The Dizzy Patient 2016 - swedish.org/media/Images/Swedish/CME1/SyllabusPDFs...6 History Onset...
Transcript of The Dizzy Patient 2016 - swedish.org/media/Images/Swedish/CME1/SyllabusPDFs...6 History Onset...
Sixth Annual Intensive Update in Neurology 0915-162016
1
The Dizzy Patient 2016
Judith White MD PhD
Medical Director Balance Center
Swedish Neuroscience Institute
Sixth Annual Intensive Update in Neurology 0915-162016
2
VESTIBULAR LABYRINTH
Sixth Annual Intensive Update in Neurology 0915-162016
3
Objectives
bull Differentiate nystagmus of benign paroxysmal
positional vertigo canal variants
bull Discuss benefits of steroids in acute vestibular
neuritis
bull Review efficacy of intratympanic therapies in
Menierersquos syndrome
bull Describe symptoms of superior semicircular
canal dehiscence
bull Summarize evidence for acute vestibular
syndrome evaluation
Sixth Annual Intensive Update in Neurology 0915-162016
4
Dizziness
bull Most common complaint over age 75
bull 8 million annual US visits
bull Chronic dizziness (gt 2 weeks) affects 16 of self-
reported US population
bull 354 of US adults aged 40 and older have vestibular
dysfunction (National Health and Nutrition Examination
Survey 2001-2004Arch Inter Med 169 (10) 938-44
2009)
Sixth Annual Intensive Update in Neurology 0915-162016
5
History
bull 70 of dizzy patients can be diagnosed with a careful
history (Gufoni 2005)
bull Equilibrium is an unconscious sensation Few
descriptions exist to describe its absence
Sixth Annual Intensive Update in Neurology 0915-162016
6
History
Onset ndash detailed description of first episode and most
recent episode
ndash Character
ndash Frequency
ndash Duration (vertigo andor milder symptoms)
ndash Associated hearing loss or tinnitus
ndash Provoking factors (noise pressure changes position change)
Sixth Annual Intensive Update in Neurology 0915-162016
7
Neuro-otology Physical Exam
bull Otologic ndash check hearing and rule out active infection or
cholesteatoma
bull Oculomotor- saccade pursuit in vertical and horizontal
planes
bull Nystagmus ndash spontaneous gaze evoked and
positionalpositioning testing (best observed using
Frenzel lenses)
bull Special testing ndash head thrust head shake
Sixth Annual Intensive Update in Neurology 0915-162016
8
Peripheral Nystagmus
bull Jerk nystagmus (slow
and fast phase)
bull Direction fixed
bull Generally beats away
from affected ear
bull Worse in the
direction of gaze that
is towards the fast
phase
Sixth Annual Intensive Update in Neurology 0915-162016
9
Benign Paroxysmal Positional Vertigo (BPPV)
bull Vertigo of sudden onset and brief duration provoked by changes in head positionndash Lying down
ndash Rolling over in bed
ndash Bending over
ndash Looking up
ndash Washing hair in shower
ndash Dentist or beauty parlor
Sixth Annual Intensive Update in Neurology 0915-162016
10
Right Posterior BPPV
Sixth Annual Intensive Update in Neurology 0915-162016
11
IncidencePrevalence of BPPV
bull Most common referred diagnosis in tertiary centers
bull 9 of randomly selected community dwelling elderly
(Oghalai JS et al 2000)
bull Incidence increases 38 with each decade of life
(Froehling 1991)
Sixth Annual Intensive Update in Neurology 0915-162016
12
Benign Positional Vertigo (BPPV)
bull Diagnosed with lateral supine head turns and Dix-
Hallpike positioning
ndash Latency
ndash Duration
ndash Fatigue
ndash Habituation
Sixth Annual Intensive Update in Neurology 0915-162016
13
DHT - Sitting
Sixth Annual Intensive Update in Neurology 0915-162016
14
DHT - HHR Orientation
Sixth Annual Intensive Update in Neurology 0915-162016
15
Dix-Hallpike to the Right
Sixth Annual Intensive Update in Neurology 0915-162016
16
Right posterior BPPV
Sixth Annual Intensive Update in Neurology 0915-162016
17
Canalith Repositioning Procedure
Sixth Annual Intensive Update in Neurology 0915-162016
18
Canalith Repositioning Procedure
Sixth Annual Intensive Update in Neurology 0915-162016
19
Canalith Repositioning Procedure
Sixth Annual Intensive Update in Neurology 0915-162016
20
Canalith Repositioning Procedure
Sixth Annual Intensive Update in Neurology 0915-162016
21
Canalith Repositioning
bull R posterior
BPPV
CPT 95992
Sixth Annual Intensive Update in Neurology 0915-162016
22
Posterior semicircular canalith repositioning
Sixth Annual Intensive Update in Neurology 0915-162016
23
Supine Positional Tests
Sixth Annual Intensive Update in Neurology 0915-162016
24
Lateral SCC-BPPV
bull Short latency horizontal nystagmus provoked by supine bilateral head turns with prolonged duration and poor fatigability
bull Uncommon (2-15 of total BPPV)
bull Two formsndash Geotropic ndash beating towards undermost ear
ndash Apogeotropic ndash beating away from undermost ear
Sixth Annual Intensive Update in Neurology 0915-162016
25
Geotropic Lateral Canal BPPV
bull Patient is positioned
first to the right lateral
position ndash nystagmus
beats right
bull Patient is then
positioned to the left
lateral position and
nystagmus reverses
and beats left
Sixth Annual Intensive Update in Neurology 0915-162016
26
Left Geotropic Lateral Semicircular Canal BPPV
Sixth Annual Intensive Update in Neurology 0915-162016
27
Repositioning Maneuvers for Geotropic Lateral Semicircular Canal (LSC) BPPV
bull Roll maneuver (Lempert Baloh)
ndash Patient rolls in 90 degree increments towards the good side
completely rolling over R LSC-BPPV repositioning shown
Sixth Annual Intensive Update in Neurology 0915-162016
28
Repositioning Maneuvers for Geotropic LSC BPPV
bull Gufoni maneuver
ndash Patient lies quickly onto good side and turns face-down to
floor for two minutes
Sixth Annual Intensive Update in Neurology 0915-162016
29
Left Apogeotropic LSC BPPV
Sixth Annual Intensive Update in Neurology 0915-162016
30
Nystagmus observed During Repositioning
bull Nystagmus should remain in the same torsional
direction as seen on DHT throughout the treatment of
posterior BPPV
bull Reversal of nystagmus direction suggests the particle
has fallen back into the canal and predicts failure
bull In Lateral BPPV nystagmus should beat towards the
good ear
Sixth Annual Intensive Update in Neurology 0915-162016
31
Posterior to Lateral canal conversion
Sixth Annual Intensive Update in Neurology 0915-162016
32
R Posterior to Lateral Canal conversion - geotropic
Sixth Annual Intensive Update in Neurology 0915-162016
33
BPPV Recurrence
bull Recurrence is common (15yr)
bull Otoconial adherence or mineralization demineralization
abnormalities may contribute to recurrence
bull Home canalith repositioning is effective (Radke 2004)
Sixth Annual Intensive Update in Neurology 0915-162016
34
Question 1
bull The most likely semicircular canal to be affected in
BPPV is
ndash 1) superior
ndash 2) lateral
ndash 3) posterior
ndash 4) combined
Sixth Annual Intensive Update in Neurology 0915-162016
35
Question 1
bull The most likely semicircular canal to be affected in
BPPV is
ndash 1) superior
ndash 2) lateral
ndash 3) posterior
ndash 4) combined
Answer 3 See references at end of lecture
Sixth Annual Intensive Update in Neurology 0915-162016
36
Acute Vestibular Syndrome
bull Rapid unilateral injury to either peripheral or central
vestibular structures produces prolonged vertigo
(days to weeks)
bull Severe vertigo nausea and vomiting spontaneous
nystagmus and postural instability
Sixth Annual Intensive Update in Neurology 0915-162016
37
Acute Vestibular Syndrome
bull 25 of patients with risk factors who present with isolated severe vertigo nystagmus and postural instability in the ER have an acute infarction of the inferior cerebellum (AICA PICA) Central imaging is suggested (Baloh 1998 NEJM)
ndash HTN diabetes smoking age gt65 Afib valvular heart disease occlusive vascular disease
Sixth Annual Intensive Update in Neurology 0915-162016
38
HINTS test
bull Head Impulse
bull Nystagmus
bull Test of Skew
ndash More sensitive than MRI in the diagnosis of acute stroke in
patients with acute vestibular syndrome with at least one risk
factor (Kattah J et al Stroke 2009)
Sixth Annual Intensive Update in Neurology 0915-162016
39
Head Impulse Test
bull No special equipment needed
bull Examiner stands in front of seated patient and
randomly moves head left and right Patient is asked to
focus on examinerrsquos nose
bull Rapid low amplitude thrust from lateral to straight
ahead causes a compensatory saccade when
performed towards the weak side
bull This finding is a permanent feature of unilateral
vestibular loss
Sixth Annual Intensive Update in Neurology 0915-162016
40
Head Impulse Test
Sixth Annual Intensive Update in Neurology 0915-162016
41
Head CT ndash Academy of Emergency Medicine 2013
bull The clinical value of head CT in isolated
dizzinessvertigo without any associated neurological
symptoms is very low
bull Less than 1 of head CTs ordered for dizziness and
vertigo have significant abnormalities and those
patients had severe headache and neurological deficits
in addition to dizziness
bull Forgoing unnecessary emergency head CTs could
save 360 million dollars annually
Sixth Annual Intensive Update in Neurology 0915-162016
42
Question 2
Head CT is not indicated for the evaluation of dizziness
and vertigo without additional neurological complaints because
1 It is too expensive
2 Less than 1 of CTs done for dizziness and vertigo are
abnormal and those patients had associated severe
neurological complaints
3 CT is the best imaging choice for the posterior fossa
4 Acoustic neuromas are well seen on CT
Sixth Annual Intensive Update in Neurology 0915-162016
43
Question 2
Head CT is not indicated for the evaluation of dizziness
and vertigo without additional neurological complaints because
1 It is too expensive
2 Less than 1 of CTs done for dizziness and vertigo are
abnormal and those patients had associated severe
neurological complaints
3 CT is the best imaging choice for the posterior fossa
4 Acoustic neuromas are well seen on CT
Answer 2 See references at end of lecture
Sixth Annual Intensive Update in Neurology 0915-162016
44
Acute Vestibular Syndrome
bull Usually peripheral and can be associated with
hearing loss (labyrinthitis)
bull Timely diagnosis improves recovery (ASAP
prednisone 1mgkgday for ten days and taper 10
mgday ndash Strupp M 2004 NEJM)
bull Avoid vestibular suppressants after 3 days and begin
vestibular rehabilitation to hasten compensation
Sixth Annual Intensive Update in Neurology 0915-162016
45
Question 3
bull Treatment for Acute Vestibular Syndrome
1) oral prednisone 1 mgkgday
2) Treat dehydration if necessary
3) Minimize vestibular suppressants after 3 days
4) Consider vestibular physical therapy
5) All of the above
Sixth Annual Intensive Update in Neurology 0915-162016
46
Question 3
bull Treatment for Acute Vestibular Syndrome
1) oral prednisone 1 mgkgday
2) Treat dehydration if necessary
3) Minimize vestibular suppressants after 3 days
4) Consider vestibular physical therapy
5) All of the above
Answer 5 See references at end of lecture
Sixth Annual Intensive Update in Neurology 0915-162016
47
Menierersquos syndrome
bull Fluctuating low frequency hearing loss
bull Tinnitus andor aural fullness
bull Vertigo lasting hours
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Normal
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Hydrops
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Menierersquos syndrome
ndash Diagnosis- Exclusion (RO acoustic)
ndash 23 of Menierersquos patients will improve spontaneously
ndash Treatment-1500 mgday low sodium triamterene375-
hydrochlorothiazide 25 vestibular suppressants (lorazepam
1 mg tablet sublingual Q 8 prn attacks) off label use
Sixth Annual Intensive Update in Neurology 0915-162016
51
Menierersquos syndrome ndash intratympanic steroids
ndash A newer treatment option for those failing medical management
ndash 90 response rate (Boleus-Aguirre MS Otology Neurotology
2008)
ndash Outcomes data confirm excellent clinical response in 3941
Menierersquos patients treated with intratympanic steroids in 2008
Sixth Annual Intensive Update in Neurology 0915-162016
52
Question 4
bull Menierersquos syndrome diagnosis is based on
1) Recurrent vertigo lasting at least 20 minutes
2) Aural fullness
3) Documented hearing loss
4) Other causes excluded
5) All of the above
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53
Question 4
bull Menierersquos syndrome diagnosis is based on
1) Recurrent vertigo lasting at least 20 minutes
2) Aural fullness
3) Documented hearing loss
4) Other causes excluded
5) All of the above
Answer 5 See references at end
Sixth Annual Intensive Update in Neurology 0915-162016
54
Superior Semicircular Canal Dehiscence (SSCD)
bull Sound andor pressure induced oscillopsia
bull CT reformatted in the plane of the superior canals
shows dehiscence of overlying bone
bull Low frequency ldquoconductive hyperacusisrdquo with elevated
bone thresholds normal reflexes and a conductive loss
Sixth Annual Intensive Update in Neurology 0915-162016
55
SSCD
bull Positive middle ear pressure or loud sound stimulates ampullofugal endolymph flow -canal activation
bull Eye movements are tonic upwards with ipsilateral intorsion
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56
SSCD
bull Valsalva against a closed glottis or jugular compression causes increased intracranial pressure and ampullopetal endolymph flow -canal inhibition
bull Eye movements are tonic downwards with ipsilateral extorsion
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57
Reconstructed left superior semicircular canal
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Sixth Annual Intensive Update in Neurology 0915-162016
59
SCHEMATIC DRAWING OF AN OTOLITHIC MACULA
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60
MACULAE OF THE UTRICLESACCULE
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61
HAIR CELLS
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62
SCANNING ELECTRON MICROGRAPH OF OTOCONIA
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63
Ocular VEMP
bull Response to loud clicks (80-110 dB) or forehead tap (Halmagyi and Curthoys 2003)
bull Contralateral oculomotor nucleus (CN III)
bull Inferior oblique muscle contraction (8 msec) detected by 5sec event triggered averaging
bull Noninverting electrode inferior to eye
bull Inverting below non-inverting electrode
bull Ground to forehead
bull Normal subjects have very low response
Sixth Annual Intensive Update in Neurology 0915-162016
64
Clinical applications
bull The o-VEMP is a sensitive test of otolithutricular
function
bull Complements vestibular test procedures that assess
semicircular canals
bull Abnormally robust responses are associated with third
window abnormalities such as semicircular canal
dehiscence
bull Serves as an excellent screening test for semicircular
canal dehiscence
Sixth Annual Intensive Update in Neurology 0915-162016
65
Question 5
bull Superior semicircular canal dehiscence
1) Causes increased ability to hear vibratory sounds
2) Improves hearing
3) Causes meningitis
4) Always needs surgical repair
Sixth Annual Intensive Update in Neurology 0915-162016
66
Question 5
bull Superior semicircular canal dehiscence
1) Causes increased ability to hear vibratory sounds
2) Improves hearing
3) Causes meningitis
4) Always needs surgical repair
Answer 1 References at end of lecture
Sixth Annual Intensive Update in Neurology 0915-162016
67
Enlarged vestibular aqueduct
bull Axial temporal bone CT showing right temporal bone with enlarged vestibular aqueduct (arrow)
bull Vestibular symptoms accompany progressive sensorineural hearing loss in 4-50 of patients in selected series
Sixth Annual Intensive Update in Neurology 0915-162016
68
Migraine
bull Family or personal history of migraine
bull 25 of migraine patients experience spontaneous vertigo (Baloh 1997)
bull Neuhauser criteria(2001) have been used to construct structured diagnostic interviews (Furman 2003)
bull Not always associated with headache
bull Motion sensitivity
bull Medication responsive
Sixth Annual Intensive Update in Neurology 0915-162016
69
ICHD-III beta Criteria for Vestibular Migraine
bull A At least five attacks fulfilling criteria C and D
bull B A current or past history of 11 migraine without aura or 12 migraine with aura
bull C Vestibular symptoms of moderate or severe intensity lasting between 5 minutes and 72 hours
bull D At least 50 associated with one of the following migrainous features
ndash 1 Headaches
ndash 2 Photophobia and phonophobia
ndash 3 Visual Aura
bull E Not better accounted for by another vestibular disorder
Sixth Annual Intensive Update in Neurology 0915-162016
70
Incidence of vertigo in MS
bull True vertigo (the perception of environmental
movement typically rotational) occurs in about
20 of MS patients and is the presenting
manifestation in 5
bull Up to 78 of MS patients report abnormalities
in balance at some point in their illness
Frohman et al Multiple Sclerosis 2003 9250
Sixth Annual Intensive Update in Neurology 0915-162016
71
Vertigo in MS due to demyelination
bull The most common neuroanatomic locations are the
root entry zone of CN VIII (giving absent calorics but
normal hearing and fixation suppression) and the
medial vestibular nucleus
bull Usually treated with steroids and vestibular
suppressants
Brandt 1993 Gass 1998 Thomke 1999
Sixth Annual Intensive Update in Neurology 0915-162016
72
Vertigo in MS
Frohmanrsquos large series (1153) reported that 52 of MS patients with acute vertigo had benign paroxysmal positional vertigo while 32 had new brainstem lesions
To avoid misdiagnoses and inappropriate treatment interventions alternative etiologies should be considered in all acute vertigo episodes in MS
E M Frohman Neurology 2000 and Multiple Sclerosis 2003
Sixth Annual Intensive Update in Neurology 0915-162016
73
Vestibular compensation
bull Acute unilateral or bilateral vestibular loss will prompt compensation thru central cervico-ocular visual and proprioceptive pathways
bull Unilateral loss may require several weeks for compensation to occur and activity especially head movements hastens compensation
bull Vestibular suppressant medication prolongs compensation
Sixth Annual Intensive Update in Neurology 0915-162016
74
Vestibular Rehabilitation
bull Performed by certified physical therapists many sites (Epic order vestibular PT) or check Vestibular Disorders of America website for providers
bull Assessment and treatment of benign positional vertigo
bull Directed rehab programs for central andor peripheral vestibular compensationndash Unilateral vestibulopathies
ndash Bilateral vestibular hypofunction
ndash Mutifactorial balance deficits and fall risk assessment
Sixth Annual Intensive Update in Neurology 0915-162016
75
Summary of whatrsquos new in peripheral vestibular disorders
bull Lateral semicircular canal benign paroxysmal positional vertigo presents with horizontal positioning nystagmus
bull Oral steroids appear to improve outcome in acute vestibular neuritis
bull Intratympanic steroids offer promise in Menierersquos syndrome
bull Superior semicircular canal dehiscence causes pressure and noise induced dizziness VEMP offers diagnostic information
Sixth Annual Intensive Update in Neurology 0915-162016
76
References
BPPVndash White J Benign paroxysmal positional vertigo Cleveland Clinic Journal of
Medicine 71(9)722-728 2004
ndash White J Coale K Catalano P Oas J Diagnosis and management of lateral semicircular canal benign positional vertigo Otolaryngology-Head and Neck Surgery 133 278-284 2005
ndash White J Savvides P Cherian N Oas J Canalith repositioning for benign paroxysmal positional vertigo Otology and Neurotology 26 704-710 2005
ndash White J Oas J Diagnosis and management of lateral semicircular canal conversions during particle repositioning therapy Laryngoscope 115(10) 1895-1897 2005
ndash Acute Vestibular Syndrome Hotson JR Baloh RW N Engl J Med 1998 Sep 3339(10)680-5
ndash Methylprednisolone valacyclovir or the combination for vestibular neuritis Strupp M Zingler VC Arbusow V Niklas D Maag KP Dieterich M Bense S Theil D Jahn K Brandt T N Engl J Med 2004 Jul 22351(4)354-61
ndash Intratympanic steroids for Meacuteniegraveres disease or syndrome Phillips JS Westerberg BCochrane Database Syst Rev 2011 Jul 6(7)CD008514 Review
ndash Sound- andor pressure-induced vertigo due to bone dehiscence of the superior semicircular canal Minor LB Solomon D Zinreich JS Zee DSArch Otolaryngol Head Neck Surg 1998 Mar124(3)249-58
Sixth Annual Intensive Update in Neurology 0915-162016
2
VESTIBULAR LABYRINTH
Sixth Annual Intensive Update in Neurology 0915-162016
3
Objectives
bull Differentiate nystagmus of benign paroxysmal
positional vertigo canal variants
bull Discuss benefits of steroids in acute vestibular
neuritis
bull Review efficacy of intratympanic therapies in
Menierersquos syndrome
bull Describe symptoms of superior semicircular
canal dehiscence
bull Summarize evidence for acute vestibular
syndrome evaluation
Sixth Annual Intensive Update in Neurology 0915-162016
4
Dizziness
bull Most common complaint over age 75
bull 8 million annual US visits
bull Chronic dizziness (gt 2 weeks) affects 16 of self-
reported US population
bull 354 of US adults aged 40 and older have vestibular
dysfunction (National Health and Nutrition Examination
Survey 2001-2004Arch Inter Med 169 (10) 938-44
2009)
Sixth Annual Intensive Update in Neurology 0915-162016
5
History
bull 70 of dizzy patients can be diagnosed with a careful
history (Gufoni 2005)
bull Equilibrium is an unconscious sensation Few
descriptions exist to describe its absence
Sixth Annual Intensive Update in Neurology 0915-162016
6
History
Onset ndash detailed description of first episode and most
recent episode
ndash Character
ndash Frequency
ndash Duration (vertigo andor milder symptoms)
ndash Associated hearing loss or tinnitus
ndash Provoking factors (noise pressure changes position change)
Sixth Annual Intensive Update in Neurology 0915-162016
7
Neuro-otology Physical Exam
bull Otologic ndash check hearing and rule out active infection or
cholesteatoma
bull Oculomotor- saccade pursuit in vertical and horizontal
planes
bull Nystagmus ndash spontaneous gaze evoked and
positionalpositioning testing (best observed using
Frenzel lenses)
bull Special testing ndash head thrust head shake
Sixth Annual Intensive Update in Neurology 0915-162016
8
Peripheral Nystagmus
bull Jerk nystagmus (slow
and fast phase)
bull Direction fixed
bull Generally beats away
from affected ear
bull Worse in the
direction of gaze that
is towards the fast
phase
Sixth Annual Intensive Update in Neurology 0915-162016
9
Benign Paroxysmal Positional Vertigo (BPPV)
bull Vertigo of sudden onset and brief duration provoked by changes in head positionndash Lying down
ndash Rolling over in bed
ndash Bending over
ndash Looking up
ndash Washing hair in shower
ndash Dentist or beauty parlor
Sixth Annual Intensive Update in Neurology 0915-162016
10
Right Posterior BPPV
Sixth Annual Intensive Update in Neurology 0915-162016
11
IncidencePrevalence of BPPV
bull Most common referred diagnosis in tertiary centers
bull 9 of randomly selected community dwelling elderly
(Oghalai JS et al 2000)
bull Incidence increases 38 with each decade of life
(Froehling 1991)
Sixth Annual Intensive Update in Neurology 0915-162016
12
Benign Positional Vertigo (BPPV)
bull Diagnosed with lateral supine head turns and Dix-
Hallpike positioning
ndash Latency
ndash Duration
ndash Fatigue
ndash Habituation
Sixth Annual Intensive Update in Neurology 0915-162016
13
DHT - Sitting
Sixth Annual Intensive Update in Neurology 0915-162016
14
DHT - HHR Orientation
Sixth Annual Intensive Update in Neurology 0915-162016
15
Dix-Hallpike to the Right
Sixth Annual Intensive Update in Neurology 0915-162016
16
Right posterior BPPV
Sixth Annual Intensive Update in Neurology 0915-162016
17
Canalith Repositioning Procedure
Sixth Annual Intensive Update in Neurology 0915-162016
18
Canalith Repositioning Procedure
Sixth Annual Intensive Update in Neurology 0915-162016
19
Canalith Repositioning Procedure
Sixth Annual Intensive Update in Neurology 0915-162016
20
Canalith Repositioning Procedure
Sixth Annual Intensive Update in Neurology 0915-162016
21
Canalith Repositioning
bull R posterior
BPPV
CPT 95992
Sixth Annual Intensive Update in Neurology 0915-162016
22
Posterior semicircular canalith repositioning
Sixth Annual Intensive Update in Neurology 0915-162016
23
Supine Positional Tests
Sixth Annual Intensive Update in Neurology 0915-162016
24
Lateral SCC-BPPV
bull Short latency horizontal nystagmus provoked by supine bilateral head turns with prolonged duration and poor fatigability
bull Uncommon (2-15 of total BPPV)
bull Two formsndash Geotropic ndash beating towards undermost ear
ndash Apogeotropic ndash beating away from undermost ear
Sixth Annual Intensive Update in Neurology 0915-162016
25
Geotropic Lateral Canal BPPV
bull Patient is positioned
first to the right lateral
position ndash nystagmus
beats right
bull Patient is then
positioned to the left
lateral position and
nystagmus reverses
and beats left
Sixth Annual Intensive Update in Neurology 0915-162016
26
Left Geotropic Lateral Semicircular Canal BPPV
Sixth Annual Intensive Update in Neurology 0915-162016
27
Repositioning Maneuvers for Geotropic Lateral Semicircular Canal (LSC) BPPV
bull Roll maneuver (Lempert Baloh)
ndash Patient rolls in 90 degree increments towards the good side
completely rolling over R LSC-BPPV repositioning shown
Sixth Annual Intensive Update in Neurology 0915-162016
28
Repositioning Maneuvers for Geotropic LSC BPPV
bull Gufoni maneuver
ndash Patient lies quickly onto good side and turns face-down to
floor for two minutes
Sixth Annual Intensive Update in Neurology 0915-162016
29
Left Apogeotropic LSC BPPV
Sixth Annual Intensive Update in Neurology 0915-162016
30
Nystagmus observed During Repositioning
bull Nystagmus should remain in the same torsional
direction as seen on DHT throughout the treatment of
posterior BPPV
bull Reversal of nystagmus direction suggests the particle
has fallen back into the canal and predicts failure
bull In Lateral BPPV nystagmus should beat towards the
good ear
Sixth Annual Intensive Update in Neurology 0915-162016
31
Posterior to Lateral canal conversion
Sixth Annual Intensive Update in Neurology 0915-162016
32
R Posterior to Lateral Canal conversion - geotropic
Sixth Annual Intensive Update in Neurology 0915-162016
33
BPPV Recurrence
bull Recurrence is common (15yr)
bull Otoconial adherence or mineralization demineralization
abnormalities may contribute to recurrence
bull Home canalith repositioning is effective (Radke 2004)
Sixth Annual Intensive Update in Neurology 0915-162016
34
Question 1
bull The most likely semicircular canal to be affected in
BPPV is
ndash 1) superior
ndash 2) lateral
ndash 3) posterior
ndash 4) combined
Sixth Annual Intensive Update in Neurology 0915-162016
35
Question 1
bull The most likely semicircular canal to be affected in
BPPV is
ndash 1) superior
ndash 2) lateral
ndash 3) posterior
ndash 4) combined
Answer 3 See references at end of lecture
Sixth Annual Intensive Update in Neurology 0915-162016
36
Acute Vestibular Syndrome
bull Rapid unilateral injury to either peripheral or central
vestibular structures produces prolonged vertigo
(days to weeks)
bull Severe vertigo nausea and vomiting spontaneous
nystagmus and postural instability
Sixth Annual Intensive Update in Neurology 0915-162016
37
Acute Vestibular Syndrome
bull 25 of patients with risk factors who present with isolated severe vertigo nystagmus and postural instability in the ER have an acute infarction of the inferior cerebellum (AICA PICA) Central imaging is suggested (Baloh 1998 NEJM)
ndash HTN diabetes smoking age gt65 Afib valvular heart disease occlusive vascular disease
Sixth Annual Intensive Update in Neurology 0915-162016
38
HINTS test
bull Head Impulse
bull Nystagmus
bull Test of Skew
ndash More sensitive than MRI in the diagnosis of acute stroke in
patients with acute vestibular syndrome with at least one risk
factor (Kattah J et al Stroke 2009)
Sixth Annual Intensive Update in Neurology 0915-162016
39
Head Impulse Test
bull No special equipment needed
bull Examiner stands in front of seated patient and
randomly moves head left and right Patient is asked to
focus on examinerrsquos nose
bull Rapid low amplitude thrust from lateral to straight
ahead causes a compensatory saccade when
performed towards the weak side
bull This finding is a permanent feature of unilateral
vestibular loss
Sixth Annual Intensive Update in Neurology 0915-162016
40
Head Impulse Test
Sixth Annual Intensive Update in Neurology 0915-162016
41
Head CT ndash Academy of Emergency Medicine 2013
bull The clinical value of head CT in isolated
dizzinessvertigo without any associated neurological
symptoms is very low
bull Less than 1 of head CTs ordered for dizziness and
vertigo have significant abnormalities and those
patients had severe headache and neurological deficits
in addition to dizziness
bull Forgoing unnecessary emergency head CTs could
save 360 million dollars annually
Sixth Annual Intensive Update in Neurology 0915-162016
42
Question 2
Head CT is not indicated for the evaluation of dizziness
and vertigo without additional neurological complaints because
1 It is too expensive
2 Less than 1 of CTs done for dizziness and vertigo are
abnormal and those patients had associated severe
neurological complaints
3 CT is the best imaging choice for the posterior fossa
4 Acoustic neuromas are well seen on CT
Sixth Annual Intensive Update in Neurology 0915-162016
43
Question 2
Head CT is not indicated for the evaluation of dizziness
and vertigo without additional neurological complaints because
1 It is too expensive
2 Less than 1 of CTs done for dizziness and vertigo are
abnormal and those patients had associated severe
neurological complaints
3 CT is the best imaging choice for the posterior fossa
4 Acoustic neuromas are well seen on CT
Answer 2 See references at end of lecture
Sixth Annual Intensive Update in Neurology 0915-162016
44
Acute Vestibular Syndrome
bull Usually peripheral and can be associated with
hearing loss (labyrinthitis)
bull Timely diagnosis improves recovery (ASAP
prednisone 1mgkgday for ten days and taper 10
mgday ndash Strupp M 2004 NEJM)
bull Avoid vestibular suppressants after 3 days and begin
vestibular rehabilitation to hasten compensation
Sixth Annual Intensive Update in Neurology 0915-162016
45
Question 3
bull Treatment for Acute Vestibular Syndrome
1) oral prednisone 1 mgkgday
2) Treat dehydration if necessary
3) Minimize vestibular suppressants after 3 days
4) Consider vestibular physical therapy
5) All of the above
Sixth Annual Intensive Update in Neurology 0915-162016
46
Question 3
bull Treatment for Acute Vestibular Syndrome
1) oral prednisone 1 mgkgday
2) Treat dehydration if necessary
3) Minimize vestibular suppressants after 3 days
4) Consider vestibular physical therapy
5) All of the above
Answer 5 See references at end of lecture
Sixth Annual Intensive Update in Neurology 0915-162016
47
Menierersquos syndrome
bull Fluctuating low frequency hearing loss
bull Tinnitus andor aural fullness
bull Vertigo lasting hours
Sixth Annual Intensive Update in Neurology 0915-162016
48
Normal
Sixth Annual Intensive Update in Neurology 0915-162016
49
Hydrops
Sixth Annual Intensive Update in Neurology 0915-162016
50
Menierersquos syndrome
ndash Diagnosis- Exclusion (RO acoustic)
ndash 23 of Menierersquos patients will improve spontaneously
ndash Treatment-1500 mgday low sodium triamterene375-
hydrochlorothiazide 25 vestibular suppressants (lorazepam
1 mg tablet sublingual Q 8 prn attacks) off label use
Sixth Annual Intensive Update in Neurology 0915-162016
51
Menierersquos syndrome ndash intratympanic steroids
ndash A newer treatment option for those failing medical management
ndash 90 response rate (Boleus-Aguirre MS Otology Neurotology
2008)
ndash Outcomes data confirm excellent clinical response in 3941
Menierersquos patients treated with intratympanic steroids in 2008
Sixth Annual Intensive Update in Neurology 0915-162016
52
Question 4
bull Menierersquos syndrome diagnosis is based on
1) Recurrent vertigo lasting at least 20 minutes
2) Aural fullness
3) Documented hearing loss
4) Other causes excluded
5) All of the above
Sixth Annual Intensive Update in Neurology 0915-162016
53
Question 4
bull Menierersquos syndrome diagnosis is based on
1) Recurrent vertigo lasting at least 20 minutes
2) Aural fullness
3) Documented hearing loss
4) Other causes excluded
5) All of the above
Answer 5 See references at end
Sixth Annual Intensive Update in Neurology 0915-162016
54
Superior Semicircular Canal Dehiscence (SSCD)
bull Sound andor pressure induced oscillopsia
bull CT reformatted in the plane of the superior canals
shows dehiscence of overlying bone
bull Low frequency ldquoconductive hyperacusisrdquo with elevated
bone thresholds normal reflexes and a conductive loss
Sixth Annual Intensive Update in Neurology 0915-162016
55
SSCD
bull Positive middle ear pressure or loud sound stimulates ampullofugal endolymph flow -canal activation
bull Eye movements are tonic upwards with ipsilateral intorsion
Sixth Annual Intensive Update in Neurology 0915-162016
56
SSCD
bull Valsalva against a closed glottis or jugular compression causes increased intracranial pressure and ampullopetal endolymph flow -canal inhibition
bull Eye movements are tonic downwards with ipsilateral extorsion
Sixth Annual Intensive Update in Neurology 0915-162016
57
Reconstructed left superior semicircular canal
Sixth Annual Intensive Update in Neurology 0915-162016
58
Sixth Annual Intensive Update in Neurology 0915-162016
59
SCHEMATIC DRAWING OF AN OTOLITHIC MACULA
Sixth Annual Intensive Update in Neurology 0915-162016
60
MACULAE OF THE UTRICLESACCULE
Sixth Annual Intensive Update in Neurology 0915-162016
61
HAIR CELLS
Sixth Annual Intensive Update in Neurology 0915-162016
62
SCANNING ELECTRON MICROGRAPH OF OTOCONIA
Sixth Annual Intensive Update in Neurology 0915-162016
63
Ocular VEMP
bull Response to loud clicks (80-110 dB) or forehead tap (Halmagyi and Curthoys 2003)
bull Contralateral oculomotor nucleus (CN III)
bull Inferior oblique muscle contraction (8 msec) detected by 5sec event triggered averaging
bull Noninverting electrode inferior to eye
bull Inverting below non-inverting electrode
bull Ground to forehead
bull Normal subjects have very low response
Sixth Annual Intensive Update in Neurology 0915-162016
64
Clinical applications
bull The o-VEMP is a sensitive test of otolithutricular
function
bull Complements vestibular test procedures that assess
semicircular canals
bull Abnormally robust responses are associated with third
window abnormalities such as semicircular canal
dehiscence
bull Serves as an excellent screening test for semicircular
canal dehiscence
Sixth Annual Intensive Update in Neurology 0915-162016
65
Question 5
bull Superior semicircular canal dehiscence
1) Causes increased ability to hear vibratory sounds
2) Improves hearing
3) Causes meningitis
4) Always needs surgical repair
Sixth Annual Intensive Update in Neurology 0915-162016
66
Question 5
bull Superior semicircular canal dehiscence
1) Causes increased ability to hear vibratory sounds
2) Improves hearing
3) Causes meningitis
4) Always needs surgical repair
Answer 1 References at end of lecture
Sixth Annual Intensive Update in Neurology 0915-162016
67
Enlarged vestibular aqueduct
bull Axial temporal bone CT showing right temporal bone with enlarged vestibular aqueduct (arrow)
bull Vestibular symptoms accompany progressive sensorineural hearing loss in 4-50 of patients in selected series
Sixth Annual Intensive Update in Neurology 0915-162016
68
Migraine
bull Family or personal history of migraine
bull 25 of migraine patients experience spontaneous vertigo (Baloh 1997)
bull Neuhauser criteria(2001) have been used to construct structured diagnostic interviews (Furman 2003)
bull Not always associated with headache
bull Motion sensitivity
bull Medication responsive
Sixth Annual Intensive Update in Neurology 0915-162016
69
ICHD-III beta Criteria for Vestibular Migraine
bull A At least five attacks fulfilling criteria C and D
bull B A current or past history of 11 migraine without aura or 12 migraine with aura
bull C Vestibular symptoms of moderate or severe intensity lasting between 5 minutes and 72 hours
bull D At least 50 associated with one of the following migrainous features
ndash 1 Headaches
ndash 2 Photophobia and phonophobia
ndash 3 Visual Aura
bull E Not better accounted for by another vestibular disorder
Sixth Annual Intensive Update in Neurology 0915-162016
70
Incidence of vertigo in MS
bull True vertigo (the perception of environmental
movement typically rotational) occurs in about
20 of MS patients and is the presenting
manifestation in 5
bull Up to 78 of MS patients report abnormalities
in balance at some point in their illness
Frohman et al Multiple Sclerosis 2003 9250
Sixth Annual Intensive Update in Neurology 0915-162016
71
Vertigo in MS due to demyelination
bull The most common neuroanatomic locations are the
root entry zone of CN VIII (giving absent calorics but
normal hearing and fixation suppression) and the
medial vestibular nucleus
bull Usually treated with steroids and vestibular
suppressants
Brandt 1993 Gass 1998 Thomke 1999
Sixth Annual Intensive Update in Neurology 0915-162016
72
Vertigo in MS
Frohmanrsquos large series (1153) reported that 52 of MS patients with acute vertigo had benign paroxysmal positional vertigo while 32 had new brainstem lesions
To avoid misdiagnoses and inappropriate treatment interventions alternative etiologies should be considered in all acute vertigo episodes in MS
E M Frohman Neurology 2000 and Multiple Sclerosis 2003
Sixth Annual Intensive Update in Neurology 0915-162016
73
Vestibular compensation
bull Acute unilateral or bilateral vestibular loss will prompt compensation thru central cervico-ocular visual and proprioceptive pathways
bull Unilateral loss may require several weeks for compensation to occur and activity especially head movements hastens compensation
bull Vestibular suppressant medication prolongs compensation
Sixth Annual Intensive Update in Neurology 0915-162016
74
Vestibular Rehabilitation
bull Performed by certified physical therapists many sites (Epic order vestibular PT) or check Vestibular Disorders of America website for providers
bull Assessment and treatment of benign positional vertigo
bull Directed rehab programs for central andor peripheral vestibular compensationndash Unilateral vestibulopathies
ndash Bilateral vestibular hypofunction
ndash Mutifactorial balance deficits and fall risk assessment
Sixth Annual Intensive Update in Neurology 0915-162016
75
Summary of whatrsquos new in peripheral vestibular disorders
bull Lateral semicircular canal benign paroxysmal positional vertigo presents with horizontal positioning nystagmus
bull Oral steroids appear to improve outcome in acute vestibular neuritis
bull Intratympanic steroids offer promise in Menierersquos syndrome
bull Superior semicircular canal dehiscence causes pressure and noise induced dizziness VEMP offers diagnostic information
Sixth Annual Intensive Update in Neurology 0915-162016
76
References
BPPVndash White J Benign paroxysmal positional vertigo Cleveland Clinic Journal of
Medicine 71(9)722-728 2004
ndash White J Coale K Catalano P Oas J Diagnosis and management of lateral semicircular canal benign positional vertigo Otolaryngology-Head and Neck Surgery 133 278-284 2005
ndash White J Savvides P Cherian N Oas J Canalith repositioning for benign paroxysmal positional vertigo Otology and Neurotology 26 704-710 2005
ndash White J Oas J Diagnosis and management of lateral semicircular canal conversions during particle repositioning therapy Laryngoscope 115(10) 1895-1897 2005
ndash Acute Vestibular Syndrome Hotson JR Baloh RW N Engl J Med 1998 Sep 3339(10)680-5
ndash Methylprednisolone valacyclovir or the combination for vestibular neuritis Strupp M Zingler VC Arbusow V Niklas D Maag KP Dieterich M Bense S Theil D Jahn K Brandt T N Engl J Med 2004 Jul 22351(4)354-61
ndash Intratympanic steroids for Meacuteniegraveres disease or syndrome Phillips JS Westerberg BCochrane Database Syst Rev 2011 Jul 6(7)CD008514 Review
ndash Sound- andor pressure-induced vertigo due to bone dehiscence of the superior semicircular canal Minor LB Solomon D Zinreich JS Zee DSArch Otolaryngol Head Neck Surg 1998 Mar124(3)249-58
Sixth Annual Intensive Update in Neurology 0915-162016
3
Objectives
bull Differentiate nystagmus of benign paroxysmal
positional vertigo canal variants
bull Discuss benefits of steroids in acute vestibular
neuritis
bull Review efficacy of intratympanic therapies in
Menierersquos syndrome
bull Describe symptoms of superior semicircular
canal dehiscence
bull Summarize evidence for acute vestibular
syndrome evaluation
Sixth Annual Intensive Update in Neurology 0915-162016
4
Dizziness
bull Most common complaint over age 75
bull 8 million annual US visits
bull Chronic dizziness (gt 2 weeks) affects 16 of self-
reported US population
bull 354 of US adults aged 40 and older have vestibular
dysfunction (National Health and Nutrition Examination
Survey 2001-2004Arch Inter Med 169 (10) 938-44
2009)
Sixth Annual Intensive Update in Neurology 0915-162016
5
History
bull 70 of dizzy patients can be diagnosed with a careful
history (Gufoni 2005)
bull Equilibrium is an unconscious sensation Few
descriptions exist to describe its absence
Sixth Annual Intensive Update in Neurology 0915-162016
6
History
Onset ndash detailed description of first episode and most
recent episode
ndash Character
ndash Frequency
ndash Duration (vertigo andor milder symptoms)
ndash Associated hearing loss or tinnitus
ndash Provoking factors (noise pressure changes position change)
Sixth Annual Intensive Update in Neurology 0915-162016
7
Neuro-otology Physical Exam
bull Otologic ndash check hearing and rule out active infection or
cholesteatoma
bull Oculomotor- saccade pursuit in vertical and horizontal
planes
bull Nystagmus ndash spontaneous gaze evoked and
positionalpositioning testing (best observed using
Frenzel lenses)
bull Special testing ndash head thrust head shake
Sixth Annual Intensive Update in Neurology 0915-162016
8
Peripheral Nystagmus
bull Jerk nystagmus (slow
and fast phase)
bull Direction fixed
bull Generally beats away
from affected ear
bull Worse in the
direction of gaze that
is towards the fast
phase
Sixth Annual Intensive Update in Neurology 0915-162016
9
Benign Paroxysmal Positional Vertigo (BPPV)
bull Vertigo of sudden onset and brief duration provoked by changes in head positionndash Lying down
ndash Rolling over in bed
ndash Bending over
ndash Looking up
ndash Washing hair in shower
ndash Dentist or beauty parlor
Sixth Annual Intensive Update in Neurology 0915-162016
10
Right Posterior BPPV
Sixth Annual Intensive Update in Neurology 0915-162016
11
IncidencePrevalence of BPPV
bull Most common referred diagnosis in tertiary centers
bull 9 of randomly selected community dwelling elderly
(Oghalai JS et al 2000)
bull Incidence increases 38 with each decade of life
(Froehling 1991)
Sixth Annual Intensive Update in Neurology 0915-162016
12
Benign Positional Vertigo (BPPV)
bull Diagnosed with lateral supine head turns and Dix-
Hallpike positioning
ndash Latency
ndash Duration
ndash Fatigue
ndash Habituation
Sixth Annual Intensive Update in Neurology 0915-162016
13
DHT - Sitting
Sixth Annual Intensive Update in Neurology 0915-162016
14
DHT - HHR Orientation
Sixth Annual Intensive Update in Neurology 0915-162016
15
Dix-Hallpike to the Right
Sixth Annual Intensive Update in Neurology 0915-162016
16
Right posterior BPPV
Sixth Annual Intensive Update in Neurology 0915-162016
17
Canalith Repositioning Procedure
Sixth Annual Intensive Update in Neurology 0915-162016
18
Canalith Repositioning Procedure
Sixth Annual Intensive Update in Neurology 0915-162016
19
Canalith Repositioning Procedure
Sixth Annual Intensive Update in Neurology 0915-162016
20
Canalith Repositioning Procedure
Sixth Annual Intensive Update in Neurology 0915-162016
21
Canalith Repositioning
bull R posterior
BPPV
CPT 95992
Sixth Annual Intensive Update in Neurology 0915-162016
22
Posterior semicircular canalith repositioning
Sixth Annual Intensive Update in Neurology 0915-162016
23
Supine Positional Tests
Sixth Annual Intensive Update in Neurology 0915-162016
24
Lateral SCC-BPPV
bull Short latency horizontal nystagmus provoked by supine bilateral head turns with prolonged duration and poor fatigability
bull Uncommon (2-15 of total BPPV)
bull Two formsndash Geotropic ndash beating towards undermost ear
ndash Apogeotropic ndash beating away from undermost ear
Sixth Annual Intensive Update in Neurology 0915-162016
25
Geotropic Lateral Canal BPPV
bull Patient is positioned
first to the right lateral
position ndash nystagmus
beats right
bull Patient is then
positioned to the left
lateral position and
nystagmus reverses
and beats left
Sixth Annual Intensive Update in Neurology 0915-162016
26
Left Geotropic Lateral Semicircular Canal BPPV
Sixth Annual Intensive Update in Neurology 0915-162016
27
Repositioning Maneuvers for Geotropic Lateral Semicircular Canal (LSC) BPPV
bull Roll maneuver (Lempert Baloh)
ndash Patient rolls in 90 degree increments towards the good side
completely rolling over R LSC-BPPV repositioning shown
Sixth Annual Intensive Update in Neurology 0915-162016
28
Repositioning Maneuvers for Geotropic LSC BPPV
bull Gufoni maneuver
ndash Patient lies quickly onto good side and turns face-down to
floor for two minutes
Sixth Annual Intensive Update in Neurology 0915-162016
29
Left Apogeotropic LSC BPPV
Sixth Annual Intensive Update in Neurology 0915-162016
30
Nystagmus observed During Repositioning
bull Nystagmus should remain in the same torsional
direction as seen on DHT throughout the treatment of
posterior BPPV
bull Reversal of nystagmus direction suggests the particle
has fallen back into the canal and predicts failure
bull In Lateral BPPV nystagmus should beat towards the
good ear
Sixth Annual Intensive Update in Neurology 0915-162016
31
Posterior to Lateral canal conversion
Sixth Annual Intensive Update in Neurology 0915-162016
32
R Posterior to Lateral Canal conversion - geotropic
Sixth Annual Intensive Update in Neurology 0915-162016
33
BPPV Recurrence
bull Recurrence is common (15yr)
bull Otoconial adherence or mineralization demineralization
abnormalities may contribute to recurrence
bull Home canalith repositioning is effective (Radke 2004)
Sixth Annual Intensive Update in Neurology 0915-162016
34
Question 1
bull The most likely semicircular canal to be affected in
BPPV is
ndash 1) superior
ndash 2) lateral
ndash 3) posterior
ndash 4) combined
Sixth Annual Intensive Update in Neurology 0915-162016
35
Question 1
bull The most likely semicircular canal to be affected in
BPPV is
ndash 1) superior
ndash 2) lateral
ndash 3) posterior
ndash 4) combined
Answer 3 See references at end of lecture
Sixth Annual Intensive Update in Neurology 0915-162016
36
Acute Vestibular Syndrome
bull Rapid unilateral injury to either peripheral or central
vestibular structures produces prolonged vertigo
(days to weeks)
bull Severe vertigo nausea and vomiting spontaneous
nystagmus and postural instability
Sixth Annual Intensive Update in Neurology 0915-162016
37
Acute Vestibular Syndrome
bull 25 of patients with risk factors who present with isolated severe vertigo nystagmus and postural instability in the ER have an acute infarction of the inferior cerebellum (AICA PICA) Central imaging is suggested (Baloh 1998 NEJM)
ndash HTN diabetes smoking age gt65 Afib valvular heart disease occlusive vascular disease
Sixth Annual Intensive Update in Neurology 0915-162016
38
HINTS test
bull Head Impulse
bull Nystagmus
bull Test of Skew
ndash More sensitive than MRI in the diagnosis of acute stroke in
patients with acute vestibular syndrome with at least one risk
factor (Kattah J et al Stroke 2009)
Sixth Annual Intensive Update in Neurology 0915-162016
39
Head Impulse Test
bull No special equipment needed
bull Examiner stands in front of seated patient and
randomly moves head left and right Patient is asked to
focus on examinerrsquos nose
bull Rapid low amplitude thrust from lateral to straight
ahead causes a compensatory saccade when
performed towards the weak side
bull This finding is a permanent feature of unilateral
vestibular loss
Sixth Annual Intensive Update in Neurology 0915-162016
40
Head Impulse Test
Sixth Annual Intensive Update in Neurology 0915-162016
41
Head CT ndash Academy of Emergency Medicine 2013
bull The clinical value of head CT in isolated
dizzinessvertigo without any associated neurological
symptoms is very low
bull Less than 1 of head CTs ordered for dizziness and
vertigo have significant abnormalities and those
patients had severe headache and neurological deficits
in addition to dizziness
bull Forgoing unnecessary emergency head CTs could
save 360 million dollars annually
Sixth Annual Intensive Update in Neurology 0915-162016
42
Question 2
Head CT is not indicated for the evaluation of dizziness
and vertigo without additional neurological complaints because
1 It is too expensive
2 Less than 1 of CTs done for dizziness and vertigo are
abnormal and those patients had associated severe
neurological complaints
3 CT is the best imaging choice for the posterior fossa
4 Acoustic neuromas are well seen on CT
Sixth Annual Intensive Update in Neurology 0915-162016
43
Question 2
Head CT is not indicated for the evaluation of dizziness
and vertigo without additional neurological complaints because
1 It is too expensive
2 Less than 1 of CTs done for dizziness and vertigo are
abnormal and those patients had associated severe
neurological complaints
3 CT is the best imaging choice for the posterior fossa
4 Acoustic neuromas are well seen on CT
Answer 2 See references at end of lecture
Sixth Annual Intensive Update in Neurology 0915-162016
44
Acute Vestibular Syndrome
bull Usually peripheral and can be associated with
hearing loss (labyrinthitis)
bull Timely diagnosis improves recovery (ASAP
prednisone 1mgkgday for ten days and taper 10
mgday ndash Strupp M 2004 NEJM)
bull Avoid vestibular suppressants after 3 days and begin
vestibular rehabilitation to hasten compensation
Sixth Annual Intensive Update in Neurology 0915-162016
45
Question 3
bull Treatment for Acute Vestibular Syndrome
1) oral prednisone 1 mgkgday
2) Treat dehydration if necessary
3) Minimize vestibular suppressants after 3 days
4) Consider vestibular physical therapy
5) All of the above
Sixth Annual Intensive Update in Neurology 0915-162016
46
Question 3
bull Treatment for Acute Vestibular Syndrome
1) oral prednisone 1 mgkgday
2) Treat dehydration if necessary
3) Minimize vestibular suppressants after 3 days
4) Consider vestibular physical therapy
5) All of the above
Answer 5 See references at end of lecture
Sixth Annual Intensive Update in Neurology 0915-162016
47
Menierersquos syndrome
bull Fluctuating low frequency hearing loss
bull Tinnitus andor aural fullness
bull Vertigo lasting hours
Sixth Annual Intensive Update in Neurology 0915-162016
48
Normal
Sixth Annual Intensive Update in Neurology 0915-162016
49
Hydrops
Sixth Annual Intensive Update in Neurology 0915-162016
50
Menierersquos syndrome
ndash Diagnosis- Exclusion (RO acoustic)
ndash 23 of Menierersquos patients will improve spontaneously
ndash Treatment-1500 mgday low sodium triamterene375-
hydrochlorothiazide 25 vestibular suppressants (lorazepam
1 mg tablet sublingual Q 8 prn attacks) off label use
Sixth Annual Intensive Update in Neurology 0915-162016
51
Menierersquos syndrome ndash intratympanic steroids
ndash A newer treatment option for those failing medical management
ndash 90 response rate (Boleus-Aguirre MS Otology Neurotology
2008)
ndash Outcomes data confirm excellent clinical response in 3941
Menierersquos patients treated with intratympanic steroids in 2008
Sixth Annual Intensive Update in Neurology 0915-162016
52
Question 4
bull Menierersquos syndrome diagnosis is based on
1) Recurrent vertigo lasting at least 20 minutes
2) Aural fullness
3) Documented hearing loss
4) Other causes excluded
5) All of the above
Sixth Annual Intensive Update in Neurology 0915-162016
53
Question 4
bull Menierersquos syndrome diagnosis is based on
1) Recurrent vertigo lasting at least 20 minutes
2) Aural fullness
3) Documented hearing loss
4) Other causes excluded
5) All of the above
Answer 5 See references at end
Sixth Annual Intensive Update in Neurology 0915-162016
54
Superior Semicircular Canal Dehiscence (SSCD)
bull Sound andor pressure induced oscillopsia
bull CT reformatted in the plane of the superior canals
shows dehiscence of overlying bone
bull Low frequency ldquoconductive hyperacusisrdquo with elevated
bone thresholds normal reflexes and a conductive loss
Sixth Annual Intensive Update in Neurology 0915-162016
55
SSCD
bull Positive middle ear pressure or loud sound stimulates ampullofugal endolymph flow -canal activation
bull Eye movements are tonic upwards with ipsilateral intorsion
Sixth Annual Intensive Update in Neurology 0915-162016
56
SSCD
bull Valsalva against a closed glottis or jugular compression causes increased intracranial pressure and ampullopetal endolymph flow -canal inhibition
bull Eye movements are tonic downwards with ipsilateral extorsion
Sixth Annual Intensive Update in Neurology 0915-162016
57
Reconstructed left superior semicircular canal
Sixth Annual Intensive Update in Neurology 0915-162016
58
Sixth Annual Intensive Update in Neurology 0915-162016
59
SCHEMATIC DRAWING OF AN OTOLITHIC MACULA
Sixth Annual Intensive Update in Neurology 0915-162016
60
MACULAE OF THE UTRICLESACCULE
Sixth Annual Intensive Update in Neurology 0915-162016
61
HAIR CELLS
Sixth Annual Intensive Update in Neurology 0915-162016
62
SCANNING ELECTRON MICROGRAPH OF OTOCONIA
Sixth Annual Intensive Update in Neurology 0915-162016
63
Ocular VEMP
bull Response to loud clicks (80-110 dB) or forehead tap (Halmagyi and Curthoys 2003)
bull Contralateral oculomotor nucleus (CN III)
bull Inferior oblique muscle contraction (8 msec) detected by 5sec event triggered averaging
bull Noninverting electrode inferior to eye
bull Inverting below non-inverting electrode
bull Ground to forehead
bull Normal subjects have very low response
Sixth Annual Intensive Update in Neurology 0915-162016
64
Clinical applications
bull The o-VEMP is a sensitive test of otolithutricular
function
bull Complements vestibular test procedures that assess
semicircular canals
bull Abnormally robust responses are associated with third
window abnormalities such as semicircular canal
dehiscence
bull Serves as an excellent screening test for semicircular
canal dehiscence
Sixth Annual Intensive Update in Neurology 0915-162016
65
Question 5
bull Superior semicircular canal dehiscence
1) Causes increased ability to hear vibratory sounds
2) Improves hearing
3) Causes meningitis
4) Always needs surgical repair
Sixth Annual Intensive Update in Neurology 0915-162016
66
Question 5
bull Superior semicircular canal dehiscence
1) Causes increased ability to hear vibratory sounds
2) Improves hearing
3) Causes meningitis
4) Always needs surgical repair
Answer 1 References at end of lecture
Sixth Annual Intensive Update in Neurology 0915-162016
67
Enlarged vestibular aqueduct
bull Axial temporal bone CT showing right temporal bone with enlarged vestibular aqueduct (arrow)
bull Vestibular symptoms accompany progressive sensorineural hearing loss in 4-50 of patients in selected series
Sixth Annual Intensive Update in Neurology 0915-162016
68
Migraine
bull Family or personal history of migraine
bull 25 of migraine patients experience spontaneous vertigo (Baloh 1997)
bull Neuhauser criteria(2001) have been used to construct structured diagnostic interviews (Furman 2003)
bull Not always associated with headache
bull Motion sensitivity
bull Medication responsive
Sixth Annual Intensive Update in Neurology 0915-162016
69
ICHD-III beta Criteria for Vestibular Migraine
bull A At least five attacks fulfilling criteria C and D
bull B A current or past history of 11 migraine without aura or 12 migraine with aura
bull C Vestibular symptoms of moderate or severe intensity lasting between 5 minutes and 72 hours
bull D At least 50 associated with one of the following migrainous features
ndash 1 Headaches
ndash 2 Photophobia and phonophobia
ndash 3 Visual Aura
bull E Not better accounted for by another vestibular disorder
Sixth Annual Intensive Update in Neurology 0915-162016
70
Incidence of vertigo in MS
bull True vertigo (the perception of environmental
movement typically rotational) occurs in about
20 of MS patients and is the presenting
manifestation in 5
bull Up to 78 of MS patients report abnormalities
in balance at some point in their illness
Frohman et al Multiple Sclerosis 2003 9250
Sixth Annual Intensive Update in Neurology 0915-162016
71
Vertigo in MS due to demyelination
bull The most common neuroanatomic locations are the
root entry zone of CN VIII (giving absent calorics but
normal hearing and fixation suppression) and the
medial vestibular nucleus
bull Usually treated with steroids and vestibular
suppressants
Brandt 1993 Gass 1998 Thomke 1999
Sixth Annual Intensive Update in Neurology 0915-162016
72
Vertigo in MS
Frohmanrsquos large series (1153) reported that 52 of MS patients with acute vertigo had benign paroxysmal positional vertigo while 32 had new brainstem lesions
To avoid misdiagnoses and inappropriate treatment interventions alternative etiologies should be considered in all acute vertigo episodes in MS
E M Frohman Neurology 2000 and Multiple Sclerosis 2003
Sixth Annual Intensive Update in Neurology 0915-162016
73
Vestibular compensation
bull Acute unilateral or bilateral vestibular loss will prompt compensation thru central cervico-ocular visual and proprioceptive pathways
bull Unilateral loss may require several weeks for compensation to occur and activity especially head movements hastens compensation
bull Vestibular suppressant medication prolongs compensation
Sixth Annual Intensive Update in Neurology 0915-162016
74
Vestibular Rehabilitation
bull Performed by certified physical therapists many sites (Epic order vestibular PT) or check Vestibular Disorders of America website for providers
bull Assessment and treatment of benign positional vertigo
bull Directed rehab programs for central andor peripheral vestibular compensationndash Unilateral vestibulopathies
ndash Bilateral vestibular hypofunction
ndash Mutifactorial balance deficits and fall risk assessment
Sixth Annual Intensive Update in Neurology 0915-162016
75
Summary of whatrsquos new in peripheral vestibular disorders
bull Lateral semicircular canal benign paroxysmal positional vertigo presents with horizontal positioning nystagmus
bull Oral steroids appear to improve outcome in acute vestibular neuritis
bull Intratympanic steroids offer promise in Menierersquos syndrome
bull Superior semicircular canal dehiscence causes pressure and noise induced dizziness VEMP offers diagnostic information
Sixth Annual Intensive Update in Neurology 0915-162016
76
References
BPPVndash White J Benign paroxysmal positional vertigo Cleveland Clinic Journal of
Medicine 71(9)722-728 2004
ndash White J Coale K Catalano P Oas J Diagnosis and management of lateral semicircular canal benign positional vertigo Otolaryngology-Head and Neck Surgery 133 278-284 2005
ndash White J Savvides P Cherian N Oas J Canalith repositioning for benign paroxysmal positional vertigo Otology and Neurotology 26 704-710 2005
ndash White J Oas J Diagnosis and management of lateral semicircular canal conversions during particle repositioning therapy Laryngoscope 115(10) 1895-1897 2005
ndash Acute Vestibular Syndrome Hotson JR Baloh RW N Engl J Med 1998 Sep 3339(10)680-5
ndash Methylprednisolone valacyclovir or the combination for vestibular neuritis Strupp M Zingler VC Arbusow V Niklas D Maag KP Dieterich M Bense S Theil D Jahn K Brandt T N Engl J Med 2004 Jul 22351(4)354-61
ndash Intratympanic steroids for Meacuteniegraveres disease or syndrome Phillips JS Westerberg BCochrane Database Syst Rev 2011 Jul 6(7)CD008514 Review
ndash Sound- andor pressure-induced vertigo due to bone dehiscence of the superior semicircular canal Minor LB Solomon D Zinreich JS Zee DSArch Otolaryngol Head Neck Surg 1998 Mar124(3)249-58
Sixth Annual Intensive Update in Neurology 0915-162016
4
Dizziness
bull Most common complaint over age 75
bull 8 million annual US visits
bull Chronic dizziness (gt 2 weeks) affects 16 of self-
reported US population
bull 354 of US adults aged 40 and older have vestibular
dysfunction (National Health and Nutrition Examination
Survey 2001-2004Arch Inter Med 169 (10) 938-44
2009)
Sixth Annual Intensive Update in Neurology 0915-162016
5
History
bull 70 of dizzy patients can be diagnosed with a careful
history (Gufoni 2005)
bull Equilibrium is an unconscious sensation Few
descriptions exist to describe its absence
Sixth Annual Intensive Update in Neurology 0915-162016
6
History
Onset ndash detailed description of first episode and most
recent episode
ndash Character
ndash Frequency
ndash Duration (vertigo andor milder symptoms)
ndash Associated hearing loss or tinnitus
ndash Provoking factors (noise pressure changes position change)
Sixth Annual Intensive Update in Neurology 0915-162016
7
Neuro-otology Physical Exam
bull Otologic ndash check hearing and rule out active infection or
cholesteatoma
bull Oculomotor- saccade pursuit in vertical and horizontal
planes
bull Nystagmus ndash spontaneous gaze evoked and
positionalpositioning testing (best observed using
Frenzel lenses)
bull Special testing ndash head thrust head shake
Sixth Annual Intensive Update in Neurology 0915-162016
8
Peripheral Nystagmus
bull Jerk nystagmus (slow
and fast phase)
bull Direction fixed
bull Generally beats away
from affected ear
bull Worse in the
direction of gaze that
is towards the fast
phase
Sixth Annual Intensive Update in Neurology 0915-162016
9
Benign Paroxysmal Positional Vertigo (BPPV)
bull Vertigo of sudden onset and brief duration provoked by changes in head positionndash Lying down
ndash Rolling over in bed
ndash Bending over
ndash Looking up
ndash Washing hair in shower
ndash Dentist or beauty parlor
Sixth Annual Intensive Update in Neurology 0915-162016
10
Right Posterior BPPV
Sixth Annual Intensive Update in Neurology 0915-162016
11
IncidencePrevalence of BPPV
bull Most common referred diagnosis in tertiary centers
bull 9 of randomly selected community dwelling elderly
(Oghalai JS et al 2000)
bull Incidence increases 38 with each decade of life
(Froehling 1991)
Sixth Annual Intensive Update in Neurology 0915-162016
12
Benign Positional Vertigo (BPPV)
bull Diagnosed with lateral supine head turns and Dix-
Hallpike positioning
ndash Latency
ndash Duration
ndash Fatigue
ndash Habituation
Sixth Annual Intensive Update in Neurology 0915-162016
13
DHT - Sitting
Sixth Annual Intensive Update in Neurology 0915-162016
14
DHT - HHR Orientation
Sixth Annual Intensive Update in Neurology 0915-162016
15
Dix-Hallpike to the Right
Sixth Annual Intensive Update in Neurology 0915-162016
16
Right posterior BPPV
Sixth Annual Intensive Update in Neurology 0915-162016
17
Canalith Repositioning Procedure
Sixth Annual Intensive Update in Neurology 0915-162016
18
Canalith Repositioning Procedure
Sixth Annual Intensive Update in Neurology 0915-162016
19
Canalith Repositioning Procedure
Sixth Annual Intensive Update in Neurology 0915-162016
20
Canalith Repositioning Procedure
Sixth Annual Intensive Update in Neurology 0915-162016
21
Canalith Repositioning
bull R posterior
BPPV
CPT 95992
Sixth Annual Intensive Update in Neurology 0915-162016
22
Posterior semicircular canalith repositioning
Sixth Annual Intensive Update in Neurology 0915-162016
23
Supine Positional Tests
Sixth Annual Intensive Update in Neurology 0915-162016
24
Lateral SCC-BPPV
bull Short latency horizontal nystagmus provoked by supine bilateral head turns with prolonged duration and poor fatigability
bull Uncommon (2-15 of total BPPV)
bull Two formsndash Geotropic ndash beating towards undermost ear
ndash Apogeotropic ndash beating away from undermost ear
Sixth Annual Intensive Update in Neurology 0915-162016
25
Geotropic Lateral Canal BPPV
bull Patient is positioned
first to the right lateral
position ndash nystagmus
beats right
bull Patient is then
positioned to the left
lateral position and
nystagmus reverses
and beats left
Sixth Annual Intensive Update in Neurology 0915-162016
26
Left Geotropic Lateral Semicircular Canal BPPV
Sixth Annual Intensive Update in Neurology 0915-162016
27
Repositioning Maneuvers for Geotropic Lateral Semicircular Canal (LSC) BPPV
bull Roll maneuver (Lempert Baloh)
ndash Patient rolls in 90 degree increments towards the good side
completely rolling over R LSC-BPPV repositioning shown
Sixth Annual Intensive Update in Neurology 0915-162016
28
Repositioning Maneuvers for Geotropic LSC BPPV
bull Gufoni maneuver
ndash Patient lies quickly onto good side and turns face-down to
floor for two minutes
Sixth Annual Intensive Update in Neurology 0915-162016
29
Left Apogeotropic LSC BPPV
Sixth Annual Intensive Update in Neurology 0915-162016
30
Nystagmus observed During Repositioning
bull Nystagmus should remain in the same torsional
direction as seen on DHT throughout the treatment of
posterior BPPV
bull Reversal of nystagmus direction suggests the particle
has fallen back into the canal and predicts failure
bull In Lateral BPPV nystagmus should beat towards the
good ear
Sixth Annual Intensive Update in Neurology 0915-162016
31
Posterior to Lateral canal conversion
Sixth Annual Intensive Update in Neurology 0915-162016
32
R Posterior to Lateral Canal conversion - geotropic
Sixth Annual Intensive Update in Neurology 0915-162016
33
BPPV Recurrence
bull Recurrence is common (15yr)
bull Otoconial adherence or mineralization demineralization
abnormalities may contribute to recurrence
bull Home canalith repositioning is effective (Radke 2004)
Sixth Annual Intensive Update in Neurology 0915-162016
34
Question 1
bull The most likely semicircular canal to be affected in
BPPV is
ndash 1) superior
ndash 2) lateral
ndash 3) posterior
ndash 4) combined
Sixth Annual Intensive Update in Neurology 0915-162016
35
Question 1
bull The most likely semicircular canal to be affected in
BPPV is
ndash 1) superior
ndash 2) lateral
ndash 3) posterior
ndash 4) combined
Answer 3 See references at end of lecture
Sixth Annual Intensive Update in Neurology 0915-162016
36
Acute Vestibular Syndrome
bull Rapid unilateral injury to either peripheral or central
vestibular structures produces prolonged vertigo
(days to weeks)
bull Severe vertigo nausea and vomiting spontaneous
nystagmus and postural instability
Sixth Annual Intensive Update in Neurology 0915-162016
37
Acute Vestibular Syndrome
bull 25 of patients with risk factors who present with isolated severe vertigo nystagmus and postural instability in the ER have an acute infarction of the inferior cerebellum (AICA PICA) Central imaging is suggested (Baloh 1998 NEJM)
ndash HTN diabetes smoking age gt65 Afib valvular heart disease occlusive vascular disease
Sixth Annual Intensive Update in Neurology 0915-162016
38
HINTS test
bull Head Impulse
bull Nystagmus
bull Test of Skew
ndash More sensitive than MRI in the diagnosis of acute stroke in
patients with acute vestibular syndrome with at least one risk
factor (Kattah J et al Stroke 2009)
Sixth Annual Intensive Update in Neurology 0915-162016
39
Head Impulse Test
bull No special equipment needed
bull Examiner stands in front of seated patient and
randomly moves head left and right Patient is asked to
focus on examinerrsquos nose
bull Rapid low amplitude thrust from lateral to straight
ahead causes a compensatory saccade when
performed towards the weak side
bull This finding is a permanent feature of unilateral
vestibular loss
Sixth Annual Intensive Update in Neurology 0915-162016
40
Head Impulse Test
Sixth Annual Intensive Update in Neurology 0915-162016
41
Head CT ndash Academy of Emergency Medicine 2013
bull The clinical value of head CT in isolated
dizzinessvertigo without any associated neurological
symptoms is very low
bull Less than 1 of head CTs ordered for dizziness and
vertigo have significant abnormalities and those
patients had severe headache and neurological deficits
in addition to dizziness
bull Forgoing unnecessary emergency head CTs could
save 360 million dollars annually
Sixth Annual Intensive Update in Neurology 0915-162016
42
Question 2
Head CT is not indicated for the evaluation of dizziness
and vertigo without additional neurological complaints because
1 It is too expensive
2 Less than 1 of CTs done for dizziness and vertigo are
abnormal and those patients had associated severe
neurological complaints
3 CT is the best imaging choice for the posterior fossa
4 Acoustic neuromas are well seen on CT
Sixth Annual Intensive Update in Neurology 0915-162016
43
Question 2
Head CT is not indicated for the evaluation of dizziness
and vertigo without additional neurological complaints because
1 It is too expensive
2 Less than 1 of CTs done for dizziness and vertigo are
abnormal and those patients had associated severe
neurological complaints
3 CT is the best imaging choice for the posterior fossa
4 Acoustic neuromas are well seen on CT
Answer 2 See references at end of lecture
Sixth Annual Intensive Update in Neurology 0915-162016
44
Acute Vestibular Syndrome
bull Usually peripheral and can be associated with
hearing loss (labyrinthitis)
bull Timely diagnosis improves recovery (ASAP
prednisone 1mgkgday for ten days and taper 10
mgday ndash Strupp M 2004 NEJM)
bull Avoid vestibular suppressants after 3 days and begin
vestibular rehabilitation to hasten compensation
Sixth Annual Intensive Update in Neurology 0915-162016
45
Question 3
bull Treatment for Acute Vestibular Syndrome
1) oral prednisone 1 mgkgday
2) Treat dehydration if necessary
3) Minimize vestibular suppressants after 3 days
4) Consider vestibular physical therapy
5) All of the above
Sixth Annual Intensive Update in Neurology 0915-162016
46
Question 3
bull Treatment for Acute Vestibular Syndrome
1) oral prednisone 1 mgkgday
2) Treat dehydration if necessary
3) Minimize vestibular suppressants after 3 days
4) Consider vestibular physical therapy
5) All of the above
Answer 5 See references at end of lecture
Sixth Annual Intensive Update in Neurology 0915-162016
47
Menierersquos syndrome
bull Fluctuating low frequency hearing loss
bull Tinnitus andor aural fullness
bull Vertigo lasting hours
Sixth Annual Intensive Update in Neurology 0915-162016
48
Normal
Sixth Annual Intensive Update in Neurology 0915-162016
49
Hydrops
Sixth Annual Intensive Update in Neurology 0915-162016
50
Menierersquos syndrome
ndash Diagnosis- Exclusion (RO acoustic)
ndash 23 of Menierersquos patients will improve spontaneously
ndash Treatment-1500 mgday low sodium triamterene375-
hydrochlorothiazide 25 vestibular suppressants (lorazepam
1 mg tablet sublingual Q 8 prn attacks) off label use
Sixth Annual Intensive Update in Neurology 0915-162016
51
Menierersquos syndrome ndash intratympanic steroids
ndash A newer treatment option for those failing medical management
ndash 90 response rate (Boleus-Aguirre MS Otology Neurotology
2008)
ndash Outcomes data confirm excellent clinical response in 3941
Menierersquos patients treated with intratympanic steroids in 2008
Sixth Annual Intensive Update in Neurology 0915-162016
52
Question 4
bull Menierersquos syndrome diagnosis is based on
1) Recurrent vertigo lasting at least 20 minutes
2) Aural fullness
3) Documented hearing loss
4) Other causes excluded
5) All of the above
Sixth Annual Intensive Update in Neurology 0915-162016
53
Question 4
bull Menierersquos syndrome diagnosis is based on
1) Recurrent vertigo lasting at least 20 minutes
2) Aural fullness
3) Documented hearing loss
4) Other causes excluded
5) All of the above
Answer 5 See references at end
Sixth Annual Intensive Update in Neurology 0915-162016
54
Superior Semicircular Canal Dehiscence (SSCD)
bull Sound andor pressure induced oscillopsia
bull CT reformatted in the plane of the superior canals
shows dehiscence of overlying bone
bull Low frequency ldquoconductive hyperacusisrdquo with elevated
bone thresholds normal reflexes and a conductive loss
Sixth Annual Intensive Update in Neurology 0915-162016
55
SSCD
bull Positive middle ear pressure or loud sound stimulates ampullofugal endolymph flow -canal activation
bull Eye movements are tonic upwards with ipsilateral intorsion
Sixth Annual Intensive Update in Neurology 0915-162016
56
SSCD
bull Valsalva against a closed glottis or jugular compression causes increased intracranial pressure and ampullopetal endolymph flow -canal inhibition
bull Eye movements are tonic downwards with ipsilateral extorsion
Sixth Annual Intensive Update in Neurology 0915-162016
57
Reconstructed left superior semicircular canal
Sixth Annual Intensive Update in Neurology 0915-162016
58
Sixth Annual Intensive Update in Neurology 0915-162016
59
SCHEMATIC DRAWING OF AN OTOLITHIC MACULA
Sixth Annual Intensive Update in Neurology 0915-162016
60
MACULAE OF THE UTRICLESACCULE
Sixth Annual Intensive Update in Neurology 0915-162016
61
HAIR CELLS
Sixth Annual Intensive Update in Neurology 0915-162016
62
SCANNING ELECTRON MICROGRAPH OF OTOCONIA
Sixth Annual Intensive Update in Neurology 0915-162016
63
Ocular VEMP
bull Response to loud clicks (80-110 dB) or forehead tap (Halmagyi and Curthoys 2003)
bull Contralateral oculomotor nucleus (CN III)
bull Inferior oblique muscle contraction (8 msec) detected by 5sec event triggered averaging
bull Noninverting electrode inferior to eye
bull Inverting below non-inverting electrode
bull Ground to forehead
bull Normal subjects have very low response
Sixth Annual Intensive Update in Neurology 0915-162016
64
Clinical applications
bull The o-VEMP is a sensitive test of otolithutricular
function
bull Complements vestibular test procedures that assess
semicircular canals
bull Abnormally robust responses are associated with third
window abnormalities such as semicircular canal
dehiscence
bull Serves as an excellent screening test for semicircular
canal dehiscence
Sixth Annual Intensive Update in Neurology 0915-162016
65
Question 5
bull Superior semicircular canal dehiscence
1) Causes increased ability to hear vibratory sounds
2) Improves hearing
3) Causes meningitis
4) Always needs surgical repair
Sixth Annual Intensive Update in Neurology 0915-162016
66
Question 5
bull Superior semicircular canal dehiscence
1) Causes increased ability to hear vibratory sounds
2) Improves hearing
3) Causes meningitis
4) Always needs surgical repair
Answer 1 References at end of lecture
Sixth Annual Intensive Update in Neurology 0915-162016
67
Enlarged vestibular aqueduct
bull Axial temporal bone CT showing right temporal bone with enlarged vestibular aqueduct (arrow)
bull Vestibular symptoms accompany progressive sensorineural hearing loss in 4-50 of patients in selected series
Sixth Annual Intensive Update in Neurology 0915-162016
68
Migraine
bull Family or personal history of migraine
bull 25 of migraine patients experience spontaneous vertigo (Baloh 1997)
bull Neuhauser criteria(2001) have been used to construct structured diagnostic interviews (Furman 2003)
bull Not always associated with headache
bull Motion sensitivity
bull Medication responsive
Sixth Annual Intensive Update in Neurology 0915-162016
69
ICHD-III beta Criteria for Vestibular Migraine
bull A At least five attacks fulfilling criteria C and D
bull B A current or past history of 11 migraine without aura or 12 migraine with aura
bull C Vestibular symptoms of moderate or severe intensity lasting between 5 minutes and 72 hours
bull D At least 50 associated with one of the following migrainous features
ndash 1 Headaches
ndash 2 Photophobia and phonophobia
ndash 3 Visual Aura
bull E Not better accounted for by another vestibular disorder
Sixth Annual Intensive Update in Neurology 0915-162016
70
Incidence of vertigo in MS
bull True vertigo (the perception of environmental
movement typically rotational) occurs in about
20 of MS patients and is the presenting
manifestation in 5
bull Up to 78 of MS patients report abnormalities
in balance at some point in their illness
Frohman et al Multiple Sclerosis 2003 9250
Sixth Annual Intensive Update in Neurology 0915-162016
71
Vertigo in MS due to demyelination
bull The most common neuroanatomic locations are the
root entry zone of CN VIII (giving absent calorics but
normal hearing and fixation suppression) and the
medial vestibular nucleus
bull Usually treated with steroids and vestibular
suppressants
Brandt 1993 Gass 1998 Thomke 1999
Sixth Annual Intensive Update in Neurology 0915-162016
72
Vertigo in MS
Frohmanrsquos large series (1153) reported that 52 of MS patients with acute vertigo had benign paroxysmal positional vertigo while 32 had new brainstem lesions
To avoid misdiagnoses and inappropriate treatment interventions alternative etiologies should be considered in all acute vertigo episodes in MS
E M Frohman Neurology 2000 and Multiple Sclerosis 2003
Sixth Annual Intensive Update in Neurology 0915-162016
73
Vestibular compensation
bull Acute unilateral or bilateral vestibular loss will prompt compensation thru central cervico-ocular visual and proprioceptive pathways
bull Unilateral loss may require several weeks for compensation to occur and activity especially head movements hastens compensation
bull Vestibular suppressant medication prolongs compensation
Sixth Annual Intensive Update in Neurology 0915-162016
74
Vestibular Rehabilitation
bull Performed by certified physical therapists many sites (Epic order vestibular PT) or check Vestibular Disorders of America website for providers
bull Assessment and treatment of benign positional vertigo
bull Directed rehab programs for central andor peripheral vestibular compensationndash Unilateral vestibulopathies
ndash Bilateral vestibular hypofunction
ndash Mutifactorial balance deficits and fall risk assessment
Sixth Annual Intensive Update in Neurology 0915-162016
75
Summary of whatrsquos new in peripheral vestibular disorders
bull Lateral semicircular canal benign paroxysmal positional vertigo presents with horizontal positioning nystagmus
bull Oral steroids appear to improve outcome in acute vestibular neuritis
bull Intratympanic steroids offer promise in Menierersquos syndrome
bull Superior semicircular canal dehiscence causes pressure and noise induced dizziness VEMP offers diagnostic information
Sixth Annual Intensive Update in Neurology 0915-162016
76
References
BPPVndash White J Benign paroxysmal positional vertigo Cleveland Clinic Journal of
Medicine 71(9)722-728 2004
ndash White J Coale K Catalano P Oas J Diagnosis and management of lateral semicircular canal benign positional vertigo Otolaryngology-Head and Neck Surgery 133 278-284 2005
ndash White J Savvides P Cherian N Oas J Canalith repositioning for benign paroxysmal positional vertigo Otology and Neurotology 26 704-710 2005
ndash White J Oas J Diagnosis and management of lateral semicircular canal conversions during particle repositioning therapy Laryngoscope 115(10) 1895-1897 2005
ndash Acute Vestibular Syndrome Hotson JR Baloh RW N Engl J Med 1998 Sep 3339(10)680-5
ndash Methylprednisolone valacyclovir or the combination for vestibular neuritis Strupp M Zingler VC Arbusow V Niklas D Maag KP Dieterich M Bense S Theil D Jahn K Brandt T N Engl J Med 2004 Jul 22351(4)354-61
ndash Intratympanic steroids for Meacuteniegraveres disease or syndrome Phillips JS Westerberg BCochrane Database Syst Rev 2011 Jul 6(7)CD008514 Review
ndash Sound- andor pressure-induced vertigo due to bone dehiscence of the superior semicircular canal Minor LB Solomon D Zinreich JS Zee DSArch Otolaryngol Head Neck Surg 1998 Mar124(3)249-58
Sixth Annual Intensive Update in Neurology 0915-162016
5
History
bull 70 of dizzy patients can be diagnosed with a careful
history (Gufoni 2005)
bull Equilibrium is an unconscious sensation Few
descriptions exist to describe its absence
Sixth Annual Intensive Update in Neurology 0915-162016
6
History
Onset ndash detailed description of first episode and most
recent episode
ndash Character
ndash Frequency
ndash Duration (vertigo andor milder symptoms)
ndash Associated hearing loss or tinnitus
ndash Provoking factors (noise pressure changes position change)
Sixth Annual Intensive Update in Neurology 0915-162016
7
Neuro-otology Physical Exam
bull Otologic ndash check hearing and rule out active infection or
cholesteatoma
bull Oculomotor- saccade pursuit in vertical and horizontal
planes
bull Nystagmus ndash spontaneous gaze evoked and
positionalpositioning testing (best observed using
Frenzel lenses)
bull Special testing ndash head thrust head shake
Sixth Annual Intensive Update in Neurology 0915-162016
8
Peripheral Nystagmus
bull Jerk nystagmus (slow
and fast phase)
bull Direction fixed
bull Generally beats away
from affected ear
bull Worse in the
direction of gaze that
is towards the fast
phase
Sixth Annual Intensive Update in Neurology 0915-162016
9
Benign Paroxysmal Positional Vertigo (BPPV)
bull Vertigo of sudden onset and brief duration provoked by changes in head positionndash Lying down
ndash Rolling over in bed
ndash Bending over
ndash Looking up
ndash Washing hair in shower
ndash Dentist or beauty parlor
Sixth Annual Intensive Update in Neurology 0915-162016
10
Right Posterior BPPV
Sixth Annual Intensive Update in Neurology 0915-162016
11
IncidencePrevalence of BPPV
bull Most common referred diagnosis in tertiary centers
bull 9 of randomly selected community dwelling elderly
(Oghalai JS et al 2000)
bull Incidence increases 38 with each decade of life
(Froehling 1991)
Sixth Annual Intensive Update in Neurology 0915-162016
12
Benign Positional Vertigo (BPPV)
bull Diagnosed with lateral supine head turns and Dix-
Hallpike positioning
ndash Latency
ndash Duration
ndash Fatigue
ndash Habituation
Sixth Annual Intensive Update in Neurology 0915-162016
13
DHT - Sitting
Sixth Annual Intensive Update in Neurology 0915-162016
14
DHT - HHR Orientation
Sixth Annual Intensive Update in Neurology 0915-162016
15
Dix-Hallpike to the Right
Sixth Annual Intensive Update in Neurology 0915-162016
16
Right posterior BPPV
Sixth Annual Intensive Update in Neurology 0915-162016
17
Canalith Repositioning Procedure
Sixth Annual Intensive Update in Neurology 0915-162016
18
Canalith Repositioning Procedure
Sixth Annual Intensive Update in Neurology 0915-162016
19
Canalith Repositioning Procedure
Sixth Annual Intensive Update in Neurology 0915-162016
20
Canalith Repositioning Procedure
Sixth Annual Intensive Update in Neurology 0915-162016
21
Canalith Repositioning
bull R posterior
BPPV
CPT 95992
Sixth Annual Intensive Update in Neurology 0915-162016
22
Posterior semicircular canalith repositioning
Sixth Annual Intensive Update in Neurology 0915-162016
23
Supine Positional Tests
Sixth Annual Intensive Update in Neurology 0915-162016
24
Lateral SCC-BPPV
bull Short latency horizontal nystagmus provoked by supine bilateral head turns with prolonged duration and poor fatigability
bull Uncommon (2-15 of total BPPV)
bull Two formsndash Geotropic ndash beating towards undermost ear
ndash Apogeotropic ndash beating away from undermost ear
Sixth Annual Intensive Update in Neurology 0915-162016
25
Geotropic Lateral Canal BPPV
bull Patient is positioned
first to the right lateral
position ndash nystagmus
beats right
bull Patient is then
positioned to the left
lateral position and
nystagmus reverses
and beats left
Sixth Annual Intensive Update in Neurology 0915-162016
26
Left Geotropic Lateral Semicircular Canal BPPV
Sixth Annual Intensive Update in Neurology 0915-162016
27
Repositioning Maneuvers for Geotropic Lateral Semicircular Canal (LSC) BPPV
bull Roll maneuver (Lempert Baloh)
ndash Patient rolls in 90 degree increments towards the good side
completely rolling over R LSC-BPPV repositioning shown
Sixth Annual Intensive Update in Neurology 0915-162016
28
Repositioning Maneuvers for Geotropic LSC BPPV
bull Gufoni maneuver
ndash Patient lies quickly onto good side and turns face-down to
floor for two minutes
Sixth Annual Intensive Update in Neurology 0915-162016
29
Left Apogeotropic LSC BPPV
Sixth Annual Intensive Update in Neurology 0915-162016
30
Nystagmus observed During Repositioning
bull Nystagmus should remain in the same torsional
direction as seen on DHT throughout the treatment of
posterior BPPV
bull Reversal of nystagmus direction suggests the particle
has fallen back into the canal and predicts failure
bull In Lateral BPPV nystagmus should beat towards the
good ear
Sixth Annual Intensive Update in Neurology 0915-162016
31
Posterior to Lateral canal conversion
Sixth Annual Intensive Update in Neurology 0915-162016
32
R Posterior to Lateral Canal conversion - geotropic
Sixth Annual Intensive Update in Neurology 0915-162016
33
BPPV Recurrence
bull Recurrence is common (15yr)
bull Otoconial adherence or mineralization demineralization
abnormalities may contribute to recurrence
bull Home canalith repositioning is effective (Radke 2004)
Sixth Annual Intensive Update in Neurology 0915-162016
34
Question 1
bull The most likely semicircular canal to be affected in
BPPV is
ndash 1) superior
ndash 2) lateral
ndash 3) posterior
ndash 4) combined
Sixth Annual Intensive Update in Neurology 0915-162016
35
Question 1
bull The most likely semicircular canal to be affected in
BPPV is
ndash 1) superior
ndash 2) lateral
ndash 3) posterior
ndash 4) combined
Answer 3 See references at end of lecture
Sixth Annual Intensive Update in Neurology 0915-162016
36
Acute Vestibular Syndrome
bull Rapid unilateral injury to either peripheral or central
vestibular structures produces prolonged vertigo
(days to weeks)
bull Severe vertigo nausea and vomiting spontaneous
nystagmus and postural instability
Sixth Annual Intensive Update in Neurology 0915-162016
37
Acute Vestibular Syndrome
bull 25 of patients with risk factors who present with isolated severe vertigo nystagmus and postural instability in the ER have an acute infarction of the inferior cerebellum (AICA PICA) Central imaging is suggested (Baloh 1998 NEJM)
ndash HTN diabetes smoking age gt65 Afib valvular heart disease occlusive vascular disease
Sixth Annual Intensive Update in Neurology 0915-162016
38
HINTS test
bull Head Impulse
bull Nystagmus
bull Test of Skew
ndash More sensitive than MRI in the diagnosis of acute stroke in
patients with acute vestibular syndrome with at least one risk
factor (Kattah J et al Stroke 2009)
Sixth Annual Intensive Update in Neurology 0915-162016
39
Head Impulse Test
bull No special equipment needed
bull Examiner stands in front of seated patient and
randomly moves head left and right Patient is asked to
focus on examinerrsquos nose
bull Rapid low amplitude thrust from lateral to straight
ahead causes a compensatory saccade when
performed towards the weak side
bull This finding is a permanent feature of unilateral
vestibular loss
Sixth Annual Intensive Update in Neurology 0915-162016
40
Head Impulse Test
Sixth Annual Intensive Update in Neurology 0915-162016
41
Head CT ndash Academy of Emergency Medicine 2013
bull The clinical value of head CT in isolated
dizzinessvertigo without any associated neurological
symptoms is very low
bull Less than 1 of head CTs ordered for dizziness and
vertigo have significant abnormalities and those
patients had severe headache and neurological deficits
in addition to dizziness
bull Forgoing unnecessary emergency head CTs could
save 360 million dollars annually
Sixth Annual Intensive Update in Neurology 0915-162016
42
Question 2
Head CT is not indicated for the evaluation of dizziness
and vertigo without additional neurological complaints because
1 It is too expensive
2 Less than 1 of CTs done for dizziness and vertigo are
abnormal and those patients had associated severe
neurological complaints
3 CT is the best imaging choice for the posterior fossa
4 Acoustic neuromas are well seen on CT
Sixth Annual Intensive Update in Neurology 0915-162016
43
Question 2
Head CT is not indicated for the evaluation of dizziness
and vertigo without additional neurological complaints because
1 It is too expensive
2 Less than 1 of CTs done for dizziness and vertigo are
abnormal and those patients had associated severe
neurological complaints
3 CT is the best imaging choice for the posterior fossa
4 Acoustic neuromas are well seen on CT
Answer 2 See references at end of lecture
Sixth Annual Intensive Update in Neurology 0915-162016
44
Acute Vestibular Syndrome
bull Usually peripheral and can be associated with
hearing loss (labyrinthitis)
bull Timely diagnosis improves recovery (ASAP
prednisone 1mgkgday for ten days and taper 10
mgday ndash Strupp M 2004 NEJM)
bull Avoid vestibular suppressants after 3 days and begin
vestibular rehabilitation to hasten compensation
Sixth Annual Intensive Update in Neurology 0915-162016
45
Question 3
bull Treatment for Acute Vestibular Syndrome
1) oral prednisone 1 mgkgday
2) Treat dehydration if necessary
3) Minimize vestibular suppressants after 3 days
4) Consider vestibular physical therapy
5) All of the above
Sixth Annual Intensive Update in Neurology 0915-162016
46
Question 3
bull Treatment for Acute Vestibular Syndrome
1) oral prednisone 1 mgkgday
2) Treat dehydration if necessary
3) Minimize vestibular suppressants after 3 days
4) Consider vestibular physical therapy
5) All of the above
Answer 5 See references at end of lecture
Sixth Annual Intensive Update in Neurology 0915-162016
47
Menierersquos syndrome
bull Fluctuating low frequency hearing loss
bull Tinnitus andor aural fullness
bull Vertigo lasting hours
Sixth Annual Intensive Update in Neurology 0915-162016
48
Normal
Sixth Annual Intensive Update in Neurology 0915-162016
49
Hydrops
Sixth Annual Intensive Update in Neurology 0915-162016
50
Menierersquos syndrome
ndash Diagnosis- Exclusion (RO acoustic)
ndash 23 of Menierersquos patients will improve spontaneously
ndash Treatment-1500 mgday low sodium triamterene375-
hydrochlorothiazide 25 vestibular suppressants (lorazepam
1 mg tablet sublingual Q 8 prn attacks) off label use
Sixth Annual Intensive Update in Neurology 0915-162016
51
Menierersquos syndrome ndash intratympanic steroids
ndash A newer treatment option for those failing medical management
ndash 90 response rate (Boleus-Aguirre MS Otology Neurotology
2008)
ndash Outcomes data confirm excellent clinical response in 3941
Menierersquos patients treated with intratympanic steroids in 2008
Sixth Annual Intensive Update in Neurology 0915-162016
52
Question 4
bull Menierersquos syndrome diagnosis is based on
1) Recurrent vertigo lasting at least 20 minutes
2) Aural fullness
3) Documented hearing loss
4) Other causes excluded
5) All of the above
Sixth Annual Intensive Update in Neurology 0915-162016
53
Question 4
bull Menierersquos syndrome diagnosis is based on
1) Recurrent vertigo lasting at least 20 minutes
2) Aural fullness
3) Documented hearing loss
4) Other causes excluded
5) All of the above
Answer 5 See references at end
Sixth Annual Intensive Update in Neurology 0915-162016
54
Superior Semicircular Canal Dehiscence (SSCD)
bull Sound andor pressure induced oscillopsia
bull CT reformatted in the plane of the superior canals
shows dehiscence of overlying bone
bull Low frequency ldquoconductive hyperacusisrdquo with elevated
bone thresholds normal reflexes and a conductive loss
Sixth Annual Intensive Update in Neurology 0915-162016
55
SSCD
bull Positive middle ear pressure or loud sound stimulates ampullofugal endolymph flow -canal activation
bull Eye movements are tonic upwards with ipsilateral intorsion
Sixth Annual Intensive Update in Neurology 0915-162016
56
SSCD
bull Valsalva against a closed glottis or jugular compression causes increased intracranial pressure and ampullopetal endolymph flow -canal inhibition
bull Eye movements are tonic downwards with ipsilateral extorsion
Sixth Annual Intensive Update in Neurology 0915-162016
57
Reconstructed left superior semicircular canal
Sixth Annual Intensive Update in Neurology 0915-162016
58
Sixth Annual Intensive Update in Neurology 0915-162016
59
SCHEMATIC DRAWING OF AN OTOLITHIC MACULA
Sixth Annual Intensive Update in Neurology 0915-162016
60
MACULAE OF THE UTRICLESACCULE
Sixth Annual Intensive Update in Neurology 0915-162016
61
HAIR CELLS
Sixth Annual Intensive Update in Neurology 0915-162016
62
SCANNING ELECTRON MICROGRAPH OF OTOCONIA
Sixth Annual Intensive Update in Neurology 0915-162016
63
Ocular VEMP
bull Response to loud clicks (80-110 dB) or forehead tap (Halmagyi and Curthoys 2003)
bull Contralateral oculomotor nucleus (CN III)
bull Inferior oblique muscle contraction (8 msec) detected by 5sec event triggered averaging
bull Noninverting electrode inferior to eye
bull Inverting below non-inverting electrode
bull Ground to forehead
bull Normal subjects have very low response
Sixth Annual Intensive Update in Neurology 0915-162016
64
Clinical applications
bull The o-VEMP is a sensitive test of otolithutricular
function
bull Complements vestibular test procedures that assess
semicircular canals
bull Abnormally robust responses are associated with third
window abnormalities such as semicircular canal
dehiscence
bull Serves as an excellent screening test for semicircular
canal dehiscence
Sixth Annual Intensive Update in Neurology 0915-162016
65
Question 5
bull Superior semicircular canal dehiscence
1) Causes increased ability to hear vibratory sounds
2) Improves hearing
3) Causes meningitis
4) Always needs surgical repair
Sixth Annual Intensive Update in Neurology 0915-162016
66
Question 5
bull Superior semicircular canal dehiscence
1) Causes increased ability to hear vibratory sounds
2) Improves hearing
3) Causes meningitis
4) Always needs surgical repair
Answer 1 References at end of lecture
Sixth Annual Intensive Update in Neurology 0915-162016
67
Enlarged vestibular aqueduct
bull Axial temporal bone CT showing right temporal bone with enlarged vestibular aqueduct (arrow)
bull Vestibular symptoms accompany progressive sensorineural hearing loss in 4-50 of patients in selected series
Sixth Annual Intensive Update in Neurology 0915-162016
68
Migraine
bull Family or personal history of migraine
bull 25 of migraine patients experience spontaneous vertigo (Baloh 1997)
bull Neuhauser criteria(2001) have been used to construct structured diagnostic interviews (Furman 2003)
bull Not always associated with headache
bull Motion sensitivity
bull Medication responsive
Sixth Annual Intensive Update in Neurology 0915-162016
69
ICHD-III beta Criteria for Vestibular Migraine
bull A At least five attacks fulfilling criteria C and D
bull B A current or past history of 11 migraine without aura or 12 migraine with aura
bull C Vestibular symptoms of moderate or severe intensity lasting between 5 minutes and 72 hours
bull D At least 50 associated with one of the following migrainous features
ndash 1 Headaches
ndash 2 Photophobia and phonophobia
ndash 3 Visual Aura
bull E Not better accounted for by another vestibular disorder
Sixth Annual Intensive Update in Neurology 0915-162016
70
Incidence of vertigo in MS
bull True vertigo (the perception of environmental
movement typically rotational) occurs in about
20 of MS patients and is the presenting
manifestation in 5
bull Up to 78 of MS patients report abnormalities
in balance at some point in their illness
Frohman et al Multiple Sclerosis 2003 9250
Sixth Annual Intensive Update in Neurology 0915-162016
71
Vertigo in MS due to demyelination
bull The most common neuroanatomic locations are the
root entry zone of CN VIII (giving absent calorics but
normal hearing and fixation suppression) and the
medial vestibular nucleus
bull Usually treated with steroids and vestibular
suppressants
Brandt 1993 Gass 1998 Thomke 1999
Sixth Annual Intensive Update in Neurology 0915-162016
72
Vertigo in MS
Frohmanrsquos large series (1153) reported that 52 of MS patients with acute vertigo had benign paroxysmal positional vertigo while 32 had new brainstem lesions
To avoid misdiagnoses and inappropriate treatment interventions alternative etiologies should be considered in all acute vertigo episodes in MS
E M Frohman Neurology 2000 and Multiple Sclerosis 2003
Sixth Annual Intensive Update in Neurology 0915-162016
73
Vestibular compensation
bull Acute unilateral or bilateral vestibular loss will prompt compensation thru central cervico-ocular visual and proprioceptive pathways
bull Unilateral loss may require several weeks for compensation to occur and activity especially head movements hastens compensation
bull Vestibular suppressant medication prolongs compensation
Sixth Annual Intensive Update in Neurology 0915-162016
74
Vestibular Rehabilitation
bull Performed by certified physical therapists many sites (Epic order vestibular PT) or check Vestibular Disorders of America website for providers
bull Assessment and treatment of benign positional vertigo
bull Directed rehab programs for central andor peripheral vestibular compensationndash Unilateral vestibulopathies
ndash Bilateral vestibular hypofunction
ndash Mutifactorial balance deficits and fall risk assessment
Sixth Annual Intensive Update in Neurology 0915-162016
75
Summary of whatrsquos new in peripheral vestibular disorders
bull Lateral semicircular canal benign paroxysmal positional vertigo presents with horizontal positioning nystagmus
bull Oral steroids appear to improve outcome in acute vestibular neuritis
bull Intratympanic steroids offer promise in Menierersquos syndrome
bull Superior semicircular canal dehiscence causes pressure and noise induced dizziness VEMP offers diagnostic information
Sixth Annual Intensive Update in Neurology 0915-162016
76
References
BPPVndash White J Benign paroxysmal positional vertigo Cleveland Clinic Journal of
Medicine 71(9)722-728 2004
ndash White J Coale K Catalano P Oas J Diagnosis and management of lateral semicircular canal benign positional vertigo Otolaryngology-Head and Neck Surgery 133 278-284 2005
ndash White J Savvides P Cherian N Oas J Canalith repositioning for benign paroxysmal positional vertigo Otology and Neurotology 26 704-710 2005
ndash White J Oas J Diagnosis and management of lateral semicircular canal conversions during particle repositioning therapy Laryngoscope 115(10) 1895-1897 2005
ndash Acute Vestibular Syndrome Hotson JR Baloh RW N Engl J Med 1998 Sep 3339(10)680-5
ndash Methylprednisolone valacyclovir or the combination for vestibular neuritis Strupp M Zingler VC Arbusow V Niklas D Maag KP Dieterich M Bense S Theil D Jahn K Brandt T N Engl J Med 2004 Jul 22351(4)354-61
ndash Intratympanic steroids for Meacuteniegraveres disease or syndrome Phillips JS Westerberg BCochrane Database Syst Rev 2011 Jul 6(7)CD008514 Review
ndash Sound- andor pressure-induced vertigo due to bone dehiscence of the superior semicircular canal Minor LB Solomon D Zinreich JS Zee DSArch Otolaryngol Head Neck Surg 1998 Mar124(3)249-58
Sixth Annual Intensive Update in Neurology 0915-162016
6
History
Onset ndash detailed description of first episode and most
recent episode
ndash Character
ndash Frequency
ndash Duration (vertigo andor milder symptoms)
ndash Associated hearing loss or tinnitus
ndash Provoking factors (noise pressure changes position change)
Sixth Annual Intensive Update in Neurology 0915-162016
7
Neuro-otology Physical Exam
bull Otologic ndash check hearing and rule out active infection or
cholesteatoma
bull Oculomotor- saccade pursuit in vertical and horizontal
planes
bull Nystagmus ndash spontaneous gaze evoked and
positionalpositioning testing (best observed using
Frenzel lenses)
bull Special testing ndash head thrust head shake
Sixth Annual Intensive Update in Neurology 0915-162016
8
Peripheral Nystagmus
bull Jerk nystagmus (slow
and fast phase)
bull Direction fixed
bull Generally beats away
from affected ear
bull Worse in the
direction of gaze that
is towards the fast
phase
Sixth Annual Intensive Update in Neurology 0915-162016
9
Benign Paroxysmal Positional Vertigo (BPPV)
bull Vertigo of sudden onset and brief duration provoked by changes in head positionndash Lying down
ndash Rolling over in bed
ndash Bending over
ndash Looking up
ndash Washing hair in shower
ndash Dentist or beauty parlor
Sixth Annual Intensive Update in Neurology 0915-162016
10
Right Posterior BPPV
Sixth Annual Intensive Update in Neurology 0915-162016
11
IncidencePrevalence of BPPV
bull Most common referred diagnosis in tertiary centers
bull 9 of randomly selected community dwelling elderly
(Oghalai JS et al 2000)
bull Incidence increases 38 with each decade of life
(Froehling 1991)
Sixth Annual Intensive Update in Neurology 0915-162016
12
Benign Positional Vertigo (BPPV)
bull Diagnosed with lateral supine head turns and Dix-
Hallpike positioning
ndash Latency
ndash Duration
ndash Fatigue
ndash Habituation
Sixth Annual Intensive Update in Neurology 0915-162016
13
DHT - Sitting
Sixth Annual Intensive Update in Neurology 0915-162016
14
DHT - HHR Orientation
Sixth Annual Intensive Update in Neurology 0915-162016
15
Dix-Hallpike to the Right
Sixth Annual Intensive Update in Neurology 0915-162016
16
Right posterior BPPV
Sixth Annual Intensive Update in Neurology 0915-162016
17
Canalith Repositioning Procedure
Sixth Annual Intensive Update in Neurology 0915-162016
18
Canalith Repositioning Procedure
Sixth Annual Intensive Update in Neurology 0915-162016
19
Canalith Repositioning Procedure
Sixth Annual Intensive Update in Neurology 0915-162016
20
Canalith Repositioning Procedure
Sixth Annual Intensive Update in Neurology 0915-162016
21
Canalith Repositioning
bull R posterior
BPPV
CPT 95992
Sixth Annual Intensive Update in Neurology 0915-162016
22
Posterior semicircular canalith repositioning
Sixth Annual Intensive Update in Neurology 0915-162016
23
Supine Positional Tests
Sixth Annual Intensive Update in Neurology 0915-162016
24
Lateral SCC-BPPV
bull Short latency horizontal nystagmus provoked by supine bilateral head turns with prolonged duration and poor fatigability
bull Uncommon (2-15 of total BPPV)
bull Two formsndash Geotropic ndash beating towards undermost ear
ndash Apogeotropic ndash beating away from undermost ear
Sixth Annual Intensive Update in Neurology 0915-162016
25
Geotropic Lateral Canal BPPV
bull Patient is positioned
first to the right lateral
position ndash nystagmus
beats right
bull Patient is then
positioned to the left
lateral position and
nystagmus reverses
and beats left
Sixth Annual Intensive Update in Neurology 0915-162016
26
Left Geotropic Lateral Semicircular Canal BPPV
Sixth Annual Intensive Update in Neurology 0915-162016
27
Repositioning Maneuvers for Geotropic Lateral Semicircular Canal (LSC) BPPV
bull Roll maneuver (Lempert Baloh)
ndash Patient rolls in 90 degree increments towards the good side
completely rolling over R LSC-BPPV repositioning shown
Sixth Annual Intensive Update in Neurology 0915-162016
28
Repositioning Maneuvers for Geotropic LSC BPPV
bull Gufoni maneuver
ndash Patient lies quickly onto good side and turns face-down to
floor for two minutes
Sixth Annual Intensive Update in Neurology 0915-162016
29
Left Apogeotropic LSC BPPV
Sixth Annual Intensive Update in Neurology 0915-162016
30
Nystagmus observed During Repositioning
bull Nystagmus should remain in the same torsional
direction as seen on DHT throughout the treatment of
posterior BPPV
bull Reversal of nystagmus direction suggests the particle
has fallen back into the canal and predicts failure
bull In Lateral BPPV nystagmus should beat towards the
good ear
Sixth Annual Intensive Update in Neurology 0915-162016
31
Posterior to Lateral canal conversion
Sixth Annual Intensive Update in Neurology 0915-162016
32
R Posterior to Lateral Canal conversion - geotropic
Sixth Annual Intensive Update in Neurology 0915-162016
33
BPPV Recurrence
bull Recurrence is common (15yr)
bull Otoconial adherence or mineralization demineralization
abnormalities may contribute to recurrence
bull Home canalith repositioning is effective (Radke 2004)
Sixth Annual Intensive Update in Neurology 0915-162016
34
Question 1
bull The most likely semicircular canal to be affected in
BPPV is
ndash 1) superior
ndash 2) lateral
ndash 3) posterior
ndash 4) combined
Sixth Annual Intensive Update in Neurology 0915-162016
35
Question 1
bull The most likely semicircular canal to be affected in
BPPV is
ndash 1) superior
ndash 2) lateral
ndash 3) posterior
ndash 4) combined
Answer 3 See references at end of lecture
Sixth Annual Intensive Update in Neurology 0915-162016
36
Acute Vestibular Syndrome
bull Rapid unilateral injury to either peripheral or central
vestibular structures produces prolonged vertigo
(days to weeks)
bull Severe vertigo nausea and vomiting spontaneous
nystagmus and postural instability
Sixth Annual Intensive Update in Neurology 0915-162016
37
Acute Vestibular Syndrome
bull 25 of patients with risk factors who present with isolated severe vertigo nystagmus and postural instability in the ER have an acute infarction of the inferior cerebellum (AICA PICA) Central imaging is suggested (Baloh 1998 NEJM)
ndash HTN diabetes smoking age gt65 Afib valvular heart disease occlusive vascular disease
Sixth Annual Intensive Update in Neurology 0915-162016
38
HINTS test
bull Head Impulse
bull Nystagmus
bull Test of Skew
ndash More sensitive than MRI in the diagnosis of acute stroke in
patients with acute vestibular syndrome with at least one risk
factor (Kattah J et al Stroke 2009)
Sixth Annual Intensive Update in Neurology 0915-162016
39
Head Impulse Test
bull No special equipment needed
bull Examiner stands in front of seated patient and
randomly moves head left and right Patient is asked to
focus on examinerrsquos nose
bull Rapid low amplitude thrust from lateral to straight
ahead causes a compensatory saccade when
performed towards the weak side
bull This finding is a permanent feature of unilateral
vestibular loss
Sixth Annual Intensive Update in Neurology 0915-162016
40
Head Impulse Test
Sixth Annual Intensive Update in Neurology 0915-162016
41
Head CT ndash Academy of Emergency Medicine 2013
bull The clinical value of head CT in isolated
dizzinessvertigo without any associated neurological
symptoms is very low
bull Less than 1 of head CTs ordered for dizziness and
vertigo have significant abnormalities and those
patients had severe headache and neurological deficits
in addition to dizziness
bull Forgoing unnecessary emergency head CTs could
save 360 million dollars annually
Sixth Annual Intensive Update in Neurology 0915-162016
42
Question 2
Head CT is not indicated for the evaluation of dizziness
and vertigo without additional neurological complaints because
1 It is too expensive
2 Less than 1 of CTs done for dizziness and vertigo are
abnormal and those patients had associated severe
neurological complaints
3 CT is the best imaging choice for the posterior fossa
4 Acoustic neuromas are well seen on CT
Sixth Annual Intensive Update in Neurology 0915-162016
43
Question 2
Head CT is not indicated for the evaluation of dizziness
and vertigo without additional neurological complaints because
1 It is too expensive
2 Less than 1 of CTs done for dizziness and vertigo are
abnormal and those patients had associated severe
neurological complaints
3 CT is the best imaging choice for the posterior fossa
4 Acoustic neuromas are well seen on CT
Answer 2 See references at end of lecture
Sixth Annual Intensive Update in Neurology 0915-162016
44
Acute Vestibular Syndrome
bull Usually peripheral and can be associated with
hearing loss (labyrinthitis)
bull Timely diagnosis improves recovery (ASAP
prednisone 1mgkgday for ten days and taper 10
mgday ndash Strupp M 2004 NEJM)
bull Avoid vestibular suppressants after 3 days and begin
vestibular rehabilitation to hasten compensation
Sixth Annual Intensive Update in Neurology 0915-162016
45
Question 3
bull Treatment for Acute Vestibular Syndrome
1) oral prednisone 1 mgkgday
2) Treat dehydration if necessary
3) Minimize vestibular suppressants after 3 days
4) Consider vestibular physical therapy
5) All of the above
Sixth Annual Intensive Update in Neurology 0915-162016
46
Question 3
bull Treatment for Acute Vestibular Syndrome
1) oral prednisone 1 mgkgday
2) Treat dehydration if necessary
3) Minimize vestibular suppressants after 3 days
4) Consider vestibular physical therapy
5) All of the above
Answer 5 See references at end of lecture
Sixth Annual Intensive Update in Neurology 0915-162016
47
Menierersquos syndrome
bull Fluctuating low frequency hearing loss
bull Tinnitus andor aural fullness
bull Vertigo lasting hours
Sixth Annual Intensive Update in Neurology 0915-162016
48
Normal
Sixth Annual Intensive Update in Neurology 0915-162016
49
Hydrops
Sixth Annual Intensive Update in Neurology 0915-162016
50
Menierersquos syndrome
ndash Diagnosis- Exclusion (RO acoustic)
ndash 23 of Menierersquos patients will improve spontaneously
ndash Treatment-1500 mgday low sodium triamterene375-
hydrochlorothiazide 25 vestibular suppressants (lorazepam
1 mg tablet sublingual Q 8 prn attacks) off label use
Sixth Annual Intensive Update in Neurology 0915-162016
51
Menierersquos syndrome ndash intratympanic steroids
ndash A newer treatment option for those failing medical management
ndash 90 response rate (Boleus-Aguirre MS Otology Neurotology
2008)
ndash Outcomes data confirm excellent clinical response in 3941
Menierersquos patients treated with intratympanic steroids in 2008
Sixth Annual Intensive Update in Neurology 0915-162016
52
Question 4
bull Menierersquos syndrome diagnosis is based on
1) Recurrent vertigo lasting at least 20 minutes
2) Aural fullness
3) Documented hearing loss
4) Other causes excluded
5) All of the above
Sixth Annual Intensive Update in Neurology 0915-162016
53
Question 4
bull Menierersquos syndrome diagnosis is based on
1) Recurrent vertigo lasting at least 20 minutes
2) Aural fullness
3) Documented hearing loss
4) Other causes excluded
5) All of the above
Answer 5 See references at end
Sixth Annual Intensive Update in Neurology 0915-162016
54
Superior Semicircular Canal Dehiscence (SSCD)
bull Sound andor pressure induced oscillopsia
bull CT reformatted in the plane of the superior canals
shows dehiscence of overlying bone
bull Low frequency ldquoconductive hyperacusisrdquo with elevated
bone thresholds normal reflexes and a conductive loss
Sixth Annual Intensive Update in Neurology 0915-162016
55
SSCD
bull Positive middle ear pressure or loud sound stimulates ampullofugal endolymph flow -canal activation
bull Eye movements are tonic upwards with ipsilateral intorsion
Sixth Annual Intensive Update in Neurology 0915-162016
56
SSCD
bull Valsalva against a closed glottis or jugular compression causes increased intracranial pressure and ampullopetal endolymph flow -canal inhibition
bull Eye movements are tonic downwards with ipsilateral extorsion
Sixth Annual Intensive Update in Neurology 0915-162016
57
Reconstructed left superior semicircular canal
Sixth Annual Intensive Update in Neurology 0915-162016
58
Sixth Annual Intensive Update in Neurology 0915-162016
59
SCHEMATIC DRAWING OF AN OTOLITHIC MACULA
Sixth Annual Intensive Update in Neurology 0915-162016
60
MACULAE OF THE UTRICLESACCULE
Sixth Annual Intensive Update in Neurology 0915-162016
61
HAIR CELLS
Sixth Annual Intensive Update in Neurology 0915-162016
62
SCANNING ELECTRON MICROGRAPH OF OTOCONIA
Sixth Annual Intensive Update in Neurology 0915-162016
63
Ocular VEMP
bull Response to loud clicks (80-110 dB) or forehead tap (Halmagyi and Curthoys 2003)
bull Contralateral oculomotor nucleus (CN III)
bull Inferior oblique muscle contraction (8 msec) detected by 5sec event triggered averaging
bull Noninverting electrode inferior to eye
bull Inverting below non-inverting electrode
bull Ground to forehead
bull Normal subjects have very low response
Sixth Annual Intensive Update in Neurology 0915-162016
64
Clinical applications
bull The o-VEMP is a sensitive test of otolithutricular
function
bull Complements vestibular test procedures that assess
semicircular canals
bull Abnormally robust responses are associated with third
window abnormalities such as semicircular canal
dehiscence
bull Serves as an excellent screening test for semicircular
canal dehiscence
Sixth Annual Intensive Update in Neurology 0915-162016
65
Question 5
bull Superior semicircular canal dehiscence
1) Causes increased ability to hear vibratory sounds
2) Improves hearing
3) Causes meningitis
4) Always needs surgical repair
Sixth Annual Intensive Update in Neurology 0915-162016
66
Question 5
bull Superior semicircular canal dehiscence
1) Causes increased ability to hear vibratory sounds
2) Improves hearing
3) Causes meningitis
4) Always needs surgical repair
Answer 1 References at end of lecture
Sixth Annual Intensive Update in Neurology 0915-162016
67
Enlarged vestibular aqueduct
bull Axial temporal bone CT showing right temporal bone with enlarged vestibular aqueduct (arrow)
bull Vestibular symptoms accompany progressive sensorineural hearing loss in 4-50 of patients in selected series
Sixth Annual Intensive Update in Neurology 0915-162016
68
Migraine
bull Family or personal history of migraine
bull 25 of migraine patients experience spontaneous vertigo (Baloh 1997)
bull Neuhauser criteria(2001) have been used to construct structured diagnostic interviews (Furman 2003)
bull Not always associated with headache
bull Motion sensitivity
bull Medication responsive
Sixth Annual Intensive Update in Neurology 0915-162016
69
ICHD-III beta Criteria for Vestibular Migraine
bull A At least five attacks fulfilling criteria C and D
bull B A current or past history of 11 migraine without aura or 12 migraine with aura
bull C Vestibular symptoms of moderate or severe intensity lasting between 5 minutes and 72 hours
bull D At least 50 associated with one of the following migrainous features
ndash 1 Headaches
ndash 2 Photophobia and phonophobia
ndash 3 Visual Aura
bull E Not better accounted for by another vestibular disorder
Sixth Annual Intensive Update in Neurology 0915-162016
70
Incidence of vertigo in MS
bull True vertigo (the perception of environmental
movement typically rotational) occurs in about
20 of MS patients and is the presenting
manifestation in 5
bull Up to 78 of MS patients report abnormalities
in balance at some point in their illness
Frohman et al Multiple Sclerosis 2003 9250
Sixth Annual Intensive Update in Neurology 0915-162016
71
Vertigo in MS due to demyelination
bull The most common neuroanatomic locations are the
root entry zone of CN VIII (giving absent calorics but
normal hearing and fixation suppression) and the
medial vestibular nucleus
bull Usually treated with steroids and vestibular
suppressants
Brandt 1993 Gass 1998 Thomke 1999
Sixth Annual Intensive Update in Neurology 0915-162016
72
Vertigo in MS
Frohmanrsquos large series (1153) reported that 52 of MS patients with acute vertigo had benign paroxysmal positional vertigo while 32 had new brainstem lesions
To avoid misdiagnoses and inappropriate treatment interventions alternative etiologies should be considered in all acute vertigo episodes in MS
E M Frohman Neurology 2000 and Multiple Sclerosis 2003
Sixth Annual Intensive Update in Neurology 0915-162016
73
Vestibular compensation
bull Acute unilateral or bilateral vestibular loss will prompt compensation thru central cervico-ocular visual and proprioceptive pathways
bull Unilateral loss may require several weeks for compensation to occur and activity especially head movements hastens compensation
bull Vestibular suppressant medication prolongs compensation
Sixth Annual Intensive Update in Neurology 0915-162016
74
Vestibular Rehabilitation
bull Performed by certified physical therapists many sites (Epic order vestibular PT) or check Vestibular Disorders of America website for providers
bull Assessment and treatment of benign positional vertigo
bull Directed rehab programs for central andor peripheral vestibular compensationndash Unilateral vestibulopathies
ndash Bilateral vestibular hypofunction
ndash Mutifactorial balance deficits and fall risk assessment
Sixth Annual Intensive Update in Neurology 0915-162016
75
Summary of whatrsquos new in peripheral vestibular disorders
bull Lateral semicircular canal benign paroxysmal positional vertigo presents with horizontal positioning nystagmus
bull Oral steroids appear to improve outcome in acute vestibular neuritis
bull Intratympanic steroids offer promise in Menierersquos syndrome
bull Superior semicircular canal dehiscence causes pressure and noise induced dizziness VEMP offers diagnostic information
Sixth Annual Intensive Update in Neurology 0915-162016
76
References
BPPVndash White J Benign paroxysmal positional vertigo Cleveland Clinic Journal of
Medicine 71(9)722-728 2004
ndash White J Coale K Catalano P Oas J Diagnosis and management of lateral semicircular canal benign positional vertigo Otolaryngology-Head and Neck Surgery 133 278-284 2005
ndash White J Savvides P Cherian N Oas J Canalith repositioning for benign paroxysmal positional vertigo Otology and Neurotology 26 704-710 2005
ndash White J Oas J Diagnosis and management of lateral semicircular canal conversions during particle repositioning therapy Laryngoscope 115(10) 1895-1897 2005
ndash Acute Vestibular Syndrome Hotson JR Baloh RW N Engl J Med 1998 Sep 3339(10)680-5
ndash Methylprednisolone valacyclovir or the combination for vestibular neuritis Strupp M Zingler VC Arbusow V Niklas D Maag KP Dieterich M Bense S Theil D Jahn K Brandt T N Engl J Med 2004 Jul 22351(4)354-61
ndash Intratympanic steroids for Meacuteniegraveres disease or syndrome Phillips JS Westerberg BCochrane Database Syst Rev 2011 Jul 6(7)CD008514 Review
ndash Sound- andor pressure-induced vertigo due to bone dehiscence of the superior semicircular canal Minor LB Solomon D Zinreich JS Zee DSArch Otolaryngol Head Neck Surg 1998 Mar124(3)249-58
Sixth Annual Intensive Update in Neurology 0915-162016
7
Neuro-otology Physical Exam
bull Otologic ndash check hearing and rule out active infection or
cholesteatoma
bull Oculomotor- saccade pursuit in vertical and horizontal
planes
bull Nystagmus ndash spontaneous gaze evoked and
positionalpositioning testing (best observed using
Frenzel lenses)
bull Special testing ndash head thrust head shake
Sixth Annual Intensive Update in Neurology 0915-162016
8
Peripheral Nystagmus
bull Jerk nystagmus (slow
and fast phase)
bull Direction fixed
bull Generally beats away
from affected ear
bull Worse in the
direction of gaze that
is towards the fast
phase
Sixth Annual Intensive Update in Neurology 0915-162016
9
Benign Paroxysmal Positional Vertigo (BPPV)
bull Vertigo of sudden onset and brief duration provoked by changes in head positionndash Lying down
ndash Rolling over in bed
ndash Bending over
ndash Looking up
ndash Washing hair in shower
ndash Dentist or beauty parlor
Sixth Annual Intensive Update in Neurology 0915-162016
10
Right Posterior BPPV
Sixth Annual Intensive Update in Neurology 0915-162016
11
IncidencePrevalence of BPPV
bull Most common referred diagnosis in tertiary centers
bull 9 of randomly selected community dwelling elderly
(Oghalai JS et al 2000)
bull Incidence increases 38 with each decade of life
(Froehling 1991)
Sixth Annual Intensive Update in Neurology 0915-162016
12
Benign Positional Vertigo (BPPV)
bull Diagnosed with lateral supine head turns and Dix-
Hallpike positioning
ndash Latency
ndash Duration
ndash Fatigue
ndash Habituation
Sixth Annual Intensive Update in Neurology 0915-162016
13
DHT - Sitting
Sixth Annual Intensive Update in Neurology 0915-162016
14
DHT - HHR Orientation
Sixth Annual Intensive Update in Neurology 0915-162016
15
Dix-Hallpike to the Right
Sixth Annual Intensive Update in Neurology 0915-162016
16
Right posterior BPPV
Sixth Annual Intensive Update in Neurology 0915-162016
17
Canalith Repositioning Procedure
Sixth Annual Intensive Update in Neurology 0915-162016
18
Canalith Repositioning Procedure
Sixth Annual Intensive Update in Neurology 0915-162016
19
Canalith Repositioning Procedure
Sixth Annual Intensive Update in Neurology 0915-162016
20
Canalith Repositioning Procedure
Sixth Annual Intensive Update in Neurology 0915-162016
21
Canalith Repositioning
bull R posterior
BPPV
CPT 95992
Sixth Annual Intensive Update in Neurology 0915-162016
22
Posterior semicircular canalith repositioning
Sixth Annual Intensive Update in Neurology 0915-162016
23
Supine Positional Tests
Sixth Annual Intensive Update in Neurology 0915-162016
24
Lateral SCC-BPPV
bull Short latency horizontal nystagmus provoked by supine bilateral head turns with prolonged duration and poor fatigability
bull Uncommon (2-15 of total BPPV)
bull Two formsndash Geotropic ndash beating towards undermost ear
ndash Apogeotropic ndash beating away from undermost ear
Sixth Annual Intensive Update in Neurology 0915-162016
25
Geotropic Lateral Canal BPPV
bull Patient is positioned
first to the right lateral
position ndash nystagmus
beats right
bull Patient is then
positioned to the left
lateral position and
nystagmus reverses
and beats left
Sixth Annual Intensive Update in Neurology 0915-162016
26
Left Geotropic Lateral Semicircular Canal BPPV
Sixth Annual Intensive Update in Neurology 0915-162016
27
Repositioning Maneuvers for Geotropic Lateral Semicircular Canal (LSC) BPPV
bull Roll maneuver (Lempert Baloh)
ndash Patient rolls in 90 degree increments towards the good side
completely rolling over R LSC-BPPV repositioning shown
Sixth Annual Intensive Update in Neurology 0915-162016
28
Repositioning Maneuvers for Geotropic LSC BPPV
bull Gufoni maneuver
ndash Patient lies quickly onto good side and turns face-down to
floor for two minutes
Sixth Annual Intensive Update in Neurology 0915-162016
29
Left Apogeotropic LSC BPPV
Sixth Annual Intensive Update in Neurology 0915-162016
30
Nystagmus observed During Repositioning
bull Nystagmus should remain in the same torsional
direction as seen on DHT throughout the treatment of
posterior BPPV
bull Reversal of nystagmus direction suggests the particle
has fallen back into the canal and predicts failure
bull In Lateral BPPV nystagmus should beat towards the
good ear
Sixth Annual Intensive Update in Neurology 0915-162016
31
Posterior to Lateral canal conversion
Sixth Annual Intensive Update in Neurology 0915-162016
32
R Posterior to Lateral Canal conversion - geotropic
Sixth Annual Intensive Update in Neurology 0915-162016
33
BPPV Recurrence
bull Recurrence is common (15yr)
bull Otoconial adherence or mineralization demineralization
abnormalities may contribute to recurrence
bull Home canalith repositioning is effective (Radke 2004)
Sixth Annual Intensive Update in Neurology 0915-162016
34
Question 1
bull The most likely semicircular canal to be affected in
BPPV is
ndash 1) superior
ndash 2) lateral
ndash 3) posterior
ndash 4) combined
Sixth Annual Intensive Update in Neurology 0915-162016
35
Question 1
bull The most likely semicircular canal to be affected in
BPPV is
ndash 1) superior
ndash 2) lateral
ndash 3) posterior
ndash 4) combined
Answer 3 See references at end of lecture
Sixth Annual Intensive Update in Neurology 0915-162016
36
Acute Vestibular Syndrome
bull Rapid unilateral injury to either peripheral or central
vestibular structures produces prolonged vertigo
(days to weeks)
bull Severe vertigo nausea and vomiting spontaneous
nystagmus and postural instability
Sixth Annual Intensive Update in Neurology 0915-162016
37
Acute Vestibular Syndrome
bull 25 of patients with risk factors who present with isolated severe vertigo nystagmus and postural instability in the ER have an acute infarction of the inferior cerebellum (AICA PICA) Central imaging is suggested (Baloh 1998 NEJM)
ndash HTN diabetes smoking age gt65 Afib valvular heart disease occlusive vascular disease
Sixth Annual Intensive Update in Neurology 0915-162016
38
HINTS test
bull Head Impulse
bull Nystagmus
bull Test of Skew
ndash More sensitive than MRI in the diagnosis of acute stroke in
patients with acute vestibular syndrome with at least one risk
factor (Kattah J et al Stroke 2009)
Sixth Annual Intensive Update in Neurology 0915-162016
39
Head Impulse Test
bull No special equipment needed
bull Examiner stands in front of seated patient and
randomly moves head left and right Patient is asked to
focus on examinerrsquos nose
bull Rapid low amplitude thrust from lateral to straight
ahead causes a compensatory saccade when
performed towards the weak side
bull This finding is a permanent feature of unilateral
vestibular loss
Sixth Annual Intensive Update in Neurology 0915-162016
40
Head Impulse Test
Sixth Annual Intensive Update in Neurology 0915-162016
41
Head CT ndash Academy of Emergency Medicine 2013
bull The clinical value of head CT in isolated
dizzinessvertigo without any associated neurological
symptoms is very low
bull Less than 1 of head CTs ordered for dizziness and
vertigo have significant abnormalities and those
patients had severe headache and neurological deficits
in addition to dizziness
bull Forgoing unnecessary emergency head CTs could
save 360 million dollars annually
Sixth Annual Intensive Update in Neurology 0915-162016
42
Question 2
Head CT is not indicated for the evaluation of dizziness
and vertigo without additional neurological complaints because
1 It is too expensive
2 Less than 1 of CTs done for dizziness and vertigo are
abnormal and those patients had associated severe
neurological complaints
3 CT is the best imaging choice for the posterior fossa
4 Acoustic neuromas are well seen on CT
Sixth Annual Intensive Update in Neurology 0915-162016
43
Question 2
Head CT is not indicated for the evaluation of dizziness
and vertigo without additional neurological complaints because
1 It is too expensive
2 Less than 1 of CTs done for dizziness and vertigo are
abnormal and those patients had associated severe
neurological complaints
3 CT is the best imaging choice for the posterior fossa
4 Acoustic neuromas are well seen on CT
Answer 2 See references at end of lecture
Sixth Annual Intensive Update in Neurology 0915-162016
44
Acute Vestibular Syndrome
bull Usually peripheral and can be associated with
hearing loss (labyrinthitis)
bull Timely diagnosis improves recovery (ASAP
prednisone 1mgkgday for ten days and taper 10
mgday ndash Strupp M 2004 NEJM)
bull Avoid vestibular suppressants after 3 days and begin
vestibular rehabilitation to hasten compensation
Sixth Annual Intensive Update in Neurology 0915-162016
45
Question 3
bull Treatment for Acute Vestibular Syndrome
1) oral prednisone 1 mgkgday
2) Treat dehydration if necessary
3) Minimize vestibular suppressants after 3 days
4) Consider vestibular physical therapy
5) All of the above
Sixth Annual Intensive Update in Neurology 0915-162016
46
Question 3
bull Treatment for Acute Vestibular Syndrome
1) oral prednisone 1 mgkgday
2) Treat dehydration if necessary
3) Minimize vestibular suppressants after 3 days
4) Consider vestibular physical therapy
5) All of the above
Answer 5 See references at end of lecture
Sixth Annual Intensive Update in Neurology 0915-162016
47
Menierersquos syndrome
bull Fluctuating low frequency hearing loss
bull Tinnitus andor aural fullness
bull Vertigo lasting hours
Sixth Annual Intensive Update in Neurology 0915-162016
48
Normal
Sixth Annual Intensive Update in Neurology 0915-162016
49
Hydrops
Sixth Annual Intensive Update in Neurology 0915-162016
50
Menierersquos syndrome
ndash Diagnosis- Exclusion (RO acoustic)
ndash 23 of Menierersquos patients will improve spontaneously
ndash Treatment-1500 mgday low sodium triamterene375-
hydrochlorothiazide 25 vestibular suppressants (lorazepam
1 mg tablet sublingual Q 8 prn attacks) off label use
Sixth Annual Intensive Update in Neurology 0915-162016
51
Menierersquos syndrome ndash intratympanic steroids
ndash A newer treatment option for those failing medical management
ndash 90 response rate (Boleus-Aguirre MS Otology Neurotology
2008)
ndash Outcomes data confirm excellent clinical response in 3941
Menierersquos patients treated with intratympanic steroids in 2008
Sixth Annual Intensive Update in Neurology 0915-162016
52
Question 4
bull Menierersquos syndrome diagnosis is based on
1) Recurrent vertigo lasting at least 20 minutes
2) Aural fullness
3) Documented hearing loss
4) Other causes excluded
5) All of the above
Sixth Annual Intensive Update in Neurology 0915-162016
53
Question 4
bull Menierersquos syndrome diagnosis is based on
1) Recurrent vertigo lasting at least 20 minutes
2) Aural fullness
3) Documented hearing loss
4) Other causes excluded
5) All of the above
Answer 5 See references at end
Sixth Annual Intensive Update in Neurology 0915-162016
54
Superior Semicircular Canal Dehiscence (SSCD)
bull Sound andor pressure induced oscillopsia
bull CT reformatted in the plane of the superior canals
shows dehiscence of overlying bone
bull Low frequency ldquoconductive hyperacusisrdquo with elevated
bone thresholds normal reflexes and a conductive loss
Sixth Annual Intensive Update in Neurology 0915-162016
55
SSCD
bull Positive middle ear pressure or loud sound stimulates ampullofugal endolymph flow -canal activation
bull Eye movements are tonic upwards with ipsilateral intorsion
Sixth Annual Intensive Update in Neurology 0915-162016
56
SSCD
bull Valsalva against a closed glottis or jugular compression causes increased intracranial pressure and ampullopetal endolymph flow -canal inhibition
bull Eye movements are tonic downwards with ipsilateral extorsion
Sixth Annual Intensive Update in Neurology 0915-162016
57
Reconstructed left superior semicircular canal
Sixth Annual Intensive Update in Neurology 0915-162016
58
Sixth Annual Intensive Update in Neurology 0915-162016
59
SCHEMATIC DRAWING OF AN OTOLITHIC MACULA
Sixth Annual Intensive Update in Neurology 0915-162016
60
MACULAE OF THE UTRICLESACCULE
Sixth Annual Intensive Update in Neurology 0915-162016
61
HAIR CELLS
Sixth Annual Intensive Update in Neurology 0915-162016
62
SCANNING ELECTRON MICROGRAPH OF OTOCONIA
Sixth Annual Intensive Update in Neurology 0915-162016
63
Ocular VEMP
bull Response to loud clicks (80-110 dB) or forehead tap (Halmagyi and Curthoys 2003)
bull Contralateral oculomotor nucleus (CN III)
bull Inferior oblique muscle contraction (8 msec) detected by 5sec event triggered averaging
bull Noninverting electrode inferior to eye
bull Inverting below non-inverting electrode
bull Ground to forehead
bull Normal subjects have very low response
Sixth Annual Intensive Update in Neurology 0915-162016
64
Clinical applications
bull The o-VEMP is a sensitive test of otolithutricular
function
bull Complements vestibular test procedures that assess
semicircular canals
bull Abnormally robust responses are associated with third
window abnormalities such as semicircular canal
dehiscence
bull Serves as an excellent screening test for semicircular
canal dehiscence
Sixth Annual Intensive Update in Neurology 0915-162016
65
Question 5
bull Superior semicircular canal dehiscence
1) Causes increased ability to hear vibratory sounds
2) Improves hearing
3) Causes meningitis
4) Always needs surgical repair
Sixth Annual Intensive Update in Neurology 0915-162016
66
Question 5
bull Superior semicircular canal dehiscence
1) Causes increased ability to hear vibratory sounds
2) Improves hearing
3) Causes meningitis
4) Always needs surgical repair
Answer 1 References at end of lecture
Sixth Annual Intensive Update in Neurology 0915-162016
67
Enlarged vestibular aqueduct
bull Axial temporal bone CT showing right temporal bone with enlarged vestibular aqueduct (arrow)
bull Vestibular symptoms accompany progressive sensorineural hearing loss in 4-50 of patients in selected series
Sixth Annual Intensive Update in Neurology 0915-162016
68
Migraine
bull Family or personal history of migraine
bull 25 of migraine patients experience spontaneous vertigo (Baloh 1997)
bull Neuhauser criteria(2001) have been used to construct structured diagnostic interviews (Furman 2003)
bull Not always associated with headache
bull Motion sensitivity
bull Medication responsive
Sixth Annual Intensive Update in Neurology 0915-162016
69
ICHD-III beta Criteria for Vestibular Migraine
bull A At least five attacks fulfilling criteria C and D
bull B A current or past history of 11 migraine without aura or 12 migraine with aura
bull C Vestibular symptoms of moderate or severe intensity lasting between 5 minutes and 72 hours
bull D At least 50 associated with one of the following migrainous features
ndash 1 Headaches
ndash 2 Photophobia and phonophobia
ndash 3 Visual Aura
bull E Not better accounted for by another vestibular disorder
Sixth Annual Intensive Update in Neurology 0915-162016
70
Incidence of vertigo in MS
bull True vertigo (the perception of environmental
movement typically rotational) occurs in about
20 of MS patients and is the presenting
manifestation in 5
bull Up to 78 of MS patients report abnormalities
in balance at some point in their illness
Frohman et al Multiple Sclerosis 2003 9250
Sixth Annual Intensive Update in Neurology 0915-162016
71
Vertigo in MS due to demyelination
bull The most common neuroanatomic locations are the
root entry zone of CN VIII (giving absent calorics but
normal hearing and fixation suppression) and the
medial vestibular nucleus
bull Usually treated with steroids and vestibular
suppressants
Brandt 1993 Gass 1998 Thomke 1999
Sixth Annual Intensive Update in Neurology 0915-162016
72
Vertigo in MS
Frohmanrsquos large series (1153) reported that 52 of MS patients with acute vertigo had benign paroxysmal positional vertigo while 32 had new brainstem lesions
To avoid misdiagnoses and inappropriate treatment interventions alternative etiologies should be considered in all acute vertigo episodes in MS
E M Frohman Neurology 2000 and Multiple Sclerosis 2003
Sixth Annual Intensive Update in Neurology 0915-162016
73
Vestibular compensation
bull Acute unilateral or bilateral vestibular loss will prompt compensation thru central cervico-ocular visual and proprioceptive pathways
bull Unilateral loss may require several weeks for compensation to occur and activity especially head movements hastens compensation
bull Vestibular suppressant medication prolongs compensation
Sixth Annual Intensive Update in Neurology 0915-162016
74
Vestibular Rehabilitation
bull Performed by certified physical therapists many sites (Epic order vestibular PT) or check Vestibular Disorders of America website for providers
bull Assessment and treatment of benign positional vertigo
bull Directed rehab programs for central andor peripheral vestibular compensationndash Unilateral vestibulopathies
ndash Bilateral vestibular hypofunction
ndash Mutifactorial balance deficits and fall risk assessment
Sixth Annual Intensive Update in Neurology 0915-162016
75
Summary of whatrsquos new in peripheral vestibular disorders
bull Lateral semicircular canal benign paroxysmal positional vertigo presents with horizontal positioning nystagmus
bull Oral steroids appear to improve outcome in acute vestibular neuritis
bull Intratympanic steroids offer promise in Menierersquos syndrome
bull Superior semicircular canal dehiscence causes pressure and noise induced dizziness VEMP offers diagnostic information
Sixth Annual Intensive Update in Neurology 0915-162016
76
References
BPPVndash White J Benign paroxysmal positional vertigo Cleveland Clinic Journal of
Medicine 71(9)722-728 2004
ndash White J Coale K Catalano P Oas J Diagnosis and management of lateral semicircular canal benign positional vertigo Otolaryngology-Head and Neck Surgery 133 278-284 2005
ndash White J Savvides P Cherian N Oas J Canalith repositioning for benign paroxysmal positional vertigo Otology and Neurotology 26 704-710 2005
ndash White J Oas J Diagnosis and management of lateral semicircular canal conversions during particle repositioning therapy Laryngoscope 115(10) 1895-1897 2005
ndash Acute Vestibular Syndrome Hotson JR Baloh RW N Engl J Med 1998 Sep 3339(10)680-5
ndash Methylprednisolone valacyclovir or the combination for vestibular neuritis Strupp M Zingler VC Arbusow V Niklas D Maag KP Dieterich M Bense S Theil D Jahn K Brandt T N Engl J Med 2004 Jul 22351(4)354-61
ndash Intratympanic steroids for Meacuteniegraveres disease or syndrome Phillips JS Westerberg BCochrane Database Syst Rev 2011 Jul 6(7)CD008514 Review
ndash Sound- andor pressure-induced vertigo due to bone dehiscence of the superior semicircular canal Minor LB Solomon D Zinreich JS Zee DSArch Otolaryngol Head Neck Surg 1998 Mar124(3)249-58
Sixth Annual Intensive Update in Neurology 0915-162016
8
Peripheral Nystagmus
bull Jerk nystagmus (slow
and fast phase)
bull Direction fixed
bull Generally beats away
from affected ear
bull Worse in the
direction of gaze that
is towards the fast
phase
Sixth Annual Intensive Update in Neurology 0915-162016
9
Benign Paroxysmal Positional Vertigo (BPPV)
bull Vertigo of sudden onset and brief duration provoked by changes in head positionndash Lying down
ndash Rolling over in bed
ndash Bending over
ndash Looking up
ndash Washing hair in shower
ndash Dentist or beauty parlor
Sixth Annual Intensive Update in Neurology 0915-162016
10
Right Posterior BPPV
Sixth Annual Intensive Update in Neurology 0915-162016
11
IncidencePrevalence of BPPV
bull Most common referred diagnosis in tertiary centers
bull 9 of randomly selected community dwelling elderly
(Oghalai JS et al 2000)
bull Incidence increases 38 with each decade of life
(Froehling 1991)
Sixth Annual Intensive Update in Neurology 0915-162016
12
Benign Positional Vertigo (BPPV)
bull Diagnosed with lateral supine head turns and Dix-
Hallpike positioning
ndash Latency
ndash Duration
ndash Fatigue
ndash Habituation
Sixth Annual Intensive Update in Neurology 0915-162016
13
DHT - Sitting
Sixth Annual Intensive Update in Neurology 0915-162016
14
DHT - HHR Orientation
Sixth Annual Intensive Update in Neurology 0915-162016
15
Dix-Hallpike to the Right
Sixth Annual Intensive Update in Neurology 0915-162016
16
Right posterior BPPV
Sixth Annual Intensive Update in Neurology 0915-162016
17
Canalith Repositioning Procedure
Sixth Annual Intensive Update in Neurology 0915-162016
18
Canalith Repositioning Procedure
Sixth Annual Intensive Update in Neurology 0915-162016
19
Canalith Repositioning Procedure
Sixth Annual Intensive Update in Neurology 0915-162016
20
Canalith Repositioning Procedure
Sixth Annual Intensive Update in Neurology 0915-162016
21
Canalith Repositioning
bull R posterior
BPPV
CPT 95992
Sixth Annual Intensive Update in Neurology 0915-162016
22
Posterior semicircular canalith repositioning
Sixth Annual Intensive Update in Neurology 0915-162016
23
Supine Positional Tests
Sixth Annual Intensive Update in Neurology 0915-162016
24
Lateral SCC-BPPV
bull Short latency horizontal nystagmus provoked by supine bilateral head turns with prolonged duration and poor fatigability
bull Uncommon (2-15 of total BPPV)
bull Two formsndash Geotropic ndash beating towards undermost ear
ndash Apogeotropic ndash beating away from undermost ear
Sixth Annual Intensive Update in Neurology 0915-162016
25
Geotropic Lateral Canal BPPV
bull Patient is positioned
first to the right lateral
position ndash nystagmus
beats right
bull Patient is then
positioned to the left
lateral position and
nystagmus reverses
and beats left
Sixth Annual Intensive Update in Neurology 0915-162016
26
Left Geotropic Lateral Semicircular Canal BPPV
Sixth Annual Intensive Update in Neurology 0915-162016
27
Repositioning Maneuvers for Geotropic Lateral Semicircular Canal (LSC) BPPV
bull Roll maneuver (Lempert Baloh)
ndash Patient rolls in 90 degree increments towards the good side
completely rolling over R LSC-BPPV repositioning shown
Sixth Annual Intensive Update in Neurology 0915-162016
28
Repositioning Maneuvers for Geotropic LSC BPPV
bull Gufoni maneuver
ndash Patient lies quickly onto good side and turns face-down to
floor for two minutes
Sixth Annual Intensive Update in Neurology 0915-162016
29
Left Apogeotropic LSC BPPV
Sixth Annual Intensive Update in Neurology 0915-162016
30
Nystagmus observed During Repositioning
bull Nystagmus should remain in the same torsional
direction as seen on DHT throughout the treatment of
posterior BPPV
bull Reversal of nystagmus direction suggests the particle
has fallen back into the canal and predicts failure
bull In Lateral BPPV nystagmus should beat towards the
good ear
Sixth Annual Intensive Update in Neurology 0915-162016
31
Posterior to Lateral canal conversion
Sixth Annual Intensive Update in Neurology 0915-162016
32
R Posterior to Lateral Canal conversion - geotropic
Sixth Annual Intensive Update in Neurology 0915-162016
33
BPPV Recurrence
bull Recurrence is common (15yr)
bull Otoconial adherence or mineralization demineralization
abnormalities may contribute to recurrence
bull Home canalith repositioning is effective (Radke 2004)
Sixth Annual Intensive Update in Neurology 0915-162016
34
Question 1
bull The most likely semicircular canal to be affected in
BPPV is
ndash 1) superior
ndash 2) lateral
ndash 3) posterior
ndash 4) combined
Sixth Annual Intensive Update in Neurology 0915-162016
35
Question 1
bull The most likely semicircular canal to be affected in
BPPV is
ndash 1) superior
ndash 2) lateral
ndash 3) posterior
ndash 4) combined
Answer 3 See references at end of lecture
Sixth Annual Intensive Update in Neurology 0915-162016
36
Acute Vestibular Syndrome
bull Rapid unilateral injury to either peripheral or central
vestibular structures produces prolonged vertigo
(days to weeks)
bull Severe vertigo nausea and vomiting spontaneous
nystagmus and postural instability
Sixth Annual Intensive Update in Neurology 0915-162016
37
Acute Vestibular Syndrome
bull 25 of patients with risk factors who present with isolated severe vertigo nystagmus and postural instability in the ER have an acute infarction of the inferior cerebellum (AICA PICA) Central imaging is suggested (Baloh 1998 NEJM)
ndash HTN diabetes smoking age gt65 Afib valvular heart disease occlusive vascular disease
Sixth Annual Intensive Update in Neurology 0915-162016
38
HINTS test
bull Head Impulse
bull Nystagmus
bull Test of Skew
ndash More sensitive than MRI in the diagnosis of acute stroke in
patients with acute vestibular syndrome with at least one risk
factor (Kattah J et al Stroke 2009)
Sixth Annual Intensive Update in Neurology 0915-162016
39
Head Impulse Test
bull No special equipment needed
bull Examiner stands in front of seated patient and
randomly moves head left and right Patient is asked to
focus on examinerrsquos nose
bull Rapid low amplitude thrust from lateral to straight
ahead causes a compensatory saccade when
performed towards the weak side
bull This finding is a permanent feature of unilateral
vestibular loss
Sixth Annual Intensive Update in Neurology 0915-162016
40
Head Impulse Test
Sixth Annual Intensive Update in Neurology 0915-162016
41
Head CT ndash Academy of Emergency Medicine 2013
bull The clinical value of head CT in isolated
dizzinessvertigo without any associated neurological
symptoms is very low
bull Less than 1 of head CTs ordered for dizziness and
vertigo have significant abnormalities and those
patients had severe headache and neurological deficits
in addition to dizziness
bull Forgoing unnecessary emergency head CTs could
save 360 million dollars annually
Sixth Annual Intensive Update in Neurology 0915-162016
42
Question 2
Head CT is not indicated for the evaluation of dizziness
and vertigo without additional neurological complaints because
1 It is too expensive
2 Less than 1 of CTs done for dizziness and vertigo are
abnormal and those patients had associated severe
neurological complaints
3 CT is the best imaging choice for the posterior fossa
4 Acoustic neuromas are well seen on CT
Sixth Annual Intensive Update in Neurology 0915-162016
43
Question 2
Head CT is not indicated for the evaluation of dizziness
and vertigo without additional neurological complaints because
1 It is too expensive
2 Less than 1 of CTs done for dizziness and vertigo are
abnormal and those patients had associated severe
neurological complaints
3 CT is the best imaging choice for the posterior fossa
4 Acoustic neuromas are well seen on CT
Answer 2 See references at end of lecture
Sixth Annual Intensive Update in Neurology 0915-162016
44
Acute Vestibular Syndrome
bull Usually peripheral and can be associated with
hearing loss (labyrinthitis)
bull Timely diagnosis improves recovery (ASAP
prednisone 1mgkgday for ten days and taper 10
mgday ndash Strupp M 2004 NEJM)
bull Avoid vestibular suppressants after 3 days and begin
vestibular rehabilitation to hasten compensation
Sixth Annual Intensive Update in Neurology 0915-162016
45
Question 3
bull Treatment for Acute Vestibular Syndrome
1) oral prednisone 1 mgkgday
2) Treat dehydration if necessary
3) Minimize vestibular suppressants after 3 days
4) Consider vestibular physical therapy
5) All of the above
Sixth Annual Intensive Update in Neurology 0915-162016
46
Question 3
bull Treatment for Acute Vestibular Syndrome
1) oral prednisone 1 mgkgday
2) Treat dehydration if necessary
3) Minimize vestibular suppressants after 3 days
4) Consider vestibular physical therapy
5) All of the above
Answer 5 See references at end of lecture
Sixth Annual Intensive Update in Neurology 0915-162016
47
Menierersquos syndrome
bull Fluctuating low frequency hearing loss
bull Tinnitus andor aural fullness
bull Vertigo lasting hours
Sixth Annual Intensive Update in Neurology 0915-162016
48
Normal
Sixth Annual Intensive Update in Neurology 0915-162016
49
Hydrops
Sixth Annual Intensive Update in Neurology 0915-162016
50
Menierersquos syndrome
ndash Diagnosis- Exclusion (RO acoustic)
ndash 23 of Menierersquos patients will improve spontaneously
ndash Treatment-1500 mgday low sodium triamterene375-
hydrochlorothiazide 25 vestibular suppressants (lorazepam
1 mg tablet sublingual Q 8 prn attacks) off label use
Sixth Annual Intensive Update in Neurology 0915-162016
51
Menierersquos syndrome ndash intratympanic steroids
ndash A newer treatment option for those failing medical management
ndash 90 response rate (Boleus-Aguirre MS Otology Neurotology
2008)
ndash Outcomes data confirm excellent clinical response in 3941
Menierersquos patients treated with intratympanic steroids in 2008
Sixth Annual Intensive Update in Neurology 0915-162016
52
Question 4
bull Menierersquos syndrome diagnosis is based on
1) Recurrent vertigo lasting at least 20 minutes
2) Aural fullness
3) Documented hearing loss
4) Other causes excluded
5) All of the above
Sixth Annual Intensive Update in Neurology 0915-162016
53
Question 4
bull Menierersquos syndrome diagnosis is based on
1) Recurrent vertigo lasting at least 20 minutes
2) Aural fullness
3) Documented hearing loss
4) Other causes excluded
5) All of the above
Answer 5 See references at end
Sixth Annual Intensive Update in Neurology 0915-162016
54
Superior Semicircular Canal Dehiscence (SSCD)
bull Sound andor pressure induced oscillopsia
bull CT reformatted in the plane of the superior canals
shows dehiscence of overlying bone
bull Low frequency ldquoconductive hyperacusisrdquo with elevated
bone thresholds normal reflexes and a conductive loss
Sixth Annual Intensive Update in Neurology 0915-162016
55
SSCD
bull Positive middle ear pressure or loud sound stimulates ampullofugal endolymph flow -canal activation
bull Eye movements are tonic upwards with ipsilateral intorsion
Sixth Annual Intensive Update in Neurology 0915-162016
56
SSCD
bull Valsalva against a closed glottis or jugular compression causes increased intracranial pressure and ampullopetal endolymph flow -canal inhibition
bull Eye movements are tonic downwards with ipsilateral extorsion
Sixth Annual Intensive Update in Neurology 0915-162016
57
Reconstructed left superior semicircular canal
Sixth Annual Intensive Update in Neurology 0915-162016
58
Sixth Annual Intensive Update in Neurology 0915-162016
59
SCHEMATIC DRAWING OF AN OTOLITHIC MACULA
Sixth Annual Intensive Update in Neurology 0915-162016
60
MACULAE OF THE UTRICLESACCULE
Sixth Annual Intensive Update in Neurology 0915-162016
61
HAIR CELLS
Sixth Annual Intensive Update in Neurology 0915-162016
62
SCANNING ELECTRON MICROGRAPH OF OTOCONIA
Sixth Annual Intensive Update in Neurology 0915-162016
63
Ocular VEMP
bull Response to loud clicks (80-110 dB) or forehead tap (Halmagyi and Curthoys 2003)
bull Contralateral oculomotor nucleus (CN III)
bull Inferior oblique muscle contraction (8 msec) detected by 5sec event triggered averaging
bull Noninverting electrode inferior to eye
bull Inverting below non-inverting electrode
bull Ground to forehead
bull Normal subjects have very low response
Sixth Annual Intensive Update in Neurology 0915-162016
64
Clinical applications
bull The o-VEMP is a sensitive test of otolithutricular
function
bull Complements vestibular test procedures that assess
semicircular canals
bull Abnormally robust responses are associated with third
window abnormalities such as semicircular canal
dehiscence
bull Serves as an excellent screening test for semicircular
canal dehiscence
Sixth Annual Intensive Update in Neurology 0915-162016
65
Question 5
bull Superior semicircular canal dehiscence
1) Causes increased ability to hear vibratory sounds
2) Improves hearing
3) Causes meningitis
4) Always needs surgical repair
Sixth Annual Intensive Update in Neurology 0915-162016
66
Question 5
bull Superior semicircular canal dehiscence
1) Causes increased ability to hear vibratory sounds
2) Improves hearing
3) Causes meningitis
4) Always needs surgical repair
Answer 1 References at end of lecture
Sixth Annual Intensive Update in Neurology 0915-162016
67
Enlarged vestibular aqueduct
bull Axial temporal bone CT showing right temporal bone with enlarged vestibular aqueduct (arrow)
bull Vestibular symptoms accompany progressive sensorineural hearing loss in 4-50 of patients in selected series
Sixth Annual Intensive Update in Neurology 0915-162016
68
Migraine
bull Family or personal history of migraine
bull 25 of migraine patients experience spontaneous vertigo (Baloh 1997)
bull Neuhauser criteria(2001) have been used to construct structured diagnostic interviews (Furman 2003)
bull Not always associated with headache
bull Motion sensitivity
bull Medication responsive
Sixth Annual Intensive Update in Neurology 0915-162016
69
ICHD-III beta Criteria for Vestibular Migraine
bull A At least five attacks fulfilling criteria C and D
bull B A current or past history of 11 migraine without aura or 12 migraine with aura
bull C Vestibular symptoms of moderate or severe intensity lasting between 5 minutes and 72 hours
bull D At least 50 associated with one of the following migrainous features
ndash 1 Headaches
ndash 2 Photophobia and phonophobia
ndash 3 Visual Aura
bull E Not better accounted for by another vestibular disorder
Sixth Annual Intensive Update in Neurology 0915-162016
70
Incidence of vertigo in MS
bull True vertigo (the perception of environmental
movement typically rotational) occurs in about
20 of MS patients and is the presenting
manifestation in 5
bull Up to 78 of MS patients report abnormalities
in balance at some point in their illness
Frohman et al Multiple Sclerosis 2003 9250
Sixth Annual Intensive Update in Neurology 0915-162016
71
Vertigo in MS due to demyelination
bull The most common neuroanatomic locations are the
root entry zone of CN VIII (giving absent calorics but
normal hearing and fixation suppression) and the
medial vestibular nucleus
bull Usually treated with steroids and vestibular
suppressants
Brandt 1993 Gass 1998 Thomke 1999
Sixth Annual Intensive Update in Neurology 0915-162016
72
Vertigo in MS
Frohmanrsquos large series (1153) reported that 52 of MS patients with acute vertigo had benign paroxysmal positional vertigo while 32 had new brainstem lesions
To avoid misdiagnoses and inappropriate treatment interventions alternative etiologies should be considered in all acute vertigo episodes in MS
E M Frohman Neurology 2000 and Multiple Sclerosis 2003
Sixth Annual Intensive Update in Neurology 0915-162016
73
Vestibular compensation
bull Acute unilateral or bilateral vestibular loss will prompt compensation thru central cervico-ocular visual and proprioceptive pathways
bull Unilateral loss may require several weeks for compensation to occur and activity especially head movements hastens compensation
bull Vestibular suppressant medication prolongs compensation
Sixth Annual Intensive Update in Neurology 0915-162016
74
Vestibular Rehabilitation
bull Performed by certified physical therapists many sites (Epic order vestibular PT) or check Vestibular Disorders of America website for providers
bull Assessment and treatment of benign positional vertigo
bull Directed rehab programs for central andor peripheral vestibular compensationndash Unilateral vestibulopathies
ndash Bilateral vestibular hypofunction
ndash Mutifactorial balance deficits and fall risk assessment
Sixth Annual Intensive Update in Neurology 0915-162016
75
Summary of whatrsquos new in peripheral vestibular disorders
bull Lateral semicircular canal benign paroxysmal positional vertigo presents with horizontal positioning nystagmus
bull Oral steroids appear to improve outcome in acute vestibular neuritis
bull Intratympanic steroids offer promise in Menierersquos syndrome
bull Superior semicircular canal dehiscence causes pressure and noise induced dizziness VEMP offers diagnostic information
Sixth Annual Intensive Update in Neurology 0915-162016
76
References
BPPVndash White J Benign paroxysmal positional vertigo Cleveland Clinic Journal of
Medicine 71(9)722-728 2004
ndash White J Coale K Catalano P Oas J Diagnosis and management of lateral semicircular canal benign positional vertigo Otolaryngology-Head and Neck Surgery 133 278-284 2005
ndash White J Savvides P Cherian N Oas J Canalith repositioning for benign paroxysmal positional vertigo Otology and Neurotology 26 704-710 2005
ndash White J Oas J Diagnosis and management of lateral semicircular canal conversions during particle repositioning therapy Laryngoscope 115(10) 1895-1897 2005
ndash Acute Vestibular Syndrome Hotson JR Baloh RW N Engl J Med 1998 Sep 3339(10)680-5
ndash Methylprednisolone valacyclovir or the combination for vestibular neuritis Strupp M Zingler VC Arbusow V Niklas D Maag KP Dieterich M Bense S Theil D Jahn K Brandt T N Engl J Med 2004 Jul 22351(4)354-61
ndash Intratympanic steroids for Meacuteniegraveres disease or syndrome Phillips JS Westerberg BCochrane Database Syst Rev 2011 Jul 6(7)CD008514 Review
ndash Sound- andor pressure-induced vertigo due to bone dehiscence of the superior semicircular canal Minor LB Solomon D Zinreich JS Zee DSArch Otolaryngol Head Neck Surg 1998 Mar124(3)249-58
Sixth Annual Intensive Update in Neurology 0915-162016
9
Benign Paroxysmal Positional Vertigo (BPPV)
bull Vertigo of sudden onset and brief duration provoked by changes in head positionndash Lying down
ndash Rolling over in bed
ndash Bending over
ndash Looking up
ndash Washing hair in shower
ndash Dentist or beauty parlor
Sixth Annual Intensive Update in Neurology 0915-162016
10
Right Posterior BPPV
Sixth Annual Intensive Update in Neurology 0915-162016
11
IncidencePrevalence of BPPV
bull Most common referred diagnosis in tertiary centers
bull 9 of randomly selected community dwelling elderly
(Oghalai JS et al 2000)
bull Incidence increases 38 with each decade of life
(Froehling 1991)
Sixth Annual Intensive Update in Neurology 0915-162016
12
Benign Positional Vertigo (BPPV)
bull Diagnosed with lateral supine head turns and Dix-
Hallpike positioning
ndash Latency
ndash Duration
ndash Fatigue
ndash Habituation
Sixth Annual Intensive Update in Neurology 0915-162016
13
DHT - Sitting
Sixth Annual Intensive Update in Neurology 0915-162016
14
DHT - HHR Orientation
Sixth Annual Intensive Update in Neurology 0915-162016
15
Dix-Hallpike to the Right
Sixth Annual Intensive Update in Neurology 0915-162016
16
Right posterior BPPV
Sixth Annual Intensive Update in Neurology 0915-162016
17
Canalith Repositioning Procedure
Sixth Annual Intensive Update in Neurology 0915-162016
18
Canalith Repositioning Procedure
Sixth Annual Intensive Update in Neurology 0915-162016
19
Canalith Repositioning Procedure
Sixth Annual Intensive Update in Neurology 0915-162016
20
Canalith Repositioning Procedure
Sixth Annual Intensive Update in Neurology 0915-162016
21
Canalith Repositioning
bull R posterior
BPPV
CPT 95992
Sixth Annual Intensive Update in Neurology 0915-162016
22
Posterior semicircular canalith repositioning
Sixth Annual Intensive Update in Neurology 0915-162016
23
Supine Positional Tests
Sixth Annual Intensive Update in Neurology 0915-162016
24
Lateral SCC-BPPV
bull Short latency horizontal nystagmus provoked by supine bilateral head turns with prolonged duration and poor fatigability
bull Uncommon (2-15 of total BPPV)
bull Two formsndash Geotropic ndash beating towards undermost ear
ndash Apogeotropic ndash beating away from undermost ear
Sixth Annual Intensive Update in Neurology 0915-162016
25
Geotropic Lateral Canal BPPV
bull Patient is positioned
first to the right lateral
position ndash nystagmus
beats right
bull Patient is then
positioned to the left
lateral position and
nystagmus reverses
and beats left
Sixth Annual Intensive Update in Neurology 0915-162016
26
Left Geotropic Lateral Semicircular Canal BPPV
Sixth Annual Intensive Update in Neurology 0915-162016
27
Repositioning Maneuvers for Geotropic Lateral Semicircular Canal (LSC) BPPV
bull Roll maneuver (Lempert Baloh)
ndash Patient rolls in 90 degree increments towards the good side
completely rolling over R LSC-BPPV repositioning shown
Sixth Annual Intensive Update in Neurology 0915-162016
28
Repositioning Maneuvers for Geotropic LSC BPPV
bull Gufoni maneuver
ndash Patient lies quickly onto good side and turns face-down to
floor for two minutes
Sixth Annual Intensive Update in Neurology 0915-162016
29
Left Apogeotropic LSC BPPV
Sixth Annual Intensive Update in Neurology 0915-162016
30
Nystagmus observed During Repositioning
bull Nystagmus should remain in the same torsional
direction as seen on DHT throughout the treatment of
posterior BPPV
bull Reversal of nystagmus direction suggests the particle
has fallen back into the canal and predicts failure
bull In Lateral BPPV nystagmus should beat towards the
good ear
Sixth Annual Intensive Update in Neurology 0915-162016
31
Posterior to Lateral canal conversion
Sixth Annual Intensive Update in Neurology 0915-162016
32
R Posterior to Lateral Canal conversion - geotropic
Sixth Annual Intensive Update in Neurology 0915-162016
33
BPPV Recurrence
bull Recurrence is common (15yr)
bull Otoconial adherence or mineralization demineralization
abnormalities may contribute to recurrence
bull Home canalith repositioning is effective (Radke 2004)
Sixth Annual Intensive Update in Neurology 0915-162016
34
Question 1
bull The most likely semicircular canal to be affected in
BPPV is
ndash 1) superior
ndash 2) lateral
ndash 3) posterior
ndash 4) combined
Sixth Annual Intensive Update in Neurology 0915-162016
35
Question 1
bull The most likely semicircular canal to be affected in
BPPV is
ndash 1) superior
ndash 2) lateral
ndash 3) posterior
ndash 4) combined
Answer 3 See references at end of lecture
Sixth Annual Intensive Update in Neurology 0915-162016
36
Acute Vestibular Syndrome
bull Rapid unilateral injury to either peripheral or central
vestibular structures produces prolonged vertigo
(days to weeks)
bull Severe vertigo nausea and vomiting spontaneous
nystagmus and postural instability
Sixth Annual Intensive Update in Neurology 0915-162016
37
Acute Vestibular Syndrome
bull 25 of patients with risk factors who present with isolated severe vertigo nystagmus and postural instability in the ER have an acute infarction of the inferior cerebellum (AICA PICA) Central imaging is suggested (Baloh 1998 NEJM)
ndash HTN diabetes smoking age gt65 Afib valvular heart disease occlusive vascular disease
Sixth Annual Intensive Update in Neurology 0915-162016
38
HINTS test
bull Head Impulse
bull Nystagmus
bull Test of Skew
ndash More sensitive than MRI in the diagnosis of acute stroke in
patients with acute vestibular syndrome with at least one risk
factor (Kattah J et al Stroke 2009)
Sixth Annual Intensive Update in Neurology 0915-162016
39
Head Impulse Test
bull No special equipment needed
bull Examiner stands in front of seated patient and
randomly moves head left and right Patient is asked to
focus on examinerrsquos nose
bull Rapid low amplitude thrust from lateral to straight
ahead causes a compensatory saccade when
performed towards the weak side
bull This finding is a permanent feature of unilateral
vestibular loss
Sixth Annual Intensive Update in Neurology 0915-162016
40
Head Impulse Test
Sixth Annual Intensive Update in Neurology 0915-162016
41
Head CT ndash Academy of Emergency Medicine 2013
bull The clinical value of head CT in isolated
dizzinessvertigo without any associated neurological
symptoms is very low
bull Less than 1 of head CTs ordered for dizziness and
vertigo have significant abnormalities and those
patients had severe headache and neurological deficits
in addition to dizziness
bull Forgoing unnecessary emergency head CTs could
save 360 million dollars annually
Sixth Annual Intensive Update in Neurology 0915-162016
42
Question 2
Head CT is not indicated for the evaluation of dizziness
and vertigo without additional neurological complaints because
1 It is too expensive
2 Less than 1 of CTs done for dizziness and vertigo are
abnormal and those patients had associated severe
neurological complaints
3 CT is the best imaging choice for the posterior fossa
4 Acoustic neuromas are well seen on CT
Sixth Annual Intensive Update in Neurology 0915-162016
43
Question 2
Head CT is not indicated for the evaluation of dizziness
and vertigo without additional neurological complaints because
1 It is too expensive
2 Less than 1 of CTs done for dizziness and vertigo are
abnormal and those patients had associated severe
neurological complaints
3 CT is the best imaging choice for the posterior fossa
4 Acoustic neuromas are well seen on CT
Answer 2 See references at end of lecture
Sixth Annual Intensive Update in Neurology 0915-162016
44
Acute Vestibular Syndrome
bull Usually peripheral and can be associated with
hearing loss (labyrinthitis)
bull Timely diagnosis improves recovery (ASAP
prednisone 1mgkgday for ten days and taper 10
mgday ndash Strupp M 2004 NEJM)
bull Avoid vestibular suppressants after 3 days and begin
vestibular rehabilitation to hasten compensation
Sixth Annual Intensive Update in Neurology 0915-162016
45
Question 3
bull Treatment for Acute Vestibular Syndrome
1) oral prednisone 1 mgkgday
2) Treat dehydration if necessary
3) Minimize vestibular suppressants after 3 days
4) Consider vestibular physical therapy
5) All of the above
Sixth Annual Intensive Update in Neurology 0915-162016
46
Question 3
bull Treatment for Acute Vestibular Syndrome
1) oral prednisone 1 mgkgday
2) Treat dehydration if necessary
3) Minimize vestibular suppressants after 3 days
4) Consider vestibular physical therapy
5) All of the above
Answer 5 See references at end of lecture
Sixth Annual Intensive Update in Neurology 0915-162016
47
Menierersquos syndrome
bull Fluctuating low frequency hearing loss
bull Tinnitus andor aural fullness
bull Vertigo lasting hours
Sixth Annual Intensive Update in Neurology 0915-162016
48
Normal
Sixth Annual Intensive Update in Neurology 0915-162016
49
Hydrops
Sixth Annual Intensive Update in Neurology 0915-162016
50
Menierersquos syndrome
ndash Diagnosis- Exclusion (RO acoustic)
ndash 23 of Menierersquos patients will improve spontaneously
ndash Treatment-1500 mgday low sodium triamterene375-
hydrochlorothiazide 25 vestibular suppressants (lorazepam
1 mg tablet sublingual Q 8 prn attacks) off label use
Sixth Annual Intensive Update in Neurology 0915-162016
51
Menierersquos syndrome ndash intratympanic steroids
ndash A newer treatment option for those failing medical management
ndash 90 response rate (Boleus-Aguirre MS Otology Neurotology
2008)
ndash Outcomes data confirm excellent clinical response in 3941
Menierersquos patients treated with intratympanic steroids in 2008
Sixth Annual Intensive Update in Neurology 0915-162016
52
Question 4
bull Menierersquos syndrome diagnosis is based on
1) Recurrent vertigo lasting at least 20 minutes
2) Aural fullness
3) Documented hearing loss
4) Other causes excluded
5) All of the above
Sixth Annual Intensive Update in Neurology 0915-162016
53
Question 4
bull Menierersquos syndrome diagnosis is based on
1) Recurrent vertigo lasting at least 20 minutes
2) Aural fullness
3) Documented hearing loss
4) Other causes excluded
5) All of the above
Answer 5 See references at end
Sixth Annual Intensive Update in Neurology 0915-162016
54
Superior Semicircular Canal Dehiscence (SSCD)
bull Sound andor pressure induced oscillopsia
bull CT reformatted in the plane of the superior canals
shows dehiscence of overlying bone
bull Low frequency ldquoconductive hyperacusisrdquo with elevated
bone thresholds normal reflexes and a conductive loss
Sixth Annual Intensive Update in Neurology 0915-162016
55
SSCD
bull Positive middle ear pressure or loud sound stimulates ampullofugal endolymph flow -canal activation
bull Eye movements are tonic upwards with ipsilateral intorsion
Sixth Annual Intensive Update in Neurology 0915-162016
56
SSCD
bull Valsalva against a closed glottis or jugular compression causes increased intracranial pressure and ampullopetal endolymph flow -canal inhibition
bull Eye movements are tonic downwards with ipsilateral extorsion
Sixth Annual Intensive Update in Neurology 0915-162016
57
Reconstructed left superior semicircular canal
Sixth Annual Intensive Update in Neurology 0915-162016
58
Sixth Annual Intensive Update in Neurology 0915-162016
59
SCHEMATIC DRAWING OF AN OTOLITHIC MACULA
Sixth Annual Intensive Update in Neurology 0915-162016
60
MACULAE OF THE UTRICLESACCULE
Sixth Annual Intensive Update in Neurology 0915-162016
61
HAIR CELLS
Sixth Annual Intensive Update in Neurology 0915-162016
62
SCANNING ELECTRON MICROGRAPH OF OTOCONIA
Sixth Annual Intensive Update in Neurology 0915-162016
63
Ocular VEMP
bull Response to loud clicks (80-110 dB) or forehead tap (Halmagyi and Curthoys 2003)
bull Contralateral oculomotor nucleus (CN III)
bull Inferior oblique muscle contraction (8 msec) detected by 5sec event triggered averaging
bull Noninverting electrode inferior to eye
bull Inverting below non-inverting electrode
bull Ground to forehead
bull Normal subjects have very low response
Sixth Annual Intensive Update in Neurology 0915-162016
64
Clinical applications
bull The o-VEMP is a sensitive test of otolithutricular
function
bull Complements vestibular test procedures that assess
semicircular canals
bull Abnormally robust responses are associated with third
window abnormalities such as semicircular canal
dehiscence
bull Serves as an excellent screening test for semicircular
canal dehiscence
Sixth Annual Intensive Update in Neurology 0915-162016
65
Question 5
bull Superior semicircular canal dehiscence
1) Causes increased ability to hear vibratory sounds
2) Improves hearing
3) Causes meningitis
4) Always needs surgical repair
Sixth Annual Intensive Update in Neurology 0915-162016
66
Question 5
bull Superior semicircular canal dehiscence
1) Causes increased ability to hear vibratory sounds
2) Improves hearing
3) Causes meningitis
4) Always needs surgical repair
Answer 1 References at end of lecture
Sixth Annual Intensive Update in Neurology 0915-162016
67
Enlarged vestibular aqueduct
bull Axial temporal bone CT showing right temporal bone with enlarged vestibular aqueduct (arrow)
bull Vestibular symptoms accompany progressive sensorineural hearing loss in 4-50 of patients in selected series
Sixth Annual Intensive Update in Neurology 0915-162016
68
Migraine
bull Family or personal history of migraine
bull 25 of migraine patients experience spontaneous vertigo (Baloh 1997)
bull Neuhauser criteria(2001) have been used to construct structured diagnostic interviews (Furman 2003)
bull Not always associated with headache
bull Motion sensitivity
bull Medication responsive
Sixth Annual Intensive Update in Neurology 0915-162016
69
ICHD-III beta Criteria for Vestibular Migraine
bull A At least five attacks fulfilling criteria C and D
bull B A current or past history of 11 migraine without aura or 12 migraine with aura
bull C Vestibular symptoms of moderate or severe intensity lasting between 5 minutes and 72 hours
bull D At least 50 associated with one of the following migrainous features
ndash 1 Headaches
ndash 2 Photophobia and phonophobia
ndash 3 Visual Aura
bull E Not better accounted for by another vestibular disorder
Sixth Annual Intensive Update in Neurology 0915-162016
70
Incidence of vertigo in MS
bull True vertigo (the perception of environmental
movement typically rotational) occurs in about
20 of MS patients and is the presenting
manifestation in 5
bull Up to 78 of MS patients report abnormalities
in balance at some point in their illness
Frohman et al Multiple Sclerosis 2003 9250
Sixth Annual Intensive Update in Neurology 0915-162016
71
Vertigo in MS due to demyelination
bull The most common neuroanatomic locations are the
root entry zone of CN VIII (giving absent calorics but
normal hearing and fixation suppression) and the
medial vestibular nucleus
bull Usually treated with steroids and vestibular
suppressants
Brandt 1993 Gass 1998 Thomke 1999
Sixth Annual Intensive Update in Neurology 0915-162016
72
Vertigo in MS
Frohmanrsquos large series (1153) reported that 52 of MS patients with acute vertigo had benign paroxysmal positional vertigo while 32 had new brainstem lesions
To avoid misdiagnoses and inappropriate treatment interventions alternative etiologies should be considered in all acute vertigo episodes in MS
E M Frohman Neurology 2000 and Multiple Sclerosis 2003
Sixth Annual Intensive Update in Neurology 0915-162016
73
Vestibular compensation
bull Acute unilateral or bilateral vestibular loss will prompt compensation thru central cervico-ocular visual and proprioceptive pathways
bull Unilateral loss may require several weeks for compensation to occur and activity especially head movements hastens compensation
bull Vestibular suppressant medication prolongs compensation
Sixth Annual Intensive Update in Neurology 0915-162016
74
Vestibular Rehabilitation
bull Performed by certified physical therapists many sites (Epic order vestibular PT) or check Vestibular Disorders of America website for providers
bull Assessment and treatment of benign positional vertigo
bull Directed rehab programs for central andor peripheral vestibular compensationndash Unilateral vestibulopathies
ndash Bilateral vestibular hypofunction
ndash Mutifactorial balance deficits and fall risk assessment
Sixth Annual Intensive Update in Neurology 0915-162016
75
Summary of whatrsquos new in peripheral vestibular disorders
bull Lateral semicircular canal benign paroxysmal positional vertigo presents with horizontal positioning nystagmus
bull Oral steroids appear to improve outcome in acute vestibular neuritis
bull Intratympanic steroids offer promise in Menierersquos syndrome
bull Superior semicircular canal dehiscence causes pressure and noise induced dizziness VEMP offers diagnostic information
Sixth Annual Intensive Update in Neurology 0915-162016
76
References
BPPVndash White J Benign paroxysmal positional vertigo Cleveland Clinic Journal of
Medicine 71(9)722-728 2004
ndash White J Coale K Catalano P Oas J Diagnosis and management of lateral semicircular canal benign positional vertigo Otolaryngology-Head and Neck Surgery 133 278-284 2005
ndash White J Savvides P Cherian N Oas J Canalith repositioning for benign paroxysmal positional vertigo Otology and Neurotology 26 704-710 2005
ndash White J Oas J Diagnosis and management of lateral semicircular canal conversions during particle repositioning therapy Laryngoscope 115(10) 1895-1897 2005
ndash Acute Vestibular Syndrome Hotson JR Baloh RW N Engl J Med 1998 Sep 3339(10)680-5
ndash Methylprednisolone valacyclovir or the combination for vestibular neuritis Strupp M Zingler VC Arbusow V Niklas D Maag KP Dieterich M Bense S Theil D Jahn K Brandt T N Engl J Med 2004 Jul 22351(4)354-61
ndash Intratympanic steroids for Meacuteniegraveres disease or syndrome Phillips JS Westerberg BCochrane Database Syst Rev 2011 Jul 6(7)CD008514 Review
ndash Sound- andor pressure-induced vertigo due to bone dehiscence of the superior semicircular canal Minor LB Solomon D Zinreich JS Zee DSArch Otolaryngol Head Neck Surg 1998 Mar124(3)249-58
Sixth Annual Intensive Update in Neurology 0915-162016
10
Right Posterior BPPV
Sixth Annual Intensive Update in Neurology 0915-162016
11
IncidencePrevalence of BPPV
bull Most common referred diagnosis in tertiary centers
bull 9 of randomly selected community dwelling elderly
(Oghalai JS et al 2000)
bull Incidence increases 38 with each decade of life
(Froehling 1991)
Sixth Annual Intensive Update in Neurology 0915-162016
12
Benign Positional Vertigo (BPPV)
bull Diagnosed with lateral supine head turns and Dix-
Hallpike positioning
ndash Latency
ndash Duration
ndash Fatigue
ndash Habituation
Sixth Annual Intensive Update in Neurology 0915-162016
13
DHT - Sitting
Sixth Annual Intensive Update in Neurology 0915-162016
14
DHT - HHR Orientation
Sixth Annual Intensive Update in Neurology 0915-162016
15
Dix-Hallpike to the Right
Sixth Annual Intensive Update in Neurology 0915-162016
16
Right posterior BPPV
Sixth Annual Intensive Update in Neurology 0915-162016
17
Canalith Repositioning Procedure
Sixth Annual Intensive Update in Neurology 0915-162016
18
Canalith Repositioning Procedure
Sixth Annual Intensive Update in Neurology 0915-162016
19
Canalith Repositioning Procedure
Sixth Annual Intensive Update in Neurology 0915-162016
20
Canalith Repositioning Procedure
Sixth Annual Intensive Update in Neurology 0915-162016
21
Canalith Repositioning
bull R posterior
BPPV
CPT 95992
Sixth Annual Intensive Update in Neurology 0915-162016
22
Posterior semicircular canalith repositioning
Sixth Annual Intensive Update in Neurology 0915-162016
23
Supine Positional Tests
Sixth Annual Intensive Update in Neurology 0915-162016
24
Lateral SCC-BPPV
bull Short latency horizontal nystagmus provoked by supine bilateral head turns with prolonged duration and poor fatigability
bull Uncommon (2-15 of total BPPV)
bull Two formsndash Geotropic ndash beating towards undermost ear
ndash Apogeotropic ndash beating away from undermost ear
Sixth Annual Intensive Update in Neurology 0915-162016
25
Geotropic Lateral Canal BPPV
bull Patient is positioned
first to the right lateral
position ndash nystagmus
beats right
bull Patient is then
positioned to the left
lateral position and
nystagmus reverses
and beats left
Sixth Annual Intensive Update in Neurology 0915-162016
26
Left Geotropic Lateral Semicircular Canal BPPV
Sixth Annual Intensive Update in Neurology 0915-162016
27
Repositioning Maneuvers for Geotropic Lateral Semicircular Canal (LSC) BPPV
bull Roll maneuver (Lempert Baloh)
ndash Patient rolls in 90 degree increments towards the good side
completely rolling over R LSC-BPPV repositioning shown
Sixth Annual Intensive Update in Neurology 0915-162016
28
Repositioning Maneuvers for Geotropic LSC BPPV
bull Gufoni maneuver
ndash Patient lies quickly onto good side and turns face-down to
floor for two minutes
Sixth Annual Intensive Update in Neurology 0915-162016
29
Left Apogeotropic LSC BPPV
Sixth Annual Intensive Update in Neurology 0915-162016
30
Nystagmus observed During Repositioning
bull Nystagmus should remain in the same torsional
direction as seen on DHT throughout the treatment of
posterior BPPV
bull Reversal of nystagmus direction suggests the particle
has fallen back into the canal and predicts failure
bull In Lateral BPPV nystagmus should beat towards the
good ear
Sixth Annual Intensive Update in Neurology 0915-162016
31
Posterior to Lateral canal conversion
Sixth Annual Intensive Update in Neurology 0915-162016
32
R Posterior to Lateral Canal conversion - geotropic
Sixth Annual Intensive Update in Neurology 0915-162016
33
BPPV Recurrence
bull Recurrence is common (15yr)
bull Otoconial adherence or mineralization demineralization
abnormalities may contribute to recurrence
bull Home canalith repositioning is effective (Radke 2004)
Sixth Annual Intensive Update in Neurology 0915-162016
34
Question 1
bull The most likely semicircular canal to be affected in
BPPV is
ndash 1) superior
ndash 2) lateral
ndash 3) posterior
ndash 4) combined
Sixth Annual Intensive Update in Neurology 0915-162016
35
Question 1
bull The most likely semicircular canal to be affected in
BPPV is
ndash 1) superior
ndash 2) lateral
ndash 3) posterior
ndash 4) combined
Answer 3 See references at end of lecture
Sixth Annual Intensive Update in Neurology 0915-162016
36
Acute Vestibular Syndrome
bull Rapid unilateral injury to either peripheral or central
vestibular structures produces prolonged vertigo
(days to weeks)
bull Severe vertigo nausea and vomiting spontaneous
nystagmus and postural instability
Sixth Annual Intensive Update in Neurology 0915-162016
37
Acute Vestibular Syndrome
bull 25 of patients with risk factors who present with isolated severe vertigo nystagmus and postural instability in the ER have an acute infarction of the inferior cerebellum (AICA PICA) Central imaging is suggested (Baloh 1998 NEJM)
ndash HTN diabetes smoking age gt65 Afib valvular heart disease occlusive vascular disease
Sixth Annual Intensive Update in Neurology 0915-162016
38
HINTS test
bull Head Impulse
bull Nystagmus
bull Test of Skew
ndash More sensitive than MRI in the diagnosis of acute stroke in
patients with acute vestibular syndrome with at least one risk
factor (Kattah J et al Stroke 2009)
Sixth Annual Intensive Update in Neurology 0915-162016
39
Head Impulse Test
bull No special equipment needed
bull Examiner stands in front of seated patient and
randomly moves head left and right Patient is asked to
focus on examinerrsquos nose
bull Rapid low amplitude thrust from lateral to straight
ahead causes a compensatory saccade when
performed towards the weak side
bull This finding is a permanent feature of unilateral
vestibular loss
Sixth Annual Intensive Update in Neurology 0915-162016
40
Head Impulse Test
Sixth Annual Intensive Update in Neurology 0915-162016
41
Head CT ndash Academy of Emergency Medicine 2013
bull The clinical value of head CT in isolated
dizzinessvertigo without any associated neurological
symptoms is very low
bull Less than 1 of head CTs ordered for dizziness and
vertigo have significant abnormalities and those
patients had severe headache and neurological deficits
in addition to dizziness
bull Forgoing unnecessary emergency head CTs could
save 360 million dollars annually
Sixth Annual Intensive Update in Neurology 0915-162016
42
Question 2
Head CT is not indicated for the evaluation of dizziness
and vertigo without additional neurological complaints because
1 It is too expensive
2 Less than 1 of CTs done for dizziness and vertigo are
abnormal and those patients had associated severe
neurological complaints
3 CT is the best imaging choice for the posterior fossa
4 Acoustic neuromas are well seen on CT
Sixth Annual Intensive Update in Neurology 0915-162016
43
Question 2
Head CT is not indicated for the evaluation of dizziness
and vertigo without additional neurological complaints because
1 It is too expensive
2 Less than 1 of CTs done for dizziness and vertigo are
abnormal and those patients had associated severe
neurological complaints
3 CT is the best imaging choice for the posterior fossa
4 Acoustic neuromas are well seen on CT
Answer 2 See references at end of lecture
Sixth Annual Intensive Update in Neurology 0915-162016
44
Acute Vestibular Syndrome
bull Usually peripheral and can be associated with
hearing loss (labyrinthitis)
bull Timely diagnosis improves recovery (ASAP
prednisone 1mgkgday for ten days and taper 10
mgday ndash Strupp M 2004 NEJM)
bull Avoid vestibular suppressants after 3 days and begin
vestibular rehabilitation to hasten compensation
Sixth Annual Intensive Update in Neurology 0915-162016
45
Question 3
bull Treatment for Acute Vestibular Syndrome
1) oral prednisone 1 mgkgday
2) Treat dehydration if necessary
3) Minimize vestibular suppressants after 3 days
4) Consider vestibular physical therapy
5) All of the above
Sixth Annual Intensive Update in Neurology 0915-162016
46
Question 3
bull Treatment for Acute Vestibular Syndrome
1) oral prednisone 1 mgkgday
2) Treat dehydration if necessary
3) Minimize vestibular suppressants after 3 days
4) Consider vestibular physical therapy
5) All of the above
Answer 5 See references at end of lecture
Sixth Annual Intensive Update in Neurology 0915-162016
47
Menierersquos syndrome
bull Fluctuating low frequency hearing loss
bull Tinnitus andor aural fullness
bull Vertigo lasting hours
Sixth Annual Intensive Update in Neurology 0915-162016
48
Normal
Sixth Annual Intensive Update in Neurology 0915-162016
49
Hydrops
Sixth Annual Intensive Update in Neurology 0915-162016
50
Menierersquos syndrome
ndash Diagnosis- Exclusion (RO acoustic)
ndash 23 of Menierersquos patients will improve spontaneously
ndash Treatment-1500 mgday low sodium triamterene375-
hydrochlorothiazide 25 vestibular suppressants (lorazepam
1 mg tablet sublingual Q 8 prn attacks) off label use
Sixth Annual Intensive Update in Neurology 0915-162016
51
Menierersquos syndrome ndash intratympanic steroids
ndash A newer treatment option for those failing medical management
ndash 90 response rate (Boleus-Aguirre MS Otology Neurotology
2008)
ndash Outcomes data confirm excellent clinical response in 3941
Menierersquos patients treated with intratympanic steroids in 2008
Sixth Annual Intensive Update in Neurology 0915-162016
52
Question 4
bull Menierersquos syndrome diagnosis is based on
1) Recurrent vertigo lasting at least 20 minutes
2) Aural fullness
3) Documented hearing loss
4) Other causes excluded
5) All of the above
Sixth Annual Intensive Update in Neurology 0915-162016
53
Question 4
bull Menierersquos syndrome diagnosis is based on
1) Recurrent vertigo lasting at least 20 minutes
2) Aural fullness
3) Documented hearing loss
4) Other causes excluded
5) All of the above
Answer 5 See references at end
Sixth Annual Intensive Update in Neurology 0915-162016
54
Superior Semicircular Canal Dehiscence (SSCD)
bull Sound andor pressure induced oscillopsia
bull CT reformatted in the plane of the superior canals
shows dehiscence of overlying bone
bull Low frequency ldquoconductive hyperacusisrdquo with elevated
bone thresholds normal reflexes and a conductive loss
Sixth Annual Intensive Update in Neurology 0915-162016
55
SSCD
bull Positive middle ear pressure or loud sound stimulates ampullofugal endolymph flow -canal activation
bull Eye movements are tonic upwards with ipsilateral intorsion
Sixth Annual Intensive Update in Neurology 0915-162016
56
SSCD
bull Valsalva against a closed glottis or jugular compression causes increased intracranial pressure and ampullopetal endolymph flow -canal inhibition
bull Eye movements are tonic downwards with ipsilateral extorsion
Sixth Annual Intensive Update in Neurology 0915-162016
57
Reconstructed left superior semicircular canal
Sixth Annual Intensive Update in Neurology 0915-162016
58
Sixth Annual Intensive Update in Neurology 0915-162016
59
SCHEMATIC DRAWING OF AN OTOLITHIC MACULA
Sixth Annual Intensive Update in Neurology 0915-162016
60
MACULAE OF THE UTRICLESACCULE
Sixth Annual Intensive Update in Neurology 0915-162016
61
HAIR CELLS
Sixth Annual Intensive Update in Neurology 0915-162016
62
SCANNING ELECTRON MICROGRAPH OF OTOCONIA
Sixth Annual Intensive Update in Neurology 0915-162016
63
Ocular VEMP
bull Response to loud clicks (80-110 dB) or forehead tap (Halmagyi and Curthoys 2003)
bull Contralateral oculomotor nucleus (CN III)
bull Inferior oblique muscle contraction (8 msec) detected by 5sec event triggered averaging
bull Noninverting electrode inferior to eye
bull Inverting below non-inverting electrode
bull Ground to forehead
bull Normal subjects have very low response
Sixth Annual Intensive Update in Neurology 0915-162016
64
Clinical applications
bull The o-VEMP is a sensitive test of otolithutricular
function
bull Complements vestibular test procedures that assess
semicircular canals
bull Abnormally robust responses are associated with third
window abnormalities such as semicircular canal
dehiscence
bull Serves as an excellent screening test for semicircular
canal dehiscence
Sixth Annual Intensive Update in Neurology 0915-162016
65
Question 5
bull Superior semicircular canal dehiscence
1) Causes increased ability to hear vibratory sounds
2) Improves hearing
3) Causes meningitis
4) Always needs surgical repair
Sixth Annual Intensive Update in Neurology 0915-162016
66
Question 5
bull Superior semicircular canal dehiscence
1) Causes increased ability to hear vibratory sounds
2) Improves hearing
3) Causes meningitis
4) Always needs surgical repair
Answer 1 References at end of lecture
Sixth Annual Intensive Update in Neurology 0915-162016
67
Enlarged vestibular aqueduct
bull Axial temporal bone CT showing right temporal bone with enlarged vestibular aqueduct (arrow)
bull Vestibular symptoms accompany progressive sensorineural hearing loss in 4-50 of patients in selected series
Sixth Annual Intensive Update in Neurology 0915-162016
68
Migraine
bull Family or personal history of migraine
bull 25 of migraine patients experience spontaneous vertigo (Baloh 1997)
bull Neuhauser criteria(2001) have been used to construct structured diagnostic interviews (Furman 2003)
bull Not always associated with headache
bull Motion sensitivity
bull Medication responsive
Sixth Annual Intensive Update in Neurology 0915-162016
69
ICHD-III beta Criteria for Vestibular Migraine
bull A At least five attacks fulfilling criteria C and D
bull B A current or past history of 11 migraine without aura or 12 migraine with aura
bull C Vestibular symptoms of moderate or severe intensity lasting between 5 minutes and 72 hours
bull D At least 50 associated with one of the following migrainous features
ndash 1 Headaches
ndash 2 Photophobia and phonophobia
ndash 3 Visual Aura
bull E Not better accounted for by another vestibular disorder
Sixth Annual Intensive Update in Neurology 0915-162016
70
Incidence of vertigo in MS
bull True vertigo (the perception of environmental
movement typically rotational) occurs in about
20 of MS patients and is the presenting
manifestation in 5
bull Up to 78 of MS patients report abnormalities
in balance at some point in their illness
Frohman et al Multiple Sclerosis 2003 9250
Sixth Annual Intensive Update in Neurology 0915-162016
71
Vertigo in MS due to demyelination
bull The most common neuroanatomic locations are the
root entry zone of CN VIII (giving absent calorics but
normal hearing and fixation suppression) and the
medial vestibular nucleus
bull Usually treated with steroids and vestibular
suppressants
Brandt 1993 Gass 1998 Thomke 1999
Sixth Annual Intensive Update in Neurology 0915-162016
72
Vertigo in MS
Frohmanrsquos large series (1153) reported that 52 of MS patients with acute vertigo had benign paroxysmal positional vertigo while 32 had new brainstem lesions
To avoid misdiagnoses and inappropriate treatment interventions alternative etiologies should be considered in all acute vertigo episodes in MS
E M Frohman Neurology 2000 and Multiple Sclerosis 2003
Sixth Annual Intensive Update in Neurology 0915-162016
73
Vestibular compensation
bull Acute unilateral or bilateral vestibular loss will prompt compensation thru central cervico-ocular visual and proprioceptive pathways
bull Unilateral loss may require several weeks for compensation to occur and activity especially head movements hastens compensation
bull Vestibular suppressant medication prolongs compensation
Sixth Annual Intensive Update in Neurology 0915-162016
74
Vestibular Rehabilitation
bull Performed by certified physical therapists many sites (Epic order vestibular PT) or check Vestibular Disorders of America website for providers
bull Assessment and treatment of benign positional vertigo
bull Directed rehab programs for central andor peripheral vestibular compensationndash Unilateral vestibulopathies
ndash Bilateral vestibular hypofunction
ndash Mutifactorial balance deficits and fall risk assessment
Sixth Annual Intensive Update in Neurology 0915-162016
75
Summary of whatrsquos new in peripheral vestibular disorders
bull Lateral semicircular canal benign paroxysmal positional vertigo presents with horizontal positioning nystagmus
bull Oral steroids appear to improve outcome in acute vestibular neuritis
bull Intratympanic steroids offer promise in Menierersquos syndrome
bull Superior semicircular canal dehiscence causes pressure and noise induced dizziness VEMP offers diagnostic information
Sixth Annual Intensive Update in Neurology 0915-162016
76
References
BPPVndash White J Benign paroxysmal positional vertigo Cleveland Clinic Journal of
Medicine 71(9)722-728 2004
ndash White J Coale K Catalano P Oas J Diagnosis and management of lateral semicircular canal benign positional vertigo Otolaryngology-Head and Neck Surgery 133 278-284 2005
ndash White J Savvides P Cherian N Oas J Canalith repositioning for benign paroxysmal positional vertigo Otology and Neurotology 26 704-710 2005
ndash White J Oas J Diagnosis and management of lateral semicircular canal conversions during particle repositioning therapy Laryngoscope 115(10) 1895-1897 2005
ndash Acute Vestibular Syndrome Hotson JR Baloh RW N Engl J Med 1998 Sep 3339(10)680-5
ndash Methylprednisolone valacyclovir or the combination for vestibular neuritis Strupp M Zingler VC Arbusow V Niklas D Maag KP Dieterich M Bense S Theil D Jahn K Brandt T N Engl J Med 2004 Jul 22351(4)354-61
ndash Intratympanic steroids for Meacuteniegraveres disease or syndrome Phillips JS Westerberg BCochrane Database Syst Rev 2011 Jul 6(7)CD008514 Review
ndash Sound- andor pressure-induced vertigo due to bone dehiscence of the superior semicircular canal Minor LB Solomon D Zinreich JS Zee DSArch Otolaryngol Head Neck Surg 1998 Mar124(3)249-58
Sixth Annual Intensive Update in Neurology 0915-162016
11
IncidencePrevalence of BPPV
bull Most common referred diagnosis in tertiary centers
bull 9 of randomly selected community dwelling elderly
(Oghalai JS et al 2000)
bull Incidence increases 38 with each decade of life
(Froehling 1991)
Sixth Annual Intensive Update in Neurology 0915-162016
12
Benign Positional Vertigo (BPPV)
bull Diagnosed with lateral supine head turns and Dix-
Hallpike positioning
ndash Latency
ndash Duration
ndash Fatigue
ndash Habituation
Sixth Annual Intensive Update in Neurology 0915-162016
13
DHT - Sitting
Sixth Annual Intensive Update in Neurology 0915-162016
14
DHT - HHR Orientation
Sixth Annual Intensive Update in Neurology 0915-162016
15
Dix-Hallpike to the Right
Sixth Annual Intensive Update in Neurology 0915-162016
16
Right posterior BPPV
Sixth Annual Intensive Update in Neurology 0915-162016
17
Canalith Repositioning Procedure
Sixth Annual Intensive Update in Neurology 0915-162016
18
Canalith Repositioning Procedure
Sixth Annual Intensive Update in Neurology 0915-162016
19
Canalith Repositioning Procedure
Sixth Annual Intensive Update in Neurology 0915-162016
20
Canalith Repositioning Procedure
Sixth Annual Intensive Update in Neurology 0915-162016
21
Canalith Repositioning
bull R posterior
BPPV
CPT 95992
Sixth Annual Intensive Update in Neurology 0915-162016
22
Posterior semicircular canalith repositioning
Sixth Annual Intensive Update in Neurology 0915-162016
23
Supine Positional Tests
Sixth Annual Intensive Update in Neurology 0915-162016
24
Lateral SCC-BPPV
bull Short latency horizontal nystagmus provoked by supine bilateral head turns with prolonged duration and poor fatigability
bull Uncommon (2-15 of total BPPV)
bull Two formsndash Geotropic ndash beating towards undermost ear
ndash Apogeotropic ndash beating away from undermost ear
Sixth Annual Intensive Update in Neurology 0915-162016
25
Geotropic Lateral Canal BPPV
bull Patient is positioned
first to the right lateral
position ndash nystagmus
beats right
bull Patient is then
positioned to the left
lateral position and
nystagmus reverses
and beats left
Sixth Annual Intensive Update in Neurology 0915-162016
26
Left Geotropic Lateral Semicircular Canal BPPV
Sixth Annual Intensive Update in Neurology 0915-162016
27
Repositioning Maneuvers for Geotropic Lateral Semicircular Canal (LSC) BPPV
bull Roll maneuver (Lempert Baloh)
ndash Patient rolls in 90 degree increments towards the good side
completely rolling over R LSC-BPPV repositioning shown
Sixth Annual Intensive Update in Neurology 0915-162016
28
Repositioning Maneuvers for Geotropic LSC BPPV
bull Gufoni maneuver
ndash Patient lies quickly onto good side and turns face-down to
floor for two minutes
Sixth Annual Intensive Update in Neurology 0915-162016
29
Left Apogeotropic LSC BPPV
Sixth Annual Intensive Update in Neurology 0915-162016
30
Nystagmus observed During Repositioning
bull Nystagmus should remain in the same torsional
direction as seen on DHT throughout the treatment of
posterior BPPV
bull Reversal of nystagmus direction suggests the particle
has fallen back into the canal and predicts failure
bull In Lateral BPPV nystagmus should beat towards the
good ear
Sixth Annual Intensive Update in Neurology 0915-162016
31
Posterior to Lateral canal conversion
Sixth Annual Intensive Update in Neurology 0915-162016
32
R Posterior to Lateral Canal conversion - geotropic
Sixth Annual Intensive Update in Neurology 0915-162016
33
BPPV Recurrence
bull Recurrence is common (15yr)
bull Otoconial adherence or mineralization demineralization
abnormalities may contribute to recurrence
bull Home canalith repositioning is effective (Radke 2004)
Sixth Annual Intensive Update in Neurology 0915-162016
34
Question 1
bull The most likely semicircular canal to be affected in
BPPV is
ndash 1) superior
ndash 2) lateral
ndash 3) posterior
ndash 4) combined
Sixth Annual Intensive Update in Neurology 0915-162016
35
Question 1
bull The most likely semicircular canal to be affected in
BPPV is
ndash 1) superior
ndash 2) lateral
ndash 3) posterior
ndash 4) combined
Answer 3 See references at end of lecture
Sixth Annual Intensive Update in Neurology 0915-162016
36
Acute Vestibular Syndrome
bull Rapid unilateral injury to either peripheral or central
vestibular structures produces prolonged vertigo
(days to weeks)
bull Severe vertigo nausea and vomiting spontaneous
nystagmus and postural instability
Sixth Annual Intensive Update in Neurology 0915-162016
37
Acute Vestibular Syndrome
bull 25 of patients with risk factors who present with isolated severe vertigo nystagmus and postural instability in the ER have an acute infarction of the inferior cerebellum (AICA PICA) Central imaging is suggested (Baloh 1998 NEJM)
ndash HTN diabetes smoking age gt65 Afib valvular heart disease occlusive vascular disease
Sixth Annual Intensive Update in Neurology 0915-162016
38
HINTS test
bull Head Impulse
bull Nystagmus
bull Test of Skew
ndash More sensitive than MRI in the diagnosis of acute stroke in
patients with acute vestibular syndrome with at least one risk
factor (Kattah J et al Stroke 2009)
Sixth Annual Intensive Update in Neurology 0915-162016
39
Head Impulse Test
bull No special equipment needed
bull Examiner stands in front of seated patient and
randomly moves head left and right Patient is asked to
focus on examinerrsquos nose
bull Rapid low amplitude thrust from lateral to straight
ahead causes a compensatory saccade when
performed towards the weak side
bull This finding is a permanent feature of unilateral
vestibular loss
Sixth Annual Intensive Update in Neurology 0915-162016
40
Head Impulse Test
Sixth Annual Intensive Update in Neurology 0915-162016
41
Head CT ndash Academy of Emergency Medicine 2013
bull The clinical value of head CT in isolated
dizzinessvertigo without any associated neurological
symptoms is very low
bull Less than 1 of head CTs ordered for dizziness and
vertigo have significant abnormalities and those
patients had severe headache and neurological deficits
in addition to dizziness
bull Forgoing unnecessary emergency head CTs could
save 360 million dollars annually
Sixth Annual Intensive Update in Neurology 0915-162016
42
Question 2
Head CT is not indicated for the evaluation of dizziness
and vertigo without additional neurological complaints because
1 It is too expensive
2 Less than 1 of CTs done for dizziness and vertigo are
abnormal and those patients had associated severe
neurological complaints
3 CT is the best imaging choice for the posterior fossa
4 Acoustic neuromas are well seen on CT
Sixth Annual Intensive Update in Neurology 0915-162016
43
Question 2
Head CT is not indicated for the evaluation of dizziness
and vertigo without additional neurological complaints because
1 It is too expensive
2 Less than 1 of CTs done for dizziness and vertigo are
abnormal and those patients had associated severe
neurological complaints
3 CT is the best imaging choice for the posterior fossa
4 Acoustic neuromas are well seen on CT
Answer 2 See references at end of lecture
Sixth Annual Intensive Update in Neurology 0915-162016
44
Acute Vestibular Syndrome
bull Usually peripheral and can be associated with
hearing loss (labyrinthitis)
bull Timely diagnosis improves recovery (ASAP
prednisone 1mgkgday for ten days and taper 10
mgday ndash Strupp M 2004 NEJM)
bull Avoid vestibular suppressants after 3 days and begin
vestibular rehabilitation to hasten compensation
Sixth Annual Intensive Update in Neurology 0915-162016
45
Question 3
bull Treatment for Acute Vestibular Syndrome
1) oral prednisone 1 mgkgday
2) Treat dehydration if necessary
3) Minimize vestibular suppressants after 3 days
4) Consider vestibular physical therapy
5) All of the above
Sixth Annual Intensive Update in Neurology 0915-162016
46
Question 3
bull Treatment for Acute Vestibular Syndrome
1) oral prednisone 1 mgkgday
2) Treat dehydration if necessary
3) Minimize vestibular suppressants after 3 days
4) Consider vestibular physical therapy
5) All of the above
Answer 5 See references at end of lecture
Sixth Annual Intensive Update in Neurology 0915-162016
47
Menierersquos syndrome
bull Fluctuating low frequency hearing loss
bull Tinnitus andor aural fullness
bull Vertigo lasting hours
Sixth Annual Intensive Update in Neurology 0915-162016
48
Normal
Sixth Annual Intensive Update in Neurology 0915-162016
49
Hydrops
Sixth Annual Intensive Update in Neurology 0915-162016
50
Menierersquos syndrome
ndash Diagnosis- Exclusion (RO acoustic)
ndash 23 of Menierersquos patients will improve spontaneously
ndash Treatment-1500 mgday low sodium triamterene375-
hydrochlorothiazide 25 vestibular suppressants (lorazepam
1 mg tablet sublingual Q 8 prn attacks) off label use
Sixth Annual Intensive Update in Neurology 0915-162016
51
Menierersquos syndrome ndash intratympanic steroids
ndash A newer treatment option for those failing medical management
ndash 90 response rate (Boleus-Aguirre MS Otology Neurotology
2008)
ndash Outcomes data confirm excellent clinical response in 3941
Menierersquos patients treated with intratympanic steroids in 2008
Sixth Annual Intensive Update in Neurology 0915-162016
52
Question 4
bull Menierersquos syndrome diagnosis is based on
1) Recurrent vertigo lasting at least 20 minutes
2) Aural fullness
3) Documented hearing loss
4) Other causes excluded
5) All of the above
Sixth Annual Intensive Update in Neurology 0915-162016
53
Question 4
bull Menierersquos syndrome diagnosis is based on
1) Recurrent vertigo lasting at least 20 minutes
2) Aural fullness
3) Documented hearing loss
4) Other causes excluded
5) All of the above
Answer 5 See references at end
Sixth Annual Intensive Update in Neurology 0915-162016
54
Superior Semicircular Canal Dehiscence (SSCD)
bull Sound andor pressure induced oscillopsia
bull CT reformatted in the plane of the superior canals
shows dehiscence of overlying bone
bull Low frequency ldquoconductive hyperacusisrdquo with elevated
bone thresholds normal reflexes and a conductive loss
Sixth Annual Intensive Update in Neurology 0915-162016
55
SSCD
bull Positive middle ear pressure or loud sound stimulates ampullofugal endolymph flow -canal activation
bull Eye movements are tonic upwards with ipsilateral intorsion
Sixth Annual Intensive Update in Neurology 0915-162016
56
SSCD
bull Valsalva against a closed glottis or jugular compression causes increased intracranial pressure and ampullopetal endolymph flow -canal inhibition
bull Eye movements are tonic downwards with ipsilateral extorsion
Sixth Annual Intensive Update in Neurology 0915-162016
57
Reconstructed left superior semicircular canal
Sixth Annual Intensive Update in Neurology 0915-162016
58
Sixth Annual Intensive Update in Neurology 0915-162016
59
SCHEMATIC DRAWING OF AN OTOLITHIC MACULA
Sixth Annual Intensive Update in Neurology 0915-162016
60
MACULAE OF THE UTRICLESACCULE
Sixth Annual Intensive Update in Neurology 0915-162016
61
HAIR CELLS
Sixth Annual Intensive Update in Neurology 0915-162016
62
SCANNING ELECTRON MICROGRAPH OF OTOCONIA
Sixth Annual Intensive Update in Neurology 0915-162016
63
Ocular VEMP
bull Response to loud clicks (80-110 dB) or forehead tap (Halmagyi and Curthoys 2003)
bull Contralateral oculomotor nucleus (CN III)
bull Inferior oblique muscle contraction (8 msec) detected by 5sec event triggered averaging
bull Noninverting electrode inferior to eye
bull Inverting below non-inverting electrode
bull Ground to forehead
bull Normal subjects have very low response
Sixth Annual Intensive Update in Neurology 0915-162016
64
Clinical applications
bull The o-VEMP is a sensitive test of otolithutricular
function
bull Complements vestibular test procedures that assess
semicircular canals
bull Abnormally robust responses are associated with third
window abnormalities such as semicircular canal
dehiscence
bull Serves as an excellent screening test for semicircular
canal dehiscence
Sixth Annual Intensive Update in Neurology 0915-162016
65
Question 5
bull Superior semicircular canal dehiscence
1) Causes increased ability to hear vibratory sounds
2) Improves hearing
3) Causes meningitis
4) Always needs surgical repair
Sixth Annual Intensive Update in Neurology 0915-162016
66
Question 5
bull Superior semicircular canal dehiscence
1) Causes increased ability to hear vibratory sounds
2) Improves hearing
3) Causes meningitis
4) Always needs surgical repair
Answer 1 References at end of lecture
Sixth Annual Intensive Update in Neurology 0915-162016
67
Enlarged vestibular aqueduct
bull Axial temporal bone CT showing right temporal bone with enlarged vestibular aqueduct (arrow)
bull Vestibular symptoms accompany progressive sensorineural hearing loss in 4-50 of patients in selected series
Sixth Annual Intensive Update in Neurology 0915-162016
68
Migraine
bull Family or personal history of migraine
bull 25 of migraine patients experience spontaneous vertigo (Baloh 1997)
bull Neuhauser criteria(2001) have been used to construct structured diagnostic interviews (Furman 2003)
bull Not always associated with headache
bull Motion sensitivity
bull Medication responsive
Sixth Annual Intensive Update in Neurology 0915-162016
69
ICHD-III beta Criteria for Vestibular Migraine
bull A At least five attacks fulfilling criteria C and D
bull B A current or past history of 11 migraine without aura or 12 migraine with aura
bull C Vestibular symptoms of moderate or severe intensity lasting between 5 minutes and 72 hours
bull D At least 50 associated with one of the following migrainous features
ndash 1 Headaches
ndash 2 Photophobia and phonophobia
ndash 3 Visual Aura
bull E Not better accounted for by another vestibular disorder
Sixth Annual Intensive Update in Neurology 0915-162016
70
Incidence of vertigo in MS
bull True vertigo (the perception of environmental
movement typically rotational) occurs in about
20 of MS patients and is the presenting
manifestation in 5
bull Up to 78 of MS patients report abnormalities
in balance at some point in their illness
Frohman et al Multiple Sclerosis 2003 9250
Sixth Annual Intensive Update in Neurology 0915-162016
71
Vertigo in MS due to demyelination
bull The most common neuroanatomic locations are the
root entry zone of CN VIII (giving absent calorics but
normal hearing and fixation suppression) and the
medial vestibular nucleus
bull Usually treated with steroids and vestibular
suppressants
Brandt 1993 Gass 1998 Thomke 1999
Sixth Annual Intensive Update in Neurology 0915-162016
72
Vertigo in MS
Frohmanrsquos large series (1153) reported that 52 of MS patients with acute vertigo had benign paroxysmal positional vertigo while 32 had new brainstem lesions
To avoid misdiagnoses and inappropriate treatment interventions alternative etiologies should be considered in all acute vertigo episodes in MS
E M Frohman Neurology 2000 and Multiple Sclerosis 2003
Sixth Annual Intensive Update in Neurology 0915-162016
73
Vestibular compensation
bull Acute unilateral or bilateral vestibular loss will prompt compensation thru central cervico-ocular visual and proprioceptive pathways
bull Unilateral loss may require several weeks for compensation to occur and activity especially head movements hastens compensation
bull Vestibular suppressant medication prolongs compensation
Sixth Annual Intensive Update in Neurology 0915-162016
74
Vestibular Rehabilitation
bull Performed by certified physical therapists many sites (Epic order vestibular PT) or check Vestibular Disorders of America website for providers
bull Assessment and treatment of benign positional vertigo
bull Directed rehab programs for central andor peripheral vestibular compensationndash Unilateral vestibulopathies
ndash Bilateral vestibular hypofunction
ndash Mutifactorial balance deficits and fall risk assessment
Sixth Annual Intensive Update in Neurology 0915-162016
75
Summary of whatrsquos new in peripheral vestibular disorders
bull Lateral semicircular canal benign paroxysmal positional vertigo presents with horizontal positioning nystagmus
bull Oral steroids appear to improve outcome in acute vestibular neuritis
bull Intratympanic steroids offer promise in Menierersquos syndrome
bull Superior semicircular canal dehiscence causes pressure and noise induced dizziness VEMP offers diagnostic information
Sixth Annual Intensive Update in Neurology 0915-162016
76
References
BPPVndash White J Benign paroxysmal positional vertigo Cleveland Clinic Journal of
Medicine 71(9)722-728 2004
ndash White J Coale K Catalano P Oas J Diagnosis and management of lateral semicircular canal benign positional vertigo Otolaryngology-Head and Neck Surgery 133 278-284 2005
ndash White J Savvides P Cherian N Oas J Canalith repositioning for benign paroxysmal positional vertigo Otology and Neurotology 26 704-710 2005
ndash White J Oas J Diagnosis and management of lateral semicircular canal conversions during particle repositioning therapy Laryngoscope 115(10) 1895-1897 2005
ndash Acute Vestibular Syndrome Hotson JR Baloh RW N Engl J Med 1998 Sep 3339(10)680-5
ndash Methylprednisolone valacyclovir or the combination for vestibular neuritis Strupp M Zingler VC Arbusow V Niklas D Maag KP Dieterich M Bense S Theil D Jahn K Brandt T N Engl J Med 2004 Jul 22351(4)354-61
ndash Intratympanic steroids for Meacuteniegraveres disease or syndrome Phillips JS Westerberg BCochrane Database Syst Rev 2011 Jul 6(7)CD008514 Review
ndash Sound- andor pressure-induced vertigo due to bone dehiscence of the superior semicircular canal Minor LB Solomon D Zinreich JS Zee DSArch Otolaryngol Head Neck Surg 1998 Mar124(3)249-58
Sixth Annual Intensive Update in Neurology 0915-162016
12
Benign Positional Vertigo (BPPV)
bull Diagnosed with lateral supine head turns and Dix-
Hallpike positioning
ndash Latency
ndash Duration
ndash Fatigue
ndash Habituation
Sixth Annual Intensive Update in Neurology 0915-162016
13
DHT - Sitting
Sixth Annual Intensive Update in Neurology 0915-162016
14
DHT - HHR Orientation
Sixth Annual Intensive Update in Neurology 0915-162016
15
Dix-Hallpike to the Right
Sixth Annual Intensive Update in Neurology 0915-162016
16
Right posterior BPPV
Sixth Annual Intensive Update in Neurology 0915-162016
17
Canalith Repositioning Procedure
Sixth Annual Intensive Update in Neurology 0915-162016
18
Canalith Repositioning Procedure
Sixth Annual Intensive Update in Neurology 0915-162016
19
Canalith Repositioning Procedure
Sixth Annual Intensive Update in Neurology 0915-162016
20
Canalith Repositioning Procedure
Sixth Annual Intensive Update in Neurology 0915-162016
21
Canalith Repositioning
bull R posterior
BPPV
CPT 95992
Sixth Annual Intensive Update in Neurology 0915-162016
22
Posterior semicircular canalith repositioning
Sixth Annual Intensive Update in Neurology 0915-162016
23
Supine Positional Tests
Sixth Annual Intensive Update in Neurology 0915-162016
24
Lateral SCC-BPPV
bull Short latency horizontal nystagmus provoked by supine bilateral head turns with prolonged duration and poor fatigability
bull Uncommon (2-15 of total BPPV)
bull Two formsndash Geotropic ndash beating towards undermost ear
ndash Apogeotropic ndash beating away from undermost ear
Sixth Annual Intensive Update in Neurology 0915-162016
25
Geotropic Lateral Canal BPPV
bull Patient is positioned
first to the right lateral
position ndash nystagmus
beats right
bull Patient is then
positioned to the left
lateral position and
nystagmus reverses
and beats left
Sixth Annual Intensive Update in Neurology 0915-162016
26
Left Geotropic Lateral Semicircular Canal BPPV
Sixth Annual Intensive Update in Neurology 0915-162016
27
Repositioning Maneuvers for Geotropic Lateral Semicircular Canal (LSC) BPPV
bull Roll maneuver (Lempert Baloh)
ndash Patient rolls in 90 degree increments towards the good side
completely rolling over R LSC-BPPV repositioning shown
Sixth Annual Intensive Update in Neurology 0915-162016
28
Repositioning Maneuvers for Geotropic LSC BPPV
bull Gufoni maneuver
ndash Patient lies quickly onto good side and turns face-down to
floor for two minutes
Sixth Annual Intensive Update in Neurology 0915-162016
29
Left Apogeotropic LSC BPPV
Sixth Annual Intensive Update in Neurology 0915-162016
30
Nystagmus observed During Repositioning
bull Nystagmus should remain in the same torsional
direction as seen on DHT throughout the treatment of
posterior BPPV
bull Reversal of nystagmus direction suggests the particle
has fallen back into the canal and predicts failure
bull In Lateral BPPV nystagmus should beat towards the
good ear
Sixth Annual Intensive Update in Neurology 0915-162016
31
Posterior to Lateral canal conversion
Sixth Annual Intensive Update in Neurology 0915-162016
32
R Posterior to Lateral Canal conversion - geotropic
Sixth Annual Intensive Update in Neurology 0915-162016
33
BPPV Recurrence
bull Recurrence is common (15yr)
bull Otoconial adherence or mineralization demineralization
abnormalities may contribute to recurrence
bull Home canalith repositioning is effective (Radke 2004)
Sixth Annual Intensive Update in Neurology 0915-162016
34
Question 1
bull The most likely semicircular canal to be affected in
BPPV is
ndash 1) superior
ndash 2) lateral
ndash 3) posterior
ndash 4) combined
Sixth Annual Intensive Update in Neurology 0915-162016
35
Question 1
bull The most likely semicircular canal to be affected in
BPPV is
ndash 1) superior
ndash 2) lateral
ndash 3) posterior
ndash 4) combined
Answer 3 See references at end of lecture
Sixth Annual Intensive Update in Neurology 0915-162016
36
Acute Vestibular Syndrome
bull Rapid unilateral injury to either peripheral or central
vestibular structures produces prolonged vertigo
(days to weeks)
bull Severe vertigo nausea and vomiting spontaneous
nystagmus and postural instability
Sixth Annual Intensive Update in Neurology 0915-162016
37
Acute Vestibular Syndrome
bull 25 of patients with risk factors who present with isolated severe vertigo nystagmus and postural instability in the ER have an acute infarction of the inferior cerebellum (AICA PICA) Central imaging is suggested (Baloh 1998 NEJM)
ndash HTN diabetes smoking age gt65 Afib valvular heart disease occlusive vascular disease
Sixth Annual Intensive Update in Neurology 0915-162016
38
HINTS test
bull Head Impulse
bull Nystagmus
bull Test of Skew
ndash More sensitive than MRI in the diagnosis of acute stroke in
patients with acute vestibular syndrome with at least one risk
factor (Kattah J et al Stroke 2009)
Sixth Annual Intensive Update in Neurology 0915-162016
39
Head Impulse Test
bull No special equipment needed
bull Examiner stands in front of seated patient and
randomly moves head left and right Patient is asked to
focus on examinerrsquos nose
bull Rapid low amplitude thrust from lateral to straight
ahead causes a compensatory saccade when
performed towards the weak side
bull This finding is a permanent feature of unilateral
vestibular loss
Sixth Annual Intensive Update in Neurology 0915-162016
40
Head Impulse Test
Sixth Annual Intensive Update in Neurology 0915-162016
41
Head CT ndash Academy of Emergency Medicine 2013
bull The clinical value of head CT in isolated
dizzinessvertigo without any associated neurological
symptoms is very low
bull Less than 1 of head CTs ordered for dizziness and
vertigo have significant abnormalities and those
patients had severe headache and neurological deficits
in addition to dizziness
bull Forgoing unnecessary emergency head CTs could
save 360 million dollars annually
Sixth Annual Intensive Update in Neurology 0915-162016
42
Question 2
Head CT is not indicated for the evaluation of dizziness
and vertigo without additional neurological complaints because
1 It is too expensive
2 Less than 1 of CTs done for dizziness and vertigo are
abnormal and those patients had associated severe
neurological complaints
3 CT is the best imaging choice for the posterior fossa
4 Acoustic neuromas are well seen on CT
Sixth Annual Intensive Update in Neurology 0915-162016
43
Question 2
Head CT is not indicated for the evaluation of dizziness
and vertigo without additional neurological complaints because
1 It is too expensive
2 Less than 1 of CTs done for dizziness and vertigo are
abnormal and those patients had associated severe
neurological complaints
3 CT is the best imaging choice for the posterior fossa
4 Acoustic neuromas are well seen on CT
Answer 2 See references at end of lecture
Sixth Annual Intensive Update in Neurology 0915-162016
44
Acute Vestibular Syndrome
bull Usually peripheral and can be associated with
hearing loss (labyrinthitis)
bull Timely diagnosis improves recovery (ASAP
prednisone 1mgkgday for ten days and taper 10
mgday ndash Strupp M 2004 NEJM)
bull Avoid vestibular suppressants after 3 days and begin
vestibular rehabilitation to hasten compensation
Sixth Annual Intensive Update in Neurology 0915-162016
45
Question 3
bull Treatment for Acute Vestibular Syndrome
1) oral prednisone 1 mgkgday
2) Treat dehydration if necessary
3) Minimize vestibular suppressants after 3 days
4) Consider vestibular physical therapy
5) All of the above
Sixth Annual Intensive Update in Neurology 0915-162016
46
Question 3
bull Treatment for Acute Vestibular Syndrome
1) oral prednisone 1 mgkgday
2) Treat dehydration if necessary
3) Minimize vestibular suppressants after 3 days
4) Consider vestibular physical therapy
5) All of the above
Answer 5 See references at end of lecture
Sixth Annual Intensive Update in Neurology 0915-162016
47
Menierersquos syndrome
bull Fluctuating low frequency hearing loss
bull Tinnitus andor aural fullness
bull Vertigo lasting hours
Sixth Annual Intensive Update in Neurology 0915-162016
48
Normal
Sixth Annual Intensive Update in Neurology 0915-162016
49
Hydrops
Sixth Annual Intensive Update in Neurology 0915-162016
50
Menierersquos syndrome
ndash Diagnosis- Exclusion (RO acoustic)
ndash 23 of Menierersquos patients will improve spontaneously
ndash Treatment-1500 mgday low sodium triamterene375-
hydrochlorothiazide 25 vestibular suppressants (lorazepam
1 mg tablet sublingual Q 8 prn attacks) off label use
Sixth Annual Intensive Update in Neurology 0915-162016
51
Menierersquos syndrome ndash intratympanic steroids
ndash A newer treatment option for those failing medical management
ndash 90 response rate (Boleus-Aguirre MS Otology Neurotology
2008)
ndash Outcomes data confirm excellent clinical response in 3941
Menierersquos patients treated with intratympanic steroids in 2008
Sixth Annual Intensive Update in Neurology 0915-162016
52
Question 4
bull Menierersquos syndrome diagnosis is based on
1) Recurrent vertigo lasting at least 20 minutes
2) Aural fullness
3) Documented hearing loss
4) Other causes excluded
5) All of the above
Sixth Annual Intensive Update in Neurology 0915-162016
53
Question 4
bull Menierersquos syndrome diagnosis is based on
1) Recurrent vertigo lasting at least 20 minutes
2) Aural fullness
3) Documented hearing loss
4) Other causes excluded
5) All of the above
Answer 5 See references at end
Sixth Annual Intensive Update in Neurology 0915-162016
54
Superior Semicircular Canal Dehiscence (SSCD)
bull Sound andor pressure induced oscillopsia
bull CT reformatted in the plane of the superior canals
shows dehiscence of overlying bone
bull Low frequency ldquoconductive hyperacusisrdquo with elevated
bone thresholds normal reflexes and a conductive loss
Sixth Annual Intensive Update in Neurology 0915-162016
55
SSCD
bull Positive middle ear pressure or loud sound stimulates ampullofugal endolymph flow -canal activation
bull Eye movements are tonic upwards with ipsilateral intorsion
Sixth Annual Intensive Update in Neurology 0915-162016
56
SSCD
bull Valsalva against a closed glottis or jugular compression causes increased intracranial pressure and ampullopetal endolymph flow -canal inhibition
bull Eye movements are tonic downwards with ipsilateral extorsion
Sixth Annual Intensive Update in Neurology 0915-162016
57
Reconstructed left superior semicircular canal
Sixth Annual Intensive Update in Neurology 0915-162016
58
Sixth Annual Intensive Update in Neurology 0915-162016
59
SCHEMATIC DRAWING OF AN OTOLITHIC MACULA
Sixth Annual Intensive Update in Neurology 0915-162016
60
MACULAE OF THE UTRICLESACCULE
Sixth Annual Intensive Update in Neurology 0915-162016
61
HAIR CELLS
Sixth Annual Intensive Update in Neurology 0915-162016
62
SCANNING ELECTRON MICROGRAPH OF OTOCONIA
Sixth Annual Intensive Update in Neurology 0915-162016
63
Ocular VEMP
bull Response to loud clicks (80-110 dB) or forehead tap (Halmagyi and Curthoys 2003)
bull Contralateral oculomotor nucleus (CN III)
bull Inferior oblique muscle contraction (8 msec) detected by 5sec event triggered averaging
bull Noninverting electrode inferior to eye
bull Inverting below non-inverting electrode
bull Ground to forehead
bull Normal subjects have very low response
Sixth Annual Intensive Update in Neurology 0915-162016
64
Clinical applications
bull The o-VEMP is a sensitive test of otolithutricular
function
bull Complements vestibular test procedures that assess
semicircular canals
bull Abnormally robust responses are associated with third
window abnormalities such as semicircular canal
dehiscence
bull Serves as an excellent screening test for semicircular
canal dehiscence
Sixth Annual Intensive Update in Neurology 0915-162016
65
Question 5
bull Superior semicircular canal dehiscence
1) Causes increased ability to hear vibratory sounds
2) Improves hearing
3) Causes meningitis
4) Always needs surgical repair
Sixth Annual Intensive Update in Neurology 0915-162016
66
Question 5
bull Superior semicircular canal dehiscence
1) Causes increased ability to hear vibratory sounds
2) Improves hearing
3) Causes meningitis
4) Always needs surgical repair
Answer 1 References at end of lecture
Sixth Annual Intensive Update in Neurology 0915-162016
67
Enlarged vestibular aqueduct
bull Axial temporal bone CT showing right temporal bone with enlarged vestibular aqueduct (arrow)
bull Vestibular symptoms accompany progressive sensorineural hearing loss in 4-50 of patients in selected series
Sixth Annual Intensive Update in Neurology 0915-162016
68
Migraine
bull Family or personal history of migraine
bull 25 of migraine patients experience spontaneous vertigo (Baloh 1997)
bull Neuhauser criteria(2001) have been used to construct structured diagnostic interviews (Furman 2003)
bull Not always associated with headache
bull Motion sensitivity
bull Medication responsive
Sixth Annual Intensive Update in Neurology 0915-162016
69
ICHD-III beta Criteria for Vestibular Migraine
bull A At least five attacks fulfilling criteria C and D
bull B A current or past history of 11 migraine without aura or 12 migraine with aura
bull C Vestibular symptoms of moderate or severe intensity lasting between 5 minutes and 72 hours
bull D At least 50 associated with one of the following migrainous features
ndash 1 Headaches
ndash 2 Photophobia and phonophobia
ndash 3 Visual Aura
bull E Not better accounted for by another vestibular disorder
Sixth Annual Intensive Update in Neurology 0915-162016
70
Incidence of vertigo in MS
bull True vertigo (the perception of environmental
movement typically rotational) occurs in about
20 of MS patients and is the presenting
manifestation in 5
bull Up to 78 of MS patients report abnormalities
in balance at some point in their illness
Frohman et al Multiple Sclerosis 2003 9250
Sixth Annual Intensive Update in Neurology 0915-162016
71
Vertigo in MS due to demyelination
bull The most common neuroanatomic locations are the
root entry zone of CN VIII (giving absent calorics but
normal hearing and fixation suppression) and the
medial vestibular nucleus
bull Usually treated with steroids and vestibular
suppressants
Brandt 1993 Gass 1998 Thomke 1999
Sixth Annual Intensive Update in Neurology 0915-162016
72
Vertigo in MS
Frohmanrsquos large series (1153) reported that 52 of MS patients with acute vertigo had benign paroxysmal positional vertigo while 32 had new brainstem lesions
To avoid misdiagnoses and inappropriate treatment interventions alternative etiologies should be considered in all acute vertigo episodes in MS
E M Frohman Neurology 2000 and Multiple Sclerosis 2003
Sixth Annual Intensive Update in Neurology 0915-162016
73
Vestibular compensation
bull Acute unilateral or bilateral vestibular loss will prompt compensation thru central cervico-ocular visual and proprioceptive pathways
bull Unilateral loss may require several weeks for compensation to occur and activity especially head movements hastens compensation
bull Vestibular suppressant medication prolongs compensation
Sixth Annual Intensive Update in Neurology 0915-162016
74
Vestibular Rehabilitation
bull Performed by certified physical therapists many sites (Epic order vestibular PT) or check Vestibular Disorders of America website for providers
bull Assessment and treatment of benign positional vertigo
bull Directed rehab programs for central andor peripheral vestibular compensationndash Unilateral vestibulopathies
ndash Bilateral vestibular hypofunction
ndash Mutifactorial balance deficits and fall risk assessment
Sixth Annual Intensive Update in Neurology 0915-162016
75
Summary of whatrsquos new in peripheral vestibular disorders
bull Lateral semicircular canal benign paroxysmal positional vertigo presents with horizontal positioning nystagmus
bull Oral steroids appear to improve outcome in acute vestibular neuritis
bull Intratympanic steroids offer promise in Menierersquos syndrome
bull Superior semicircular canal dehiscence causes pressure and noise induced dizziness VEMP offers diagnostic information
Sixth Annual Intensive Update in Neurology 0915-162016
76
References
BPPVndash White J Benign paroxysmal positional vertigo Cleveland Clinic Journal of
Medicine 71(9)722-728 2004
ndash White J Coale K Catalano P Oas J Diagnosis and management of lateral semicircular canal benign positional vertigo Otolaryngology-Head and Neck Surgery 133 278-284 2005
ndash White J Savvides P Cherian N Oas J Canalith repositioning for benign paroxysmal positional vertigo Otology and Neurotology 26 704-710 2005
ndash White J Oas J Diagnosis and management of lateral semicircular canal conversions during particle repositioning therapy Laryngoscope 115(10) 1895-1897 2005
ndash Acute Vestibular Syndrome Hotson JR Baloh RW N Engl J Med 1998 Sep 3339(10)680-5
ndash Methylprednisolone valacyclovir or the combination for vestibular neuritis Strupp M Zingler VC Arbusow V Niklas D Maag KP Dieterich M Bense S Theil D Jahn K Brandt T N Engl J Med 2004 Jul 22351(4)354-61
ndash Intratympanic steroids for Meacuteniegraveres disease or syndrome Phillips JS Westerberg BCochrane Database Syst Rev 2011 Jul 6(7)CD008514 Review
ndash Sound- andor pressure-induced vertigo due to bone dehiscence of the superior semicircular canal Minor LB Solomon D Zinreich JS Zee DSArch Otolaryngol Head Neck Surg 1998 Mar124(3)249-58
Sixth Annual Intensive Update in Neurology 0915-162016
13
DHT - Sitting
Sixth Annual Intensive Update in Neurology 0915-162016
14
DHT - HHR Orientation
Sixth Annual Intensive Update in Neurology 0915-162016
15
Dix-Hallpike to the Right
Sixth Annual Intensive Update in Neurology 0915-162016
16
Right posterior BPPV
Sixth Annual Intensive Update in Neurology 0915-162016
17
Canalith Repositioning Procedure
Sixth Annual Intensive Update in Neurology 0915-162016
18
Canalith Repositioning Procedure
Sixth Annual Intensive Update in Neurology 0915-162016
19
Canalith Repositioning Procedure
Sixth Annual Intensive Update in Neurology 0915-162016
20
Canalith Repositioning Procedure
Sixth Annual Intensive Update in Neurology 0915-162016
21
Canalith Repositioning
bull R posterior
BPPV
CPT 95992
Sixth Annual Intensive Update in Neurology 0915-162016
22
Posterior semicircular canalith repositioning
Sixth Annual Intensive Update in Neurology 0915-162016
23
Supine Positional Tests
Sixth Annual Intensive Update in Neurology 0915-162016
24
Lateral SCC-BPPV
bull Short latency horizontal nystagmus provoked by supine bilateral head turns with prolonged duration and poor fatigability
bull Uncommon (2-15 of total BPPV)
bull Two formsndash Geotropic ndash beating towards undermost ear
ndash Apogeotropic ndash beating away from undermost ear
Sixth Annual Intensive Update in Neurology 0915-162016
25
Geotropic Lateral Canal BPPV
bull Patient is positioned
first to the right lateral
position ndash nystagmus
beats right
bull Patient is then
positioned to the left
lateral position and
nystagmus reverses
and beats left
Sixth Annual Intensive Update in Neurology 0915-162016
26
Left Geotropic Lateral Semicircular Canal BPPV
Sixth Annual Intensive Update in Neurology 0915-162016
27
Repositioning Maneuvers for Geotropic Lateral Semicircular Canal (LSC) BPPV
bull Roll maneuver (Lempert Baloh)
ndash Patient rolls in 90 degree increments towards the good side
completely rolling over R LSC-BPPV repositioning shown
Sixth Annual Intensive Update in Neurology 0915-162016
28
Repositioning Maneuvers for Geotropic LSC BPPV
bull Gufoni maneuver
ndash Patient lies quickly onto good side and turns face-down to
floor for two minutes
Sixth Annual Intensive Update in Neurology 0915-162016
29
Left Apogeotropic LSC BPPV
Sixth Annual Intensive Update in Neurology 0915-162016
30
Nystagmus observed During Repositioning
bull Nystagmus should remain in the same torsional
direction as seen on DHT throughout the treatment of
posterior BPPV
bull Reversal of nystagmus direction suggests the particle
has fallen back into the canal and predicts failure
bull In Lateral BPPV nystagmus should beat towards the
good ear
Sixth Annual Intensive Update in Neurology 0915-162016
31
Posterior to Lateral canal conversion
Sixth Annual Intensive Update in Neurology 0915-162016
32
R Posterior to Lateral Canal conversion - geotropic
Sixth Annual Intensive Update in Neurology 0915-162016
33
BPPV Recurrence
bull Recurrence is common (15yr)
bull Otoconial adherence or mineralization demineralization
abnormalities may contribute to recurrence
bull Home canalith repositioning is effective (Radke 2004)
Sixth Annual Intensive Update in Neurology 0915-162016
34
Question 1
bull The most likely semicircular canal to be affected in
BPPV is
ndash 1) superior
ndash 2) lateral
ndash 3) posterior
ndash 4) combined
Sixth Annual Intensive Update in Neurology 0915-162016
35
Question 1
bull The most likely semicircular canal to be affected in
BPPV is
ndash 1) superior
ndash 2) lateral
ndash 3) posterior
ndash 4) combined
Answer 3 See references at end of lecture
Sixth Annual Intensive Update in Neurology 0915-162016
36
Acute Vestibular Syndrome
bull Rapid unilateral injury to either peripheral or central
vestibular structures produces prolonged vertigo
(days to weeks)
bull Severe vertigo nausea and vomiting spontaneous
nystagmus and postural instability
Sixth Annual Intensive Update in Neurology 0915-162016
37
Acute Vestibular Syndrome
bull 25 of patients with risk factors who present with isolated severe vertigo nystagmus and postural instability in the ER have an acute infarction of the inferior cerebellum (AICA PICA) Central imaging is suggested (Baloh 1998 NEJM)
ndash HTN diabetes smoking age gt65 Afib valvular heart disease occlusive vascular disease
Sixth Annual Intensive Update in Neurology 0915-162016
38
HINTS test
bull Head Impulse
bull Nystagmus
bull Test of Skew
ndash More sensitive than MRI in the diagnosis of acute stroke in
patients with acute vestibular syndrome with at least one risk
factor (Kattah J et al Stroke 2009)
Sixth Annual Intensive Update in Neurology 0915-162016
39
Head Impulse Test
bull No special equipment needed
bull Examiner stands in front of seated patient and
randomly moves head left and right Patient is asked to
focus on examinerrsquos nose
bull Rapid low amplitude thrust from lateral to straight
ahead causes a compensatory saccade when
performed towards the weak side
bull This finding is a permanent feature of unilateral
vestibular loss
Sixth Annual Intensive Update in Neurology 0915-162016
40
Head Impulse Test
Sixth Annual Intensive Update in Neurology 0915-162016
41
Head CT ndash Academy of Emergency Medicine 2013
bull The clinical value of head CT in isolated
dizzinessvertigo without any associated neurological
symptoms is very low
bull Less than 1 of head CTs ordered for dizziness and
vertigo have significant abnormalities and those
patients had severe headache and neurological deficits
in addition to dizziness
bull Forgoing unnecessary emergency head CTs could
save 360 million dollars annually
Sixth Annual Intensive Update in Neurology 0915-162016
42
Question 2
Head CT is not indicated for the evaluation of dizziness
and vertigo without additional neurological complaints because
1 It is too expensive
2 Less than 1 of CTs done for dizziness and vertigo are
abnormal and those patients had associated severe
neurological complaints
3 CT is the best imaging choice for the posterior fossa
4 Acoustic neuromas are well seen on CT
Sixth Annual Intensive Update in Neurology 0915-162016
43
Question 2
Head CT is not indicated for the evaluation of dizziness
and vertigo without additional neurological complaints because
1 It is too expensive
2 Less than 1 of CTs done for dizziness and vertigo are
abnormal and those patients had associated severe
neurological complaints
3 CT is the best imaging choice for the posterior fossa
4 Acoustic neuromas are well seen on CT
Answer 2 See references at end of lecture
Sixth Annual Intensive Update in Neurology 0915-162016
44
Acute Vestibular Syndrome
bull Usually peripheral and can be associated with
hearing loss (labyrinthitis)
bull Timely diagnosis improves recovery (ASAP
prednisone 1mgkgday for ten days and taper 10
mgday ndash Strupp M 2004 NEJM)
bull Avoid vestibular suppressants after 3 days and begin
vestibular rehabilitation to hasten compensation
Sixth Annual Intensive Update in Neurology 0915-162016
45
Question 3
bull Treatment for Acute Vestibular Syndrome
1) oral prednisone 1 mgkgday
2) Treat dehydration if necessary
3) Minimize vestibular suppressants after 3 days
4) Consider vestibular physical therapy
5) All of the above
Sixth Annual Intensive Update in Neurology 0915-162016
46
Question 3
bull Treatment for Acute Vestibular Syndrome
1) oral prednisone 1 mgkgday
2) Treat dehydration if necessary
3) Minimize vestibular suppressants after 3 days
4) Consider vestibular physical therapy
5) All of the above
Answer 5 See references at end of lecture
Sixth Annual Intensive Update in Neurology 0915-162016
47
Menierersquos syndrome
bull Fluctuating low frequency hearing loss
bull Tinnitus andor aural fullness
bull Vertigo lasting hours
Sixth Annual Intensive Update in Neurology 0915-162016
48
Normal
Sixth Annual Intensive Update in Neurology 0915-162016
49
Hydrops
Sixth Annual Intensive Update in Neurology 0915-162016
50
Menierersquos syndrome
ndash Diagnosis- Exclusion (RO acoustic)
ndash 23 of Menierersquos patients will improve spontaneously
ndash Treatment-1500 mgday low sodium triamterene375-
hydrochlorothiazide 25 vestibular suppressants (lorazepam
1 mg tablet sublingual Q 8 prn attacks) off label use
Sixth Annual Intensive Update in Neurology 0915-162016
51
Menierersquos syndrome ndash intratympanic steroids
ndash A newer treatment option for those failing medical management
ndash 90 response rate (Boleus-Aguirre MS Otology Neurotology
2008)
ndash Outcomes data confirm excellent clinical response in 3941
Menierersquos patients treated with intratympanic steroids in 2008
Sixth Annual Intensive Update in Neurology 0915-162016
52
Question 4
bull Menierersquos syndrome diagnosis is based on
1) Recurrent vertigo lasting at least 20 minutes
2) Aural fullness
3) Documented hearing loss
4) Other causes excluded
5) All of the above
Sixth Annual Intensive Update in Neurology 0915-162016
53
Question 4
bull Menierersquos syndrome diagnosis is based on
1) Recurrent vertigo lasting at least 20 minutes
2) Aural fullness
3) Documented hearing loss
4) Other causes excluded
5) All of the above
Answer 5 See references at end
Sixth Annual Intensive Update in Neurology 0915-162016
54
Superior Semicircular Canal Dehiscence (SSCD)
bull Sound andor pressure induced oscillopsia
bull CT reformatted in the plane of the superior canals
shows dehiscence of overlying bone
bull Low frequency ldquoconductive hyperacusisrdquo with elevated
bone thresholds normal reflexes and a conductive loss
Sixth Annual Intensive Update in Neurology 0915-162016
55
SSCD
bull Positive middle ear pressure or loud sound stimulates ampullofugal endolymph flow -canal activation
bull Eye movements are tonic upwards with ipsilateral intorsion
Sixth Annual Intensive Update in Neurology 0915-162016
56
SSCD
bull Valsalva against a closed glottis or jugular compression causes increased intracranial pressure and ampullopetal endolymph flow -canal inhibition
bull Eye movements are tonic downwards with ipsilateral extorsion
Sixth Annual Intensive Update in Neurology 0915-162016
57
Reconstructed left superior semicircular canal
Sixth Annual Intensive Update in Neurology 0915-162016
58
Sixth Annual Intensive Update in Neurology 0915-162016
59
SCHEMATIC DRAWING OF AN OTOLITHIC MACULA
Sixth Annual Intensive Update in Neurology 0915-162016
60
MACULAE OF THE UTRICLESACCULE
Sixth Annual Intensive Update in Neurology 0915-162016
61
HAIR CELLS
Sixth Annual Intensive Update in Neurology 0915-162016
62
SCANNING ELECTRON MICROGRAPH OF OTOCONIA
Sixth Annual Intensive Update in Neurology 0915-162016
63
Ocular VEMP
bull Response to loud clicks (80-110 dB) or forehead tap (Halmagyi and Curthoys 2003)
bull Contralateral oculomotor nucleus (CN III)
bull Inferior oblique muscle contraction (8 msec) detected by 5sec event triggered averaging
bull Noninverting electrode inferior to eye
bull Inverting below non-inverting electrode
bull Ground to forehead
bull Normal subjects have very low response
Sixth Annual Intensive Update in Neurology 0915-162016
64
Clinical applications
bull The o-VEMP is a sensitive test of otolithutricular
function
bull Complements vestibular test procedures that assess
semicircular canals
bull Abnormally robust responses are associated with third
window abnormalities such as semicircular canal
dehiscence
bull Serves as an excellent screening test for semicircular
canal dehiscence
Sixth Annual Intensive Update in Neurology 0915-162016
65
Question 5
bull Superior semicircular canal dehiscence
1) Causes increased ability to hear vibratory sounds
2) Improves hearing
3) Causes meningitis
4) Always needs surgical repair
Sixth Annual Intensive Update in Neurology 0915-162016
66
Question 5
bull Superior semicircular canal dehiscence
1) Causes increased ability to hear vibratory sounds
2) Improves hearing
3) Causes meningitis
4) Always needs surgical repair
Answer 1 References at end of lecture
Sixth Annual Intensive Update in Neurology 0915-162016
67
Enlarged vestibular aqueduct
bull Axial temporal bone CT showing right temporal bone with enlarged vestibular aqueduct (arrow)
bull Vestibular symptoms accompany progressive sensorineural hearing loss in 4-50 of patients in selected series
Sixth Annual Intensive Update in Neurology 0915-162016
68
Migraine
bull Family or personal history of migraine
bull 25 of migraine patients experience spontaneous vertigo (Baloh 1997)
bull Neuhauser criteria(2001) have been used to construct structured diagnostic interviews (Furman 2003)
bull Not always associated with headache
bull Motion sensitivity
bull Medication responsive
Sixth Annual Intensive Update in Neurology 0915-162016
69
ICHD-III beta Criteria for Vestibular Migraine
bull A At least five attacks fulfilling criteria C and D
bull B A current or past history of 11 migraine without aura or 12 migraine with aura
bull C Vestibular symptoms of moderate or severe intensity lasting between 5 minutes and 72 hours
bull D At least 50 associated with one of the following migrainous features
ndash 1 Headaches
ndash 2 Photophobia and phonophobia
ndash 3 Visual Aura
bull E Not better accounted for by another vestibular disorder
Sixth Annual Intensive Update in Neurology 0915-162016
70
Incidence of vertigo in MS
bull True vertigo (the perception of environmental
movement typically rotational) occurs in about
20 of MS patients and is the presenting
manifestation in 5
bull Up to 78 of MS patients report abnormalities
in balance at some point in their illness
Frohman et al Multiple Sclerosis 2003 9250
Sixth Annual Intensive Update in Neurology 0915-162016
71
Vertigo in MS due to demyelination
bull The most common neuroanatomic locations are the
root entry zone of CN VIII (giving absent calorics but
normal hearing and fixation suppression) and the
medial vestibular nucleus
bull Usually treated with steroids and vestibular
suppressants
Brandt 1993 Gass 1998 Thomke 1999
Sixth Annual Intensive Update in Neurology 0915-162016
72
Vertigo in MS
Frohmanrsquos large series (1153) reported that 52 of MS patients with acute vertigo had benign paroxysmal positional vertigo while 32 had new brainstem lesions
To avoid misdiagnoses and inappropriate treatment interventions alternative etiologies should be considered in all acute vertigo episodes in MS
E M Frohman Neurology 2000 and Multiple Sclerosis 2003
Sixth Annual Intensive Update in Neurology 0915-162016
73
Vestibular compensation
bull Acute unilateral or bilateral vestibular loss will prompt compensation thru central cervico-ocular visual and proprioceptive pathways
bull Unilateral loss may require several weeks for compensation to occur and activity especially head movements hastens compensation
bull Vestibular suppressant medication prolongs compensation
Sixth Annual Intensive Update in Neurology 0915-162016
74
Vestibular Rehabilitation
bull Performed by certified physical therapists many sites (Epic order vestibular PT) or check Vestibular Disorders of America website for providers
bull Assessment and treatment of benign positional vertigo
bull Directed rehab programs for central andor peripheral vestibular compensationndash Unilateral vestibulopathies
ndash Bilateral vestibular hypofunction
ndash Mutifactorial balance deficits and fall risk assessment
Sixth Annual Intensive Update in Neurology 0915-162016
75
Summary of whatrsquos new in peripheral vestibular disorders
bull Lateral semicircular canal benign paroxysmal positional vertigo presents with horizontal positioning nystagmus
bull Oral steroids appear to improve outcome in acute vestibular neuritis
bull Intratympanic steroids offer promise in Menierersquos syndrome
bull Superior semicircular canal dehiscence causes pressure and noise induced dizziness VEMP offers diagnostic information
Sixth Annual Intensive Update in Neurology 0915-162016
76
References
BPPVndash White J Benign paroxysmal positional vertigo Cleveland Clinic Journal of
Medicine 71(9)722-728 2004
ndash White J Coale K Catalano P Oas J Diagnosis and management of lateral semicircular canal benign positional vertigo Otolaryngology-Head and Neck Surgery 133 278-284 2005
ndash White J Savvides P Cherian N Oas J Canalith repositioning for benign paroxysmal positional vertigo Otology and Neurotology 26 704-710 2005
ndash White J Oas J Diagnosis and management of lateral semicircular canal conversions during particle repositioning therapy Laryngoscope 115(10) 1895-1897 2005
ndash Acute Vestibular Syndrome Hotson JR Baloh RW N Engl J Med 1998 Sep 3339(10)680-5
ndash Methylprednisolone valacyclovir or the combination for vestibular neuritis Strupp M Zingler VC Arbusow V Niklas D Maag KP Dieterich M Bense S Theil D Jahn K Brandt T N Engl J Med 2004 Jul 22351(4)354-61
ndash Intratympanic steroids for Meacuteniegraveres disease or syndrome Phillips JS Westerberg BCochrane Database Syst Rev 2011 Jul 6(7)CD008514 Review
ndash Sound- andor pressure-induced vertigo due to bone dehiscence of the superior semicircular canal Minor LB Solomon D Zinreich JS Zee DSArch Otolaryngol Head Neck Surg 1998 Mar124(3)249-58
Sixth Annual Intensive Update in Neurology 0915-162016
14
DHT - HHR Orientation
Sixth Annual Intensive Update in Neurology 0915-162016
15
Dix-Hallpike to the Right
Sixth Annual Intensive Update in Neurology 0915-162016
16
Right posterior BPPV
Sixth Annual Intensive Update in Neurology 0915-162016
17
Canalith Repositioning Procedure
Sixth Annual Intensive Update in Neurology 0915-162016
18
Canalith Repositioning Procedure
Sixth Annual Intensive Update in Neurology 0915-162016
19
Canalith Repositioning Procedure
Sixth Annual Intensive Update in Neurology 0915-162016
20
Canalith Repositioning Procedure
Sixth Annual Intensive Update in Neurology 0915-162016
21
Canalith Repositioning
bull R posterior
BPPV
CPT 95992
Sixth Annual Intensive Update in Neurology 0915-162016
22
Posterior semicircular canalith repositioning
Sixth Annual Intensive Update in Neurology 0915-162016
23
Supine Positional Tests
Sixth Annual Intensive Update in Neurology 0915-162016
24
Lateral SCC-BPPV
bull Short latency horizontal nystagmus provoked by supine bilateral head turns with prolonged duration and poor fatigability
bull Uncommon (2-15 of total BPPV)
bull Two formsndash Geotropic ndash beating towards undermost ear
ndash Apogeotropic ndash beating away from undermost ear
Sixth Annual Intensive Update in Neurology 0915-162016
25
Geotropic Lateral Canal BPPV
bull Patient is positioned
first to the right lateral
position ndash nystagmus
beats right
bull Patient is then
positioned to the left
lateral position and
nystagmus reverses
and beats left
Sixth Annual Intensive Update in Neurology 0915-162016
26
Left Geotropic Lateral Semicircular Canal BPPV
Sixth Annual Intensive Update in Neurology 0915-162016
27
Repositioning Maneuvers for Geotropic Lateral Semicircular Canal (LSC) BPPV
bull Roll maneuver (Lempert Baloh)
ndash Patient rolls in 90 degree increments towards the good side
completely rolling over R LSC-BPPV repositioning shown
Sixth Annual Intensive Update in Neurology 0915-162016
28
Repositioning Maneuvers for Geotropic LSC BPPV
bull Gufoni maneuver
ndash Patient lies quickly onto good side and turns face-down to
floor for two minutes
Sixth Annual Intensive Update in Neurology 0915-162016
29
Left Apogeotropic LSC BPPV
Sixth Annual Intensive Update in Neurology 0915-162016
30
Nystagmus observed During Repositioning
bull Nystagmus should remain in the same torsional
direction as seen on DHT throughout the treatment of
posterior BPPV
bull Reversal of nystagmus direction suggests the particle
has fallen back into the canal and predicts failure
bull In Lateral BPPV nystagmus should beat towards the
good ear
Sixth Annual Intensive Update in Neurology 0915-162016
31
Posterior to Lateral canal conversion
Sixth Annual Intensive Update in Neurology 0915-162016
32
R Posterior to Lateral Canal conversion - geotropic
Sixth Annual Intensive Update in Neurology 0915-162016
33
BPPV Recurrence
bull Recurrence is common (15yr)
bull Otoconial adherence or mineralization demineralization
abnormalities may contribute to recurrence
bull Home canalith repositioning is effective (Radke 2004)
Sixth Annual Intensive Update in Neurology 0915-162016
34
Question 1
bull The most likely semicircular canal to be affected in
BPPV is
ndash 1) superior
ndash 2) lateral
ndash 3) posterior
ndash 4) combined
Sixth Annual Intensive Update in Neurology 0915-162016
35
Question 1
bull The most likely semicircular canal to be affected in
BPPV is
ndash 1) superior
ndash 2) lateral
ndash 3) posterior
ndash 4) combined
Answer 3 See references at end of lecture
Sixth Annual Intensive Update in Neurology 0915-162016
36
Acute Vestibular Syndrome
bull Rapid unilateral injury to either peripheral or central
vestibular structures produces prolonged vertigo
(days to weeks)
bull Severe vertigo nausea and vomiting spontaneous
nystagmus and postural instability
Sixth Annual Intensive Update in Neurology 0915-162016
37
Acute Vestibular Syndrome
bull 25 of patients with risk factors who present with isolated severe vertigo nystagmus and postural instability in the ER have an acute infarction of the inferior cerebellum (AICA PICA) Central imaging is suggested (Baloh 1998 NEJM)
ndash HTN diabetes smoking age gt65 Afib valvular heart disease occlusive vascular disease
Sixth Annual Intensive Update in Neurology 0915-162016
38
HINTS test
bull Head Impulse
bull Nystagmus
bull Test of Skew
ndash More sensitive than MRI in the diagnosis of acute stroke in
patients with acute vestibular syndrome with at least one risk
factor (Kattah J et al Stroke 2009)
Sixth Annual Intensive Update in Neurology 0915-162016
39
Head Impulse Test
bull No special equipment needed
bull Examiner stands in front of seated patient and
randomly moves head left and right Patient is asked to
focus on examinerrsquos nose
bull Rapid low amplitude thrust from lateral to straight
ahead causes a compensatory saccade when
performed towards the weak side
bull This finding is a permanent feature of unilateral
vestibular loss
Sixth Annual Intensive Update in Neurology 0915-162016
40
Head Impulse Test
Sixth Annual Intensive Update in Neurology 0915-162016
41
Head CT ndash Academy of Emergency Medicine 2013
bull The clinical value of head CT in isolated
dizzinessvertigo without any associated neurological
symptoms is very low
bull Less than 1 of head CTs ordered for dizziness and
vertigo have significant abnormalities and those
patients had severe headache and neurological deficits
in addition to dizziness
bull Forgoing unnecessary emergency head CTs could
save 360 million dollars annually
Sixth Annual Intensive Update in Neurology 0915-162016
42
Question 2
Head CT is not indicated for the evaluation of dizziness
and vertigo without additional neurological complaints because
1 It is too expensive
2 Less than 1 of CTs done for dizziness and vertigo are
abnormal and those patients had associated severe
neurological complaints
3 CT is the best imaging choice for the posterior fossa
4 Acoustic neuromas are well seen on CT
Sixth Annual Intensive Update in Neurology 0915-162016
43
Question 2
Head CT is not indicated for the evaluation of dizziness
and vertigo without additional neurological complaints because
1 It is too expensive
2 Less than 1 of CTs done for dizziness and vertigo are
abnormal and those patients had associated severe
neurological complaints
3 CT is the best imaging choice for the posterior fossa
4 Acoustic neuromas are well seen on CT
Answer 2 See references at end of lecture
Sixth Annual Intensive Update in Neurology 0915-162016
44
Acute Vestibular Syndrome
bull Usually peripheral and can be associated with
hearing loss (labyrinthitis)
bull Timely diagnosis improves recovery (ASAP
prednisone 1mgkgday for ten days and taper 10
mgday ndash Strupp M 2004 NEJM)
bull Avoid vestibular suppressants after 3 days and begin
vestibular rehabilitation to hasten compensation
Sixth Annual Intensive Update in Neurology 0915-162016
45
Question 3
bull Treatment for Acute Vestibular Syndrome
1) oral prednisone 1 mgkgday
2) Treat dehydration if necessary
3) Minimize vestibular suppressants after 3 days
4) Consider vestibular physical therapy
5) All of the above
Sixth Annual Intensive Update in Neurology 0915-162016
46
Question 3
bull Treatment for Acute Vestibular Syndrome
1) oral prednisone 1 mgkgday
2) Treat dehydration if necessary
3) Minimize vestibular suppressants after 3 days
4) Consider vestibular physical therapy
5) All of the above
Answer 5 See references at end of lecture
Sixth Annual Intensive Update in Neurology 0915-162016
47
Menierersquos syndrome
bull Fluctuating low frequency hearing loss
bull Tinnitus andor aural fullness
bull Vertigo lasting hours
Sixth Annual Intensive Update in Neurology 0915-162016
48
Normal
Sixth Annual Intensive Update in Neurology 0915-162016
49
Hydrops
Sixth Annual Intensive Update in Neurology 0915-162016
50
Menierersquos syndrome
ndash Diagnosis- Exclusion (RO acoustic)
ndash 23 of Menierersquos patients will improve spontaneously
ndash Treatment-1500 mgday low sodium triamterene375-
hydrochlorothiazide 25 vestibular suppressants (lorazepam
1 mg tablet sublingual Q 8 prn attacks) off label use
Sixth Annual Intensive Update in Neurology 0915-162016
51
Menierersquos syndrome ndash intratympanic steroids
ndash A newer treatment option for those failing medical management
ndash 90 response rate (Boleus-Aguirre MS Otology Neurotology
2008)
ndash Outcomes data confirm excellent clinical response in 3941
Menierersquos patients treated with intratympanic steroids in 2008
Sixth Annual Intensive Update in Neurology 0915-162016
52
Question 4
bull Menierersquos syndrome diagnosis is based on
1) Recurrent vertigo lasting at least 20 minutes
2) Aural fullness
3) Documented hearing loss
4) Other causes excluded
5) All of the above
Sixth Annual Intensive Update in Neurology 0915-162016
53
Question 4
bull Menierersquos syndrome diagnosis is based on
1) Recurrent vertigo lasting at least 20 minutes
2) Aural fullness
3) Documented hearing loss
4) Other causes excluded
5) All of the above
Answer 5 See references at end
Sixth Annual Intensive Update in Neurology 0915-162016
54
Superior Semicircular Canal Dehiscence (SSCD)
bull Sound andor pressure induced oscillopsia
bull CT reformatted in the plane of the superior canals
shows dehiscence of overlying bone
bull Low frequency ldquoconductive hyperacusisrdquo with elevated
bone thresholds normal reflexes and a conductive loss
Sixth Annual Intensive Update in Neurology 0915-162016
55
SSCD
bull Positive middle ear pressure or loud sound stimulates ampullofugal endolymph flow -canal activation
bull Eye movements are tonic upwards with ipsilateral intorsion
Sixth Annual Intensive Update in Neurology 0915-162016
56
SSCD
bull Valsalva against a closed glottis or jugular compression causes increased intracranial pressure and ampullopetal endolymph flow -canal inhibition
bull Eye movements are tonic downwards with ipsilateral extorsion
Sixth Annual Intensive Update in Neurology 0915-162016
57
Reconstructed left superior semicircular canal
Sixth Annual Intensive Update in Neurology 0915-162016
58
Sixth Annual Intensive Update in Neurology 0915-162016
59
SCHEMATIC DRAWING OF AN OTOLITHIC MACULA
Sixth Annual Intensive Update in Neurology 0915-162016
60
MACULAE OF THE UTRICLESACCULE
Sixth Annual Intensive Update in Neurology 0915-162016
61
HAIR CELLS
Sixth Annual Intensive Update in Neurology 0915-162016
62
SCANNING ELECTRON MICROGRAPH OF OTOCONIA
Sixth Annual Intensive Update in Neurology 0915-162016
63
Ocular VEMP
bull Response to loud clicks (80-110 dB) or forehead tap (Halmagyi and Curthoys 2003)
bull Contralateral oculomotor nucleus (CN III)
bull Inferior oblique muscle contraction (8 msec) detected by 5sec event triggered averaging
bull Noninverting electrode inferior to eye
bull Inverting below non-inverting electrode
bull Ground to forehead
bull Normal subjects have very low response
Sixth Annual Intensive Update in Neurology 0915-162016
64
Clinical applications
bull The o-VEMP is a sensitive test of otolithutricular
function
bull Complements vestibular test procedures that assess
semicircular canals
bull Abnormally robust responses are associated with third
window abnormalities such as semicircular canal
dehiscence
bull Serves as an excellent screening test for semicircular
canal dehiscence
Sixth Annual Intensive Update in Neurology 0915-162016
65
Question 5
bull Superior semicircular canal dehiscence
1) Causes increased ability to hear vibratory sounds
2) Improves hearing
3) Causes meningitis
4) Always needs surgical repair
Sixth Annual Intensive Update in Neurology 0915-162016
66
Question 5
bull Superior semicircular canal dehiscence
1) Causes increased ability to hear vibratory sounds
2) Improves hearing
3) Causes meningitis
4) Always needs surgical repair
Answer 1 References at end of lecture
Sixth Annual Intensive Update in Neurology 0915-162016
67
Enlarged vestibular aqueduct
bull Axial temporal bone CT showing right temporal bone with enlarged vestibular aqueduct (arrow)
bull Vestibular symptoms accompany progressive sensorineural hearing loss in 4-50 of patients in selected series
Sixth Annual Intensive Update in Neurology 0915-162016
68
Migraine
bull Family or personal history of migraine
bull 25 of migraine patients experience spontaneous vertigo (Baloh 1997)
bull Neuhauser criteria(2001) have been used to construct structured diagnostic interviews (Furman 2003)
bull Not always associated with headache
bull Motion sensitivity
bull Medication responsive
Sixth Annual Intensive Update in Neurology 0915-162016
69
ICHD-III beta Criteria for Vestibular Migraine
bull A At least five attacks fulfilling criteria C and D
bull B A current or past history of 11 migraine without aura or 12 migraine with aura
bull C Vestibular symptoms of moderate or severe intensity lasting between 5 minutes and 72 hours
bull D At least 50 associated with one of the following migrainous features
ndash 1 Headaches
ndash 2 Photophobia and phonophobia
ndash 3 Visual Aura
bull E Not better accounted for by another vestibular disorder
Sixth Annual Intensive Update in Neurology 0915-162016
70
Incidence of vertigo in MS
bull True vertigo (the perception of environmental
movement typically rotational) occurs in about
20 of MS patients and is the presenting
manifestation in 5
bull Up to 78 of MS patients report abnormalities
in balance at some point in their illness
Frohman et al Multiple Sclerosis 2003 9250
Sixth Annual Intensive Update in Neurology 0915-162016
71
Vertigo in MS due to demyelination
bull The most common neuroanatomic locations are the
root entry zone of CN VIII (giving absent calorics but
normal hearing and fixation suppression) and the
medial vestibular nucleus
bull Usually treated with steroids and vestibular
suppressants
Brandt 1993 Gass 1998 Thomke 1999
Sixth Annual Intensive Update in Neurology 0915-162016
72
Vertigo in MS
Frohmanrsquos large series (1153) reported that 52 of MS patients with acute vertigo had benign paroxysmal positional vertigo while 32 had new brainstem lesions
To avoid misdiagnoses and inappropriate treatment interventions alternative etiologies should be considered in all acute vertigo episodes in MS
E M Frohman Neurology 2000 and Multiple Sclerosis 2003
Sixth Annual Intensive Update in Neurology 0915-162016
73
Vestibular compensation
bull Acute unilateral or bilateral vestibular loss will prompt compensation thru central cervico-ocular visual and proprioceptive pathways
bull Unilateral loss may require several weeks for compensation to occur and activity especially head movements hastens compensation
bull Vestibular suppressant medication prolongs compensation
Sixth Annual Intensive Update in Neurology 0915-162016
74
Vestibular Rehabilitation
bull Performed by certified physical therapists many sites (Epic order vestibular PT) or check Vestibular Disorders of America website for providers
bull Assessment and treatment of benign positional vertigo
bull Directed rehab programs for central andor peripheral vestibular compensationndash Unilateral vestibulopathies
ndash Bilateral vestibular hypofunction
ndash Mutifactorial balance deficits and fall risk assessment
Sixth Annual Intensive Update in Neurology 0915-162016
75
Summary of whatrsquos new in peripheral vestibular disorders
bull Lateral semicircular canal benign paroxysmal positional vertigo presents with horizontal positioning nystagmus
bull Oral steroids appear to improve outcome in acute vestibular neuritis
bull Intratympanic steroids offer promise in Menierersquos syndrome
bull Superior semicircular canal dehiscence causes pressure and noise induced dizziness VEMP offers diagnostic information
Sixth Annual Intensive Update in Neurology 0915-162016
76
References
BPPVndash White J Benign paroxysmal positional vertigo Cleveland Clinic Journal of
Medicine 71(9)722-728 2004
ndash White J Coale K Catalano P Oas J Diagnosis and management of lateral semicircular canal benign positional vertigo Otolaryngology-Head and Neck Surgery 133 278-284 2005
ndash White J Savvides P Cherian N Oas J Canalith repositioning for benign paroxysmal positional vertigo Otology and Neurotology 26 704-710 2005
ndash White J Oas J Diagnosis and management of lateral semicircular canal conversions during particle repositioning therapy Laryngoscope 115(10) 1895-1897 2005
ndash Acute Vestibular Syndrome Hotson JR Baloh RW N Engl J Med 1998 Sep 3339(10)680-5
ndash Methylprednisolone valacyclovir or the combination for vestibular neuritis Strupp M Zingler VC Arbusow V Niklas D Maag KP Dieterich M Bense S Theil D Jahn K Brandt T N Engl J Med 2004 Jul 22351(4)354-61
ndash Intratympanic steroids for Meacuteniegraveres disease or syndrome Phillips JS Westerberg BCochrane Database Syst Rev 2011 Jul 6(7)CD008514 Review
ndash Sound- andor pressure-induced vertigo due to bone dehiscence of the superior semicircular canal Minor LB Solomon D Zinreich JS Zee DSArch Otolaryngol Head Neck Surg 1998 Mar124(3)249-58
Sixth Annual Intensive Update in Neurology 0915-162016
15
Dix-Hallpike to the Right
Sixth Annual Intensive Update in Neurology 0915-162016
16
Right posterior BPPV
Sixth Annual Intensive Update in Neurology 0915-162016
17
Canalith Repositioning Procedure
Sixth Annual Intensive Update in Neurology 0915-162016
18
Canalith Repositioning Procedure
Sixth Annual Intensive Update in Neurology 0915-162016
19
Canalith Repositioning Procedure
Sixth Annual Intensive Update in Neurology 0915-162016
20
Canalith Repositioning Procedure
Sixth Annual Intensive Update in Neurology 0915-162016
21
Canalith Repositioning
bull R posterior
BPPV
CPT 95992
Sixth Annual Intensive Update in Neurology 0915-162016
22
Posterior semicircular canalith repositioning
Sixth Annual Intensive Update in Neurology 0915-162016
23
Supine Positional Tests
Sixth Annual Intensive Update in Neurology 0915-162016
24
Lateral SCC-BPPV
bull Short latency horizontal nystagmus provoked by supine bilateral head turns with prolonged duration and poor fatigability
bull Uncommon (2-15 of total BPPV)
bull Two formsndash Geotropic ndash beating towards undermost ear
ndash Apogeotropic ndash beating away from undermost ear
Sixth Annual Intensive Update in Neurology 0915-162016
25
Geotropic Lateral Canal BPPV
bull Patient is positioned
first to the right lateral
position ndash nystagmus
beats right
bull Patient is then
positioned to the left
lateral position and
nystagmus reverses
and beats left
Sixth Annual Intensive Update in Neurology 0915-162016
26
Left Geotropic Lateral Semicircular Canal BPPV
Sixth Annual Intensive Update in Neurology 0915-162016
27
Repositioning Maneuvers for Geotropic Lateral Semicircular Canal (LSC) BPPV
bull Roll maneuver (Lempert Baloh)
ndash Patient rolls in 90 degree increments towards the good side
completely rolling over R LSC-BPPV repositioning shown
Sixth Annual Intensive Update in Neurology 0915-162016
28
Repositioning Maneuvers for Geotropic LSC BPPV
bull Gufoni maneuver
ndash Patient lies quickly onto good side and turns face-down to
floor for two minutes
Sixth Annual Intensive Update in Neurology 0915-162016
29
Left Apogeotropic LSC BPPV
Sixth Annual Intensive Update in Neurology 0915-162016
30
Nystagmus observed During Repositioning
bull Nystagmus should remain in the same torsional
direction as seen on DHT throughout the treatment of
posterior BPPV
bull Reversal of nystagmus direction suggests the particle
has fallen back into the canal and predicts failure
bull In Lateral BPPV nystagmus should beat towards the
good ear
Sixth Annual Intensive Update in Neurology 0915-162016
31
Posterior to Lateral canal conversion
Sixth Annual Intensive Update in Neurology 0915-162016
32
R Posterior to Lateral Canal conversion - geotropic
Sixth Annual Intensive Update in Neurology 0915-162016
33
BPPV Recurrence
bull Recurrence is common (15yr)
bull Otoconial adherence or mineralization demineralization
abnormalities may contribute to recurrence
bull Home canalith repositioning is effective (Radke 2004)
Sixth Annual Intensive Update in Neurology 0915-162016
34
Question 1
bull The most likely semicircular canal to be affected in
BPPV is
ndash 1) superior
ndash 2) lateral
ndash 3) posterior
ndash 4) combined
Sixth Annual Intensive Update in Neurology 0915-162016
35
Question 1
bull The most likely semicircular canal to be affected in
BPPV is
ndash 1) superior
ndash 2) lateral
ndash 3) posterior
ndash 4) combined
Answer 3 See references at end of lecture
Sixth Annual Intensive Update in Neurology 0915-162016
36
Acute Vestibular Syndrome
bull Rapid unilateral injury to either peripheral or central
vestibular structures produces prolonged vertigo
(days to weeks)
bull Severe vertigo nausea and vomiting spontaneous
nystagmus and postural instability
Sixth Annual Intensive Update in Neurology 0915-162016
37
Acute Vestibular Syndrome
bull 25 of patients with risk factors who present with isolated severe vertigo nystagmus and postural instability in the ER have an acute infarction of the inferior cerebellum (AICA PICA) Central imaging is suggested (Baloh 1998 NEJM)
ndash HTN diabetes smoking age gt65 Afib valvular heart disease occlusive vascular disease
Sixth Annual Intensive Update in Neurology 0915-162016
38
HINTS test
bull Head Impulse
bull Nystagmus
bull Test of Skew
ndash More sensitive than MRI in the diagnosis of acute stroke in
patients with acute vestibular syndrome with at least one risk
factor (Kattah J et al Stroke 2009)
Sixth Annual Intensive Update in Neurology 0915-162016
39
Head Impulse Test
bull No special equipment needed
bull Examiner stands in front of seated patient and
randomly moves head left and right Patient is asked to
focus on examinerrsquos nose
bull Rapid low amplitude thrust from lateral to straight
ahead causes a compensatory saccade when
performed towards the weak side
bull This finding is a permanent feature of unilateral
vestibular loss
Sixth Annual Intensive Update in Neurology 0915-162016
40
Head Impulse Test
Sixth Annual Intensive Update in Neurology 0915-162016
41
Head CT ndash Academy of Emergency Medicine 2013
bull The clinical value of head CT in isolated
dizzinessvertigo without any associated neurological
symptoms is very low
bull Less than 1 of head CTs ordered for dizziness and
vertigo have significant abnormalities and those
patients had severe headache and neurological deficits
in addition to dizziness
bull Forgoing unnecessary emergency head CTs could
save 360 million dollars annually
Sixth Annual Intensive Update in Neurology 0915-162016
42
Question 2
Head CT is not indicated for the evaluation of dizziness
and vertigo without additional neurological complaints because
1 It is too expensive
2 Less than 1 of CTs done for dizziness and vertigo are
abnormal and those patients had associated severe
neurological complaints
3 CT is the best imaging choice for the posterior fossa
4 Acoustic neuromas are well seen on CT
Sixth Annual Intensive Update in Neurology 0915-162016
43
Question 2
Head CT is not indicated for the evaluation of dizziness
and vertigo without additional neurological complaints because
1 It is too expensive
2 Less than 1 of CTs done for dizziness and vertigo are
abnormal and those patients had associated severe
neurological complaints
3 CT is the best imaging choice for the posterior fossa
4 Acoustic neuromas are well seen on CT
Answer 2 See references at end of lecture
Sixth Annual Intensive Update in Neurology 0915-162016
44
Acute Vestibular Syndrome
bull Usually peripheral and can be associated with
hearing loss (labyrinthitis)
bull Timely diagnosis improves recovery (ASAP
prednisone 1mgkgday for ten days and taper 10
mgday ndash Strupp M 2004 NEJM)
bull Avoid vestibular suppressants after 3 days and begin
vestibular rehabilitation to hasten compensation
Sixth Annual Intensive Update in Neurology 0915-162016
45
Question 3
bull Treatment for Acute Vestibular Syndrome
1) oral prednisone 1 mgkgday
2) Treat dehydration if necessary
3) Minimize vestibular suppressants after 3 days
4) Consider vestibular physical therapy
5) All of the above
Sixth Annual Intensive Update in Neurology 0915-162016
46
Question 3
bull Treatment for Acute Vestibular Syndrome
1) oral prednisone 1 mgkgday
2) Treat dehydration if necessary
3) Minimize vestibular suppressants after 3 days
4) Consider vestibular physical therapy
5) All of the above
Answer 5 See references at end of lecture
Sixth Annual Intensive Update in Neurology 0915-162016
47
Menierersquos syndrome
bull Fluctuating low frequency hearing loss
bull Tinnitus andor aural fullness
bull Vertigo lasting hours
Sixth Annual Intensive Update in Neurology 0915-162016
48
Normal
Sixth Annual Intensive Update in Neurology 0915-162016
49
Hydrops
Sixth Annual Intensive Update in Neurology 0915-162016
50
Menierersquos syndrome
ndash Diagnosis- Exclusion (RO acoustic)
ndash 23 of Menierersquos patients will improve spontaneously
ndash Treatment-1500 mgday low sodium triamterene375-
hydrochlorothiazide 25 vestibular suppressants (lorazepam
1 mg tablet sublingual Q 8 prn attacks) off label use
Sixth Annual Intensive Update in Neurology 0915-162016
51
Menierersquos syndrome ndash intratympanic steroids
ndash A newer treatment option for those failing medical management
ndash 90 response rate (Boleus-Aguirre MS Otology Neurotology
2008)
ndash Outcomes data confirm excellent clinical response in 3941
Menierersquos patients treated with intratympanic steroids in 2008
Sixth Annual Intensive Update in Neurology 0915-162016
52
Question 4
bull Menierersquos syndrome diagnosis is based on
1) Recurrent vertigo lasting at least 20 minutes
2) Aural fullness
3) Documented hearing loss
4) Other causes excluded
5) All of the above
Sixth Annual Intensive Update in Neurology 0915-162016
53
Question 4
bull Menierersquos syndrome diagnosis is based on
1) Recurrent vertigo lasting at least 20 minutes
2) Aural fullness
3) Documented hearing loss
4) Other causes excluded
5) All of the above
Answer 5 See references at end
Sixth Annual Intensive Update in Neurology 0915-162016
54
Superior Semicircular Canal Dehiscence (SSCD)
bull Sound andor pressure induced oscillopsia
bull CT reformatted in the plane of the superior canals
shows dehiscence of overlying bone
bull Low frequency ldquoconductive hyperacusisrdquo with elevated
bone thresholds normal reflexes and a conductive loss
Sixth Annual Intensive Update in Neurology 0915-162016
55
SSCD
bull Positive middle ear pressure or loud sound stimulates ampullofugal endolymph flow -canal activation
bull Eye movements are tonic upwards with ipsilateral intorsion
Sixth Annual Intensive Update in Neurology 0915-162016
56
SSCD
bull Valsalva against a closed glottis or jugular compression causes increased intracranial pressure and ampullopetal endolymph flow -canal inhibition
bull Eye movements are tonic downwards with ipsilateral extorsion
Sixth Annual Intensive Update in Neurology 0915-162016
57
Reconstructed left superior semicircular canal
Sixth Annual Intensive Update in Neurology 0915-162016
58
Sixth Annual Intensive Update in Neurology 0915-162016
59
SCHEMATIC DRAWING OF AN OTOLITHIC MACULA
Sixth Annual Intensive Update in Neurology 0915-162016
60
MACULAE OF THE UTRICLESACCULE
Sixth Annual Intensive Update in Neurology 0915-162016
61
HAIR CELLS
Sixth Annual Intensive Update in Neurology 0915-162016
62
SCANNING ELECTRON MICROGRAPH OF OTOCONIA
Sixth Annual Intensive Update in Neurology 0915-162016
63
Ocular VEMP
bull Response to loud clicks (80-110 dB) or forehead tap (Halmagyi and Curthoys 2003)
bull Contralateral oculomotor nucleus (CN III)
bull Inferior oblique muscle contraction (8 msec) detected by 5sec event triggered averaging
bull Noninverting electrode inferior to eye
bull Inverting below non-inverting electrode
bull Ground to forehead
bull Normal subjects have very low response
Sixth Annual Intensive Update in Neurology 0915-162016
64
Clinical applications
bull The o-VEMP is a sensitive test of otolithutricular
function
bull Complements vestibular test procedures that assess
semicircular canals
bull Abnormally robust responses are associated with third
window abnormalities such as semicircular canal
dehiscence
bull Serves as an excellent screening test for semicircular
canal dehiscence
Sixth Annual Intensive Update in Neurology 0915-162016
65
Question 5
bull Superior semicircular canal dehiscence
1) Causes increased ability to hear vibratory sounds
2) Improves hearing
3) Causes meningitis
4) Always needs surgical repair
Sixth Annual Intensive Update in Neurology 0915-162016
66
Question 5
bull Superior semicircular canal dehiscence
1) Causes increased ability to hear vibratory sounds
2) Improves hearing
3) Causes meningitis
4) Always needs surgical repair
Answer 1 References at end of lecture
Sixth Annual Intensive Update in Neurology 0915-162016
67
Enlarged vestibular aqueduct
bull Axial temporal bone CT showing right temporal bone with enlarged vestibular aqueduct (arrow)
bull Vestibular symptoms accompany progressive sensorineural hearing loss in 4-50 of patients in selected series
Sixth Annual Intensive Update in Neurology 0915-162016
68
Migraine
bull Family or personal history of migraine
bull 25 of migraine patients experience spontaneous vertigo (Baloh 1997)
bull Neuhauser criteria(2001) have been used to construct structured diagnostic interviews (Furman 2003)
bull Not always associated with headache
bull Motion sensitivity
bull Medication responsive
Sixth Annual Intensive Update in Neurology 0915-162016
69
ICHD-III beta Criteria for Vestibular Migraine
bull A At least five attacks fulfilling criteria C and D
bull B A current or past history of 11 migraine without aura or 12 migraine with aura
bull C Vestibular symptoms of moderate or severe intensity lasting between 5 minutes and 72 hours
bull D At least 50 associated with one of the following migrainous features
ndash 1 Headaches
ndash 2 Photophobia and phonophobia
ndash 3 Visual Aura
bull E Not better accounted for by another vestibular disorder
Sixth Annual Intensive Update in Neurology 0915-162016
70
Incidence of vertigo in MS
bull True vertigo (the perception of environmental
movement typically rotational) occurs in about
20 of MS patients and is the presenting
manifestation in 5
bull Up to 78 of MS patients report abnormalities
in balance at some point in their illness
Frohman et al Multiple Sclerosis 2003 9250
Sixth Annual Intensive Update in Neurology 0915-162016
71
Vertigo in MS due to demyelination
bull The most common neuroanatomic locations are the
root entry zone of CN VIII (giving absent calorics but
normal hearing and fixation suppression) and the
medial vestibular nucleus
bull Usually treated with steroids and vestibular
suppressants
Brandt 1993 Gass 1998 Thomke 1999
Sixth Annual Intensive Update in Neurology 0915-162016
72
Vertigo in MS
Frohmanrsquos large series (1153) reported that 52 of MS patients with acute vertigo had benign paroxysmal positional vertigo while 32 had new brainstem lesions
To avoid misdiagnoses and inappropriate treatment interventions alternative etiologies should be considered in all acute vertigo episodes in MS
E M Frohman Neurology 2000 and Multiple Sclerosis 2003
Sixth Annual Intensive Update in Neurology 0915-162016
73
Vestibular compensation
bull Acute unilateral or bilateral vestibular loss will prompt compensation thru central cervico-ocular visual and proprioceptive pathways
bull Unilateral loss may require several weeks for compensation to occur and activity especially head movements hastens compensation
bull Vestibular suppressant medication prolongs compensation
Sixth Annual Intensive Update in Neurology 0915-162016
74
Vestibular Rehabilitation
bull Performed by certified physical therapists many sites (Epic order vestibular PT) or check Vestibular Disorders of America website for providers
bull Assessment and treatment of benign positional vertigo
bull Directed rehab programs for central andor peripheral vestibular compensationndash Unilateral vestibulopathies
ndash Bilateral vestibular hypofunction
ndash Mutifactorial balance deficits and fall risk assessment
Sixth Annual Intensive Update in Neurology 0915-162016
75
Summary of whatrsquos new in peripheral vestibular disorders
bull Lateral semicircular canal benign paroxysmal positional vertigo presents with horizontal positioning nystagmus
bull Oral steroids appear to improve outcome in acute vestibular neuritis
bull Intratympanic steroids offer promise in Menierersquos syndrome
bull Superior semicircular canal dehiscence causes pressure and noise induced dizziness VEMP offers diagnostic information
Sixth Annual Intensive Update in Neurology 0915-162016
76
References
BPPVndash White J Benign paroxysmal positional vertigo Cleveland Clinic Journal of
Medicine 71(9)722-728 2004
ndash White J Coale K Catalano P Oas J Diagnosis and management of lateral semicircular canal benign positional vertigo Otolaryngology-Head and Neck Surgery 133 278-284 2005
ndash White J Savvides P Cherian N Oas J Canalith repositioning for benign paroxysmal positional vertigo Otology and Neurotology 26 704-710 2005
ndash White J Oas J Diagnosis and management of lateral semicircular canal conversions during particle repositioning therapy Laryngoscope 115(10) 1895-1897 2005
ndash Acute Vestibular Syndrome Hotson JR Baloh RW N Engl J Med 1998 Sep 3339(10)680-5
ndash Methylprednisolone valacyclovir or the combination for vestibular neuritis Strupp M Zingler VC Arbusow V Niklas D Maag KP Dieterich M Bense S Theil D Jahn K Brandt T N Engl J Med 2004 Jul 22351(4)354-61
ndash Intratympanic steroids for Meacuteniegraveres disease or syndrome Phillips JS Westerberg BCochrane Database Syst Rev 2011 Jul 6(7)CD008514 Review
ndash Sound- andor pressure-induced vertigo due to bone dehiscence of the superior semicircular canal Minor LB Solomon D Zinreich JS Zee DSArch Otolaryngol Head Neck Surg 1998 Mar124(3)249-58
Sixth Annual Intensive Update in Neurology 0915-162016
16
Right posterior BPPV
Sixth Annual Intensive Update in Neurology 0915-162016
17
Canalith Repositioning Procedure
Sixth Annual Intensive Update in Neurology 0915-162016
18
Canalith Repositioning Procedure
Sixth Annual Intensive Update in Neurology 0915-162016
19
Canalith Repositioning Procedure
Sixth Annual Intensive Update in Neurology 0915-162016
20
Canalith Repositioning Procedure
Sixth Annual Intensive Update in Neurology 0915-162016
21
Canalith Repositioning
bull R posterior
BPPV
CPT 95992
Sixth Annual Intensive Update in Neurology 0915-162016
22
Posterior semicircular canalith repositioning
Sixth Annual Intensive Update in Neurology 0915-162016
23
Supine Positional Tests
Sixth Annual Intensive Update in Neurology 0915-162016
24
Lateral SCC-BPPV
bull Short latency horizontal nystagmus provoked by supine bilateral head turns with prolonged duration and poor fatigability
bull Uncommon (2-15 of total BPPV)
bull Two formsndash Geotropic ndash beating towards undermost ear
ndash Apogeotropic ndash beating away from undermost ear
Sixth Annual Intensive Update in Neurology 0915-162016
25
Geotropic Lateral Canal BPPV
bull Patient is positioned
first to the right lateral
position ndash nystagmus
beats right
bull Patient is then
positioned to the left
lateral position and
nystagmus reverses
and beats left
Sixth Annual Intensive Update in Neurology 0915-162016
26
Left Geotropic Lateral Semicircular Canal BPPV
Sixth Annual Intensive Update in Neurology 0915-162016
27
Repositioning Maneuvers for Geotropic Lateral Semicircular Canal (LSC) BPPV
bull Roll maneuver (Lempert Baloh)
ndash Patient rolls in 90 degree increments towards the good side
completely rolling over R LSC-BPPV repositioning shown
Sixth Annual Intensive Update in Neurology 0915-162016
28
Repositioning Maneuvers for Geotropic LSC BPPV
bull Gufoni maneuver
ndash Patient lies quickly onto good side and turns face-down to
floor for two minutes
Sixth Annual Intensive Update in Neurology 0915-162016
29
Left Apogeotropic LSC BPPV
Sixth Annual Intensive Update in Neurology 0915-162016
30
Nystagmus observed During Repositioning
bull Nystagmus should remain in the same torsional
direction as seen on DHT throughout the treatment of
posterior BPPV
bull Reversal of nystagmus direction suggests the particle
has fallen back into the canal and predicts failure
bull In Lateral BPPV nystagmus should beat towards the
good ear
Sixth Annual Intensive Update in Neurology 0915-162016
31
Posterior to Lateral canal conversion
Sixth Annual Intensive Update in Neurology 0915-162016
32
R Posterior to Lateral Canal conversion - geotropic
Sixth Annual Intensive Update in Neurology 0915-162016
33
BPPV Recurrence
bull Recurrence is common (15yr)
bull Otoconial adherence or mineralization demineralization
abnormalities may contribute to recurrence
bull Home canalith repositioning is effective (Radke 2004)
Sixth Annual Intensive Update in Neurology 0915-162016
34
Question 1
bull The most likely semicircular canal to be affected in
BPPV is
ndash 1) superior
ndash 2) lateral
ndash 3) posterior
ndash 4) combined
Sixth Annual Intensive Update in Neurology 0915-162016
35
Question 1
bull The most likely semicircular canal to be affected in
BPPV is
ndash 1) superior
ndash 2) lateral
ndash 3) posterior
ndash 4) combined
Answer 3 See references at end of lecture
Sixth Annual Intensive Update in Neurology 0915-162016
36
Acute Vestibular Syndrome
bull Rapid unilateral injury to either peripheral or central
vestibular structures produces prolonged vertigo
(days to weeks)
bull Severe vertigo nausea and vomiting spontaneous
nystagmus and postural instability
Sixth Annual Intensive Update in Neurology 0915-162016
37
Acute Vestibular Syndrome
bull 25 of patients with risk factors who present with isolated severe vertigo nystagmus and postural instability in the ER have an acute infarction of the inferior cerebellum (AICA PICA) Central imaging is suggested (Baloh 1998 NEJM)
ndash HTN diabetes smoking age gt65 Afib valvular heart disease occlusive vascular disease
Sixth Annual Intensive Update in Neurology 0915-162016
38
HINTS test
bull Head Impulse
bull Nystagmus
bull Test of Skew
ndash More sensitive than MRI in the diagnosis of acute stroke in
patients with acute vestibular syndrome with at least one risk
factor (Kattah J et al Stroke 2009)
Sixth Annual Intensive Update in Neurology 0915-162016
39
Head Impulse Test
bull No special equipment needed
bull Examiner stands in front of seated patient and
randomly moves head left and right Patient is asked to
focus on examinerrsquos nose
bull Rapid low amplitude thrust from lateral to straight
ahead causes a compensatory saccade when
performed towards the weak side
bull This finding is a permanent feature of unilateral
vestibular loss
Sixth Annual Intensive Update in Neurology 0915-162016
40
Head Impulse Test
Sixth Annual Intensive Update in Neurology 0915-162016
41
Head CT ndash Academy of Emergency Medicine 2013
bull The clinical value of head CT in isolated
dizzinessvertigo without any associated neurological
symptoms is very low
bull Less than 1 of head CTs ordered for dizziness and
vertigo have significant abnormalities and those
patients had severe headache and neurological deficits
in addition to dizziness
bull Forgoing unnecessary emergency head CTs could
save 360 million dollars annually
Sixth Annual Intensive Update in Neurology 0915-162016
42
Question 2
Head CT is not indicated for the evaluation of dizziness
and vertigo without additional neurological complaints because
1 It is too expensive
2 Less than 1 of CTs done for dizziness and vertigo are
abnormal and those patients had associated severe
neurological complaints
3 CT is the best imaging choice for the posterior fossa
4 Acoustic neuromas are well seen on CT
Sixth Annual Intensive Update in Neurology 0915-162016
43
Question 2
Head CT is not indicated for the evaluation of dizziness
and vertigo without additional neurological complaints because
1 It is too expensive
2 Less than 1 of CTs done for dizziness and vertigo are
abnormal and those patients had associated severe
neurological complaints
3 CT is the best imaging choice for the posterior fossa
4 Acoustic neuromas are well seen on CT
Answer 2 See references at end of lecture
Sixth Annual Intensive Update in Neurology 0915-162016
44
Acute Vestibular Syndrome
bull Usually peripheral and can be associated with
hearing loss (labyrinthitis)
bull Timely diagnosis improves recovery (ASAP
prednisone 1mgkgday for ten days and taper 10
mgday ndash Strupp M 2004 NEJM)
bull Avoid vestibular suppressants after 3 days and begin
vestibular rehabilitation to hasten compensation
Sixth Annual Intensive Update in Neurology 0915-162016
45
Question 3
bull Treatment for Acute Vestibular Syndrome
1) oral prednisone 1 mgkgday
2) Treat dehydration if necessary
3) Minimize vestibular suppressants after 3 days
4) Consider vestibular physical therapy
5) All of the above
Sixth Annual Intensive Update in Neurology 0915-162016
46
Question 3
bull Treatment for Acute Vestibular Syndrome
1) oral prednisone 1 mgkgday
2) Treat dehydration if necessary
3) Minimize vestibular suppressants after 3 days
4) Consider vestibular physical therapy
5) All of the above
Answer 5 See references at end of lecture
Sixth Annual Intensive Update in Neurology 0915-162016
47
Menierersquos syndrome
bull Fluctuating low frequency hearing loss
bull Tinnitus andor aural fullness
bull Vertigo lasting hours
Sixth Annual Intensive Update in Neurology 0915-162016
48
Normal
Sixth Annual Intensive Update in Neurology 0915-162016
49
Hydrops
Sixth Annual Intensive Update in Neurology 0915-162016
50
Menierersquos syndrome
ndash Diagnosis- Exclusion (RO acoustic)
ndash 23 of Menierersquos patients will improve spontaneously
ndash Treatment-1500 mgday low sodium triamterene375-
hydrochlorothiazide 25 vestibular suppressants (lorazepam
1 mg tablet sublingual Q 8 prn attacks) off label use
Sixth Annual Intensive Update in Neurology 0915-162016
51
Menierersquos syndrome ndash intratympanic steroids
ndash A newer treatment option for those failing medical management
ndash 90 response rate (Boleus-Aguirre MS Otology Neurotology
2008)
ndash Outcomes data confirm excellent clinical response in 3941
Menierersquos patients treated with intratympanic steroids in 2008
Sixth Annual Intensive Update in Neurology 0915-162016
52
Question 4
bull Menierersquos syndrome diagnosis is based on
1) Recurrent vertigo lasting at least 20 minutes
2) Aural fullness
3) Documented hearing loss
4) Other causes excluded
5) All of the above
Sixth Annual Intensive Update in Neurology 0915-162016
53
Question 4
bull Menierersquos syndrome diagnosis is based on
1) Recurrent vertigo lasting at least 20 minutes
2) Aural fullness
3) Documented hearing loss
4) Other causes excluded
5) All of the above
Answer 5 See references at end
Sixth Annual Intensive Update in Neurology 0915-162016
54
Superior Semicircular Canal Dehiscence (SSCD)
bull Sound andor pressure induced oscillopsia
bull CT reformatted in the plane of the superior canals
shows dehiscence of overlying bone
bull Low frequency ldquoconductive hyperacusisrdquo with elevated
bone thresholds normal reflexes and a conductive loss
Sixth Annual Intensive Update in Neurology 0915-162016
55
SSCD
bull Positive middle ear pressure or loud sound stimulates ampullofugal endolymph flow -canal activation
bull Eye movements are tonic upwards with ipsilateral intorsion
Sixth Annual Intensive Update in Neurology 0915-162016
56
SSCD
bull Valsalva against a closed glottis or jugular compression causes increased intracranial pressure and ampullopetal endolymph flow -canal inhibition
bull Eye movements are tonic downwards with ipsilateral extorsion
Sixth Annual Intensive Update in Neurology 0915-162016
57
Reconstructed left superior semicircular canal
Sixth Annual Intensive Update in Neurology 0915-162016
58
Sixth Annual Intensive Update in Neurology 0915-162016
59
SCHEMATIC DRAWING OF AN OTOLITHIC MACULA
Sixth Annual Intensive Update in Neurology 0915-162016
60
MACULAE OF THE UTRICLESACCULE
Sixth Annual Intensive Update in Neurology 0915-162016
61
HAIR CELLS
Sixth Annual Intensive Update in Neurology 0915-162016
62
SCANNING ELECTRON MICROGRAPH OF OTOCONIA
Sixth Annual Intensive Update in Neurology 0915-162016
63
Ocular VEMP
bull Response to loud clicks (80-110 dB) or forehead tap (Halmagyi and Curthoys 2003)
bull Contralateral oculomotor nucleus (CN III)
bull Inferior oblique muscle contraction (8 msec) detected by 5sec event triggered averaging
bull Noninverting electrode inferior to eye
bull Inverting below non-inverting electrode
bull Ground to forehead
bull Normal subjects have very low response
Sixth Annual Intensive Update in Neurology 0915-162016
64
Clinical applications
bull The o-VEMP is a sensitive test of otolithutricular
function
bull Complements vestibular test procedures that assess
semicircular canals
bull Abnormally robust responses are associated with third
window abnormalities such as semicircular canal
dehiscence
bull Serves as an excellent screening test for semicircular
canal dehiscence
Sixth Annual Intensive Update in Neurology 0915-162016
65
Question 5
bull Superior semicircular canal dehiscence
1) Causes increased ability to hear vibratory sounds
2) Improves hearing
3) Causes meningitis
4) Always needs surgical repair
Sixth Annual Intensive Update in Neurology 0915-162016
66
Question 5
bull Superior semicircular canal dehiscence
1) Causes increased ability to hear vibratory sounds
2) Improves hearing
3) Causes meningitis
4) Always needs surgical repair
Answer 1 References at end of lecture
Sixth Annual Intensive Update in Neurology 0915-162016
67
Enlarged vestibular aqueduct
bull Axial temporal bone CT showing right temporal bone with enlarged vestibular aqueduct (arrow)
bull Vestibular symptoms accompany progressive sensorineural hearing loss in 4-50 of patients in selected series
Sixth Annual Intensive Update in Neurology 0915-162016
68
Migraine
bull Family or personal history of migraine
bull 25 of migraine patients experience spontaneous vertigo (Baloh 1997)
bull Neuhauser criteria(2001) have been used to construct structured diagnostic interviews (Furman 2003)
bull Not always associated with headache
bull Motion sensitivity
bull Medication responsive
Sixth Annual Intensive Update in Neurology 0915-162016
69
ICHD-III beta Criteria for Vestibular Migraine
bull A At least five attacks fulfilling criteria C and D
bull B A current or past history of 11 migraine without aura or 12 migraine with aura
bull C Vestibular symptoms of moderate or severe intensity lasting between 5 minutes and 72 hours
bull D At least 50 associated with one of the following migrainous features
ndash 1 Headaches
ndash 2 Photophobia and phonophobia
ndash 3 Visual Aura
bull E Not better accounted for by another vestibular disorder
Sixth Annual Intensive Update in Neurology 0915-162016
70
Incidence of vertigo in MS
bull True vertigo (the perception of environmental
movement typically rotational) occurs in about
20 of MS patients and is the presenting
manifestation in 5
bull Up to 78 of MS patients report abnormalities
in balance at some point in their illness
Frohman et al Multiple Sclerosis 2003 9250
Sixth Annual Intensive Update in Neurology 0915-162016
71
Vertigo in MS due to demyelination
bull The most common neuroanatomic locations are the
root entry zone of CN VIII (giving absent calorics but
normal hearing and fixation suppression) and the
medial vestibular nucleus
bull Usually treated with steroids and vestibular
suppressants
Brandt 1993 Gass 1998 Thomke 1999
Sixth Annual Intensive Update in Neurology 0915-162016
72
Vertigo in MS
Frohmanrsquos large series (1153) reported that 52 of MS patients with acute vertigo had benign paroxysmal positional vertigo while 32 had new brainstem lesions
To avoid misdiagnoses and inappropriate treatment interventions alternative etiologies should be considered in all acute vertigo episodes in MS
E M Frohman Neurology 2000 and Multiple Sclerosis 2003
Sixth Annual Intensive Update in Neurology 0915-162016
73
Vestibular compensation
bull Acute unilateral or bilateral vestibular loss will prompt compensation thru central cervico-ocular visual and proprioceptive pathways
bull Unilateral loss may require several weeks for compensation to occur and activity especially head movements hastens compensation
bull Vestibular suppressant medication prolongs compensation
Sixth Annual Intensive Update in Neurology 0915-162016
74
Vestibular Rehabilitation
bull Performed by certified physical therapists many sites (Epic order vestibular PT) or check Vestibular Disorders of America website for providers
bull Assessment and treatment of benign positional vertigo
bull Directed rehab programs for central andor peripheral vestibular compensationndash Unilateral vestibulopathies
ndash Bilateral vestibular hypofunction
ndash Mutifactorial balance deficits and fall risk assessment
Sixth Annual Intensive Update in Neurology 0915-162016
75
Summary of whatrsquos new in peripheral vestibular disorders
bull Lateral semicircular canal benign paroxysmal positional vertigo presents with horizontal positioning nystagmus
bull Oral steroids appear to improve outcome in acute vestibular neuritis
bull Intratympanic steroids offer promise in Menierersquos syndrome
bull Superior semicircular canal dehiscence causes pressure and noise induced dizziness VEMP offers diagnostic information
Sixth Annual Intensive Update in Neurology 0915-162016
76
References
BPPVndash White J Benign paroxysmal positional vertigo Cleveland Clinic Journal of
Medicine 71(9)722-728 2004
ndash White J Coale K Catalano P Oas J Diagnosis and management of lateral semicircular canal benign positional vertigo Otolaryngology-Head and Neck Surgery 133 278-284 2005
ndash White J Savvides P Cherian N Oas J Canalith repositioning for benign paroxysmal positional vertigo Otology and Neurotology 26 704-710 2005
ndash White J Oas J Diagnosis and management of lateral semicircular canal conversions during particle repositioning therapy Laryngoscope 115(10) 1895-1897 2005
ndash Acute Vestibular Syndrome Hotson JR Baloh RW N Engl J Med 1998 Sep 3339(10)680-5
ndash Methylprednisolone valacyclovir or the combination for vestibular neuritis Strupp M Zingler VC Arbusow V Niklas D Maag KP Dieterich M Bense S Theil D Jahn K Brandt T N Engl J Med 2004 Jul 22351(4)354-61
ndash Intratympanic steroids for Meacuteniegraveres disease or syndrome Phillips JS Westerberg BCochrane Database Syst Rev 2011 Jul 6(7)CD008514 Review
ndash Sound- andor pressure-induced vertigo due to bone dehiscence of the superior semicircular canal Minor LB Solomon D Zinreich JS Zee DSArch Otolaryngol Head Neck Surg 1998 Mar124(3)249-58
Sixth Annual Intensive Update in Neurology 0915-162016
17
Canalith Repositioning Procedure
Sixth Annual Intensive Update in Neurology 0915-162016
18
Canalith Repositioning Procedure
Sixth Annual Intensive Update in Neurology 0915-162016
19
Canalith Repositioning Procedure
Sixth Annual Intensive Update in Neurology 0915-162016
20
Canalith Repositioning Procedure
Sixth Annual Intensive Update in Neurology 0915-162016
21
Canalith Repositioning
bull R posterior
BPPV
CPT 95992
Sixth Annual Intensive Update in Neurology 0915-162016
22
Posterior semicircular canalith repositioning
Sixth Annual Intensive Update in Neurology 0915-162016
23
Supine Positional Tests
Sixth Annual Intensive Update in Neurology 0915-162016
24
Lateral SCC-BPPV
bull Short latency horizontal nystagmus provoked by supine bilateral head turns with prolonged duration and poor fatigability
bull Uncommon (2-15 of total BPPV)
bull Two formsndash Geotropic ndash beating towards undermost ear
ndash Apogeotropic ndash beating away from undermost ear
Sixth Annual Intensive Update in Neurology 0915-162016
25
Geotropic Lateral Canal BPPV
bull Patient is positioned
first to the right lateral
position ndash nystagmus
beats right
bull Patient is then
positioned to the left
lateral position and
nystagmus reverses
and beats left
Sixth Annual Intensive Update in Neurology 0915-162016
26
Left Geotropic Lateral Semicircular Canal BPPV
Sixth Annual Intensive Update in Neurology 0915-162016
27
Repositioning Maneuvers for Geotropic Lateral Semicircular Canal (LSC) BPPV
bull Roll maneuver (Lempert Baloh)
ndash Patient rolls in 90 degree increments towards the good side
completely rolling over R LSC-BPPV repositioning shown
Sixth Annual Intensive Update in Neurology 0915-162016
28
Repositioning Maneuvers for Geotropic LSC BPPV
bull Gufoni maneuver
ndash Patient lies quickly onto good side and turns face-down to
floor for two minutes
Sixth Annual Intensive Update in Neurology 0915-162016
29
Left Apogeotropic LSC BPPV
Sixth Annual Intensive Update in Neurology 0915-162016
30
Nystagmus observed During Repositioning
bull Nystagmus should remain in the same torsional
direction as seen on DHT throughout the treatment of
posterior BPPV
bull Reversal of nystagmus direction suggests the particle
has fallen back into the canal and predicts failure
bull In Lateral BPPV nystagmus should beat towards the
good ear
Sixth Annual Intensive Update in Neurology 0915-162016
31
Posterior to Lateral canal conversion
Sixth Annual Intensive Update in Neurology 0915-162016
32
R Posterior to Lateral Canal conversion - geotropic
Sixth Annual Intensive Update in Neurology 0915-162016
33
BPPV Recurrence
bull Recurrence is common (15yr)
bull Otoconial adherence or mineralization demineralization
abnormalities may contribute to recurrence
bull Home canalith repositioning is effective (Radke 2004)
Sixth Annual Intensive Update in Neurology 0915-162016
34
Question 1
bull The most likely semicircular canal to be affected in
BPPV is
ndash 1) superior
ndash 2) lateral
ndash 3) posterior
ndash 4) combined
Sixth Annual Intensive Update in Neurology 0915-162016
35
Question 1
bull The most likely semicircular canal to be affected in
BPPV is
ndash 1) superior
ndash 2) lateral
ndash 3) posterior
ndash 4) combined
Answer 3 See references at end of lecture
Sixth Annual Intensive Update in Neurology 0915-162016
36
Acute Vestibular Syndrome
bull Rapid unilateral injury to either peripheral or central
vestibular structures produces prolonged vertigo
(days to weeks)
bull Severe vertigo nausea and vomiting spontaneous
nystagmus and postural instability
Sixth Annual Intensive Update in Neurology 0915-162016
37
Acute Vestibular Syndrome
bull 25 of patients with risk factors who present with isolated severe vertigo nystagmus and postural instability in the ER have an acute infarction of the inferior cerebellum (AICA PICA) Central imaging is suggested (Baloh 1998 NEJM)
ndash HTN diabetes smoking age gt65 Afib valvular heart disease occlusive vascular disease
Sixth Annual Intensive Update in Neurology 0915-162016
38
HINTS test
bull Head Impulse
bull Nystagmus
bull Test of Skew
ndash More sensitive than MRI in the diagnosis of acute stroke in
patients with acute vestibular syndrome with at least one risk
factor (Kattah J et al Stroke 2009)
Sixth Annual Intensive Update in Neurology 0915-162016
39
Head Impulse Test
bull No special equipment needed
bull Examiner stands in front of seated patient and
randomly moves head left and right Patient is asked to
focus on examinerrsquos nose
bull Rapid low amplitude thrust from lateral to straight
ahead causes a compensatory saccade when
performed towards the weak side
bull This finding is a permanent feature of unilateral
vestibular loss
Sixth Annual Intensive Update in Neurology 0915-162016
40
Head Impulse Test
Sixth Annual Intensive Update in Neurology 0915-162016
41
Head CT ndash Academy of Emergency Medicine 2013
bull The clinical value of head CT in isolated
dizzinessvertigo without any associated neurological
symptoms is very low
bull Less than 1 of head CTs ordered for dizziness and
vertigo have significant abnormalities and those
patients had severe headache and neurological deficits
in addition to dizziness
bull Forgoing unnecessary emergency head CTs could
save 360 million dollars annually
Sixth Annual Intensive Update in Neurology 0915-162016
42
Question 2
Head CT is not indicated for the evaluation of dizziness
and vertigo without additional neurological complaints because
1 It is too expensive
2 Less than 1 of CTs done for dizziness and vertigo are
abnormal and those patients had associated severe
neurological complaints
3 CT is the best imaging choice for the posterior fossa
4 Acoustic neuromas are well seen on CT
Sixth Annual Intensive Update in Neurology 0915-162016
43
Question 2
Head CT is not indicated for the evaluation of dizziness
and vertigo without additional neurological complaints because
1 It is too expensive
2 Less than 1 of CTs done for dizziness and vertigo are
abnormal and those patients had associated severe
neurological complaints
3 CT is the best imaging choice for the posterior fossa
4 Acoustic neuromas are well seen on CT
Answer 2 See references at end of lecture
Sixth Annual Intensive Update in Neurology 0915-162016
44
Acute Vestibular Syndrome
bull Usually peripheral and can be associated with
hearing loss (labyrinthitis)
bull Timely diagnosis improves recovery (ASAP
prednisone 1mgkgday for ten days and taper 10
mgday ndash Strupp M 2004 NEJM)
bull Avoid vestibular suppressants after 3 days and begin
vestibular rehabilitation to hasten compensation
Sixth Annual Intensive Update in Neurology 0915-162016
45
Question 3
bull Treatment for Acute Vestibular Syndrome
1) oral prednisone 1 mgkgday
2) Treat dehydration if necessary
3) Minimize vestibular suppressants after 3 days
4) Consider vestibular physical therapy
5) All of the above
Sixth Annual Intensive Update in Neurology 0915-162016
46
Question 3
bull Treatment for Acute Vestibular Syndrome
1) oral prednisone 1 mgkgday
2) Treat dehydration if necessary
3) Minimize vestibular suppressants after 3 days
4) Consider vestibular physical therapy
5) All of the above
Answer 5 See references at end of lecture
Sixth Annual Intensive Update in Neurology 0915-162016
47
Menierersquos syndrome
bull Fluctuating low frequency hearing loss
bull Tinnitus andor aural fullness
bull Vertigo lasting hours
Sixth Annual Intensive Update in Neurology 0915-162016
48
Normal
Sixth Annual Intensive Update in Neurology 0915-162016
49
Hydrops
Sixth Annual Intensive Update in Neurology 0915-162016
50
Menierersquos syndrome
ndash Diagnosis- Exclusion (RO acoustic)
ndash 23 of Menierersquos patients will improve spontaneously
ndash Treatment-1500 mgday low sodium triamterene375-
hydrochlorothiazide 25 vestibular suppressants (lorazepam
1 mg tablet sublingual Q 8 prn attacks) off label use
Sixth Annual Intensive Update in Neurology 0915-162016
51
Menierersquos syndrome ndash intratympanic steroids
ndash A newer treatment option for those failing medical management
ndash 90 response rate (Boleus-Aguirre MS Otology Neurotology
2008)
ndash Outcomes data confirm excellent clinical response in 3941
Menierersquos patients treated with intratympanic steroids in 2008
Sixth Annual Intensive Update in Neurology 0915-162016
52
Question 4
bull Menierersquos syndrome diagnosis is based on
1) Recurrent vertigo lasting at least 20 minutes
2) Aural fullness
3) Documented hearing loss
4) Other causes excluded
5) All of the above
Sixth Annual Intensive Update in Neurology 0915-162016
53
Question 4
bull Menierersquos syndrome diagnosis is based on
1) Recurrent vertigo lasting at least 20 minutes
2) Aural fullness
3) Documented hearing loss
4) Other causes excluded
5) All of the above
Answer 5 See references at end
Sixth Annual Intensive Update in Neurology 0915-162016
54
Superior Semicircular Canal Dehiscence (SSCD)
bull Sound andor pressure induced oscillopsia
bull CT reformatted in the plane of the superior canals
shows dehiscence of overlying bone
bull Low frequency ldquoconductive hyperacusisrdquo with elevated
bone thresholds normal reflexes and a conductive loss
Sixth Annual Intensive Update in Neurology 0915-162016
55
SSCD
bull Positive middle ear pressure or loud sound stimulates ampullofugal endolymph flow -canal activation
bull Eye movements are tonic upwards with ipsilateral intorsion
Sixth Annual Intensive Update in Neurology 0915-162016
56
SSCD
bull Valsalva against a closed glottis or jugular compression causes increased intracranial pressure and ampullopetal endolymph flow -canal inhibition
bull Eye movements are tonic downwards with ipsilateral extorsion
Sixth Annual Intensive Update in Neurology 0915-162016
57
Reconstructed left superior semicircular canal
Sixth Annual Intensive Update in Neurology 0915-162016
58
Sixth Annual Intensive Update in Neurology 0915-162016
59
SCHEMATIC DRAWING OF AN OTOLITHIC MACULA
Sixth Annual Intensive Update in Neurology 0915-162016
60
MACULAE OF THE UTRICLESACCULE
Sixth Annual Intensive Update in Neurology 0915-162016
61
HAIR CELLS
Sixth Annual Intensive Update in Neurology 0915-162016
62
SCANNING ELECTRON MICROGRAPH OF OTOCONIA
Sixth Annual Intensive Update in Neurology 0915-162016
63
Ocular VEMP
bull Response to loud clicks (80-110 dB) or forehead tap (Halmagyi and Curthoys 2003)
bull Contralateral oculomotor nucleus (CN III)
bull Inferior oblique muscle contraction (8 msec) detected by 5sec event triggered averaging
bull Noninverting electrode inferior to eye
bull Inverting below non-inverting electrode
bull Ground to forehead
bull Normal subjects have very low response
Sixth Annual Intensive Update in Neurology 0915-162016
64
Clinical applications
bull The o-VEMP is a sensitive test of otolithutricular
function
bull Complements vestibular test procedures that assess
semicircular canals
bull Abnormally robust responses are associated with third
window abnormalities such as semicircular canal
dehiscence
bull Serves as an excellent screening test for semicircular
canal dehiscence
Sixth Annual Intensive Update in Neurology 0915-162016
65
Question 5
bull Superior semicircular canal dehiscence
1) Causes increased ability to hear vibratory sounds
2) Improves hearing
3) Causes meningitis
4) Always needs surgical repair
Sixth Annual Intensive Update in Neurology 0915-162016
66
Question 5
bull Superior semicircular canal dehiscence
1) Causes increased ability to hear vibratory sounds
2) Improves hearing
3) Causes meningitis
4) Always needs surgical repair
Answer 1 References at end of lecture
Sixth Annual Intensive Update in Neurology 0915-162016
67
Enlarged vestibular aqueduct
bull Axial temporal bone CT showing right temporal bone with enlarged vestibular aqueduct (arrow)
bull Vestibular symptoms accompany progressive sensorineural hearing loss in 4-50 of patients in selected series
Sixth Annual Intensive Update in Neurology 0915-162016
68
Migraine
bull Family or personal history of migraine
bull 25 of migraine patients experience spontaneous vertigo (Baloh 1997)
bull Neuhauser criteria(2001) have been used to construct structured diagnostic interviews (Furman 2003)
bull Not always associated with headache
bull Motion sensitivity
bull Medication responsive
Sixth Annual Intensive Update in Neurology 0915-162016
69
ICHD-III beta Criteria for Vestibular Migraine
bull A At least five attacks fulfilling criteria C and D
bull B A current or past history of 11 migraine without aura or 12 migraine with aura
bull C Vestibular symptoms of moderate or severe intensity lasting between 5 minutes and 72 hours
bull D At least 50 associated with one of the following migrainous features
ndash 1 Headaches
ndash 2 Photophobia and phonophobia
ndash 3 Visual Aura
bull E Not better accounted for by another vestibular disorder
Sixth Annual Intensive Update in Neurology 0915-162016
70
Incidence of vertigo in MS
bull True vertigo (the perception of environmental
movement typically rotational) occurs in about
20 of MS patients and is the presenting
manifestation in 5
bull Up to 78 of MS patients report abnormalities
in balance at some point in their illness
Frohman et al Multiple Sclerosis 2003 9250
Sixth Annual Intensive Update in Neurology 0915-162016
71
Vertigo in MS due to demyelination
bull The most common neuroanatomic locations are the
root entry zone of CN VIII (giving absent calorics but
normal hearing and fixation suppression) and the
medial vestibular nucleus
bull Usually treated with steroids and vestibular
suppressants
Brandt 1993 Gass 1998 Thomke 1999
Sixth Annual Intensive Update in Neurology 0915-162016
72
Vertigo in MS
Frohmanrsquos large series (1153) reported that 52 of MS patients with acute vertigo had benign paroxysmal positional vertigo while 32 had new brainstem lesions
To avoid misdiagnoses and inappropriate treatment interventions alternative etiologies should be considered in all acute vertigo episodes in MS
E M Frohman Neurology 2000 and Multiple Sclerosis 2003
Sixth Annual Intensive Update in Neurology 0915-162016
73
Vestibular compensation
bull Acute unilateral or bilateral vestibular loss will prompt compensation thru central cervico-ocular visual and proprioceptive pathways
bull Unilateral loss may require several weeks for compensation to occur and activity especially head movements hastens compensation
bull Vestibular suppressant medication prolongs compensation
Sixth Annual Intensive Update in Neurology 0915-162016
74
Vestibular Rehabilitation
bull Performed by certified physical therapists many sites (Epic order vestibular PT) or check Vestibular Disorders of America website for providers
bull Assessment and treatment of benign positional vertigo
bull Directed rehab programs for central andor peripheral vestibular compensationndash Unilateral vestibulopathies
ndash Bilateral vestibular hypofunction
ndash Mutifactorial balance deficits and fall risk assessment
Sixth Annual Intensive Update in Neurology 0915-162016
75
Summary of whatrsquos new in peripheral vestibular disorders
bull Lateral semicircular canal benign paroxysmal positional vertigo presents with horizontal positioning nystagmus
bull Oral steroids appear to improve outcome in acute vestibular neuritis
bull Intratympanic steroids offer promise in Menierersquos syndrome
bull Superior semicircular canal dehiscence causes pressure and noise induced dizziness VEMP offers diagnostic information
Sixth Annual Intensive Update in Neurology 0915-162016
76
References
BPPVndash White J Benign paroxysmal positional vertigo Cleveland Clinic Journal of
Medicine 71(9)722-728 2004
ndash White J Coale K Catalano P Oas J Diagnosis and management of lateral semicircular canal benign positional vertigo Otolaryngology-Head and Neck Surgery 133 278-284 2005
ndash White J Savvides P Cherian N Oas J Canalith repositioning for benign paroxysmal positional vertigo Otology and Neurotology 26 704-710 2005
ndash White J Oas J Diagnosis and management of lateral semicircular canal conversions during particle repositioning therapy Laryngoscope 115(10) 1895-1897 2005
ndash Acute Vestibular Syndrome Hotson JR Baloh RW N Engl J Med 1998 Sep 3339(10)680-5
ndash Methylprednisolone valacyclovir or the combination for vestibular neuritis Strupp M Zingler VC Arbusow V Niklas D Maag KP Dieterich M Bense S Theil D Jahn K Brandt T N Engl J Med 2004 Jul 22351(4)354-61
ndash Intratympanic steroids for Meacuteniegraveres disease or syndrome Phillips JS Westerberg BCochrane Database Syst Rev 2011 Jul 6(7)CD008514 Review
ndash Sound- andor pressure-induced vertigo due to bone dehiscence of the superior semicircular canal Minor LB Solomon D Zinreich JS Zee DSArch Otolaryngol Head Neck Surg 1998 Mar124(3)249-58
Sixth Annual Intensive Update in Neurology 0915-162016
18
Canalith Repositioning Procedure
Sixth Annual Intensive Update in Neurology 0915-162016
19
Canalith Repositioning Procedure
Sixth Annual Intensive Update in Neurology 0915-162016
20
Canalith Repositioning Procedure
Sixth Annual Intensive Update in Neurology 0915-162016
21
Canalith Repositioning
bull R posterior
BPPV
CPT 95992
Sixth Annual Intensive Update in Neurology 0915-162016
22
Posterior semicircular canalith repositioning
Sixth Annual Intensive Update in Neurology 0915-162016
23
Supine Positional Tests
Sixth Annual Intensive Update in Neurology 0915-162016
24
Lateral SCC-BPPV
bull Short latency horizontal nystagmus provoked by supine bilateral head turns with prolonged duration and poor fatigability
bull Uncommon (2-15 of total BPPV)
bull Two formsndash Geotropic ndash beating towards undermost ear
ndash Apogeotropic ndash beating away from undermost ear
Sixth Annual Intensive Update in Neurology 0915-162016
25
Geotropic Lateral Canal BPPV
bull Patient is positioned
first to the right lateral
position ndash nystagmus
beats right
bull Patient is then
positioned to the left
lateral position and
nystagmus reverses
and beats left
Sixth Annual Intensive Update in Neurology 0915-162016
26
Left Geotropic Lateral Semicircular Canal BPPV
Sixth Annual Intensive Update in Neurology 0915-162016
27
Repositioning Maneuvers for Geotropic Lateral Semicircular Canal (LSC) BPPV
bull Roll maneuver (Lempert Baloh)
ndash Patient rolls in 90 degree increments towards the good side
completely rolling over R LSC-BPPV repositioning shown
Sixth Annual Intensive Update in Neurology 0915-162016
28
Repositioning Maneuvers for Geotropic LSC BPPV
bull Gufoni maneuver
ndash Patient lies quickly onto good side and turns face-down to
floor for two minutes
Sixth Annual Intensive Update in Neurology 0915-162016
29
Left Apogeotropic LSC BPPV
Sixth Annual Intensive Update in Neurology 0915-162016
30
Nystagmus observed During Repositioning
bull Nystagmus should remain in the same torsional
direction as seen on DHT throughout the treatment of
posterior BPPV
bull Reversal of nystagmus direction suggests the particle
has fallen back into the canal and predicts failure
bull In Lateral BPPV nystagmus should beat towards the
good ear
Sixth Annual Intensive Update in Neurology 0915-162016
31
Posterior to Lateral canal conversion
Sixth Annual Intensive Update in Neurology 0915-162016
32
R Posterior to Lateral Canal conversion - geotropic
Sixth Annual Intensive Update in Neurology 0915-162016
33
BPPV Recurrence
bull Recurrence is common (15yr)
bull Otoconial adherence or mineralization demineralization
abnormalities may contribute to recurrence
bull Home canalith repositioning is effective (Radke 2004)
Sixth Annual Intensive Update in Neurology 0915-162016
34
Question 1
bull The most likely semicircular canal to be affected in
BPPV is
ndash 1) superior
ndash 2) lateral
ndash 3) posterior
ndash 4) combined
Sixth Annual Intensive Update in Neurology 0915-162016
35
Question 1
bull The most likely semicircular canal to be affected in
BPPV is
ndash 1) superior
ndash 2) lateral
ndash 3) posterior
ndash 4) combined
Answer 3 See references at end of lecture
Sixth Annual Intensive Update in Neurology 0915-162016
36
Acute Vestibular Syndrome
bull Rapid unilateral injury to either peripheral or central
vestibular structures produces prolonged vertigo
(days to weeks)
bull Severe vertigo nausea and vomiting spontaneous
nystagmus and postural instability
Sixth Annual Intensive Update in Neurology 0915-162016
37
Acute Vestibular Syndrome
bull 25 of patients with risk factors who present with isolated severe vertigo nystagmus and postural instability in the ER have an acute infarction of the inferior cerebellum (AICA PICA) Central imaging is suggested (Baloh 1998 NEJM)
ndash HTN diabetes smoking age gt65 Afib valvular heart disease occlusive vascular disease
Sixth Annual Intensive Update in Neurology 0915-162016
38
HINTS test
bull Head Impulse
bull Nystagmus
bull Test of Skew
ndash More sensitive than MRI in the diagnosis of acute stroke in
patients with acute vestibular syndrome with at least one risk
factor (Kattah J et al Stroke 2009)
Sixth Annual Intensive Update in Neurology 0915-162016
39
Head Impulse Test
bull No special equipment needed
bull Examiner stands in front of seated patient and
randomly moves head left and right Patient is asked to
focus on examinerrsquos nose
bull Rapid low amplitude thrust from lateral to straight
ahead causes a compensatory saccade when
performed towards the weak side
bull This finding is a permanent feature of unilateral
vestibular loss
Sixth Annual Intensive Update in Neurology 0915-162016
40
Head Impulse Test
Sixth Annual Intensive Update in Neurology 0915-162016
41
Head CT ndash Academy of Emergency Medicine 2013
bull The clinical value of head CT in isolated
dizzinessvertigo without any associated neurological
symptoms is very low
bull Less than 1 of head CTs ordered for dizziness and
vertigo have significant abnormalities and those
patients had severe headache and neurological deficits
in addition to dizziness
bull Forgoing unnecessary emergency head CTs could
save 360 million dollars annually
Sixth Annual Intensive Update in Neurology 0915-162016
42
Question 2
Head CT is not indicated for the evaluation of dizziness
and vertigo without additional neurological complaints because
1 It is too expensive
2 Less than 1 of CTs done for dizziness and vertigo are
abnormal and those patients had associated severe
neurological complaints
3 CT is the best imaging choice for the posterior fossa
4 Acoustic neuromas are well seen on CT
Sixth Annual Intensive Update in Neurology 0915-162016
43
Question 2
Head CT is not indicated for the evaluation of dizziness
and vertigo without additional neurological complaints because
1 It is too expensive
2 Less than 1 of CTs done for dizziness and vertigo are
abnormal and those patients had associated severe
neurological complaints
3 CT is the best imaging choice for the posterior fossa
4 Acoustic neuromas are well seen on CT
Answer 2 See references at end of lecture
Sixth Annual Intensive Update in Neurology 0915-162016
44
Acute Vestibular Syndrome
bull Usually peripheral and can be associated with
hearing loss (labyrinthitis)
bull Timely diagnosis improves recovery (ASAP
prednisone 1mgkgday for ten days and taper 10
mgday ndash Strupp M 2004 NEJM)
bull Avoid vestibular suppressants after 3 days and begin
vestibular rehabilitation to hasten compensation
Sixth Annual Intensive Update in Neurology 0915-162016
45
Question 3
bull Treatment for Acute Vestibular Syndrome
1) oral prednisone 1 mgkgday
2) Treat dehydration if necessary
3) Minimize vestibular suppressants after 3 days
4) Consider vestibular physical therapy
5) All of the above
Sixth Annual Intensive Update in Neurology 0915-162016
46
Question 3
bull Treatment for Acute Vestibular Syndrome
1) oral prednisone 1 mgkgday
2) Treat dehydration if necessary
3) Minimize vestibular suppressants after 3 days
4) Consider vestibular physical therapy
5) All of the above
Answer 5 See references at end of lecture
Sixth Annual Intensive Update in Neurology 0915-162016
47
Menierersquos syndrome
bull Fluctuating low frequency hearing loss
bull Tinnitus andor aural fullness
bull Vertigo lasting hours
Sixth Annual Intensive Update in Neurology 0915-162016
48
Normal
Sixth Annual Intensive Update in Neurology 0915-162016
49
Hydrops
Sixth Annual Intensive Update in Neurology 0915-162016
50
Menierersquos syndrome
ndash Diagnosis- Exclusion (RO acoustic)
ndash 23 of Menierersquos patients will improve spontaneously
ndash Treatment-1500 mgday low sodium triamterene375-
hydrochlorothiazide 25 vestibular suppressants (lorazepam
1 mg tablet sublingual Q 8 prn attacks) off label use
Sixth Annual Intensive Update in Neurology 0915-162016
51
Menierersquos syndrome ndash intratympanic steroids
ndash A newer treatment option for those failing medical management
ndash 90 response rate (Boleus-Aguirre MS Otology Neurotology
2008)
ndash Outcomes data confirm excellent clinical response in 3941
Menierersquos patients treated with intratympanic steroids in 2008
Sixth Annual Intensive Update in Neurology 0915-162016
52
Question 4
bull Menierersquos syndrome diagnosis is based on
1) Recurrent vertigo lasting at least 20 minutes
2) Aural fullness
3) Documented hearing loss
4) Other causes excluded
5) All of the above
Sixth Annual Intensive Update in Neurology 0915-162016
53
Question 4
bull Menierersquos syndrome diagnosis is based on
1) Recurrent vertigo lasting at least 20 minutes
2) Aural fullness
3) Documented hearing loss
4) Other causes excluded
5) All of the above
Answer 5 See references at end
Sixth Annual Intensive Update in Neurology 0915-162016
54
Superior Semicircular Canal Dehiscence (SSCD)
bull Sound andor pressure induced oscillopsia
bull CT reformatted in the plane of the superior canals
shows dehiscence of overlying bone
bull Low frequency ldquoconductive hyperacusisrdquo with elevated
bone thresholds normal reflexes and a conductive loss
Sixth Annual Intensive Update in Neurology 0915-162016
55
SSCD
bull Positive middle ear pressure or loud sound stimulates ampullofugal endolymph flow -canal activation
bull Eye movements are tonic upwards with ipsilateral intorsion
Sixth Annual Intensive Update in Neurology 0915-162016
56
SSCD
bull Valsalva against a closed glottis or jugular compression causes increased intracranial pressure and ampullopetal endolymph flow -canal inhibition
bull Eye movements are tonic downwards with ipsilateral extorsion
Sixth Annual Intensive Update in Neurology 0915-162016
57
Reconstructed left superior semicircular canal
Sixth Annual Intensive Update in Neurology 0915-162016
58
Sixth Annual Intensive Update in Neurology 0915-162016
59
SCHEMATIC DRAWING OF AN OTOLITHIC MACULA
Sixth Annual Intensive Update in Neurology 0915-162016
60
MACULAE OF THE UTRICLESACCULE
Sixth Annual Intensive Update in Neurology 0915-162016
61
HAIR CELLS
Sixth Annual Intensive Update in Neurology 0915-162016
62
SCANNING ELECTRON MICROGRAPH OF OTOCONIA
Sixth Annual Intensive Update in Neurology 0915-162016
63
Ocular VEMP
bull Response to loud clicks (80-110 dB) or forehead tap (Halmagyi and Curthoys 2003)
bull Contralateral oculomotor nucleus (CN III)
bull Inferior oblique muscle contraction (8 msec) detected by 5sec event triggered averaging
bull Noninverting electrode inferior to eye
bull Inverting below non-inverting electrode
bull Ground to forehead
bull Normal subjects have very low response
Sixth Annual Intensive Update in Neurology 0915-162016
64
Clinical applications
bull The o-VEMP is a sensitive test of otolithutricular
function
bull Complements vestibular test procedures that assess
semicircular canals
bull Abnormally robust responses are associated with third
window abnormalities such as semicircular canal
dehiscence
bull Serves as an excellent screening test for semicircular
canal dehiscence
Sixth Annual Intensive Update in Neurology 0915-162016
65
Question 5
bull Superior semicircular canal dehiscence
1) Causes increased ability to hear vibratory sounds
2) Improves hearing
3) Causes meningitis
4) Always needs surgical repair
Sixth Annual Intensive Update in Neurology 0915-162016
66
Question 5
bull Superior semicircular canal dehiscence
1) Causes increased ability to hear vibratory sounds
2) Improves hearing
3) Causes meningitis
4) Always needs surgical repair
Answer 1 References at end of lecture
Sixth Annual Intensive Update in Neurology 0915-162016
67
Enlarged vestibular aqueduct
bull Axial temporal bone CT showing right temporal bone with enlarged vestibular aqueduct (arrow)
bull Vestibular symptoms accompany progressive sensorineural hearing loss in 4-50 of patients in selected series
Sixth Annual Intensive Update in Neurology 0915-162016
68
Migraine
bull Family or personal history of migraine
bull 25 of migraine patients experience spontaneous vertigo (Baloh 1997)
bull Neuhauser criteria(2001) have been used to construct structured diagnostic interviews (Furman 2003)
bull Not always associated with headache
bull Motion sensitivity
bull Medication responsive
Sixth Annual Intensive Update in Neurology 0915-162016
69
ICHD-III beta Criteria for Vestibular Migraine
bull A At least five attacks fulfilling criteria C and D
bull B A current or past history of 11 migraine without aura or 12 migraine with aura
bull C Vestibular symptoms of moderate or severe intensity lasting between 5 minutes and 72 hours
bull D At least 50 associated with one of the following migrainous features
ndash 1 Headaches
ndash 2 Photophobia and phonophobia
ndash 3 Visual Aura
bull E Not better accounted for by another vestibular disorder
Sixth Annual Intensive Update in Neurology 0915-162016
70
Incidence of vertigo in MS
bull True vertigo (the perception of environmental
movement typically rotational) occurs in about
20 of MS patients and is the presenting
manifestation in 5
bull Up to 78 of MS patients report abnormalities
in balance at some point in their illness
Frohman et al Multiple Sclerosis 2003 9250
Sixth Annual Intensive Update in Neurology 0915-162016
71
Vertigo in MS due to demyelination
bull The most common neuroanatomic locations are the
root entry zone of CN VIII (giving absent calorics but
normal hearing and fixation suppression) and the
medial vestibular nucleus
bull Usually treated with steroids and vestibular
suppressants
Brandt 1993 Gass 1998 Thomke 1999
Sixth Annual Intensive Update in Neurology 0915-162016
72
Vertigo in MS
Frohmanrsquos large series (1153) reported that 52 of MS patients with acute vertigo had benign paroxysmal positional vertigo while 32 had new brainstem lesions
To avoid misdiagnoses and inappropriate treatment interventions alternative etiologies should be considered in all acute vertigo episodes in MS
E M Frohman Neurology 2000 and Multiple Sclerosis 2003
Sixth Annual Intensive Update in Neurology 0915-162016
73
Vestibular compensation
bull Acute unilateral or bilateral vestibular loss will prompt compensation thru central cervico-ocular visual and proprioceptive pathways
bull Unilateral loss may require several weeks for compensation to occur and activity especially head movements hastens compensation
bull Vestibular suppressant medication prolongs compensation
Sixth Annual Intensive Update in Neurology 0915-162016
74
Vestibular Rehabilitation
bull Performed by certified physical therapists many sites (Epic order vestibular PT) or check Vestibular Disorders of America website for providers
bull Assessment and treatment of benign positional vertigo
bull Directed rehab programs for central andor peripheral vestibular compensationndash Unilateral vestibulopathies
ndash Bilateral vestibular hypofunction
ndash Mutifactorial balance deficits and fall risk assessment
Sixth Annual Intensive Update in Neurology 0915-162016
75
Summary of whatrsquos new in peripheral vestibular disorders
bull Lateral semicircular canal benign paroxysmal positional vertigo presents with horizontal positioning nystagmus
bull Oral steroids appear to improve outcome in acute vestibular neuritis
bull Intratympanic steroids offer promise in Menierersquos syndrome
bull Superior semicircular canal dehiscence causes pressure and noise induced dizziness VEMP offers diagnostic information
Sixth Annual Intensive Update in Neurology 0915-162016
76
References
BPPVndash White J Benign paroxysmal positional vertigo Cleveland Clinic Journal of
Medicine 71(9)722-728 2004
ndash White J Coale K Catalano P Oas J Diagnosis and management of lateral semicircular canal benign positional vertigo Otolaryngology-Head and Neck Surgery 133 278-284 2005
ndash White J Savvides P Cherian N Oas J Canalith repositioning for benign paroxysmal positional vertigo Otology and Neurotology 26 704-710 2005
ndash White J Oas J Diagnosis and management of lateral semicircular canal conversions during particle repositioning therapy Laryngoscope 115(10) 1895-1897 2005
ndash Acute Vestibular Syndrome Hotson JR Baloh RW N Engl J Med 1998 Sep 3339(10)680-5
ndash Methylprednisolone valacyclovir or the combination for vestibular neuritis Strupp M Zingler VC Arbusow V Niklas D Maag KP Dieterich M Bense S Theil D Jahn K Brandt T N Engl J Med 2004 Jul 22351(4)354-61
ndash Intratympanic steroids for Meacuteniegraveres disease or syndrome Phillips JS Westerberg BCochrane Database Syst Rev 2011 Jul 6(7)CD008514 Review
ndash Sound- andor pressure-induced vertigo due to bone dehiscence of the superior semicircular canal Minor LB Solomon D Zinreich JS Zee DSArch Otolaryngol Head Neck Surg 1998 Mar124(3)249-58
Sixth Annual Intensive Update in Neurology 0915-162016
19
Canalith Repositioning Procedure
Sixth Annual Intensive Update in Neurology 0915-162016
20
Canalith Repositioning Procedure
Sixth Annual Intensive Update in Neurology 0915-162016
21
Canalith Repositioning
bull R posterior
BPPV
CPT 95992
Sixth Annual Intensive Update in Neurology 0915-162016
22
Posterior semicircular canalith repositioning
Sixth Annual Intensive Update in Neurology 0915-162016
23
Supine Positional Tests
Sixth Annual Intensive Update in Neurology 0915-162016
24
Lateral SCC-BPPV
bull Short latency horizontal nystagmus provoked by supine bilateral head turns with prolonged duration and poor fatigability
bull Uncommon (2-15 of total BPPV)
bull Two formsndash Geotropic ndash beating towards undermost ear
ndash Apogeotropic ndash beating away from undermost ear
Sixth Annual Intensive Update in Neurology 0915-162016
25
Geotropic Lateral Canal BPPV
bull Patient is positioned
first to the right lateral
position ndash nystagmus
beats right
bull Patient is then
positioned to the left
lateral position and
nystagmus reverses
and beats left
Sixth Annual Intensive Update in Neurology 0915-162016
26
Left Geotropic Lateral Semicircular Canal BPPV
Sixth Annual Intensive Update in Neurology 0915-162016
27
Repositioning Maneuvers for Geotropic Lateral Semicircular Canal (LSC) BPPV
bull Roll maneuver (Lempert Baloh)
ndash Patient rolls in 90 degree increments towards the good side
completely rolling over R LSC-BPPV repositioning shown
Sixth Annual Intensive Update in Neurology 0915-162016
28
Repositioning Maneuvers for Geotropic LSC BPPV
bull Gufoni maneuver
ndash Patient lies quickly onto good side and turns face-down to
floor for two minutes
Sixth Annual Intensive Update in Neurology 0915-162016
29
Left Apogeotropic LSC BPPV
Sixth Annual Intensive Update in Neurology 0915-162016
30
Nystagmus observed During Repositioning
bull Nystagmus should remain in the same torsional
direction as seen on DHT throughout the treatment of
posterior BPPV
bull Reversal of nystagmus direction suggests the particle
has fallen back into the canal and predicts failure
bull In Lateral BPPV nystagmus should beat towards the
good ear
Sixth Annual Intensive Update in Neurology 0915-162016
31
Posterior to Lateral canal conversion
Sixth Annual Intensive Update in Neurology 0915-162016
32
R Posterior to Lateral Canal conversion - geotropic
Sixth Annual Intensive Update in Neurology 0915-162016
33
BPPV Recurrence
bull Recurrence is common (15yr)
bull Otoconial adherence or mineralization demineralization
abnormalities may contribute to recurrence
bull Home canalith repositioning is effective (Radke 2004)
Sixth Annual Intensive Update in Neurology 0915-162016
34
Question 1
bull The most likely semicircular canal to be affected in
BPPV is
ndash 1) superior
ndash 2) lateral
ndash 3) posterior
ndash 4) combined
Sixth Annual Intensive Update in Neurology 0915-162016
35
Question 1
bull The most likely semicircular canal to be affected in
BPPV is
ndash 1) superior
ndash 2) lateral
ndash 3) posterior
ndash 4) combined
Answer 3 See references at end of lecture
Sixth Annual Intensive Update in Neurology 0915-162016
36
Acute Vestibular Syndrome
bull Rapid unilateral injury to either peripheral or central
vestibular structures produces prolonged vertigo
(days to weeks)
bull Severe vertigo nausea and vomiting spontaneous
nystagmus and postural instability
Sixth Annual Intensive Update in Neurology 0915-162016
37
Acute Vestibular Syndrome
bull 25 of patients with risk factors who present with isolated severe vertigo nystagmus and postural instability in the ER have an acute infarction of the inferior cerebellum (AICA PICA) Central imaging is suggested (Baloh 1998 NEJM)
ndash HTN diabetes smoking age gt65 Afib valvular heart disease occlusive vascular disease
Sixth Annual Intensive Update in Neurology 0915-162016
38
HINTS test
bull Head Impulse
bull Nystagmus
bull Test of Skew
ndash More sensitive than MRI in the diagnosis of acute stroke in
patients with acute vestibular syndrome with at least one risk
factor (Kattah J et al Stroke 2009)
Sixth Annual Intensive Update in Neurology 0915-162016
39
Head Impulse Test
bull No special equipment needed
bull Examiner stands in front of seated patient and
randomly moves head left and right Patient is asked to
focus on examinerrsquos nose
bull Rapid low amplitude thrust from lateral to straight
ahead causes a compensatory saccade when
performed towards the weak side
bull This finding is a permanent feature of unilateral
vestibular loss
Sixth Annual Intensive Update in Neurology 0915-162016
40
Head Impulse Test
Sixth Annual Intensive Update in Neurology 0915-162016
41
Head CT ndash Academy of Emergency Medicine 2013
bull The clinical value of head CT in isolated
dizzinessvertigo without any associated neurological
symptoms is very low
bull Less than 1 of head CTs ordered for dizziness and
vertigo have significant abnormalities and those
patients had severe headache and neurological deficits
in addition to dizziness
bull Forgoing unnecessary emergency head CTs could
save 360 million dollars annually
Sixth Annual Intensive Update in Neurology 0915-162016
42
Question 2
Head CT is not indicated for the evaluation of dizziness
and vertigo without additional neurological complaints because
1 It is too expensive
2 Less than 1 of CTs done for dizziness and vertigo are
abnormal and those patients had associated severe
neurological complaints
3 CT is the best imaging choice for the posterior fossa
4 Acoustic neuromas are well seen on CT
Sixth Annual Intensive Update in Neurology 0915-162016
43
Question 2
Head CT is not indicated for the evaluation of dizziness
and vertigo without additional neurological complaints because
1 It is too expensive
2 Less than 1 of CTs done for dizziness and vertigo are
abnormal and those patients had associated severe
neurological complaints
3 CT is the best imaging choice for the posterior fossa
4 Acoustic neuromas are well seen on CT
Answer 2 See references at end of lecture
Sixth Annual Intensive Update in Neurology 0915-162016
44
Acute Vestibular Syndrome
bull Usually peripheral and can be associated with
hearing loss (labyrinthitis)
bull Timely diagnosis improves recovery (ASAP
prednisone 1mgkgday for ten days and taper 10
mgday ndash Strupp M 2004 NEJM)
bull Avoid vestibular suppressants after 3 days and begin
vestibular rehabilitation to hasten compensation
Sixth Annual Intensive Update in Neurology 0915-162016
45
Question 3
bull Treatment for Acute Vestibular Syndrome
1) oral prednisone 1 mgkgday
2) Treat dehydration if necessary
3) Minimize vestibular suppressants after 3 days
4) Consider vestibular physical therapy
5) All of the above
Sixth Annual Intensive Update in Neurology 0915-162016
46
Question 3
bull Treatment for Acute Vestibular Syndrome
1) oral prednisone 1 mgkgday
2) Treat dehydration if necessary
3) Minimize vestibular suppressants after 3 days
4) Consider vestibular physical therapy
5) All of the above
Answer 5 See references at end of lecture
Sixth Annual Intensive Update in Neurology 0915-162016
47
Menierersquos syndrome
bull Fluctuating low frequency hearing loss
bull Tinnitus andor aural fullness
bull Vertigo lasting hours
Sixth Annual Intensive Update in Neurology 0915-162016
48
Normal
Sixth Annual Intensive Update in Neurology 0915-162016
49
Hydrops
Sixth Annual Intensive Update in Neurology 0915-162016
50
Menierersquos syndrome
ndash Diagnosis- Exclusion (RO acoustic)
ndash 23 of Menierersquos patients will improve spontaneously
ndash Treatment-1500 mgday low sodium triamterene375-
hydrochlorothiazide 25 vestibular suppressants (lorazepam
1 mg tablet sublingual Q 8 prn attacks) off label use
Sixth Annual Intensive Update in Neurology 0915-162016
51
Menierersquos syndrome ndash intratympanic steroids
ndash A newer treatment option for those failing medical management
ndash 90 response rate (Boleus-Aguirre MS Otology Neurotology
2008)
ndash Outcomes data confirm excellent clinical response in 3941
Menierersquos patients treated with intratympanic steroids in 2008
Sixth Annual Intensive Update in Neurology 0915-162016
52
Question 4
bull Menierersquos syndrome diagnosis is based on
1) Recurrent vertigo lasting at least 20 minutes
2) Aural fullness
3) Documented hearing loss
4) Other causes excluded
5) All of the above
Sixth Annual Intensive Update in Neurology 0915-162016
53
Question 4
bull Menierersquos syndrome diagnosis is based on
1) Recurrent vertigo lasting at least 20 minutes
2) Aural fullness
3) Documented hearing loss
4) Other causes excluded
5) All of the above
Answer 5 See references at end
Sixth Annual Intensive Update in Neurology 0915-162016
54
Superior Semicircular Canal Dehiscence (SSCD)
bull Sound andor pressure induced oscillopsia
bull CT reformatted in the plane of the superior canals
shows dehiscence of overlying bone
bull Low frequency ldquoconductive hyperacusisrdquo with elevated
bone thresholds normal reflexes and a conductive loss
Sixth Annual Intensive Update in Neurology 0915-162016
55
SSCD
bull Positive middle ear pressure or loud sound stimulates ampullofugal endolymph flow -canal activation
bull Eye movements are tonic upwards with ipsilateral intorsion
Sixth Annual Intensive Update in Neurology 0915-162016
56
SSCD
bull Valsalva against a closed glottis or jugular compression causes increased intracranial pressure and ampullopetal endolymph flow -canal inhibition
bull Eye movements are tonic downwards with ipsilateral extorsion
Sixth Annual Intensive Update in Neurology 0915-162016
57
Reconstructed left superior semicircular canal
Sixth Annual Intensive Update in Neurology 0915-162016
58
Sixth Annual Intensive Update in Neurology 0915-162016
59
SCHEMATIC DRAWING OF AN OTOLITHIC MACULA
Sixth Annual Intensive Update in Neurology 0915-162016
60
MACULAE OF THE UTRICLESACCULE
Sixth Annual Intensive Update in Neurology 0915-162016
61
HAIR CELLS
Sixth Annual Intensive Update in Neurology 0915-162016
62
SCANNING ELECTRON MICROGRAPH OF OTOCONIA
Sixth Annual Intensive Update in Neurology 0915-162016
63
Ocular VEMP
bull Response to loud clicks (80-110 dB) or forehead tap (Halmagyi and Curthoys 2003)
bull Contralateral oculomotor nucleus (CN III)
bull Inferior oblique muscle contraction (8 msec) detected by 5sec event triggered averaging
bull Noninverting electrode inferior to eye
bull Inverting below non-inverting electrode
bull Ground to forehead
bull Normal subjects have very low response
Sixth Annual Intensive Update in Neurology 0915-162016
64
Clinical applications
bull The o-VEMP is a sensitive test of otolithutricular
function
bull Complements vestibular test procedures that assess
semicircular canals
bull Abnormally robust responses are associated with third
window abnormalities such as semicircular canal
dehiscence
bull Serves as an excellent screening test for semicircular
canal dehiscence
Sixth Annual Intensive Update in Neurology 0915-162016
65
Question 5
bull Superior semicircular canal dehiscence
1) Causes increased ability to hear vibratory sounds
2) Improves hearing
3) Causes meningitis
4) Always needs surgical repair
Sixth Annual Intensive Update in Neurology 0915-162016
66
Question 5
bull Superior semicircular canal dehiscence
1) Causes increased ability to hear vibratory sounds
2) Improves hearing
3) Causes meningitis
4) Always needs surgical repair
Answer 1 References at end of lecture
Sixth Annual Intensive Update in Neurology 0915-162016
67
Enlarged vestibular aqueduct
bull Axial temporal bone CT showing right temporal bone with enlarged vestibular aqueduct (arrow)
bull Vestibular symptoms accompany progressive sensorineural hearing loss in 4-50 of patients in selected series
Sixth Annual Intensive Update in Neurology 0915-162016
68
Migraine
bull Family or personal history of migraine
bull 25 of migraine patients experience spontaneous vertigo (Baloh 1997)
bull Neuhauser criteria(2001) have been used to construct structured diagnostic interviews (Furman 2003)
bull Not always associated with headache
bull Motion sensitivity
bull Medication responsive
Sixth Annual Intensive Update in Neurology 0915-162016
69
ICHD-III beta Criteria for Vestibular Migraine
bull A At least five attacks fulfilling criteria C and D
bull B A current or past history of 11 migraine without aura or 12 migraine with aura
bull C Vestibular symptoms of moderate or severe intensity lasting between 5 minutes and 72 hours
bull D At least 50 associated with one of the following migrainous features
ndash 1 Headaches
ndash 2 Photophobia and phonophobia
ndash 3 Visual Aura
bull E Not better accounted for by another vestibular disorder
Sixth Annual Intensive Update in Neurology 0915-162016
70
Incidence of vertigo in MS
bull True vertigo (the perception of environmental
movement typically rotational) occurs in about
20 of MS patients and is the presenting
manifestation in 5
bull Up to 78 of MS patients report abnormalities
in balance at some point in their illness
Frohman et al Multiple Sclerosis 2003 9250
Sixth Annual Intensive Update in Neurology 0915-162016
71
Vertigo in MS due to demyelination
bull The most common neuroanatomic locations are the
root entry zone of CN VIII (giving absent calorics but
normal hearing and fixation suppression) and the
medial vestibular nucleus
bull Usually treated with steroids and vestibular
suppressants
Brandt 1993 Gass 1998 Thomke 1999
Sixth Annual Intensive Update in Neurology 0915-162016
72
Vertigo in MS
Frohmanrsquos large series (1153) reported that 52 of MS patients with acute vertigo had benign paroxysmal positional vertigo while 32 had new brainstem lesions
To avoid misdiagnoses and inappropriate treatment interventions alternative etiologies should be considered in all acute vertigo episodes in MS
E M Frohman Neurology 2000 and Multiple Sclerosis 2003
Sixth Annual Intensive Update in Neurology 0915-162016
73
Vestibular compensation
bull Acute unilateral or bilateral vestibular loss will prompt compensation thru central cervico-ocular visual and proprioceptive pathways
bull Unilateral loss may require several weeks for compensation to occur and activity especially head movements hastens compensation
bull Vestibular suppressant medication prolongs compensation
Sixth Annual Intensive Update in Neurology 0915-162016
74
Vestibular Rehabilitation
bull Performed by certified physical therapists many sites (Epic order vestibular PT) or check Vestibular Disorders of America website for providers
bull Assessment and treatment of benign positional vertigo
bull Directed rehab programs for central andor peripheral vestibular compensationndash Unilateral vestibulopathies
ndash Bilateral vestibular hypofunction
ndash Mutifactorial balance deficits and fall risk assessment
Sixth Annual Intensive Update in Neurology 0915-162016
75
Summary of whatrsquos new in peripheral vestibular disorders
bull Lateral semicircular canal benign paroxysmal positional vertigo presents with horizontal positioning nystagmus
bull Oral steroids appear to improve outcome in acute vestibular neuritis
bull Intratympanic steroids offer promise in Menierersquos syndrome
bull Superior semicircular canal dehiscence causes pressure and noise induced dizziness VEMP offers diagnostic information
Sixth Annual Intensive Update in Neurology 0915-162016
76
References
BPPVndash White J Benign paroxysmal positional vertigo Cleveland Clinic Journal of
Medicine 71(9)722-728 2004
ndash White J Coale K Catalano P Oas J Diagnosis and management of lateral semicircular canal benign positional vertigo Otolaryngology-Head and Neck Surgery 133 278-284 2005
ndash White J Savvides P Cherian N Oas J Canalith repositioning for benign paroxysmal positional vertigo Otology and Neurotology 26 704-710 2005
ndash White J Oas J Diagnosis and management of lateral semicircular canal conversions during particle repositioning therapy Laryngoscope 115(10) 1895-1897 2005
ndash Acute Vestibular Syndrome Hotson JR Baloh RW N Engl J Med 1998 Sep 3339(10)680-5
ndash Methylprednisolone valacyclovir or the combination for vestibular neuritis Strupp M Zingler VC Arbusow V Niklas D Maag KP Dieterich M Bense S Theil D Jahn K Brandt T N Engl J Med 2004 Jul 22351(4)354-61
ndash Intratympanic steroids for Meacuteniegraveres disease or syndrome Phillips JS Westerberg BCochrane Database Syst Rev 2011 Jul 6(7)CD008514 Review
ndash Sound- andor pressure-induced vertigo due to bone dehiscence of the superior semicircular canal Minor LB Solomon D Zinreich JS Zee DSArch Otolaryngol Head Neck Surg 1998 Mar124(3)249-58
Sixth Annual Intensive Update in Neurology 0915-162016
20
Canalith Repositioning Procedure
Sixth Annual Intensive Update in Neurology 0915-162016
21
Canalith Repositioning
bull R posterior
BPPV
CPT 95992
Sixth Annual Intensive Update in Neurology 0915-162016
22
Posterior semicircular canalith repositioning
Sixth Annual Intensive Update in Neurology 0915-162016
23
Supine Positional Tests
Sixth Annual Intensive Update in Neurology 0915-162016
24
Lateral SCC-BPPV
bull Short latency horizontal nystagmus provoked by supine bilateral head turns with prolonged duration and poor fatigability
bull Uncommon (2-15 of total BPPV)
bull Two formsndash Geotropic ndash beating towards undermost ear
ndash Apogeotropic ndash beating away from undermost ear
Sixth Annual Intensive Update in Neurology 0915-162016
25
Geotropic Lateral Canal BPPV
bull Patient is positioned
first to the right lateral
position ndash nystagmus
beats right
bull Patient is then
positioned to the left
lateral position and
nystagmus reverses
and beats left
Sixth Annual Intensive Update in Neurology 0915-162016
26
Left Geotropic Lateral Semicircular Canal BPPV
Sixth Annual Intensive Update in Neurology 0915-162016
27
Repositioning Maneuvers for Geotropic Lateral Semicircular Canal (LSC) BPPV
bull Roll maneuver (Lempert Baloh)
ndash Patient rolls in 90 degree increments towards the good side
completely rolling over R LSC-BPPV repositioning shown
Sixth Annual Intensive Update in Neurology 0915-162016
28
Repositioning Maneuvers for Geotropic LSC BPPV
bull Gufoni maneuver
ndash Patient lies quickly onto good side and turns face-down to
floor for two minutes
Sixth Annual Intensive Update in Neurology 0915-162016
29
Left Apogeotropic LSC BPPV
Sixth Annual Intensive Update in Neurology 0915-162016
30
Nystagmus observed During Repositioning
bull Nystagmus should remain in the same torsional
direction as seen on DHT throughout the treatment of
posterior BPPV
bull Reversal of nystagmus direction suggests the particle
has fallen back into the canal and predicts failure
bull In Lateral BPPV nystagmus should beat towards the
good ear
Sixth Annual Intensive Update in Neurology 0915-162016
31
Posterior to Lateral canal conversion
Sixth Annual Intensive Update in Neurology 0915-162016
32
R Posterior to Lateral Canal conversion - geotropic
Sixth Annual Intensive Update in Neurology 0915-162016
33
BPPV Recurrence
bull Recurrence is common (15yr)
bull Otoconial adherence or mineralization demineralization
abnormalities may contribute to recurrence
bull Home canalith repositioning is effective (Radke 2004)
Sixth Annual Intensive Update in Neurology 0915-162016
34
Question 1
bull The most likely semicircular canal to be affected in
BPPV is
ndash 1) superior
ndash 2) lateral
ndash 3) posterior
ndash 4) combined
Sixth Annual Intensive Update in Neurology 0915-162016
35
Question 1
bull The most likely semicircular canal to be affected in
BPPV is
ndash 1) superior
ndash 2) lateral
ndash 3) posterior
ndash 4) combined
Answer 3 See references at end of lecture
Sixth Annual Intensive Update in Neurology 0915-162016
36
Acute Vestibular Syndrome
bull Rapid unilateral injury to either peripheral or central
vestibular structures produces prolonged vertigo
(days to weeks)
bull Severe vertigo nausea and vomiting spontaneous
nystagmus and postural instability
Sixth Annual Intensive Update in Neurology 0915-162016
37
Acute Vestibular Syndrome
bull 25 of patients with risk factors who present with isolated severe vertigo nystagmus and postural instability in the ER have an acute infarction of the inferior cerebellum (AICA PICA) Central imaging is suggested (Baloh 1998 NEJM)
ndash HTN diabetes smoking age gt65 Afib valvular heart disease occlusive vascular disease
Sixth Annual Intensive Update in Neurology 0915-162016
38
HINTS test
bull Head Impulse
bull Nystagmus
bull Test of Skew
ndash More sensitive than MRI in the diagnosis of acute stroke in
patients with acute vestibular syndrome with at least one risk
factor (Kattah J et al Stroke 2009)
Sixth Annual Intensive Update in Neurology 0915-162016
39
Head Impulse Test
bull No special equipment needed
bull Examiner stands in front of seated patient and
randomly moves head left and right Patient is asked to
focus on examinerrsquos nose
bull Rapid low amplitude thrust from lateral to straight
ahead causes a compensatory saccade when
performed towards the weak side
bull This finding is a permanent feature of unilateral
vestibular loss
Sixth Annual Intensive Update in Neurology 0915-162016
40
Head Impulse Test
Sixth Annual Intensive Update in Neurology 0915-162016
41
Head CT ndash Academy of Emergency Medicine 2013
bull The clinical value of head CT in isolated
dizzinessvertigo without any associated neurological
symptoms is very low
bull Less than 1 of head CTs ordered for dizziness and
vertigo have significant abnormalities and those
patients had severe headache and neurological deficits
in addition to dizziness
bull Forgoing unnecessary emergency head CTs could
save 360 million dollars annually
Sixth Annual Intensive Update in Neurology 0915-162016
42
Question 2
Head CT is not indicated for the evaluation of dizziness
and vertigo without additional neurological complaints because
1 It is too expensive
2 Less than 1 of CTs done for dizziness and vertigo are
abnormal and those patients had associated severe
neurological complaints
3 CT is the best imaging choice for the posterior fossa
4 Acoustic neuromas are well seen on CT
Sixth Annual Intensive Update in Neurology 0915-162016
43
Question 2
Head CT is not indicated for the evaluation of dizziness
and vertigo without additional neurological complaints because
1 It is too expensive
2 Less than 1 of CTs done for dizziness and vertigo are
abnormal and those patients had associated severe
neurological complaints
3 CT is the best imaging choice for the posterior fossa
4 Acoustic neuromas are well seen on CT
Answer 2 See references at end of lecture
Sixth Annual Intensive Update in Neurology 0915-162016
44
Acute Vestibular Syndrome
bull Usually peripheral and can be associated with
hearing loss (labyrinthitis)
bull Timely diagnosis improves recovery (ASAP
prednisone 1mgkgday for ten days and taper 10
mgday ndash Strupp M 2004 NEJM)
bull Avoid vestibular suppressants after 3 days and begin
vestibular rehabilitation to hasten compensation
Sixth Annual Intensive Update in Neurology 0915-162016
45
Question 3
bull Treatment for Acute Vestibular Syndrome
1) oral prednisone 1 mgkgday
2) Treat dehydration if necessary
3) Minimize vestibular suppressants after 3 days
4) Consider vestibular physical therapy
5) All of the above
Sixth Annual Intensive Update in Neurology 0915-162016
46
Question 3
bull Treatment for Acute Vestibular Syndrome
1) oral prednisone 1 mgkgday
2) Treat dehydration if necessary
3) Minimize vestibular suppressants after 3 days
4) Consider vestibular physical therapy
5) All of the above
Answer 5 See references at end of lecture
Sixth Annual Intensive Update in Neurology 0915-162016
47
Menierersquos syndrome
bull Fluctuating low frequency hearing loss
bull Tinnitus andor aural fullness
bull Vertigo lasting hours
Sixth Annual Intensive Update in Neurology 0915-162016
48
Normal
Sixth Annual Intensive Update in Neurology 0915-162016
49
Hydrops
Sixth Annual Intensive Update in Neurology 0915-162016
50
Menierersquos syndrome
ndash Diagnosis- Exclusion (RO acoustic)
ndash 23 of Menierersquos patients will improve spontaneously
ndash Treatment-1500 mgday low sodium triamterene375-
hydrochlorothiazide 25 vestibular suppressants (lorazepam
1 mg tablet sublingual Q 8 prn attacks) off label use
Sixth Annual Intensive Update in Neurology 0915-162016
51
Menierersquos syndrome ndash intratympanic steroids
ndash A newer treatment option for those failing medical management
ndash 90 response rate (Boleus-Aguirre MS Otology Neurotology
2008)
ndash Outcomes data confirm excellent clinical response in 3941
Menierersquos patients treated with intratympanic steroids in 2008
Sixth Annual Intensive Update in Neurology 0915-162016
52
Question 4
bull Menierersquos syndrome diagnosis is based on
1) Recurrent vertigo lasting at least 20 minutes
2) Aural fullness
3) Documented hearing loss
4) Other causes excluded
5) All of the above
Sixth Annual Intensive Update in Neurology 0915-162016
53
Question 4
bull Menierersquos syndrome diagnosis is based on
1) Recurrent vertigo lasting at least 20 minutes
2) Aural fullness
3) Documented hearing loss
4) Other causes excluded
5) All of the above
Answer 5 See references at end
Sixth Annual Intensive Update in Neurology 0915-162016
54
Superior Semicircular Canal Dehiscence (SSCD)
bull Sound andor pressure induced oscillopsia
bull CT reformatted in the plane of the superior canals
shows dehiscence of overlying bone
bull Low frequency ldquoconductive hyperacusisrdquo with elevated
bone thresholds normal reflexes and a conductive loss
Sixth Annual Intensive Update in Neurology 0915-162016
55
SSCD
bull Positive middle ear pressure or loud sound stimulates ampullofugal endolymph flow -canal activation
bull Eye movements are tonic upwards with ipsilateral intorsion
Sixth Annual Intensive Update in Neurology 0915-162016
56
SSCD
bull Valsalva against a closed glottis or jugular compression causes increased intracranial pressure and ampullopetal endolymph flow -canal inhibition
bull Eye movements are tonic downwards with ipsilateral extorsion
Sixth Annual Intensive Update in Neurology 0915-162016
57
Reconstructed left superior semicircular canal
Sixth Annual Intensive Update in Neurology 0915-162016
58
Sixth Annual Intensive Update in Neurology 0915-162016
59
SCHEMATIC DRAWING OF AN OTOLITHIC MACULA
Sixth Annual Intensive Update in Neurology 0915-162016
60
MACULAE OF THE UTRICLESACCULE
Sixth Annual Intensive Update in Neurology 0915-162016
61
HAIR CELLS
Sixth Annual Intensive Update in Neurology 0915-162016
62
SCANNING ELECTRON MICROGRAPH OF OTOCONIA
Sixth Annual Intensive Update in Neurology 0915-162016
63
Ocular VEMP
bull Response to loud clicks (80-110 dB) or forehead tap (Halmagyi and Curthoys 2003)
bull Contralateral oculomotor nucleus (CN III)
bull Inferior oblique muscle contraction (8 msec) detected by 5sec event triggered averaging
bull Noninverting electrode inferior to eye
bull Inverting below non-inverting electrode
bull Ground to forehead
bull Normal subjects have very low response
Sixth Annual Intensive Update in Neurology 0915-162016
64
Clinical applications
bull The o-VEMP is a sensitive test of otolithutricular
function
bull Complements vestibular test procedures that assess
semicircular canals
bull Abnormally robust responses are associated with third
window abnormalities such as semicircular canal
dehiscence
bull Serves as an excellent screening test for semicircular
canal dehiscence
Sixth Annual Intensive Update in Neurology 0915-162016
65
Question 5
bull Superior semicircular canal dehiscence
1) Causes increased ability to hear vibratory sounds
2) Improves hearing
3) Causes meningitis
4) Always needs surgical repair
Sixth Annual Intensive Update in Neurology 0915-162016
66
Question 5
bull Superior semicircular canal dehiscence
1) Causes increased ability to hear vibratory sounds
2) Improves hearing
3) Causes meningitis
4) Always needs surgical repair
Answer 1 References at end of lecture
Sixth Annual Intensive Update in Neurology 0915-162016
67
Enlarged vestibular aqueduct
bull Axial temporal bone CT showing right temporal bone with enlarged vestibular aqueduct (arrow)
bull Vestibular symptoms accompany progressive sensorineural hearing loss in 4-50 of patients in selected series
Sixth Annual Intensive Update in Neurology 0915-162016
68
Migraine
bull Family or personal history of migraine
bull 25 of migraine patients experience spontaneous vertigo (Baloh 1997)
bull Neuhauser criteria(2001) have been used to construct structured diagnostic interviews (Furman 2003)
bull Not always associated with headache
bull Motion sensitivity
bull Medication responsive
Sixth Annual Intensive Update in Neurology 0915-162016
69
ICHD-III beta Criteria for Vestibular Migraine
bull A At least five attacks fulfilling criteria C and D
bull B A current or past history of 11 migraine without aura or 12 migraine with aura
bull C Vestibular symptoms of moderate or severe intensity lasting between 5 minutes and 72 hours
bull D At least 50 associated with one of the following migrainous features
ndash 1 Headaches
ndash 2 Photophobia and phonophobia
ndash 3 Visual Aura
bull E Not better accounted for by another vestibular disorder
Sixth Annual Intensive Update in Neurology 0915-162016
70
Incidence of vertigo in MS
bull True vertigo (the perception of environmental
movement typically rotational) occurs in about
20 of MS patients and is the presenting
manifestation in 5
bull Up to 78 of MS patients report abnormalities
in balance at some point in their illness
Frohman et al Multiple Sclerosis 2003 9250
Sixth Annual Intensive Update in Neurology 0915-162016
71
Vertigo in MS due to demyelination
bull The most common neuroanatomic locations are the
root entry zone of CN VIII (giving absent calorics but
normal hearing and fixation suppression) and the
medial vestibular nucleus
bull Usually treated with steroids and vestibular
suppressants
Brandt 1993 Gass 1998 Thomke 1999
Sixth Annual Intensive Update in Neurology 0915-162016
72
Vertigo in MS
Frohmanrsquos large series (1153) reported that 52 of MS patients with acute vertigo had benign paroxysmal positional vertigo while 32 had new brainstem lesions
To avoid misdiagnoses and inappropriate treatment interventions alternative etiologies should be considered in all acute vertigo episodes in MS
E M Frohman Neurology 2000 and Multiple Sclerosis 2003
Sixth Annual Intensive Update in Neurology 0915-162016
73
Vestibular compensation
bull Acute unilateral or bilateral vestibular loss will prompt compensation thru central cervico-ocular visual and proprioceptive pathways
bull Unilateral loss may require several weeks for compensation to occur and activity especially head movements hastens compensation
bull Vestibular suppressant medication prolongs compensation
Sixth Annual Intensive Update in Neurology 0915-162016
74
Vestibular Rehabilitation
bull Performed by certified physical therapists many sites (Epic order vestibular PT) or check Vestibular Disorders of America website for providers
bull Assessment and treatment of benign positional vertigo
bull Directed rehab programs for central andor peripheral vestibular compensationndash Unilateral vestibulopathies
ndash Bilateral vestibular hypofunction
ndash Mutifactorial balance deficits and fall risk assessment
Sixth Annual Intensive Update in Neurology 0915-162016
75
Summary of whatrsquos new in peripheral vestibular disorders
bull Lateral semicircular canal benign paroxysmal positional vertigo presents with horizontal positioning nystagmus
bull Oral steroids appear to improve outcome in acute vestibular neuritis
bull Intratympanic steroids offer promise in Menierersquos syndrome
bull Superior semicircular canal dehiscence causes pressure and noise induced dizziness VEMP offers diagnostic information
Sixth Annual Intensive Update in Neurology 0915-162016
76
References
BPPVndash White J Benign paroxysmal positional vertigo Cleveland Clinic Journal of
Medicine 71(9)722-728 2004
ndash White J Coale K Catalano P Oas J Diagnosis and management of lateral semicircular canal benign positional vertigo Otolaryngology-Head and Neck Surgery 133 278-284 2005
ndash White J Savvides P Cherian N Oas J Canalith repositioning for benign paroxysmal positional vertigo Otology and Neurotology 26 704-710 2005
ndash White J Oas J Diagnosis and management of lateral semicircular canal conversions during particle repositioning therapy Laryngoscope 115(10) 1895-1897 2005
ndash Acute Vestibular Syndrome Hotson JR Baloh RW N Engl J Med 1998 Sep 3339(10)680-5
ndash Methylprednisolone valacyclovir or the combination for vestibular neuritis Strupp M Zingler VC Arbusow V Niklas D Maag KP Dieterich M Bense S Theil D Jahn K Brandt T N Engl J Med 2004 Jul 22351(4)354-61
ndash Intratympanic steroids for Meacuteniegraveres disease or syndrome Phillips JS Westerberg BCochrane Database Syst Rev 2011 Jul 6(7)CD008514 Review
ndash Sound- andor pressure-induced vertigo due to bone dehiscence of the superior semicircular canal Minor LB Solomon D Zinreich JS Zee DSArch Otolaryngol Head Neck Surg 1998 Mar124(3)249-58
Sixth Annual Intensive Update in Neurology 0915-162016
21
Canalith Repositioning
bull R posterior
BPPV
CPT 95992
Sixth Annual Intensive Update in Neurology 0915-162016
22
Posterior semicircular canalith repositioning
Sixth Annual Intensive Update in Neurology 0915-162016
23
Supine Positional Tests
Sixth Annual Intensive Update in Neurology 0915-162016
24
Lateral SCC-BPPV
bull Short latency horizontal nystagmus provoked by supine bilateral head turns with prolonged duration and poor fatigability
bull Uncommon (2-15 of total BPPV)
bull Two formsndash Geotropic ndash beating towards undermost ear
ndash Apogeotropic ndash beating away from undermost ear
Sixth Annual Intensive Update in Neurology 0915-162016
25
Geotropic Lateral Canal BPPV
bull Patient is positioned
first to the right lateral
position ndash nystagmus
beats right
bull Patient is then
positioned to the left
lateral position and
nystagmus reverses
and beats left
Sixth Annual Intensive Update in Neurology 0915-162016
26
Left Geotropic Lateral Semicircular Canal BPPV
Sixth Annual Intensive Update in Neurology 0915-162016
27
Repositioning Maneuvers for Geotropic Lateral Semicircular Canal (LSC) BPPV
bull Roll maneuver (Lempert Baloh)
ndash Patient rolls in 90 degree increments towards the good side
completely rolling over R LSC-BPPV repositioning shown
Sixth Annual Intensive Update in Neurology 0915-162016
28
Repositioning Maneuvers for Geotropic LSC BPPV
bull Gufoni maneuver
ndash Patient lies quickly onto good side and turns face-down to
floor for two minutes
Sixth Annual Intensive Update in Neurology 0915-162016
29
Left Apogeotropic LSC BPPV
Sixth Annual Intensive Update in Neurology 0915-162016
30
Nystagmus observed During Repositioning
bull Nystagmus should remain in the same torsional
direction as seen on DHT throughout the treatment of
posterior BPPV
bull Reversal of nystagmus direction suggests the particle
has fallen back into the canal and predicts failure
bull In Lateral BPPV nystagmus should beat towards the
good ear
Sixth Annual Intensive Update in Neurology 0915-162016
31
Posterior to Lateral canal conversion
Sixth Annual Intensive Update in Neurology 0915-162016
32
R Posterior to Lateral Canal conversion - geotropic
Sixth Annual Intensive Update in Neurology 0915-162016
33
BPPV Recurrence
bull Recurrence is common (15yr)
bull Otoconial adherence or mineralization demineralization
abnormalities may contribute to recurrence
bull Home canalith repositioning is effective (Radke 2004)
Sixth Annual Intensive Update in Neurology 0915-162016
34
Question 1
bull The most likely semicircular canal to be affected in
BPPV is
ndash 1) superior
ndash 2) lateral
ndash 3) posterior
ndash 4) combined
Sixth Annual Intensive Update in Neurology 0915-162016
35
Question 1
bull The most likely semicircular canal to be affected in
BPPV is
ndash 1) superior
ndash 2) lateral
ndash 3) posterior
ndash 4) combined
Answer 3 See references at end of lecture
Sixth Annual Intensive Update in Neurology 0915-162016
36
Acute Vestibular Syndrome
bull Rapid unilateral injury to either peripheral or central
vestibular structures produces prolonged vertigo
(days to weeks)
bull Severe vertigo nausea and vomiting spontaneous
nystagmus and postural instability
Sixth Annual Intensive Update in Neurology 0915-162016
37
Acute Vestibular Syndrome
bull 25 of patients with risk factors who present with isolated severe vertigo nystagmus and postural instability in the ER have an acute infarction of the inferior cerebellum (AICA PICA) Central imaging is suggested (Baloh 1998 NEJM)
ndash HTN diabetes smoking age gt65 Afib valvular heart disease occlusive vascular disease
Sixth Annual Intensive Update in Neurology 0915-162016
38
HINTS test
bull Head Impulse
bull Nystagmus
bull Test of Skew
ndash More sensitive than MRI in the diagnosis of acute stroke in
patients with acute vestibular syndrome with at least one risk
factor (Kattah J et al Stroke 2009)
Sixth Annual Intensive Update in Neurology 0915-162016
39
Head Impulse Test
bull No special equipment needed
bull Examiner stands in front of seated patient and
randomly moves head left and right Patient is asked to
focus on examinerrsquos nose
bull Rapid low amplitude thrust from lateral to straight
ahead causes a compensatory saccade when
performed towards the weak side
bull This finding is a permanent feature of unilateral
vestibular loss
Sixth Annual Intensive Update in Neurology 0915-162016
40
Head Impulse Test
Sixth Annual Intensive Update in Neurology 0915-162016
41
Head CT ndash Academy of Emergency Medicine 2013
bull The clinical value of head CT in isolated
dizzinessvertigo without any associated neurological
symptoms is very low
bull Less than 1 of head CTs ordered for dizziness and
vertigo have significant abnormalities and those
patients had severe headache and neurological deficits
in addition to dizziness
bull Forgoing unnecessary emergency head CTs could
save 360 million dollars annually
Sixth Annual Intensive Update in Neurology 0915-162016
42
Question 2
Head CT is not indicated for the evaluation of dizziness
and vertigo without additional neurological complaints because
1 It is too expensive
2 Less than 1 of CTs done for dizziness and vertigo are
abnormal and those patients had associated severe
neurological complaints
3 CT is the best imaging choice for the posterior fossa
4 Acoustic neuromas are well seen on CT
Sixth Annual Intensive Update in Neurology 0915-162016
43
Question 2
Head CT is not indicated for the evaluation of dizziness
and vertigo without additional neurological complaints because
1 It is too expensive
2 Less than 1 of CTs done for dizziness and vertigo are
abnormal and those patients had associated severe
neurological complaints
3 CT is the best imaging choice for the posterior fossa
4 Acoustic neuromas are well seen on CT
Answer 2 See references at end of lecture
Sixth Annual Intensive Update in Neurology 0915-162016
44
Acute Vestibular Syndrome
bull Usually peripheral and can be associated with
hearing loss (labyrinthitis)
bull Timely diagnosis improves recovery (ASAP
prednisone 1mgkgday for ten days and taper 10
mgday ndash Strupp M 2004 NEJM)
bull Avoid vestibular suppressants after 3 days and begin
vestibular rehabilitation to hasten compensation
Sixth Annual Intensive Update in Neurology 0915-162016
45
Question 3
bull Treatment for Acute Vestibular Syndrome
1) oral prednisone 1 mgkgday
2) Treat dehydration if necessary
3) Minimize vestibular suppressants after 3 days
4) Consider vestibular physical therapy
5) All of the above
Sixth Annual Intensive Update in Neurology 0915-162016
46
Question 3
bull Treatment for Acute Vestibular Syndrome
1) oral prednisone 1 mgkgday
2) Treat dehydration if necessary
3) Minimize vestibular suppressants after 3 days
4) Consider vestibular physical therapy
5) All of the above
Answer 5 See references at end of lecture
Sixth Annual Intensive Update in Neurology 0915-162016
47
Menierersquos syndrome
bull Fluctuating low frequency hearing loss
bull Tinnitus andor aural fullness
bull Vertigo lasting hours
Sixth Annual Intensive Update in Neurology 0915-162016
48
Normal
Sixth Annual Intensive Update in Neurology 0915-162016
49
Hydrops
Sixth Annual Intensive Update in Neurology 0915-162016
50
Menierersquos syndrome
ndash Diagnosis- Exclusion (RO acoustic)
ndash 23 of Menierersquos patients will improve spontaneously
ndash Treatment-1500 mgday low sodium triamterene375-
hydrochlorothiazide 25 vestibular suppressants (lorazepam
1 mg tablet sublingual Q 8 prn attacks) off label use
Sixth Annual Intensive Update in Neurology 0915-162016
51
Menierersquos syndrome ndash intratympanic steroids
ndash A newer treatment option for those failing medical management
ndash 90 response rate (Boleus-Aguirre MS Otology Neurotology
2008)
ndash Outcomes data confirm excellent clinical response in 3941
Menierersquos patients treated with intratympanic steroids in 2008
Sixth Annual Intensive Update in Neurology 0915-162016
52
Question 4
bull Menierersquos syndrome diagnosis is based on
1) Recurrent vertigo lasting at least 20 minutes
2) Aural fullness
3) Documented hearing loss
4) Other causes excluded
5) All of the above
Sixth Annual Intensive Update in Neurology 0915-162016
53
Question 4
bull Menierersquos syndrome diagnosis is based on
1) Recurrent vertigo lasting at least 20 minutes
2) Aural fullness
3) Documented hearing loss
4) Other causes excluded
5) All of the above
Answer 5 See references at end
Sixth Annual Intensive Update in Neurology 0915-162016
54
Superior Semicircular Canal Dehiscence (SSCD)
bull Sound andor pressure induced oscillopsia
bull CT reformatted in the plane of the superior canals
shows dehiscence of overlying bone
bull Low frequency ldquoconductive hyperacusisrdquo with elevated
bone thresholds normal reflexes and a conductive loss
Sixth Annual Intensive Update in Neurology 0915-162016
55
SSCD
bull Positive middle ear pressure or loud sound stimulates ampullofugal endolymph flow -canal activation
bull Eye movements are tonic upwards with ipsilateral intorsion
Sixth Annual Intensive Update in Neurology 0915-162016
56
SSCD
bull Valsalva against a closed glottis or jugular compression causes increased intracranial pressure and ampullopetal endolymph flow -canal inhibition
bull Eye movements are tonic downwards with ipsilateral extorsion
Sixth Annual Intensive Update in Neurology 0915-162016
57
Reconstructed left superior semicircular canal
Sixth Annual Intensive Update in Neurology 0915-162016
58
Sixth Annual Intensive Update in Neurology 0915-162016
59
SCHEMATIC DRAWING OF AN OTOLITHIC MACULA
Sixth Annual Intensive Update in Neurology 0915-162016
60
MACULAE OF THE UTRICLESACCULE
Sixth Annual Intensive Update in Neurology 0915-162016
61
HAIR CELLS
Sixth Annual Intensive Update in Neurology 0915-162016
62
SCANNING ELECTRON MICROGRAPH OF OTOCONIA
Sixth Annual Intensive Update in Neurology 0915-162016
63
Ocular VEMP
bull Response to loud clicks (80-110 dB) or forehead tap (Halmagyi and Curthoys 2003)
bull Contralateral oculomotor nucleus (CN III)
bull Inferior oblique muscle contraction (8 msec) detected by 5sec event triggered averaging
bull Noninverting electrode inferior to eye
bull Inverting below non-inverting electrode
bull Ground to forehead
bull Normal subjects have very low response
Sixth Annual Intensive Update in Neurology 0915-162016
64
Clinical applications
bull The o-VEMP is a sensitive test of otolithutricular
function
bull Complements vestibular test procedures that assess
semicircular canals
bull Abnormally robust responses are associated with third
window abnormalities such as semicircular canal
dehiscence
bull Serves as an excellent screening test for semicircular
canal dehiscence
Sixth Annual Intensive Update in Neurology 0915-162016
65
Question 5
bull Superior semicircular canal dehiscence
1) Causes increased ability to hear vibratory sounds
2) Improves hearing
3) Causes meningitis
4) Always needs surgical repair
Sixth Annual Intensive Update in Neurology 0915-162016
66
Question 5
bull Superior semicircular canal dehiscence
1) Causes increased ability to hear vibratory sounds
2) Improves hearing
3) Causes meningitis
4) Always needs surgical repair
Answer 1 References at end of lecture
Sixth Annual Intensive Update in Neurology 0915-162016
67
Enlarged vestibular aqueduct
bull Axial temporal bone CT showing right temporal bone with enlarged vestibular aqueduct (arrow)
bull Vestibular symptoms accompany progressive sensorineural hearing loss in 4-50 of patients in selected series
Sixth Annual Intensive Update in Neurology 0915-162016
68
Migraine
bull Family or personal history of migraine
bull 25 of migraine patients experience spontaneous vertigo (Baloh 1997)
bull Neuhauser criteria(2001) have been used to construct structured diagnostic interviews (Furman 2003)
bull Not always associated with headache
bull Motion sensitivity
bull Medication responsive
Sixth Annual Intensive Update in Neurology 0915-162016
69
ICHD-III beta Criteria for Vestibular Migraine
bull A At least five attacks fulfilling criteria C and D
bull B A current or past history of 11 migraine without aura or 12 migraine with aura
bull C Vestibular symptoms of moderate or severe intensity lasting between 5 minutes and 72 hours
bull D At least 50 associated with one of the following migrainous features
ndash 1 Headaches
ndash 2 Photophobia and phonophobia
ndash 3 Visual Aura
bull E Not better accounted for by another vestibular disorder
Sixth Annual Intensive Update in Neurology 0915-162016
70
Incidence of vertigo in MS
bull True vertigo (the perception of environmental
movement typically rotational) occurs in about
20 of MS patients and is the presenting
manifestation in 5
bull Up to 78 of MS patients report abnormalities
in balance at some point in their illness
Frohman et al Multiple Sclerosis 2003 9250
Sixth Annual Intensive Update in Neurology 0915-162016
71
Vertigo in MS due to demyelination
bull The most common neuroanatomic locations are the
root entry zone of CN VIII (giving absent calorics but
normal hearing and fixation suppression) and the
medial vestibular nucleus
bull Usually treated with steroids and vestibular
suppressants
Brandt 1993 Gass 1998 Thomke 1999
Sixth Annual Intensive Update in Neurology 0915-162016
72
Vertigo in MS
Frohmanrsquos large series (1153) reported that 52 of MS patients with acute vertigo had benign paroxysmal positional vertigo while 32 had new brainstem lesions
To avoid misdiagnoses and inappropriate treatment interventions alternative etiologies should be considered in all acute vertigo episodes in MS
E M Frohman Neurology 2000 and Multiple Sclerosis 2003
Sixth Annual Intensive Update in Neurology 0915-162016
73
Vestibular compensation
bull Acute unilateral or bilateral vestibular loss will prompt compensation thru central cervico-ocular visual and proprioceptive pathways
bull Unilateral loss may require several weeks for compensation to occur and activity especially head movements hastens compensation
bull Vestibular suppressant medication prolongs compensation
Sixth Annual Intensive Update in Neurology 0915-162016
74
Vestibular Rehabilitation
bull Performed by certified physical therapists many sites (Epic order vestibular PT) or check Vestibular Disorders of America website for providers
bull Assessment and treatment of benign positional vertigo
bull Directed rehab programs for central andor peripheral vestibular compensationndash Unilateral vestibulopathies
ndash Bilateral vestibular hypofunction
ndash Mutifactorial balance deficits and fall risk assessment
Sixth Annual Intensive Update in Neurology 0915-162016
75
Summary of whatrsquos new in peripheral vestibular disorders
bull Lateral semicircular canal benign paroxysmal positional vertigo presents with horizontal positioning nystagmus
bull Oral steroids appear to improve outcome in acute vestibular neuritis
bull Intratympanic steroids offer promise in Menierersquos syndrome
bull Superior semicircular canal dehiscence causes pressure and noise induced dizziness VEMP offers diagnostic information
Sixth Annual Intensive Update in Neurology 0915-162016
76
References
BPPVndash White J Benign paroxysmal positional vertigo Cleveland Clinic Journal of
Medicine 71(9)722-728 2004
ndash White J Coale K Catalano P Oas J Diagnosis and management of lateral semicircular canal benign positional vertigo Otolaryngology-Head and Neck Surgery 133 278-284 2005
ndash White J Savvides P Cherian N Oas J Canalith repositioning for benign paroxysmal positional vertigo Otology and Neurotology 26 704-710 2005
ndash White J Oas J Diagnosis and management of lateral semicircular canal conversions during particle repositioning therapy Laryngoscope 115(10) 1895-1897 2005
ndash Acute Vestibular Syndrome Hotson JR Baloh RW N Engl J Med 1998 Sep 3339(10)680-5
ndash Methylprednisolone valacyclovir or the combination for vestibular neuritis Strupp M Zingler VC Arbusow V Niklas D Maag KP Dieterich M Bense S Theil D Jahn K Brandt T N Engl J Med 2004 Jul 22351(4)354-61
ndash Intratympanic steroids for Meacuteniegraveres disease or syndrome Phillips JS Westerberg BCochrane Database Syst Rev 2011 Jul 6(7)CD008514 Review
ndash Sound- andor pressure-induced vertigo due to bone dehiscence of the superior semicircular canal Minor LB Solomon D Zinreich JS Zee DSArch Otolaryngol Head Neck Surg 1998 Mar124(3)249-58
Sixth Annual Intensive Update in Neurology 0915-162016
22
Posterior semicircular canalith repositioning
Sixth Annual Intensive Update in Neurology 0915-162016
23
Supine Positional Tests
Sixth Annual Intensive Update in Neurology 0915-162016
24
Lateral SCC-BPPV
bull Short latency horizontal nystagmus provoked by supine bilateral head turns with prolonged duration and poor fatigability
bull Uncommon (2-15 of total BPPV)
bull Two formsndash Geotropic ndash beating towards undermost ear
ndash Apogeotropic ndash beating away from undermost ear
Sixth Annual Intensive Update in Neurology 0915-162016
25
Geotropic Lateral Canal BPPV
bull Patient is positioned
first to the right lateral
position ndash nystagmus
beats right
bull Patient is then
positioned to the left
lateral position and
nystagmus reverses
and beats left
Sixth Annual Intensive Update in Neurology 0915-162016
26
Left Geotropic Lateral Semicircular Canal BPPV
Sixth Annual Intensive Update in Neurology 0915-162016
27
Repositioning Maneuvers for Geotropic Lateral Semicircular Canal (LSC) BPPV
bull Roll maneuver (Lempert Baloh)
ndash Patient rolls in 90 degree increments towards the good side
completely rolling over R LSC-BPPV repositioning shown
Sixth Annual Intensive Update in Neurology 0915-162016
28
Repositioning Maneuvers for Geotropic LSC BPPV
bull Gufoni maneuver
ndash Patient lies quickly onto good side and turns face-down to
floor for two minutes
Sixth Annual Intensive Update in Neurology 0915-162016
29
Left Apogeotropic LSC BPPV
Sixth Annual Intensive Update in Neurology 0915-162016
30
Nystagmus observed During Repositioning
bull Nystagmus should remain in the same torsional
direction as seen on DHT throughout the treatment of
posterior BPPV
bull Reversal of nystagmus direction suggests the particle
has fallen back into the canal and predicts failure
bull In Lateral BPPV nystagmus should beat towards the
good ear
Sixth Annual Intensive Update in Neurology 0915-162016
31
Posterior to Lateral canal conversion
Sixth Annual Intensive Update in Neurology 0915-162016
32
R Posterior to Lateral Canal conversion - geotropic
Sixth Annual Intensive Update in Neurology 0915-162016
33
BPPV Recurrence
bull Recurrence is common (15yr)
bull Otoconial adherence or mineralization demineralization
abnormalities may contribute to recurrence
bull Home canalith repositioning is effective (Radke 2004)
Sixth Annual Intensive Update in Neurology 0915-162016
34
Question 1
bull The most likely semicircular canal to be affected in
BPPV is
ndash 1) superior
ndash 2) lateral
ndash 3) posterior
ndash 4) combined
Sixth Annual Intensive Update in Neurology 0915-162016
35
Question 1
bull The most likely semicircular canal to be affected in
BPPV is
ndash 1) superior
ndash 2) lateral
ndash 3) posterior
ndash 4) combined
Answer 3 See references at end of lecture
Sixth Annual Intensive Update in Neurology 0915-162016
36
Acute Vestibular Syndrome
bull Rapid unilateral injury to either peripheral or central
vestibular structures produces prolonged vertigo
(days to weeks)
bull Severe vertigo nausea and vomiting spontaneous
nystagmus and postural instability
Sixth Annual Intensive Update in Neurology 0915-162016
37
Acute Vestibular Syndrome
bull 25 of patients with risk factors who present with isolated severe vertigo nystagmus and postural instability in the ER have an acute infarction of the inferior cerebellum (AICA PICA) Central imaging is suggested (Baloh 1998 NEJM)
ndash HTN diabetes smoking age gt65 Afib valvular heart disease occlusive vascular disease
Sixth Annual Intensive Update in Neurology 0915-162016
38
HINTS test
bull Head Impulse
bull Nystagmus
bull Test of Skew
ndash More sensitive than MRI in the diagnosis of acute stroke in
patients with acute vestibular syndrome with at least one risk
factor (Kattah J et al Stroke 2009)
Sixth Annual Intensive Update in Neurology 0915-162016
39
Head Impulse Test
bull No special equipment needed
bull Examiner stands in front of seated patient and
randomly moves head left and right Patient is asked to
focus on examinerrsquos nose
bull Rapid low amplitude thrust from lateral to straight
ahead causes a compensatory saccade when
performed towards the weak side
bull This finding is a permanent feature of unilateral
vestibular loss
Sixth Annual Intensive Update in Neurology 0915-162016
40
Head Impulse Test
Sixth Annual Intensive Update in Neurology 0915-162016
41
Head CT ndash Academy of Emergency Medicine 2013
bull The clinical value of head CT in isolated
dizzinessvertigo without any associated neurological
symptoms is very low
bull Less than 1 of head CTs ordered for dizziness and
vertigo have significant abnormalities and those
patients had severe headache and neurological deficits
in addition to dizziness
bull Forgoing unnecessary emergency head CTs could
save 360 million dollars annually
Sixth Annual Intensive Update in Neurology 0915-162016
42
Question 2
Head CT is not indicated for the evaluation of dizziness
and vertigo without additional neurological complaints because
1 It is too expensive
2 Less than 1 of CTs done for dizziness and vertigo are
abnormal and those patients had associated severe
neurological complaints
3 CT is the best imaging choice for the posterior fossa
4 Acoustic neuromas are well seen on CT
Sixth Annual Intensive Update in Neurology 0915-162016
43
Question 2
Head CT is not indicated for the evaluation of dizziness
and vertigo without additional neurological complaints because
1 It is too expensive
2 Less than 1 of CTs done for dizziness and vertigo are
abnormal and those patients had associated severe
neurological complaints
3 CT is the best imaging choice for the posterior fossa
4 Acoustic neuromas are well seen on CT
Answer 2 See references at end of lecture
Sixth Annual Intensive Update in Neurology 0915-162016
44
Acute Vestibular Syndrome
bull Usually peripheral and can be associated with
hearing loss (labyrinthitis)
bull Timely diagnosis improves recovery (ASAP
prednisone 1mgkgday for ten days and taper 10
mgday ndash Strupp M 2004 NEJM)
bull Avoid vestibular suppressants after 3 days and begin
vestibular rehabilitation to hasten compensation
Sixth Annual Intensive Update in Neurology 0915-162016
45
Question 3
bull Treatment for Acute Vestibular Syndrome
1) oral prednisone 1 mgkgday
2) Treat dehydration if necessary
3) Minimize vestibular suppressants after 3 days
4) Consider vestibular physical therapy
5) All of the above
Sixth Annual Intensive Update in Neurology 0915-162016
46
Question 3
bull Treatment for Acute Vestibular Syndrome
1) oral prednisone 1 mgkgday
2) Treat dehydration if necessary
3) Minimize vestibular suppressants after 3 days
4) Consider vestibular physical therapy
5) All of the above
Answer 5 See references at end of lecture
Sixth Annual Intensive Update in Neurology 0915-162016
47
Menierersquos syndrome
bull Fluctuating low frequency hearing loss
bull Tinnitus andor aural fullness
bull Vertigo lasting hours
Sixth Annual Intensive Update in Neurology 0915-162016
48
Normal
Sixth Annual Intensive Update in Neurology 0915-162016
49
Hydrops
Sixth Annual Intensive Update in Neurology 0915-162016
50
Menierersquos syndrome
ndash Diagnosis- Exclusion (RO acoustic)
ndash 23 of Menierersquos patients will improve spontaneously
ndash Treatment-1500 mgday low sodium triamterene375-
hydrochlorothiazide 25 vestibular suppressants (lorazepam
1 mg tablet sublingual Q 8 prn attacks) off label use
Sixth Annual Intensive Update in Neurology 0915-162016
51
Menierersquos syndrome ndash intratympanic steroids
ndash A newer treatment option for those failing medical management
ndash 90 response rate (Boleus-Aguirre MS Otology Neurotology
2008)
ndash Outcomes data confirm excellent clinical response in 3941
Menierersquos patients treated with intratympanic steroids in 2008
Sixth Annual Intensive Update in Neurology 0915-162016
52
Question 4
bull Menierersquos syndrome diagnosis is based on
1) Recurrent vertigo lasting at least 20 minutes
2) Aural fullness
3) Documented hearing loss
4) Other causes excluded
5) All of the above
Sixth Annual Intensive Update in Neurology 0915-162016
53
Question 4
bull Menierersquos syndrome diagnosis is based on
1) Recurrent vertigo lasting at least 20 minutes
2) Aural fullness
3) Documented hearing loss
4) Other causes excluded
5) All of the above
Answer 5 See references at end
Sixth Annual Intensive Update in Neurology 0915-162016
54
Superior Semicircular Canal Dehiscence (SSCD)
bull Sound andor pressure induced oscillopsia
bull CT reformatted in the plane of the superior canals
shows dehiscence of overlying bone
bull Low frequency ldquoconductive hyperacusisrdquo with elevated
bone thresholds normal reflexes and a conductive loss
Sixth Annual Intensive Update in Neurology 0915-162016
55
SSCD
bull Positive middle ear pressure or loud sound stimulates ampullofugal endolymph flow -canal activation
bull Eye movements are tonic upwards with ipsilateral intorsion
Sixth Annual Intensive Update in Neurology 0915-162016
56
SSCD
bull Valsalva against a closed glottis or jugular compression causes increased intracranial pressure and ampullopetal endolymph flow -canal inhibition
bull Eye movements are tonic downwards with ipsilateral extorsion
Sixth Annual Intensive Update in Neurology 0915-162016
57
Reconstructed left superior semicircular canal
Sixth Annual Intensive Update in Neurology 0915-162016
58
Sixth Annual Intensive Update in Neurology 0915-162016
59
SCHEMATIC DRAWING OF AN OTOLITHIC MACULA
Sixth Annual Intensive Update in Neurology 0915-162016
60
MACULAE OF THE UTRICLESACCULE
Sixth Annual Intensive Update in Neurology 0915-162016
61
HAIR CELLS
Sixth Annual Intensive Update in Neurology 0915-162016
62
SCANNING ELECTRON MICROGRAPH OF OTOCONIA
Sixth Annual Intensive Update in Neurology 0915-162016
63
Ocular VEMP
bull Response to loud clicks (80-110 dB) or forehead tap (Halmagyi and Curthoys 2003)
bull Contralateral oculomotor nucleus (CN III)
bull Inferior oblique muscle contraction (8 msec) detected by 5sec event triggered averaging
bull Noninverting electrode inferior to eye
bull Inverting below non-inverting electrode
bull Ground to forehead
bull Normal subjects have very low response
Sixth Annual Intensive Update in Neurology 0915-162016
64
Clinical applications
bull The o-VEMP is a sensitive test of otolithutricular
function
bull Complements vestibular test procedures that assess
semicircular canals
bull Abnormally robust responses are associated with third
window abnormalities such as semicircular canal
dehiscence
bull Serves as an excellent screening test for semicircular
canal dehiscence
Sixth Annual Intensive Update in Neurology 0915-162016
65
Question 5
bull Superior semicircular canal dehiscence
1) Causes increased ability to hear vibratory sounds
2) Improves hearing
3) Causes meningitis
4) Always needs surgical repair
Sixth Annual Intensive Update in Neurology 0915-162016
66
Question 5
bull Superior semicircular canal dehiscence
1) Causes increased ability to hear vibratory sounds
2) Improves hearing
3) Causes meningitis
4) Always needs surgical repair
Answer 1 References at end of lecture
Sixth Annual Intensive Update in Neurology 0915-162016
67
Enlarged vestibular aqueduct
bull Axial temporal bone CT showing right temporal bone with enlarged vestibular aqueduct (arrow)
bull Vestibular symptoms accompany progressive sensorineural hearing loss in 4-50 of patients in selected series
Sixth Annual Intensive Update in Neurology 0915-162016
68
Migraine
bull Family or personal history of migraine
bull 25 of migraine patients experience spontaneous vertigo (Baloh 1997)
bull Neuhauser criteria(2001) have been used to construct structured diagnostic interviews (Furman 2003)
bull Not always associated with headache
bull Motion sensitivity
bull Medication responsive
Sixth Annual Intensive Update in Neurology 0915-162016
69
ICHD-III beta Criteria for Vestibular Migraine
bull A At least five attacks fulfilling criteria C and D
bull B A current or past history of 11 migraine without aura or 12 migraine with aura
bull C Vestibular symptoms of moderate or severe intensity lasting between 5 minutes and 72 hours
bull D At least 50 associated with one of the following migrainous features
ndash 1 Headaches
ndash 2 Photophobia and phonophobia
ndash 3 Visual Aura
bull E Not better accounted for by another vestibular disorder
Sixth Annual Intensive Update in Neurology 0915-162016
70
Incidence of vertigo in MS
bull True vertigo (the perception of environmental
movement typically rotational) occurs in about
20 of MS patients and is the presenting
manifestation in 5
bull Up to 78 of MS patients report abnormalities
in balance at some point in their illness
Frohman et al Multiple Sclerosis 2003 9250
Sixth Annual Intensive Update in Neurology 0915-162016
71
Vertigo in MS due to demyelination
bull The most common neuroanatomic locations are the
root entry zone of CN VIII (giving absent calorics but
normal hearing and fixation suppression) and the
medial vestibular nucleus
bull Usually treated with steroids and vestibular
suppressants
Brandt 1993 Gass 1998 Thomke 1999
Sixth Annual Intensive Update in Neurology 0915-162016
72
Vertigo in MS
Frohmanrsquos large series (1153) reported that 52 of MS patients with acute vertigo had benign paroxysmal positional vertigo while 32 had new brainstem lesions
To avoid misdiagnoses and inappropriate treatment interventions alternative etiologies should be considered in all acute vertigo episodes in MS
E M Frohman Neurology 2000 and Multiple Sclerosis 2003
Sixth Annual Intensive Update in Neurology 0915-162016
73
Vestibular compensation
bull Acute unilateral or bilateral vestibular loss will prompt compensation thru central cervico-ocular visual and proprioceptive pathways
bull Unilateral loss may require several weeks for compensation to occur and activity especially head movements hastens compensation
bull Vestibular suppressant medication prolongs compensation
Sixth Annual Intensive Update in Neurology 0915-162016
74
Vestibular Rehabilitation
bull Performed by certified physical therapists many sites (Epic order vestibular PT) or check Vestibular Disorders of America website for providers
bull Assessment and treatment of benign positional vertigo
bull Directed rehab programs for central andor peripheral vestibular compensationndash Unilateral vestibulopathies
ndash Bilateral vestibular hypofunction
ndash Mutifactorial balance deficits and fall risk assessment
Sixth Annual Intensive Update in Neurology 0915-162016
75
Summary of whatrsquos new in peripheral vestibular disorders
bull Lateral semicircular canal benign paroxysmal positional vertigo presents with horizontal positioning nystagmus
bull Oral steroids appear to improve outcome in acute vestibular neuritis
bull Intratympanic steroids offer promise in Menierersquos syndrome
bull Superior semicircular canal dehiscence causes pressure and noise induced dizziness VEMP offers diagnostic information
Sixth Annual Intensive Update in Neurology 0915-162016
76
References
BPPVndash White J Benign paroxysmal positional vertigo Cleveland Clinic Journal of
Medicine 71(9)722-728 2004
ndash White J Coale K Catalano P Oas J Diagnosis and management of lateral semicircular canal benign positional vertigo Otolaryngology-Head and Neck Surgery 133 278-284 2005
ndash White J Savvides P Cherian N Oas J Canalith repositioning for benign paroxysmal positional vertigo Otology and Neurotology 26 704-710 2005
ndash White J Oas J Diagnosis and management of lateral semicircular canal conversions during particle repositioning therapy Laryngoscope 115(10) 1895-1897 2005
ndash Acute Vestibular Syndrome Hotson JR Baloh RW N Engl J Med 1998 Sep 3339(10)680-5
ndash Methylprednisolone valacyclovir or the combination for vestibular neuritis Strupp M Zingler VC Arbusow V Niklas D Maag KP Dieterich M Bense S Theil D Jahn K Brandt T N Engl J Med 2004 Jul 22351(4)354-61
ndash Intratympanic steroids for Meacuteniegraveres disease or syndrome Phillips JS Westerberg BCochrane Database Syst Rev 2011 Jul 6(7)CD008514 Review
ndash Sound- andor pressure-induced vertigo due to bone dehiscence of the superior semicircular canal Minor LB Solomon D Zinreich JS Zee DSArch Otolaryngol Head Neck Surg 1998 Mar124(3)249-58
Sixth Annual Intensive Update in Neurology 0915-162016
23
Supine Positional Tests
Sixth Annual Intensive Update in Neurology 0915-162016
24
Lateral SCC-BPPV
bull Short latency horizontal nystagmus provoked by supine bilateral head turns with prolonged duration and poor fatigability
bull Uncommon (2-15 of total BPPV)
bull Two formsndash Geotropic ndash beating towards undermost ear
ndash Apogeotropic ndash beating away from undermost ear
Sixth Annual Intensive Update in Neurology 0915-162016
25
Geotropic Lateral Canal BPPV
bull Patient is positioned
first to the right lateral
position ndash nystagmus
beats right
bull Patient is then
positioned to the left
lateral position and
nystagmus reverses
and beats left
Sixth Annual Intensive Update in Neurology 0915-162016
26
Left Geotropic Lateral Semicircular Canal BPPV
Sixth Annual Intensive Update in Neurology 0915-162016
27
Repositioning Maneuvers for Geotropic Lateral Semicircular Canal (LSC) BPPV
bull Roll maneuver (Lempert Baloh)
ndash Patient rolls in 90 degree increments towards the good side
completely rolling over R LSC-BPPV repositioning shown
Sixth Annual Intensive Update in Neurology 0915-162016
28
Repositioning Maneuvers for Geotropic LSC BPPV
bull Gufoni maneuver
ndash Patient lies quickly onto good side and turns face-down to
floor for two minutes
Sixth Annual Intensive Update in Neurology 0915-162016
29
Left Apogeotropic LSC BPPV
Sixth Annual Intensive Update in Neurology 0915-162016
30
Nystagmus observed During Repositioning
bull Nystagmus should remain in the same torsional
direction as seen on DHT throughout the treatment of
posterior BPPV
bull Reversal of nystagmus direction suggests the particle
has fallen back into the canal and predicts failure
bull In Lateral BPPV nystagmus should beat towards the
good ear
Sixth Annual Intensive Update in Neurology 0915-162016
31
Posterior to Lateral canal conversion
Sixth Annual Intensive Update in Neurology 0915-162016
32
R Posterior to Lateral Canal conversion - geotropic
Sixth Annual Intensive Update in Neurology 0915-162016
33
BPPV Recurrence
bull Recurrence is common (15yr)
bull Otoconial adherence or mineralization demineralization
abnormalities may contribute to recurrence
bull Home canalith repositioning is effective (Radke 2004)
Sixth Annual Intensive Update in Neurology 0915-162016
34
Question 1
bull The most likely semicircular canal to be affected in
BPPV is
ndash 1) superior
ndash 2) lateral
ndash 3) posterior
ndash 4) combined
Sixth Annual Intensive Update in Neurology 0915-162016
35
Question 1
bull The most likely semicircular canal to be affected in
BPPV is
ndash 1) superior
ndash 2) lateral
ndash 3) posterior
ndash 4) combined
Answer 3 See references at end of lecture
Sixth Annual Intensive Update in Neurology 0915-162016
36
Acute Vestibular Syndrome
bull Rapid unilateral injury to either peripheral or central
vestibular structures produces prolonged vertigo
(days to weeks)
bull Severe vertigo nausea and vomiting spontaneous
nystagmus and postural instability
Sixth Annual Intensive Update in Neurology 0915-162016
37
Acute Vestibular Syndrome
bull 25 of patients with risk factors who present with isolated severe vertigo nystagmus and postural instability in the ER have an acute infarction of the inferior cerebellum (AICA PICA) Central imaging is suggested (Baloh 1998 NEJM)
ndash HTN diabetes smoking age gt65 Afib valvular heart disease occlusive vascular disease
Sixth Annual Intensive Update in Neurology 0915-162016
38
HINTS test
bull Head Impulse
bull Nystagmus
bull Test of Skew
ndash More sensitive than MRI in the diagnosis of acute stroke in
patients with acute vestibular syndrome with at least one risk
factor (Kattah J et al Stroke 2009)
Sixth Annual Intensive Update in Neurology 0915-162016
39
Head Impulse Test
bull No special equipment needed
bull Examiner stands in front of seated patient and
randomly moves head left and right Patient is asked to
focus on examinerrsquos nose
bull Rapid low amplitude thrust from lateral to straight
ahead causes a compensatory saccade when
performed towards the weak side
bull This finding is a permanent feature of unilateral
vestibular loss
Sixth Annual Intensive Update in Neurology 0915-162016
40
Head Impulse Test
Sixth Annual Intensive Update in Neurology 0915-162016
41
Head CT ndash Academy of Emergency Medicine 2013
bull The clinical value of head CT in isolated
dizzinessvertigo without any associated neurological
symptoms is very low
bull Less than 1 of head CTs ordered for dizziness and
vertigo have significant abnormalities and those
patients had severe headache and neurological deficits
in addition to dizziness
bull Forgoing unnecessary emergency head CTs could
save 360 million dollars annually
Sixth Annual Intensive Update in Neurology 0915-162016
42
Question 2
Head CT is not indicated for the evaluation of dizziness
and vertigo without additional neurological complaints because
1 It is too expensive
2 Less than 1 of CTs done for dizziness and vertigo are
abnormal and those patients had associated severe
neurological complaints
3 CT is the best imaging choice for the posterior fossa
4 Acoustic neuromas are well seen on CT
Sixth Annual Intensive Update in Neurology 0915-162016
43
Question 2
Head CT is not indicated for the evaluation of dizziness
and vertigo without additional neurological complaints because
1 It is too expensive
2 Less than 1 of CTs done for dizziness and vertigo are
abnormal and those patients had associated severe
neurological complaints
3 CT is the best imaging choice for the posterior fossa
4 Acoustic neuromas are well seen on CT
Answer 2 See references at end of lecture
Sixth Annual Intensive Update in Neurology 0915-162016
44
Acute Vestibular Syndrome
bull Usually peripheral and can be associated with
hearing loss (labyrinthitis)
bull Timely diagnosis improves recovery (ASAP
prednisone 1mgkgday for ten days and taper 10
mgday ndash Strupp M 2004 NEJM)
bull Avoid vestibular suppressants after 3 days and begin
vestibular rehabilitation to hasten compensation
Sixth Annual Intensive Update in Neurology 0915-162016
45
Question 3
bull Treatment for Acute Vestibular Syndrome
1) oral prednisone 1 mgkgday
2) Treat dehydration if necessary
3) Minimize vestibular suppressants after 3 days
4) Consider vestibular physical therapy
5) All of the above
Sixth Annual Intensive Update in Neurology 0915-162016
46
Question 3
bull Treatment for Acute Vestibular Syndrome
1) oral prednisone 1 mgkgday
2) Treat dehydration if necessary
3) Minimize vestibular suppressants after 3 days
4) Consider vestibular physical therapy
5) All of the above
Answer 5 See references at end of lecture
Sixth Annual Intensive Update in Neurology 0915-162016
47
Menierersquos syndrome
bull Fluctuating low frequency hearing loss
bull Tinnitus andor aural fullness
bull Vertigo lasting hours
Sixth Annual Intensive Update in Neurology 0915-162016
48
Normal
Sixth Annual Intensive Update in Neurology 0915-162016
49
Hydrops
Sixth Annual Intensive Update in Neurology 0915-162016
50
Menierersquos syndrome
ndash Diagnosis- Exclusion (RO acoustic)
ndash 23 of Menierersquos patients will improve spontaneously
ndash Treatment-1500 mgday low sodium triamterene375-
hydrochlorothiazide 25 vestibular suppressants (lorazepam
1 mg tablet sublingual Q 8 prn attacks) off label use
Sixth Annual Intensive Update in Neurology 0915-162016
51
Menierersquos syndrome ndash intratympanic steroids
ndash A newer treatment option for those failing medical management
ndash 90 response rate (Boleus-Aguirre MS Otology Neurotology
2008)
ndash Outcomes data confirm excellent clinical response in 3941
Menierersquos patients treated with intratympanic steroids in 2008
Sixth Annual Intensive Update in Neurology 0915-162016
52
Question 4
bull Menierersquos syndrome diagnosis is based on
1) Recurrent vertigo lasting at least 20 minutes
2) Aural fullness
3) Documented hearing loss
4) Other causes excluded
5) All of the above
Sixth Annual Intensive Update in Neurology 0915-162016
53
Question 4
bull Menierersquos syndrome diagnosis is based on
1) Recurrent vertigo lasting at least 20 minutes
2) Aural fullness
3) Documented hearing loss
4) Other causes excluded
5) All of the above
Answer 5 See references at end
Sixth Annual Intensive Update in Neurology 0915-162016
54
Superior Semicircular Canal Dehiscence (SSCD)
bull Sound andor pressure induced oscillopsia
bull CT reformatted in the plane of the superior canals
shows dehiscence of overlying bone
bull Low frequency ldquoconductive hyperacusisrdquo with elevated
bone thresholds normal reflexes and a conductive loss
Sixth Annual Intensive Update in Neurology 0915-162016
55
SSCD
bull Positive middle ear pressure or loud sound stimulates ampullofugal endolymph flow -canal activation
bull Eye movements are tonic upwards with ipsilateral intorsion
Sixth Annual Intensive Update in Neurology 0915-162016
56
SSCD
bull Valsalva against a closed glottis or jugular compression causes increased intracranial pressure and ampullopetal endolymph flow -canal inhibition
bull Eye movements are tonic downwards with ipsilateral extorsion
Sixth Annual Intensive Update in Neurology 0915-162016
57
Reconstructed left superior semicircular canal
Sixth Annual Intensive Update in Neurology 0915-162016
58
Sixth Annual Intensive Update in Neurology 0915-162016
59
SCHEMATIC DRAWING OF AN OTOLITHIC MACULA
Sixth Annual Intensive Update in Neurology 0915-162016
60
MACULAE OF THE UTRICLESACCULE
Sixth Annual Intensive Update in Neurology 0915-162016
61
HAIR CELLS
Sixth Annual Intensive Update in Neurology 0915-162016
62
SCANNING ELECTRON MICROGRAPH OF OTOCONIA
Sixth Annual Intensive Update in Neurology 0915-162016
63
Ocular VEMP
bull Response to loud clicks (80-110 dB) or forehead tap (Halmagyi and Curthoys 2003)
bull Contralateral oculomotor nucleus (CN III)
bull Inferior oblique muscle contraction (8 msec) detected by 5sec event triggered averaging
bull Noninverting electrode inferior to eye
bull Inverting below non-inverting electrode
bull Ground to forehead
bull Normal subjects have very low response
Sixth Annual Intensive Update in Neurology 0915-162016
64
Clinical applications
bull The o-VEMP is a sensitive test of otolithutricular
function
bull Complements vestibular test procedures that assess
semicircular canals
bull Abnormally robust responses are associated with third
window abnormalities such as semicircular canal
dehiscence
bull Serves as an excellent screening test for semicircular
canal dehiscence
Sixth Annual Intensive Update in Neurology 0915-162016
65
Question 5
bull Superior semicircular canal dehiscence
1) Causes increased ability to hear vibratory sounds
2) Improves hearing
3) Causes meningitis
4) Always needs surgical repair
Sixth Annual Intensive Update in Neurology 0915-162016
66
Question 5
bull Superior semicircular canal dehiscence
1) Causes increased ability to hear vibratory sounds
2) Improves hearing
3) Causes meningitis
4) Always needs surgical repair
Answer 1 References at end of lecture
Sixth Annual Intensive Update in Neurology 0915-162016
67
Enlarged vestibular aqueduct
bull Axial temporal bone CT showing right temporal bone with enlarged vestibular aqueduct (arrow)
bull Vestibular symptoms accompany progressive sensorineural hearing loss in 4-50 of patients in selected series
Sixth Annual Intensive Update in Neurology 0915-162016
68
Migraine
bull Family or personal history of migraine
bull 25 of migraine patients experience spontaneous vertigo (Baloh 1997)
bull Neuhauser criteria(2001) have been used to construct structured diagnostic interviews (Furman 2003)
bull Not always associated with headache
bull Motion sensitivity
bull Medication responsive
Sixth Annual Intensive Update in Neurology 0915-162016
69
ICHD-III beta Criteria for Vestibular Migraine
bull A At least five attacks fulfilling criteria C and D
bull B A current or past history of 11 migraine without aura or 12 migraine with aura
bull C Vestibular symptoms of moderate or severe intensity lasting between 5 minutes and 72 hours
bull D At least 50 associated with one of the following migrainous features
ndash 1 Headaches
ndash 2 Photophobia and phonophobia
ndash 3 Visual Aura
bull E Not better accounted for by another vestibular disorder
Sixth Annual Intensive Update in Neurology 0915-162016
70
Incidence of vertigo in MS
bull True vertigo (the perception of environmental
movement typically rotational) occurs in about
20 of MS patients and is the presenting
manifestation in 5
bull Up to 78 of MS patients report abnormalities
in balance at some point in their illness
Frohman et al Multiple Sclerosis 2003 9250
Sixth Annual Intensive Update in Neurology 0915-162016
71
Vertigo in MS due to demyelination
bull The most common neuroanatomic locations are the
root entry zone of CN VIII (giving absent calorics but
normal hearing and fixation suppression) and the
medial vestibular nucleus
bull Usually treated with steroids and vestibular
suppressants
Brandt 1993 Gass 1998 Thomke 1999
Sixth Annual Intensive Update in Neurology 0915-162016
72
Vertigo in MS
Frohmanrsquos large series (1153) reported that 52 of MS patients with acute vertigo had benign paroxysmal positional vertigo while 32 had new brainstem lesions
To avoid misdiagnoses and inappropriate treatment interventions alternative etiologies should be considered in all acute vertigo episodes in MS
E M Frohman Neurology 2000 and Multiple Sclerosis 2003
Sixth Annual Intensive Update in Neurology 0915-162016
73
Vestibular compensation
bull Acute unilateral or bilateral vestibular loss will prompt compensation thru central cervico-ocular visual and proprioceptive pathways
bull Unilateral loss may require several weeks for compensation to occur and activity especially head movements hastens compensation
bull Vestibular suppressant medication prolongs compensation
Sixth Annual Intensive Update in Neurology 0915-162016
74
Vestibular Rehabilitation
bull Performed by certified physical therapists many sites (Epic order vestibular PT) or check Vestibular Disorders of America website for providers
bull Assessment and treatment of benign positional vertigo
bull Directed rehab programs for central andor peripheral vestibular compensationndash Unilateral vestibulopathies
ndash Bilateral vestibular hypofunction
ndash Mutifactorial balance deficits and fall risk assessment
Sixth Annual Intensive Update in Neurology 0915-162016
75
Summary of whatrsquos new in peripheral vestibular disorders
bull Lateral semicircular canal benign paroxysmal positional vertigo presents with horizontal positioning nystagmus
bull Oral steroids appear to improve outcome in acute vestibular neuritis
bull Intratympanic steroids offer promise in Menierersquos syndrome
bull Superior semicircular canal dehiscence causes pressure and noise induced dizziness VEMP offers diagnostic information
Sixth Annual Intensive Update in Neurology 0915-162016
76
References
BPPVndash White J Benign paroxysmal positional vertigo Cleveland Clinic Journal of
Medicine 71(9)722-728 2004
ndash White J Coale K Catalano P Oas J Diagnosis and management of lateral semicircular canal benign positional vertigo Otolaryngology-Head and Neck Surgery 133 278-284 2005
ndash White J Savvides P Cherian N Oas J Canalith repositioning for benign paroxysmal positional vertigo Otology and Neurotology 26 704-710 2005
ndash White J Oas J Diagnosis and management of lateral semicircular canal conversions during particle repositioning therapy Laryngoscope 115(10) 1895-1897 2005
ndash Acute Vestibular Syndrome Hotson JR Baloh RW N Engl J Med 1998 Sep 3339(10)680-5
ndash Methylprednisolone valacyclovir or the combination for vestibular neuritis Strupp M Zingler VC Arbusow V Niklas D Maag KP Dieterich M Bense S Theil D Jahn K Brandt T N Engl J Med 2004 Jul 22351(4)354-61
ndash Intratympanic steroids for Meacuteniegraveres disease or syndrome Phillips JS Westerberg BCochrane Database Syst Rev 2011 Jul 6(7)CD008514 Review
ndash Sound- andor pressure-induced vertigo due to bone dehiscence of the superior semicircular canal Minor LB Solomon D Zinreich JS Zee DSArch Otolaryngol Head Neck Surg 1998 Mar124(3)249-58
Sixth Annual Intensive Update in Neurology 0915-162016
24
Lateral SCC-BPPV
bull Short latency horizontal nystagmus provoked by supine bilateral head turns with prolonged duration and poor fatigability
bull Uncommon (2-15 of total BPPV)
bull Two formsndash Geotropic ndash beating towards undermost ear
ndash Apogeotropic ndash beating away from undermost ear
Sixth Annual Intensive Update in Neurology 0915-162016
25
Geotropic Lateral Canal BPPV
bull Patient is positioned
first to the right lateral
position ndash nystagmus
beats right
bull Patient is then
positioned to the left
lateral position and
nystagmus reverses
and beats left
Sixth Annual Intensive Update in Neurology 0915-162016
26
Left Geotropic Lateral Semicircular Canal BPPV
Sixth Annual Intensive Update in Neurology 0915-162016
27
Repositioning Maneuvers for Geotropic Lateral Semicircular Canal (LSC) BPPV
bull Roll maneuver (Lempert Baloh)
ndash Patient rolls in 90 degree increments towards the good side
completely rolling over R LSC-BPPV repositioning shown
Sixth Annual Intensive Update in Neurology 0915-162016
28
Repositioning Maneuvers for Geotropic LSC BPPV
bull Gufoni maneuver
ndash Patient lies quickly onto good side and turns face-down to
floor for two minutes
Sixth Annual Intensive Update in Neurology 0915-162016
29
Left Apogeotropic LSC BPPV
Sixth Annual Intensive Update in Neurology 0915-162016
30
Nystagmus observed During Repositioning
bull Nystagmus should remain in the same torsional
direction as seen on DHT throughout the treatment of
posterior BPPV
bull Reversal of nystagmus direction suggests the particle
has fallen back into the canal and predicts failure
bull In Lateral BPPV nystagmus should beat towards the
good ear
Sixth Annual Intensive Update in Neurology 0915-162016
31
Posterior to Lateral canal conversion
Sixth Annual Intensive Update in Neurology 0915-162016
32
R Posterior to Lateral Canal conversion - geotropic
Sixth Annual Intensive Update in Neurology 0915-162016
33
BPPV Recurrence
bull Recurrence is common (15yr)
bull Otoconial adherence or mineralization demineralization
abnormalities may contribute to recurrence
bull Home canalith repositioning is effective (Radke 2004)
Sixth Annual Intensive Update in Neurology 0915-162016
34
Question 1
bull The most likely semicircular canal to be affected in
BPPV is
ndash 1) superior
ndash 2) lateral
ndash 3) posterior
ndash 4) combined
Sixth Annual Intensive Update in Neurology 0915-162016
35
Question 1
bull The most likely semicircular canal to be affected in
BPPV is
ndash 1) superior
ndash 2) lateral
ndash 3) posterior
ndash 4) combined
Answer 3 See references at end of lecture
Sixth Annual Intensive Update in Neurology 0915-162016
36
Acute Vestibular Syndrome
bull Rapid unilateral injury to either peripheral or central
vestibular structures produces prolonged vertigo
(days to weeks)
bull Severe vertigo nausea and vomiting spontaneous
nystagmus and postural instability
Sixth Annual Intensive Update in Neurology 0915-162016
37
Acute Vestibular Syndrome
bull 25 of patients with risk factors who present with isolated severe vertigo nystagmus and postural instability in the ER have an acute infarction of the inferior cerebellum (AICA PICA) Central imaging is suggested (Baloh 1998 NEJM)
ndash HTN diabetes smoking age gt65 Afib valvular heart disease occlusive vascular disease
Sixth Annual Intensive Update in Neurology 0915-162016
38
HINTS test
bull Head Impulse
bull Nystagmus
bull Test of Skew
ndash More sensitive than MRI in the diagnosis of acute stroke in
patients with acute vestibular syndrome with at least one risk
factor (Kattah J et al Stroke 2009)
Sixth Annual Intensive Update in Neurology 0915-162016
39
Head Impulse Test
bull No special equipment needed
bull Examiner stands in front of seated patient and
randomly moves head left and right Patient is asked to
focus on examinerrsquos nose
bull Rapid low amplitude thrust from lateral to straight
ahead causes a compensatory saccade when
performed towards the weak side
bull This finding is a permanent feature of unilateral
vestibular loss
Sixth Annual Intensive Update in Neurology 0915-162016
40
Head Impulse Test
Sixth Annual Intensive Update in Neurology 0915-162016
41
Head CT ndash Academy of Emergency Medicine 2013
bull The clinical value of head CT in isolated
dizzinessvertigo without any associated neurological
symptoms is very low
bull Less than 1 of head CTs ordered for dizziness and
vertigo have significant abnormalities and those
patients had severe headache and neurological deficits
in addition to dizziness
bull Forgoing unnecessary emergency head CTs could
save 360 million dollars annually
Sixth Annual Intensive Update in Neurology 0915-162016
42
Question 2
Head CT is not indicated for the evaluation of dizziness
and vertigo without additional neurological complaints because
1 It is too expensive
2 Less than 1 of CTs done for dizziness and vertigo are
abnormal and those patients had associated severe
neurological complaints
3 CT is the best imaging choice for the posterior fossa
4 Acoustic neuromas are well seen on CT
Sixth Annual Intensive Update in Neurology 0915-162016
43
Question 2
Head CT is not indicated for the evaluation of dizziness
and vertigo without additional neurological complaints because
1 It is too expensive
2 Less than 1 of CTs done for dizziness and vertigo are
abnormal and those patients had associated severe
neurological complaints
3 CT is the best imaging choice for the posterior fossa
4 Acoustic neuromas are well seen on CT
Answer 2 See references at end of lecture
Sixth Annual Intensive Update in Neurology 0915-162016
44
Acute Vestibular Syndrome
bull Usually peripheral and can be associated with
hearing loss (labyrinthitis)
bull Timely diagnosis improves recovery (ASAP
prednisone 1mgkgday for ten days and taper 10
mgday ndash Strupp M 2004 NEJM)
bull Avoid vestibular suppressants after 3 days and begin
vestibular rehabilitation to hasten compensation
Sixth Annual Intensive Update in Neurology 0915-162016
45
Question 3
bull Treatment for Acute Vestibular Syndrome
1) oral prednisone 1 mgkgday
2) Treat dehydration if necessary
3) Minimize vestibular suppressants after 3 days
4) Consider vestibular physical therapy
5) All of the above
Sixth Annual Intensive Update in Neurology 0915-162016
46
Question 3
bull Treatment for Acute Vestibular Syndrome
1) oral prednisone 1 mgkgday
2) Treat dehydration if necessary
3) Minimize vestibular suppressants after 3 days
4) Consider vestibular physical therapy
5) All of the above
Answer 5 See references at end of lecture
Sixth Annual Intensive Update in Neurology 0915-162016
47
Menierersquos syndrome
bull Fluctuating low frequency hearing loss
bull Tinnitus andor aural fullness
bull Vertigo lasting hours
Sixth Annual Intensive Update in Neurology 0915-162016
48
Normal
Sixth Annual Intensive Update in Neurology 0915-162016
49
Hydrops
Sixth Annual Intensive Update in Neurology 0915-162016
50
Menierersquos syndrome
ndash Diagnosis- Exclusion (RO acoustic)
ndash 23 of Menierersquos patients will improve spontaneously
ndash Treatment-1500 mgday low sodium triamterene375-
hydrochlorothiazide 25 vestibular suppressants (lorazepam
1 mg tablet sublingual Q 8 prn attacks) off label use
Sixth Annual Intensive Update in Neurology 0915-162016
51
Menierersquos syndrome ndash intratympanic steroids
ndash A newer treatment option for those failing medical management
ndash 90 response rate (Boleus-Aguirre MS Otology Neurotology
2008)
ndash Outcomes data confirm excellent clinical response in 3941
Menierersquos patients treated with intratympanic steroids in 2008
Sixth Annual Intensive Update in Neurology 0915-162016
52
Question 4
bull Menierersquos syndrome diagnosis is based on
1) Recurrent vertigo lasting at least 20 minutes
2) Aural fullness
3) Documented hearing loss
4) Other causes excluded
5) All of the above
Sixth Annual Intensive Update in Neurology 0915-162016
53
Question 4
bull Menierersquos syndrome diagnosis is based on
1) Recurrent vertigo lasting at least 20 minutes
2) Aural fullness
3) Documented hearing loss
4) Other causes excluded
5) All of the above
Answer 5 See references at end
Sixth Annual Intensive Update in Neurology 0915-162016
54
Superior Semicircular Canal Dehiscence (SSCD)
bull Sound andor pressure induced oscillopsia
bull CT reformatted in the plane of the superior canals
shows dehiscence of overlying bone
bull Low frequency ldquoconductive hyperacusisrdquo with elevated
bone thresholds normal reflexes and a conductive loss
Sixth Annual Intensive Update in Neurology 0915-162016
55
SSCD
bull Positive middle ear pressure or loud sound stimulates ampullofugal endolymph flow -canal activation
bull Eye movements are tonic upwards with ipsilateral intorsion
Sixth Annual Intensive Update in Neurology 0915-162016
56
SSCD
bull Valsalva against a closed glottis or jugular compression causes increased intracranial pressure and ampullopetal endolymph flow -canal inhibition
bull Eye movements are tonic downwards with ipsilateral extorsion
Sixth Annual Intensive Update in Neurology 0915-162016
57
Reconstructed left superior semicircular canal
Sixth Annual Intensive Update in Neurology 0915-162016
58
Sixth Annual Intensive Update in Neurology 0915-162016
59
SCHEMATIC DRAWING OF AN OTOLITHIC MACULA
Sixth Annual Intensive Update in Neurology 0915-162016
60
MACULAE OF THE UTRICLESACCULE
Sixth Annual Intensive Update in Neurology 0915-162016
61
HAIR CELLS
Sixth Annual Intensive Update in Neurology 0915-162016
62
SCANNING ELECTRON MICROGRAPH OF OTOCONIA
Sixth Annual Intensive Update in Neurology 0915-162016
63
Ocular VEMP
bull Response to loud clicks (80-110 dB) or forehead tap (Halmagyi and Curthoys 2003)
bull Contralateral oculomotor nucleus (CN III)
bull Inferior oblique muscle contraction (8 msec) detected by 5sec event triggered averaging
bull Noninverting electrode inferior to eye
bull Inverting below non-inverting electrode
bull Ground to forehead
bull Normal subjects have very low response
Sixth Annual Intensive Update in Neurology 0915-162016
64
Clinical applications
bull The o-VEMP is a sensitive test of otolithutricular
function
bull Complements vestibular test procedures that assess
semicircular canals
bull Abnormally robust responses are associated with third
window abnormalities such as semicircular canal
dehiscence
bull Serves as an excellent screening test for semicircular
canal dehiscence
Sixth Annual Intensive Update in Neurology 0915-162016
65
Question 5
bull Superior semicircular canal dehiscence
1) Causes increased ability to hear vibratory sounds
2) Improves hearing
3) Causes meningitis
4) Always needs surgical repair
Sixth Annual Intensive Update in Neurology 0915-162016
66
Question 5
bull Superior semicircular canal dehiscence
1) Causes increased ability to hear vibratory sounds
2) Improves hearing
3) Causes meningitis
4) Always needs surgical repair
Answer 1 References at end of lecture
Sixth Annual Intensive Update in Neurology 0915-162016
67
Enlarged vestibular aqueduct
bull Axial temporal bone CT showing right temporal bone with enlarged vestibular aqueduct (arrow)
bull Vestibular symptoms accompany progressive sensorineural hearing loss in 4-50 of patients in selected series
Sixth Annual Intensive Update in Neurology 0915-162016
68
Migraine
bull Family or personal history of migraine
bull 25 of migraine patients experience spontaneous vertigo (Baloh 1997)
bull Neuhauser criteria(2001) have been used to construct structured diagnostic interviews (Furman 2003)
bull Not always associated with headache
bull Motion sensitivity
bull Medication responsive
Sixth Annual Intensive Update in Neurology 0915-162016
69
ICHD-III beta Criteria for Vestibular Migraine
bull A At least five attacks fulfilling criteria C and D
bull B A current or past history of 11 migraine without aura or 12 migraine with aura
bull C Vestibular symptoms of moderate or severe intensity lasting between 5 minutes and 72 hours
bull D At least 50 associated with one of the following migrainous features
ndash 1 Headaches
ndash 2 Photophobia and phonophobia
ndash 3 Visual Aura
bull E Not better accounted for by another vestibular disorder
Sixth Annual Intensive Update in Neurology 0915-162016
70
Incidence of vertigo in MS
bull True vertigo (the perception of environmental
movement typically rotational) occurs in about
20 of MS patients and is the presenting
manifestation in 5
bull Up to 78 of MS patients report abnormalities
in balance at some point in their illness
Frohman et al Multiple Sclerosis 2003 9250
Sixth Annual Intensive Update in Neurology 0915-162016
71
Vertigo in MS due to demyelination
bull The most common neuroanatomic locations are the
root entry zone of CN VIII (giving absent calorics but
normal hearing and fixation suppression) and the
medial vestibular nucleus
bull Usually treated with steroids and vestibular
suppressants
Brandt 1993 Gass 1998 Thomke 1999
Sixth Annual Intensive Update in Neurology 0915-162016
72
Vertigo in MS
Frohmanrsquos large series (1153) reported that 52 of MS patients with acute vertigo had benign paroxysmal positional vertigo while 32 had new brainstem lesions
To avoid misdiagnoses and inappropriate treatment interventions alternative etiologies should be considered in all acute vertigo episodes in MS
E M Frohman Neurology 2000 and Multiple Sclerosis 2003
Sixth Annual Intensive Update in Neurology 0915-162016
73
Vestibular compensation
bull Acute unilateral or bilateral vestibular loss will prompt compensation thru central cervico-ocular visual and proprioceptive pathways
bull Unilateral loss may require several weeks for compensation to occur and activity especially head movements hastens compensation
bull Vestibular suppressant medication prolongs compensation
Sixth Annual Intensive Update in Neurology 0915-162016
74
Vestibular Rehabilitation
bull Performed by certified physical therapists many sites (Epic order vestibular PT) or check Vestibular Disorders of America website for providers
bull Assessment and treatment of benign positional vertigo
bull Directed rehab programs for central andor peripheral vestibular compensationndash Unilateral vestibulopathies
ndash Bilateral vestibular hypofunction
ndash Mutifactorial balance deficits and fall risk assessment
Sixth Annual Intensive Update in Neurology 0915-162016
75
Summary of whatrsquos new in peripheral vestibular disorders
bull Lateral semicircular canal benign paroxysmal positional vertigo presents with horizontal positioning nystagmus
bull Oral steroids appear to improve outcome in acute vestibular neuritis
bull Intratympanic steroids offer promise in Menierersquos syndrome
bull Superior semicircular canal dehiscence causes pressure and noise induced dizziness VEMP offers diagnostic information
Sixth Annual Intensive Update in Neurology 0915-162016
76
References
BPPVndash White J Benign paroxysmal positional vertigo Cleveland Clinic Journal of
Medicine 71(9)722-728 2004
ndash White J Coale K Catalano P Oas J Diagnosis and management of lateral semicircular canal benign positional vertigo Otolaryngology-Head and Neck Surgery 133 278-284 2005
ndash White J Savvides P Cherian N Oas J Canalith repositioning for benign paroxysmal positional vertigo Otology and Neurotology 26 704-710 2005
ndash White J Oas J Diagnosis and management of lateral semicircular canal conversions during particle repositioning therapy Laryngoscope 115(10) 1895-1897 2005
ndash Acute Vestibular Syndrome Hotson JR Baloh RW N Engl J Med 1998 Sep 3339(10)680-5
ndash Methylprednisolone valacyclovir or the combination for vestibular neuritis Strupp M Zingler VC Arbusow V Niklas D Maag KP Dieterich M Bense S Theil D Jahn K Brandt T N Engl J Med 2004 Jul 22351(4)354-61
ndash Intratympanic steroids for Meacuteniegraveres disease or syndrome Phillips JS Westerberg BCochrane Database Syst Rev 2011 Jul 6(7)CD008514 Review
ndash Sound- andor pressure-induced vertigo due to bone dehiscence of the superior semicircular canal Minor LB Solomon D Zinreich JS Zee DSArch Otolaryngol Head Neck Surg 1998 Mar124(3)249-58
Sixth Annual Intensive Update in Neurology 0915-162016
25
Geotropic Lateral Canal BPPV
bull Patient is positioned
first to the right lateral
position ndash nystagmus
beats right
bull Patient is then
positioned to the left
lateral position and
nystagmus reverses
and beats left
Sixth Annual Intensive Update in Neurology 0915-162016
26
Left Geotropic Lateral Semicircular Canal BPPV
Sixth Annual Intensive Update in Neurology 0915-162016
27
Repositioning Maneuvers for Geotropic Lateral Semicircular Canal (LSC) BPPV
bull Roll maneuver (Lempert Baloh)
ndash Patient rolls in 90 degree increments towards the good side
completely rolling over R LSC-BPPV repositioning shown
Sixth Annual Intensive Update in Neurology 0915-162016
28
Repositioning Maneuvers for Geotropic LSC BPPV
bull Gufoni maneuver
ndash Patient lies quickly onto good side and turns face-down to
floor for two minutes
Sixth Annual Intensive Update in Neurology 0915-162016
29
Left Apogeotropic LSC BPPV
Sixth Annual Intensive Update in Neurology 0915-162016
30
Nystagmus observed During Repositioning
bull Nystagmus should remain in the same torsional
direction as seen on DHT throughout the treatment of
posterior BPPV
bull Reversal of nystagmus direction suggests the particle
has fallen back into the canal and predicts failure
bull In Lateral BPPV nystagmus should beat towards the
good ear
Sixth Annual Intensive Update in Neurology 0915-162016
31
Posterior to Lateral canal conversion
Sixth Annual Intensive Update in Neurology 0915-162016
32
R Posterior to Lateral Canal conversion - geotropic
Sixth Annual Intensive Update in Neurology 0915-162016
33
BPPV Recurrence
bull Recurrence is common (15yr)
bull Otoconial adherence or mineralization demineralization
abnormalities may contribute to recurrence
bull Home canalith repositioning is effective (Radke 2004)
Sixth Annual Intensive Update in Neurology 0915-162016
34
Question 1
bull The most likely semicircular canal to be affected in
BPPV is
ndash 1) superior
ndash 2) lateral
ndash 3) posterior
ndash 4) combined
Sixth Annual Intensive Update in Neurology 0915-162016
35
Question 1
bull The most likely semicircular canal to be affected in
BPPV is
ndash 1) superior
ndash 2) lateral
ndash 3) posterior
ndash 4) combined
Answer 3 See references at end of lecture
Sixth Annual Intensive Update in Neurology 0915-162016
36
Acute Vestibular Syndrome
bull Rapid unilateral injury to either peripheral or central
vestibular structures produces prolonged vertigo
(days to weeks)
bull Severe vertigo nausea and vomiting spontaneous
nystagmus and postural instability
Sixth Annual Intensive Update in Neurology 0915-162016
37
Acute Vestibular Syndrome
bull 25 of patients with risk factors who present with isolated severe vertigo nystagmus and postural instability in the ER have an acute infarction of the inferior cerebellum (AICA PICA) Central imaging is suggested (Baloh 1998 NEJM)
ndash HTN diabetes smoking age gt65 Afib valvular heart disease occlusive vascular disease
Sixth Annual Intensive Update in Neurology 0915-162016
38
HINTS test
bull Head Impulse
bull Nystagmus
bull Test of Skew
ndash More sensitive than MRI in the diagnosis of acute stroke in
patients with acute vestibular syndrome with at least one risk
factor (Kattah J et al Stroke 2009)
Sixth Annual Intensive Update in Neurology 0915-162016
39
Head Impulse Test
bull No special equipment needed
bull Examiner stands in front of seated patient and
randomly moves head left and right Patient is asked to
focus on examinerrsquos nose
bull Rapid low amplitude thrust from lateral to straight
ahead causes a compensatory saccade when
performed towards the weak side
bull This finding is a permanent feature of unilateral
vestibular loss
Sixth Annual Intensive Update in Neurology 0915-162016
40
Head Impulse Test
Sixth Annual Intensive Update in Neurology 0915-162016
41
Head CT ndash Academy of Emergency Medicine 2013
bull The clinical value of head CT in isolated
dizzinessvertigo without any associated neurological
symptoms is very low
bull Less than 1 of head CTs ordered for dizziness and
vertigo have significant abnormalities and those
patients had severe headache and neurological deficits
in addition to dizziness
bull Forgoing unnecessary emergency head CTs could
save 360 million dollars annually
Sixth Annual Intensive Update in Neurology 0915-162016
42
Question 2
Head CT is not indicated for the evaluation of dizziness
and vertigo without additional neurological complaints because
1 It is too expensive
2 Less than 1 of CTs done for dizziness and vertigo are
abnormal and those patients had associated severe
neurological complaints
3 CT is the best imaging choice for the posterior fossa
4 Acoustic neuromas are well seen on CT
Sixth Annual Intensive Update in Neurology 0915-162016
43
Question 2
Head CT is not indicated for the evaluation of dizziness
and vertigo without additional neurological complaints because
1 It is too expensive
2 Less than 1 of CTs done for dizziness and vertigo are
abnormal and those patients had associated severe
neurological complaints
3 CT is the best imaging choice for the posterior fossa
4 Acoustic neuromas are well seen on CT
Answer 2 See references at end of lecture
Sixth Annual Intensive Update in Neurology 0915-162016
44
Acute Vestibular Syndrome
bull Usually peripheral and can be associated with
hearing loss (labyrinthitis)
bull Timely diagnosis improves recovery (ASAP
prednisone 1mgkgday for ten days and taper 10
mgday ndash Strupp M 2004 NEJM)
bull Avoid vestibular suppressants after 3 days and begin
vestibular rehabilitation to hasten compensation
Sixth Annual Intensive Update in Neurology 0915-162016
45
Question 3
bull Treatment for Acute Vestibular Syndrome
1) oral prednisone 1 mgkgday
2) Treat dehydration if necessary
3) Minimize vestibular suppressants after 3 days
4) Consider vestibular physical therapy
5) All of the above
Sixth Annual Intensive Update in Neurology 0915-162016
46
Question 3
bull Treatment for Acute Vestibular Syndrome
1) oral prednisone 1 mgkgday
2) Treat dehydration if necessary
3) Minimize vestibular suppressants after 3 days
4) Consider vestibular physical therapy
5) All of the above
Answer 5 See references at end of lecture
Sixth Annual Intensive Update in Neurology 0915-162016
47
Menierersquos syndrome
bull Fluctuating low frequency hearing loss
bull Tinnitus andor aural fullness
bull Vertigo lasting hours
Sixth Annual Intensive Update in Neurology 0915-162016
48
Normal
Sixth Annual Intensive Update in Neurology 0915-162016
49
Hydrops
Sixth Annual Intensive Update in Neurology 0915-162016
50
Menierersquos syndrome
ndash Diagnosis- Exclusion (RO acoustic)
ndash 23 of Menierersquos patients will improve spontaneously
ndash Treatment-1500 mgday low sodium triamterene375-
hydrochlorothiazide 25 vestibular suppressants (lorazepam
1 mg tablet sublingual Q 8 prn attacks) off label use
Sixth Annual Intensive Update in Neurology 0915-162016
51
Menierersquos syndrome ndash intratympanic steroids
ndash A newer treatment option for those failing medical management
ndash 90 response rate (Boleus-Aguirre MS Otology Neurotology
2008)
ndash Outcomes data confirm excellent clinical response in 3941
Menierersquos patients treated with intratympanic steroids in 2008
Sixth Annual Intensive Update in Neurology 0915-162016
52
Question 4
bull Menierersquos syndrome diagnosis is based on
1) Recurrent vertigo lasting at least 20 minutes
2) Aural fullness
3) Documented hearing loss
4) Other causes excluded
5) All of the above
Sixth Annual Intensive Update in Neurology 0915-162016
53
Question 4
bull Menierersquos syndrome diagnosis is based on
1) Recurrent vertigo lasting at least 20 minutes
2) Aural fullness
3) Documented hearing loss
4) Other causes excluded
5) All of the above
Answer 5 See references at end
Sixth Annual Intensive Update in Neurology 0915-162016
54
Superior Semicircular Canal Dehiscence (SSCD)
bull Sound andor pressure induced oscillopsia
bull CT reformatted in the plane of the superior canals
shows dehiscence of overlying bone
bull Low frequency ldquoconductive hyperacusisrdquo with elevated
bone thresholds normal reflexes and a conductive loss
Sixth Annual Intensive Update in Neurology 0915-162016
55
SSCD
bull Positive middle ear pressure or loud sound stimulates ampullofugal endolymph flow -canal activation
bull Eye movements are tonic upwards with ipsilateral intorsion
Sixth Annual Intensive Update in Neurology 0915-162016
56
SSCD
bull Valsalva against a closed glottis or jugular compression causes increased intracranial pressure and ampullopetal endolymph flow -canal inhibition
bull Eye movements are tonic downwards with ipsilateral extorsion
Sixth Annual Intensive Update in Neurology 0915-162016
57
Reconstructed left superior semicircular canal
Sixth Annual Intensive Update in Neurology 0915-162016
58
Sixth Annual Intensive Update in Neurology 0915-162016
59
SCHEMATIC DRAWING OF AN OTOLITHIC MACULA
Sixth Annual Intensive Update in Neurology 0915-162016
60
MACULAE OF THE UTRICLESACCULE
Sixth Annual Intensive Update in Neurology 0915-162016
61
HAIR CELLS
Sixth Annual Intensive Update in Neurology 0915-162016
62
SCANNING ELECTRON MICROGRAPH OF OTOCONIA
Sixth Annual Intensive Update in Neurology 0915-162016
63
Ocular VEMP
bull Response to loud clicks (80-110 dB) or forehead tap (Halmagyi and Curthoys 2003)
bull Contralateral oculomotor nucleus (CN III)
bull Inferior oblique muscle contraction (8 msec) detected by 5sec event triggered averaging
bull Noninverting electrode inferior to eye
bull Inverting below non-inverting electrode
bull Ground to forehead
bull Normal subjects have very low response
Sixth Annual Intensive Update in Neurology 0915-162016
64
Clinical applications
bull The o-VEMP is a sensitive test of otolithutricular
function
bull Complements vestibular test procedures that assess
semicircular canals
bull Abnormally robust responses are associated with third
window abnormalities such as semicircular canal
dehiscence
bull Serves as an excellent screening test for semicircular
canal dehiscence
Sixth Annual Intensive Update in Neurology 0915-162016
65
Question 5
bull Superior semicircular canal dehiscence
1) Causes increased ability to hear vibratory sounds
2) Improves hearing
3) Causes meningitis
4) Always needs surgical repair
Sixth Annual Intensive Update in Neurology 0915-162016
66
Question 5
bull Superior semicircular canal dehiscence
1) Causes increased ability to hear vibratory sounds
2) Improves hearing
3) Causes meningitis
4) Always needs surgical repair
Answer 1 References at end of lecture
Sixth Annual Intensive Update in Neurology 0915-162016
67
Enlarged vestibular aqueduct
bull Axial temporal bone CT showing right temporal bone with enlarged vestibular aqueduct (arrow)
bull Vestibular symptoms accompany progressive sensorineural hearing loss in 4-50 of patients in selected series
Sixth Annual Intensive Update in Neurology 0915-162016
68
Migraine
bull Family or personal history of migraine
bull 25 of migraine patients experience spontaneous vertigo (Baloh 1997)
bull Neuhauser criteria(2001) have been used to construct structured diagnostic interviews (Furman 2003)
bull Not always associated with headache
bull Motion sensitivity
bull Medication responsive
Sixth Annual Intensive Update in Neurology 0915-162016
69
ICHD-III beta Criteria for Vestibular Migraine
bull A At least five attacks fulfilling criteria C and D
bull B A current or past history of 11 migraine without aura or 12 migraine with aura
bull C Vestibular symptoms of moderate or severe intensity lasting between 5 minutes and 72 hours
bull D At least 50 associated with one of the following migrainous features
ndash 1 Headaches
ndash 2 Photophobia and phonophobia
ndash 3 Visual Aura
bull E Not better accounted for by another vestibular disorder
Sixth Annual Intensive Update in Neurology 0915-162016
70
Incidence of vertigo in MS
bull True vertigo (the perception of environmental
movement typically rotational) occurs in about
20 of MS patients and is the presenting
manifestation in 5
bull Up to 78 of MS patients report abnormalities
in balance at some point in their illness
Frohman et al Multiple Sclerosis 2003 9250
Sixth Annual Intensive Update in Neurology 0915-162016
71
Vertigo in MS due to demyelination
bull The most common neuroanatomic locations are the
root entry zone of CN VIII (giving absent calorics but
normal hearing and fixation suppression) and the
medial vestibular nucleus
bull Usually treated with steroids and vestibular
suppressants
Brandt 1993 Gass 1998 Thomke 1999
Sixth Annual Intensive Update in Neurology 0915-162016
72
Vertigo in MS
Frohmanrsquos large series (1153) reported that 52 of MS patients with acute vertigo had benign paroxysmal positional vertigo while 32 had new brainstem lesions
To avoid misdiagnoses and inappropriate treatment interventions alternative etiologies should be considered in all acute vertigo episodes in MS
E M Frohman Neurology 2000 and Multiple Sclerosis 2003
Sixth Annual Intensive Update in Neurology 0915-162016
73
Vestibular compensation
bull Acute unilateral or bilateral vestibular loss will prompt compensation thru central cervico-ocular visual and proprioceptive pathways
bull Unilateral loss may require several weeks for compensation to occur and activity especially head movements hastens compensation
bull Vestibular suppressant medication prolongs compensation
Sixth Annual Intensive Update in Neurology 0915-162016
74
Vestibular Rehabilitation
bull Performed by certified physical therapists many sites (Epic order vestibular PT) or check Vestibular Disorders of America website for providers
bull Assessment and treatment of benign positional vertigo
bull Directed rehab programs for central andor peripheral vestibular compensationndash Unilateral vestibulopathies
ndash Bilateral vestibular hypofunction
ndash Mutifactorial balance deficits and fall risk assessment
Sixth Annual Intensive Update in Neurology 0915-162016
75
Summary of whatrsquos new in peripheral vestibular disorders
bull Lateral semicircular canal benign paroxysmal positional vertigo presents with horizontal positioning nystagmus
bull Oral steroids appear to improve outcome in acute vestibular neuritis
bull Intratympanic steroids offer promise in Menierersquos syndrome
bull Superior semicircular canal dehiscence causes pressure and noise induced dizziness VEMP offers diagnostic information
Sixth Annual Intensive Update in Neurology 0915-162016
76
References
BPPVndash White J Benign paroxysmal positional vertigo Cleveland Clinic Journal of
Medicine 71(9)722-728 2004
ndash White J Coale K Catalano P Oas J Diagnosis and management of lateral semicircular canal benign positional vertigo Otolaryngology-Head and Neck Surgery 133 278-284 2005
ndash White J Savvides P Cherian N Oas J Canalith repositioning for benign paroxysmal positional vertigo Otology and Neurotology 26 704-710 2005
ndash White J Oas J Diagnosis and management of lateral semicircular canal conversions during particle repositioning therapy Laryngoscope 115(10) 1895-1897 2005
ndash Acute Vestibular Syndrome Hotson JR Baloh RW N Engl J Med 1998 Sep 3339(10)680-5
ndash Methylprednisolone valacyclovir or the combination for vestibular neuritis Strupp M Zingler VC Arbusow V Niklas D Maag KP Dieterich M Bense S Theil D Jahn K Brandt T N Engl J Med 2004 Jul 22351(4)354-61
ndash Intratympanic steroids for Meacuteniegraveres disease or syndrome Phillips JS Westerberg BCochrane Database Syst Rev 2011 Jul 6(7)CD008514 Review
ndash Sound- andor pressure-induced vertigo due to bone dehiscence of the superior semicircular canal Minor LB Solomon D Zinreich JS Zee DSArch Otolaryngol Head Neck Surg 1998 Mar124(3)249-58
Sixth Annual Intensive Update in Neurology 0915-162016
26
Left Geotropic Lateral Semicircular Canal BPPV
Sixth Annual Intensive Update in Neurology 0915-162016
27
Repositioning Maneuvers for Geotropic Lateral Semicircular Canal (LSC) BPPV
bull Roll maneuver (Lempert Baloh)
ndash Patient rolls in 90 degree increments towards the good side
completely rolling over R LSC-BPPV repositioning shown
Sixth Annual Intensive Update in Neurology 0915-162016
28
Repositioning Maneuvers for Geotropic LSC BPPV
bull Gufoni maneuver
ndash Patient lies quickly onto good side and turns face-down to
floor for two minutes
Sixth Annual Intensive Update in Neurology 0915-162016
29
Left Apogeotropic LSC BPPV
Sixth Annual Intensive Update in Neurology 0915-162016
30
Nystagmus observed During Repositioning
bull Nystagmus should remain in the same torsional
direction as seen on DHT throughout the treatment of
posterior BPPV
bull Reversal of nystagmus direction suggests the particle
has fallen back into the canal and predicts failure
bull In Lateral BPPV nystagmus should beat towards the
good ear
Sixth Annual Intensive Update in Neurology 0915-162016
31
Posterior to Lateral canal conversion
Sixth Annual Intensive Update in Neurology 0915-162016
32
R Posterior to Lateral Canal conversion - geotropic
Sixth Annual Intensive Update in Neurology 0915-162016
33
BPPV Recurrence
bull Recurrence is common (15yr)
bull Otoconial adherence or mineralization demineralization
abnormalities may contribute to recurrence
bull Home canalith repositioning is effective (Radke 2004)
Sixth Annual Intensive Update in Neurology 0915-162016
34
Question 1
bull The most likely semicircular canal to be affected in
BPPV is
ndash 1) superior
ndash 2) lateral
ndash 3) posterior
ndash 4) combined
Sixth Annual Intensive Update in Neurology 0915-162016
35
Question 1
bull The most likely semicircular canal to be affected in
BPPV is
ndash 1) superior
ndash 2) lateral
ndash 3) posterior
ndash 4) combined
Answer 3 See references at end of lecture
Sixth Annual Intensive Update in Neurology 0915-162016
36
Acute Vestibular Syndrome
bull Rapid unilateral injury to either peripheral or central
vestibular structures produces prolonged vertigo
(days to weeks)
bull Severe vertigo nausea and vomiting spontaneous
nystagmus and postural instability
Sixth Annual Intensive Update in Neurology 0915-162016
37
Acute Vestibular Syndrome
bull 25 of patients with risk factors who present with isolated severe vertigo nystagmus and postural instability in the ER have an acute infarction of the inferior cerebellum (AICA PICA) Central imaging is suggested (Baloh 1998 NEJM)
ndash HTN diabetes smoking age gt65 Afib valvular heart disease occlusive vascular disease
Sixth Annual Intensive Update in Neurology 0915-162016
38
HINTS test
bull Head Impulse
bull Nystagmus
bull Test of Skew
ndash More sensitive than MRI in the diagnosis of acute stroke in
patients with acute vestibular syndrome with at least one risk
factor (Kattah J et al Stroke 2009)
Sixth Annual Intensive Update in Neurology 0915-162016
39
Head Impulse Test
bull No special equipment needed
bull Examiner stands in front of seated patient and
randomly moves head left and right Patient is asked to
focus on examinerrsquos nose
bull Rapid low amplitude thrust from lateral to straight
ahead causes a compensatory saccade when
performed towards the weak side
bull This finding is a permanent feature of unilateral
vestibular loss
Sixth Annual Intensive Update in Neurology 0915-162016
40
Head Impulse Test
Sixth Annual Intensive Update in Neurology 0915-162016
41
Head CT ndash Academy of Emergency Medicine 2013
bull The clinical value of head CT in isolated
dizzinessvertigo without any associated neurological
symptoms is very low
bull Less than 1 of head CTs ordered for dizziness and
vertigo have significant abnormalities and those
patients had severe headache and neurological deficits
in addition to dizziness
bull Forgoing unnecessary emergency head CTs could
save 360 million dollars annually
Sixth Annual Intensive Update in Neurology 0915-162016
42
Question 2
Head CT is not indicated for the evaluation of dizziness
and vertigo without additional neurological complaints because
1 It is too expensive
2 Less than 1 of CTs done for dizziness and vertigo are
abnormal and those patients had associated severe
neurological complaints
3 CT is the best imaging choice for the posterior fossa
4 Acoustic neuromas are well seen on CT
Sixth Annual Intensive Update in Neurology 0915-162016
43
Question 2
Head CT is not indicated for the evaluation of dizziness
and vertigo without additional neurological complaints because
1 It is too expensive
2 Less than 1 of CTs done for dizziness and vertigo are
abnormal and those patients had associated severe
neurological complaints
3 CT is the best imaging choice for the posterior fossa
4 Acoustic neuromas are well seen on CT
Answer 2 See references at end of lecture
Sixth Annual Intensive Update in Neurology 0915-162016
44
Acute Vestibular Syndrome
bull Usually peripheral and can be associated with
hearing loss (labyrinthitis)
bull Timely diagnosis improves recovery (ASAP
prednisone 1mgkgday for ten days and taper 10
mgday ndash Strupp M 2004 NEJM)
bull Avoid vestibular suppressants after 3 days and begin
vestibular rehabilitation to hasten compensation
Sixth Annual Intensive Update in Neurology 0915-162016
45
Question 3
bull Treatment for Acute Vestibular Syndrome
1) oral prednisone 1 mgkgday
2) Treat dehydration if necessary
3) Minimize vestibular suppressants after 3 days
4) Consider vestibular physical therapy
5) All of the above
Sixth Annual Intensive Update in Neurology 0915-162016
46
Question 3
bull Treatment for Acute Vestibular Syndrome
1) oral prednisone 1 mgkgday
2) Treat dehydration if necessary
3) Minimize vestibular suppressants after 3 days
4) Consider vestibular physical therapy
5) All of the above
Answer 5 See references at end of lecture
Sixth Annual Intensive Update in Neurology 0915-162016
47
Menierersquos syndrome
bull Fluctuating low frequency hearing loss
bull Tinnitus andor aural fullness
bull Vertigo lasting hours
Sixth Annual Intensive Update in Neurology 0915-162016
48
Normal
Sixth Annual Intensive Update in Neurology 0915-162016
49
Hydrops
Sixth Annual Intensive Update in Neurology 0915-162016
50
Menierersquos syndrome
ndash Diagnosis- Exclusion (RO acoustic)
ndash 23 of Menierersquos patients will improve spontaneously
ndash Treatment-1500 mgday low sodium triamterene375-
hydrochlorothiazide 25 vestibular suppressants (lorazepam
1 mg tablet sublingual Q 8 prn attacks) off label use
Sixth Annual Intensive Update in Neurology 0915-162016
51
Menierersquos syndrome ndash intratympanic steroids
ndash A newer treatment option for those failing medical management
ndash 90 response rate (Boleus-Aguirre MS Otology Neurotology
2008)
ndash Outcomes data confirm excellent clinical response in 3941
Menierersquos patients treated with intratympanic steroids in 2008
Sixth Annual Intensive Update in Neurology 0915-162016
52
Question 4
bull Menierersquos syndrome diagnosis is based on
1) Recurrent vertigo lasting at least 20 minutes
2) Aural fullness
3) Documented hearing loss
4) Other causes excluded
5) All of the above
Sixth Annual Intensive Update in Neurology 0915-162016
53
Question 4
bull Menierersquos syndrome diagnosis is based on
1) Recurrent vertigo lasting at least 20 minutes
2) Aural fullness
3) Documented hearing loss
4) Other causes excluded
5) All of the above
Answer 5 See references at end
Sixth Annual Intensive Update in Neurology 0915-162016
54
Superior Semicircular Canal Dehiscence (SSCD)
bull Sound andor pressure induced oscillopsia
bull CT reformatted in the plane of the superior canals
shows dehiscence of overlying bone
bull Low frequency ldquoconductive hyperacusisrdquo with elevated
bone thresholds normal reflexes and a conductive loss
Sixth Annual Intensive Update in Neurology 0915-162016
55
SSCD
bull Positive middle ear pressure or loud sound stimulates ampullofugal endolymph flow -canal activation
bull Eye movements are tonic upwards with ipsilateral intorsion
Sixth Annual Intensive Update in Neurology 0915-162016
56
SSCD
bull Valsalva against a closed glottis or jugular compression causes increased intracranial pressure and ampullopetal endolymph flow -canal inhibition
bull Eye movements are tonic downwards with ipsilateral extorsion
Sixth Annual Intensive Update in Neurology 0915-162016
57
Reconstructed left superior semicircular canal
Sixth Annual Intensive Update in Neurology 0915-162016
58
Sixth Annual Intensive Update in Neurology 0915-162016
59
SCHEMATIC DRAWING OF AN OTOLITHIC MACULA
Sixth Annual Intensive Update in Neurology 0915-162016
60
MACULAE OF THE UTRICLESACCULE
Sixth Annual Intensive Update in Neurology 0915-162016
61
HAIR CELLS
Sixth Annual Intensive Update in Neurology 0915-162016
62
SCANNING ELECTRON MICROGRAPH OF OTOCONIA
Sixth Annual Intensive Update in Neurology 0915-162016
63
Ocular VEMP
bull Response to loud clicks (80-110 dB) or forehead tap (Halmagyi and Curthoys 2003)
bull Contralateral oculomotor nucleus (CN III)
bull Inferior oblique muscle contraction (8 msec) detected by 5sec event triggered averaging
bull Noninverting electrode inferior to eye
bull Inverting below non-inverting electrode
bull Ground to forehead
bull Normal subjects have very low response
Sixth Annual Intensive Update in Neurology 0915-162016
64
Clinical applications
bull The o-VEMP is a sensitive test of otolithutricular
function
bull Complements vestibular test procedures that assess
semicircular canals
bull Abnormally robust responses are associated with third
window abnormalities such as semicircular canal
dehiscence
bull Serves as an excellent screening test for semicircular
canal dehiscence
Sixth Annual Intensive Update in Neurology 0915-162016
65
Question 5
bull Superior semicircular canal dehiscence
1) Causes increased ability to hear vibratory sounds
2) Improves hearing
3) Causes meningitis
4) Always needs surgical repair
Sixth Annual Intensive Update in Neurology 0915-162016
66
Question 5
bull Superior semicircular canal dehiscence
1) Causes increased ability to hear vibratory sounds
2) Improves hearing
3) Causes meningitis
4) Always needs surgical repair
Answer 1 References at end of lecture
Sixth Annual Intensive Update in Neurology 0915-162016
67
Enlarged vestibular aqueduct
bull Axial temporal bone CT showing right temporal bone with enlarged vestibular aqueduct (arrow)
bull Vestibular symptoms accompany progressive sensorineural hearing loss in 4-50 of patients in selected series
Sixth Annual Intensive Update in Neurology 0915-162016
68
Migraine
bull Family or personal history of migraine
bull 25 of migraine patients experience spontaneous vertigo (Baloh 1997)
bull Neuhauser criteria(2001) have been used to construct structured diagnostic interviews (Furman 2003)
bull Not always associated with headache
bull Motion sensitivity
bull Medication responsive
Sixth Annual Intensive Update in Neurology 0915-162016
69
ICHD-III beta Criteria for Vestibular Migraine
bull A At least five attacks fulfilling criteria C and D
bull B A current or past history of 11 migraine without aura or 12 migraine with aura
bull C Vestibular symptoms of moderate or severe intensity lasting between 5 minutes and 72 hours
bull D At least 50 associated with one of the following migrainous features
ndash 1 Headaches
ndash 2 Photophobia and phonophobia
ndash 3 Visual Aura
bull E Not better accounted for by another vestibular disorder
Sixth Annual Intensive Update in Neurology 0915-162016
70
Incidence of vertigo in MS
bull True vertigo (the perception of environmental
movement typically rotational) occurs in about
20 of MS patients and is the presenting
manifestation in 5
bull Up to 78 of MS patients report abnormalities
in balance at some point in their illness
Frohman et al Multiple Sclerosis 2003 9250
Sixth Annual Intensive Update in Neurology 0915-162016
71
Vertigo in MS due to demyelination
bull The most common neuroanatomic locations are the
root entry zone of CN VIII (giving absent calorics but
normal hearing and fixation suppression) and the
medial vestibular nucleus
bull Usually treated with steroids and vestibular
suppressants
Brandt 1993 Gass 1998 Thomke 1999
Sixth Annual Intensive Update in Neurology 0915-162016
72
Vertigo in MS
Frohmanrsquos large series (1153) reported that 52 of MS patients with acute vertigo had benign paroxysmal positional vertigo while 32 had new brainstem lesions
To avoid misdiagnoses and inappropriate treatment interventions alternative etiologies should be considered in all acute vertigo episodes in MS
E M Frohman Neurology 2000 and Multiple Sclerosis 2003
Sixth Annual Intensive Update in Neurology 0915-162016
73
Vestibular compensation
bull Acute unilateral or bilateral vestibular loss will prompt compensation thru central cervico-ocular visual and proprioceptive pathways
bull Unilateral loss may require several weeks for compensation to occur and activity especially head movements hastens compensation
bull Vestibular suppressant medication prolongs compensation
Sixth Annual Intensive Update in Neurology 0915-162016
74
Vestibular Rehabilitation
bull Performed by certified physical therapists many sites (Epic order vestibular PT) or check Vestibular Disorders of America website for providers
bull Assessment and treatment of benign positional vertigo
bull Directed rehab programs for central andor peripheral vestibular compensationndash Unilateral vestibulopathies
ndash Bilateral vestibular hypofunction
ndash Mutifactorial balance deficits and fall risk assessment
Sixth Annual Intensive Update in Neurology 0915-162016
75
Summary of whatrsquos new in peripheral vestibular disorders
bull Lateral semicircular canal benign paroxysmal positional vertigo presents with horizontal positioning nystagmus
bull Oral steroids appear to improve outcome in acute vestibular neuritis
bull Intratympanic steroids offer promise in Menierersquos syndrome
bull Superior semicircular canal dehiscence causes pressure and noise induced dizziness VEMP offers diagnostic information
Sixth Annual Intensive Update in Neurology 0915-162016
76
References
BPPVndash White J Benign paroxysmal positional vertigo Cleveland Clinic Journal of
Medicine 71(9)722-728 2004
ndash White J Coale K Catalano P Oas J Diagnosis and management of lateral semicircular canal benign positional vertigo Otolaryngology-Head and Neck Surgery 133 278-284 2005
ndash White J Savvides P Cherian N Oas J Canalith repositioning for benign paroxysmal positional vertigo Otology and Neurotology 26 704-710 2005
ndash White J Oas J Diagnosis and management of lateral semicircular canal conversions during particle repositioning therapy Laryngoscope 115(10) 1895-1897 2005
ndash Acute Vestibular Syndrome Hotson JR Baloh RW N Engl J Med 1998 Sep 3339(10)680-5
ndash Methylprednisolone valacyclovir or the combination for vestibular neuritis Strupp M Zingler VC Arbusow V Niklas D Maag KP Dieterich M Bense S Theil D Jahn K Brandt T N Engl J Med 2004 Jul 22351(4)354-61
ndash Intratympanic steroids for Meacuteniegraveres disease or syndrome Phillips JS Westerberg BCochrane Database Syst Rev 2011 Jul 6(7)CD008514 Review
ndash Sound- andor pressure-induced vertigo due to bone dehiscence of the superior semicircular canal Minor LB Solomon D Zinreich JS Zee DSArch Otolaryngol Head Neck Surg 1998 Mar124(3)249-58
Sixth Annual Intensive Update in Neurology 0915-162016
27
Repositioning Maneuvers for Geotropic Lateral Semicircular Canal (LSC) BPPV
bull Roll maneuver (Lempert Baloh)
ndash Patient rolls in 90 degree increments towards the good side
completely rolling over R LSC-BPPV repositioning shown
Sixth Annual Intensive Update in Neurology 0915-162016
28
Repositioning Maneuvers for Geotropic LSC BPPV
bull Gufoni maneuver
ndash Patient lies quickly onto good side and turns face-down to
floor for two minutes
Sixth Annual Intensive Update in Neurology 0915-162016
29
Left Apogeotropic LSC BPPV
Sixth Annual Intensive Update in Neurology 0915-162016
30
Nystagmus observed During Repositioning
bull Nystagmus should remain in the same torsional
direction as seen on DHT throughout the treatment of
posterior BPPV
bull Reversal of nystagmus direction suggests the particle
has fallen back into the canal and predicts failure
bull In Lateral BPPV nystagmus should beat towards the
good ear
Sixth Annual Intensive Update in Neurology 0915-162016
31
Posterior to Lateral canal conversion
Sixth Annual Intensive Update in Neurology 0915-162016
32
R Posterior to Lateral Canal conversion - geotropic
Sixth Annual Intensive Update in Neurology 0915-162016
33
BPPV Recurrence
bull Recurrence is common (15yr)
bull Otoconial adherence or mineralization demineralization
abnormalities may contribute to recurrence
bull Home canalith repositioning is effective (Radke 2004)
Sixth Annual Intensive Update in Neurology 0915-162016
34
Question 1
bull The most likely semicircular canal to be affected in
BPPV is
ndash 1) superior
ndash 2) lateral
ndash 3) posterior
ndash 4) combined
Sixth Annual Intensive Update in Neurology 0915-162016
35
Question 1
bull The most likely semicircular canal to be affected in
BPPV is
ndash 1) superior
ndash 2) lateral
ndash 3) posterior
ndash 4) combined
Answer 3 See references at end of lecture
Sixth Annual Intensive Update in Neurology 0915-162016
36
Acute Vestibular Syndrome
bull Rapid unilateral injury to either peripheral or central
vestibular structures produces prolonged vertigo
(days to weeks)
bull Severe vertigo nausea and vomiting spontaneous
nystagmus and postural instability
Sixth Annual Intensive Update in Neurology 0915-162016
37
Acute Vestibular Syndrome
bull 25 of patients with risk factors who present with isolated severe vertigo nystagmus and postural instability in the ER have an acute infarction of the inferior cerebellum (AICA PICA) Central imaging is suggested (Baloh 1998 NEJM)
ndash HTN diabetes smoking age gt65 Afib valvular heart disease occlusive vascular disease
Sixth Annual Intensive Update in Neurology 0915-162016
38
HINTS test
bull Head Impulse
bull Nystagmus
bull Test of Skew
ndash More sensitive than MRI in the diagnosis of acute stroke in
patients with acute vestibular syndrome with at least one risk
factor (Kattah J et al Stroke 2009)
Sixth Annual Intensive Update in Neurology 0915-162016
39
Head Impulse Test
bull No special equipment needed
bull Examiner stands in front of seated patient and
randomly moves head left and right Patient is asked to
focus on examinerrsquos nose
bull Rapid low amplitude thrust from lateral to straight
ahead causes a compensatory saccade when
performed towards the weak side
bull This finding is a permanent feature of unilateral
vestibular loss
Sixth Annual Intensive Update in Neurology 0915-162016
40
Head Impulse Test
Sixth Annual Intensive Update in Neurology 0915-162016
41
Head CT ndash Academy of Emergency Medicine 2013
bull The clinical value of head CT in isolated
dizzinessvertigo without any associated neurological
symptoms is very low
bull Less than 1 of head CTs ordered for dizziness and
vertigo have significant abnormalities and those
patients had severe headache and neurological deficits
in addition to dizziness
bull Forgoing unnecessary emergency head CTs could
save 360 million dollars annually
Sixth Annual Intensive Update in Neurology 0915-162016
42
Question 2
Head CT is not indicated for the evaluation of dizziness
and vertigo without additional neurological complaints because
1 It is too expensive
2 Less than 1 of CTs done for dizziness and vertigo are
abnormal and those patients had associated severe
neurological complaints
3 CT is the best imaging choice for the posterior fossa
4 Acoustic neuromas are well seen on CT
Sixth Annual Intensive Update in Neurology 0915-162016
43
Question 2
Head CT is not indicated for the evaluation of dizziness
and vertigo without additional neurological complaints because
1 It is too expensive
2 Less than 1 of CTs done for dizziness and vertigo are
abnormal and those patients had associated severe
neurological complaints
3 CT is the best imaging choice for the posterior fossa
4 Acoustic neuromas are well seen on CT
Answer 2 See references at end of lecture
Sixth Annual Intensive Update in Neurology 0915-162016
44
Acute Vestibular Syndrome
bull Usually peripheral and can be associated with
hearing loss (labyrinthitis)
bull Timely diagnosis improves recovery (ASAP
prednisone 1mgkgday for ten days and taper 10
mgday ndash Strupp M 2004 NEJM)
bull Avoid vestibular suppressants after 3 days and begin
vestibular rehabilitation to hasten compensation
Sixth Annual Intensive Update in Neurology 0915-162016
45
Question 3
bull Treatment for Acute Vestibular Syndrome
1) oral prednisone 1 mgkgday
2) Treat dehydration if necessary
3) Minimize vestibular suppressants after 3 days
4) Consider vestibular physical therapy
5) All of the above
Sixth Annual Intensive Update in Neurology 0915-162016
46
Question 3
bull Treatment for Acute Vestibular Syndrome
1) oral prednisone 1 mgkgday
2) Treat dehydration if necessary
3) Minimize vestibular suppressants after 3 days
4) Consider vestibular physical therapy
5) All of the above
Answer 5 See references at end of lecture
Sixth Annual Intensive Update in Neurology 0915-162016
47
Menierersquos syndrome
bull Fluctuating low frequency hearing loss
bull Tinnitus andor aural fullness
bull Vertigo lasting hours
Sixth Annual Intensive Update in Neurology 0915-162016
48
Normal
Sixth Annual Intensive Update in Neurology 0915-162016
49
Hydrops
Sixth Annual Intensive Update in Neurology 0915-162016
50
Menierersquos syndrome
ndash Diagnosis- Exclusion (RO acoustic)
ndash 23 of Menierersquos patients will improve spontaneously
ndash Treatment-1500 mgday low sodium triamterene375-
hydrochlorothiazide 25 vestibular suppressants (lorazepam
1 mg tablet sublingual Q 8 prn attacks) off label use
Sixth Annual Intensive Update in Neurology 0915-162016
51
Menierersquos syndrome ndash intratympanic steroids
ndash A newer treatment option for those failing medical management
ndash 90 response rate (Boleus-Aguirre MS Otology Neurotology
2008)
ndash Outcomes data confirm excellent clinical response in 3941
Menierersquos patients treated with intratympanic steroids in 2008
Sixth Annual Intensive Update in Neurology 0915-162016
52
Question 4
bull Menierersquos syndrome diagnosis is based on
1) Recurrent vertigo lasting at least 20 minutes
2) Aural fullness
3) Documented hearing loss
4) Other causes excluded
5) All of the above
Sixth Annual Intensive Update in Neurology 0915-162016
53
Question 4
bull Menierersquos syndrome diagnosis is based on
1) Recurrent vertigo lasting at least 20 minutes
2) Aural fullness
3) Documented hearing loss
4) Other causes excluded
5) All of the above
Answer 5 See references at end
Sixth Annual Intensive Update in Neurology 0915-162016
54
Superior Semicircular Canal Dehiscence (SSCD)
bull Sound andor pressure induced oscillopsia
bull CT reformatted in the plane of the superior canals
shows dehiscence of overlying bone
bull Low frequency ldquoconductive hyperacusisrdquo with elevated
bone thresholds normal reflexes and a conductive loss
Sixth Annual Intensive Update in Neurology 0915-162016
55
SSCD
bull Positive middle ear pressure or loud sound stimulates ampullofugal endolymph flow -canal activation
bull Eye movements are tonic upwards with ipsilateral intorsion
Sixth Annual Intensive Update in Neurology 0915-162016
56
SSCD
bull Valsalva against a closed glottis or jugular compression causes increased intracranial pressure and ampullopetal endolymph flow -canal inhibition
bull Eye movements are tonic downwards with ipsilateral extorsion
Sixth Annual Intensive Update in Neurology 0915-162016
57
Reconstructed left superior semicircular canal
Sixth Annual Intensive Update in Neurology 0915-162016
58
Sixth Annual Intensive Update in Neurology 0915-162016
59
SCHEMATIC DRAWING OF AN OTOLITHIC MACULA
Sixth Annual Intensive Update in Neurology 0915-162016
60
MACULAE OF THE UTRICLESACCULE
Sixth Annual Intensive Update in Neurology 0915-162016
61
HAIR CELLS
Sixth Annual Intensive Update in Neurology 0915-162016
62
SCANNING ELECTRON MICROGRAPH OF OTOCONIA
Sixth Annual Intensive Update in Neurology 0915-162016
63
Ocular VEMP
bull Response to loud clicks (80-110 dB) or forehead tap (Halmagyi and Curthoys 2003)
bull Contralateral oculomotor nucleus (CN III)
bull Inferior oblique muscle contraction (8 msec) detected by 5sec event triggered averaging
bull Noninverting electrode inferior to eye
bull Inverting below non-inverting electrode
bull Ground to forehead
bull Normal subjects have very low response
Sixth Annual Intensive Update in Neurology 0915-162016
64
Clinical applications
bull The o-VEMP is a sensitive test of otolithutricular
function
bull Complements vestibular test procedures that assess
semicircular canals
bull Abnormally robust responses are associated with third
window abnormalities such as semicircular canal
dehiscence
bull Serves as an excellent screening test for semicircular
canal dehiscence
Sixth Annual Intensive Update in Neurology 0915-162016
65
Question 5
bull Superior semicircular canal dehiscence
1) Causes increased ability to hear vibratory sounds
2) Improves hearing
3) Causes meningitis
4) Always needs surgical repair
Sixth Annual Intensive Update in Neurology 0915-162016
66
Question 5
bull Superior semicircular canal dehiscence
1) Causes increased ability to hear vibratory sounds
2) Improves hearing
3) Causes meningitis
4) Always needs surgical repair
Answer 1 References at end of lecture
Sixth Annual Intensive Update in Neurology 0915-162016
67
Enlarged vestibular aqueduct
bull Axial temporal bone CT showing right temporal bone with enlarged vestibular aqueduct (arrow)
bull Vestibular symptoms accompany progressive sensorineural hearing loss in 4-50 of patients in selected series
Sixth Annual Intensive Update in Neurology 0915-162016
68
Migraine
bull Family or personal history of migraine
bull 25 of migraine patients experience spontaneous vertigo (Baloh 1997)
bull Neuhauser criteria(2001) have been used to construct structured diagnostic interviews (Furman 2003)
bull Not always associated with headache
bull Motion sensitivity
bull Medication responsive
Sixth Annual Intensive Update in Neurology 0915-162016
69
ICHD-III beta Criteria for Vestibular Migraine
bull A At least five attacks fulfilling criteria C and D
bull B A current or past history of 11 migraine without aura or 12 migraine with aura
bull C Vestibular symptoms of moderate or severe intensity lasting between 5 minutes and 72 hours
bull D At least 50 associated with one of the following migrainous features
ndash 1 Headaches
ndash 2 Photophobia and phonophobia
ndash 3 Visual Aura
bull E Not better accounted for by another vestibular disorder
Sixth Annual Intensive Update in Neurology 0915-162016
70
Incidence of vertigo in MS
bull True vertigo (the perception of environmental
movement typically rotational) occurs in about
20 of MS patients and is the presenting
manifestation in 5
bull Up to 78 of MS patients report abnormalities
in balance at some point in their illness
Frohman et al Multiple Sclerosis 2003 9250
Sixth Annual Intensive Update in Neurology 0915-162016
71
Vertigo in MS due to demyelination
bull The most common neuroanatomic locations are the
root entry zone of CN VIII (giving absent calorics but
normal hearing and fixation suppression) and the
medial vestibular nucleus
bull Usually treated with steroids and vestibular
suppressants
Brandt 1993 Gass 1998 Thomke 1999
Sixth Annual Intensive Update in Neurology 0915-162016
72
Vertigo in MS
Frohmanrsquos large series (1153) reported that 52 of MS patients with acute vertigo had benign paroxysmal positional vertigo while 32 had new brainstem lesions
To avoid misdiagnoses and inappropriate treatment interventions alternative etiologies should be considered in all acute vertigo episodes in MS
E M Frohman Neurology 2000 and Multiple Sclerosis 2003
Sixth Annual Intensive Update in Neurology 0915-162016
73
Vestibular compensation
bull Acute unilateral or bilateral vestibular loss will prompt compensation thru central cervico-ocular visual and proprioceptive pathways
bull Unilateral loss may require several weeks for compensation to occur and activity especially head movements hastens compensation
bull Vestibular suppressant medication prolongs compensation
Sixth Annual Intensive Update in Neurology 0915-162016
74
Vestibular Rehabilitation
bull Performed by certified physical therapists many sites (Epic order vestibular PT) or check Vestibular Disorders of America website for providers
bull Assessment and treatment of benign positional vertigo
bull Directed rehab programs for central andor peripheral vestibular compensationndash Unilateral vestibulopathies
ndash Bilateral vestibular hypofunction
ndash Mutifactorial balance deficits and fall risk assessment
Sixth Annual Intensive Update in Neurology 0915-162016
75
Summary of whatrsquos new in peripheral vestibular disorders
bull Lateral semicircular canal benign paroxysmal positional vertigo presents with horizontal positioning nystagmus
bull Oral steroids appear to improve outcome in acute vestibular neuritis
bull Intratympanic steroids offer promise in Menierersquos syndrome
bull Superior semicircular canal dehiscence causes pressure and noise induced dizziness VEMP offers diagnostic information
Sixth Annual Intensive Update in Neurology 0915-162016
76
References
BPPVndash White J Benign paroxysmal positional vertigo Cleveland Clinic Journal of
Medicine 71(9)722-728 2004
ndash White J Coale K Catalano P Oas J Diagnosis and management of lateral semicircular canal benign positional vertigo Otolaryngology-Head and Neck Surgery 133 278-284 2005
ndash White J Savvides P Cherian N Oas J Canalith repositioning for benign paroxysmal positional vertigo Otology and Neurotology 26 704-710 2005
ndash White J Oas J Diagnosis and management of lateral semicircular canal conversions during particle repositioning therapy Laryngoscope 115(10) 1895-1897 2005
ndash Acute Vestibular Syndrome Hotson JR Baloh RW N Engl J Med 1998 Sep 3339(10)680-5
ndash Methylprednisolone valacyclovir or the combination for vestibular neuritis Strupp M Zingler VC Arbusow V Niklas D Maag KP Dieterich M Bense S Theil D Jahn K Brandt T N Engl J Med 2004 Jul 22351(4)354-61
ndash Intratympanic steroids for Meacuteniegraveres disease or syndrome Phillips JS Westerberg BCochrane Database Syst Rev 2011 Jul 6(7)CD008514 Review
ndash Sound- andor pressure-induced vertigo due to bone dehiscence of the superior semicircular canal Minor LB Solomon D Zinreich JS Zee DSArch Otolaryngol Head Neck Surg 1998 Mar124(3)249-58
Sixth Annual Intensive Update in Neurology 0915-162016
28
Repositioning Maneuvers for Geotropic LSC BPPV
bull Gufoni maneuver
ndash Patient lies quickly onto good side and turns face-down to
floor for two minutes
Sixth Annual Intensive Update in Neurology 0915-162016
29
Left Apogeotropic LSC BPPV
Sixth Annual Intensive Update in Neurology 0915-162016
30
Nystagmus observed During Repositioning
bull Nystagmus should remain in the same torsional
direction as seen on DHT throughout the treatment of
posterior BPPV
bull Reversal of nystagmus direction suggests the particle
has fallen back into the canal and predicts failure
bull In Lateral BPPV nystagmus should beat towards the
good ear
Sixth Annual Intensive Update in Neurology 0915-162016
31
Posterior to Lateral canal conversion
Sixth Annual Intensive Update in Neurology 0915-162016
32
R Posterior to Lateral Canal conversion - geotropic
Sixth Annual Intensive Update in Neurology 0915-162016
33
BPPV Recurrence
bull Recurrence is common (15yr)
bull Otoconial adherence or mineralization demineralization
abnormalities may contribute to recurrence
bull Home canalith repositioning is effective (Radke 2004)
Sixth Annual Intensive Update in Neurology 0915-162016
34
Question 1
bull The most likely semicircular canal to be affected in
BPPV is
ndash 1) superior
ndash 2) lateral
ndash 3) posterior
ndash 4) combined
Sixth Annual Intensive Update in Neurology 0915-162016
35
Question 1
bull The most likely semicircular canal to be affected in
BPPV is
ndash 1) superior
ndash 2) lateral
ndash 3) posterior
ndash 4) combined
Answer 3 See references at end of lecture
Sixth Annual Intensive Update in Neurology 0915-162016
36
Acute Vestibular Syndrome
bull Rapid unilateral injury to either peripheral or central
vestibular structures produces prolonged vertigo
(days to weeks)
bull Severe vertigo nausea and vomiting spontaneous
nystagmus and postural instability
Sixth Annual Intensive Update in Neurology 0915-162016
37
Acute Vestibular Syndrome
bull 25 of patients with risk factors who present with isolated severe vertigo nystagmus and postural instability in the ER have an acute infarction of the inferior cerebellum (AICA PICA) Central imaging is suggested (Baloh 1998 NEJM)
ndash HTN diabetes smoking age gt65 Afib valvular heart disease occlusive vascular disease
Sixth Annual Intensive Update in Neurology 0915-162016
38
HINTS test
bull Head Impulse
bull Nystagmus
bull Test of Skew
ndash More sensitive than MRI in the diagnosis of acute stroke in
patients with acute vestibular syndrome with at least one risk
factor (Kattah J et al Stroke 2009)
Sixth Annual Intensive Update in Neurology 0915-162016
39
Head Impulse Test
bull No special equipment needed
bull Examiner stands in front of seated patient and
randomly moves head left and right Patient is asked to
focus on examinerrsquos nose
bull Rapid low amplitude thrust from lateral to straight
ahead causes a compensatory saccade when
performed towards the weak side
bull This finding is a permanent feature of unilateral
vestibular loss
Sixth Annual Intensive Update in Neurology 0915-162016
40
Head Impulse Test
Sixth Annual Intensive Update in Neurology 0915-162016
41
Head CT ndash Academy of Emergency Medicine 2013
bull The clinical value of head CT in isolated
dizzinessvertigo without any associated neurological
symptoms is very low
bull Less than 1 of head CTs ordered for dizziness and
vertigo have significant abnormalities and those
patients had severe headache and neurological deficits
in addition to dizziness
bull Forgoing unnecessary emergency head CTs could
save 360 million dollars annually
Sixth Annual Intensive Update in Neurology 0915-162016
42
Question 2
Head CT is not indicated for the evaluation of dizziness
and vertigo without additional neurological complaints because
1 It is too expensive
2 Less than 1 of CTs done for dizziness and vertigo are
abnormal and those patients had associated severe
neurological complaints
3 CT is the best imaging choice for the posterior fossa
4 Acoustic neuromas are well seen on CT
Sixth Annual Intensive Update in Neurology 0915-162016
43
Question 2
Head CT is not indicated for the evaluation of dizziness
and vertigo without additional neurological complaints because
1 It is too expensive
2 Less than 1 of CTs done for dizziness and vertigo are
abnormal and those patients had associated severe
neurological complaints
3 CT is the best imaging choice for the posterior fossa
4 Acoustic neuromas are well seen on CT
Answer 2 See references at end of lecture
Sixth Annual Intensive Update in Neurology 0915-162016
44
Acute Vestibular Syndrome
bull Usually peripheral and can be associated with
hearing loss (labyrinthitis)
bull Timely diagnosis improves recovery (ASAP
prednisone 1mgkgday for ten days and taper 10
mgday ndash Strupp M 2004 NEJM)
bull Avoid vestibular suppressants after 3 days and begin
vestibular rehabilitation to hasten compensation
Sixth Annual Intensive Update in Neurology 0915-162016
45
Question 3
bull Treatment for Acute Vestibular Syndrome
1) oral prednisone 1 mgkgday
2) Treat dehydration if necessary
3) Minimize vestibular suppressants after 3 days
4) Consider vestibular physical therapy
5) All of the above
Sixth Annual Intensive Update in Neurology 0915-162016
46
Question 3
bull Treatment for Acute Vestibular Syndrome
1) oral prednisone 1 mgkgday
2) Treat dehydration if necessary
3) Minimize vestibular suppressants after 3 days
4) Consider vestibular physical therapy
5) All of the above
Answer 5 See references at end of lecture
Sixth Annual Intensive Update in Neurology 0915-162016
47
Menierersquos syndrome
bull Fluctuating low frequency hearing loss
bull Tinnitus andor aural fullness
bull Vertigo lasting hours
Sixth Annual Intensive Update in Neurology 0915-162016
48
Normal
Sixth Annual Intensive Update in Neurology 0915-162016
49
Hydrops
Sixth Annual Intensive Update in Neurology 0915-162016
50
Menierersquos syndrome
ndash Diagnosis- Exclusion (RO acoustic)
ndash 23 of Menierersquos patients will improve spontaneously
ndash Treatment-1500 mgday low sodium triamterene375-
hydrochlorothiazide 25 vestibular suppressants (lorazepam
1 mg tablet sublingual Q 8 prn attacks) off label use
Sixth Annual Intensive Update in Neurology 0915-162016
51
Menierersquos syndrome ndash intratympanic steroids
ndash A newer treatment option for those failing medical management
ndash 90 response rate (Boleus-Aguirre MS Otology Neurotology
2008)
ndash Outcomes data confirm excellent clinical response in 3941
Menierersquos patients treated with intratympanic steroids in 2008
Sixth Annual Intensive Update in Neurology 0915-162016
52
Question 4
bull Menierersquos syndrome diagnosis is based on
1) Recurrent vertigo lasting at least 20 minutes
2) Aural fullness
3) Documented hearing loss
4) Other causes excluded
5) All of the above
Sixth Annual Intensive Update in Neurology 0915-162016
53
Question 4
bull Menierersquos syndrome diagnosis is based on
1) Recurrent vertigo lasting at least 20 minutes
2) Aural fullness
3) Documented hearing loss
4) Other causes excluded
5) All of the above
Answer 5 See references at end
Sixth Annual Intensive Update in Neurology 0915-162016
54
Superior Semicircular Canal Dehiscence (SSCD)
bull Sound andor pressure induced oscillopsia
bull CT reformatted in the plane of the superior canals
shows dehiscence of overlying bone
bull Low frequency ldquoconductive hyperacusisrdquo with elevated
bone thresholds normal reflexes and a conductive loss
Sixth Annual Intensive Update in Neurology 0915-162016
55
SSCD
bull Positive middle ear pressure or loud sound stimulates ampullofugal endolymph flow -canal activation
bull Eye movements are tonic upwards with ipsilateral intorsion
Sixth Annual Intensive Update in Neurology 0915-162016
56
SSCD
bull Valsalva against a closed glottis or jugular compression causes increased intracranial pressure and ampullopetal endolymph flow -canal inhibition
bull Eye movements are tonic downwards with ipsilateral extorsion
Sixth Annual Intensive Update in Neurology 0915-162016
57
Reconstructed left superior semicircular canal
Sixth Annual Intensive Update in Neurology 0915-162016
58
Sixth Annual Intensive Update in Neurology 0915-162016
59
SCHEMATIC DRAWING OF AN OTOLITHIC MACULA
Sixth Annual Intensive Update in Neurology 0915-162016
60
MACULAE OF THE UTRICLESACCULE
Sixth Annual Intensive Update in Neurology 0915-162016
61
HAIR CELLS
Sixth Annual Intensive Update in Neurology 0915-162016
62
SCANNING ELECTRON MICROGRAPH OF OTOCONIA
Sixth Annual Intensive Update in Neurology 0915-162016
63
Ocular VEMP
bull Response to loud clicks (80-110 dB) or forehead tap (Halmagyi and Curthoys 2003)
bull Contralateral oculomotor nucleus (CN III)
bull Inferior oblique muscle contraction (8 msec) detected by 5sec event triggered averaging
bull Noninverting electrode inferior to eye
bull Inverting below non-inverting electrode
bull Ground to forehead
bull Normal subjects have very low response
Sixth Annual Intensive Update in Neurology 0915-162016
64
Clinical applications
bull The o-VEMP is a sensitive test of otolithutricular
function
bull Complements vestibular test procedures that assess
semicircular canals
bull Abnormally robust responses are associated with third
window abnormalities such as semicircular canal
dehiscence
bull Serves as an excellent screening test for semicircular
canal dehiscence
Sixth Annual Intensive Update in Neurology 0915-162016
65
Question 5
bull Superior semicircular canal dehiscence
1) Causes increased ability to hear vibratory sounds
2) Improves hearing
3) Causes meningitis
4) Always needs surgical repair
Sixth Annual Intensive Update in Neurology 0915-162016
66
Question 5
bull Superior semicircular canal dehiscence
1) Causes increased ability to hear vibratory sounds
2) Improves hearing
3) Causes meningitis
4) Always needs surgical repair
Answer 1 References at end of lecture
Sixth Annual Intensive Update in Neurology 0915-162016
67
Enlarged vestibular aqueduct
bull Axial temporal bone CT showing right temporal bone with enlarged vestibular aqueduct (arrow)
bull Vestibular symptoms accompany progressive sensorineural hearing loss in 4-50 of patients in selected series
Sixth Annual Intensive Update in Neurology 0915-162016
68
Migraine
bull Family or personal history of migraine
bull 25 of migraine patients experience spontaneous vertigo (Baloh 1997)
bull Neuhauser criteria(2001) have been used to construct structured diagnostic interviews (Furman 2003)
bull Not always associated with headache
bull Motion sensitivity
bull Medication responsive
Sixth Annual Intensive Update in Neurology 0915-162016
69
ICHD-III beta Criteria for Vestibular Migraine
bull A At least five attacks fulfilling criteria C and D
bull B A current or past history of 11 migraine without aura or 12 migraine with aura
bull C Vestibular symptoms of moderate or severe intensity lasting between 5 minutes and 72 hours
bull D At least 50 associated with one of the following migrainous features
ndash 1 Headaches
ndash 2 Photophobia and phonophobia
ndash 3 Visual Aura
bull E Not better accounted for by another vestibular disorder
Sixth Annual Intensive Update in Neurology 0915-162016
70
Incidence of vertigo in MS
bull True vertigo (the perception of environmental
movement typically rotational) occurs in about
20 of MS patients and is the presenting
manifestation in 5
bull Up to 78 of MS patients report abnormalities
in balance at some point in their illness
Frohman et al Multiple Sclerosis 2003 9250
Sixth Annual Intensive Update in Neurology 0915-162016
71
Vertigo in MS due to demyelination
bull The most common neuroanatomic locations are the
root entry zone of CN VIII (giving absent calorics but
normal hearing and fixation suppression) and the
medial vestibular nucleus
bull Usually treated with steroids and vestibular
suppressants
Brandt 1993 Gass 1998 Thomke 1999
Sixth Annual Intensive Update in Neurology 0915-162016
72
Vertigo in MS
Frohmanrsquos large series (1153) reported that 52 of MS patients with acute vertigo had benign paroxysmal positional vertigo while 32 had new brainstem lesions
To avoid misdiagnoses and inappropriate treatment interventions alternative etiologies should be considered in all acute vertigo episodes in MS
E M Frohman Neurology 2000 and Multiple Sclerosis 2003
Sixth Annual Intensive Update in Neurology 0915-162016
73
Vestibular compensation
bull Acute unilateral or bilateral vestibular loss will prompt compensation thru central cervico-ocular visual and proprioceptive pathways
bull Unilateral loss may require several weeks for compensation to occur and activity especially head movements hastens compensation
bull Vestibular suppressant medication prolongs compensation
Sixth Annual Intensive Update in Neurology 0915-162016
74
Vestibular Rehabilitation
bull Performed by certified physical therapists many sites (Epic order vestibular PT) or check Vestibular Disorders of America website for providers
bull Assessment and treatment of benign positional vertigo
bull Directed rehab programs for central andor peripheral vestibular compensationndash Unilateral vestibulopathies
ndash Bilateral vestibular hypofunction
ndash Mutifactorial balance deficits and fall risk assessment
Sixth Annual Intensive Update in Neurology 0915-162016
75
Summary of whatrsquos new in peripheral vestibular disorders
bull Lateral semicircular canal benign paroxysmal positional vertigo presents with horizontal positioning nystagmus
bull Oral steroids appear to improve outcome in acute vestibular neuritis
bull Intratympanic steroids offer promise in Menierersquos syndrome
bull Superior semicircular canal dehiscence causes pressure and noise induced dizziness VEMP offers diagnostic information
Sixth Annual Intensive Update in Neurology 0915-162016
76
References
BPPVndash White J Benign paroxysmal positional vertigo Cleveland Clinic Journal of
Medicine 71(9)722-728 2004
ndash White J Coale K Catalano P Oas J Diagnosis and management of lateral semicircular canal benign positional vertigo Otolaryngology-Head and Neck Surgery 133 278-284 2005
ndash White J Savvides P Cherian N Oas J Canalith repositioning for benign paroxysmal positional vertigo Otology and Neurotology 26 704-710 2005
ndash White J Oas J Diagnosis and management of lateral semicircular canal conversions during particle repositioning therapy Laryngoscope 115(10) 1895-1897 2005
ndash Acute Vestibular Syndrome Hotson JR Baloh RW N Engl J Med 1998 Sep 3339(10)680-5
ndash Methylprednisolone valacyclovir or the combination for vestibular neuritis Strupp M Zingler VC Arbusow V Niklas D Maag KP Dieterich M Bense S Theil D Jahn K Brandt T N Engl J Med 2004 Jul 22351(4)354-61
ndash Intratympanic steroids for Meacuteniegraveres disease or syndrome Phillips JS Westerberg BCochrane Database Syst Rev 2011 Jul 6(7)CD008514 Review
ndash Sound- andor pressure-induced vertigo due to bone dehiscence of the superior semicircular canal Minor LB Solomon D Zinreich JS Zee DSArch Otolaryngol Head Neck Surg 1998 Mar124(3)249-58
Sixth Annual Intensive Update in Neurology 0915-162016
29
Left Apogeotropic LSC BPPV
Sixth Annual Intensive Update in Neurology 0915-162016
30
Nystagmus observed During Repositioning
bull Nystagmus should remain in the same torsional
direction as seen on DHT throughout the treatment of
posterior BPPV
bull Reversal of nystagmus direction suggests the particle
has fallen back into the canal and predicts failure
bull In Lateral BPPV nystagmus should beat towards the
good ear
Sixth Annual Intensive Update in Neurology 0915-162016
31
Posterior to Lateral canal conversion
Sixth Annual Intensive Update in Neurology 0915-162016
32
R Posterior to Lateral Canal conversion - geotropic
Sixth Annual Intensive Update in Neurology 0915-162016
33
BPPV Recurrence
bull Recurrence is common (15yr)
bull Otoconial adherence or mineralization demineralization
abnormalities may contribute to recurrence
bull Home canalith repositioning is effective (Radke 2004)
Sixth Annual Intensive Update in Neurology 0915-162016
34
Question 1
bull The most likely semicircular canal to be affected in
BPPV is
ndash 1) superior
ndash 2) lateral
ndash 3) posterior
ndash 4) combined
Sixth Annual Intensive Update in Neurology 0915-162016
35
Question 1
bull The most likely semicircular canal to be affected in
BPPV is
ndash 1) superior
ndash 2) lateral
ndash 3) posterior
ndash 4) combined
Answer 3 See references at end of lecture
Sixth Annual Intensive Update in Neurology 0915-162016
36
Acute Vestibular Syndrome
bull Rapid unilateral injury to either peripheral or central
vestibular structures produces prolonged vertigo
(days to weeks)
bull Severe vertigo nausea and vomiting spontaneous
nystagmus and postural instability
Sixth Annual Intensive Update in Neurology 0915-162016
37
Acute Vestibular Syndrome
bull 25 of patients with risk factors who present with isolated severe vertigo nystagmus and postural instability in the ER have an acute infarction of the inferior cerebellum (AICA PICA) Central imaging is suggested (Baloh 1998 NEJM)
ndash HTN diabetes smoking age gt65 Afib valvular heart disease occlusive vascular disease
Sixth Annual Intensive Update in Neurology 0915-162016
38
HINTS test
bull Head Impulse
bull Nystagmus
bull Test of Skew
ndash More sensitive than MRI in the diagnosis of acute stroke in
patients with acute vestibular syndrome with at least one risk
factor (Kattah J et al Stroke 2009)
Sixth Annual Intensive Update in Neurology 0915-162016
39
Head Impulse Test
bull No special equipment needed
bull Examiner stands in front of seated patient and
randomly moves head left and right Patient is asked to
focus on examinerrsquos nose
bull Rapid low amplitude thrust from lateral to straight
ahead causes a compensatory saccade when
performed towards the weak side
bull This finding is a permanent feature of unilateral
vestibular loss
Sixth Annual Intensive Update in Neurology 0915-162016
40
Head Impulse Test
Sixth Annual Intensive Update in Neurology 0915-162016
41
Head CT ndash Academy of Emergency Medicine 2013
bull The clinical value of head CT in isolated
dizzinessvertigo without any associated neurological
symptoms is very low
bull Less than 1 of head CTs ordered for dizziness and
vertigo have significant abnormalities and those
patients had severe headache and neurological deficits
in addition to dizziness
bull Forgoing unnecessary emergency head CTs could
save 360 million dollars annually
Sixth Annual Intensive Update in Neurology 0915-162016
42
Question 2
Head CT is not indicated for the evaluation of dizziness
and vertigo without additional neurological complaints because
1 It is too expensive
2 Less than 1 of CTs done for dizziness and vertigo are
abnormal and those patients had associated severe
neurological complaints
3 CT is the best imaging choice for the posterior fossa
4 Acoustic neuromas are well seen on CT
Sixth Annual Intensive Update in Neurology 0915-162016
43
Question 2
Head CT is not indicated for the evaluation of dizziness
and vertigo without additional neurological complaints because
1 It is too expensive
2 Less than 1 of CTs done for dizziness and vertigo are
abnormal and those patients had associated severe
neurological complaints
3 CT is the best imaging choice for the posterior fossa
4 Acoustic neuromas are well seen on CT
Answer 2 See references at end of lecture
Sixth Annual Intensive Update in Neurology 0915-162016
44
Acute Vestibular Syndrome
bull Usually peripheral and can be associated with
hearing loss (labyrinthitis)
bull Timely diagnosis improves recovery (ASAP
prednisone 1mgkgday for ten days and taper 10
mgday ndash Strupp M 2004 NEJM)
bull Avoid vestibular suppressants after 3 days and begin
vestibular rehabilitation to hasten compensation
Sixth Annual Intensive Update in Neurology 0915-162016
45
Question 3
bull Treatment for Acute Vestibular Syndrome
1) oral prednisone 1 mgkgday
2) Treat dehydration if necessary
3) Minimize vestibular suppressants after 3 days
4) Consider vestibular physical therapy
5) All of the above
Sixth Annual Intensive Update in Neurology 0915-162016
46
Question 3
bull Treatment for Acute Vestibular Syndrome
1) oral prednisone 1 mgkgday
2) Treat dehydration if necessary
3) Minimize vestibular suppressants after 3 days
4) Consider vestibular physical therapy
5) All of the above
Answer 5 See references at end of lecture
Sixth Annual Intensive Update in Neurology 0915-162016
47
Menierersquos syndrome
bull Fluctuating low frequency hearing loss
bull Tinnitus andor aural fullness
bull Vertigo lasting hours
Sixth Annual Intensive Update in Neurology 0915-162016
48
Normal
Sixth Annual Intensive Update in Neurology 0915-162016
49
Hydrops
Sixth Annual Intensive Update in Neurology 0915-162016
50
Menierersquos syndrome
ndash Diagnosis- Exclusion (RO acoustic)
ndash 23 of Menierersquos patients will improve spontaneously
ndash Treatment-1500 mgday low sodium triamterene375-
hydrochlorothiazide 25 vestibular suppressants (lorazepam
1 mg tablet sublingual Q 8 prn attacks) off label use
Sixth Annual Intensive Update in Neurology 0915-162016
51
Menierersquos syndrome ndash intratympanic steroids
ndash A newer treatment option for those failing medical management
ndash 90 response rate (Boleus-Aguirre MS Otology Neurotology
2008)
ndash Outcomes data confirm excellent clinical response in 3941
Menierersquos patients treated with intratympanic steroids in 2008
Sixth Annual Intensive Update in Neurology 0915-162016
52
Question 4
bull Menierersquos syndrome diagnosis is based on
1) Recurrent vertigo lasting at least 20 minutes
2) Aural fullness
3) Documented hearing loss
4) Other causes excluded
5) All of the above
Sixth Annual Intensive Update in Neurology 0915-162016
53
Question 4
bull Menierersquos syndrome diagnosis is based on
1) Recurrent vertigo lasting at least 20 minutes
2) Aural fullness
3) Documented hearing loss
4) Other causes excluded
5) All of the above
Answer 5 See references at end
Sixth Annual Intensive Update in Neurology 0915-162016
54
Superior Semicircular Canal Dehiscence (SSCD)
bull Sound andor pressure induced oscillopsia
bull CT reformatted in the plane of the superior canals
shows dehiscence of overlying bone
bull Low frequency ldquoconductive hyperacusisrdquo with elevated
bone thresholds normal reflexes and a conductive loss
Sixth Annual Intensive Update in Neurology 0915-162016
55
SSCD
bull Positive middle ear pressure or loud sound stimulates ampullofugal endolymph flow -canal activation
bull Eye movements are tonic upwards with ipsilateral intorsion
Sixth Annual Intensive Update in Neurology 0915-162016
56
SSCD
bull Valsalva against a closed glottis or jugular compression causes increased intracranial pressure and ampullopetal endolymph flow -canal inhibition
bull Eye movements are tonic downwards with ipsilateral extorsion
Sixth Annual Intensive Update in Neurology 0915-162016
57
Reconstructed left superior semicircular canal
Sixth Annual Intensive Update in Neurology 0915-162016
58
Sixth Annual Intensive Update in Neurology 0915-162016
59
SCHEMATIC DRAWING OF AN OTOLITHIC MACULA
Sixth Annual Intensive Update in Neurology 0915-162016
60
MACULAE OF THE UTRICLESACCULE
Sixth Annual Intensive Update in Neurology 0915-162016
61
HAIR CELLS
Sixth Annual Intensive Update in Neurology 0915-162016
62
SCANNING ELECTRON MICROGRAPH OF OTOCONIA
Sixth Annual Intensive Update in Neurology 0915-162016
63
Ocular VEMP
bull Response to loud clicks (80-110 dB) or forehead tap (Halmagyi and Curthoys 2003)
bull Contralateral oculomotor nucleus (CN III)
bull Inferior oblique muscle contraction (8 msec) detected by 5sec event triggered averaging
bull Noninverting electrode inferior to eye
bull Inverting below non-inverting electrode
bull Ground to forehead
bull Normal subjects have very low response
Sixth Annual Intensive Update in Neurology 0915-162016
64
Clinical applications
bull The o-VEMP is a sensitive test of otolithutricular
function
bull Complements vestibular test procedures that assess
semicircular canals
bull Abnormally robust responses are associated with third
window abnormalities such as semicircular canal
dehiscence
bull Serves as an excellent screening test for semicircular
canal dehiscence
Sixth Annual Intensive Update in Neurology 0915-162016
65
Question 5
bull Superior semicircular canal dehiscence
1) Causes increased ability to hear vibratory sounds
2) Improves hearing
3) Causes meningitis
4) Always needs surgical repair
Sixth Annual Intensive Update in Neurology 0915-162016
66
Question 5
bull Superior semicircular canal dehiscence
1) Causes increased ability to hear vibratory sounds
2) Improves hearing
3) Causes meningitis
4) Always needs surgical repair
Answer 1 References at end of lecture
Sixth Annual Intensive Update in Neurology 0915-162016
67
Enlarged vestibular aqueduct
bull Axial temporal bone CT showing right temporal bone with enlarged vestibular aqueduct (arrow)
bull Vestibular symptoms accompany progressive sensorineural hearing loss in 4-50 of patients in selected series
Sixth Annual Intensive Update in Neurology 0915-162016
68
Migraine
bull Family or personal history of migraine
bull 25 of migraine patients experience spontaneous vertigo (Baloh 1997)
bull Neuhauser criteria(2001) have been used to construct structured diagnostic interviews (Furman 2003)
bull Not always associated with headache
bull Motion sensitivity
bull Medication responsive
Sixth Annual Intensive Update in Neurology 0915-162016
69
ICHD-III beta Criteria for Vestibular Migraine
bull A At least five attacks fulfilling criteria C and D
bull B A current or past history of 11 migraine without aura or 12 migraine with aura
bull C Vestibular symptoms of moderate or severe intensity lasting between 5 minutes and 72 hours
bull D At least 50 associated with one of the following migrainous features
ndash 1 Headaches
ndash 2 Photophobia and phonophobia
ndash 3 Visual Aura
bull E Not better accounted for by another vestibular disorder
Sixth Annual Intensive Update in Neurology 0915-162016
70
Incidence of vertigo in MS
bull True vertigo (the perception of environmental
movement typically rotational) occurs in about
20 of MS patients and is the presenting
manifestation in 5
bull Up to 78 of MS patients report abnormalities
in balance at some point in their illness
Frohman et al Multiple Sclerosis 2003 9250
Sixth Annual Intensive Update in Neurology 0915-162016
71
Vertigo in MS due to demyelination
bull The most common neuroanatomic locations are the
root entry zone of CN VIII (giving absent calorics but
normal hearing and fixation suppression) and the
medial vestibular nucleus
bull Usually treated with steroids and vestibular
suppressants
Brandt 1993 Gass 1998 Thomke 1999
Sixth Annual Intensive Update in Neurology 0915-162016
72
Vertigo in MS
Frohmanrsquos large series (1153) reported that 52 of MS patients with acute vertigo had benign paroxysmal positional vertigo while 32 had new brainstem lesions
To avoid misdiagnoses and inappropriate treatment interventions alternative etiologies should be considered in all acute vertigo episodes in MS
E M Frohman Neurology 2000 and Multiple Sclerosis 2003
Sixth Annual Intensive Update in Neurology 0915-162016
73
Vestibular compensation
bull Acute unilateral or bilateral vestibular loss will prompt compensation thru central cervico-ocular visual and proprioceptive pathways
bull Unilateral loss may require several weeks for compensation to occur and activity especially head movements hastens compensation
bull Vestibular suppressant medication prolongs compensation
Sixth Annual Intensive Update in Neurology 0915-162016
74
Vestibular Rehabilitation
bull Performed by certified physical therapists many sites (Epic order vestibular PT) or check Vestibular Disorders of America website for providers
bull Assessment and treatment of benign positional vertigo
bull Directed rehab programs for central andor peripheral vestibular compensationndash Unilateral vestibulopathies
ndash Bilateral vestibular hypofunction
ndash Mutifactorial balance deficits and fall risk assessment
Sixth Annual Intensive Update in Neurology 0915-162016
75
Summary of whatrsquos new in peripheral vestibular disorders
bull Lateral semicircular canal benign paroxysmal positional vertigo presents with horizontal positioning nystagmus
bull Oral steroids appear to improve outcome in acute vestibular neuritis
bull Intratympanic steroids offer promise in Menierersquos syndrome
bull Superior semicircular canal dehiscence causes pressure and noise induced dizziness VEMP offers diagnostic information
Sixth Annual Intensive Update in Neurology 0915-162016
76
References
BPPVndash White J Benign paroxysmal positional vertigo Cleveland Clinic Journal of
Medicine 71(9)722-728 2004
ndash White J Coale K Catalano P Oas J Diagnosis and management of lateral semicircular canal benign positional vertigo Otolaryngology-Head and Neck Surgery 133 278-284 2005
ndash White J Savvides P Cherian N Oas J Canalith repositioning for benign paroxysmal positional vertigo Otology and Neurotology 26 704-710 2005
ndash White J Oas J Diagnosis and management of lateral semicircular canal conversions during particle repositioning therapy Laryngoscope 115(10) 1895-1897 2005
ndash Acute Vestibular Syndrome Hotson JR Baloh RW N Engl J Med 1998 Sep 3339(10)680-5
ndash Methylprednisolone valacyclovir or the combination for vestibular neuritis Strupp M Zingler VC Arbusow V Niklas D Maag KP Dieterich M Bense S Theil D Jahn K Brandt T N Engl J Med 2004 Jul 22351(4)354-61
ndash Intratympanic steroids for Meacuteniegraveres disease or syndrome Phillips JS Westerberg BCochrane Database Syst Rev 2011 Jul 6(7)CD008514 Review
ndash Sound- andor pressure-induced vertigo due to bone dehiscence of the superior semicircular canal Minor LB Solomon D Zinreich JS Zee DSArch Otolaryngol Head Neck Surg 1998 Mar124(3)249-58
Sixth Annual Intensive Update in Neurology 0915-162016
30
Nystagmus observed During Repositioning
bull Nystagmus should remain in the same torsional
direction as seen on DHT throughout the treatment of
posterior BPPV
bull Reversal of nystagmus direction suggests the particle
has fallen back into the canal and predicts failure
bull In Lateral BPPV nystagmus should beat towards the
good ear
Sixth Annual Intensive Update in Neurology 0915-162016
31
Posterior to Lateral canal conversion
Sixth Annual Intensive Update in Neurology 0915-162016
32
R Posterior to Lateral Canal conversion - geotropic
Sixth Annual Intensive Update in Neurology 0915-162016
33
BPPV Recurrence
bull Recurrence is common (15yr)
bull Otoconial adherence or mineralization demineralization
abnormalities may contribute to recurrence
bull Home canalith repositioning is effective (Radke 2004)
Sixth Annual Intensive Update in Neurology 0915-162016
34
Question 1
bull The most likely semicircular canal to be affected in
BPPV is
ndash 1) superior
ndash 2) lateral
ndash 3) posterior
ndash 4) combined
Sixth Annual Intensive Update in Neurology 0915-162016
35
Question 1
bull The most likely semicircular canal to be affected in
BPPV is
ndash 1) superior
ndash 2) lateral
ndash 3) posterior
ndash 4) combined
Answer 3 See references at end of lecture
Sixth Annual Intensive Update in Neurology 0915-162016
36
Acute Vestibular Syndrome
bull Rapid unilateral injury to either peripheral or central
vestibular structures produces prolonged vertigo
(days to weeks)
bull Severe vertigo nausea and vomiting spontaneous
nystagmus and postural instability
Sixth Annual Intensive Update in Neurology 0915-162016
37
Acute Vestibular Syndrome
bull 25 of patients with risk factors who present with isolated severe vertigo nystagmus and postural instability in the ER have an acute infarction of the inferior cerebellum (AICA PICA) Central imaging is suggested (Baloh 1998 NEJM)
ndash HTN diabetes smoking age gt65 Afib valvular heart disease occlusive vascular disease
Sixth Annual Intensive Update in Neurology 0915-162016
38
HINTS test
bull Head Impulse
bull Nystagmus
bull Test of Skew
ndash More sensitive than MRI in the diagnosis of acute stroke in
patients with acute vestibular syndrome with at least one risk
factor (Kattah J et al Stroke 2009)
Sixth Annual Intensive Update in Neurology 0915-162016
39
Head Impulse Test
bull No special equipment needed
bull Examiner stands in front of seated patient and
randomly moves head left and right Patient is asked to
focus on examinerrsquos nose
bull Rapid low amplitude thrust from lateral to straight
ahead causes a compensatory saccade when
performed towards the weak side
bull This finding is a permanent feature of unilateral
vestibular loss
Sixth Annual Intensive Update in Neurology 0915-162016
40
Head Impulse Test
Sixth Annual Intensive Update in Neurology 0915-162016
41
Head CT ndash Academy of Emergency Medicine 2013
bull The clinical value of head CT in isolated
dizzinessvertigo without any associated neurological
symptoms is very low
bull Less than 1 of head CTs ordered for dizziness and
vertigo have significant abnormalities and those
patients had severe headache and neurological deficits
in addition to dizziness
bull Forgoing unnecessary emergency head CTs could
save 360 million dollars annually
Sixth Annual Intensive Update in Neurology 0915-162016
42
Question 2
Head CT is not indicated for the evaluation of dizziness
and vertigo without additional neurological complaints because
1 It is too expensive
2 Less than 1 of CTs done for dizziness and vertigo are
abnormal and those patients had associated severe
neurological complaints
3 CT is the best imaging choice for the posterior fossa
4 Acoustic neuromas are well seen on CT
Sixth Annual Intensive Update in Neurology 0915-162016
43
Question 2
Head CT is not indicated for the evaluation of dizziness
and vertigo without additional neurological complaints because
1 It is too expensive
2 Less than 1 of CTs done for dizziness and vertigo are
abnormal and those patients had associated severe
neurological complaints
3 CT is the best imaging choice for the posterior fossa
4 Acoustic neuromas are well seen on CT
Answer 2 See references at end of lecture
Sixth Annual Intensive Update in Neurology 0915-162016
44
Acute Vestibular Syndrome
bull Usually peripheral and can be associated with
hearing loss (labyrinthitis)
bull Timely diagnosis improves recovery (ASAP
prednisone 1mgkgday for ten days and taper 10
mgday ndash Strupp M 2004 NEJM)
bull Avoid vestibular suppressants after 3 days and begin
vestibular rehabilitation to hasten compensation
Sixth Annual Intensive Update in Neurology 0915-162016
45
Question 3
bull Treatment for Acute Vestibular Syndrome
1) oral prednisone 1 mgkgday
2) Treat dehydration if necessary
3) Minimize vestibular suppressants after 3 days
4) Consider vestibular physical therapy
5) All of the above
Sixth Annual Intensive Update in Neurology 0915-162016
46
Question 3
bull Treatment for Acute Vestibular Syndrome
1) oral prednisone 1 mgkgday
2) Treat dehydration if necessary
3) Minimize vestibular suppressants after 3 days
4) Consider vestibular physical therapy
5) All of the above
Answer 5 See references at end of lecture
Sixth Annual Intensive Update in Neurology 0915-162016
47
Menierersquos syndrome
bull Fluctuating low frequency hearing loss
bull Tinnitus andor aural fullness
bull Vertigo lasting hours
Sixth Annual Intensive Update in Neurology 0915-162016
48
Normal
Sixth Annual Intensive Update in Neurology 0915-162016
49
Hydrops
Sixth Annual Intensive Update in Neurology 0915-162016
50
Menierersquos syndrome
ndash Diagnosis- Exclusion (RO acoustic)
ndash 23 of Menierersquos patients will improve spontaneously
ndash Treatment-1500 mgday low sodium triamterene375-
hydrochlorothiazide 25 vestibular suppressants (lorazepam
1 mg tablet sublingual Q 8 prn attacks) off label use
Sixth Annual Intensive Update in Neurology 0915-162016
51
Menierersquos syndrome ndash intratympanic steroids
ndash A newer treatment option for those failing medical management
ndash 90 response rate (Boleus-Aguirre MS Otology Neurotology
2008)
ndash Outcomes data confirm excellent clinical response in 3941
Menierersquos patients treated with intratympanic steroids in 2008
Sixth Annual Intensive Update in Neurology 0915-162016
52
Question 4
bull Menierersquos syndrome diagnosis is based on
1) Recurrent vertigo lasting at least 20 minutes
2) Aural fullness
3) Documented hearing loss
4) Other causes excluded
5) All of the above
Sixth Annual Intensive Update in Neurology 0915-162016
53
Question 4
bull Menierersquos syndrome diagnosis is based on
1) Recurrent vertigo lasting at least 20 minutes
2) Aural fullness
3) Documented hearing loss
4) Other causes excluded
5) All of the above
Answer 5 See references at end
Sixth Annual Intensive Update in Neurology 0915-162016
54
Superior Semicircular Canal Dehiscence (SSCD)
bull Sound andor pressure induced oscillopsia
bull CT reformatted in the plane of the superior canals
shows dehiscence of overlying bone
bull Low frequency ldquoconductive hyperacusisrdquo with elevated
bone thresholds normal reflexes and a conductive loss
Sixth Annual Intensive Update in Neurology 0915-162016
55
SSCD
bull Positive middle ear pressure or loud sound stimulates ampullofugal endolymph flow -canal activation
bull Eye movements are tonic upwards with ipsilateral intorsion
Sixth Annual Intensive Update in Neurology 0915-162016
56
SSCD
bull Valsalva against a closed glottis or jugular compression causes increased intracranial pressure and ampullopetal endolymph flow -canal inhibition
bull Eye movements are tonic downwards with ipsilateral extorsion
Sixth Annual Intensive Update in Neurology 0915-162016
57
Reconstructed left superior semicircular canal
Sixth Annual Intensive Update in Neurology 0915-162016
58
Sixth Annual Intensive Update in Neurology 0915-162016
59
SCHEMATIC DRAWING OF AN OTOLITHIC MACULA
Sixth Annual Intensive Update in Neurology 0915-162016
60
MACULAE OF THE UTRICLESACCULE
Sixth Annual Intensive Update in Neurology 0915-162016
61
HAIR CELLS
Sixth Annual Intensive Update in Neurology 0915-162016
62
SCANNING ELECTRON MICROGRAPH OF OTOCONIA
Sixth Annual Intensive Update in Neurology 0915-162016
63
Ocular VEMP
bull Response to loud clicks (80-110 dB) or forehead tap (Halmagyi and Curthoys 2003)
bull Contralateral oculomotor nucleus (CN III)
bull Inferior oblique muscle contraction (8 msec) detected by 5sec event triggered averaging
bull Noninverting electrode inferior to eye
bull Inverting below non-inverting electrode
bull Ground to forehead
bull Normal subjects have very low response
Sixth Annual Intensive Update in Neurology 0915-162016
64
Clinical applications
bull The o-VEMP is a sensitive test of otolithutricular
function
bull Complements vestibular test procedures that assess
semicircular canals
bull Abnormally robust responses are associated with third
window abnormalities such as semicircular canal
dehiscence
bull Serves as an excellent screening test for semicircular
canal dehiscence
Sixth Annual Intensive Update in Neurology 0915-162016
65
Question 5
bull Superior semicircular canal dehiscence
1) Causes increased ability to hear vibratory sounds
2) Improves hearing
3) Causes meningitis
4) Always needs surgical repair
Sixth Annual Intensive Update in Neurology 0915-162016
66
Question 5
bull Superior semicircular canal dehiscence
1) Causes increased ability to hear vibratory sounds
2) Improves hearing
3) Causes meningitis
4) Always needs surgical repair
Answer 1 References at end of lecture
Sixth Annual Intensive Update in Neurology 0915-162016
67
Enlarged vestibular aqueduct
bull Axial temporal bone CT showing right temporal bone with enlarged vestibular aqueduct (arrow)
bull Vestibular symptoms accompany progressive sensorineural hearing loss in 4-50 of patients in selected series
Sixth Annual Intensive Update in Neurology 0915-162016
68
Migraine
bull Family or personal history of migraine
bull 25 of migraine patients experience spontaneous vertigo (Baloh 1997)
bull Neuhauser criteria(2001) have been used to construct structured diagnostic interviews (Furman 2003)
bull Not always associated with headache
bull Motion sensitivity
bull Medication responsive
Sixth Annual Intensive Update in Neurology 0915-162016
69
ICHD-III beta Criteria for Vestibular Migraine
bull A At least five attacks fulfilling criteria C and D
bull B A current or past history of 11 migraine without aura or 12 migraine with aura
bull C Vestibular symptoms of moderate or severe intensity lasting between 5 minutes and 72 hours
bull D At least 50 associated with one of the following migrainous features
ndash 1 Headaches
ndash 2 Photophobia and phonophobia
ndash 3 Visual Aura
bull E Not better accounted for by another vestibular disorder
Sixth Annual Intensive Update in Neurology 0915-162016
70
Incidence of vertigo in MS
bull True vertigo (the perception of environmental
movement typically rotational) occurs in about
20 of MS patients and is the presenting
manifestation in 5
bull Up to 78 of MS patients report abnormalities
in balance at some point in their illness
Frohman et al Multiple Sclerosis 2003 9250
Sixth Annual Intensive Update in Neurology 0915-162016
71
Vertigo in MS due to demyelination
bull The most common neuroanatomic locations are the
root entry zone of CN VIII (giving absent calorics but
normal hearing and fixation suppression) and the
medial vestibular nucleus
bull Usually treated with steroids and vestibular
suppressants
Brandt 1993 Gass 1998 Thomke 1999
Sixth Annual Intensive Update in Neurology 0915-162016
72
Vertigo in MS
Frohmanrsquos large series (1153) reported that 52 of MS patients with acute vertigo had benign paroxysmal positional vertigo while 32 had new brainstem lesions
To avoid misdiagnoses and inappropriate treatment interventions alternative etiologies should be considered in all acute vertigo episodes in MS
E M Frohman Neurology 2000 and Multiple Sclerosis 2003
Sixth Annual Intensive Update in Neurology 0915-162016
73
Vestibular compensation
bull Acute unilateral or bilateral vestibular loss will prompt compensation thru central cervico-ocular visual and proprioceptive pathways
bull Unilateral loss may require several weeks for compensation to occur and activity especially head movements hastens compensation
bull Vestibular suppressant medication prolongs compensation
Sixth Annual Intensive Update in Neurology 0915-162016
74
Vestibular Rehabilitation
bull Performed by certified physical therapists many sites (Epic order vestibular PT) or check Vestibular Disorders of America website for providers
bull Assessment and treatment of benign positional vertigo
bull Directed rehab programs for central andor peripheral vestibular compensationndash Unilateral vestibulopathies
ndash Bilateral vestibular hypofunction
ndash Mutifactorial balance deficits and fall risk assessment
Sixth Annual Intensive Update in Neurology 0915-162016
75
Summary of whatrsquos new in peripheral vestibular disorders
bull Lateral semicircular canal benign paroxysmal positional vertigo presents with horizontal positioning nystagmus
bull Oral steroids appear to improve outcome in acute vestibular neuritis
bull Intratympanic steroids offer promise in Menierersquos syndrome
bull Superior semicircular canal dehiscence causes pressure and noise induced dizziness VEMP offers diagnostic information
Sixth Annual Intensive Update in Neurology 0915-162016
76
References
BPPVndash White J Benign paroxysmal positional vertigo Cleveland Clinic Journal of
Medicine 71(9)722-728 2004
ndash White J Coale K Catalano P Oas J Diagnosis and management of lateral semicircular canal benign positional vertigo Otolaryngology-Head and Neck Surgery 133 278-284 2005
ndash White J Savvides P Cherian N Oas J Canalith repositioning for benign paroxysmal positional vertigo Otology and Neurotology 26 704-710 2005
ndash White J Oas J Diagnosis and management of lateral semicircular canal conversions during particle repositioning therapy Laryngoscope 115(10) 1895-1897 2005
ndash Acute Vestibular Syndrome Hotson JR Baloh RW N Engl J Med 1998 Sep 3339(10)680-5
ndash Methylprednisolone valacyclovir or the combination for vestibular neuritis Strupp M Zingler VC Arbusow V Niklas D Maag KP Dieterich M Bense S Theil D Jahn K Brandt T N Engl J Med 2004 Jul 22351(4)354-61
ndash Intratympanic steroids for Meacuteniegraveres disease or syndrome Phillips JS Westerberg BCochrane Database Syst Rev 2011 Jul 6(7)CD008514 Review
ndash Sound- andor pressure-induced vertigo due to bone dehiscence of the superior semicircular canal Minor LB Solomon D Zinreich JS Zee DSArch Otolaryngol Head Neck Surg 1998 Mar124(3)249-58
Sixth Annual Intensive Update in Neurology 0915-162016
31
Posterior to Lateral canal conversion
Sixth Annual Intensive Update in Neurology 0915-162016
32
R Posterior to Lateral Canal conversion - geotropic
Sixth Annual Intensive Update in Neurology 0915-162016
33
BPPV Recurrence
bull Recurrence is common (15yr)
bull Otoconial adherence or mineralization demineralization
abnormalities may contribute to recurrence
bull Home canalith repositioning is effective (Radke 2004)
Sixth Annual Intensive Update in Neurology 0915-162016
34
Question 1
bull The most likely semicircular canal to be affected in
BPPV is
ndash 1) superior
ndash 2) lateral
ndash 3) posterior
ndash 4) combined
Sixth Annual Intensive Update in Neurology 0915-162016
35
Question 1
bull The most likely semicircular canal to be affected in
BPPV is
ndash 1) superior
ndash 2) lateral
ndash 3) posterior
ndash 4) combined
Answer 3 See references at end of lecture
Sixth Annual Intensive Update in Neurology 0915-162016
36
Acute Vestibular Syndrome
bull Rapid unilateral injury to either peripheral or central
vestibular structures produces prolonged vertigo
(days to weeks)
bull Severe vertigo nausea and vomiting spontaneous
nystagmus and postural instability
Sixth Annual Intensive Update in Neurology 0915-162016
37
Acute Vestibular Syndrome
bull 25 of patients with risk factors who present with isolated severe vertigo nystagmus and postural instability in the ER have an acute infarction of the inferior cerebellum (AICA PICA) Central imaging is suggested (Baloh 1998 NEJM)
ndash HTN diabetes smoking age gt65 Afib valvular heart disease occlusive vascular disease
Sixth Annual Intensive Update in Neurology 0915-162016
38
HINTS test
bull Head Impulse
bull Nystagmus
bull Test of Skew
ndash More sensitive than MRI in the diagnosis of acute stroke in
patients with acute vestibular syndrome with at least one risk
factor (Kattah J et al Stroke 2009)
Sixth Annual Intensive Update in Neurology 0915-162016
39
Head Impulse Test
bull No special equipment needed
bull Examiner stands in front of seated patient and
randomly moves head left and right Patient is asked to
focus on examinerrsquos nose
bull Rapid low amplitude thrust from lateral to straight
ahead causes a compensatory saccade when
performed towards the weak side
bull This finding is a permanent feature of unilateral
vestibular loss
Sixth Annual Intensive Update in Neurology 0915-162016
40
Head Impulse Test
Sixth Annual Intensive Update in Neurology 0915-162016
41
Head CT ndash Academy of Emergency Medicine 2013
bull The clinical value of head CT in isolated
dizzinessvertigo without any associated neurological
symptoms is very low
bull Less than 1 of head CTs ordered for dizziness and
vertigo have significant abnormalities and those
patients had severe headache and neurological deficits
in addition to dizziness
bull Forgoing unnecessary emergency head CTs could
save 360 million dollars annually
Sixth Annual Intensive Update in Neurology 0915-162016
42
Question 2
Head CT is not indicated for the evaluation of dizziness
and vertigo without additional neurological complaints because
1 It is too expensive
2 Less than 1 of CTs done for dizziness and vertigo are
abnormal and those patients had associated severe
neurological complaints
3 CT is the best imaging choice for the posterior fossa
4 Acoustic neuromas are well seen on CT
Sixth Annual Intensive Update in Neurology 0915-162016
43
Question 2
Head CT is not indicated for the evaluation of dizziness
and vertigo without additional neurological complaints because
1 It is too expensive
2 Less than 1 of CTs done for dizziness and vertigo are
abnormal and those patients had associated severe
neurological complaints
3 CT is the best imaging choice for the posterior fossa
4 Acoustic neuromas are well seen on CT
Answer 2 See references at end of lecture
Sixth Annual Intensive Update in Neurology 0915-162016
44
Acute Vestibular Syndrome
bull Usually peripheral and can be associated with
hearing loss (labyrinthitis)
bull Timely diagnosis improves recovery (ASAP
prednisone 1mgkgday for ten days and taper 10
mgday ndash Strupp M 2004 NEJM)
bull Avoid vestibular suppressants after 3 days and begin
vestibular rehabilitation to hasten compensation
Sixth Annual Intensive Update in Neurology 0915-162016
45
Question 3
bull Treatment for Acute Vestibular Syndrome
1) oral prednisone 1 mgkgday
2) Treat dehydration if necessary
3) Minimize vestibular suppressants after 3 days
4) Consider vestibular physical therapy
5) All of the above
Sixth Annual Intensive Update in Neurology 0915-162016
46
Question 3
bull Treatment for Acute Vestibular Syndrome
1) oral prednisone 1 mgkgday
2) Treat dehydration if necessary
3) Minimize vestibular suppressants after 3 days
4) Consider vestibular physical therapy
5) All of the above
Answer 5 See references at end of lecture
Sixth Annual Intensive Update in Neurology 0915-162016
47
Menierersquos syndrome
bull Fluctuating low frequency hearing loss
bull Tinnitus andor aural fullness
bull Vertigo lasting hours
Sixth Annual Intensive Update in Neurology 0915-162016
48
Normal
Sixth Annual Intensive Update in Neurology 0915-162016
49
Hydrops
Sixth Annual Intensive Update in Neurology 0915-162016
50
Menierersquos syndrome
ndash Diagnosis- Exclusion (RO acoustic)
ndash 23 of Menierersquos patients will improve spontaneously
ndash Treatment-1500 mgday low sodium triamterene375-
hydrochlorothiazide 25 vestibular suppressants (lorazepam
1 mg tablet sublingual Q 8 prn attacks) off label use
Sixth Annual Intensive Update in Neurology 0915-162016
51
Menierersquos syndrome ndash intratympanic steroids
ndash A newer treatment option for those failing medical management
ndash 90 response rate (Boleus-Aguirre MS Otology Neurotology
2008)
ndash Outcomes data confirm excellent clinical response in 3941
Menierersquos patients treated with intratympanic steroids in 2008
Sixth Annual Intensive Update in Neurology 0915-162016
52
Question 4
bull Menierersquos syndrome diagnosis is based on
1) Recurrent vertigo lasting at least 20 minutes
2) Aural fullness
3) Documented hearing loss
4) Other causes excluded
5) All of the above
Sixth Annual Intensive Update in Neurology 0915-162016
53
Question 4
bull Menierersquos syndrome diagnosis is based on
1) Recurrent vertigo lasting at least 20 minutes
2) Aural fullness
3) Documented hearing loss
4) Other causes excluded
5) All of the above
Answer 5 See references at end
Sixth Annual Intensive Update in Neurology 0915-162016
54
Superior Semicircular Canal Dehiscence (SSCD)
bull Sound andor pressure induced oscillopsia
bull CT reformatted in the plane of the superior canals
shows dehiscence of overlying bone
bull Low frequency ldquoconductive hyperacusisrdquo with elevated
bone thresholds normal reflexes and a conductive loss
Sixth Annual Intensive Update in Neurology 0915-162016
55
SSCD
bull Positive middle ear pressure or loud sound stimulates ampullofugal endolymph flow -canal activation
bull Eye movements are tonic upwards with ipsilateral intorsion
Sixth Annual Intensive Update in Neurology 0915-162016
56
SSCD
bull Valsalva against a closed glottis or jugular compression causes increased intracranial pressure and ampullopetal endolymph flow -canal inhibition
bull Eye movements are tonic downwards with ipsilateral extorsion
Sixth Annual Intensive Update in Neurology 0915-162016
57
Reconstructed left superior semicircular canal
Sixth Annual Intensive Update in Neurology 0915-162016
58
Sixth Annual Intensive Update in Neurology 0915-162016
59
SCHEMATIC DRAWING OF AN OTOLITHIC MACULA
Sixth Annual Intensive Update in Neurology 0915-162016
60
MACULAE OF THE UTRICLESACCULE
Sixth Annual Intensive Update in Neurology 0915-162016
61
HAIR CELLS
Sixth Annual Intensive Update in Neurology 0915-162016
62
SCANNING ELECTRON MICROGRAPH OF OTOCONIA
Sixth Annual Intensive Update in Neurology 0915-162016
63
Ocular VEMP
bull Response to loud clicks (80-110 dB) or forehead tap (Halmagyi and Curthoys 2003)
bull Contralateral oculomotor nucleus (CN III)
bull Inferior oblique muscle contraction (8 msec) detected by 5sec event triggered averaging
bull Noninverting electrode inferior to eye
bull Inverting below non-inverting electrode
bull Ground to forehead
bull Normal subjects have very low response
Sixth Annual Intensive Update in Neurology 0915-162016
64
Clinical applications
bull The o-VEMP is a sensitive test of otolithutricular
function
bull Complements vestibular test procedures that assess
semicircular canals
bull Abnormally robust responses are associated with third
window abnormalities such as semicircular canal
dehiscence
bull Serves as an excellent screening test for semicircular
canal dehiscence
Sixth Annual Intensive Update in Neurology 0915-162016
65
Question 5
bull Superior semicircular canal dehiscence
1) Causes increased ability to hear vibratory sounds
2) Improves hearing
3) Causes meningitis
4) Always needs surgical repair
Sixth Annual Intensive Update in Neurology 0915-162016
66
Question 5
bull Superior semicircular canal dehiscence
1) Causes increased ability to hear vibratory sounds
2) Improves hearing
3) Causes meningitis
4) Always needs surgical repair
Answer 1 References at end of lecture
Sixth Annual Intensive Update in Neurology 0915-162016
67
Enlarged vestibular aqueduct
bull Axial temporal bone CT showing right temporal bone with enlarged vestibular aqueduct (arrow)
bull Vestibular symptoms accompany progressive sensorineural hearing loss in 4-50 of patients in selected series
Sixth Annual Intensive Update in Neurology 0915-162016
68
Migraine
bull Family or personal history of migraine
bull 25 of migraine patients experience spontaneous vertigo (Baloh 1997)
bull Neuhauser criteria(2001) have been used to construct structured diagnostic interviews (Furman 2003)
bull Not always associated with headache
bull Motion sensitivity
bull Medication responsive
Sixth Annual Intensive Update in Neurology 0915-162016
69
ICHD-III beta Criteria for Vestibular Migraine
bull A At least five attacks fulfilling criteria C and D
bull B A current or past history of 11 migraine without aura or 12 migraine with aura
bull C Vestibular symptoms of moderate or severe intensity lasting between 5 minutes and 72 hours
bull D At least 50 associated with one of the following migrainous features
ndash 1 Headaches
ndash 2 Photophobia and phonophobia
ndash 3 Visual Aura
bull E Not better accounted for by another vestibular disorder
Sixth Annual Intensive Update in Neurology 0915-162016
70
Incidence of vertigo in MS
bull True vertigo (the perception of environmental
movement typically rotational) occurs in about
20 of MS patients and is the presenting
manifestation in 5
bull Up to 78 of MS patients report abnormalities
in balance at some point in their illness
Frohman et al Multiple Sclerosis 2003 9250
Sixth Annual Intensive Update in Neurology 0915-162016
71
Vertigo in MS due to demyelination
bull The most common neuroanatomic locations are the
root entry zone of CN VIII (giving absent calorics but
normal hearing and fixation suppression) and the
medial vestibular nucleus
bull Usually treated with steroids and vestibular
suppressants
Brandt 1993 Gass 1998 Thomke 1999
Sixth Annual Intensive Update in Neurology 0915-162016
72
Vertigo in MS
Frohmanrsquos large series (1153) reported that 52 of MS patients with acute vertigo had benign paroxysmal positional vertigo while 32 had new brainstem lesions
To avoid misdiagnoses and inappropriate treatment interventions alternative etiologies should be considered in all acute vertigo episodes in MS
E M Frohman Neurology 2000 and Multiple Sclerosis 2003
Sixth Annual Intensive Update in Neurology 0915-162016
73
Vestibular compensation
bull Acute unilateral or bilateral vestibular loss will prompt compensation thru central cervico-ocular visual and proprioceptive pathways
bull Unilateral loss may require several weeks for compensation to occur and activity especially head movements hastens compensation
bull Vestibular suppressant medication prolongs compensation
Sixth Annual Intensive Update in Neurology 0915-162016
74
Vestibular Rehabilitation
bull Performed by certified physical therapists many sites (Epic order vestibular PT) or check Vestibular Disorders of America website for providers
bull Assessment and treatment of benign positional vertigo
bull Directed rehab programs for central andor peripheral vestibular compensationndash Unilateral vestibulopathies
ndash Bilateral vestibular hypofunction
ndash Mutifactorial balance deficits and fall risk assessment
Sixth Annual Intensive Update in Neurology 0915-162016
75
Summary of whatrsquos new in peripheral vestibular disorders
bull Lateral semicircular canal benign paroxysmal positional vertigo presents with horizontal positioning nystagmus
bull Oral steroids appear to improve outcome in acute vestibular neuritis
bull Intratympanic steroids offer promise in Menierersquos syndrome
bull Superior semicircular canal dehiscence causes pressure and noise induced dizziness VEMP offers diagnostic information
Sixth Annual Intensive Update in Neurology 0915-162016
76
References
BPPVndash White J Benign paroxysmal positional vertigo Cleveland Clinic Journal of
Medicine 71(9)722-728 2004
ndash White J Coale K Catalano P Oas J Diagnosis and management of lateral semicircular canal benign positional vertigo Otolaryngology-Head and Neck Surgery 133 278-284 2005
ndash White J Savvides P Cherian N Oas J Canalith repositioning for benign paroxysmal positional vertigo Otology and Neurotology 26 704-710 2005
ndash White J Oas J Diagnosis and management of lateral semicircular canal conversions during particle repositioning therapy Laryngoscope 115(10) 1895-1897 2005
ndash Acute Vestibular Syndrome Hotson JR Baloh RW N Engl J Med 1998 Sep 3339(10)680-5
ndash Methylprednisolone valacyclovir or the combination for vestibular neuritis Strupp M Zingler VC Arbusow V Niklas D Maag KP Dieterich M Bense S Theil D Jahn K Brandt T N Engl J Med 2004 Jul 22351(4)354-61
ndash Intratympanic steroids for Meacuteniegraveres disease or syndrome Phillips JS Westerberg BCochrane Database Syst Rev 2011 Jul 6(7)CD008514 Review
ndash Sound- andor pressure-induced vertigo due to bone dehiscence of the superior semicircular canal Minor LB Solomon D Zinreich JS Zee DSArch Otolaryngol Head Neck Surg 1998 Mar124(3)249-58
Sixth Annual Intensive Update in Neurology 0915-162016
32
R Posterior to Lateral Canal conversion - geotropic
Sixth Annual Intensive Update in Neurology 0915-162016
33
BPPV Recurrence
bull Recurrence is common (15yr)
bull Otoconial adherence or mineralization demineralization
abnormalities may contribute to recurrence
bull Home canalith repositioning is effective (Radke 2004)
Sixth Annual Intensive Update in Neurology 0915-162016
34
Question 1
bull The most likely semicircular canal to be affected in
BPPV is
ndash 1) superior
ndash 2) lateral
ndash 3) posterior
ndash 4) combined
Sixth Annual Intensive Update in Neurology 0915-162016
35
Question 1
bull The most likely semicircular canal to be affected in
BPPV is
ndash 1) superior
ndash 2) lateral
ndash 3) posterior
ndash 4) combined
Answer 3 See references at end of lecture
Sixth Annual Intensive Update in Neurology 0915-162016
36
Acute Vestibular Syndrome
bull Rapid unilateral injury to either peripheral or central
vestibular structures produces prolonged vertigo
(days to weeks)
bull Severe vertigo nausea and vomiting spontaneous
nystagmus and postural instability
Sixth Annual Intensive Update in Neurology 0915-162016
37
Acute Vestibular Syndrome
bull 25 of patients with risk factors who present with isolated severe vertigo nystagmus and postural instability in the ER have an acute infarction of the inferior cerebellum (AICA PICA) Central imaging is suggested (Baloh 1998 NEJM)
ndash HTN diabetes smoking age gt65 Afib valvular heart disease occlusive vascular disease
Sixth Annual Intensive Update in Neurology 0915-162016
38
HINTS test
bull Head Impulse
bull Nystagmus
bull Test of Skew
ndash More sensitive than MRI in the diagnosis of acute stroke in
patients with acute vestibular syndrome with at least one risk
factor (Kattah J et al Stroke 2009)
Sixth Annual Intensive Update in Neurology 0915-162016
39
Head Impulse Test
bull No special equipment needed
bull Examiner stands in front of seated patient and
randomly moves head left and right Patient is asked to
focus on examinerrsquos nose
bull Rapid low amplitude thrust from lateral to straight
ahead causes a compensatory saccade when
performed towards the weak side
bull This finding is a permanent feature of unilateral
vestibular loss
Sixth Annual Intensive Update in Neurology 0915-162016
40
Head Impulse Test
Sixth Annual Intensive Update in Neurology 0915-162016
41
Head CT ndash Academy of Emergency Medicine 2013
bull The clinical value of head CT in isolated
dizzinessvertigo without any associated neurological
symptoms is very low
bull Less than 1 of head CTs ordered for dizziness and
vertigo have significant abnormalities and those
patients had severe headache and neurological deficits
in addition to dizziness
bull Forgoing unnecessary emergency head CTs could
save 360 million dollars annually
Sixth Annual Intensive Update in Neurology 0915-162016
42
Question 2
Head CT is not indicated for the evaluation of dizziness
and vertigo without additional neurological complaints because
1 It is too expensive
2 Less than 1 of CTs done for dizziness and vertigo are
abnormal and those patients had associated severe
neurological complaints
3 CT is the best imaging choice for the posterior fossa
4 Acoustic neuromas are well seen on CT
Sixth Annual Intensive Update in Neurology 0915-162016
43
Question 2
Head CT is not indicated for the evaluation of dizziness
and vertigo without additional neurological complaints because
1 It is too expensive
2 Less than 1 of CTs done for dizziness and vertigo are
abnormal and those patients had associated severe
neurological complaints
3 CT is the best imaging choice for the posterior fossa
4 Acoustic neuromas are well seen on CT
Answer 2 See references at end of lecture
Sixth Annual Intensive Update in Neurology 0915-162016
44
Acute Vestibular Syndrome
bull Usually peripheral and can be associated with
hearing loss (labyrinthitis)
bull Timely diagnosis improves recovery (ASAP
prednisone 1mgkgday for ten days and taper 10
mgday ndash Strupp M 2004 NEJM)
bull Avoid vestibular suppressants after 3 days and begin
vestibular rehabilitation to hasten compensation
Sixth Annual Intensive Update in Neurology 0915-162016
45
Question 3
bull Treatment for Acute Vestibular Syndrome
1) oral prednisone 1 mgkgday
2) Treat dehydration if necessary
3) Minimize vestibular suppressants after 3 days
4) Consider vestibular physical therapy
5) All of the above
Sixth Annual Intensive Update in Neurology 0915-162016
46
Question 3
bull Treatment for Acute Vestibular Syndrome
1) oral prednisone 1 mgkgday
2) Treat dehydration if necessary
3) Minimize vestibular suppressants after 3 days
4) Consider vestibular physical therapy
5) All of the above
Answer 5 See references at end of lecture
Sixth Annual Intensive Update in Neurology 0915-162016
47
Menierersquos syndrome
bull Fluctuating low frequency hearing loss
bull Tinnitus andor aural fullness
bull Vertigo lasting hours
Sixth Annual Intensive Update in Neurology 0915-162016
48
Normal
Sixth Annual Intensive Update in Neurology 0915-162016
49
Hydrops
Sixth Annual Intensive Update in Neurology 0915-162016
50
Menierersquos syndrome
ndash Diagnosis- Exclusion (RO acoustic)
ndash 23 of Menierersquos patients will improve spontaneously
ndash Treatment-1500 mgday low sodium triamterene375-
hydrochlorothiazide 25 vestibular suppressants (lorazepam
1 mg tablet sublingual Q 8 prn attacks) off label use
Sixth Annual Intensive Update in Neurology 0915-162016
51
Menierersquos syndrome ndash intratympanic steroids
ndash A newer treatment option for those failing medical management
ndash 90 response rate (Boleus-Aguirre MS Otology Neurotology
2008)
ndash Outcomes data confirm excellent clinical response in 3941
Menierersquos patients treated with intratympanic steroids in 2008
Sixth Annual Intensive Update in Neurology 0915-162016
52
Question 4
bull Menierersquos syndrome diagnosis is based on
1) Recurrent vertigo lasting at least 20 minutes
2) Aural fullness
3) Documented hearing loss
4) Other causes excluded
5) All of the above
Sixth Annual Intensive Update in Neurology 0915-162016
53
Question 4
bull Menierersquos syndrome diagnosis is based on
1) Recurrent vertigo lasting at least 20 minutes
2) Aural fullness
3) Documented hearing loss
4) Other causes excluded
5) All of the above
Answer 5 See references at end
Sixth Annual Intensive Update in Neurology 0915-162016
54
Superior Semicircular Canal Dehiscence (SSCD)
bull Sound andor pressure induced oscillopsia
bull CT reformatted in the plane of the superior canals
shows dehiscence of overlying bone
bull Low frequency ldquoconductive hyperacusisrdquo with elevated
bone thresholds normal reflexes and a conductive loss
Sixth Annual Intensive Update in Neurology 0915-162016
55
SSCD
bull Positive middle ear pressure or loud sound stimulates ampullofugal endolymph flow -canal activation
bull Eye movements are tonic upwards with ipsilateral intorsion
Sixth Annual Intensive Update in Neurology 0915-162016
56
SSCD
bull Valsalva against a closed glottis or jugular compression causes increased intracranial pressure and ampullopetal endolymph flow -canal inhibition
bull Eye movements are tonic downwards with ipsilateral extorsion
Sixth Annual Intensive Update in Neurology 0915-162016
57
Reconstructed left superior semicircular canal
Sixth Annual Intensive Update in Neurology 0915-162016
58
Sixth Annual Intensive Update in Neurology 0915-162016
59
SCHEMATIC DRAWING OF AN OTOLITHIC MACULA
Sixth Annual Intensive Update in Neurology 0915-162016
60
MACULAE OF THE UTRICLESACCULE
Sixth Annual Intensive Update in Neurology 0915-162016
61
HAIR CELLS
Sixth Annual Intensive Update in Neurology 0915-162016
62
SCANNING ELECTRON MICROGRAPH OF OTOCONIA
Sixth Annual Intensive Update in Neurology 0915-162016
63
Ocular VEMP
bull Response to loud clicks (80-110 dB) or forehead tap (Halmagyi and Curthoys 2003)
bull Contralateral oculomotor nucleus (CN III)
bull Inferior oblique muscle contraction (8 msec) detected by 5sec event triggered averaging
bull Noninverting electrode inferior to eye
bull Inverting below non-inverting electrode
bull Ground to forehead
bull Normal subjects have very low response
Sixth Annual Intensive Update in Neurology 0915-162016
64
Clinical applications
bull The o-VEMP is a sensitive test of otolithutricular
function
bull Complements vestibular test procedures that assess
semicircular canals
bull Abnormally robust responses are associated with third
window abnormalities such as semicircular canal
dehiscence
bull Serves as an excellent screening test for semicircular
canal dehiscence
Sixth Annual Intensive Update in Neurology 0915-162016
65
Question 5
bull Superior semicircular canal dehiscence
1) Causes increased ability to hear vibratory sounds
2) Improves hearing
3) Causes meningitis
4) Always needs surgical repair
Sixth Annual Intensive Update in Neurology 0915-162016
66
Question 5
bull Superior semicircular canal dehiscence
1) Causes increased ability to hear vibratory sounds
2) Improves hearing
3) Causes meningitis
4) Always needs surgical repair
Answer 1 References at end of lecture
Sixth Annual Intensive Update in Neurology 0915-162016
67
Enlarged vestibular aqueduct
bull Axial temporal bone CT showing right temporal bone with enlarged vestibular aqueduct (arrow)
bull Vestibular symptoms accompany progressive sensorineural hearing loss in 4-50 of patients in selected series
Sixth Annual Intensive Update in Neurology 0915-162016
68
Migraine
bull Family or personal history of migraine
bull 25 of migraine patients experience spontaneous vertigo (Baloh 1997)
bull Neuhauser criteria(2001) have been used to construct structured diagnostic interviews (Furman 2003)
bull Not always associated with headache
bull Motion sensitivity
bull Medication responsive
Sixth Annual Intensive Update in Neurology 0915-162016
69
ICHD-III beta Criteria for Vestibular Migraine
bull A At least five attacks fulfilling criteria C and D
bull B A current or past history of 11 migraine without aura or 12 migraine with aura
bull C Vestibular symptoms of moderate or severe intensity lasting between 5 minutes and 72 hours
bull D At least 50 associated with one of the following migrainous features
ndash 1 Headaches
ndash 2 Photophobia and phonophobia
ndash 3 Visual Aura
bull E Not better accounted for by another vestibular disorder
Sixth Annual Intensive Update in Neurology 0915-162016
70
Incidence of vertigo in MS
bull True vertigo (the perception of environmental
movement typically rotational) occurs in about
20 of MS patients and is the presenting
manifestation in 5
bull Up to 78 of MS patients report abnormalities
in balance at some point in their illness
Frohman et al Multiple Sclerosis 2003 9250
Sixth Annual Intensive Update in Neurology 0915-162016
71
Vertigo in MS due to demyelination
bull The most common neuroanatomic locations are the
root entry zone of CN VIII (giving absent calorics but
normal hearing and fixation suppression) and the
medial vestibular nucleus
bull Usually treated with steroids and vestibular
suppressants
Brandt 1993 Gass 1998 Thomke 1999
Sixth Annual Intensive Update in Neurology 0915-162016
72
Vertigo in MS
Frohmanrsquos large series (1153) reported that 52 of MS patients with acute vertigo had benign paroxysmal positional vertigo while 32 had new brainstem lesions
To avoid misdiagnoses and inappropriate treatment interventions alternative etiologies should be considered in all acute vertigo episodes in MS
E M Frohman Neurology 2000 and Multiple Sclerosis 2003
Sixth Annual Intensive Update in Neurology 0915-162016
73
Vestibular compensation
bull Acute unilateral or bilateral vestibular loss will prompt compensation thru central cervico-ocular visual and proprioceptive pathways
bull Unilateral loss may require several weeks for compensation to occur and activity especially head movements hastens compensation
bull Vestibular suppressant medication prolongs compensation
Sixth Annual Intensive Update in Neurology 0915-162016
74
Vestibular Rehabilitation
bull Performed by certified physical therapists many sites (Epic order vestibular PT) or check Vestibular Disorders of America website for providers
bull Assessment and treatment of benign positional vertigo
bull Directed rehab programs for central andor peripheral vestibular compensationndash Unilateral vestibulopathies
ndash Bilateral vestibular hypofunction
ndash Mutifactorial balance deficits and fall risk assessment
Sixth Annual Intensive Update in Neurology 0915-162016
75
Summary of whatrsquos new in peripheral vestibular disorders
bull Lateral semicircular canal benign paroxysmal positional vertigo presents with horizontal positioning nystagmus
bull Oral steroids appear to improve outcome in acute vestibular neuritis
bull Intratympanic steroids offer promise in Menierersquos syndrome
bull Superior semicircular canal dehiscence causes pressure and noise induced dizziness VEMP offers diagnostic information
Sixth Annual Intensive Update in Neurology 0915-162016
76
References
BPPVndash White J Benign paroxysmal positional vertigo Cleveland Clinic Journal of
Medicine 71(9)722-728 2004
ndash White J Coale K Catalano P Oas J Diagnosis and management of lateral semicircular canal benign positional vertigo Otolaryngology-Head and Neck Surgery 133 278-284 2005
ndash White J Savvides P Cherian N Oas J Canalith repositioning for benign paroxysmal positional vertigo Otology and Neurotology 26 704-710 2005
ndash White J Oas J Diagnosis and management of lateral semicircular canal conversions during particle repositioning therapy Laryngoscope 115(10) 1895-1897 2005
ndash Acute Vestibular Syndrome Hotson JR Baloh RW N Engl J Med 1998 Sep 3339(10)680-5
ndash Methylprednisolone valacyclovir or the combination for vestibular neuritis Strupp M Zingler VC Arbusow V Niklas D Maag KP Dieterich M Bense S Theil D Jahn K Brandt T N Engl J Med 2004 Jul 22351(4)354-61
ndash Intratympanic steroids for Meacuteniegraveres disease or syndrome Phillips JS Westerberg BCochrane Database Syst Rev 2011 Jul 6(7)CD008514 Review
ndash Sound- andor pressure-induced vertigo due to bone dehiscence of the superior semicircular canal Minor LB Solomon D Zinreich JS Zee DSArch Otolaryngol Head Neck Surg 1998 Mar124(3)249-58
Sixth Annual Intensive Update in Neurology 0915-162016
33
BPPV Recurrence
bull Recurrence is common (15yr)
bull Otoconial adherence or mineralization demineralization
abnormalities may contribute to recurrence
bull Home canalith repositioning is effective (Radke 2004)
Sixth Annual Intensive Update in Neurology 0915-162016
34
Question 1
bull The most likely semicircular canal to be affected in
BPPV is
ndash 1) superior
ndash 2) lateral
ndash 3) posterior
ndash 4) combined
Sixth Annual Intensive Update in Neurology 0915-162016
35
Question 1
bull The most likely semicircular canal to be affected in
BPPV is
ndash 1) superior
ndash 2) lateral
ndash 3) posterior
ndash 4) combined
Answer 3 See references at end of lecture
Sixth Annual Intensive Update in Neurology 0915-162016
36
Acute Vestibular Syndrome
bull Rapid unilateral injury to either peripheral or central
vestibular structures produces prolonged vertigo
(days to weeks)
bull Severe vertigo nausea and vomiting spontaneous
nystagmus and postural instability
Sixth Annual Intensive Update in Neurology 0915-162016
37
Acute Vestibular Syndrome
bull 25 of patients with risk factors who present with isolated severe vertigo nystagmus and postural instability in the ER have an acute infarction of the inferior cerebellum (AICA PICA) Central imaging is suggested (Baloh 1998 NEJM)
ndash HTN diabetes smoking age gt65 Afib valvular heart disease occlusive vascular disease
Sixth Annual Intensive Update in Neurology 0915-162016
38
HINTS test
bull Head Impulse
bull Nystagmus
bull Test of Skew
ndash More sensitive than MRI in the diagnosis of acute stroke in
patients with acute vestibular syndrome with at least one risk
factor (Kattah J et al Stroke 2009)
Sixth Annual Intensive Update in Neurology 0915-162016
39
Head Impulse Test
bull No special equipment needed
bull Examiner stands in front of seated patient and
randomly moves head left and right Patient is asked to
focus on examinerrsquos nose
bull Rapid low amplitude thrust from lateral to straight
ahead causes a compensatory saccade when
performed towards the weak side
bull This finding is a permanent feature of unilateral
vestibular loss
Sixth Annual Intensive Update in Neurology 0915-162016
40
Head Impulse Test
Sixth Annual Intensive Update in Neurology 0915-162016
41
Head CT ndash Academy of Emergency Medicine 2013
bull The clinical value of head CT in isolated
dizzinessvertigo without any associated neurological
symptoms is very low
bull Less than 1 of head CTs ordered for dizziness and
vertigo have significant abnormalities and those
patients had severe headache and neurological deficits
in addition to dizziness
bull Forgoing unnecessary emergency head CTs could
save 360 million dollars annually
Sixth Annual Intensive Update in Neurology 0915-162016
42
Question 2
Head CT is not indicated for the evaluation of dizziness
and vertigo without additional neurological complaints because
1 It is too expensive
2 Less than 1 of CTs done for dizziness and vertigo are
abnormal and those patients had associated severe
neurological complaints
3 CT is the best imaging choice for the posterior fossa
4 Acoustic neuromas are well seen on CT
Sixth Annual Intensive Update in Neurology 0915-162016
43
Question 2
Head CT is not indicated for the evaluation of dizziness
and vertigo without additional neurological complaints because
1 It is too expensive
2 Less than 1 of CTs done for dizziness and vertigo are
abnormal and those patients had associated severe
neurological complaints
3 CT is the best imaging choice for the posterior fossa
4 Acoustic neuromas are well seen on CT
Answer 2 See references at end of lecture
Sixth Annual Intensive Update in Neurology 0915-162016
44
Acute Vestibular Syndrome
bull Usually peripheral and can be associated with
hearing loss (labyrinthitis)
bull Timely diagnosis improves recovery (ASAP
prednisone 1mgkgday for ten days and taper 10
mgday ndash Strupp M 2004 NEJM)
bull Avoid vestibular suppressants after 3 days and begin
vestibular rehabilitation to hasten compensation
Sixth Annual Intensive Update in Neurology 0915-162016
45
Question 3
bull Treatment for Acute Vestibular Syndrome
1) oral prednisone 1 mgkgday
2) Treat dehydration if necessary
3) Minimize vestibular suppressants after 3 days
4) Consider vestibular physical therapy
5) All of the above
Sixth Annual Intensive Update in Neurology 0915-162016
46
Question 3
bull Treatment for Acute Vestibular Syndrome
1) oral prednisone 1 mgkgday
2) Treat dehydration if necessary
3) Minimize vestibular suppressants after 3 days
4) Consider vestibular physical therapy
5) All of the above
Answer 5 See references at end of lecture
Sixth Annual Intensive Update in Neurology 0915-162016
47
Menierersquos syndrome
bull Fluctuating low frequency hearing loss
bull Tinnitus andor aural fullness
bull Vertigo lasting hours
Sixth Annual Intensive Update in Neurology 0915-162016
48
Normal
Sixth Annual Intensive Update in Neurology 0915-162016
49
Hydrops
Sixth Annual Intensive Update in Neurology 0915-162016
50
Menierersquos syndrome
ndash Diagnosis- Exclusion (RO acoustic)
ndash 23 of Menierersquos patients will improve spontaneously
ndash Treatment-1500 mgday low sodium triamterene375-
hydrochlorothiazide 25 vestibular suppressants (lorazepam
1 mg tablet sublingual Q 8 prn attacks) off label use
Sixth Annual Intensive Update in Neurology 0915-162016
51
Menierersquos syndrome ndash intratympanic steroids
ndash A newer treatment option for those failing medical management
ndash 90 response rate (Boleus-Aguirre MS Otology Neurotology
2008)
ndash Outcomes data confirm excellent clinical response in 3941
Menierersquos patients treated with intratympanic steroids in 2008
Sixth Annual Intensive Update in Neurology 0915-162016
52
Question 4
bull Menierersquos syndrome diagnosis is based on
1) Recurrent vertigo lasting at least 20 minutes
2) Aural fullness
3) Documented hearing loss
4) Other causes excluded
5) All of the above
Sixth Annual Intensive Update in Neurology 0915-162016
53
Question 4
bull Menierersquos syndrome diagnosis is based on
1) Recurrent vertigo lasting at least 20 minutes
2) Aural fullness
3) Documented hearing loss
4) Other causes excluded
5) All of the above
Answer 5 See references at end
Sixth Annual Intensive Update in Neurology 0915-162016
54
Superior Semicircular Canal Dehiscence (SSCD)
bull Sound andor pressure induced oscillopsia
bull CT reformatted in the plane of the superior canals
shows dehiscence of overlying bone
bull Low frequency ldquoconductive hyperacusisrdquo with elevated
bone thresholds normal reflexes and a conductive loss
Sixth Annual Intensive Update in Neurology 0915-162016
55
SSCD
bull Positive middle ear pressure or loud sound stimulates ampullofugal endolymph flow -canal activation
bull Eye movements are tonic upwards with ipsilateral intorsion
Sixth Annual Intensive Update in Neurology 0915-162016
56
SSCD
bull Valsalva against a closed glottis or jugular compression causes increased intracranial pressure and ampullopetal endolymph flow -canal inhibition
bull Eye movements are tonic downwards with ipsilateral extorsion
Sixth Annual Intensive Update in Neurology 0915-162016
57
Reconstructed left superior semicircular canal
Sixth Annual Intensive Update in Neurology 0915-162016
58
Sixth Annual Intensive Update in Neurology 0915-162016
59
SCHEMATIC DRAWING OF AN OTOLITHIC MACULA
Sixth Annual Intensive Update in Neurology 0915-162016
60
MACULAE OF THE UTRICLESACCULE
Sixth Annual Intensive Update in Neurology 0915-162016
61
HAIR CELLS
Sixth Annual Intensive Update in Neurology 0915-162016
62
SCANNING ELECTRON MICROGRAPH OF OTOCONIA
Sixth Annual Intensive Update in Neurology 0915-162016
63
Ocular VEMP
bull Response to loud clicks (80-110 dB) or forehead tap (Halmagyi and Curthoys 2003)
bull Contralateral oculomotor nucleus (CN III)
bull Inferior oblique muscle contraction (8 msec) detected by 5sec event triggered averaging
bull Noninverting electrode inferior to eye
bull Inverting below non-inverting electrode
bull Ground to forehead
bull Normal subjects have very low response
Sixth Annual Intensive Update in Neurology 0915-162016
64
Clinical applications
bull The o-VEMP is a sensitive test of otolithutricular
function
bull Complements vestibular test procedures that assess
semicircular canals
bull Abnormally robust responses are associated with third
window abnormalities such as semicircular canal
dehiscence
bull Serves as an excellent screening test for semicircular
canal dehiscence
Sixth Annual Intensive Update in Neurology 0915-162016
65
Question 5
bull Superior semicircular canal dehiscence
1) Causes increased ability to hear vibratory sounds
2) Improves hearing
3) Causes meningitis
4) Always needs surgical repair
Sixth Annual Intensive Update in Neurology 0915-162016
66
Question 5
bull Superior semicircular canal dehiscence
1) Causes increased ability to hear vibratory sounds
2) Improves hearing
3) Causes meningitis
4) Always needs surgical repair
Answer 1 References at end of lecture
Sixth Annual Intensive Update in Neurology 0915-162016
67
Enlarged vestibular aqueduct
bull Axial temporal bone CT showing right temporal bone with enlarged vestibular aqueduct (arrow)
bull Vestibular symptoms accompany progressive sensorineural hearing loss in 4-50 of patients in selected series
Sixth Annual Intensive Update in Neurology 0915-162016
68
Migraine
bull Family or personal history of migraine
bull 25 of migraine patients experience spontaneous vertigo (Baloh 1997)
bull Neuhauser criteria(2001) have been used to construct structured diagnostic interviews (Furman 2003)
bull Not always associated with headache
bull Motion sensitivity
bull Medication responsive
Sixth Annual Intensive Update in Neurology 0915-162016
69
ICHD-III beta Criteria for Vestibular Migraine
bull A At least five attacks fulfilling criteria C and D
bull B A current or past history of 11 migraine without aura or 12 migraine with aura
bull C Vestibular symptoms of moderate or severe intensity lasting between 5 minutes and 72 hours
bull D At least 50 associated with one of the following migrainous features
ndash 1 Headaches
ndash 2 Photophobia and phonophobia
ndash 3 Visual Aura
bull E Not better accounted for by another vestibular disorder
Sixth Annual Intensive Update in Neurology 0915-162016
70
Incidence of vertigo in MS
bull True vertigo (the perception of environmental
movement typically rotational) occurs in about
20 of MS patients and is the presenting
manifestation in 5
bull Up to 78 of MS patients report abnormalities
in balance at some point in their illness
Frohman et al Multiple Sclerosis 2003 9250
Sixth Annual Intensive Update in Neurology 0915-162016
71
Vertigo in MS due to demyelination
bull The most common neuroanatomic locations are the
root entry zone of CN VIII (giving absent calorics but
normal hearing and fixation suppression) and the
medial vestibular nucleus
bull Usually treated with steroids and vestibular
suppressants
Brandt 1993 Gass 1998 Thomke 1999
Sixth Annual Intensive Update in Neurology 0915-162016
72
Vertigo in MS
Frohmanrsquos large series (1153) reported that 52 of MS patients with acute vertigo had benign paroxysmal positional vertigo while 32 had new brainstem lesions
To avoid misdiagnoses and inappropriate treatment interventions alternative etiologies should be considered in all acute vertigo episodes in MS
E M Frohman Neurology 2000 and Multiple Sclerosis 2003
Sixth Annual Intensive Update in Neurology 0915-162016
73
Vestibular compensation
bull Acute unilateral or bilateral vestibular loss will prompt compensation thru central cervico-ocular visual and proprioceptive pathways
bull Unilateral loss may require several weeks for compensation to occur and activity especially head movements hastens compensation
bull Vestibular suppressant medication prolongs compensation
Sixth Annual Intensive Update in Neurology 0915-162016
74
Vestibular Rehabilitation
bull Performed by certified physical therapists many sites (Epic order vestibular PT) or check Vestibular Disorders of America website for providers
bull Assessment and treatment of benign positional vertigo
bull Directed rehab programs for central andor peripheral vestibular compensationndash Unilateral vestibulopathies
ndash Bilateral vestibular hypofunction
ndash Mutifactorial balance deficits and fall risk assessment
Sixth Annual Intensive Update in Neurology 0915-162016
75
Summary of whatrsquos new in peripheral vestibular disorders
bull Lateral semicircular canal benign paroxysmal positional vertigo presents with horizontal positioning nystagmus
bull Oral steroids appear to improve outcome in acute vestibular neuritis
bull Intratympanic steroids offer promise in Menierersquos syndrome
bull Superior semicircular canal dehiscence causes pressure and noise induced dizziness VEMP offers diagnostic information
Sixth Annual Intensive Update in Neurology 0915-162016
76
References
BPPVndash White J Benign paroxysmal positional vertigo Cleveland Clinic Journal of
Medicine 71(9)722-728 2004
ndash White J Coale K Catalano P Oas J Diagnosis and management of lateral semicircular canal benign positional vertigo Otolaryngology-Head and Neck Surgery 133 278-284 2005
ndash White J Savvides P Cherian N Oas J Canalith repositioning for benign paroxysmal positional vertigo Otology and Neurotology 26 704-710 2005
ndash White J Oas J Diagnosis and management of lateral semicircular canal conversions during particle repositioning therapy Laryngoscope 115(10) 1895-1897 2005
ndash Acute Vestibular Syndrome Hotson JR Baloh RW N Engl J Med 1998 Sep 3339(10)680-5
ndash Methylprednisolone valacyclovir or the combination for vestibular neuritis Strupp M Zingler VC Arbusow V Niklas D Maag KP Dieterich M Bense S Theil D Jahn K Brandt T N Engl J Med 2004 Jul 22351(4)354-61
ndash Intratympanic steroids for Meacuteniegraveres disease or syndrome Phillips JS Westerberg BCochrane Database Syst Rev 2011 Jul 6(7)CD008514 Review
ndash Sound- andor pressure-induced vertigo due to bone dehiscence of the superior semicircular canal Minor LB Solomon D Zinreich JS Zee DSArch Otolaryngol Head Neck Surg 1998 Mar124(3)249-58
Sixth Annual Intensive Update in Neurology 0915-162016
34
Question 1
bull The most likely semicircular canal to be affected in
BPPV is
ndash 1) superior
ndash 2) lateral
ndash 3) posterior
ndash 4) combined
Sixth Annual Intensive Update in Neurology 0915-162016
35
Question 1
bull The most likely semicircular canal to be affected in
BPPV is
ndash 1) superior
ndash 2) lateral
ndash 3) posterior
ndash 4) combined
Answer 3 See references at end of lecture
Sixth Annual Intensive Update in Neurology 0915-162016
36
Acute Vestibular Syndrome
bull Rapid unilateral injury to either peripheral or central
vestibular structures produces prolonged vertigo
(days to weeks)
bull Severe vertigo nausea and vomiting spontaneous
nystagmus and postural instability
Sixth Annual Intensive Update in Neurology 0915-162016
37
Acute Vestibular Syndrome
bull 25 of patients with risk factors who present with isolated severe vertigo nystagmus and postural instability in the ER have an acute infarction of the inferior cerebellum (AICA PICA) Central imaging is suggested (Baloh 1998 NEJM)
ndash HTN diabetes smoking age gt65 Afib valvular heart disease occlusive vascular disease
Sixth Annual Intensive Update in Neurology 0915-162016
38
HINTS test
bull Head Impulse
bull Nystagmus
bull Test of Skew
ndash More sensitive than MRI in the diagnosis of acute stroke in
patients with acute vestibular syndrome with at least one risk
factor (Kattah J et al Stroke 2009)
Sixth Annual Intensive Update in Neurology 0915-162016
39
Head Impulse Test
bull No special equipment needed
bull Examiner stands in front of seated patient and
randomly moves head left and right Patient is asked to
focus on examinerrsquos nose
bull Rapid low amplitude thrust from lateral to straight
ahead causes a compensatory saccade when
performed towards the weak side
bull This finding is a permanent feature of unilateral
vestibular loss
Sixth Annual Intensive Update in Neurology 0915-162016
40
Head Impulse Test
Sixth Annual Intensive Update in Neurology 0915-162016
41
Head CT ndash Academy of Emergency Medicine 2013
bull The clinical value of head CT in isolated
dizzinessvertigo without any associated neurological
symptoms is very low
bull Less than 1 of head CTs ordered for dizziness and
vertigo have significant abnormalities and those
patients had severe headache and neurological deficits
in addition to dizziness
bull Forgoing unnecessary emergency head CTs could
save 360 million dollars annually
Sixth Annual Intensive Update in Neurology 0915-162016
42
Question 2
Head CT is not indicated for the evaluation of dizziness
and vertigo without additional neurological complaints because
1 It is too expensive
2 Less than 1 of CTs done for dizziness and vertigo are
abnormal and those patients had associated severe
neurological complaints
3 CT is the best imaging choice for the posterior fossa
4 Acoustic neuromas are well seen on CT
Sixth Annual Intensive Update in Neurology 0915-162016
43
Question 2
Head CT is not indicated for the evaluation of dizziness
and vertigo without additional neurological complaints because
1 It is too expensive
2 Less than 1 of CTs done for dizziness and vertigo are
abnormal and those patients had associated severe
neurological complaints
3 CT is the best imaging choice for the posterior fossa
4 Acoustic neuromas are well seen on CT
Answer 2 See references at end of lecture
Sixth Annual Intensive Update in Neurology 0915-162016
44
Acute Vestibular Syndrome
bull Usually peripheral and can be associated with
hearing loss (labyrinthitis)
bull Timely diagnosis improves recovery (ASAP
prednisone 1mgkgday for ten days and taper 10
mgday ndash Strupp M 2004 NEJM)
bull Avoid vestibular suppressants after 3 days and begin
vestibular rehabilitation to hasten compensation
Sixth Annual Intensive Update in Neurology 0915-162016
45
Question 3
bull Treatment for Acute Vestibular Syndrome
1) oral prednisone 1 mgkgday
2) Treat dehydration if necessary
3) Minimize vestibular suppressants after 3 days
4) Consider vestibular physical therapy
5) All of the above
Sixth Annual Intensive Update in Neurology 0915-162016
46
Question 3
bull Treatment for Acute Vestibular Syndrome
1) oral prednisone 1 mgkgday
2) Treat dehydration if necessary
3) Minimize vestibular suppressants after 3 days
4) Consider vestibular physical therapy
5) All of the above
Answer 5 See references at end of lecture
Sixth Annual Intensive Update in Neurology 0915-162016
47
Menierersquos syndrome
bull Fluctuating low frequency hearing loss
bull Tinnitus andor aural fullness
bull Vertigo lasting hours
Sixth Annual Intensive Update in Neurology 0915-162016
48
Normal
Sixth Annual Intensive Update in Neurology 0915-162016
49
Hydrops
Sixth Annual Intensive Update in Neurology 0915-162016
50
Menierersquos syndrome
ndash Diagnosis- Exclusion (RO acoustic)
ndash 23 of Menierersquos patients will improve spontaneously
ndash Treatment-1500 mgday low sodium triamterene375-
hydrochlorothiazide 25 vestibular suppressants (lorazepam
1 mg tablet sublingual Q 8 prn attacks) off label use
Sixth Annual Intensive Update in Neurology 0915-162016
51
Menierersquos syndrome ndash intratympanic steroids
ndash A newer treatment option for those failing medical management
ndash 90 response rate (Boleus-Aguirre MS Otology Neurotology
2008)
ndash Outcomes data confirm excellent clinical response in 3941
Menierersquos patients treated with intratympanic steroids in 2008
Sixth Annual Intensive Update in Neurology 0915-162016
52
Question 4
bull Menierersquos syndrome diagnosis is based on
1) Recurrent vertigo lasting at least 20 minutes
2) Aural fullness
3) Documented hearing loss
4) Other causes excluded
5) All of the above
Sixth Annual Intensive Update in Neurology 0915-162016
53
Question 4
bull Menierersquos syndrome diagnosis is based on
1) Recurrent vertigo lasting at least 20 minutes
2) Aural fullness
3) Documented hearing loss
4) Other causes excluded
5) All of the above
Answer 5 See references at end
Sixth Annual Intensive Update in Neurology 0915-162016
54
Superior Semicircular Canal Dehiscence (SSCD)
bull Sound andor pressure induced oscillopsia
bull CT reformatted in the plane of the superior canals
shows dehiscence of overlying bone
bull Low frequency ldquoconductive hyperacusisrdquo with elevated
bone thresholds normal reflexes and a conductive loss
Sixth Annual Intensive Update in Neurology 0915-162016
55
SSCD
bull Positive middle ear pressure or loud sound stimulates ampullofugal endolymph flow -canal activation
bull Eye movements are tonic upwards with ipsilateral intorsion
Sixth Annual Intensive Update in Neurology 0915-162016
56
SSCD
bull Valsalva against a closed glottis or jugular compression causes increased intracranial pressure and ampullopetal endolymph flow -canal inhibition
bull Eye movements are tonic downwards with ipsilateral extorsion
Sixth Annual Intensive Update in Neurology 0915-162016
57
Reconstructed left superior semicircular canal
Sixth Annual Intensive Update in Neurology 0915-162016
58
Sixth Annual Intensive Update in Neurology 0915-162016
59
SCHEMATIC DRAWING OF AN OTOLITHIC MACULA
Sixth Annual Intensive Update in Neurology 0915-162016
60
MACULAE OF THE UTRICLESACCULE
Sixth Annual Intensive Update in Neurology 0915-162016
61
HAIR CELLS
Sixth Annual Intensive Update in Neurology 0915-162016
62
SCANNING ELECTRON MICROGRAPH OF OTOCONIA
Sixth Annual Intensive Update in Neurology 0915-162016
63
Ocular VEMP
bull Response to loud clicks (80-110 dB) or forehead tap (Halmagyi and Curthoys 2003)
bull Contralateral oculomotor nucleus (CN III)
bull Inferior oblique muscle contraction (8 msec) detected by 5sec event triggered averaging
bull Noninverting electrode inferior to eye
bull Inverting below non-inverting electrode
bull Ground to forehead
bull Normal subjects have very low response
Sixth Annual Intensive Update in Neurology 0915-162016
64
Clinical applications
bull The o-VEMP is a sensitive test of otolithutricular
function
bull Complements vestibular test procedures that assess
semicircular canals
bull Abnormally robust responses are associated with third
window abnormalities such as semicircular canal
dehiscence
bull Serves as an excellent screening test for semicircular
canal dehiscence
Sixth Annual Intensive Update in Neurology 0915-162016
65
Question 5
bull Superior semicircular canal dehiscence
1) Causes increased ability to hear vibratory sounds
2) Improves hearing
3) Causes meningitis
4) Always needs surgical repair
Sixth Annual Intensive Update in Neurology 0915-162016
66
Question 5
bull Superior semicircular canal dehiscence
1) Causes increased ability to hear vibratory sounds
2) Improves hearing
3) Causes meningitis
4) Always needs surgical repair
Answer 1 References at end of lecture
Sixth Annual Intensive Update in Neurology 0915-162016
67
Enlarged vestibular aqueduct
bull Axial temporal bone CT showing right temporal bone with enlarged vestibular aqueduct (arrow)
bull Vestibular symptoms accompany progressive sensorineural hearing loss in 4-50 of patients in selected series
Sixth Annual Intensive Update in Neurology 0915-162016
68
Migraine
bull Family or personal history of migraine
bull 25 of migraine patients experience spontaneous vertigo (Baloh 1997)
bull Neuhauser criteria(2001) have been used to construct structured diagnostic interviews (Furman 2003)
bull Not always associated with headache
bull Motion sensitivity
bull Medication responsive
Sixth Annual Intensive Update in Neurology 0915-162016
69
ICHD-III beta Criteria for Vestibular Migraine
bull A At least five attacks fulfilling criteria C and D
bull B A current or past history of 11 migraine without aura or 12 migraine with aura
bull C Vestibular symptoms of moderate or severe intensity lasting between 5 minutes and 72 hours
bull D At least 50 associated with one of the following migrainous features
ndash 1 Headaches
ndash 2 Photophobia and phonophobia
ndash 3 Visual Aura
bull E Not better accounted for by another vestibular disorder
Sixth Annual Intensive Update in Neurology 0915-162016
70
Incidence of vertigo in MS
bull True vertigo (the perception of environmental
movement typically rotational) occurs in about
20 of MS patients and is the presenting
manifestation in 5
bull Up to 78 of MS patients report abnormalities
in balance at some point in their illness
Frohman et al Multiple Sclerosis 2003 9250
Sixth Annual Intensive Update in Neurology 0915-162016
71
Vertigo in MS due to demyelination
bull The most common neuroanatomic locations are the
root entry zone of CN VIII (giving absent calorics but
normal hearing and fixation suppression) and the
medial vestibular nucleus
bull Usually treated with steroids and vestibular
suppressants
Brandt 1993 Gass 1998 Thomke 1999
Sixth Annual Intensive Update in Neurology 0915-162016
72
Vertigo in MS
Frohmanrsquos large series (1153) reported that 52 of MS patients with acute vertigo had benign paroxysmal positional vertigo while 32 had new brainstem lesions
To avoid misdiagnoses and inappropriate treatment interventions alternative etiologies should be considered in all acute vertigo episodes in MS
E M Frohman Neurology 2000 and Multiple Sclerosis 2003
Sixth Annual Intensive Update in Neurology 0915-162016
73
Vestibular compensation
bull Acute unilateral or bilateral vestibular loss will prompt compensation thru central cervico-ocular visual and proprioceptive pathways
bull Unilateral loss may require several weeks for compensation to occur and activity especially head movements hastens compensation
bull Vestibular suppressant medication prolongs compensation
Sixth Annual Intensive Update in Neurology 0915-162016
74
Vestibular Rehabilitation
bull Performed by certified physical therapists many sites (Epic order vestibular PT) or check Vestibular Disorders of America website for providers
bull Assessment and treatment of benign positional vertigo
bull Directed rehab programs for central andor peripheral vestibular compensationndash Unilateral vestibulopathies
ndash Bilateral vestibular hypofunction
ndash Mutifactorial balance deficits and fall risk assessment
Sixth Annual Intensive Update in Neurology 0915-162016
75
Summary of whatrsquos new in peripheral vestibular disorders
bull Lateral semicircular canal benign paroxysmal positional vertigo presents with horizontal positioning nystagmus
bull Oral steroids appear to improve outcome in acute vestibular neuritis
bull Intratympanic steroids offer promise in Menierersquos syndrome
bull Superior semicircular canal dehiscence causes pressure and noise induced dizziness VEMP offers diagnostic information
Sixth Annual Intensive Update in Neurology 0915-162016
76
References
BPPVndash White J Benign paroxysmal positional vertigo Cleveland Clinic Journal of
Medicine 71(9)722-728 2004
ndash White J Coale K Catalano P Oas J Diagnosis and management of lateral semicircular canal benign positional vertigo Otolaryngology-Head and Neck Surgery 133 278-284 2005
ndash White J Savvides P Cherian N Oas J Canalith repositioning for benign paroxysmal positional vertigo Otology and Neurotology 26 704-710 2005
ndash White J Oas J Diagnosis and management of lateral semicircular canal conversions during particle repositioning therapy Laryngoscope 115(10) 1895-1897 2005
ndash Acute Vestibular Syndrome Hotson JR Baloh RW N Engl J Med 1998 Sep 3339(10)680-5
ndash Methylprednisolone valacyclovir or the combination for vestibular neuritis Strupp M Zingler VC Arbusow V Niklas D Maag KP Dieterich M Bense S Theil D Jahn K Brandt T N Engl J Med 2004 Jul 22351(4)354-61
ndash Intratympanic steroids for Meacuteniegraveres disease or syndrome Phillips JS Westerberg BCochrane Database Syst Rev 2011 Jul 6(7)CD008514 Review
ndash Sound- andor pressure-induced vertigo due to bone dehiscence of the superior semicircular canal Minor LB Solomon D Zinreich JS Zee DSArch Otolaryngol Head Neck Surg 1998 Mar124(3)249-58
Sixth Annual Intensive Update in Neurology 0915-162016
35
Question 1
bull The most likely semicircular canal to be affected in
BPPV is
ndash 1) superior
ndash 2) lateral
ndash 3) posterior
ndash 4) combined
Answer 3 See references at end of lecture
Sixth Annual Intensive Update in Neurology 0915-162016
36
Acute Vestibular Syndrome
bull Rapid unilateral injury to either peripheral or central
vestibular structures produces prolonged vertigo
(days to weeks)
bull Severe vertigo nausea and vomiting spontaneous
nystagmus and postural instability
Sixth Annual Intensive Update in Neurology 0915-162016
37
Acute Vestibular Syndrome
bull 25 of patients with risk factors who present with isolated severe vertigo nystagmus and postural instability in the ER have an acute infarction of the inferior cerebellum (AICA PICA) Central imaging is suggested (Baloh 1998 NEJM)
ndash HTN diabetes smoking age gt65 Afib valvular heart disease occlusive vascular disease
Sixth Annual Intensive Update in Neurology 0915-162016
38
HINTS test
bull Head Impulse
bull Nystagmus
bull Test of Skew
ndash More sensitive than MRI in the diagnosis of acute stroke in
patients with acute vestibular syndrome with at least one risk
factor (Kattah J et al Stroke 2009)
Sixth Annual Intensive Update in Neurology 0915-162016
39
Head Impulse Test
bull No special equipment needed
bull Examiner stands in front of seated patient and
randomly moves head left and right Patient is asked to
focus on examinerrsquos nose
bull Rapid low amplitude thrust from lateral to straight
ahead causes a compensatory saccade when
performed towards the weak side
bull This finding is a permanent feature of unilateral
vestibular loss
Sixth Annual Intensive Update in Neurology 0915-162016
40
Head Impulse Test
Sixth Annual Intensive Update in Neurology 0915-162016
41
Head CT ndash Academy of Emergency Medicine 2013
bull The clinical value of head CT in isolated
dizzinessvertigo without any associated neurological
symptoms is very low
bull Less than 1 of head CTs ordered for dizziness and
vertigo have significant abnormalities and those
patients had severe headache and neurological deficits
in addition to dizziness
bull Forgoing unnecessary emergency head CTs could
save 360 million dollars annually
Sixth Annual Intensive Update in Neurology 0915-162016
42
Question 2
Head CT is not indicated for the evaluation of dizziness
and vertigo without additional neurological complaints because
1 It is too expensive
2 Less than 1 of CTs done for dizziness and vertigo are
abnormal and those patients had associated severe
neurological complaints
3 CT is the best imaging choice for the posterior fossa
4 Acoustic neuromas are well seen on CT
Sixth Annual Intensive Update in Neurology 0915-162016
43
Question 2
Head CT is not indicated for the evaluation of dizziness
and vertigo without additional neurological complaints because
1 It is too expensive
2 Less than 1 of CTs done for dizziness and vertigo are
abnormal and those patients had associated severe
neurological complaints
3 CT is the best imaging choice for the posterior fossa
4 Acoustic neuromas are well seen on CT
Answer 2 See references at end of lecture
Sixth Annual Intensive Update in Neurology 0915-162016
44
Acute Vestibular Syndrome
bull Usually peripheral and can be associated with
hearing loss (labyrinthitis)
bull Timely diagnosis improves recovery (ASAP
prednisone 1mgkgday for ten days and taper 10
mgday ndash Strupp M 2004 NEJM)
bull Avoid vestibular suppressants after 3 days and begin
vestibular rehabilitation to hasten compensation
Sixth Annual Intensive Update in Neurology 0915-162016
45
Question 3
bull Treatment for Acute Vestibular Syndrome
1) oral prednisone 1 mgkgday
2) Treat dehydration if necessary
3) Minimize vestibular suppressants after 3 days
4) Consider vestibular physical therapy
5) All of the above
Sixth Annual Intensive Update in Neurology 0915-162016
46
Question 3
bull Treatment for Acute Vestibular Syndrome
1) oral prednisone 1 mgkgday
2) Treat dehydration if necessary
3) Minimize vestibular suppressants after 3 days
4) Consider vestibular physical therapy
5) All of the above
Answer 5 See references at end of lecture
Sixth Annual Intensive Update in Neurology 0915-162016
47
Menierersquos syndrome
bull Fluctuating low frequency hearing loss
bull Tinnitus andor aural fullness
bull Vertigo lasting hours
Sixth Annual Intensive Update in Neurology 0915-162016
48
Normal
Sixth Annual Intensive Update in Neurology 0915-162016
49
Hydrops
Sixth Annual Intensive Update in Neurology 0915-162016
50
Menierersquos syndrome
ndash Diagnosis- Exclusion (RO acoustic)
ndash 23 of Menierersquos patients will improve spontaneously
ndash Treatment-1500 mgday low sodium triamterene375-
hydrochlorothiazide 25 vestibular suppressants (lorazepam
1 mg tablet sublingual Q 8 prn attacks) off label use
Sixth Annual Intensive Update in Neurology 0915-162016
51
Menierersquos syndrome ndash intratympanic steroids
ndash A newer treatment option for those failing medical management
ndash 90 response rate (Boleus-Aguirre MS Otology Neurotology
2008)
ndash Outcomes data confirm excellent clinical response in 3941
Menierersquos patients treated with intratympanic steroids in 2008
Sixth Annual Intensive Update in Neurology 0915-162016
52
Question 4
bull Menierersquos syndrome diagnosis is based on
1) Recurrent vertigo lasting at least 20 minutes
2) Aural fullness
3) Documented hearing loss
4) Other causes excluded
5) All of the above
Sixth Annual Intensive Update in Neurology 0915-162016
53
Question 4
bull Menierersquos syndrome diagnosis is based on
1) Recurrent vertigo lasting at least 20 minutes
2) Aural fullness
3) Documented hearing loss
4) Other causes excluded
5) All of the above
Answer 5 See references at end
Sixth Annual Intensive Update in Neurology 0915-162016
54
Superior Semicircular Canal Dehiscence (SSCD)
bull Sound andor pressure induced oscillopsia
bull CT reformatted in the plane of the superior canals
shows dehiscence of overlying bone
bull Low frequency ldquoconductive hyperacusisrdquo with elevated
bone thresholds normal reflexes and a conductive loss
Sixth Annual Intensive Update in Neurology 0915-162016
55
SSCD
bull Positive middle ear pressure or loud sound stimulates ampullofugal endolymph flow -canal activation
bull Eye movements are tonic upwards with ipsilateral intorsion
Sixth Annual Intensive Update in Neurology 0915-162016
56
SSCD
bull Valsalva against a closed glottis or jugular compression causes increased intracranial pressure and ampullopetal endolymph flow -canal inhibition
bull Eye movements are tonic downwards with ipsilateral extorsion
Sixth Annual Intensive Update in Neurology 0915-162016
57
Reconstructed left superior semicircular canal
Sixth Annual Intensive Update in Neurology 0915-162016
58
Sixth Annual Intensive Update in Neurology 0915-162016
59
SCHEMATIC DRAWING OF AN OTOLITHIC MACULA
Sixth Annual Intensive Update in Neurology 0915-162016
60
MACULAE OF THE UTRICLESACCULE
Sixth Annual Intensive Update in Neurology 0915-162016
61
HAIR CELLS
Sixth Annual Intensive Update in Neurology 0915-162016
62
SCANNING ELECTRON MICROGRAPH OF OTOCONIA
Sixth Annual Intensive Update in Neurology 0915-162016
63
Ocular VEMP
bull Response to loud clicks (80-110 dB) or forehead tap (Halmagyi and Curthoys 2003)
bull Contralateral oculomotor nucleus (CN III)
bull Inferior oblique muscle contraction (8 msec) detected by 5sec event triggered averaging
bull Noninverting electrode inferior to eye
bull Inverting below non-inverting electrode
bull Ground to forehead
bull Normal subjects have very low response
Sixth Annual Intensive Update in Neurology 0915-162016
64
Clinical applications
bull The o-VEMP is a sensitive test of otolithutricular
function
bull Complements vestibular test procedures that assess
semicircular canals
bull Abnormally robust responses are associated with third
window abnormalities such as semicircular canal
dehiscence
bull Serves as an excellent screening test for semicircular
canal dehiscence
Sixth Annual Intensive Update in Neurology 0915-162016
65
Question 5
bull Superior semicircular canal dehiscence
1) Causes increased ability to hear vibratory sounds
2) Improves hearing
3) Causes meningitis
4) Always needs surgical repair
Sixth Annual Intensive Update in Neurology 0915-162016
66
Question 5
bull Superior semicircular canal dehiscence
1) Causes increased ability to hear vibratory sounds
2) Improves hearing
3) Causes meningitis
4) Always needs surgical repair
Answer 1 References at end of lecture
Sixth Annual Intensive Update in Neurology 0915-162016
67
Enlarged vestibular aqueduct
bull Axial temporal bone CT showing right temporal bone with enlarged vestibular aqueduct (arrow)
bull Vestibular symptoms accompany progressive sensorineural hearing loss in 4-50 of patients in selected series
Sixth Annual Intensive Update in Neurology 0915-162016
68
Migraine
bull Family or personal history of migraine
bull 25 of migraine patients experience spontaneous vertigo (Baloh 1997)
bull Neuhauser criteria(2001) have been used to construct structured diagnostic interviews (Furman 2003)
bull Not always associated with headache
bull Motion sensitivity
bull Medication responsive
Sixth Annual Intensive Update in Neurology 0915-162016
69
ICHD-III beta Criteria for Vestibular Migraine
bull A At least five attacks fulfilling criteria C and D
bull B A current or past history of 11 migraine without aura or 12 migraine with aura
bull C Vestibular symptoms of moderate or severe intensity lasting between 5 minutes and 72 hours
bull D At least 50 associated with one of the following migrainous features
ndash 1 Headaches
ndash 2 Photophobia and phonophobia
ndash 3 Visual Aura
bull E Not better accounted for by another vestibular disorder
Sixth Annual Intensive Update in Neurology 0915-162016
70
Incidence of vertigo in MS
bull True vertigo (the perception of environmental
movement typically rotational) occurs in about
20 of MS patients and is the presenting
manifestation in 5
bull Up to 78 of MS patients report abnormalities
in balance at some point in their illness
Frohman et al Multiple Sclerosis 2003 9250
Sixth Annual Intensive Update in Neurology 0915-162016
71
Vertigo in MS due to demyelination
bull The most common neuroanatomic locations are the
root entry zone of CN VIII (giving absent calorics but
normal hearing and fixation suppression) and the
medial vestibular nucleus
bull Usually treated with steroids and vestibular
suppressants
Brandt 1993 Gass 1998 Thomke 1999
Sixth Annual Intensive Update in Neurology 0915-162016
72
Vertigo in MS
Frohmanrsquos large series (1153) reported that 52 of MS patients with acute vertigo had benign paroxysmal positional vertigo while 32 had new brainstem lesions
To avoid misdiagnoses and inappropriate treatment interventions alternative etiologies should be considered in all acute vertigo episodes in MS
E M Frohman Neurology 2000 and Multiple Sclerosis 2003
Sixth Annual Intensive Update in Neurology 0915-162016
73
Vestibular compensation
bull Acute unilateral or bilateral vestibular loss will prompt compensation thru central cervico-ocular visual and proprioceptive pathways
bull Unilateral loss may require several weeks for compensation to occur and activity especially head movements hastens compensation
bull Vestibular suppressant medication prolongs compensation
Sixth Annual Intensive Update in Neurology 0915-162016
74
Vestibular Rehabilitation
bull Performed by certified physical therapists many sites (Epic order vestibular PT) or check Vestibular Disorders of America website for providers
bull Assessment and treatment of benign positional vertigo
bull Directed rehab programs for central andor peripheral vestibular compensationndash Unilateral vestibulopathies
ndash Bilateral vestibular hypofunction
ndash Mutifactorial balance deficits and fall risk assessment
Sixth Annual Intensive Update in Neurology 0915-162016
75
Summary of whatrsquos new in peripheral vestibular disorders
bull Lateral semicircular canal benign paroxysmal positional vertigo presents with horizontal positioning nystagmus
bull Oral steroids appear to improve outcome in acute vestibular neuritis
bull Intratympanic steroids offer promise in Menierersquos syndrome
bull Superior semicircular canal dehiscence causes pressure and noise induced dizziness VEMP offers diagnostic information
Sixth Annual Intensive Update in Neurology 0915-162016
76
References
BPPVndash White J Benign paroxysmal positional vertigo Cleveland Clinic Journal of
Medicine 71(9)722-728 2004
ndash White J Coale K Catalano P Oas J Diagnosis and management of lateral semicircular canal benign positional vertigo Otolaryngology-Head and Neck Surgery 133 278-284 2005
ndash White J Savvides P Cherian N Oas J Canalith repositioning for benign paroxysmal positional vertigo Otology and Neurotology 26 704-710 2005
ndash White J Oas J Diagnosis and management of lateral semicircular canal conversions during particle repositioning therapy Laryngoscope 115(10) 1895-1897 2005
ndash Acute Vestibular Syndrome Hotson JR Baloh RW N Engl J Med 1998 Sep 3339(10)680-5
ndash Methylprednisolone valacyclovir or the combination for vestibular neuritis Strupp M Zingler VC Arbusow V Niklas D Maag KP Dieterich M Bense S Theil D Jahn K Brandt T N Engl J Med 2004 Jul 22351(4)354-61
ndash Intratympanic steroids for Meacuteniegraveres disease or syndrome Phillips JS Westerberg BCochrane Database Syst Rev 2011 Jul 6(7)CD008514 Review
ndash Sound- andor pressure-induced vertigo due to bone dehiscence of the superior semicircular canal Minor LB Solomon D Zinreich JS Zee DSArch Otolaryngol Head Neck Surg 1998 Mar124(3)249-58
Sixth Annual Intensive Update in Neurology 0915-162016
36
Acute Vestibular Syndrome
bull Rapid unilateral injury to either peripheral or central
vestibular structures produces prolonged vertigo
(days to weeks)
bull Severe vertigo nausea and vomiting spontaneous
nystagmus and postural instability
Sixth Annual Intensive Update in Neurology 0915-162016
37
Acute Vestibular Syndrome
bull 25 of patients with risk factors who present with isolated severe vertigo nystagmus and postural instability in the ER have an acute infarction of the inferior cerebellum (AICA PICA) Central imaging is suggested (Baloh 1998 NEJM)
ndash HTN diabetes smoking age gt65 Afib valvular heart disease occlusive vascular disease
Sixth Annual Intensive Update in Neurology 0915-162016
38
HINTS test
bull Head Impulse
bull Nystagmus
bull Test of Skew
ndash More sensitive than MRI in the diagnosis of acute stroke in
patients with acute vestibular syndrome with at least one risk
factor (Kattah J et al Stroke 2009)
Sixth Annual Intensive Update in Neurology 0915-162016
39
Head Impulse Test
bull No special equipment needed
bull Examiner stands in front of seated patient and
randomly moves head left and right Patient is asked to
focus on examinerrsquos nose
bull Rapid low amplitude thrust from lateral to straight
ahead causes a compensatory saccade when
performed towards the weak side
bull This finding is a permanent feature of unilateral
vestibular loss
Sixth Annual Intensive Update in Neurology 0915-162016
40
Head Impulse Test
Sixth Annual Intensive Update in Neurology 0915-162016
41
Head CT ndash Academy of Emergency Medicine 2013
bull The clinical value of head CT in isolated
dizzinessvertigo without any associated neurological
symptoms is very low
bull Less than 1 of head CTs ordered for dizziness and
vertigo have significant abnormalities and those
patients had severe headache and neurological deficits
in addition to dizziness
bull Forgoing unnecessary emergency head CTs could
save 360 million dollars annually
Sixth Annual Intensive Update in Neurology 0915-162016
42
Question 2
Head CT is not indicated for the evaluation of dizziness
and vertigo without additional neurological complaints because
1 It is too expensive
2 Less than 1 of CTs done for dizziness and vertigo are
abnormal and those patients had associated severe
neurological complaints
3 CT is the best imaging choice for the posterior fossa
4 Acoustic neuromas are well seen on CT
Sixth Annual Intensive Update in Neurology 0915-162016
43
Question 2
Head CT is not indicated for the evaluation of dizziness
and vertigo without additional neurological complaints because
1 It is too expensive
2 Less than 1 of CTs done for dizziness and vertigo are
abnormal and those patients had associated severe
neurological complaints
3 CT is the best imaging choice for the posterior fossa
4 Acoustic neuromas are well seen on CT
Answer 2 See references at end of lecture
Sixth Annual Intensive Update in Neurology 0915-162016
44
Acute Vestibular Syndrome
bull Usually peripheral and can be associated with
hearing loss (labyrinthitis)
bull Timely diagnosis improves recovery (ASAP
prednisone 1mgkgday for ten days and taper 10
mgday ndash Strupp M 2004 NEJM)
bull Avoid vestibular suppressants after 3 days and begin
vestibular rehabilitation to hasten compensation
Sixth Annual Intensive Update in Neurology 0915-162016
45
Question 3
bull Treatment for Acute Vestibular Syndrome
1) oral prednisone 1 mgkgday
2) Treat dehydration if necessary
3) Minimize vestibular suppressants after 3 days
4) Consider vestibular physical therapy
5) All of the above
Sixth Annual Intensive Update in Neurology 0915-162016
46
Question 3
bull Treatment for Acute Vestibular Syndrome
1) oral prednisone 1 mgkgday
2) Treat dehydration if necessary
3) Minimize vestibular suppressants after 3 days
4) Consider vestibular physical therapy
5) All of the above
Answer 5 See references at end of lecture
Sixth Annual Intensive Update in Neurology 0915-162016
47
Menierersquos syndrome
bull Fluctuating low frequency hearing loss
bull Tinnitus andor aural fullness
bull Vertigo lasting hours
Sixth Annual Intensive Update in Neurology 0915-162016
48
Normal
Sixth Annual Intensive Update in Neurology 0915-162016
49
Hydrops
Sixth Annual Intensive Update in Neurology 0915-162016
50
Menierersquos syndrome
ndash Diagnosis- Exclusion (RO acoustic)
ndash 23 of Menierersquos patients will improve spontaneously
ndash Treatment-1500 mgday low sodium triamterene375-
hydrochlorothiazide 25 vestibular suppressants (lorazepam
1 mg tablet sublingual Q 8 prn attacks) off label use
Sixth Annual Intensive Update in Neurology 0915-162016
51
Menierersquos syndrome ndash intratympanic steroids
ndash A newer treatment option for those failing medical management
ndash 90 response rate (Boleus-Aguirre MS Otology Neurotology
2008)
ndash Outcomes data confirm excellent clinical response in 3941
Menierersquos patients treated with intratympanic steroids in 2008
Sixth Annual Intensive Update in Neurology 0915-162016
52
Question 4
bull Menierersquos syndrome diagnosis is based on
1) Recurrent vertigo lasting at least 20 minutes
2) Aural fullness
3) Documented hearing loss
4) Other causes excluded
5) All of the above
Sixth Annual Intensive Update in Neurology 0915-162016
53
Question 4
bull Menierersquos syndrome diagnosis is based on
1) Recurrent vertigo lasting at least 20 minutes
2) Aural fullness
3) Documented hearing loss
4) Other causes excluded
5) All of the above
Answer 5 See references at end
Sixth Annual Intensive Update in Neurology 0915-162016
54
Superior Semicircular Canal Dehiscence (SSCD)
bull Sound andor pressure induced oscillopsia
bull CT reformatted in the plane of the superior canals
shows dehiscence of overlying bone
bull Low frequency ldquoconductive hyperacusisrdquo with elevated
bone thresholds normal reflexes and a conductive loss
Sixth Annual Intensive Update in Neurology 0915-162016
55
SSCD
bull Positive middle ear pressure or loud sound stimulates ampullofugal endolymph flow -canal activation
bull Eye movements are tonic upwards with ipsilateral intorsion
Sixth Annual Intensive Update in Neurology 0915-162016
56
SSCD
bull Valsalva against a closed glottis or jugular compression causes increased intracranial pressure and ampullopetal endolymph flow -canal inhibition
bull Eye movements are tonic downwards with ipsilateral extorsion
Sixth Annual Intensive Update in Neurology 0915-162016
57
Reconstructed left superior semicircular canal
Sixth Annual Intensive Update in Neurology 0915-162016
58
Sixth Annual Intensive Update in Neurology 0915-162016
59
SCHEMATIC DRAWING OF AN OTOLITHIC MACULA
Sixth Annual Intensive Update in Neurology 0915-162016
60
MACULAE OF THE UTRICLESACCULE
Sixth Annual Intensive Update in Neurology 0915-162016
61
HAIR CELLS
Sixth Annual Intensive Update in Neurology 0915-162016
62
SCANNING ELECTRON MICROGRAPH OF OTOCONIA
Sixth Annual Intensive Update in Neurology 0915-162016
63
Ocular VEMP
bull Response to loud clicks (80-110 dB) or forehead tap (Halmagyi and Curthoys 2003)
bull Contralateral oculomotor nucleus (CN III)
bull Inferior oblique muscle contraction (8 msec) detected by 5sec event triggered averaging
bull Noninverting electrode inferior to eye
bull Inverting below non-inverting electrode
bull Ground to forehead
bull Normal subjects have very low response
Sixth Annual Intensive Update in Neurology 0915-162016
64
Clinical applications
bull The o-VEMP is a sensitive test of otolithutricular
function
bull Complements vestibular test procedures that assess
semicircular canals
bull Abnormally robust responses are associated with third
window abnormalities such as semicircular canal
dehiscence
bull Serves as an excellent screening test for semicircular
canal dehiscence
Sixth Annual Intensive Update in Neurology 0915-162016
65
Question 5
bull Superior semicircular canal dehiscence
1) Causes increased ability to hear vibratory sounds
2) Improves hearing
3) Causes meningitis
4) Always needs surgical repair
Sixth Annual Intensive Update in Neurology 0915-162016
66
Question 5
bull Superior semicircular canal dehiscence
1) Causes increased ability to hear vibratory sounds
2) Improves hearing
3) Causes meningitis
4) Always needs surgical repair
Answer 1 References at end of lecture
Sixth Annual Intensive Update in Neurology 0915-162016
67
Enlarged vestibular aqueduct
bull Axial temporal bone CT showing right temporal bone with enlarged vestibular aqueduct (arrow)
bull Vestibular symptoms accompany progressive sensorineural hearing loss in 4-50 of patients in selected series
Sixth Annual Intensive Update in Neurology 0915-162016
68
Migraine
bull Family or personal history of migraine
bull 25 of migraine patients experience spontaneous vertigo (Baloh 1997)
bull Neuhauser criteria(2001) have been used to construct structured diagnostic interviews (Furman 2003)
bull Not always associated with headache
bull Motion sensitivity
bull Medication responsive
Sixth Annual Intensive Update in Neurology 0915-162016
69
ICHD-III beta Criteria for Vestibular Migraine
bull A At least five attacks fulfilling criteria C and D
bull B A current or past history of 11 migraine without aura or 12 migraine with aura
bull C Vestibular symptoms of moderate or severe intensity lasting between 5 minutes and 72 hours
bull D At least 50 associated with one of the following migrainous features
ndash 1 Headaches
ndash 2 Photophobia and phonophobia
ndash 3 Visual Aura
bull E Not better accounted for by another vestibular disorder
Sixth Annual Intensive Update in Neurology 0915-162016
70
Incidence of vertigo in MS
bull True vertigo (the perception of environmental
movement typically rotational) occurs in about
20 of MS patients and is the presenting
manifestation in 5
bull Up to 78 of MS patients report abnormalities
in balance at some point in their illness
Frohman et al Multiple Sclerosis 2003 9250
Sixth Annual Intensive Update in Neurology 0915-162016
71
Vertigo in MS due to demyelination
bull The most common neuroanatomic locations are the
root entry zone of CN VIII (giving absent calorics but
normal hearing and fixation suppression) and the
medial vestibular nucleus
bull Usually treated with steroids and vestibular
suppressants
Brandt 1993 Gass 1998 Thomke 1999
Sixth Annual Intensive Update in Neurology 0915-162016
72
Vertigo in MS
Frohmanrsquos large series (1153) reported that 52 of MS patients with acute vertigo had benign paroxysmal positional vertigo while 32 had new brainstem lesions
To avoid misdiagnoses and inappropriate treatment interventions alternative etiologies should be considered in all acute vertigo episodes in MS
E M Frohman Neurology 2000 and Multiple Sclerosis 2003
Sixth Annual Intensive Update in Neurology 0915-162016
73
Vestibular compensation
bull Acute unilateral or bilateral vestibular loss will prompt compensation thru central cervico-ocular visual and proprioceptive pathways
bull Unilateral loss may require several weeks for compensation to occur and activity especially head movements hastens compensation
bull Vestibular suppressant medication prolongs compensation
Sixth Annual Intensive Update in Neurology 0915-162016
74
Vestibular Rehabilitation
bull Performed by certified physical therapists many sites (Epic order vestibular PT) or check Vestibular Disorders of America website for providers
bull Assessment and treatment of benign positional vertigo
bull Directed rehab programs for central andor peripheral vestibular compensationndash Unilateral vestibulopathies
ndash Bilateral vestibular hypofunction
ndash Mutifactorial balance deficits and fall risk assessment
Sixth Annual Intensive Update in Neurology 0915-162016
75
Summary of whatrsquos new in peripheral vestibular disorders
bull Lateral semicircular canal benign paroxysmal positional vertigo presents with horizontal positioning nystagmus
bull Oral steroids appear to improve outcome in acute vestibular neuritis
bull Intratympanic steroids offer promise in Menierersquos syndrome
bull Superior semicircular canal dehiscence causes pressure and noise induced dizziness VEMP offers diagnostic information
Sixth Annual Intensive Update in Neurology 0915-162016
76
References
BPPVndash White J Benign paroxysmal positional vertigo Cleveland Clinic Journal of
Medicine 71(9)722-728 2004
ndash White J Coale K Catalano P Oas J Diagnosis and management of lateral semicircular canal benign positional vertigo Otolaryngology-Head and Neck Surgery 133 278-284 2005
ndash White J Savvides P Cherian N Oas J Canalith repositioning for benign paroxysmal positional vertigo Otology and Neurotology 26 704-710 2005
ndash White J Oas J Diagnosis and management of lateral semicircular canal conversions during particle repositioning therapy Laryngoscope 115(10) 1895-1897 2005
ndash Acute Vestibular Syndrome Hotson JR Baloh RW N Engl J Med 1998 Sep 3339(10)680-5
ndash Methylprednisolone valacyclovir or the combination for vestibular neuritis Strupp M Zingler VC Arbusow V Niklas D Maag KP Dieterich M Bense S Theil D Jahn K Brandt T N Engl J Med 2004 Jul 22351(4)354-61
ndash Intratympanic steroids for Meacuteniegraveres disease or syndrome Phillips JS Westerberg BCochrane Database Syst Rev 2011 Jul 6(7)CD008514 Review
ndash Sound- andor pressure-induced vertigo due to bone dehiscence of the superior semicircular canal Minor LB Solomon D Zinreich JS Zee DSArch Otolaryngol Head Neck Surg 1998 Mar124(3)249-58
Sixth Annual Intensive Update in Neurology 0915-162016
37
Acute Vestibular Syndrome
bull 25 of patients with risk factors who present with isolated severe vertigo nystagmus and postural instability in the ER have an acute infarction of the inferior cerebellum (AICA PICA) Central imaging is suggested (Baloh 1998 NEJM)
ndash HTN diabetes smoking age gt65 Afib valvular heart disease occlusive vascular disease
Sixth Annual Intensive Update in Neurology 0915-162016
38
HINTS test
bull Head Impulse
bull Nystagmus
bull Test of Skew
ndash More sensitive than MRI in the diagnosis of acute stroke in
patients with acute vestibular syndrome with at least one risk
factor (Kattah J et al Stroke 2009)
Sixth Annual Intensive Update in Neurology 0915-162016
39
Head Impulse Test
bull No special equipment needed
bull Examiner stands in front of seated patient and
randomly moves head left and right Patient is asked to
focus on examinerrsquos nose
bull Rapid low amplitude thrust from lateral to straight
ahead causes a compensatory saccade when
performed towards the weak side
bull This finding is a permanent feature of unilateral
vestibular loss
Sixth Annual Intensive Update in Neurology 0915-162016
40
Head Impulse Test
Sixth Annual Intensive Update in Neurology 0915-162016
41
Head CT ndash Academy of Emergency Medicine 2013
bull The clinical value of head CT in isolated
dizzinessvertigo without any associated neurological
symptoms is very low
bull Less than 1 of head CTs ordered for dizziness and
vertigo have significant abnormalities and those
patients had severe headache and neurological deficits
in addition to dizziness
bull Forgoing unnecessary emergency head CTs could
save 360 million dollars annually
Sixth Annual Intensive Update in Neurology 0915-162016
42
Question 2
Head CT is not indicated for the evaluation of dizziness
and vertigo without additional neurological complaints because
1 It is too expensive
2 Less than 1 of CTs done for dizziness and vertigo are
abnormal and those patients had associated severe
neurological complaints
3 CT is the best imaging choice for the posterior fossa
4 Acoustic neuromas are well seen on CT
Sixth Annual Intensive Update in Neurology 0915-162016
43
Question 2
Head CT is not indicated for the evaluation of dizziness
and vertigo without additional neurological complaints because
1 It is too expensive
2 Less than 1 of CTs done for dizziness and vertigo are
abnormal and those patients had associated severe
neurological complaints
3 CT is the best imaging choice for the posterior fossa
4 Acoustic neuromas are well seen on CT
Answer 2 See references at end of lecture
Sixth Annual Intensive Update in Neurology 0915-162016
44
Acute Vestibular Syndrome
bull Usually peripheral and can be associated with
hearing loss (labyrinthitis)
bull Timely diagnosis improves recovery (ASAP
prednisone 1mgkgday for ten days and taper 10
mgday ndash Strupp M 2004 NEJM)
bull Avoid vestibular suppressants after 3 days and begin
vestibular rehabilitation to hasten compensation
Sixth Annual Intensive Update in Neurology 0915-162016
45
Question 3
bull Treatment for Acute Vestibular Syndrome
1) oral prednisone 1 mgkgday
2) Treat dehydration if necessary
3) Minimize vestibular suppressants after 3 days
4) Consider vestibular physical therapy
5) All of the above
Sixth Annual Intensive Update in Neurology 0915-162016
46
Question 3
bull Treatment for Acute Vestibular Syndrome
1) oral prednisone 1 mgkgday
2) Treat dehydration if necessary
3) Minimize vestibular suppressants after 3 days
4) Consider vestibular physical therapy
5) All of the above
Answer 5 See references at end of lecture
Sixth Annual Intensive Update in Neurology 0915-162016
47
Menierersquos syndrome
bull Fluctuating low frequency hearing loss
bull Tinnitus andor aural fullness
bull Vertigo lasting hours
Sixth Annual Intensive Update in Neurology 0915-162016
48
Normal
Sixth Annual Intensive Update in Neurology 0915-162016
49
Hydrops
Sixth Annual Intensive Update in Neurology 0915-162016
50
Menierersquos syndrome
ndash Diagnosis- Exclusion (RO acoustic)
ndash 23 of Menierersquos patients will improve spontaneously
ndash Treatment-1500 mgday low sodium triamterene375-
hydrochlorothiazide 25 vestibular suppressants (lorazepam
1 mg tablet sublingual Q 8 prn attacks) off label use
Sixth Annual Intensive Update in Neurology 0915-162016
51
Menierersquos syndrome ndash intratympanic steroids
ndash A newer treatment option for those failing medical management
ndash 90 response rate (Boleus-Aguirre MS Otology Neurotology
2008)
ndash Outcomes data confirm excellent clinical response in 3941
Menierersquos patients treated with intratympanic steroids in 2008
Sixth Annual Intensive Update in Neurology 0915-162016
52
Question 4
bull Menierersquos syndrome diagnosis is based on
1) Recurrent vertigo lasting at least 20 minutes
2) Aural fullness
3) Documented hearing loss
4) Other causes excluded
5) All of the above
Sixth Annual Intensive Update in Neurology 0915-162016
53
Question 4
bull Menierersquos syndrome diagnosis is based on
1) Recurrent vertigo lasting at least 20 minutes
2) Aural fullness
3) Documented hearing loss
4) Other causes excluded
5) All of the above
Answer 5 See references at end
Sixth Annual Intensive Update in Neurology 0915-162016
54
Superior Semicircular Canal Dehiscence (SSCD)
bull Sound andor pressure induced oscillopsia
bull CT reformatted in the plane of the superior canals
shows dehiscence of overlying bone
bull Low frequency ldquoconductive hyperacusisrdquo with elevated
bone thresholds normal reflexes and a conductive loss
Sixth Annual Intensive Update in Neurology 0915-162016
55
SSCD
bull Positive middle ear pressure or loud sound stimulates ampullofugal endolymph flow -canal activation
bull Eye movements are tonic upwards with ipsilateral intorsion
Sixth Annual Intensive Update in Neurology 0915-162016
56
SSCD
bull Valsalva against a closed glottis or jugular compression causes increased intracranial pressure and ampullopetal endolymph flow -canal inhibition
bull Eye movements are tonic downwards with ipsilateral extorsion
Sixth Annual Intensive Update in Neurology 0915-162016
57
Reconstructed left superior semicircular canal
Sixth Annual Intensive Update in Neurology 0915-162016
58
Sixth Annual Intensive Update in Neurology 0915-162016
59
SCHEMATIC DRAWING OF AN OTOLITHIC MACULA
Sixth Annual Intensive Update in Neurology 0915-162016
60
MACULAE OF THE UTRICLESACCULE
Sixth Annual Intensive Update in Neurology 0915-162016
61
HAIR CELLS
Sixth Annual Intensive Update in Neurology 0915-162016
62
SCANNING ELECTRON MICROGRAPH OF OTOCONIA
Sixth Annual Intensive Update in Neurology 0915-162016
63
Ocular VEMP
bull Response to loud clicks (80-110 dB) or forehead tap (Halmagyi and Curthoys 2003)
bull Contralateral oculomotor nucleus (CN III)
bull Inferior oblique muscle contraction (8 msec) detected by 5sec event triggered averaging
bull Noninverting electrode inferior to eye
bull Inverting below non-inverting electrode
bull Ground to forehead
bull Normal subjects have very low response
Sixth Annual Intensive Update in Neurology 0915-162016
64
Clinical applications
bull The o-VEMP is a sensitive test of otolithutricular
function
bull Complements vestibular test procedures that assess
semicircular canals
bull Abnormally robust responses are associated with third
window abnormalities such as semicircular canal
dehiscence
bull Serves as an excellent screening test for semicircular
canal dehiscence
Sixth Annual Intensive Update in Neurology 0915-162016
65
Question 5
bull Superior semicircular canal dehiscence
1) Causes increased ability to hear vibratory sounds
2) Improves hearing
3) Causes meningitis
4) Always needs surgical repair
Sixth Annual Intensive Update in Neurology 0915-162016
66
Question 5
bull Superior semicircular canal dehiscence
1) Causes increased ability to hear vibratory sounds
2) Improves hearing
3) Causes meningitis
4) Always needs surgical repair
Answer 1 References at end of lecture
Sixth Annual Intensive Update in Neurology 0915-162016
67
Enlarged vestibular aqueduct
bull Axial temporal bone CT showing right temporal bone with enlarged vestibular aqueduct (arrow)
bull Vestibular symptoms accompany progressive sensorineural hearing loss in 4-50 of patients in selected series
Sixth Annual Intensive Update in Neurology 0915-162016
68
Migraine
bull Family or personal history of migraine
bull 25 of migraine patients experience spontaneous vertigo (Baloh 1997)
bull Neuhauser criteria(2001) have been used to construct structured diagnostic interviews (Furman 2003)
bull Not always associated with headache
bull Motion sensitivity
bull Medication responsive
Sixth Annual Intensive Update in Neurology 0915-162016
69
ICHD-III beta Criteria for Vestibular Migraine
bull A At least five attacks fulfilling criteria C and D
bull B A current or past history of 11 migraine without aura or 12 migraine with aura
bull C Vestibular symptoms of moderate or severe intensity lasting between 5 minutes and 72 hours
bull D At least 50 associated with one of the following migrainous features
ndash 1 Headaches
ndash 2 Photophobia and phonophobia
ndash 3 Visual Aura
bull E Not better accounted for by another vestibular disorder
Sixth Annual Intensive Update in Neurology 0915-162016
70
Incidence of vertigo in MS
bull True vertigo (the perception of environmental
movement typically rotational) occurs in about
20 of MS patients and is the presenting
manifestation in 5
bull Up to 78 of MS patients report abnormalities
in balance at some point in their illness
Frohman et al Multiple Sclerosis 2003 9250
Sixth Annual Intensive Update in Neurology 0915-162016
71
Vertigo in MS due to demyelination
bull The most common neuroanatomic locations are the
root entry zone of CN VIII (giving absent calorics but
normal hearing and fixation suppression) and the
medial vestibular nucleus
bull Usually treated with steroids and vestibular
suppressants
Brandt 1993 Gass 1998 Thomke 1999
Sixth Annual Intensive Update in Neurology 0915-162016
72
Vertigo in MS
Frohmanrsquos large series (1153) reported that 52 of MS patients with acute vertigo had benign paroxysmal positional vertigo while 32 had new brainstem lesions
To avoid misdiagnoses and inappropriate treatment interventions alternative etiologies should be considered in all acute vertigo episodes in MS
E M Frohman Neurology 2000 and Multiple Sclerosis 2003
Sixth Annual Intensive Update in Neurology 0915-162016
73
Vestibular compensation
bull Acute unilateral or bilateral vestibular loss will prompt compensation thru central cervico-ocular visual and proprioceptive pathways
bull Unilateral loss may require several weeks for compensation to occur and activity especially head movements hastens compensation
bull Vestibular suppressant medication prolongs compensation
Sixth Annual Intensive Update in Neurology 0915-162016
74
Vestibular Rehabilitation
bull Performed by certified physical therapists many sites (Epic order vestibular PT) or check Vestibular Disorders of America website for providers
bull Assessment and treatment of benign positional vertigo
bull Directed rehab programs for central andor peripheral vestibular compensationndash Unilateral vestibulopathies
ndash Bilateral vestibular hypofunction
ndash Mutifactorial balance deficits and fall risk assessment
Sixth Annual Intensive Update in Neurology 0915-162016
75
Summary of whatrsquos new in peripheral vestibular disorders
bull Lateral semicircular canal benign paroxysmal positional vertigo presents with horizontal positioning nystagmus
bull Oral steroids appear to improve outcome in acute vestibular neuritis
bull Intratympanic steroids offer promise in Menierersquos syndrome
bull Superior semicircular canal dehiscence causes pressure and noise induced dizziness VEMP offers diagnostic information
Sixth Annual Intensive Update in Neurology 0915-162016
76
References
BPPVndash White J Benign paroxysmal positional vertigo Cleveland Clinic Journal of
Medicine 71(9)722-728 2004
ndash White J Coale K Catalano P Oas J Diagnosis and management of lateral semicircular canal benign positional vertigo Otolaryngology-Head and Neck Surgery 133 278-284 2005
ndash White J Savvides P Cherian N Oas J Canalith repositioning for benign paroxysmal positional vertigo Otology and Neurotology 26 704-710 2005
ndash White J Oas J Diagnosis and management of lateral semicircular canal conversions during particle repositioning therapy Laryngoscope 115(10) 1895-1897 2005
ndash Acute Vestibular Syndrome Hotson JR Baloh RW N Engl J Med 1998 Sep 3339(10)680-5
ndash Methylprednisolone valacyclovir or the combination for vestibular neuritis Strupp M Zingler VC Arbusow V Niklas D Maag KP Dieterich M Bense S Theil D Jahn K Brandt T N Engl J Med 2004 Jul 22351(4)354-61
ndash Intratympanic steroids for Meacuteniegraveres disease or syndrome Phillips JS Westerberg BCochrane Database Syst Rev 2011 Jul 6(7)CD008514 Review
ndash Sound- andor pressure-induced vertigo due to bone dehiscence of the superior semicircular canal Minor LB Solomon D Zinreich JS Zee DSArch Otolaryngol Head Neck Surg 1998 Mar124(3)249-58
Sixth Annual Intensive Update in Neurology 0915-162016
38
HINTS test
bull Head Impulse
bull Nystagmus
bull Test of Skew
ndash More sensitive than MRI in the diagnosis of acute stroke in
patients with acute vestibular syndrome with at least one risk
factor (Kattah J et al Stroke 2009)
Sixth Annual Intensive Update in Neurology 0915-162016
39
Head Impulse Test
bull No special equipment needed
bull Examiner stands in front of seated patient and
randomly moves head left and right Patient is asked to
focus on examinerrsquos nose
bull Rapid low amplitude thrust from lateral to straight
ahead causes a compensatory saccade when
performed towards the weak side
bull This finding is a permanent feature of unilateral
vestibular loss
Sixth Annual Intensive Update in Neurology 0915-162016
40
Head Impulse Test
Sixth Annual Intensive Update in Neurology 0915-162016
41
Head CT ndash Academy of Emergency Medicine 2013
bull The clinical value of head CT in isolated
dizzinessvertigo without any associated neurological
symptoms is very low
bull Less than 1 of head CTs ordered for dizziness and
vertigo have significant abnormalities and those
patients had severe headache and neurological deficits
in addition to dizziness
bull Forgoing unnecessary emergency head CTs could
save 360 million dollars annually
Sixth Annual Intensive Update in Neurology 0915-162016
42
Question 2
Head CT is not indicated for the evaluation of dizziness
and vertigo without additional neurological complaints because
1 It is too expensive
2 Less than 1 of CTs done for dizziness and vertigo are
abnormal and those patients had associated severe
neurological complaints
3 CT is the best imaging choice for the posterior fossa
4 Acoustic neuromas are well seen on CT
Sixth Annual Intensive Update in Neurology 0915-162016
43
Question 2
Head CT is not indicated for the evaluation of dizziness
and vertigo without additional neurological complaints because
1 It is too expensive
2 Less than 1 of CTs done for dizziness and vertigo are
abnormal and those patients had associated severe
neurological complaints
3 CT is the best imaging choice for the posterior fossa
4 Acoustic neuromas are well seen on CT
Answer 2 See references at end of lecture
Sixth Annual Intensive Update in Neurology 0915-162016
44
Acute Vestibular Syndrome
bull Usually peripheral and can be associated with
hearing loss (labyrinthitis)
bull Timely diagnosis improves recovery (ASAP
prednisone 1mgkgday for ten days and taper 10
mgday ndash Strupp M 2004 NEJM)
bull Avoid vestibular suppressants after 3 days and begin
vestibular rehabilitation to hasten compensation
Sixth Annual Intensive Update in Neurology 0915-162016
45
Question 3
bull Treatment for Acute Vestibular Syndrome
1) oral prednisone 1 mgkgday
2) Treat dehydration if necessary
3) Minimize vestibular suppressants after 3 days
4) Consider vestibular physical therapy
5) All of the above
Sixth Annual Intensive Update in Neurology 0915-162016
46
Question 3
bull Treatment for Acute Vestibular Syndrome
1) oral prednisone 1 mgkgday
2) Treat dehydration if necessary
3) Minimize vestibular suppressants after 3 days
4) Consider vestibular physical therapy
5) All of the above
Answer 5 See references at end of lecture
Sixth Annual Intensive Update in Neurology 0915-162016
47
Menierersquos syndrome
bull Fluctuating low frequency hearing loss
bull Tinnitus andor aural fullness
bull Vertigo lasting hours
Sixth Annual Intensive Update in Neurology 0915-162016
48
Normal
Sixth Annual Intensive Update in Neurology 0915-162016
49
Hydrops
Sixth Annual Intensive Update in Neurology 0915-162016
50
Menierersquos syndrome
ndash Diagnosis- Exclusion (RO acoustic)
ndash 23 of Menierersquos patients will improve spontaneously
ndash Treatment-1500 mgday low sodium triamterene375-
hydrochlorothiazide 25 vestibular suppressants (lorazepam
1 mg tablet sublingual Q 8 prn attacks) off label use
Sixth Annual Intensive Update in Neurology 0915-162016
51
Menierersquos syndrome ndash intratympanic steroids
ndash A newer treatment option for those failing medical management
ndash 90 response rate (Boleus-Aguirre MS Otology Neurotology
2008)
ndash Outcomes data confirm excellent clinical response in 3941
Menierersquos patients treated with intratympanic steroids in 2008
Sixth Annual Intensive Update in Neurology 0915-162016
52
Question 4
bull Menierersquos syndrome diagnosis is based on
1) Recurrent vertigo lasting at least 20 minutes
2) Aural fullness
3) Documented hearing loss
4) Other causes excluded
5) All of the above
Sixth Annual Intensive Update in Neurology 0915-162016
53
Question 4
bull Menierersquos syndrome diagnosis is based on
1) Recurrent vertigo lasting at least 20 minutes
2) Aural fullness
3) Documented hearing loss
4) Other causes excluded
5) All of the above
Answer 5 See references at end
Sixth Annual Intensive Update in Neurology 0915-162016
54
Superior Semicircular Canal Dehiscence (SSCD)
bull Sound andor pressure induced oscillopsia
bull CT reformatted in the plane of the superior canals
shows dehiscence of overlying bone
bull Low frequency ldquoconductive hyperacusisrdquo with elevated
bone thresholds normal reflexes and a conductive loss
Sixth Annual Intensive Update in Neurology 0915-162016
55
SSCD
bull Positive middle ear pressure or loud sound stimulates ampullofugal endolymph flow -canal activation
bull Eye movements are tonic upwards with ipsilateral intorsion
Sixth Annual Intensive Update in Neurology 0915-162016
56
SSCD
bull Valsalva against a closed glottis or jugular compression causes increased intracranial pressure and ampullopetal endolymph flow -canal inhibition
bull Eye movements are tonic downwards with ipsilateral extorsion
Sixth Annual Intensive Update in Neurology 0915-162016
57
Reconstructed left superior semicircular canal
Sixth Annual Intensive Update in Neurology 0915-162016
58
Sixth Annual Intensive Update in Neurology 0915-162016
59
SCHEMATIC DRAWING OF AN OTOLITHIC MACULA
Sixth Annual Intensive Update in Neurology 0915-162016
60
MACULAE OF THE UTRICLESACCULE
Sixth Annual Intensive Update in Neurology 0915-162016
61
HAIR CELLS
Sixth Annual Intensive Update in Neurology 0915-162016
62
SCANNING ELECTRON MICROGRAPH OF OTOCONIA
Sixth Annual Intensive Update in Neurology 0915-162016
63
Ocular VEMP
bull Response to loud clicks (80-110 dB) or forehead tap (Halmagyi and Curthoys 2003)
bull Contralateral oculomotor nucleus (CN III)
bull Inferior oblique muscle contraction (8 msec) detected by 5sec event triggered averaging
bull Noninverting electrode inferior to eye
bull Inverting below non-inverting electrode
bull Ground to forehead
bull Normal subjects have very low response
Sixth Annual Intensive Update in Neurology 0915-162016
64
Clinical applications
bull The o-VEMP is a sensitive test of otolithutricular
function
bull Complements vestibular test procedures that assess
semicircular canals
bull Abnormally robust responses are associated with third
window abnormalities such as semicircular canal
dehiscence
bull Serves as an excellent screening test for semicircular
canal dehiscence
Sixth Annual Intensive Update in Neurology 0915-162016
65
Question 5
bull Superior semicircular canal dehiscence
1) Causes increased ability to hear vibratory sounds
2) Improves hearing
3) Causes meningitis
4) Always needs surgical repair
Sixth Annual Intensive Update in Neurology 0915-162016
66
Question 5
bull Superior semicircular canal dehiscence
1) Causes increased ability to hear vibratory sounds
2) Improves hearing
3) Causes meningitis
4) Always needs surgical repair
Answer 1 References at end of lecture
Sixth Annual Intensive Update in Neurology 0915-162016
67
Enlarged vestibular aqueduct
bull Axial temporal bone CT showing right temporal bone with enlarged vestibular aqueduct (arrow)
bull Vestibular symptoms accompany progressive sensorineural hearing loss in 4-50 of patients in selected series
Sixth Annual Intensive Update in Neurology 0915-162016
68
Migraine
bull Family or personal history of migraine
bull 25 of migraine patients experience spontaneous vertigo (Baloh 1997)
bull Neuhauser criteria(2001) have been used to construct structured diagnostic interviews (Furman 2003)
bull Not always associated with headache
bull Motion sensitivity
bull Medication responsive
Sixth Annual Intensive Update in Neurology 0915-162016
69
ICHD-III beta Criteria for Vestibular Migraine
bull A At least five attacks fulfilling criteria C and D
bull B A current or past history of 11 migraine without aura or 12 migraine with aura
bull C Vestibular symptoms of moderate or severe intensity lasting between 5 minutes and 72 hours
bull D At least 50 associated with one of the following migrainous features
ndash 1 Headaches
ndash 2 Photophobia and phonophobia
ndash 3 Visual Aura
bull E Not better accounted for by another vestibular disorder
Sixth Annual Intensive Update in Neurology 0915-162016
70
Incidence of vertigo in MS
bull True vertigo (the perception of environmental
movement typically rotational) occurs in about
20 of MS patients and is the presenting
manifestation in 5
bull Up to 78 of MS patients report abnormalities
in balance at some point in their illness
Frohman et al Multiple Sclerosis 2003 9250
Sixth Annual Intensive Update in Neurology 0915-162016
71
Vertigo in MS due to demyelination
bull The most common neuroanatomic locations are the
root entry zone of CN VIII (giving absent calorics but
normal hearing and fixation suppression) and the
medial vestibular nucleus
bull Usually treated with steroids and vestibular
suppressants
Brandt 1993 Gass 1998 Thomke 1999
Sixth Annual Intensive Update in Neurology 0915-162016
72
Vertigo in MS
Frohmanrsquos large series (1153) reported that 52 of MS patients with acute vertigo had benign paroxysmal positional vertigo while 32 had new brainstem lesions
To avoid misdiagnoses and inappropriate treatment interventions alternative etiologies should be considered in all acute vertigo episodes in MS
E M Frohman Neurology 2000 and Multiple Sclerosis 2003
Sixth Annual Intensive Update in Neurology 0915-162016
73
Vestibular compensation
bull Acute unilateral or bilateral vestibular loss will prompt compensation thru central cervico-ocular visual and proprioceptive pathways
bull Unilateral loss may require several weeks for compensation to occur and activity especially head movements hastens compensation
bull Vestibular suppressant medication prolongs compensation
Sixth Annual Intensive Update in Neurology 0915-162016
74
Vestibular Rehabilitation
bull Performed by certified physical therapists many sites (Epic order vestibular PT) or check Vestibular Disorders of America website for providers
bull Assessment and treatment of benign positional vertigo
bull Directed rehab programs for central andor peripheral vestibular compensationndash Unilateral vestibulopathies
ndash Bilateral vestibular hypofunction
ndash Mutifactorial balance deficits and fall risk assessment
Sixth Annual Intensive Update in Neurology 0915-162016
75
Summary of whatrsquos new in peripheral vestibular disorders
bull Lateral semicircular canal benign paroxysmal positional vertigo presents with horizontal positioning nystagmus
bull Oral steroids appear to improve outcome in acute vestibular neuritis
bull Intratympanic steroids offer promise in Menierersquos syndrome
bull Superior semicircular canal dehiscence causes pressure and noise induced dizziness VEMP offers diagnostic information
Sixth Annual Intensive Update in Neurology 0915-162016
76
References
BPPVndash White J Benign paroxysmal positional vertigo Cleveland Clinic Journal of
Medicine 71(9)722-728 2004
ndash White J Coale K Catalano P Oas J Diagnosis and management of lateral semicircular canal benign positional vertigo Otolaryngology-Head and Neck Surgery 133 278-284 2005
ndash White J Savvides P Cherian N Oas J Canalith repositioning for benign paroxysmal positional vertigo Otology and Neurotology 26 704-710 2005
ndash White J Oas J Diagnosis and management of lateral semicircular canal conversions during particle repositioning therapy Laryngoscope 115(10) 1895-1897 2005
ndash Acute Vestibular Syndrome Hotson JR Baloh RW N Engl J Med 1998 Sep 3339(10)680-5
ndash Methylprednisolone valacyclovir or the combination for vestibular neuritis Strupp M Zingler VC Arbusow V Niklas D Maag KP Dieterich M Bense S Theil D Jahn K Brandt T N Engl J Med 2004 Jul 22351(4)354-61
ndash Intratympanic steroids for Meacuteniegraveres disease or syndrome Phillips JS Westerberg BCochrane Database Syst Rev 2011 Jul 6(7)CD008514 Review
ndash Sound- andor pressure-induced vertigo due to bone dehiscence of the superior semicircular canal Minor LB Solomon D Zinreich JS Zee DSArch Otolaryngol Head Neck Surg 1998 Mar124(3)249-58
Sixth Annual Intensive Update in Neurology 0915-162016
39
Head Impulse Test
bull No special equipment needed
bull Examiner stands in front of seated patient and
randomly moves head left and right Patient is asked to
focus on examinerrsquos nose
bull Rapid low amplitude thrust from lateral to straight
ahead causes a compensatory saccade when
performed towards the weak side
bull This finding is a permanent feature of unilateral
vestibular loss
Sixth Annual Intensive Update in Neurology 0915-162016
40
Head Impulse Test
Sixth Annual Intensive Update in Neurology 0915-162016
41
Head CT ndash Academy of Emergency Medicine 2013
bull The clinical value of head CT in isolated
dizzinessvertigo without any associated neurological
symptoms is very low
bull Less than 1 of head CTs ordered for dizziness and
vertigo have significant abnormalities and those
patients had severe headache and neurological deficits
in addition to dizziness
bull Forgoing unnecessary emergency head CTs could
save 360 million dollars annually
Sixth Annual Intensive Update in Neurology 0915-162016
42
Question 2
Head CT is not indicated for the evaluation of dizziness
and vertigo without additional neurological complaints because
1 It is too expensive
2 Less than 1 of CTs done for dizziness and vertigo are
abnormal and those patients had associated severe
neurological complaints
3 CT is the best imaging choice for the posterior fossa
4 Acoustic neuromas are well seen on CT
Sixth Annual Intensive Update in Neurology 0915-162016
43
Question 2
Head CT is not indicated for the evaluation of dizziness
and vertigo without additional neurological complaints because
1 It is too expensive
2 Less than 1 of CTs done for dizziness and vertigo are
abnormal and those patients had associated severe
neurological complaints
3 CT is the best imaging choice for the posterior fossa
4 Acoustic neuromas are well seen on CT
Answer 2 See references at end of lecture
Sixth Annual Intensive Update in Neurology 0915-162016
44
Acute Vestibular Syndrome
bull Usually peripheral and can be associated with
hearing loss (labyrinthitis)
bull Timely diagnosis improves recovery (ASAP
prednisone 1mgkgday for ten days and taper 10
mgday ndash Strupp M 2004 NEJM)
bull Avoid vestibular suppressants after 3 days and begin
vestibular rehabilitation to hasten compensation
Sixth Annual Intensive Update in Neurology 0915-162016
45
Question 3
bull Treatment for Acute Vestibular Syndrome
1) oral prednisone 1 mgkgday
2) Treat dehydration if necessary
3) Minimize vestibular suppressants after 3 days
4) Consider vestibular physical therapy
5) All of the above
Sixth Annual Intensive Update in Neurology 0915-162016
46
Question 3
bull Treatment for Acute Vestibular Syndrome
1) oral prednisone 1 mgkgday
2) Treat dehydration if necessary
3) Minimize vestibular suppressants after 3 days
4) Consider vestibular physical therapy
5) All of the above
Answer 5 See references at end of lecture
Sixth Annual Intensive Update in Neurology 0915-162016
47
Menierersquos syndrome
bull Fluctuating low frequency hearing loss
bull Tinnitus andor aural fullness
bull Vertigo lasting hours
Sixth Annual Intensive Update in Neurology 0915-162016
48
Normal
Sixth Annual Intensive Update in Neurology 0915-162016
49
Hydrops
Sixth Annual Intensive Update in Neurology 0915-162016
50
Menierersquos syndrome
ndash Diagnosis- Exclusion (RO acoustic)
ndash 23 of Menierersquos patients will improve spontaneously
ndash Treatment-1500 mgday low sodium triamterene375-
hydrochlorothiazide 25 vestibular suppressants (lorazepam
1 mg tablet sublingual Q 8 prn attacks) off label use
Sixth Annual Intensive Update in Neurology 0915-162016
51
Menierersquos syndrome ndash intratympanic steroids
ndash A newer treatment option for those failing medical management
ndash 90 response rate (Boleus-Aguirre MS Otology Neurotology
2008)
ndash Outcomes data confirm excellent clinical response in 3941
Menierersquos patients treated with intratympanic steroids in 2008
Sixth Annual Intensive Update in Neurology 0915-162016
52
Question 4
bull Menierersquos syndrome diagnosis is based on
1) Recurrent vertigo lasting at least 20 minutes
2) Aural fullness
3) Documented hearing loss
4) Other causes excluded
5) All of the above
Sixth Annual Intensive Update in Neurology 0915-162016
53
Question 4
bull Menierersquos syndrome diagnosis is based on
1) Recurrent vertigo lasting at least 20 minutes
2) Aural fullness
3) Documented hearing loss
4) Other causes excluded
5) All of the above
Answer 5 See references at end
Sixth Annual Intensive Update in Neurology 0915-162016
54
Superior Semicircular Canal Dehiscence (SSCD)
bull Sound andor pressure induced oscillopsia
bull CT reformatted in the plane of the superior canals
shows dehiscence of overlying bone
bull Low frequency ldquoconductive hyperacusisrdquo with elevated
bone thresholds normal reflexes and a conductive loss
Sixth Annual Intensive Update in Neurology 0915-162016
55
SSCD
bull Positive middle ear pressure or loud sound stimulates ampullofugal endolymph flow -canal activation
bull Eye movements are tonic upwards with ipsilateral intorsion
Sixth Annual Intensive Update in Neurology 0915-162016
56
SSCD
bull Valsalva against a closed glottis or jugular compression causes increased intracranial pressure and ampullopetal endolymph flow -canal inhibition
bull Eye movements are tonic downwards with ipsilateral extorsion
Sixth Annual Intensive Update in Neurology 0915-162016
57
Reconstructed left superior semicircular canal
Sixth Annual Intensive Update in Neurology 0915-162016
58
Sixth Annual Intensive Update in Neurology 0915-162016
59
SCHEMATIC DRAWING OF AN OTOLITHIC MACULA
Sixth Annual Intensive Update in Neurology 0915-162016
60
MACULAE OF THE UTRICLESACCULE
Sixth Annual Intensive Update in Neurology 0915-162016
61
HAIR CELLS
Sixth Annual Intensive Update in Neurology 0915-162016
62
SCANNING ELECTRON MICROGRAPH OF OTOCONIA
Sixth Annual Intensive Update in Neurology 0915-162016
63
Ocular VEMP
bull Response to loud clicks (80-110 dB) or forehead tap (Halmagyi and Curthoys 2003)
bull Contralateral oculomotor nucleus (CN III)
bull Inferior oblique muscle contraction (8 msec) detected by 5sec event triggered averaging
bull Noninverting electrode inferior to eye
bull Inverting below non-inverting electrode
bull Ground to forehead
bull Normal subjects have very low response
Sixth Annual Intensive Update in Neurology 0915-162016
64
Clinical applications
bull The o-VEMP is a sensitive test of otolithutricular
function
bull Complements vestibular test procedures that assess
semicircular canals
bull Abnormally robust responses are associated with third
window abnormalities such as semicircular canal
dehiscence
bull Serves as an excellent screening test for semicircular
canal dehiscence
Sixth Annual Intensive Update in Neurology 0915-162016
65
Question 5
bull Superior semicircular canal dehiscence
1) Causes increased ability to hear vibratory sounds
2) Improves hearing
3) Causes meningitis
4) Always needs surgical repair
Sixth Annual Intensive Update in Neurology 0915-162016
66
Question 5
bull Superior semicircular canal dehiscence
1) Causes increased ability to hear vibratory sounds
2) Improves hearing
3) Causes meningitis
4) Always needs surgical repair
Answer 1 References at end of lecture
Sixth Annual Intensive Update in Neurology 0915-162016
67
Enlarged vestibular aqueduct
bull Axial temporal bone CT showing right temporal bone with enlarged vestibular aqueduct (arrow)
bull Vestibular symptoms accompany progressive sensorineural hearing loss in 4-50 of patients in selected series
Sixth Annual Intensive Update in Neurology 0915-162016
68
Migraine
bull Family or personal history of migraine
bull 25 of migraine patients experience spontaneous vertigo (Baloh 1997)
bull Neuhauser criteria(2001) have been used to construct structured diagnostic interviews (Furman 2003)
bull Not always associated with headache
bull Motion sensitivity
bull Medication responsive
Sixth Annual Intensive Update in Neurology 0915-162016
69
ICHD-III beta Criteria for Vestibular Migraine
bull A At least five attacks fulfilling criteria C and D
bull B A current or past history of 11 migraine without aura or 12 migraine with aura
bull C Vestibular symptoms of moderate or severe intensity lasting between 5 minutes and 72 hours
bull D At least 50 associated with one of the following migrainous features
ndash 1 Headaches
ndash 2 Photophobia and phonophobia
ndash 3 Visual Aura
bull E Not better accounted for by another vestibular disorder
Sixth Annual Intensive Update in Neurology 0915-162016
70
Incidence of vertigo in MS
bull True vertigo (the perception of environmental
movement typically rotational) occurs in about
20 of MS patients and is the presenting
manifestation in 5
bull Up to 78 of MS patients report abnormalities
in balance at some point in their illness
Frohman et al Multiple Sclerosis 2003 9250
Sixth Annual Intensive Update in Neurology 0915-162016
71
Vertigo in MS due to demyelination
bull The most common neuroanatomic locations are the
root entry zone of CN VIII (giving absent calorics but
normal hearing and fixation suppression) and the
medial vestibular nucleus
bull Usually treated with steroids and vestibular
suppressants
Brandt 1993 Gass 1998 Thomke 1999
Sixth Annual Intensive Update in Neurology 0915-162016
72
Vertigo in MS
Frohmanrsquos large series (1153) reported that 52 of MS patients with acute vertigo had benign paroxysmal positional vertigo while 32 had new brainstem lesions
To avoid misdiagnoses and inappropriate treatment interventions alternative etiologies should be considered in all acute vertigo episodes in MS
E M Frohman Neurology 2000 and Multiple Sclerosis 2003
Sixth Annual Intensive Update in Neurology 0915-162016
73
Vestibular compensation
bull Acute unilateral or bilateral vestibular loss will prompt compensation thru central cervico-ocular visual and proprioceptive pathways
bull Unilateral loss may require several weeks for compensation to occur and activity especially head movements hastens compensation
bull Vestibular suppressant medication prolongs compensation
Sixth Annual Intensive Update in Neurology 0915-162016
74
Vestibular Rehabilitation
bull Performed by certified physical therapists many sites (Epic order vestibular PT) or check Vestibular Disorders of America website for providers
bull Assessment and treatment of benign positional vertigo
bull Directed rehab programs for central andor peripheral vestibular compensationndash Unilateral vestibulopathies
ndash Bilateral vestibular hypofunction
ndash Mutifactorial balance deficits and fall risk assessment
Sixth Annual Intensive Update in Neurology 0915-162016
75
Summary of whatrsquos new in peripheral vestibular disorders
bull Lateral semicircular canal benign paroxysmal positional vertigo presents with horizontal positioning nystagmus
bull Oral steroids appear to improve outcome in acute vestibular neuritis
bull Intratympanic steroids offer promise in Menierersquos syndrome
bull Superior semicircular canal dehiscence causes pressure and noise induced dizziness VEMP offers diagnostic information
Sixth Annual Intensive Update in Neurology 0915-162016
76
References
BPPVndash White J Benign paroxysmal positional vertigo Cleveland Clinic Journal of
Medicine 71(9)722-728 2004
ndash White J Coale K Catalano P Oas J Diagnosis and management of lateral semicircular canal benign positional vertigo Otolaryngology-Head and Neck Surgery 133 278-284 2005
ndash White J Savvides P Cherian N Oas J Canalith repositioning for benign paroxysmal positional vertigo Otology and Neurotology 26 704-710 2005
ndash White J Oas J Diagnosis and management of lateral semicircular canal conversions during particle repositioning therapy Laryngoscope 115(10) 1895-1897 2005
ndash Acute Vestibular Syndrome Hotson JR Baloh RW N Engl J Med 1998 Sep 3339(10)680-5
ndash Methylprednisolone valacyclovir or the combination for vestibular neuritis Strupp M Zingler VC Arbusow V Niklas D Maag KP Dieterich M Bense S Theil D Jahn K Brandt T N Engl J Med 2004 Jul 22351(4)354-61
ndash Intratympanic steroids for Meacuteniegraveres disease or syndrome Phillips JS Westerberg BCochrane Database Syst Rev 2011 Jul 6(7)CD008514 Review
ndash Sound- andor pressure-induced vertigo due to bone dehiscence of the superior semicircular canal Minor LB Solomon D Zinreich JS Zee DSArch Otolaryngol Head Neck Surg 1998 Mar124(3)249-58
Sixth Annual Intensive Update in Neurology 0915-162016
40
Head Impulse Test
Sixth Annual Intensive Update in Neurology 0915-162016
41
Head CT ndash Academy of Emergency Medicine 2013
bull The clinical value of head CT in isolated
dizzinessvertigo without any associated neurological
symptoms is very low
bull Less than 1 of head CTs ordered for dizziness and
vertigo have significant abnormalities and those
patients had severe headache and neurological deficits
in addition to dizziness
bull Forgoing unnecessary emergency head CTs could
save 360 million dollars annually
Sixth Annual Intensive Update in Neurology 0915-162016
42
Question 2
Head CT is not indicated for the evaluation of dizziness
and vertigo without additional neurological complaints because
1 It is too expensive
2 Less than 1 of CTs done for dizziness and vertigo are
abnormal and those patients had associated severe
neurological complaints
3 CT is the best imaging choice for the posterior fossa
4 Acoustic neuromas are well seen on CT
Sixth Annual Intensive Update in Neurology 0915-162016
43
Question 2
Head CT is not indicated for the evaluation of dizziness
and vertigo without additional neurological complaints because
1 It is too expensive
2 Less than 1 of CTs done for dizziness and vertigo are
abnormal and those patients had associated severe
neurological complaints
3 CT is the best imaging choice for the posterior fossa
4 Acoustic neuromas are well seen on CT
Answer 2 See references at end of lecture
Sixth Annual Intensive Update in Neurology 0915-162016
44
Acute Vestibular Syndrome
bull Usually peripheral and can be associated with
hearing loss (labyrinthitis)
bull Timely diagnosis improves recovery (ASAP
prednisone 1mgkgday for ten days and taper 10
mgday ndash Strupp M 2004 NEJM)
bull Avoid vestibular suppressants after 3 days and begin
vestibular rehabilitation to hasten compensation
Sixth Annual Intensive Update in Neurology 0915-162016
45
Question 3
bull Treatment for Acute Vestibular Syndrome
1) oral prednisone 1 mgkgday
2) Treat dehydration if necessary
3) Minimize vestibular suppressants after 3 days
4) Consider vestibular physical therapy
5) All of the above
Sixth Annual Intensive Update in Neurology 0915-162016
46
Question 3
bull Treatment for Acute Vestibular Syndrome
1) oral prednisone 1 mgkgday
2) Treat dehydration if necessary
3) Minimize vestibular suppressants after 3 days
4) Consider vestibular physical therapy
5) All of the above
Answer 5 See references at end of lecture
Sixth Annual Intensive Update in Neurology 0915-162016
47
Menierersquos syndrome
bull Fluctuating low frequency hearing loss
bull Tinnitus andor aural fullness
bull Vertigo lasting hours
Sixth Annual Intensive Update in Neurology 0915-162016
48
Normal
Sixth Annual Intensive Update in Neurology 0915-162016
49
Hydrops
Sixth Annual Intensive Update in Neurology 0915-162016
50
Menierersquos syndrome
ndash Diagnosis- Exclusion (RO acoustic)
ndash 23 of Menierersquos patients will improve spontaneously
ndash Treatment-1500 mgday low sodium triamterene375-
hydrochlorothiazide 25 vestibular suppressants (lorazepam
1 mg tablet sublingual Q 8 prn attacks) off label use
Sixth Annual Intensive Update in Neurology 0915-162016
51
Menierersquos syndrome ndash intratympanic steroids
ndash A newer treatment option for those failing medical management
ndash 90 response rate (Boleus-Aguirre MS Otology Neurotology
2008)
ndash Outcomes data confirm excellent clinical response in 3941
Menierersquos patients treated with intratympanic steroids in 2008
Sixth Annual Intensive Update in Neurology 0915-162016
52
Question 4
bull Menierersquos syndrome diagnosis is based on
1) Recurrent vertigo lasting at least 20 minutes
2) Aural fullness
3) Documented hearing loss
4) Other causes excluded
5) All of the above
Sixth Annual Intensive Update in Neurology 0915-162016
53
Question 4
bull Menierersquos syndrome diagnosis is based on
1) Recurrent vertigo lasting at least 20 minutes
2) Aural fullness
3) Documented hearing loss
4) Other causes excluded
5) All of the above
Answer 5 See references at end
Sixth Annual Intensive Update in Neurology 0915-162016
54
Superior Semicircular Canal Dehiscence (SSCD)
bull Sound andor pressure induced oscillopsia
bull CT reformatted in the plane of the superior canals
shows dehiscence of overlying bone
bull Low frequency ldquoconductive hyperacusisrdquo with elevated
bone thresholds normal reflexes and a conductive loss
Sixth Annual Intensive Update in Neurology 0915-162016
55
SSCD
bull Positive middle ear pressure or loud sound stimulates ampullofugal endolymph flow -canal activation
bull Eye movements are tonic upwards with ipsilateral intorsion
Sixth Annual Intensive Update in Neurology 0915-162016
56
SSCD
bull Valsalva against a closed glottis or jugular compression causes increased intracranial pressure and ampullopetal endolymph flow -canal inhibition
bull Eye movements are tonic downwards with ipsilateral extorsion
Sixth Annual Intensive Update in Neurology 0915-162016
57
Reconstructed left superior semicircular canal
Sixth Annual Intensive Update in Neurology 0915-162016
58
Sixth Annual Intensive Update in Neurology 0915-162016
59
SCHEMATIC DRAWING OF AN OTOLITHIC MACULA
Sixth Annual Intensive Update in Neurology 0915-162016
60
MACULAE OF THE UTRICLESACCULE
Sixth Annual Intensive Update in Neurology 0915-162016
61
HAIR CELLS
Sixth Annual Intensive Update in Neurology 0915-162016
62
SCANNING ELECTRON MICROGRAPH OF OTOCONIA
Sixth Annual Intensive Update in Neurology 0915-162016
63
Ocular VEMP
bull Response to loud clicks (80-110 dB) or forehead tap (Halmagyi and Curthoys 2003)
bull Contralateral oculomotor nucleus (CN III)
bull Inferior oblique muscle contraction (8 msec) detected by 5sec event triggered averaging
bull Noninverting electrode inferior to eye
bull Inverting below non-inverting electrode
bull Ground to forehead
bull Normal subjects have very low response
Sixth Annual Intensive Update in Neurology 0915-162016
64
Clinical applications
bull The o-VEMP is a sensitive test of otolithutricular
function
bull Complements vestibular test procedures that assess
semicircular canals
bull Abnormally robust responses are associated with third
window abnormalities such as semicircular canal
dehiscence
bull Serves as an excellent screening test for semicircular
canal dehiscence
Sixth Annual Intensive Update in Neurology 0915-162016
65
Question 5
bull Superior semicircular canal dehiscence
1) Causes increased ability to hear vibratory sounds
2) Improves hearing
3) Causes meningitis
4) Always needs surgical repair
Sixth Annual Intensive Update in Neurology 0915-162016
66
Question 5
bull Superior semicircular canal dehiscence
1) Causes increased ability to hear vibratory sounds
2) Improves hearing
3) Causes meningitis
4) Always needs surgical repair
Answer 1 References at end of lecture
Sixth Annual Intensive Update in Neurology 0915-162016
67
Enlarged vestibular aqueduct
bull Axial temporal bone CT showing right temporal bone with enlarged vestibular aqueduct (arrow)
bull Vestibular symptoms accompany progressive sensorineural hearing loss in 4-50 of patients in selected series
Sixth Annual Intensive Update in Neurology 0915-162016
68
Migraine
bull Family or personal history of migraine
bull 25 of migraine patients experience spontaneous vertigo (Baloh 1997)
bull Neuhauser criteria(2001) have been used to construct structured diagnostic interviews (Furman 2003)
bull Not always associated with headache
bull Motion sensitivity
bull Medication responsive
Sixth Annual Intensive Update in Neurology 0915-162016
69
ICHD-III beta Criteria for Vestibular Migraine
bull A At least five attacks fulfilling criteria C and D
bull B A current or past history of 11 migraine without aura or 12 migraine with aura
bull C Vestibular symptoms of moderate or severe intensity lasting between 5 minutes and 72 hours
bull D At least 50 associated with one of the following migrainous features
ndash 1 Headaches
ndash 2 Photophobia and phonophobia
ndash 3 Visual Aura
bull E Not better accounted for by another vestibular disorder
Sixth Annual Intensive Update in Neurology 0915-162016
70
Incidence of vertigo in MS
bull True vertigo (the perception of environmental
movement typically rotational) occurs in about
20 of MS patients and is the presenting
manifestation in 5
bull Up to 78 of MS patients report abnormalities
in balance at some point in their illness
Frohman et al Multiple Sclerosis 2003 9250
Sixth Annual Intensive Update in Neurology 0915-162016
71
Vertigo in MS due to demyelination
bull The most common neuroanatomic locations are the
root entry zone of CN VIII (giving absent calorics but
normal hearing and fixation suppression) and the
medial vestibular nucleus
bull Usually treated with steroids and vestibular
suppressants
Brandt 1993 Gass 1998 Thomke 1999
Sixth Annual Intensive Update in Neurology 0915-162016
72
Vertigo in MS
Frohmanrsquos large series (1153) reported that 52 of MS patients with acute vertigo had benign paroxysmal positional vertigo while 32 had new brainstem lesions
To avoid misdiagnoses and inappropriate treatment interventions alternative etiologies should be considered in all acute vertigo episodes in MS
E M Frohman Neurology 2000 and Multiple Sclerosis 2003
Sixth Annual Intensive Update in Neurology 0915-162016
73
Vestibular compensation
bull Acute unilateral or bilateral vestibular loss will prompt compensation thru central cervico-ocular visual and proprioceptive pathways
bull Unilateral loss may require several weeks for compensation to occur and activity especially head movements hastens compensation
bull Vestibular suppressant medication prolongs compensation
Sixth Annual Intensive Update in Neurology 0915-162016
74
Vestibular Rehabilitation
bull Performed by certified physical therapists many sites (Epic order vestibular PT) or check Vestibular Disorders of America website for providers
bull Assessment and treatment of benign positional vertigo
bull Directed rehab programs for central andor peripheral vestibular compensationndash Unilateral vestibulopathies
ndash Bilateral vestibular hypofunction
ndash Mutifactorial balance deficits and fall risk assessment
Sixth Annual Intensive Update in Neurology 0915-162016
75
Summary of whatrsquos new in peripheral vestibular disorders
bull Lateral semicircular canal benign paroxysmal positional vertigo presents with horizontal positioning nystagmus
bull Oral steroids appear to improve outcome in acute vestibular neuritis
bull Intratympanic steroids offer promise in Menierersquos syndrome
bull Superior semicircular canal dehiscence causes pressure and noise induced dizziness VEMP offers diagnostic information
Sixth Annual Intensive Update in Neurology 0915-162016
76
References
BPPVndash White J Benign paroxysmal positional vertigo Cleveland Clinic Journal of
Medicine 71(9)722-728 2004
ndash White J Coale K Catalano P Oas J Diagnosis and management of lateral semicircular canal benign positional vertigo Otolaryngology-Head and Neck Surgery 133 278-284 2005
ndash White J Savvides P Cherian N Oas J Canalith repositioning for benign paroxysmal positional vertigo Otology and Neurotology 26 704-710 2005
ndash White J Oas J Diagnosis and management of lateral semicircular canal conversions during particle repositioning therapy Laryngoscope 115(10) 1895-1897 2005
ndash Acute Vestibular Syndrome Hotson JR Baloh RW N Engl J Med 1998 Sep 3339(10)680-5
ndash Methylprednisolone valacyclovir or the combination for vestibular neuritis Strupp M Zingler VC Arbusow V Niklas D Maag KP Dieterich M Bense S Theil D Jahn K Brandt T N Engl J Med 2004 Jul 22351(4)354-61
ndash Intratympanic steroids for Meacuteniegraveres disease or syndrome Phillips JS Westerberg BCochrane Database Syst Rev 2011 Jul 6(7)CD008514 Review
ndash Sound- andor pressure-induced vertigo due to bone dehiscence of the superior semicircular canal Minor LB Solomon D Zinreich JS Zee DSArch Otolaryngol Head Neck Surg 1998 Mar124(3)249-58
Sixth Annual Intensive Update in Neurology 0915-162016
41
Head CT ndash Academy of Emergency Medicine 2013
bull The clinical value of head CT in isolated
dizzinessvertigo without any associated neurological
symptoms is very low
bull Less than 1 of head CTs ordered for dizziness and
vertigo have significant abnormalities and those
patients had severe headache and neurological deficits
in addition to dizziness
bull Forgoing unnecessary emergency head CTs could
save 360 million dollars annually
Sixth Annual Intensive Update in Neurology 0915-162016
42
Question 2
Head CT is not indicated for the evaluation of dizziness
and vertigo without additional neurological complaints because
1 It is too expensive
2 Less than 1 of CTs done for dizziness and vertigo are
abnormal and those patients had associated severe
neurological complaints
3 CT is the best imaging choice for the posterior fossa
4 Acoustic neuromas are well seen on CT
Sixth Annual Intensive Update in Neurology 0915-162016
43
Question 2
Head CT is not indicated for the evaluation of dizziness
and vertigo without additional neurological complaints because
1 It is too expensive
2 Less than 1 of CTs done for dizziness and vertigo are
abnormal and those patients had associated severe
neurological complaints
3 CT is the best imaging choice for the posterior fossa
4 Acoustic neuromas are well seen on CT
Answer 2 See references at end of lecture
Sixth Annual Intensive Update in Neurology 0915-162016
44
Acute Vestibular Syndrome
bull Usually peripheral and can be associated with
hearing loss (labyrinthitis)
bull Timely diagnosis improves recovery (ASAP
prednisone 1mgkgday for ten days and taper 10
mgday ndash Strupp M 2004 NEJM)
bull Avoid vestibular suppressants after 3 days and begin
vestibular rehabilitation to hasten compensation
Sixth Annual Intensive Update in Neurology 0915-162016
45
Question 3
bull Treatment for Acute Vestibular Syndrome
1) oral prednisone 1 mgkgday
2) Treat dehydration if necessary
3) Minimize vestibular suppressants after 3 days
4) Consider vestibular physical therapy
5) All of the above
Sixth Annual Intensive Update in Neurology 0915-162016
46
Question 3
bull Treatment for Acute Vestibular Syndrome
1) oral prednisone 1 mgkgday
2) Treat dehydration if necessary
3) Minimize vestibular suppressants after 3 days
4) Consider vestibular physical therapy
5) All of the above
Answer 5 See references at end of lecture
Sixth Annual Intensive Update in Neurology 0915-162016
47
Menierersquos syndrome
bull Fluctuating low frequency hearing loss
bull Tinnitus andor aural fullness
bull Vertigo lasting hours
Sixth Annual Intensive Update in Neurology 0915-162016
48
Normal
Sixth Annual Intensive Update in Neurology 0915-162016
49
Hydrops
Sixth Annual Intensive Update in Neurology 0915-162016
50
Menierersquos syndrome
ndash Diagnosis- Exclusion (RO acoustic)
ndash 23 of Menierersquos patients will improve spontaneously
ndash Treatment-1500 mgday low sodium triamterene375-
hydrochlorothiazide 25 vestibular suppressants (lorazepam
1 mg tablet sublingual Q 8 prn attacks) off label use
Sixth Annual Intensive Update in Neurology 0915-162016
51
Menierersquos syndrome ndash intratympanic steroids
ndash A newer treatment option for those failing medical management
ndash 90 response rate (Boleus-Aguirre MS Otology Neurotology
2008)
ndash Outcomes data confirm excellent clinical response in 3941
Menierersquos patients treated with intratympanic steroids in 2008
Sixth Annual Intensive Update in Neurology 0915-162016
52
Question 4
bull Menierersquos syndrome diagnosis is based on
1) Recurrent vertigo lasting at least 20 minutes
2) Aural fullness
3) Documented hearing loss
4) Other causes excluded
5) All of the above
Sixth Annual Intensive Update in Neurology 0915-162016
53
Question 4
bull Menierersquos syndrome diagnosis is based on
1) Recurrent vertigo lasting at least 20 minutes
2) Aural fullness
3) Documented hearing loss
4) Other causes excluded
5) All of the above
Answer 5 See references at end
Sixth Annual Intensive Update in Neurology 0915-162016
54
Superior Semicircular Canal Dehiscence (SSCD)
bull Sound andor pressure induced oscillopsia
bull CT reformatted in the plane of the superior canals
shows dehiscence of overlying bone
bull Low frequency ldquoconductive hyperacusisrdquo with elevated
bone thresholds normal reflexes and a conductive loss
Sixth Annual Intensive Update in Neurology 0915-162016
55
SSCD
bull Positive middle ear pressure or loud sound stimulates ampullofugal endolymph flow -canal activation
bull Eye movements are tonic upwards with ipsilateral intorsion
Sixth Annual Intensive Update in Neurology 0915-162016
56
SSCD
bull Valsalva against a closed glottis or jugular compression causes increased intracranial pressure and ampullopetal endolymph flow -canal inhibition
bull Eye movements are tonic downwards with ipsilateral extorsion
Sixth Annual Intensive Update in Neurology 0915-162016
57
Reconstructed left superior semicircular canal
Sixth Annual Intensive Update in Neurology 0915-162016
58
Sixth Annual Intensive Update in Neurology 0915-162016
59
SCHEMATIC DRAWING OF AN OTOLITHIC MACULA
Sixth Annual Intensive Update in Neurology 0915-162016
60
MACULAE OF THE UTRICLESACCULE
Sixth Annual Intensive Update in Neurology 0915-162016
61
HAIR CELLS
Sixth Annual Intensive Update in Neurology 0915-162016
62
SCANNING ELECTRON MICROGRAPH OF OTOCONIA
Sixth Annual Intensive Update in Neurology 0915-162016
63
Ocular VEMP
bull Response to loud clicks (80-110 dB) or forehead tap (Halmagyi and Curthoys 2003)
bull Contralateral oculomotor nucleus (CN III)
bull Inferior oblique muscle contraction (8 msec) detected by 5sec event triggered averaging
bull Noninverting electrode inferior to eye
bull Inverting below non-inverting electrode
bull Ground to forehead
bull Normal subjects have very low response
Sixth Annual Intensive Update in Neurology 0915-162016
64
Clinical applications
bull The o-VEMP is a sensitive test of otolithutricular
function
bull Complements vestibular test procedures that assess
semicircular canals
bull Abnormally robust responses are associated with third
window abnormalities such as semicircular canal
dehiscence
bull Serves as an excellent screening test for semicircular
canal dehiscence
Sixth Annual Intensive Update in Neurology 0915-162016
65
Question 5
bull Superior semicircular canal dehiscence
1) Causes increased ability to hear vibratory sounds
2) Improves hearing
3) Causes meningitis
4) Always needs surgical repair
Sixth Annual Intensive Update in Neurology 0915-162016
66
Question 5
bull Superior semicircular canal dehiscence
1) Causes increased ability to hear vibratory sounds
2) Improves hearing
3) Causes meningitis
4) Always needs surgical repair
Answer 1 References at end of lecture
Sixth Annual Intensive Update in Neurology 0915-162016
67
Enlarged vestibular aqueduct
bull Axial temporal bone CT showing right temporal bone with enlarged vestibular aqueduct (arrow)
bull Vestibular symptoms accompany progressive sensorineural hearing loss in 4-50 of patients in selected series
Sixth Annual Intensive Update in Neurology 0915-162016
68
Migraine
bull Family or personal history of migraine
bull 25 of migraine patients experience spontaneous vertigo (Baloh 1997)
bull Neuhauser criteria(2001) have been used to construct structured diagnostic interviews (Furman 2003)
bull Not always associated with headache
bull Motion sensitivity
bull Medication responsive
Sixth Annual Intensive Update in Neurology 0915-162016
69
ICHD-III beta Criteria for Vestibular Migraine
bull A At least five attacks fulfilling criteria C and D
bull B A current or past history of 11 migraine without aura or 12 migraine with aura
bull C Vestibular symptoms of moderate or severe intensity lasting between 5 minutes and 72 hours
bull D At least 50 associated with one of the following migrainous features
ndash 1 Headaches
ndash 2 Photophobia and phonophobia
ndash 3 Visual Aura
bull E Not better accounted for by another vestibular disorder
Sixth Annual Intensive Update in Neurology 0915-162016
70
Incidence of vertigo in MS
bull True vertigo (the perception of environmental
movement typically rotational) occurs in about
20 of MS patients and is the presenting
manifestation in 5
bull Up to 78 of MS patients report abnormalities
in balance at some point in their illness
Frohman et al Multiple Sclerosis 2003 9250
Sixth Annual Intensive Update in Neurology 0915-162016
71
Vertigo in MS due to demyelination
bull The most common neuroanatomic locations are the
root entry zone of CN VIII (giving absent calorics but
normal hearing and fixation suppression) and the
medial vestibular nucleus
bull Usually treated with steroids and vestibular
suppressants
Brandt 1993 Gass 1998 Thomke 1999
Sixth Annual Intensive Update in Neurology 0915-162016
72
Vertigo in MS
Frohmanrsquos large series (1153) reported that 52 of MS patients with acute vertigo had benign paroxysmal positional vertigo while 32 had new brainstem lesions
To avoid misdiagnoses and inappropriate treatment interventions alternative etiologies should be considered in all acute vertigo episodes in MS
E M Frohman Neurology 2000 and Multiple Sclerosis 2003
Sixth Annual Intensive Update in Neurology 0915-162016
73
Vestibular compensation
bull Acute unilateral or bilateral vestibular loss will prompt compensation thru central cervico-ocular visual and proprioceptive pathways
bull Unilateral loss may require several weeks for compensation to occur and activity especially head movements hastens compensation
bull Vestibular suppressant medication prolongs compensation
Sixth Annual Intensive Update in Neurology 0915-162016
74
Vestibular Rehabilitation
bull Performed by certified physical therapists many sites (Epic order vestibular PT) or check Vestibular Disorders of America website for providers
bull Assessment and treatment of benign positional vertigo
bull Directed rehab programs for central andor peripheral vestibular compensationndash Unilateral vestibulopathies
ndash Bilateral vestibular hypofunction
ndash Mutifactorial balance deficits and fall risk assessment
Sixth Annual Intensive Update in Neurology 0915-162016
75
Summary of whatrsquos new in peripheral vestibular disorders
bull Lateral semicircular canal benign paroxysmal positional vertigo presents with horizontal positioning nystagmus
bull Oral steroids appear to improve outcome in acute vestibular neuritis
bull Intratympanic steroids offer promise in Menierersquos syndrome
bull Superior semicircular canal dehiscence causes pressure and noise induced dizziness VEMP offers diagnostic information
Sixth Annual Intensive Update in Neurology 0915-162016
76
References
BPPVndash White J Benign paroxysmal positional vertigo Cleveland Clinic Journal of
Medicine 71(9)722-728 2004
ndash White J Coale K Catalano P Oas J Diagnosis and management of lateral semicircular canal benign positional vertigo Otolaryngology-Head and Neck Surgery 133 278-284 2005
ndash White J Savvides P Cherian N Oas J Canalith repositioning for benign paroxysmal positional vertigo Otology and Neurotology 26 704-710 2005
ndash White J Oas J Diagnosis and management of lateral semicircular canal conversions during particle repositioning therapy Laryngoscope 115(10) 1895-1897 2005
ndash Acute Vestibular Syndrome Hotson JR Baloh RW N Engl J Med 1998 Sep 3339(10)680-5
ndash Methylprednisolone valacyclovir or the combination for vestibular neuritis Strupp M Zingler VC Arbusow V Niklas D Maag KP Dieterich M Bense S Theil D Jahn K Brandt T N Engl J Med 2004 Jul 22351(4)354-61
ndash Intratympanic steroids for Meacuteniegraveres disease or syndrome Phillips JS Westerberg BCochrane Database Syst Rev 2011 Jul 6(7)CD008514 Review
ndash Sound- andor pressure-induced vertigo due to bone dehiscence of the superior semicircular canal Minor LB Solomon D Zinreich JS Zee DSArch Otolaryngol Head Neck Surg 1998 Mar124(3)249-58
Sixth Annual Intensive Update in Neurology 0915-162016
42
Question 2
Head CT is not indicated for the evaluation of dizziness
and vertigo without additional neurological complaints because
1 It is too expensive
2 Less than 1 of CTs done for dizziness and vertigo are
abnormal and those patients had associated severe
neurological complaints
3 CT is the best imaging choice for the posterior fossa
4 Acoustic neuromas are well seen on CT
Sixth Annual Intensive Update in Neurology 0915-162016
43
Question 2
Head CT is not indicated for the evaluation of dizziness
and vertigo without additional neurological complaints because
1 It is too expensive
2 Less than 1 of CTs done for dizziness and vertigo are
abnormal and those patients had associated severe
neurological complaints
3 CT is the best imaging choice for the posterior fossa
4 Acoustic neuromas are well seen on CT
Answer 2 See references at end of lecture
Sixth Annual Intensive Update in Neurology 0915-162016
44
Acute Vestibular Syndrome
bull Usually peripheral and can be associated with
hearing loss (labyrinthitis)
bull Timely diagnosis improves recovery (ASAP
prednisone 1mgkgday for ten days and taper 10
mgday ndash Strupp M 2004 NEJM)
bull Avoid vestibular suppressants after 3 days and begin
vestibular rehabilitation to hasten compensation
Sixth Annual Intensive Update in Neurology 0915-162016
45
Question 3
bull Treatment for Acute Vestibular Syndrome
1) oral prednisone 1 mgkgday
2) Treat dehydration if necessary
3) Minimize vestibular suppressants after 3 days
4) Consider vestibular physical therapy
5) All of the above
Sixth Annual Intensive Update in Neurology 0915-162016
46
Question 3
bull Treatment for Acute Vestibular Syndrome
1) oral prednisone 1 mgkgday
2) Treat dehydration if necessary
3) Minimize vestibular suppressants after 3 days
4) Consider vestibular physical therapy
5) All of the above
Answer 5 See references at end of lecture
Sixth Annual Intensive Update in Neurology 0915-162016
47
Menierersquos syndrome
bull Fluctuating low frequency hearing loss
bull Tinnitus andor aural fullness
bull Vertigo lasting hours
Sixth Annual Intensive Update in Neurology 0915-162016
48
Normal
Sixth Annual Intensive Update in Neurology 0915-162016
49
Hydrops
Sixth Annual Intensive Update in Neurology 0915-162016
50
Menierersquos syndrome
ndash Diagnosis- Exclusion (RO acoustic)
ndash 23 of Menierersquos patients will improve spontaneously
ndash Treatment-1500 mgday low sodium triamterene375-
hydrochlorothiazide 25 vestibular suppressants (lorazepam
1 mg tablet sublingual Q 8 prn attacks) off label use
Sixth Annual Intensive Update in Neurology 0915-162016
51
Menierersquos syndrome ndash intratympanic steroids
ndash A newer treatment option for those failing medical management
ndash 90 response rate (Boleus-Aguirre MS Otology Neurotology
2008)
ndash Outcomes data confirm excellent clinical response in 3941
Menierersquos patients treated with intratympanic steroids in 2008
Sixth Annual Intensive Update in Neurology 0915-162016
52
Question 4
bull Menierersquos syndrome diagnosis is based on
1) Recurrent vertigo lasting at least 20 minutes
2) Aural fullness
3) Documented hearing loss
4) Other causes excluded
5) All of the above
Sixth Annual Intensive Update in Neurology 0915-162016
53
Question 4
bull Menierersquos syndrome diagnosis is based on
1) Recurrent vertigo lasting at least 20 minutes
2) Aural fullness
3) Documented hearing loss
4) Other causes excluded
5) All of the above
Answer 5 See references at end
Sixth Annual Intensive Update in Neurology 0915-162016
54
Superior Semicircular Canal Dehiscence (SSCD)
bull Sound andor pressure induced oscillopsia
bull CT reformatted in the plane of the superior canals
shows dehiscence of overlying bone
bull Low frequency ldquoconductive hyperacusisrdquo with elevated
bone thresholds normal reflexes and a conductive loss
Sixth Annual Intensive Update in Neurology 0915-162016
55
SSCD
bull Positive middle ear pressure or loud sound stimulates ampullofugal endolymph flow -canal activation
bull Eye movements are tonic upwards with ipsilateral intorsion
Sixth Annual Intensive Update in Neurology 0915-162016
56
SSCD
bull Valsalva against a closed glottis or jugular compression causes increased intracranial pressure and ampullopetal endolymph flow -canal inhibition
bull Eye movements are tonic downwards with ipsilateral extorsion
Sixth Annual Intensive Update in Neurology 0915-162016
57
Reconstructed left superior semicircular canal
Sixth Annual Intensive Update in Neurology 0915-162016
58
Sixth Annual Intensive Update in Neurology 0915-162016
59
SCHEMATIC DRAWING OF AN OTOLITHIC MACULA
Sixth Annual Intensive Update in Neurology 0915-162016
60
MACULAE OF THE UTRICLESACCULE
Sixth Annual Intensive Update in Neurology 0915-162016
61
HAIR CELLS
Sixth Annual Intensive Update in Neurology 0915-162016
62
SCANNING ELECTRON MICROGRAPH OF OTOCONIA
Sixth Annual Intensive Update in Neurology 0915-162016
63
Ocular VEMP
bull Response to loud clicks (80-110 dB) or forehead tap (Halmagyi and Curthoys 2003)
bull Contralateral oculomotor nucleus (CN III)
bull Inferior oblique muscle contraction (8 msec) detected by 5sec event triggered averaging
bull Noninverting electrode inferior to eye
bull Inverting below non-inverting electrode
bull Ground to forehead
bull Normal subjects have very low response
Sixth Annual Intensive Update in Neurology 0915-162016
64
Clinical applications
bull The o-VEMP is a sensitive test of otolithutricular
function
bull Complements vestibular test procedures that assess
semicircular canals
bull Abnormally robust responses are associated with third
window abnormalities such as semicircular canal
dehiscence
bull Serves as an excellent screening test for semicircular
canal dehiscence
Sixth Annual Intensive Update in Neurology 0915-162016
65
Question 5
bull Superior semicircular canal dehiscence
1) Causes increased ability to hear vibratory sounds
2) Improves hearing
3) Causes meningitis
4) Always needs surgical repair
Sixth Annual Intensive Update in Neurology 0915-162016
66
Question 5
bull Superior semicircular canal dehiscence
1) Causes increased ability to hear vibratory sounds
2) Improves hearing
3) Causes meningitis
4) Always needs surgical repair
Answer 1 References at end of lecture
Sixth Annual Intensive Update in Neurology 0915-162016
67
Enlarged vestibular aqueduct
bull Axial temporal bone CT showing right temporal bone with enlarged vestibular aqueduct (arrow)
bull Vestibular symptoms accompany progressive sensorineural hearing loss in 4-50 of patients in selected series
Sixth Annual Intensive Update in Neurology 0915-162016
68
Migraine
bull Family or personal history of migraine
bull 25 of migraine patients experience spontaneous vertigo (Baloh 1997)
bull Neuhauser criteria(2001) have been used to construct structured diagnostic interviews (Furman 2003)
bull Not always associated with headache
bull Motion sensitivity
bull Medication responsive
Sixth Annual Intensive Update in Neurology 0915-162016
69
ICHD-III beta Criteria for Vestibular Migraine
bull A At least five attacks fulfilling criteria C and D
bull B A current or past history of 11 migraine without aura or 12 migraine with aura
bull C Vestibular symptoms of moderate or severe intensity lasting between 5 minutes and 72 hours
bull D At least 50 associated with one of the following migrainous features
ndash 1 Headaches
ndash 2 Photophobia and phonophobia
ndash 3 Visual Aura
bull E Not better accounted for by another vestibular disorder
Sixth Annual Intensive Update in Neurology 0915-162016
70
Incidence of vertigo in MS
bull True vertigo (the perception of environmental
movement typically rotational) occurs in about
20 of MS patients and is the presenting
manifestation in 5
bull Up to 78 of MS patients report abnormalities
in balance at some point in their illness
Frohman et al Multiple Sclerosis 2003 9250
Sixth Annual Intensive Update in Neurology 0915-162016
71
Vertigo in MS due to demyelination
bull The most common neuroanatomic locations are the
root entry zone of CN VIII (giving absent calorics but
normal hearing and fixation suppression) and the
medial vestibular nucleus
bull Usually treated with steroids and vestibular
suppressants
Brandt 1993 Gass 1998 Thomke 1999
Sixth Annual Intensive Update in Neurology 0915-162016
72
Vertigo in MS
Frohmanrsquos large series (1153) reported that 52 of MS patients with acute vertigo had benign paroxysmal positional vertigo while 32 had new brainstem lesions
To avoid misdiagnoses and inappropriate treatment interventions alternative etiologies should be considered in all acute vertigo episodes in MS
E M Frohman Neurology 2000 and Multiple Sclerosis 2003
Sixth Annual Intensive Update in Neurology 0915-162016
73
Vestibular compensation
bull Acute unilateral or bilateral vestibular loss will prompt compensation thru central cervico-ocular visual and proprioceptive pathways
bull Unilateral loss may require several weeks for compensation to occur and activity especially head movements hastens compensation
bull Vestibular suppressant medication prolongs compensation
Sixth Annual Intensive Update in Neurology 0915-162016
74
Vestibular Rehabilitation
bull Performed by certified physical therapists many sites (Epic order vestibular PT) or check Vestibular Disorders of America website for providers
bull Assessment and treatment of benign positional vertigo
bull Directed rehab programs for central andor peripheral vestibular compensationndash Unilateral vestibulopathies
ndash Bilateral vestibular hypofunction
ndash Mutifactorial balance deficits and fall risk assessment
Sixth Annual Intensive Update in Neurology 0915-162016
75
Summary of whatrsquos new in peripheral vestibular disorders
bull Lateral semicircular canal benign paroxysmal positional vertigo presents with horizontal positioning nystagmus
bull Oral steroids appear to improve outcome in acute vestibular neuritis
bull Intratympanic steroids offer promise in Menierersquos syndrome
bull Superior semicircular canal dehiscence causes pressure and noise induced dizziness VEMP offers diagnostic information
Sixth Annual Intensive Update in Neurology 0915-162016
76
References
BPPVndash White J Benign paroxysmal positional vertigo Cleveland Clinic Journal of
Medicine 71(9)722-728 2004
ndash White J Coale K Catalano P Oas J Diagnosis and management of lateral semicircular canal benign positional vertigo Otolaryngology-Head and Neck Surgery 133 278-284 2005
ndash White J Savvides P Cherian N Oas J Canalith repositioning for benign paroxysmal positional vertigo Otology and Neurotology 26 704-710 2005
ndash White J Oas J Diagnosis and management of lateral semicircular canal conversions during particle repositioning therapy Laryngoscope 115(10) 1895-1897 2005
ndash Acute Vestibular Syndrome Hotson JR Baloh RW N Engl J Med 1998 Sep 3339(10)680-5
ndash Methylprednisolone valacyclovir or the combination for vestibular neuritis Strupp M Zingler VC Arbusow V Niklas D Maag KP Dieterich M Bense S Theil D Jahn K Brandt T N Engl J Med 2004 Jul 22351(4)354-61
ndash Intratympanic steroids for Meacuteniegraveres disease or syndrome Phillips JS Westerberg BCochrane Database Syst Rev 2011 Jul 6(7)CD008514 Review
ndash Sound- andor pressure-induced vertigo due to bone dehiscence of the superior semicircular canal Minor LB Solomon D Zinreich JS Zee DSArch Otolaryngol Head Neck Surg 1998 Mar124(3)249-58
Sixth Annual Intensive Update in Neurology 0915-162016
43
Question 2
Head CT is not indicated for the evaluation of dizziness
and vertigo without additional neurological complaints because
1 It is too expensive
2 Less than 1 of CTs done for dizziness and vertigo are
abnormal and those patients had associated severe
neurological complaints
3 CT is the best imaging choice for the posterior fossa
4 Acoustic neuromas are well seen on CT
Answer 2 See references at end of lecture
Sixth Annual Intensive Update in Neurology 0915-162016
44
Acute Vestibular Syndrome
bull Usually peripheral and can be associated with
hearing loss (labyrinthitis)
bull Timely diagnosis improves recovery (ASAP
prednisone 1mgkgday for ten days and taper 10
mgday ndash Strupp M 2004 NEJM)
bull Avoid vestibular suppressants after 3 days and begin
vestibular rehabilitation to hasten compensation
Sixth Annual Intensive Update in Neurology 0915-162016
45
Question 3
bull Treatment for Acute Vestibular Syndrome
1) oral prednisone 1 mgkgday
2) Treat dehydration if necessary
3) Minimize vestibular suppressants after 3 days
4) Consider vestibular physical therapy
5) All of the above
Sixth Annual Intensive Update in Neurology 0915-162016
46
Question 3
bull Treatment for Acute Vestibular Syndrome
1) oral prednisone 1 mgkgday
2) Treat dehydration if necessary
3) Minimize vestibular suppressants after 3 days
4) Consider vestibular physical therapy
5) All of the above
Answer 5 See references at end of lecture
Sixth Annual Intensive Update in Neurology 0915-162016
47
Menierersquos syndrome
bull Fluctuating low frequency hearing loss
bull Tinnitus andor aural fullness
bull Vertigo lasting hours
Sixth Annual Intensive Update in Neurology 0915-162016
48
Normal
Sixth Annual Intensive Update in Neurology 0915-162016
49
Hydrops
Sixth Annual Intensive Update in Neurology 0915-162016
50
Menierersquos syndrome
ndash Diagnosis- Exclusion (RO acoustic)
ndash 23 of Menierersquos patients will improve spontaneously
ndash Treatment-1500 mgday low sodium triamterene375-
hydrochlorothiazide 25 vestibular suppressants (lorazepam
1 mg tablet sublingual Q 8 prn attacks) off label use
Sixth Annual Intensive Update in Neurology 0915-162016
51
Menierersquos syndrome ndash intratympanic steroids
ndash A newer treatment option for those failing medical management
ndash 90 response rate (Boleus-Aguirre MS Otology Neurotology
2008)
ndash Outcomes data confirm excellent clinical response in 3941
Menierersquos patients treated with intratympanic steroids in 2008
Sixth Annual Intensive Update in Neurology 0915-162016
52
Question 4
bull Menierersquos syndrome diagnosis is based on
1) Recurrent vertigo lasting at least 20 minutes
2) Aural fullness
3) Documented hearing loss
4) Other causes excluded
5) All of the above
Sixth Annual Intensive Update in Neurology 0915-162016
53
Question 4
bull Menierersquos syndrome diagnosis is based on
1) Recurrent vertigo lasting at least 20 minutes
2) Aural fullness
3) Documented hearing loss
4) Other causes excluded
5) All of the above
Answer 5 See references at end
Sixth Annual Intensive Update in Neurology 0915-162016
54
Superior Semicircular Canal Dehiscence (SSCD)
bull Sound andor pressure induced oscillopsia
bull CT reformatted in the plane of the superior canals
shows dehiscence of overlying bone
bull Low frequency ldquoconductive hyperacusisrdquo with elevated
bone thresholds normal reflexes and a conductive loss
Sixth Annual Intensive Update in Neurology 0915-162016
55
SSCD
bull Positive middle ear pressure or loud sound stimulates ampullofugal endolymph flow -canal activation
bull Eye movements are tonic upwards with ipsilateral intorsion
Sixth Annual Intensive Update in Neurology 0915-162016
56
SSCD
bull Valsalva against a closed glottis or jugular compression causes increased intracranial pressure and ampullopetal endolymph flow -canal inhibition
bull Eye movements are tonic downwards with ipsilateral extorsion
Sixth Annual Intensive Update in Neurology 0915-162016
57
Reconstructed left superior semicircular canal
Sixth Annual Intensive Update in Neurology 0915-162016
58
Sixth Annual Intensive Update in Neurology 0915-162016
59
SCHEMATIC DRAWING OF AN OTOLITHIC MACULA
Sixth Annual Intensive Update in Neurology 0915-162016
60
MACULAE OF THE UTRICLESACCULE
Sixth Annual Intensive Update in Neurology 0915-162016
61
HAIR CELLS
Sixth Annual Intensive Update in Neurology 0915-162016
62
SCANNING ELECTRON MICROGRAPH OF OTOCONIA
Sixth Annual Intensive Update in Neurology 0915-162016
63
Ocular VEMP
bull Response to loud clicks (80-110 dB) or forehead tap (Halmagyi and Curthoys 2003)
bull Contralateral oculomotor nucleus (CN III)
bull Inferior oblique muscle contraction (8 msec) detected by 5sec event triggered averaging
bull Noninverting electrode inferior to eye
bull Inverting below non-inverting electrode
bull Ground to forehead
bull Normal subjects have very low response
Sixth Annual Intensive Update in Neurology 0915-162016
64
Clinical applications
bull The o-VEMP is a sensitive test of otolithutricular
function
bull Complements vestibular test procedures that assess
semicircular canals
bull Abnormally robust responses are associated with third
window abnormalities such as semicircular canal
dehiscence
bull Serves as an excellent screening test for semicircular
canal dehiscence
Sixth Annual Intensive Update in Neurology 0915-162016
65
Question 5
bull Superior semicircular canal dehiscence
1) Causes increased ability to hear vibratory sounds
2) Improves hearing
3) Causes meningitis
4) Always needs surgical repair
Sixth Annual Intensive Update in Neurology 0915-162016
66
Question 5
bull Superior semicircular canal dehiscence
1) Causes increased ability to hear vibratory sounds
2) Improves hearing
3) Causes meningitis
4) Always needs surgical repair
Answer 1 References at end of lecture
Sixth Annual Intensive Update in Neurology 0915-162016
67
Enlarged vestibular aqueduct
bull Axial temporal bone CT showing right temporal bone with enlarged vestibular aqueduct (arrow)
bull Vestibular symptoms accompany progressive sensorineural hearing loss in 4-50 of patients in selected series
Sixth Annual Intensive Update in Neurology 0915-162016
68
Migraine
bull Family or personal history of migraine
bull 25 of migraine patients experience spontaneous vertigo (Baloh 1997)
bull Neuhauser criteria(2001) have been used to construct structured diagnostic interviews (Furman 2003)
bull Not always associated with headache
bull Motion sensitivity
bull Medication responsive
Sixth Annual Intensive Update in Neurology 0915-162016
69
ICHD-III beta Criteria for Vestibular Migraine
bull A At least five attacks fulfilling criteria C and D
bull B A current or past history of 11 migraine without aura or 12 migraine with aura
bull C Vestibular symptoms of moderate or severe intensity lasting between 5 minutes and 72 hours
bull D At least 50 associated with one of the following migrainous features
ndash 1 Headaches
ndash 2 Photophobia and phonophobia
ndash 3 Visual Aura
bull E Not better accounted for by another vestibular disorder
Sixth Annual Intensive Update in Neurology 0915-162016
70
Incidence of vertigo in MS
bull True vertigo (the perception of environmental
movement typically rotational) occurs in about
20 of MS patients and is the presenting
manifestation in 5
bull Up to 78 of MS patients report abnormalities
in balance at some point in their illness
Frohman et al Multiple Sclerosis 2003 9250
Sixth Annual Intensive Update in Neurology 0915-162016
71
Vertigo in MS due to demyelination
bull The most common neuroanatomic locations are the
root entry zone of CN VIII (giving absent calorics but
normal hearing and fixation suppression) and the
medial vestibular nucleus
bull Usually treated with steroids and vestibular
suppressants
Brandt 1993 Gass 1998 Thomke 1999
Sixth Annual Intensive Update in Neurology 0915-162016
72
Vertigo in MS
Frohmanrsquos large series (1153) reported that 52 of MS patients with acute vertigo had benign paroxysmal positional vertigo while 32 had new brainstem lesions
To avoid misdiagnoses and inappropriate treatment interventions alternative etiologies should be considered in all acute vertigo episodes in MS
E M Frohman Neurology 2000 and Multiple Sclerosis 2003
Sixth Annual Intensive Update in Neurology 0915-162016
73
Vestibular compensation
bull Acute unilateral or bilateral vestibular loss will prompt compensation thru central cervico-ocular visual and proprioceptive pathways
bull Unilateral loss may require several weeks for compensation to occur and activity especially head movements hastens compensation
bull Vestibular suppressant medication prolongs compensation
Sixth Annual Intensive Update in Neurology 0915-162016
74
Vestibular Rehabilitation
bull Performed by certified physical therapists many sites (Epic order vestibular PT) or check Vestibular Disorders of America website for providers
bull Assessment and treatment of benign positional vertigo
bull Directed rehab programs for central andor peripheral vestibular compensationndash Unilateral vestibulopathies
ndash Bilateral vestibular hypofunction
ndash Mutifactorial balance deficits and fall risk assessment
Sixth Annual Intensive Update in Neurology 0915-162016
75
Summary of whatrsquos new in peripheral vestibular disorders
bull Lateral semicircular canal benign paroxysmal positional vertigo presents with horizontal positioning nystagmus
bull Oral steroids appear to improve outcome in acute vestibular neuritis
bull Intratympanic steroids offer promise in Menierersquos syndrome
bull Superior semicircular canal dehiscence causes pressure and noise induced dizziness VEMP offers diagnostic information
Sixth Annual Intensive Update in Neurology 0915-162016
76
References
BPPVndash White J Benign paroxysmal positional vertigo Cleveland Clinic Journal of
Medicine 71(9)722-728 2004
ndash White J Coale K Catalano P Oas J Diagnosis and management of lateral semicircular canal benign positional vertigo Otolaryngology-Head and Neck Surgery 133 278-284 2005
ndash White J Savvides P Cherian N Oas J Canalith repositioning for benign paroxysmal positional vertigo Otology and Neurotology 26 704-710 2005
ndash White J Oas J Diagnosis and management of lateral semicircular canal conversions during particle repositioning therapy Laryngoscope 115(10) 1895-1897 2005
ndash Acute Vestibular Syndrome Hotson JR Baloh RW N Engl J Med 1998 Sep 3339(10)680-5
ndash Methylprednisolone valacyclovir or the combination for vestibular neuritis Strupp M Zingler VC Arbusow V Niklas D Maag KP Dieterich M Bense S Theil D Jahn K Brandt T N Engl J Med 2004 Jul 22351(4)354-61
ndash Intratympanic steroids for Meacuteniegraveres disease or syndrome Phillips JS Westerberg BCochrane Database Syst Rev 2011 Jul 6(7)CD008514 Review
ndash Sound- andor pressure-induced vertigo due to bone dehiscence of the superior semicircular canal Minor LB Solomon D Zinreich JS Zee DSArch Otolaryngol Head Neck Surg 1998 Mar124(3)249-58
Sixth Annual Intensive Update in Neurology 0915-162016
44
Acute Vestibular Syndrome
bull Usually peripheral and can be associated with
hearing loss (labyrinthitis)
bull Timely diagnosis improves recovery (ASAP
prednisone 1mgkgday for ten days and taper 10
mgday ndash Strupp M 2004 NEJM)
bull Avoid vestibular suppressants after 3 days and begin
vestibular rehabilitation to hasten compensation
Sixth Annual Intensive Update in Neurology 0915-162016
45
Question 3
bull Treatment for Acute Vestibular Syndrome
1) oral prednisone 1 mgkgday
2) Treat dehydration if necessary
3) Minimize vestibular suppressants after 3 days
4) Consider vestibular physical therapy
5) All of the above
Sixth Annual Intensive Update in Neurology 0915-162016
46
Question 3
bull Treatment for Acute Vestibular Syndrome
1) oral prednisone 1 mgkgday
2) Treat dehydration if necessary
3) Minimize vestibular suppressants after 3 days
4) Consider vestibular physical therapy
5) All of the above
Answer 5 See references at end of lecture
Sixth Annual Intensive Update in Neurology 0915-162016
47
Menierersquos syndrome
bull Fluctuating low frequency hearing loss
bull Tinnitus andor aural fullness
bull Vertigo lasting hours
Sixth Annual Intensive Update in Neurology 0915-162016
48
Normal
Sixth Annual Intensive Update in Neurology 0915-162016
49
Hydrops
Sixth Annual Intensive Update in Neurology 0915-162016
50
Menierersquos syndrome
ndash Diagnosis- Exclusion (RO acoustic)
ndash 23 of Menierersquos patients will improve spontaneously
ndash Treatment-1500 mgday low sodium triamterene375-
hydrochlorothiazide 25 vestibular suppressants (lorazepam
1 mg tablet sublingual Q 8 prn attacks) off label use
Sixth Annual Intensive Update in Neurology 0915-162016
51
Menierersquos syndrome ndash intratympanic steroids
ndash A newer treatment option for those failing medical management
ndash 90 response rate (Boleus-Aguirre MS Otology Neurotology
2008)
ndash Outcomes data confirm excellent clinical response in 3941
Menierersquos patients treated with intratympanic steroids in 2008
Sixth Annual Intensive Update in Neurology 0915-162016
52
Question 4
bull Menierersquos syndrome diagnosis is based on
1) Recurrent vertigo lasting at least 20 minutes
2) Aural fullness
3) Documented hearing loss
4) Other causes excluded
5) All of the above
Sixth Annual Intensive Update in Neurology 0915-162016
53
Question 4
bull Menierersquos syndrome diagnosis is based on
1) Recurrent vertigo lasting at least 20 minutes
2) Aural fullness
3) Documented hearing loss
4) Other causes excluded
5) All of the above
Answer 5 See references at end
Sixth Annual Intensive Update in Neurology 0915-162016
54
Superior Semicircular Canal Dehiscence (SSCD)
bull Sound andor pressure induced oscillopsia
bull CT reformatted in the plane of the superior canals
shows dehiscence of overlying bone
bull Low frequency ldquoconductive hyperacusisrdquo with elevated
bone thresholds normal reflexes and a conductive loss
Sixth Annual Intensive Update in Neurology 0915-162016
55
SSCD
bull Positive middle ear pressure or loud sound stimulates ampullofugal endolymph flow -canal activation
bull Eye movements are tonic upwards with ipsilateral intorsion
Sixth Annual Intensive Update in Neurology 0915-162016
56
SSCD
bull Valsalva against a closed glottis or jugular compression causes increased intracranial pressure and ampullopetal endolymph flow -canal inhibition
bull Eye movements are tonic downwards with ipsilateral extorsion
Sixth Annual Intensive Update in Neurology 0915-162016
57
Reconstructed left superior semicircular canal
Sixth Annual Intensive Update in Neurology 0915-162016
58
Sixth Annual Intensive Update in Neurology 0915-162016
59
SCHEMATIC DRAWING OF AN OTOLITHIC MACULA
Sixth Annual Intensive Update in Neurology 0915-162016
60
MACULAE OF THE UTRICLESACCULE
Sixth Annual Intensive Update in Neurology 0915-162016
61
HAIR CELLS
Sixth Annual Intensive Update in Neurology 0915-162016
62
SCANNING ELECTRON MICROGRAPH OF OTOCONIA
Sixth Annual Intensive Update in Neurology 0915-162016
63
Ocular VEMP
bull Response to loud clicks (80-110 dB) or forehead tap (Halmagyi and Curthoys 2003)
bull Contralateral oculomotor nucleus (CN III)
bull Inferior oblique muscle contraction (8 msec) detected by 5sec event triggered averaging
bull Noninverting electrode inferior to eye
bull Inverting below non-inverting electrode
bull Ground to forehead
bull Normal subjects have very low response
Sixth Annual Intensive Update in Neurology 0915-162016
64
Clinical applications
bull The o-VEMP is a sensitive test of otolithutricular
function
bull Complements vestibular test procedures that assess
semicircular canals
bull Abnormally robust responses are associated with third
window abnormalities such as semicircular canal
dehiscence
bull Serves as an excellent screening test for semicircular
canal dehiscence
Sixth Annual Intensive Update in Neurology 0915-162016
65
Question 5
bull Superior semicircular canal dehiscence
1) Causes increased ability to hear vibratory sounds
2) Improves hearing
3) Causes meningitis
4) Always needs surgical repair
Sixth Annual Intensive Update in Neurology 0915-162016
66
Question 5
bull Superior semicircular canal dehiscence
1) Causes increased ability to hear vibratory sounds
2) Improves hearing
3) Causes meningitis
4) Always needs surgical repair
Answer 1 References at end of lecture
Sixth Annual Intensive Update in Neurology 0915-162016
67
Enlarged vestibular aqueduct
bull Axial temporal bone CT showing right temporal bone with enlarged vestibular aqueduct (arrow)
bull Vestibular symptoms accompany progressive sensorineural hearing loss in 4-50 of patients in selected series
Sixth Annual Intensive Update in Neurology 0915-162016
68
Migraine
bull Family or personal history of migraine
bull 25 of migraine patients experience spontaneous vertigo (Baloh 1997)
bull Neuhauser criteria(2001) have been used to construct structured diagnostic interviews (Furman 2003)
bull Not always associated with headache
bull Motion sensitivity
bull Medication responsive
Sixth Annual Intensive Update in Neurology 0915-162016
69
ICHD-III beta Criteria for Vestibular Migraine
bull A At least five attacks fulfilling criteria C and D
bull B A current or past history of 11 migraine without aura or 12 migraine with aura
bull C Vestibular symptoms of moderate or severe intensity lasting between 5 minutes and 72 hours
bull D At least 50 associated with one of the following migrainous features
ndash 1 Headaches
ndash 2 Photophobia and phonophobia
ndash 3 Visual Aura
bull E Not better accounted for by another vestibular disorder
Sixth Annual Intensive Update in Neurology 0915-162016
70
Incidence of vertigo in MS
bull True vertigo (the perception of environmental
movement typically rotational) occurs in about
20 of MS patients and is the presenting
manifestation in 5
bull Up to 78 of MS patients report abnormalities
in balance at some point in their illness
Frohman et al Multiple Sclerosis 2003 9250
Sixth Annual Intensive Update in Neurology 0915-162016
71
Vertigo in MS due to demyelination
bull The most common neuroanatomic locations are the
root entry zone of CN VIII (giving absent calorics but
normal hearing and fixation suppression) and the
medial vestibular nucleus
bull Usually treated with steroids and vestibular
suppressants
Brandt 1993 Gass 1998 Thomke 1999
Sixth Annual Intensive Update in Neurology 0915-162016
72
Vertigo in MS
Frohmanrsquos large series (1153) reported that 52 of MS patients with acute vertigo had benign paroxysmal positional vertigo while 32 had new brainstem lesions
To avoid misdiagnoses and inappropriate treatment interventions alternative etiologies should be considered in all acute vertigo episodes in MS
E M Frohman Neurology 2000 and Multiple Sclerosis 2003
Sixth Annual Intensive Update in Neurology 0915-162016
73
Vestibular compensation
bull Acute unilateral or bilateral vestibular loss will prompt compensation thru central cervico-ocular visual and proprioceptive pathways
bull Unilateral loss may require several weeks for compensation to occur and activity especially head movements hastens compensation
bull Vestibular suppressant medication prolongs compensation
Sixth Annual Intensive Update in Neurology 0915-162016
74
Vestibular Rehabilitation
bull Performed by certified physical therapists many sites (Epic order vestibular PT) or check Vestibular Disorders of America website for providers
bull Assessment and treatment of benign positional vertigo
bull Directed rehab programs for central andor peripheral vestibular compensationndash Unilateral vestibulopathies
ndash Bilateral vestibular hypofunction
ndash Mutifactorial balance deficits and fall risk assessment
Sixth Annual Intensive Update in Neurology 0915-162016
75
Summary of whatrsquos new in peripheral vestibular disorders
bull Lateral semicircular canal benign paroxysmal positional vertigo presents with horizontal positioning nystagmus
bull Oral steroids appear to improve outcome in acute vestibular neuritis
bull Intratympanic steroids offer promise in Menierersquos syndrome
bull Superior semicircular canal dehiscence causes pressure and noise induced dizziness VEMP offers diagnostic information
Sixth Annual Intensive Update in Neurology 0915-162016
76
References
BPPVndash White J Benign paroxysmal positional vertigo Cleveland Clinic Journal of
Medicine 71(9)722-728 2004
ndash White J Coale K Catalano P Oas J Diagnosis and management of lateral semicircular canal benign positional vertigo Otolaryngology-Head and Neck Surgery 133 278-284 2005
ndash White J Savvides P Cherian N Oas J Canalith repositioning for benign paroxysmal positional vertigo Otology and Neurotology 26 704-710 2005
ndash White J Oas J Diagnosis and management of lateral semicircular canal conversions during particle repositioning therapy Laryngoscope 115(10) 1895-1897 2005
ndash Acute Vestibular Syndrome Hotson JR Baloh RW N Engl J Med 1998 Sep 3339(10)680-5
ndash Methylprednisolone valacyclovir or the combination for vestibular neuritis Strupp M Zingler VC Arbusow V Niklas D Maag KP Dieterich M Bense S Theil D Jahn K Brandt T N Engl J Med 2004 Jul 22351(4)354-61
ndash Intratympanic steroids for Meacuteniegraveres disease or syndrome Phillips JS Westerberg BCochrane Database Syst Rev 2011 Jul 6(7)CD008514 Review
ndash Sound- andor pressure-induced vertigo due to bone dehiscence of the superior semicircular canal Minor LB Solomon D Zinreich JS Zee DSArch Otolaryngol Head Neck Surg 1998 Mar124(3)249-58
Sixth Annual Intensive Update in Neurology 0915-162016
45
Question 3
bull Treatment for Acute Vestibular Syndrome
1) oral prednisone 1 mgkgday
2) Treat dehydration if necessary
3) Minimize vestibular suppressants after 3 days
4) Consider vestibular physical therapy
5) All of the above
Sixth Annual Intensive Update in Neurology 0915-162016
46
Question 3
bull Treatment for Acute Vestibular Syndrome
1) oral prednisone 1 mgkgday
2) Treat dehydration if necessary
3) Minimize vestibular suppressants after 3 days
4) Consider vestibular physical therapy
5) All of the above
Answer 5 See references at end of lecture
Sixth Annual Intensive Update in Neurology 0915-162016
47
Menierersquos syndrome
bull Fluctuating low frequency hearing loss
bull Tinnitus andor aural fullness
bull Vertigo lasting hours
Sixth Annual Intensive Update in Neurology 0915-162016
48
Normal
Sixth Annual Intensive Update in Neurology 0915-162016
49
Hydrops
Sixth Annual Intensive Update in Neurology 0915-162016
50
Menierersquos syndrome
ndash Diagnosis- Exclusion (RO acoustic)
ndash 23 of Menierersquos patients will improve spontaneously
ndash Treatment-1500 mgday low sodium triamterene375-
hydrochlorothiazide 25 vestibular suppressants (lorazepam
1 mg tablet sublingual Q 8 prn attacks) off label use
Sixth Annual Intensive Update in Neurology 0915-162016
51
Menierersquos syndrome ndash intratympanic steroids
ndash A newer treatment option for those failing medical management
ndash 90 response rate (Boleus-Aguirre MS Otology Neurotology
2008)
ndash Outcomes data confirm excellent clinical response in 3941
Menierersquos patients treated with intratympanic steroids in 2008
Sixth Annual Intensive Update in Neurology 0915-162016
52
Question 4
bull Menierersquos syndrome diagnosis is based on
1) Recurrent vertigo lasting at least 20 minutes
2) Aural fullness
3) Documented hearing loss
4) Other causes excluded
5) All of the above
Sixth Annual Intensive Update in Neurology 0915-162016
53
Question 4
bull Menierersquos syndrome diagnosis is based on
1) Recurrent vertigo lasting at least 20 minutes
2) Aural fullness
3) Documented hearing loss
4) Other causes excluded
5) All of the above
Answer 5 See references at end
Sixth Annual Intensive Update in Neurology 0915-162016
54
Superior Semicircular Canal Dehiscence (SSCD)
bull Sound andor pressure induced oscillopsia
bull CT reformatted in the plane of the superior canals
shows dehiscence of overlying bone
bull Low frequency ldquoconductive hyperacusisrdquo with elevated
bone thresholds normal reflexes and a conductive loss
Sixth Annual Intensive Update in Neurology 0915-162016
55
SSCD
bull Positive middle ear pressure or loud sound stimulates ampullofugal endolymph flow -canal activation
bull Eye movements are tonic upwards with ipsilateral intorsion
Sixth Annual Intensive Update in Neurology 0915-162016
56
SSCD
bull Valsalva against a closed glottis or jugular compression causes increased intracranial pressure and ampullopetal endolymph flow -canal inhibition
bull Eye movements are tonic downwards with ipsilateral extorsion
Sixth Annual Intensive Update in Neurology 0915-162016
57
Reconstructed left superior semicircular canal
Sixth Annual Intensive Update in Neurology 0915-162016
58
Sixth Annual Intensive Update in Neurology 0915-162016
59
SCHEMATIC DRAWING OF AN OTOLITHIC MACULA
Sixth Annual Intensive Update in Neurology 0915-162016
60
MACULAE OF THE UTRICLESACCULE
Sixth Annual Intensive Update in Neurology 0915-162016
61
HAIR CELLS
Sixth Annual Intensive Update in Neurology 0915-162016
62
SCANNING ELECTRON MICROGRAPH OF OTOCONIA
Sixth Annual Intensive Update in Neurology 0915-162016
63
Ocular VEMP
bull Response to loud clicks (80-110 dB) or forehead tap (Halmagyi and Curthoys 2003)
bull Contralateral oculomotor nucleus (CN III)
bull Inferior oblique muscle contraction (8 msec) detected by 5sec event triggered averaging
bull Noninverting electrode inferior to eye
bull Inverting below non-inverting electrode
bull Ground to forehead
bull Normal subjects have very low response
Sixth Annual Intensive Update in Neurology 0915-162016
64
Clinical applications
bull The o-VEMP is a sensitive test of otolithutricular
function
bull Complements vestibular test procedures that assess
semicircular canals
bull Abnormally robust responses are associated with third
window abnormalities such as semicircular canal
dehiscence
bull Serves as an excellent screening test for semicircular
canal dehiscence
Sixth Annual Intensive Update in Neurology 0915-162016
65
Question 5
bull Superior semicircular canal dehiscence
1) Causes increased ability to hear vibratory sounds
2) Improves hearing
3) Causes meningitis
4) Always needs surgical repair
Sixth Annual Intensive Update in Neurology 0915-162016
66
Question 5
bull Superior semicircular canal dehiscence
1) Causes increased ability to hear vibratory sounds
2) Improves hearing
3) Causes meningitis
4) Always needs surgical repair
Answer 1 References at end of lecture
Sixth Annual Intensive Update in Neurology 0915-162016
67
Enlarged vestibular aqueduct
bull Axial temporal bone CT showing right temporal bone with enlarged vestibular aqueduct (arrow)
bull Vestibular symptoms accompany progressive sensorineural hearing loss in 4-50 of patients in selected series
Sixth Annual Intensive Update in Neurology 0915-162016
68
Migraine
bull Family or personal history of migraine
bull 25 of migraine patients experience spontaneous vertigo (Baloh 1997)
bull Neuhauser criteria(2001) have been used to construct structured diagnostic interviews (Furman 2003)
bull Not always associated with headache
bull Motion sensitivity
bull Medication responsive
Sixth Annual Intensive Update in Neurology 0915-162016
69
ICHD-III beta Criteria for Vestibular Migraine
bull A At least five attacks fulfilling criteria C and D
bull B A current or past history of 11 migraine without aura or 12 migraine with aura
bull C Vestibular symptoms of moderate or severe intensity lasting between 5 minutes and 72 hours
bull D At least 50 associated with one of the following migrainous features
ndash 1 Headaches
ndash 2 Photophobia and phonophobia
ndash 3 Visual Aura
bull E Not better accounted for by another vestibular disorder
Sixth Annual Intensive Update in Neurology 0915-162016
70
Incidence of vertigo in MS
bull True vertigo (the perception of environmental
movement typically rotational) occurs in about
20 of MS patients and is the presenting
manifestation in 5
bull Up to 78 of MS patients report abnormalities
in balance at some point in their illness
Frohman et al Multiple Sclerosis 2003 9250
Sixth Annual Intensive Update in Neurology 0915-162016
71
Vertigo in MS due to demyelination
bull The most common neuroanatomic locations are the
root entry zone of CN VIII (giving absent calorics but
normal hearing and fixation suppression) and the
medial vestibular nucleus
bull Usually treated with steroids and vestibular
suppressants
Brandt 1993 Gass 1998 Thomke 1999
Sixth Annual Intensive Update in Neurology 0915-162016
72
Vertigo in MS
Frohmanrsquos large series (1153) reported that 52 of MS patients with acute vertigo had benign paroxysmal positional vertigo while 32 had new brainstem lesions
To avoid misdiagnoses and inappropriate treatment interventions alternative etiologies should be considered in all acute vertigo episodes in MS
E M Frohman Neurology 2000 and Multiple Sclerosis 2003
Sixth Annual Intensive Update in Neurology 0915-162016
73
Vestibular compensation
bull Acute unilateral or bilateral vestibular loss will prompt compensation thru central cervico-ocular visual and proprioceptive pathways
bull Unilateral loss may require several weeks for compensation to occur and activity especially head movements hastens compensation
bull Vestibular suppressant medication prolongs compensation
Sixth Annual Intensive Update in Neurology 0915-162016
74
Vestibular Rehabilitation
bull Performed by certified physical therapists many sites (Epic order vestibular PT) or check Vestibular Disorders of America website for providers
bull Assessment and treatment of benign positional vertigo
bull Directed rehab programs for central andor peripheral vestibular compensationndash Unilateral vestibulopathies
ndash Bilateral vestibular hypofunction
ndash Mutifactorial balance deficits and fall risk assessment
Sixth Annual Intensive Update in Neurology 0915-162016
75
Summary of whatrsquos new in peripheral vestibular disorders
bull Lateral semicircular canal benign paroxysmal positional vertigo presents with horizontal positioning nystagmus
bull Oral steroids appear to improve outcome in acute vestibular neuritis
bull Intratympanic steroids offer promise in Menierersquos syndrome
bull Superior semicircular canal dehiscence causes pressure and noise induced dizziness VEMP offers diagnostic information
Sixth Annual Intensive Update in Neurology 0915-162016
76
References
BPPVndash White J Benign paroxysmal positional vertigo Cleveland Clinic Journal of
Medicine 71(9)722-728 2004
ndash White J Coale K Catalano P Oas J Diagnosis and management of lateral semicircular canal benign positional vertigo Otolaryngology-Head and Neck Surgery 133 278-284 2005
ndash White J Savvides P Cherian N Oas J Canalith repositioning for benign paroxysmal positional vertigo Otology and Neurotology 26 704-710 2005
ndash White J Oas J Diagnosis and management of lateral semicircular canal conversions during particle repositioning therapy Laryngoscope 115(10) 1895-1897 2005
ndash Acute Vestibular Syndrome Hotson JR Baloh RW N Engl J Med 1998 Sep 3339(10)680-5
ndash Methylprednisolone valacyclovir or the combination for vestibular neuritis Strupp M Zingler VC Arbusow V Niklas D Maag KP Dieterich M Bense S Theil D Jahn K Brandt T N Engl J Med 2004 Jul 22351(4)354-61
ndash Intratympanic steroids for Meacuteniegraveres disease or syndrome Phillips JS Westerberg BCochrane Database Syst Rev 2011 Jul 6(7)CD008514 Review
ndash Sound- andor pressure-induced vertigo due to bone dehiscence of the superior semicircular canal Minor LB Solomon D Zinreich JS Zee DSArch Otolaryngol Head Neck Surg 1998 Mar124(3)249-58
Sixth Annual Intensive Update in Neurology 0915-162016
46
Question 3
bull Treatment for Acute Vestibular Syndrome
1) oral prednisone 1 mgkgday
2) Treat dehydration if necessary
3) Minimize vestibular suppressants after 3 days
4) Consider vestibular physical therapy
5) All of the above
Answer 5 See references at end of lecture
Sixth Annual Intensive Update in Neurology 0915-162016
47
Menierersquos syndrome
bull Fluctuating low frequency hearing loss
bull Tinnitus andor aural fullness
bull Vertigo lasting hours
Sixth Annual Intensive Update in Neurology 0915-162016
48
Normal
Sixth Annual Intensive Update in Neurology 0915-162016
49
Hydrops
Sixth Annual Intensive Update in Neurology 0915-162016
50
Menierersquos syndrome
ndash Diagnosis- Exclusion (RO acoustic)
ndash 23 of Menierersquos patients will improve spontaneously
ndash Treatment-1500 mgday low sodium triamterene375-
hydrochlorothiazide 25 vestibular suppressants (lorazepam
1 mg tablet sublingual Q 8 prn attacks) off label use
Sixth Annual Intensive Update in Neurology 0915-162016
51
Menierersquos syndrome ndash intratympanic steroids
ndash A newer treatment option for those failing medical management
ndash 90 response rate (Boleus-Aguirre MS Otology Neurotology
2008)
ndash Outcomes data confirm excellent clinical response in 3941
Menierersquos patients treated with intratympanic steroids in 2008
Sixth Annual Intensive Update in Neurology 0915-162016
52
Question 4
bull Menierersquos syndrome diagnosis is based on
1) Recurrent vertigo lasting at least 20 minutes
2) Aural fullness
3) Documented hearing loss
4) Other causes excluded
5) All of the above
Sixth Annual Intensive Update in Neurology 0915-162016
53
Question 4
bull Menierersquos syndrome diagnosis is based on
1) Recurrent vertigo lasting at least 20 minutes
2) Aural fullness
3) Documented hearing loss
4) Other causes excluded
5) All of the above
Answer 5 See references at end
Sixth Annual Intensive Update in Neurology 0915-162016
54
Superior Semicircular Canal Dehiscence (SSCD)
bull Sound andor pressure induced oscillopsia
bull CT reformatted in the plane of the superior canals
shows dehiscence of overlying bone
bull Low frequency ldquoconductive hyperacusisrdquo with elevated
bone thresholds normal reflexes and a conductive loss
Sixth Annual Intensive Update in Neurology 0915-162016
55
SSCD
bull Positive middle ear pressure or loud sound stimulates ampullofugal endolymph flow -canal activation
bull Eye movements are tonic upwards with ipsilateral intorsion
Sixth Annual Intensive Update in Neurology 0915-162016
56
SSCD
bull Valsalva against a closed glottis or jugular compression causes increased intracranial pressure and ampullopetal endolymph flow -canal inhibition
bull Eye movements are tonic downwards with ipsilateral extorsion
Sixth Annual Intensive Update in Neurology 0915-162016
57
Reconstructed left superior semicircular canal
Sixth Annual Intensive Update in Neurology 0915-162016
58
Sixth Annual Intensive Update in Neurology 0915-162016
59
SCHEMATIC DRAWING OF AN OTOLITHIC MACULA
Sixth Annual Intensive Update in Neurology 0915-162016
60
MACULAE OF THE UTRICLESACCULE
Sixth Annual Intensive Update in Neurology 0915-162016
61
HAIR CELLS
Sixth Annual Intensive Update in Neurology 0915-162016
62
SCANNING ELECTRON MICROGRAPH OF OTOCONIA
Sixth Annual Intensive Update in Neurology 0915-162016
63
Ocular VEMP
bull Response to loud clicks (80-110 dB) or forehead tap (Halmagyi and Curthoys 2003)
bull Contralateral oculomotor nucleus (CN III)
bull Inferior oblique muscle contraction (8 msec) detected by 5sec event triggered averaging
bull Noninverting electrode inferior to eye
bull Inverting below non-inverting electrode
bull Ground to forehead
bull Normal subjects have very low response
Sixth Annual Intensive Update in Neurology 0915-162016
64
Clinical applications
bull The o-VEMP is a sensitive test of otolithutricular
function
bull Complements vestibular test procedures that assess
semicircular canals
bull Abnormally robust responses are associated with third
window abnormalities such as semicircular canal
dehiscence
bull Serves as an excellent screening test for semicircular
canal dehiscence
Sixth Annual Intensive Update in Neurology 0915-162016
65
Question 5
bull Superior semicircular canal dehiscence
1) Causes increased ability to hear vibratory sounds
2) Improves hearing
3) Causes meningitis
4) Always needs surgical repair
Sixth Annual Intensive Update in Neurology 0915-162016
66
Question 5
bull Superior semicircular canal dehiscence
1) Causes increased ability to hear vibratory sounds
2) Improves hearing
3) Causes meningitis
4) Always needs surgical repair
Answer 1 References at end of lecture
Sixth Annual Intensive Update in Neurology 0915-162016
67
Enlarged vestibular aqueduct
bull Axial temporal bone CT showing right temporal bone with enlarged vestibular aqueduct (arrow)
bull Vestibular symptoms accompany progressive sensorineural hearing loss in 4-50 of patients in selected series
Sixth Annual Intensive Update in Neurology 0915-162016
68
Migraine
bull Family or personal history of migraine
bull 25 of migraine patients experience spontaneous vertigo (Baloh 1997)
bull Neuhauser criteria(2001) have been used to construct structured diagnostic interviews (Furman 2003)
bull Not always associated with headache
bull Motion sensitivity
bull Medication responsive
Sixth Annual Intensive Update in Neurology 0915-162016
69
ICHD-III beta Criteria for Vestibular Migraine
bull A At least five attacks fulfilling criteria C and D
bull B A current or past history of 11 migraine without aura or 12 migraine with aura
bull C Vestibular symptoms of moderate or severe intensity lasting between 5 minutes and 72 hours
bull D At least 50 associated with one of the following migrainous features
ndash 1 Headaches
ndash 2 Photophobia and phonophobia
ndash 3 Visual Aura
bull E Not better accounted for by another vestibular disorder
Sixth Annual Intensive Update in Neurology 0915-162016
70
Incidence of vertigo in MS
bull True vertigo (the perception of environmental
movement typically rotational) occurs in about
20 of MS patients and is the presenting
manifestation in 5
bull Up to 78 of MS patients report abnormalities
in balance at some point in their illness
Frohman et al Multiple Sclerosis 2003 9250
Sixth Annual Intensive Update in Neurology 0915-162016
71
Vertigo in MS due to demyelination
bull The most common neuroanatomic locations are the
root entry zone of CN VIII (giving absent calorics but
normal hearing and fixation suppression) and the
medial vestibular nucleus
bull Usually treated with steroids and vestibular
suppressants
Brandt 1993 Gass 1998 Thomke 1999
Sixth Annual Intensive Update in Neurology 0915-162016
72
Vertigo in MS
Frohmanrsquos large series (1153) reported that 52 of MS patients with acute vertigo had benign paroxysmal positional vertigo while 32 had new brainstem lesions
To avoid misdiagnoses and inappropriate treatment interventions alternative etiologies should be considered in all acute vertigo episodes in MS
E M Frohman Neurology 2000 and Multiple Sclerosis 2003
Sixth Annual Intensive Update in Neurology 0915-162016
73
Vestibular compensation
bull Acute unilateral or bilateral vestibular loss will prompt compensation thru central cervico-ocular visual and proprioceptive pathways
bull Unilateral loss may require several weeks for compensation to occur and activity especially head movements hastens compensation
bull Vestibular suppressant medication prolongs compensation
Sixth Annual Intensive Update in Neurology 0915-162016
74
Vestibular Rehabilitation
bull Performed by certified physical therapists many sites (Epic order vestibular PT) or check Vestibular Disorders of America website for providers
bull Assessment and treatment of benign positional vertigo
bull Directed rehab programs for central andor peripheral vestibular compensationndash Unilateral vestibulopathies
ndash Bilateral vestibular hypofunction
ndash Mutifactorial balance deficits and fall risk assessment
Sixth Annual Intensive Update in Neurology 0915-162016
75
Summary of whatrsquos new in peripheral vestibular disorders
bull Lateral semicircular canal benign paroxysmal positional vertigo presents with horizontal positioning nystagmus
bull Oral steroids appear to improve outcome in acute vestibular neuritis
bull Intratympanic steroids offer promise in Menierersquos syndrome
bull Superior semicircular canal dehiscence causes pressure and noise induced dizziness VEMP offers diagnostic information
Sixth Annual Intensive Update in Neurology 0915-162016
76
References
BPPVndash White J Benign paroxysmal positional vertigo Cleveland Clinic Journal of
Medicine 71(9)722-728 2004
ndash White J Coale K Catalano P Oas J Diagnosis and management of lateral semicircular canal benign positional vertigo Otolaryngology-Head and Neck Surgery 133 278-284 2005
ndash White J Savvides P Cherian N Oas J Canalith repositioning for benign paroxysmal positional vertigo Otology and Neurotology 26 704-710 2005
ndash White J Oas J Diagnosis and management of lateral semicircular canal conversions during particle repositioning therapy Laryngoscope 115(10) 1895-1897 2005
ndash Acute Vestibular Syndrome Hotson JR Baloh RW N Engl J Med 1998 Sep 3339(10)680-5
ndash Methylprednisolone valacyclovir or the combination for vestibular neuritis Strupp M Zingler VC Arbusow V Niklas D Maag KP Dieterich M Bense S Theil D Jahn K Brandt T N Engl J Med 2004 Jul 22351(4)354-61
ndash Intratympanic steroids for Meacuteniegraveres disease or syndrome Phillips JS Westerberg BCochrane Database Syst Rev 2011 Jul 6(7)CD008514 Review
ndash Sound- andor pressure-induced vertigo due to bone dehiscence of the superior semicircular canal Minor LB Solomon D Zinreich JS Zee DSArch Otolaryngol Head Neck Surg 1998 Mar124(3)249-58
Sixth Annual Intensive Update in Neurology 0915-162016
47
Menierersquos syndrome
bull Fluctuating low frequency hearing loss
bull Tinnitus andor aural fullness
bull Vertigo lasting hours
Sixth Annual Intensive Update in Neurology 0915-162016
48
Normal
Sixth Annual Intensive Update in Neurology 0915-162016
49
Hydrops
Sixth Annual Intensive Update in Neurology 0915-162016
50
Menierersquos syndrome
ndash Diagnosis- Exclusion (RO acoustic)
ndash 23 of Menierersquos patients will improve spontaneously
ndash Treatment-1500 mgday low sodium triamterene375-
hydrochlorothiazide 25 vestibular suppressants (lorazepam
1 mg tablet sublingual Q 8 prn attacks) off label use
Sixth Annual Intensive Update in Neurology 0915-162016
51
Menierersquos syndrome ndash intratympanic steroids
ndash A newer treatment option for those failing medical management
ndash 90 response rate (Boleus-Aguirre MS Otology Neurotology
2008)
ndash Outcomes data confirm excellent clinical response in 3941
Menierersquos patients treated with intratympanic steroids in 2008
Sixth Annual Intensive Update in Neurology 0915-162016
52
Question 4
bull Menierersquos syndrome diagnosis is based on
1) Recurrent vertigo lasting at least 20 minutes
2) Aural fullness
3) Documented hearing loss
4) Other causes excluded
5) All of the above
Sixth Annual Intensive Update in Neurology 0915-162016
53
Question 4
bull Menierersquos syndrome diagnosis is based on
1) Recurrent vertigo lasting at least 20 minutes
2) Aural fullness
3) Documented hearing loss
4) Other causes excluded
5) All of the above
Answer 5 See references at end
Sixth Annual Intensive Update in Neurology 0915-162016
54
Superior Semicircular Canal Dehiscence (SSCD)
bull Sound andor pressure induced oscillopsia
bull CT reformatted in the plane of the superior canals
shows dehiscence of overlying bone
bull Low frequency ldquoconductive hyperacusisrdquo with elevated
bone thresholds normal reflexes and a conductive loss
Sixth Annual Intensive Update in Neurology 0915-162016
55
SSCD
bull Positive middle ear pressure or loud sound stimulates ampullofugal endolymph flow -canal activation
bull Eye movements are tonic upwards with ipsilateral intorsion
Sixth Annual Intensive Update in Neurology 0915-162016
56
SSCD
bull Valsalva against a closed glottis or jugular compression causes increased intracranial pressure and ampullopetal endolymph flow -canal inhibition
bull Eye movements are tonic downwards with ipsilateral extorsion
Sixth Annual Intensive Update in Neurology 0915-162016
57
Reconstructed left superior semicircular canal
Sixth Annual Intensive Update in Neurology 0915-162016
58
Sixth Annual Intensive Update in Neurology 0915-162016
59
SCHEMATIC DRAWING OF AN OTOLITHIC MACULA
Sixth Annual Intensive Update in Neurology 0915-162016
60
MACULAE OF THE UTRICLESACCULE
Sixth Annual Intensive Update in Neurology 0915-162016
61
HAIR CELLS
Sixth Annual Intensive Update in Neurology 0915-162016
62
SCANNING ELECTRON MICROGRAPH OF OTOCONIA
Sixth Annual Intensive Update in Neurology 0915-162016
63
Ocular VEMP
bull Response to loud clicks (80-110 dB) or forehead tap (Halmagyi and Curthoys 2003)
bull Contralateral oculomotor nucleus (CN III)
bull Inferior oblique muscle contraction (8 msec) detected by 5sec event triggered averaging
bull Noninverting electrode inferior to eye
bull Inverting below non-inverting electrode
bull Ground to forehead
bull Normal subjects have very low response
Sixth Annual Intensive Update in Neurology 0915-162016
64
Clinical applications
bull The o-VEMP is a sensitive test of otolithutricular
function
bull Complements vestibular test procedures that assess
semicircular canals
bull Abnormally robust responses are associated with third
window abnormalities such as semicircular canal
dehiscence
bull Serves as an excellent screening test for semicircular
canal dehiscence
Sixth Annual Intensive Update in Neurology 0915-162016
65
Question 5
bull Superior semicircular canal dehiscence
1) Causes increased ability to hear vibratory sounds
2) Improves hearing
3) Causes meningitis
4) Always needs surgical repair
Sixth Annual Intensive Update in Neurology 0915-162016
66
Question 5
bull Superior semicircular canal dehiscence
1) Causes increased ability to hear vibratory sounds
2) Improves hearing
3) Causes meningitis
4) Always needs surgical repair
Answer 1 References at end of lecture
Sixth Annual Intensive Update in Neurology 0915-162016
67
Enlarged vestibular aqueduct
bull Axial temporal bone CT showing right temporal bone with enlarged vestibular aqueduct (arrow)
bull Vestibular symptoms accompany progressive sensorineural hearing loss in 4-50 of patients in selected series
Sixth Annual Intensive Update in Neurology 0915-162016
68
Migraine
bull Family or personal history of migraine
bull 25 of migraine patients experience spontaneous vertigo (Baloh 1997)
bull Neuhauser criteria(2001) have been used to construct structured diagnostic interviews (Furman 2003)
bull Not always associated with headache
bull Motion sensitivity
bull Medication responsive
Sixth Annual Intensive Update in Neurology 0915-162016
69
ICHD-III beta Criteria for Vestibular Migraine
bull A At least five attacks fulfilling criteria C and D
bull B A current or past history of 11 migraine without aura or 12 migraine with aura
bull C Vestibular symptoms of moderate or severe intensity lasting between 5 minutes and 72 hours
bull D At least 50 associated with one of the following migrainous features
ndash 1 Headaches
ndash 2 Photophobia and phonophobia
ndash 3 Visual Aura
bull E Not better accounted for by another vestibular disorder
Sixth Annual Intensive Update in Neurology 0915-162016
70
Incidence of vertigo in MS
bull True vertigo (the perception of environmental
movement typically rotational) occurs in about
20 of MS patients and is the presenting
manifestation in 5
bull Up to 78 of MS patients report abnormalities
in balance at some point in their illness
Frohman et al Multiple Sclerosis 2003 9250
Sixth Annual Intensive Update in Neurology 0915-162016
71
Vertigo in MS due to demyelination
bull The most common neuroanatomic locations are the
root entry zone of CN VIII (giving absent calorics but
normal hearing and fixation suppression) and the
medial vestibular nucleus
bull Usually treated with steroids and vestibular
suppressants
Brandt 1993 Gass 1998 Thomke 1999
Sixth Annual Intensive Update in Neurology 0915-162016
72
Vertigo in MS
Frohmanrsquos large series (1153) reported that 52 of MS patients with acute vertigo had benign paroxysmal positional vertigo while 32 had new brainstem lesions
To avoid misdiagnoses and inappropriate treatment interventions alternative etiologies should be considered in all acute vertigo episodes in MS
E M Frohman Neurology 2000 and Multiple Sclerosis 2003
Sixth Annual Intensive Update in Neurology 0915-162016
73
Vestibular compensation
bull Acute unilateral or bilateral vestibular loss will prompt compensation thru central cervico-ocular visual and proprioceptive pathways
bull Unilateral loss may require several weeks for compensation to occur and activity especially head movements hastens compensation
bull Vestibular suppressant medication prolongs compensation
Sixth Annual Intensive Update in Neurology 0915-162016
74
Vestibular Rehabilitation
bull Performed by certified physical therapists many sites (Epic order vestibular PT) or check Vestibular Disorders of America website for providers
bull Assessment and treatment of benign positional vertigo
bull Directed rehab programs for central andor peripheral vestibular compensationndash Unilateral vestibulopathies
ndash Bilateral vestibular hypofunction
ndash Mutifactorial balance deficits and fall risk assessment
Sixth Annual Intensive Update in Neurology 0915-162016
75
Summary of whatrsquos new in peripheral vestibular disorders
bull Lateral semicircular canal benign paroxysmal positional vertigo presents with horizontal positioning nystagmus
bull Oral steroids appear to improve outcome in acute vestibular neuritis
bull Intratympanic steroids offer promise in Menierersquos syndrome
bull Superior semicircular canal dehiscence causes pressure and noise induced dizziness VEMP offers diagnostic information
Sixth Annual Intensive Update in Neurology 0915-162016
76
References
BPPVndash White J Benign paroxysmal positional vertigo Cleveland Clinic Journal of
Medicine 71(9)722-728 2004
ndash White J Coale K Catalano P Oas J Diagnosis and management of lateral semicircular canal benign positional vertigo Otolaryngology-Head and Neck Surgery 133 278-284 2005
ndash White J Savvides P Cherian N Oas J Canalith repositioning for benign paroxysmal positional vertigo Otology and Neurotology 26 704-710 2005
ndash White J Oas J Diagnosis and management of lateral semicircular canal conversions during particle repositioning therapy Laryngoscope 115(10) 1895-1897 2005
ndash Acute Vestibular Syndrome Hotson JR Baloh RW N Engl J Med 1998 Sep 3339(10)680-5
ndash Methylprednisolone valacyclovir or the combination for vestibular neuritis Strupp M Zingler VC Arbusow V Niklas D Maag KP Dieterich M Bense S Theil D Jahn K Brandt T N Engl J Med 2004 Jul 22351(4)354-61
ndash Intratympanic steroids for Meacuteniegraveres disease or syndrome Phillips JS Westerberg BCochrane Database Syst Rev 2011 Jul 6(7)CD008514 Review
ndash Sound- andor pressure-induced vertigo due to bone dehiscence of the superior semicircular canal Minor LB Solomon D Zinreich JS Zee DSArch Otolaryngol Head Neck Surg 1998 Mar124(3)249-58
Sixth Annual Intensive Update in Neurology 0915-162016
48
Normal
Sixth Annual Intensive Update in Neurology 0915-162016
49
Hydrops
Sixth Annual Intensive Update in Neurology 0915-162016
50
Menierersquos syndrome
ndash Diagnosis- Exclusion (RO acoustic)
ndash 23 of Menierersquos patients will improve spontaneously
ndash Treatment-1500 mgday low sodium triamterene375-
hydrochlorothiazide 25 vestibular suppressants (lorazepam
1 mg tablet sublingual Q 8 prn attacks) off label use
Sixth Annual Intensive Update in Neurology 0915-162016
51
Menierersquos syndrome ndash intratympanic steroids
ndash A newer treatment option for those failing medical management
ndash 90 response rate (Boleus-Aguirre MS Otology Neurotology
2008)
ndash Outcomes data confirm excellent clinical response in 3941
Menierersquos patients treated with intratympanic steroids in 2008
Sixth Annual Intensive Update in Neurology 0915-162016
52
Question 4
bull Menierersquos syndrome diagnosis is based on
1) Recurrent vertigo lasting at least 20 minutes
2) Aural fullness
3) Documented hearing loss
4) Other causes excluded
5) All of the above
Sixth Annual Intensive Update in Neurology 0915-162016
53
Question 4
bull Menierersquos syndrome diagnosis is based on
1) Recurrent vertigo lasting at least 20 minutes
2) Aural fullness
3) Documented hearing loss
4) Other causes excluded
5) All of the above
Answer 5 See references at end
Sixth Annual Intensive Update in Neurology 0915-162016
54
Superior Semicircular Canal Dehiscence (SSCD)
bull Sound andor pressure induced oscillopsia
bull CT reformatted in the plane of the superior canals
shows dehiscence of overlying bone
bull Low frequency ldquoconductive hyperacusisrdquo with elevated
bone thresholds normal reflexes and a conductive loss
Sixth Annual Intensive Update in Neurology 0915-162016
55
SSCD
bull Positive middle ear pressure or loud sound stimulates ampullofugal endolymph flow -canal activation
bull Eye movements are tonic upwards with ipsilateral intorsion
Sixth Annual Intensive Update in Neurology 0915-162016
56
SSCD
bull Valsalva against a closed glottis or jugular compression causes increased intracranial pressure and ampullopetal endolymph flow -canal inhibition
bull Eye movements are tonic downwards with ipsilateral extorsion
Sixth Annual Intensive Update in Neurology 0915-162016
57
Reconstructed left superior semicircular canal
Sixth Annual Intensive Update in Neurology 0915-162016
58
Sixth Annual Intensive Update in Neurology 0915-162016
59
SCHEMATIC DRAWING OF AN OTOLITHIC MACULA
Sixth Annual Intensive Update in Neurology 0915-162016
60
MACULAE OF THE UTRICLESACCULE
Sixth Annual Intensive Update in Neurology 0915-162016
61
HAIR CELLS
Sixth Annual Intensive Update in Neurology 0915-162016
62
SCANNING ELECTRON MICROGRAPH OF OTOCONIA
Sixth Annual Intensive Update in Neurology 0915-162016
63
Ocular VEMP
bull Response to loud clicks (80-110 dB) or forehead tap (Halmagyi and Curthoys 2003)
bull Contralateral oculomotor nucleus (CN III)
bull Inferior oblique muscle contraction (8 msec) detected by 5sec event triggered averaging
bull Noninverting electrode inferior to eye
bull Inverting below non-inverting electrode
bull Ground to forehead
bull Normal subjects have very low response
Sixth Annual Intensive Update in Neurology 0915-162016
64
Clinical applications
bull The o-VEMP is a sensitive test of otolithutricular
function
bull Complements vestibular test procedures that assess
semicircular canals
bull Abnormally robust responses are associated with third
window abnormalities such as semicircular canal
dehiscence
bull Serves as an excellent screening test for semicircular
canal dehiscence
Sixth Annual Intensive Update in Neurology 0915-162016
65
Question 5
bull Superior semicircular canal dehiscence
1) Causes increased ability to hear vibratory sounds
2) Improves hearing
3) Causes meningitis
4) Always needs surgical repair
Sixth Annual Intensive Update in Neurology 0915-162016
66
Question 5
bull Superior semicircular canal dehiscence
1) Causes increased ability to hear vibratory sounds
2) Improves hearing
3) Causes meningitis
4) Always needs surgical repair
Answer 1 References at end of lecture
Sixth Annual Intensive Update in Neurology 0915-162016
67
Enlarged vestibular aqueduct
bull Axial temporal bone CT showing right temporal bone with enlarged vestibular aqueduct (arrow)
bull Vestibular symptoms accompany progressive sensorineural hearing loss in 4-50 of patients in selected series
Sixth Annual Intensive Update in Neurology 0915-162016
68
Migraine
bull Family or personal history of migraine
bull 25 of migraine patients experience spontaneous vertigo (Baloh 1997)
bull Neuhauser criteria(2001) have been used to construct structured diagnostic interviews (Furman 2003)
bull Not always associated with headache
bull Motion sensitivity
bull Medication responsive
Sixth Annual Intensive Update in Neurology 0915-162016
69
ICHD-III beta Criteria for Vestibular Migraine
bull A At least five attacks fulfilling criteria C and D
bull B A current or past history of 11 migraine without aura or 12 migraine with aura
bull C Vestibular symptoms of moderate or severe intensity lasting between 5 minutes and 72 hours
bull D At least 50 associated with one of the following migrainous features
ndash 1 Headaches
ndash 2 Photophobia and phonophobia
ndash 3 Visual Aura
bull E Not better accounted for by another vestibular disorder
Sixth Annual Intensive Update in Neurology 0915-162016
70
Incidence of vertigo in MS
bull True vertigo (the perception of environmental
movement typically rotational) occurs in about
20 of MS patients and is the presenting
manifestation in 5
bull Up to 78 of MS patients report abnormalities
in balance at some point in their illness
Frohman et al Multiple Sclerosis 2003 9250
Sixth Annual Intensive Update in Neurology 0915-162016
71
Vertigo in MS due to demyelination
bull The most common neuroanatomic locations are the
root entry zone of CN VIII (giving absent calorics but
normal hearing and fixation suppression) and the
medial vestibular nucleus
bull Usually treated with steroids and vestibular
suppressants
Brandt 1993 Gass 1998 Thomke 1999
Sixth Annual Intensive Update in Neurology 0915-162016
72
Vertigo in MS
Frohmanrsquos large series (1153) reported that 52 of MS patients with acute vertigo had benign paroxysmal positional vertigo while 32 had new brainstem lesions
To avoid misdiagnoses and inappropriate treatment interventions alternative etiologies should be considered in all acute vertigo episodes in MS
E M Frohman Neurology 2000 and Multiple Sclerosis 2003
Sixth Annual Intensive Update in Neurology 0915-162016
73
Vestibular compensation
bull Acute unilateral or bilateral vestibular loss will prompt compensation thru central cervico-ocular visual and proprioceptive pathways
bull Unilateral loss may require several weeks for compensation to occur and activity especially head movements hastens compensation
bull Vestibular suppressant medication prolongs compensation
Sixth Annual Intensive Update in Neurology 0915-162016
74
Vestibular Rehabilitation
bull Performed by certified physical therapists many sites (Epic order vestibular PT) or check Vestibular Disorders of America website for providers
bull Assessment and treatment of benign positional vertigo
bull Directed rehab programs for central andor peripheral vestibular compensationndash Unilateral vestibulopathies
ndash Bilateral vestibular hypofunction
ndash Mutifactorial balance deficits and fall risk assessment
Sixth Annual Intensive Update in Neurology 0915-162016
75
Summary of whatrsquos new in peripheral vestibular disorders
bull Lateral semicircular canal benign paroxysmal positional vertigo presents with horizontal positioning nystagmus
bull Oral steroids appear to improve outcome in acute vestibular neuritis
bull Intratympanic steroids offer promise in Menierersquos syndrome
bull Superior semicircular canal dehiscence causes pressure and noise induced dizziness VEMP offers diagnostic information
Sixth Annual Intensive Update in Neurology 0915-162016
76
References
BPPVndash White J Benign paroxysmal positional vertigo Cleveland Clinic Journal of
Medicine 71(9)722-728 2004
ndash White J Coale K Catalano P Oas J Diagnosis and management of lateral semicircular canal benign positional vertigo Otolaryngology-Head and Neck Surgery 133 278-284 2005
ndash White J Savvides P Cherian N Oas J Canalith repositioning for benign paroxysmal positional vertigo Otology and Neurotology 26 704-710 2005
ndash White J Oas J Diagnosis and management of lateral semicircular canal conversions during particle repositioning therapy Laryngoscope 115(10) 1895-1897 2005
ndash Acute Vestibular Syndrome Hotson JR Baloh RW N Engl J Med 1998 Sep 3339(10)680-5
ndash Methylprednisolone valacyclovir or the combination for vestibular neuritis Strupp M Zingler VC Arbusow V Niklas D Maag KP Dieterich M Bense S Theil D Jahn K Brandt T N Engl J Med 2004 Jul 22351(4)354-61
ndash Intratympanic steroids for Meacuteniegraveres disease or syndrome Phillips JS Westerberg BCochrane Database Syst Rev 2011 Jul 6(7)CD008514 Review
ndash Sound- andor pressure-induced vertigo due to bone dehiscence of the superior semicircular canal Minor LB Solomon D Zinreich JS Zee DSArch Otolaryngol Head Neck Surg 1998 Mar124(3)249-58
Sixth Annual Intensive Update in Neurology 0915-162016
49
Hydrops
Sixth Annual Intensive Update in Neurology 0915-162016
50
Menierersquos syndrome
ndash Diagnosis- Exclusion (RO acoustic)
ndash 23 of Menierersquos patients will improve spontaneously
ndash Treatment-1500 mgday low sodium triamterene375-
hydrochlorothiazide 25 vestibular suppressants (lorazepam
1 mg tablet sublingual Q 8 prn attacks) off label use
Sixth Annual Intensive Update in Neurology 0915-162016
51
Menierersquos syndrome ndash intratympanic steroids
ndash A newer treatment option for those failing medical management
ndash 90 response rate (Boleus-Aguirre MS Otology Neurotology
2008)
ndash Outcomes data confirm excellent clinical response in 3941
Menierersquos patients treated with intratympanic steroids in 2008
Sixth Annual Intensive Update in Neurology 0915-162016
52
Question 4
bull Menierersquos syndrome diagnosis is based on
1) Recurrent vertigo lasting at least 20 minutes
2) Aural fullness
3) Documented hearing loss
4) Other causes excluded
5) All of the above
Sixth Annual Intensive Update in Neurology 0915-162016
53
Question 4
bull Menierersquos syndrome diagnosis is based on
1) Recurrent vertigo lasting at least 20 minutes
2) Aural fullness
3) Documented hearing loss
4) Other causes excluded
5) All of the above
Answer 5 See references at end
Sixth Annual Intensive Update in Neurology 0915-162016
54
Superior Semicircular Canal Dehiscence (SSCD)
bull Sound andor pressure induced oscillopsia
bull CT reformatted in the plane of the superior canals
shows dehiscence of overlying bone
bull Low frequency ldquoconductive hyperacusisrdquo with elevated
bone thresholds normal reflexes and a conductive loss
Sixth Annual Intensive Update in Neurology 0915-162016
55
SSCD
bull Positive middle ear pressure or loud sound stimulates ampullofugal endolymph flow -canal activation
bull Eye movements are tonic upwards with ipsilateral intorsion
Sixth Annual Intensive Update in Neurology 0915-162016
56
SSCD
bull Valsalva against a closed glottis or jugular compression causes increased intracranial pressure and ampullopetal endolymph flow -canal inhibition
bull Eye movements are tonic downwards with ipsilateral extorsion
Sixth Annual Intensive Update in Neurology 0915-162016
57
Reconstructed left superior semicircular canal
Sixth Annual Intensive Update in Neurology 0915-162016
58
Sixth Annual Intensive Update in Neurology 0915-162016
59
SCHEMATIC DRAWING OF AN OTOLITHIC MACULA
Sixth Annual Intensive Update in Neurology 0915-162016
60
MACULAE OF THE UTRICLESACCULE
Sixth Annual Intensive Update in Neurology 0915-162016
61
HAIR CELLS
Sixth Annual Intensive Update in Neurology 0915-162016
62
SCANNING ELECTRON MICROGRAPH OF OTOCONIA
Sixth Annual Intensive Update in Neurology 0915-162016
63
Ocular VEMP
bull Response to loud clicks (80-110 dB) or forehead tap (Halmagyi and Curthoys 2003)
bull Contralateral oculomotor nucleus (CN III)
bull Inferior oblique muscle contraction (8 msec) detected by 5sec event triggered averaging
bull Noninverting electrode inferior to eye
bull Inverting below non-inverting electrode
bull Ground to forehead
bull Normal subjects have very low response
Sixth Annual Intensive Update in Neurology 0915-162016
64
Clinical applications
bull The o-VEMP is a sensitive test of otolithutricular
function
bull Complements vestibular test procedures that assess
semicircular canals
bull Abnormally robust responses are associated with third
window abnormalities such as semicircular canal
dehiscence
bull Serves as an excellent screening test for semicircular
canal dehiscence
Sixth Annual Intensive Update in Neurology 0915-162016
65
Question 5
bull Superior semicircular canal dehiscence
1) Causes increased ability to hear vibratory sounds
2) Improves hearing
3) Causes meningitis
4) Always needs surgical repair
Sixth Annual Intensive Update in Neurology 0915-162016
66
Question 5
bull Superior semicircular canal dehiscence
1) Causes increased ability to hear vibratory sounds
2) Improves hearing
3) Causes meningitis
4) Always needs surgical repair
Answer 1 References at end of lecture
Sixth Annual Intensive Update in Neurology 0915-162016
67
Enlarged vestibular aqueduct
bull Axial temporal bone CT showing right temporal bone with enlarged vestibular aqueduct (arrow)
bull Vestibular symptoms accompany progressive sensorineural hearing loss in 4-50 of patients in selected series
Sixth Annual Intensive Update in Neurology 0915-162016
68
Migraine
bull Family or personal history of migraine
bull 25 of migraine patients experience spontaneous vertigo (Baloh 1997)
bull Neuhauser criteria(2001) have been used to construct structured diagnostic interviews (Furman 2003)
bull Not always associated with headache
bull Motion sensitivity
bull Medication responsive
Sixth Annual Intensive Update in Neurology 0915-162016
69
ICHD-III beta Criteria for Vestibular Migraine
bull A At least five attacks fulfilling criteria C and D
bull B A current or past history of 11 migraine without aura or 12 migraine with aura
bull C Vestibular symptoms of moderate or severe intensity lasting between 5 minutes and 72 hours
bull D At least 50 associated with one of the following migrainous features
ndash 1 Headaches
ndash 2 Photophobia and phonophobia
ndash 3 Visual Aura
bull E Not better accounted for by another vestibular disorder
Sixth Annual Intensive Update in Neurology 0915-162016
70
Incidence of vertigo in MS
bull True vertigo (the perception of environmental
movement typically rotational) occurs in about
20 of MS patients and is the presenting
manifestation in 5
bull Up to 78 of MS patients report abnormalities
in balance at some point in their illness
Frohman et al Multiple Sclerosis 2003 9250
Sixth Annual Intensive Update in Neurology 0915-162016
71
Vertigo in MS due to demyelination
bull The most common neuroanatomic locations are the
root entry zone of CN VIII (giving absent calorics but
normal hearing and fixation suppression) and the
medial vestibular nucleus
bull Usually treated with steroids and vestibular
suppressants
Brandt 1993 Gass 1998 Thomke 1999
Sixth Annual Intensive Update in Neurology 0915-162016
72
Vertigo in MS
Frohmanrsquos large series (1153) reported that 52 of MS patients with acute vertigo had benign paroxysmal positional vertigo while 32 had new brainstem lesions
To avoid misdiagnoses and inappropriate treatment interventions alternative etiologies should be considered in all acute vertigo episodes in MS
E M Frohman Neurology 2000 and Multiple Sclerosis 2003
Sixth Annual Intensive Update in Neurology 0915-162016
73
Vestibular compensation
bull Acute unilateral or bilateral vestibular loss will prompt compensation thru central cervico-ocular visual and proprioceptive pathways
bull Unilateral loss may require several weeks for compensation to occur and activity especially head movements hastens compensation
bull Vestibular suppressant medication prolongs compensation
Sixth Annual Intensive Update in Neurology 0915-162016
74
Vestibular Rehabilitation
bull Performed by certified physical therapists many sites (Epic order vestibular PT) or check Vestibular Disorders of America website for providers
bull Assessment and treatment of benign positional vertigo
bull Directed rehab programs for central andor peripheral vestibular compensationndash Unilateral vestibulopathies
ndash Bilateral vestibular hypofunction
ndash Mutifactorial balance deficits and fall risk assessment
Sixth Annual Intensive Update in Neurology 0915-162016
75
Summary of whatrsquos new in peripheral vestibular disorders
bull Lateral semicircular canal benign paroxysmal positional vertigo presents with horizontal positioning nystagmus
bull Oral steroids appear to improve outcome in acute vestibular neuritis
bull Intratympanic steroids offer promise in Menierersquos syndrome
bull Superior semicircular canal dehiscence causes pressure and noise induced dizziness VEMP offers diagnostic information
Sixth Annual Intensive Update in Neurology 0915-162016
76
References
BPPVndash White J Benign paroxysmal positional vertigo Cleveland Clinic Journal of
Medicine 71(9)722-728 2004
ndash White J Coale K Catalano P Oas J Diagnosis and management of lateral semicircular canal benign positional vertigo Otolaryngology-Head and Neck Surgery 133 278-284 2005
ndash White J Savvides P Cherian N Oas J Canalith repositioning for benign paroxysmal positional vertigo Otology and Neurotology 26 704-710 2005
ndash White J Oas J Diagnosis and management of lateral semicircular canal conversions during particle repositioning therapy Laryngoscope 115(10) 1895-1897 2005
ndash Acute Vestibular Syndrome Hotson JR Baloh RW N Engl J Med 1998 Sep 3339(10)680-5
ndash Methylprednisolone valacyclovir or the combination for vestibular neuritis Strupp M Zingler VC Arbusow V Niklas D Maag KP Dieterich M Bense S Theil D Jahn K Brandt T N Engl J Med 2004 Jul 22351(4)354-61
ndash Intratympanic steroids for Meacuteniegraveres disease or syndrome Phillips JS Westerberg BCochrane Database Syst Rev 2011 Jul 6(7)CD008514 Review
ndash Sound- andor pressure-induced vertigo due to bone dehiscence of the superior semicircular canal Minor LB Solomon D Zinreich JS Zee DSArch Otolaryngol Head Neck Surg 1998 Mar124(3)249-58
Sixth Annual Intensive Update in Neurology 0915-162016
50
Menierersquos syndrome
ndash Diagnosis- Exclusion (RO acoustic)
ndash 23 of Menierersquos patients will improve spontaneously
ndash Treatment-1500 mgday low sodium triamterene375-
hydrochlorothiazide 25 vestibular suppressants (lorazepam
1 mg tablet sublingual Q 8 prn attacks) off label use
Sixth Annual Intensive Update in Neurology 0915-162016
51
Menierersquos syndrome ndash intratympanic steroids
ndash A newer treatment option for those failing medical management
ndash 90 response rate (Boleus-Aguirre MS Otology Neurotology
2008)
ndash Outcomes data confirm excellent clinical response in 3941
Menierersquos patients treated with intratympanic steroids in 2008
Sixth Annual Intensive Update in Neurology 0915-162016
52
Question 4
bull Menierersquos syndrome diagnosis is based on
1) Recurrent vertigo lasting at least 20 minutes
2) Aural fullness
3) Documented hearing loss
4) Other causes excluded
5) All of the above
Sixth Annual Intensive Update in Neurology 0915-162016
53
Question 4
bull Menierersquos syndrome diagnosis is based on
1) Recurrent vertigo lasting at least 20 minutes
2) Aural fullness
3) Documented hearing loss
4) Other causes excluded
5) All of the above
Answer 5 See references at end
Sixth Annual Intensive Update in Neurology 0915-162016
54
Superior Semicircular Canal Dehiscence (SSCD)
bull Sound andor pressure induced oscillopsia
bull CT reformatted in the plane of the superior canals
shows dehiscence of overlying bone
bull Low frequency ldquoconductive hyperacusisrdquo with elevated
bone thresholds normal reflexes and a conductive loss
Sixth Annual Intensive Update in Neurology 0915-162016
55
SSCD
bull Positive middle ear pressure or loud sound stimulates ampullofugal endolymph flow -canal activation
bull Eye movements are tonic upwards with ipsilateral intorsion
Sixth Annual Intensive Update in Neurology 0915-162016
56
SSCD
bull Valsalva against a closed glottis or jugular compression causes increased intracranial pressure and ampullopetal endolymph flow -canal inhibition
bull Eye movements are tonic downwards with ipsilateral extorsion
Sixth Annual Intensive Update in Neurology 0915-162016
57
Reconstructed left superior semicircular canal
Sixth Annual Intensive Update in Neurology 0915-162016
58
Sixth Annual Intensive Update in Neurology 0915-162016
59
SCHEMATIC DRAWING OF AN OTOLITHIC MACULA
Sixth Annual Intensive Update in Neurology 0915-162016
60
MACULAE OF THE UTRICLESACCULE
Sixth Annual Intensive Update in Neurology 0915-162016
61
HAIR CELLS
Sixth Annual Intensive Update in Neurology 0915-162016
62
SCANNING ELECTRON MICROGRAPH OF OTOCONIA
Sixth Annual Intensive Update in Neurology 0915-162016
63
Ocular VEMP
bull Response to loud clicks (80-110 dB) or forehead tap (Halmagyi and Curthoys 2003)
bull Contralateral oculomotor nucleus (CN III)
bull Inferior oblique muscle contraction (8 msec) detected by 5sec event triggered averaging
bull Noninverting electrode inferior to eye
bull Inverting below non-inverting electrode
bull Ground to forehead
bull Normal subjects have very low response
Sixth Annual Intensive Update in Neurology 0915-162016
64
Clinical applications
bull The o-VEMP is a sensitive test of otolithutricular
function
bull Complements vestibular test procedures that assess
semicircular canals
bull Abnormally robust responses are associated with third
window abnormalities such as semicircular canal
dehiscence
bull Serves as an excellent screening test for semicircular
canal dehiscence
Sixth Annual Intensive Update in Neurology 0915-162016
65
Question 5
bull Superior semicircular canal dehiscence
1) Causes increased ability to hear vibratory sounds
2) Improves hearing
3) Causes meningitis
4) Always needs surgical repair
Sixth Annual Intensive Update in Neurology 0915-162016
66
Question 5
bull Superior semicircular canal dehiscence
1) Causes increased ability to hear vibratory sounds
2) Improves hearing
3) Causes meningitis
4) Always needs surgical repair
Answer 1 References at end of lecture
Sixth Annual Intensive Update in Neurology 0915-162016
67
Enlarged vestibular aqueduct
bull Axial temporal bone CT showing right temporal bone with enlarged vestibular aqueduct (arrow)
bull Vestibular symptoms accompany progressive sensorineural hearing loss in 4-50 of patients in selected series
Sixth Annual Intensive Update in Neurology 0915-162016
68
Migraine
bull Family or personal history of migraine
bull 25 of migraine patients experience spontaneous vertigo (Baloh 1997)
bull Neuhauser criteria(2001) have been used to construct structured diagnostic interviews (Furman 2003)
bull Not always associated with headache
bull Motion sensitivity
bull Medication responsive
Sixth Annual Intensive Update in Neurology 0915-162016
69
ICHD-III beta Criteria for Vestibular Migraine
bull A At least five attacks fulfilling criteria C and D
bull B A current or past history of 11 migraine without aura or 12 migraine with aura
bull C Vestibular symptoms of moderate or severe intensity lasting between 5 minutes and 72 hours
bull D At least 50 associated with one of the following migrainous features
ndash 1 Headaches
ndash 2 Photophobia and phonophobia
ndash 3 Visual Aura
bull E Not better accounted for by another vestibular disorder
Sixth Annual Intensive Update in Neurology 0915-162016
70
Incidence of vertigo in MS
bull True vertigo (the perception of environmental
movement typically rotational) occurs in about
20 of MS patients and is the presenting
manifestation in 5
bull Up to 78 of MS patients report abnormalities
in balance at some point in their illness
Frohman et al Multiple Sclerosis 2003 9250
Sixth Annual Intensive Update in Neurology 0915-162016
71
Vertigo in MS due to demyelination
bull The most common neuroanatomic locations are the
root entry zone of CN VIII (giving absent calorics but
normal hearing and fixation suppression) and the
medial vestibular nucleus
bull Usually treated with steroids and vestibular
suppressants
Brandt 1993 Gass 1998 Thomke 1999
Sixth Annual Intensive Update in Neurology 0915-162016
72
Vertigo in MS
Frohmanrsquos large series (1153) reported that 52 of MS patients with acute vertigo had benign paroxysmal positional vertigo while 32 had new brainstem lesions
To avoid misdiagnoses and inappropriate treatment interventions alternative etiologies should be considered in all acute vertigo episodes in MS
E M Frohman Neurology 2000 and Multiple Sclerosis 2003
Sixth Annual Intensive Update in Neurology 0915-162016
73
Vestibular compensation
bull Acute unilateral or bilateral vestibular loss will prompt compensation thru central cervico-ocular visual and proprioceptive pathways
bull Unilateral loss may require several weeks for compensation to occur and activity especially head movements hastens compensation
bull Vestibular suppressant medication prolongs compensation
Sixth Annual Intensive Update in Neurology 0915-162016
74
Vestibular Rehabilitation
bull Performed by certified physical therapists many sites (Epic order vestibular PT) or check Vestibular Disorders of America website for providers
bull Assessment and treatment of benign positional vertigo
bull Directed rehab programs for central andor peripheral vestibular compensationndash Unilateral vestibulopathies
ndash Bilateral vestibular hypofunction
ndash Mutifactorial balance deficits and fall risk assessment
Sixth Annual Intensive Update in Neurology 0915-162016
75
Summary of whatrsquos new in peripheral vestibular disorders
bull Lateral semicircular canal benign paroxysmal positional vertigo presents with horizontal positioning nystagmus
bull Oral steroids appear to improve outcome in acute vestibular neuritis
bull Intratympanic steroids offer promise in Menierersquos syndrome
bull Superior semicircular canal dehiscence causes pressure and noise induced dizziness VEMP offers diagnostic information
Sixth Annual Intensive Update in Neurology 0915-162016
76
References
BPPVndash White J Benign paroxysmal positional vertigo Cleveland Clinic Journal of
Medicine 71(9)722-728 2004
ndash White J Coale K Catalano P Oas J Diagnosis and management of lateral semicircular canal benign positional vertigo Otolaryngology-Head and Neck Surgery 133 278-284 2005
ndash White J Savvides P Cherian N Oas J Canalith repositioning for benign paroxysmal positional vertigo Otology and Neurotology 26 704-710 2005
ndash White J Oas J Diagnosis and management of lateral semicircular canal conversions during particle repositioning therapy Laryngoscope 115(10) 1895-1897 2005
ndash Acute Vestibular Syndrome Hotson JR Baloh RW N Engl J Med 1998 Sep 3339(10)680-5
ndash Methylprednisolone valacyclovir or the combination for vestibular neuritis Strupp M Zingler VC Arbusow V Niklas D Maag KP Dieterich M Bense S Theil D Jahn K Brandt T N Engl J Med 2004 Jul 22351(4)354-61
ndash Intratympanic steroids for Meacuteniegraveres disease or syndrome Phillips JS Westerberg BCochrane Database Syst Rev 2011 Jul 6(7)CD008514 Review
ndash Sound- andor pressure-induced vertigo due to bone dehiscence of the superior semicircular canal Minor LB Solomon D Zinreich JS Zee DSArch Otolaryngol Head Neck Surg 1998 Mar124(3)249-58
Sixth Annual Intensive Update in Neurology 0915-162016
51
Menierersquos syndrome ndash intratympanic steroids
ndash A newer treatment option for those failing medical management
ndash 90 response rate (Boleus-Aguirre MS Otology Neurotology
2008)
ndash Outcomes data confirm excellent clinical response in 3941
Menierersquos patients treated with intratympanic steroids in 2008
Sixth Annual Intensive Update in Neurology 0915-162016
52
Question 4
bull Menierersquos syndrome diagnosis is based on
1) Recurrent vertigo lasting at least 20 minutes
2) Aural fullness
3) Documented hearing loss
4) Other causes excluded
5) All of the above
Sixth Annual Intensive Update in Neurology 0915-162016
53
Question 4
bull Menierersquos syndrome diagnosis is based on
1) Recurrent vertigo lasting at least 20 minutes
2) Aural fullness
3) Documented hearing loss
4) Other causes excluded
5) All of the above
Answer 5 See references at end
Sixth Annual Intensive Update in Neurology 0915-162016
54
Superior Semicircular Canal Dehiscence (SSCD)
bull Sound andor pressure induced oscillopsia
bull CT reformatted in the plane of the superior canals
shows dehiscence of overlying bone
bull Low frequency ldquoconductive hyperacusisrdquo with elevated
bone thresholds normal reflexes and a conductive loss
Sixth Annual Intensive Update in Neurology 0915-162016
55
SSCD
bull Positive middle ear pressure or loud sound stimulates ampullofugal endolymph flow -canal activation
bull Eye movements are tonic upwards with ipsilateral intorsion
Sixth Annual Intensive Update in Neurology 0915-162016
56
SSCD
bull Valsalva against a closed glottis or jugular compression causes increased intracranial pressure and ampullopetal endolymph flow -canal inhibition
bull Eye movements are tonic downwards with ipsilateral extorsion
Sixth Annual Intensive Update in Neurology 0915-162016
57
Reconstructed left superior semicircular canal
Sixth Annual Intensive Update in Neurology 0915-162016
58
Sixth Annual Intensive Update in Neurology 0915-162016
59
SCHEMATIC DRAWING OF AN OTOLITHIC MACULA
Sixth Annual Intensive Update in Neurology 0915-162016
60
MACULAE OF THE UTRICLESACCULE
Sixth Annual Intensive Update in Neurology 0915-162016
61
HAIR CELLS
Sixth Annual Intensive Update in Neurology 0915-162016
62
SCANNING ELECTRON MICROGRAPH OF OTOCONIA
Sixth Annual Intensive Update in Neurology 0915-162016
63
Ocular VEMP
bull Response to loud clicks (80-110 dB) or forehead tap (Halmagyi and Curthoys 2003)
bull Contralateral oculomotor nucleus (CN III)
bull Inferior oblique muscle contraction (8 msec) detected by 5sec event triggered averaging
bull Noninverting electrode inferior to eye
bull Inverting below non-inverting electrode
bull Ground to forehead
bull Normal subjects have very low response
Sixth Annual Intensive Update in Neurology 0915-162016
64
Clinical applications
bull The o-VEMP is a sensitive test of otolithutricular
function
bull Complements vestibular test procedures that assess
semicircular canals
bull Abnormally robust responses are associated with third
window abnormalities such as semicircular canal
dehiscence
bull Serves as an excellent screening test for semicircular
canal dehiscence
Sixth Annual Intensive Update in Neurology 0915-162016
65
Question 5
bull Superior semicircular canal dehiscence
1) Causes increased ability to hear vibratory sounds
2) Improves hearing
3) Causes meningitis
4) Always needs surgical repair
Sixth Annual Intensive Update in Neurology 0915-162016
66
Question 5
bull Superior semicircular canal dehiscence
1) Causes increased ability to hear vibratory sounds
2) Improves hearing
3) Causes meningitis
4) Always needs surgical repair
Answer 1 References at end of lecture
Sixth Annual Intensive Update in Neurology 0915-162016
67
Enlarged vestibular aqueduct
bull Axial temporal bone CT showing right temporal bone with enlarged vestibular aqueduct (arrow)
bull Vestibular symptoms accompany progressive sensorineural hearing loss in 4-50 of patients in selected series
Sixth Annual Intensive Update in Neurology 0915-162016
68
Migraine
bull Family or personal history of migraine
bull 25 of migraine patients experience spontaneous vertigo (Baloh 1997)
bull Neuhauser criteria(2001) have been used to construct structured diagnostic interviews (Furman 2003)
bull Not always associated with headache
bull Motion sensitivity
bull Medication responsive
Sixth Annual Intensive Update in Neurology 0915-162016
69
ICHD-III beta Criteria for Vestibular Migraine
bull A At least five attacks fulfilling criteria C and D
bull B A current or past history of 11 migraine without aura or 12 migraine with aura
bull C Vestibular symptoms of moderate or severe intensity lasting between 5 minutes and 72 hours
bull D At least 50 associated with one of the following migrainous features
ndash 1 Headaches
ndash 2 Photophobia and phonophobia
ndash 3 Visual Aura
bull E Not better accounted for by another vestibular disorder
Sixth Annual Intensive Update in Neurology 0915-162016
70
Incidence of vertigo in MS
bull True vertigo (the perception of environmental
movement typically rotational) occurs in about
20 of MS patients and is the presenting
manifestation in 5
bull Up to 78 of MS patients report abnormalities
in balance at some point in their illness
Frohman et al Multiple Sclerosis 2003 9250
Sixth Annual Intensive Update in Neurology 0915-162016
71
Vertigo in MS due to demyelination
bull The most common neuroanatomic locations are the
root entry zone of CN VIII (giving absent calorics but
normal hearing and fixation suppression) and the
medial vestibular nucleus
bull Usually treated with steroids and vestibular
suppressants
Brandt 1993 Gass 1998 Thomke 1999
Sixth Annual Intensive Update in Neurology 0915-162016
72
Vertigo in MS
Frohmanrsquos large series (1153) reported that 52 of MS patients with acute vertigo had benign paroxysmal positional vertigo while 32 had new brainstem lesions
To avoid misdiagnoses and inappropriate treatment interventions alternative etiologies should be considered in all acute vertigo episodes in MS
E M Frohman Neurology 2000 and Multiple Sclerosis 2003
Sixth Annual Intensive Update in Neurology 0915-162016
73
Vestibular compensation
bull Acute unilateral or bilateral vestibular loss will prompt compensation thru central cervico-ocular visual and proprioceptive pathways
bull Unilateral loss may require several weeks for compensation to occur and activity especially head movements hastens compensation
bull Vestibular suppressant medication prolongs compensation
Sixth Annual Intensive Update in Neurology 0915-162016
74
Vestibular Rehabilitation
bull Performed by certified physical therapists many sites (Epic order vestibular PT) or check Vestibular Disorders of America website for providers
bull Assessment and treatment of benign positional vertigo
bull Directed rehab programs for central andor peripheral vestibular compensationndash Unilateral vestibulopathies
ndash Bilateral vestibular hypofunction
ndash Mutifactorial balance deficits and fall risk assessment
Sixth Annual Intensive Update in Neurology 0915-162016
75
Summary of whatrsquos new in peripheral vestibular disorders
bull Lateral semicircular canal benign paroxysmal positional vertigo presents with horizontal positioning nystagmus
bull Oral steroids appear to improve outcome in acute vestibular neuritis
bull Intratympanic steroids offer promise in Menierersquos syndrome
bull Superior semicircular canal dehiscence causes pressure and noise induced dizziness VEMP offers diagnostic information
Sixth Annual Intensive Update in Neurology 0915-162016
76
References
BPPVndash White J Benign paroxysmal positional vertigo Cleveland Clinic Journal of
Medicine 71(9)722-728 2004
ndash White J Coale K Catalano P Oas J Diagnosis and management of lateral semicircular canal benign positional vertigo Otolaryngology-Head and Neck Surgery 133 278-284 2005
ndash White J Savvides P Cherian N Oas J Canalith repositioning for benign paroxysmal positional vertigo Otology and Neurotology 26 704-710 2005
ndash White J Oas J Diagnosis and management of lateral semicircular canal conversions during particle repositioning therapy Laryngoscope 115(10) 1895-1897 2005
ndash Acute Vestibular Syndrome Hotson JR Baloh RW N Engl J Med 1998 Sep 3339(10)680-5
ndash Methylprednisolone valacyclovir or the combination for vestibular neuritis Strupp M Zingler VC Arbusow V Niklas D Maag KP Dieterich M Bense S Theil D Jahn K Brandt T N Engl J Med 2004 Jul 22351(4)354-61
ndash Intratympanic steroids for Meacuteniegraveres disease or syndrome Phillips JS Westerberg BCochrane Database Syst Rev 2011 Jul 6(7)CD008514 Review
ndash Sound- andor pressure-induced vertigo due to bone dehiscence of the superior semicircular canal Minor LB Solomon D Zinreich JS Zee DSArch Otolaryngol Head Neck Surg 1998 Mar124(3)249-58
Sixth Annual Intensive Update in Neurology 0915-162016
52
Question 4
bull Menierersquos syndrome diagnosis is based on
1) Recurrent vertigo lasting at least 20 minutes
2) Aural fullness
3) Documented hearing loss
4) Other causes excluded
5) All of the above
Sixth Annual Intensive Update in Neurology 0915-162016
53
Question 4
bull Menierersquos syndrome diagnosis is based on
1) Recurrent vertigo lasting at least 20 minutes
2) Aural fullness
3) Documented hearing loss
4) Other causes excluded
5) All of the above
Answer 5 See references at end
Sixth Annual Intensive Update in Neurology 0915-162016
54
Superior Semicircular Canal Dehiscence (SSCD)
bull Sound andor pressure induced oscillopsia
bull CT reformatted in the plane of the superior canals
shows dehiscence of overlying bone
bull Low frequency ldquoconductive hyperacusisrdquo with elevated
bone thresholds normal reflexes and a conductive loss
Sixth Annual Intensive Update in Neurology 0915-162016
55
SSCD
bull Positive middle ear pressure or loud sound stimulates ampullofugal endolymph flow -canal activation
bull Eye movements are tonic upwards with ipsilateral intorsion
Sixth Annual Intensive Update in Neurology 0915-162016
56
SSCD
bull Valsalva against a closed glottis or jugular compression causes increased intracranial pressure and ampullopetal endolymph flow -canal inhibition
bull Eye movements are tonic downwards with ipsilateral extorsion
Sixth Annual Intensive Update in Neurology 0915-162016
57
Reconstructed left superior semicircular canal
Sixth Annual Intensive Update in Neurology 0915-162016
58
Sixth Annual Intensive Update in Neurology 0915-162016
59
SCHEMATIC DRAWING OF AN OTOLITHIC MACULA
Sixth Annual Intensive Update in Neurology 0915-162016
60
MACULAE OF THE UTRICLESACCULE
Sixth Annual Intensive Update in Neurology 0915-162016
61
HAIR CELLS
Sixth Annual Intensive Update in Neurology 0915-162016
62
SCANNING ELECTRON MICROGRAPH OF OTOCONIA
Sixth Annual Intensive Update in Neurology 0915-162016
63
Ocular VEMP
bull Response to loud clicks (80-110 dB) or forehead tap (Halmagyi and Curthoys 2003)
bull Contralateral oculomotor nucleus (CN III)
bull Inferior oblique muscle contraction (8 msec) detected by 5sec event triggered averaging
bull Noninverting electrode inferior to eye
bull Inverting below non-inverting electrode
bull Ground to forehead
bull Normal subjects have very low response
Sixth Annual Intensive Update in Neurology 0915-162016
64
Clinical applications
bull The o-VEMP is a sensitive test of otolithutricular
function
bull Complements vestibular test procedures that assess
semicircular canals
bull Abnormally robust responses are associated with third
window abnormalities such as semicircular canal
dehiscence
bull Serves as an excellent screening test for semicircular
canal dehiscence
Sixth Annual Intensive Update in Neurology 0915-162016
65
Question 5
bull Superior semicircular canal dehiscence
1) Causes increased ability to hear vibratory sounds
2) Improves hearing
3) Causes meningitis
4) Always needs surgical repair
Sixth Annual Intensive Update in Neurology 0915-162016
66
Question 5
bull Superior semicircular canal dehiscence
1) Causes increased ability to hear vibratory sounds
2) Improves hearing
3) Causes meningitis
4) Always needs surgical repair
Answer 1 References at end of lecture
Sixth Annual Intensive Update in Neurology 0915-162016
67
Enlarged vestibular aqueduct
bull Axial temporal bone CT showing right temporal bone with enlarged vestibular aqueduct (arrow)
bull Vestibular symptoms accompany progressive sensorineural hearing loss in 4-50 of patients in selected series
Sixth Annual Intensive Update in Neurology 0915-162016
68
Migraine
bull Family or personal history of migraine
bull 25 of migraine patients experience spontaneous vertigo (Baloh 1997)
bull Neuhauser criteria(2001) have been used to construct structured diagnostic interviews (Furman 2003)
bull Not always associated with headache
bull Motion sensitivity
bull Medication responsive
Sixth Annual Intensive Update in Neurology 0915-162016
69
ICHD-III beta Criteria for Vestibular Migraine
bull A At least five attacks fulfilling criteria C and D
bull B A current or past history of 11 migraine without aura or 12 migraine with aura
bull C Vestibular symptoms of moderate or severe intensity lasting between 5 minutes and 72 hours
bull D At least 50 associated with one of the following migrainous features
ndash 1 Headaches
ndash 2 Photophobia and phonophobia
ndash 3 Visual Aura
bull E Not better accounted for by another vestibular disorder
Sixth Annual Intensive Update in Neurology 0915-162016
70
Incidence of vertigo in MS
bull True vertigo (the perception of environmental
movement typically rotational) occurs in about
20 of MS patients and is the presenting
manifestation in 5
bull Up to 78 of MS patients report abnormalities
in balance at some point in their illness
Frohman et al Multiple Sclerosis 2003 9250
Sixth Annual Intensive Update in Neurology 0915-162016
71
Vertigo in MS due to demyelination
bull The most common neuroanatomic locations are the
root entry zone of CN VIII (giving absent calorics but
normal hearing and fixation suppression) and the
medial vestibular nucleus
bull Usually treated with steroids and vestibular
suppressants
Brandt 1993 Gass 1998 Thomke 1999
Sixth Annual Intensive Update in Neurology 0915-162016
72
Vertigo in MS
Frohmanrsquos large series (1153) reported that 52 of MS patients with acute vertigo had benign paroxysmal positional vertigo while 32 had new brainstem lesions
To avoid misdiagnoses and inappropriate treatment interventions alternative etiologies should be considered in all acute vertigo episodes in MS
E M Frohman Neurology 2000 and Multiple Sclerosis 2003
Sixth Annual Intensive Update in Neurology 0915-162016
73
Vestibular compensation
bull Acute unilateral or bilateral vestibular loss will prompt compensation thru central cervico-ocular visual and proprioceptive pathways
bull Unilateral loss may require several weeks for compensation to occur and activity especially head movements hastens compensation
bull Vestibular suppressant medication prolongs compensation
Sixth Annual Intensive Update in Neurology 0915-162016
74
Vestibular Rehabilitation
bull Performed by certified physical therapists many sites (Epic order vestibular PT) or check Vestibular Disorders of America website for providers
bull Assessment and treatment of benign positional vertigo
bull Directed rehab programs for central andor peripheral vestibular compensationndash Unilateral vestibulopathies
ndash Bilateral vestibular hypofunction
ndash Mutifactorial balance deficits and fall risk assessment
Sixth Annual Intensive Update in Neurology 0915-162016
75
Summary of whatrsquos new in peripheral vestibular disorders
bull Lateral semicircular canal benign paroxysmal positional vertigo presents with horizontal positioning nystagmus
bull Oral steroids appear to improve outcome in acute vestibular neuritis
bull Intratympanic steroids offer promise in Menierersquos syndrome
bull Superior semicircular canal dehiscence causes pressure and noise induced dizziness VEMP offers diagnostic information
Sixth Annual Intensive Update in Neurology 0915-162016
76
References
BPPVndash White J Benign paroxysmal positional vertigo Cleveland Clinic Journal of
Medicine 71(9)722-728 2004
ndash White J Coale K Catalano P Oas J Diagnosis and management of lateral semicircular canal benign positional vertigo Otolaryngology-Head and Neck Surgery 133 278-284 2005
ndash White J Savvides P Cherian N Oas J Canalith repositioning for benign paroxysmal positional vertigo Otology and Neurotology 26 704-710 2005
ndash White J Oas J Diagnosis and management of lateral semicircular canal conversions during particle repositioning therapy Laryngoscope 115(10) 1895-1897 2005
ndash Acute Vestibular Syndrome Hotson JR Baloh RW N Engl J Med 1998 Sep 3339(10)680-5
ndash Methylprednisolone valacyclovir or the combination for vestibular neuritis Strupp M Zingler VC Arbusow V Niklas D Maag KP Dieterich M Bense S Theil D Jahn K Brandt T N Engl J Med 2004 Jul 22351(4)354-61
ndash Intratympanic steroids for Meacuteniegraveres disease or syndrome Phillips JS Westerberg BCochrane Database Syst Rev 2011 Jul 6(7)CD008514 Review
ndash Sound- andor pressure-induced vertigo due to bone dehiscence of the superior semicircular canal Minor LB Solomon D Zinreich JS Zee DSArch Otolaryngol Head Neck Surg 1998 Mar124(3)249-58
Sixth Annual Intensive Update in Neurology 0915-162016
53
Question 4
bull Menierersquos syndrome diagnosis is based on
1) Recurrent vertigo lasting at least 20 minutes
2) Aural fullness
3) Documented hearing loss
4) Other causes excluded
5) All of the above
Answer 5 See references at end
Sixth Annual Intensive Update in Neurology 0915-162016
54
Superior Semicircular Canal Dehiscence (SSCD)
bull Sound andor pressure induced oscillopsia
bull CT reformatted in the plane of the superior canals
shows dehiscence of overlying bone
bull Low frequency ldquoconductive hyperacusisrdquo with elevated
bone thresholds normal reflexes and a conductive loss
Sixth Annual Intensive Update in Neurology 0915-162016
55
SSCD
bull Positive middle ear pressure or loud sound stimulates ampullofugal endolymph flow -canal activation
bull Eye movements are tonic upwards with ipsilateral intorsion
Sixth Annual Intensive Update in Neurology 0915-162016
56
SSCD
bull Valsalva against a closed glottis or jugular compression causes increased intracranial pressure and ampullopetal endolymph flow -canal inhibition
bull Eye movements are tonic downwards with ipsilateral extorsion
Sixth Annual Intensive Update in Neurology 0915-162016
57
Reconstructed left superior semicircular canal
Sixth Annual Intensive Update in Neurology 0915-162016
58
Sixth Annual Intensive Update in Neurology 0915-162016
59
SCHEMATIC DRAWING OF AN OTOLITHIC MACULA
Sixth Annual Intensive Update in Neurology 0915-162016
60
MACULAE OF THE UTRICLESACCULE
Sixth Annual Intensive Update in Neurology 0915-162016
61
HAIR CELLS
Sixth Annual Intensive Update in Neurology 0915-162016
62
SCANNING ELECTRON MICROGRAPH OF OTOCONIA
Sixth Annual Intensive Update in Neurology 0915-162016
63
Ocular VEMP
bull Response to loud clicks (80-110 dB) or forehead tap (Halmagyi and Curthoys 2003)
bull Contralateral oculomotor nucleus (CN III)
bull Inferior oblique muscle contraction (8 msec) detected by 5sec event triggered averaging
bull Noninverting electrode inferior to eye
bull Inverting below non-inverting electrode
bull Ground to forehead
bull Normal subjects have very low response
Sixth Annual Intensive Update in Neurology 0915-162016
64
Clinical applications
bull The o-VEMP is a sensitive test of otolithutricular
function
bull Complements vestibular test procedures that assess
semicircular canals
bull Abnormally robust responses are associated with third
window abnormalities such as semicircular canal
dehiscence
bull Serves as an excellent screening test for semicircular
canal dehiscence
Sixth Annual Intensive Update in Neurology 0915-162016
65
Question 5
bull Superior semicircular canal dehiscence
1) Causes increased ability to hear vibratory sounds
2) Improves hearing
3) Causes meningitis
4) Always needs surgical repair
Sixth Annual Intensive Update in Neurology 0915-162016
66
Question 5
bull Superior semicircular canal dehiscence
1) Causes increased ability to hear vibratory sounds
2) Improves hearing
3) Causes meningitis
4) Always needs surgical repair
Answer 1 References at end of lecture
Sixth Annual Intensive Update in Neurology 0915-162016
67
Enlarged vestibular aqueduct
bull Axial temporal bone CT showing right temporal bone with enlarged vestibular aqueduct (arrow)
bull Vestibular symptoms accompany progressive sensorineural hearing loss in 4-50 of patients in selected series
Sixth Annual Intensive Update in Neurology 0915-162016
68
Migraine
bull Family or personal history of migraine
bull 25 of migraine patients experience spontaneous vertigo (Baloh 1997)
bull Neuhauser criteria(2001) have been used to construct structured diagnostic interviews (Furman 2003)
bull Not always associated with headache
bull Motion sensitivity
bull Medication responsive
Sixth Annual Intensive Update in Neurology 0915-162016
69
ICHD-III beta Criteria for Vestibular Migraine
bull A At least five attacks fulfilling criteria C and D
bull B A current or past history of 11 migraine without aura or 12 migraine with aura
bull C Vestibular symptoms of moderate or severe intensity lasting between 5 minutes and 72 hours
bull D At least 50 associated with one of the following migrainous features
ndash 1 Headaches
ndash 2 Photophobia and phonophobia
ndash 3 Visual Aura
bull E Not better accounted for by another vestibular disorder
Sixth Annual Intensive Update in Neurology 0915-162016
70
Incidence of vertigo in MS
bull True vertigo (the perception of environmental
movement typically rotational) occurs in about
20 of MS patients and is the presenting
manifestation in 5
bull Up to 78 of MS patients report abnormalities
in balance at some point in their illness
Frohman et al Multiple Sclerosis 2003 9250
Sixth Annual Intensive Update in Neurology 0915-162016
71
Vertigo in MS due to demyelination
bull The most common neuroanatomic locations are the
root entry zone of CN VIII (giving absent calorics but
normal hearing and fixation suppression) and the
medial vestibular nucleus
bull Usually treated with steroids and vestibular
suppressants
Brandt 1993 Gass 1998 Thomke 1999
Sixth Annual Intensive Update in Neurology 0915-162016
72
Vertigo in MS
Frohmanrsquos large series (1153) reported that 52 of MS patients with acute vertigo had benign paroxysmal positional vertigo while 32 had new brainstem lesions
To avoid misdiagnoses and inappropriate treatment interventions alternative etiologies should be considered in all acute vertigo episodes in MS
E M Frohman Neurology 2000 and Multiple Sclerosis 2003
Sixth Annual Intensive Update in Neurology 0915-162016
73
Vestibular compensation
bull Acute unilateral or bilateral vestibular loss will prompt compensation thru central cervico-ocular visual and proprioceptive pathways
bull Unilateral loss may require several weeks for compensation to occur and activity especially head movements hastens compensation
bull Vestibular suppressant medication prolongs compensation
Sixth Annual Intensive Update in Neurology 0915-162016
74
Vestibular Rehabilitation
bull Performed by certified physical therapists many sites (Epic order vestibular PT) or check Vestibular Disorders of America website for providers
bull Assessment and treatment of benign positional vertigo
bull Directed rehab programs for central andor peripheral vestibular compensationndash Unilateral vestibulopathies
ndash Bilateral vestibular hypofunction
ndash Mutifactorial balance deficits and fall risk assessment
Sixth Annual Intensive Update in Neurology 0915-162016
75
Summary of whatrsquos new in peripheral vestibular disorders
bull Lateral semicircular canal benign paroxysmal positional vertigo presents with horizontal positioning nystagmus
bull Oral steroids appear to improve outcome in acute vestibular neuritis
bull Intratympanic steroids offer promise in Menierersquos syndrome
bull Superior semicircular canal dehiscence causes pressure and noise induced dizziness VEMP offers diagnostic information
Sixth Annual Intensive Update in Neurology 0915-162016
76
References
BPPVndash White J Benign paroxysmal positional vertigo Cleveland Clinic Journal of
Medicine 71(9)722-728 2004
ndash White J Coale K Catalano P Oas J Diagnosis and management of lateral semicircular canal benign positional vertigo Otolaryngology-Head and Neck Surgery 133 278-284 2005
ndash White J Savvides P Cherian N Oas J Canalith repositioning for benign paroxysmal positional vertigo Otology and Neurotology 26 704-710 2005
ndash White J Oas J Diagnosis and management of lateral semicircular canal conversions during particle repositioning therapy Laryngoscope 115(10) 1895-1897 2005
ndash Acute Vestibular Syndrome Hotson JR Baloh RW N Engl J Med 1998 Sep 3339(10)680-5
ndash Methylprednisolone valacyclovir or the combination for vestibular neuritis Strupp M Zingler VC Arbusow V Niklas D Maag KP Dieterich M Bense S Theil D Jahn K Brandt T N Engl J Med 2004 Jul 22351(4)354-61
ndash Intratympanic steroids for Meacuteniegraveres disease or syndrome Phillips JS Westerberg BCochrane Database Syst Rev 2011 Jul 6(7)CD008514 Review
ndash Sound- andor pressure-induced vertigo due to bone dehiscence of the superior semicircular canal Minor LB Solomon D Zinreich JS Zee DSArch Otolaryngol Head Neck Surg 1998 Mar124(3)249-58
Sixth Annual Intensive Update in Neurology 0915-162016
54
Superior Semicircular Canal Dehiscence (SSCD)
bull Sound andor pressure induced oscillopsia
bull CT reformatted in the plane of the superior canals
shows dehiscence of overlying bone
bull Low frequency ldquoconductive hyperacusisrdquo with elevated
bone thresholds normal reflexes and a conductive loss
Sixth Annual Intensive Update in Neurology 0915-162016
55
SSCD
bull Positive middle ear pressure or loud sound stimulates ampullofugal endolymph flow -canal activation
bull Eye movements are tonic upwards with ipsilateral intorsion
Sixth Annual Intensive Update in Neurology 0915-162016
56
SSCD
bull Valsalva against a closed glottis or jugular compression causes increased intracranial pressure and ampullopetal endolymph flow -canal inhibition
bull Eye movements are tonic downwards with ipsilateral extorsion
Sixth Annual Intensive Update in Neurology 0915-162016
57
Reconstructed left superior semicircular canal
Sixth Annual Intensive Update in Neurology 0915-162016
58
Sixth Annual Intensive Update in Neurology 0915-162016
59
SCHEMATIC DRAWING OF AN OTOLITHIC MACULA
Sixth Annual Intensive Update in Neurology 0915-162016
60
MACULAE OF THE UTRICLESACCULE
Sixth Annual Intensive Update in Neurology 0915-162016
61
HAIR CELLS
Sixth Annual Intensive Update in Neurology 0915-162016
62
SCANNING ELECTRON MICROGRAPH OF OTOCONIA
Sixth Annual Intensive Update in Neurology 0915-162016
63
Ocular VEMP
bull Response to loud clicks (80-110 dB) or forehead tap (Halmagyi and Curthoys 2003)
bull Contralateral oculomotor nucleus (CN III)
bull Inferior oblique muscle contraction (8 msec) detected by 5sec event triggered averaging
bull Noninverting electrode inferior to eye
bull Inverting below non-inverting electrode
bull Ground to forehead
bull Normal subjects have very low response
Sixth Annual Intensive Update in Neurology 0915-162016
64
Clinical applications
bull The o-VEMP is a sensitive test of otolithutricular
function
bull Complements vestibular test procedures that assess
semicircular canals
bull Abnormally robust responses are associated with third
window abnormalities such as semicircular canal
dehiscence
bull Serves as an excellent screening test for semicircular
canal dehiscence
Sixth Annual Intensive Update in Neurology 0915-162016
65
Question 5
bull Superior semicircular canal dehiscence
1) Causes increased ability to hear vibratory sounds
2) Improves hearing
3) Causes meningitis
4) Always needs surgical repair
Sixth Annual Intensive Update in Neurology 0915-162016
66
Question 5
bull Superior semicircular canal dehiscence
1) Causes increased ability to hear vibratory sounds
2) Improves hearing
3) Causes meningitis
4) Always needs surgical repair
Answer 1 References at end of lecture
Sixth Annual Intensive Update in Neurology 0915-162016
67
Enlarged vestibular aqueduct
bull Axial temporal bone CT showing right temporal bone with enlarged vestibular aqueduct (arrow)
bull Vestibular symptoms accompany progressive sensorineural hearing loss in 4-50 of patients in selected series
Sixth Annual Intensive Update in Neurology 0915-162016
68
Migraine
bull Family or personal history of migraine
bull 25 of migraine patients experience spontaneous vertigo (Baloh 1997)
bull Neuhauser criteria(2001) have been used to construct structured diagnostic interviews (Furman 2003)
bull Not always associated with headache
bull Motion sensitivity
bull Medication responsive
Sixth Annual Intensive Update in Neurology 0915-162016
69
ICHD-III beta Criteria for Vestibular Migraine
bull A At least five attacks fulfilling criteria C and D
bull B A current or past history of 11 migraine without aura or 12 migraine with aura
bull C Vestibular symptoms of moderate or severe intensity lasting between 5 minutes and 72 hours
bull D At least 50 associated with one of the following migrainous features
ndash 1 Headaches
ndash 2 Photophobia and phonophobia
ndash 3 Visual Aura
bull E Not better accounted for by another vestibular disorder
Sixth Annual Intensive Update in Neurology 0915-162016
70
Incidence of vertigo in MS
bull True vertigo (the perception of environmental
movement typically rotational) occurs in about
20 of MS patients and is the presenting
manifestation in 5
bull Up to 78 of MS patients report abnormalities
in balance at some point in their illness
Frohman et al Multiple Sclerosis 2003 9250
Sixth Annual Intensive Update in Neurology 0915-162016
71
Vertigo in MS due to demyelination
bull The most common neuroanatomic locations are the
root entry zone of CN VIII (giving absent calorics but
normal hearing and fixation suppression) and the
medial vestibular nucleus
bull Usually treated with steroids and vestibular
suppressants
Brandt 1993 Gass 1998 Thomke 1999
Sixth Annual Intensive Update in Neurology 0915-162016
72
Vertigo in MS
Frohmanrsquos large series (1153) reported that 52 of MS patients with acute vertigo had benign paroxysmal positional vertigo while 32 had new brainstem lesions
To avoid misdiagnoses and inappropriate treatment interventions alternative etiologies should be considered in all acute vertigo episodes in MS
E M Frohman Neurology 2000 and Multiple Sclerosis 2003
Sixth Annual Intensive Update in Neurology 0915-162016
73
Vestibular compensation
bull Acute unilateral or bilateral vestibular loss will prompt compensation thru central cervico-ocular visual and proprioceptive pathways
bull Unilateral loss may require several weeks for compensation to occur and activity especially head movements hastens compensation
bull Vestibular suppressant medication prolongs compensation
Sixth Annual Intensive Update in Neurology 0915-162016
74
Vestibular Rehabilitation
bull Performed by certified physical therapists many sites (Epic order vestibular PT) or check Vestibular Disorders of America website for providers
bull Assessment and treatment of benign positional vertigo
bull Directed rehab programs for central andor peripheral vestibular compensationndash Unilateral vestibulopathies
ndash Bilateral vestibular hypofunction
ndash Mutifactorial balance deficits and fall risk assessment
Sixth Annual Intensive Update in Neurology 0915-162016
75
Summary of whatrsquos new in peripheral vestibular disorders
bull Lateral semicircular canal benign paroxysmal positional vertigo presents with horizontal positioning nystagmus
bull Oral steroids appear to improve outcome in acute vestibular neuritis
bull Intratympanic steroids offer promise in Menierersquos syndrome
bull Superior semicircular canal dehiscence causes pressure and noise induced dizziness VEMP offers diagnostic information
Sixth Annual Intensive Update in Neurology 0915-162016
76
References
BPPVndash White J Benign paroxysmal positional vertigo Cleveland Clinic Journal of
Medicine 71(9)722-728 2004
ndash White J Coale K Catalano P Oas J Diagnosis and management of lateral semicircular canal benign positional vertigo Otolaryngology-Head and Neck Surgery 133 278-284 2005
ndash White J Savvides P Cherian N Oas J Canalith repositioning for benign paroxysmal positional vertigo Otology and Neurotology 26 704-710 2005
ndash White J Oas J Diagnosis and management of lateral semicircular canal conversions during particle repositioning therapy Laryngoscope 115(10) 1895-1897 2005
ndash Acute Vestibular Syndrome Hotson JR Baloh RW N Engl J Med 1998 Sep 3339(10)680-5
ndash Methylprednisolone valacyclovir or the combination for vestibular neuritis Strupp M Zingler VC Arbusow V Niklas D Maag KP Dieterich M Bense S Theil D Jahn K Brandt T N Engl J Med 2004 Jul 22351(4)354-61
ndash Intratympanic steroids for Meacuteniegraveres disease or syndrome Phillips JS Westerberg BCochrane Database Syst Rev 2011 Jul 6(7)CD008514 Review
ndash Sound- andor pressure-induced vertigo due to bone dehiscence of the superior semicircular canal Minor LB Solomon D Zinreich JS Zee DSArch Otolaryngol Head Neck Surg 1998 Mar124(3)249-58
Sixth Annual Intensive Update in Neurology 0915-162016
55
SSCD
bull Positive middle ear pressure or loud sound stimulates ampullofugal endolymph flow -canal activation
bull Eye movements are tonic upwards with ipsilateral intorsion
Sixth Annual Intensive Update in Neurology 0915-162016
56
SSCD
bull Valsalva against a closed glottis or jugular compression causes increased intracranial pressure and ampullopetal endolymph flow -canal inhibition
bull Eye movements are tonic downwards with ipsilateral extorsion
Sixth Annual Intensive Update in Neurology 0915-162016
57
Reconstructed left superior semicircular canal
Sixth Annual Intensive Update in Neurology 0915-162016
58
Sixth Annual Intensive Update in Neurology 0915-162016
59
SCHEMATIC DRAWING OF AN OTOLITHIC MACULA
Sixth Annual Intensive Update in Neurology 0915-162016
60
MACULAE OF THE UTRICLESACCULE
Sixth Annual Intensive Update in Neurology 0915-162016
61
HAIR CELLS
Sixth Annual Intensive Update in Neurology 0915-162016
62
SCANNING ELECTRON MICROGRAPH OF OTOCONIA
Sixth Annual Intensive Update in Neurology 0915-162016
63
Ocular VEMP
bull Response to loud clicks (80-110 dB) or forehead tap (Halmagyi and Curthoys 2003)
bull Contralateral oculomotor nucleus (CN III)
bull Inferior oblique muscle contraction (8 msec) detected by 5sec event triggered averaging
bull Noninverting electrode inferior to eye
bull Inverting below non-inverting electrode
bull Ground to forehead
bull Normal subjects have very low response
Sixth Annual Intensive Update in Neurology 0915-162016
64
Clinical applications
bull The o-VEMP is a sensitive test of otolithutricular
function
bull Complements vestibular test procedures that assess
semicircular canals
bull Abnormally robust responses are associated with third
window abnormalities such as semicircular canal
dehiscence
bull Serves as an excellent screening test for semicircular
canal dehiscence
Sixth Annual Intensive Update in Neurology 0915-162016
65
Question 5
bull Superior semicircular canal dehiscence
1) Causes increased ability to hear vibratory sounds
2) Improves hearing
3) Causes meningitis
4) Always needs surgical repair
Sixth Annual Intensive Update in Neurology 0915-162016
66
Question 5
bull Superior semicircular canal dehiscence
1) Causes increased ability to hear vibratory sounds
2) Improves hearing
3) Causes meningitis
4) Always needs surgical repair
Answer 1 References at end of lecture
Sixth Annual Intensive Update in Neurology 0915-162016
67
Enlarged vestibular aqueduct
bull Axial temporal bone CT showing right temporal bone with enlarged vestibular aqueduct (arrow)
bull Vestibular symptoms accompany progressive sensorineural hearing loss in 4-50 of patients in selected series
Sixth Annual Intensive Update in Neurology 0915-162016
68
Migraine
bull Family or personal history of migraine
bull 25 of migraine patients experience spontaneous vertigo (Baloh 1997)
bull Neuhauser criteria(2001) have been used to construct structured diagnostic interviews (Furman 2003)
bull Not always associated with headache
bull Motion sensitivity
bull Medication responsive
Sixth Annual Intensive Update in Neurology 0915-162016
69
ICHD-III beta Criteria for Vestibular Migraine
bull A At least five attacks fulfilling criteria C and D
bull B A current or past history of 11 migraine without aura or 12 migraine with aura
bull C Vestibular symptoms of moderate or severe intensity lasting between 5 minutes and 72 hours
bull D At least 50 associated with one of the following migrainous features
ndash 1 Headaches
ndash 2 Photophobia and phonophobia
ndash 3 Visual Aura
bull E Not better accounted for by another vestibular disorder
Sixth Annual Intensive Update in Neurology 0915-162016
70
Incidence of vertigo in MS
bull True vertigo (the perception of environmental
movement typically rotational) occurs in about
20 of MS patients and is the presenting
manifestation in 5
bull Up to 78 of MS patients report abnormalities
in balance at some point in their illness
Frohman et al Multiple Sclerosis 2003 9250
Sixth Annual Intensive Update in Neurology 0915-162016
71
Vertigo in MS due to demyelination
bull The most common neuroanatomic locations are the
root entry zone of CN VIII (giving absent calorics but
normal hearing and fixation suppression) and the
medial vestibular nucleus
bull Usually treated with steroids and vestibular
suppressants
Brandt 1993 Gass 1998 Thomke 1999
Sixth Annual Intensive Update in Neurology 0915-162016
72
Vertigo in MS
Frohmanrsquos large series (1153) reported that 52 of MS patients with acute vertigo had benign paroxysmal positional vertigo while 32 had new brainstem lesions
To avoid misdiagnoses and inappropriate treatment interventions alternative etiologies should be considered in all acute vertigo episodes in MS
E M Frohman Neurology 2000 and Multiple Sclerosis 2003
Sixth Annual Intensive Update in Neurology 0915-162016
73
Vestibular compensation
bull Acute unilateral or bilateral vestibular loss will prompt compensation thru central cervico-ocular visual and proprioceptive pathways
bull Unilateral loss may require several weeks for compensation to occur and activity especially head movements hastens compensation
bull Vestibular suppressant medication prolongs compensation
Sixth Annual Intensive Update in Neurology 0915-162016
74
Vestibular Rehabilitation
bull Performed by certified physical therapists many sites (Epic order vestibular PT) or check Vestibular Disorders of America website for providers
bull Assessment and treatment of benign positional vertigo
bull Directed rehab programs for central andor peripheral vestibular compensationndash Unilateral vestibulopathies
ndash Bilateral vestibular hypofunction
ndash Mutifactorial balance deficits and fall risk assessment
Sixth Annual Intensive Update in Neurology 0915-162016
75
Summary of whatrsquos new in peripheral vestibular disorders
bull Lateral semicircular canal benign paroxysmal positional vertigo presents with horizontal positioning nystagmus
bull Oral steroids appear to improve outcome in acute vestibular neuritis
bull Intratympanic steroids offer promise in Menierersquos syndrome
bull Superior semicircular canal dehiscence causes pressure and noise induced dizziness VEMP offers diagnostic information
Sixth Annual Intensive Update in Neurology 0915-162016
76
References
BPPVndash White J Benign paroxysmal positional vertigo Cleveland Clinic Journal of
Medicine 71(9)722-728 2004
ndash White J Coale K Catalano P Oas J Diagnosis and management of lateral semicircular canal benign positional vertigo Otolaryngology-Head and Neck Surgery 133 278-284 2005
ndash White J Savvides P Cherian N Oas J Canalith repositioning for benign paroxysmal positional vertigo Otology and Neurotology 26 704-710 2005
ndash White J Oas J Diagnosis and management of lateral semicircular canal conversions during particle repositioning therapy Laryngoscope 115(10) 1895-1897 2005
ndash Acute Vestibular Syndrome Hotson JR Baloh RW N Engl J Med 1998 Sep 3339(10)680-5
ndash Methylprednisolone valacyclovir or the combination for vestibular neuritis Strupp M Zingler VC Arbusow V Niklas D Maag KP Dieterich M Bense S Theil D Jahn K Brandt T N Engl J Med 2004 Jul 22351(4)354-61
ndash Intratympanic steroids for Meacuteniegraveres disease or syndrome Phillips JS Westerberg BCochrane Database Syst Rev 2011 Jul 6(7)CD008514 Review
ndash Sound- andor pressure-induced vertigo due to bone dehiscence of the superior semicircular canal Minor LB Solomon D Zinreich JS Zee DSArch Otolaryngol Head Neck Surg 1998 Mar124(3)249-58
Sixth Annual Intensive Update in Neurology 0915-162016
56
SSCD
bull Valsalva against a closed glottis or jugular compression causes increased intracranial pressure and ampullopetal endolymph flow -canal inhibition
bull Eye movements are tonic downwards with ipsilateral extorsion
Sixth Annual Intensive Update in Neurology 0915-162016
57
Reconstructed left superior semicircular canal
Sixth Annual Intensive Update in Neurology 0915-162016
58
Sixth Annual Intensive Update in Neurology 0915-162016
59
SCHEMATIC DRAWING OF AN OTOLITHIC MACULA
Sixth Annual Intensive Update in Neurology 0915-162016
60
MACULAE OF THE UTRICLESACCULE
Sixth Annual Intensive Update in Neurology 0915-162016
61
HAIR CELLS
Sixth Annual Intensive Update in Neurology 0915-162016
62
SCANNING ELECTRON MICROGRAPH OF OTOCONIA
Sixth Annual Intensive Update in Neurology 0915-162016
63
Ocular VEMP
bull Response to loud clicks (80-110 dB) or forehead tap (Halmagyi and Curthoys 2003)
bull Contralateral oculomotor nucleus (CN III)
bull Inferior oblique muscle contraction (8 msec) detected by 5sec event triggered averaging
bull Noninverting electrode inferior to eye
bull Inverting below non-inverting electrode
bull Ground to forehead
bull Normal subjects have very low response
Sixth Annual Intensive Update in Neurology 0915-162016
64
Clinical applications
bull The o-VEMP is a sensitive test of otolithutricular
function
bull Complements vestibular test procedures that assess
semicircular canals
bull Abnormally robust responses are associated with third
window abnormalities such as semicircular canal
dehiscence
bull Serves as an excellent screening test for semicircular
canal dehiscence
Sixth Annual Intensive Update in Neurology 0915-162016
65
Question 5
bull Superior semicircular canal dehiscence
1) Causes increased ability to hear vibratory sounds
2) Improves hearing
3) Causes meningitis
4) Always needs surgical repair
Sixth Annual Intensive Update in Neurology 0915-162016
66
Question 5
bull Superior semicircular canal dehiscence
1) Causes increased ability to hear vibratory sounds
2) Improves hearing
3) Causes meningitis
4) Always needs surgical repair
Answer 1 References at end of lecture
Sixth Annual Intensive Update in Neurology 0915-162016
67
Enlarged vestibular aqueduct
bull Axial temporal bone CT showing right temporal bone with enlarged vestibular aqueduct (arrow)
bull Vestibular symptoms accompany progressive sensorineural hearing loss in 4-50 of patients in selected series
Sixth Annual Intensive Update in Neurology 0915-162016
68
Migraine
bull Family or personal history of migraine
bull 25 of migraine patients experience spontaneous vertigo (Baloh 1997)
bull Neuhauser criteria(2001) have been used to construct structured diagnostic interviews (Furman 2003)
bull Not always associated with headache
bull Motion sensitivity
bull Medication responsive
Sixth Annual Intensive Update in Neurology 0915-162016
69
ICHD-III beta Criteria for Vestibular Migraine
bull A At least five attacks fulfilling criteria C and D
bull B A current or past history of 11 migraine without aura or 12 migraine with aura
bull C Vestibular symptoms of moderate or severe intensity lasting between 5 minutes and 72 hours
bull D At least 50 associated with one of the following migrainous features
ndash 1 Headaches
ndash 2 Photophobia and phonophobia
ndash 3 Visual Aura
bull E Not better accounted for by another vestibular disorder
Sixth Annual Intensive Update in Neurology 0915-162016
70
Incidence of vertigo in MS
bull True vertigo (the perception of environmental
movement typically rotational) occurs in about
20 of MS patients and is the presenting
manifestation in 5
bull Up to 78 of MS patients report abnormalities
in balance at some point in their illness
Frohman et al Multiple Sclerosis 2003 9250
Sixth Annual Intensive Update in Neurology 0915-162016
71
Vertigo in MS due to demyelination
bull The most common neuroanatomic locations are the
root entry zone of CN VIII (giving absent calorics but
normal hearing and fixation suppression) and the
medial vestibular nucleus
bull Usually treated with steroids and vestibular
suppressants
Brandt 1993 Gass 1998 Thomke 1999
Sixth Annual Intensive Update in Neurology 0915-162016
72
Vertigo in MS
Frohmanrsquos large series (1153) reported that 52 of MS patients with acute vertigo had benign paroxysmal positional vertigo while 32 had new brainstem lesions
To avoid misdiagnoses and inappropriate treatment interventions alternative etiologies should be considered in all acute vertigo episodes in MS
E M Frohman Neurology 2000 and Multiple Sclerosis 2003
Sixth Annual Intensive Update in Neurology 0915-162016
73
Vestibular compensation
bull Acute unilateral or bilateral vestibular loss will prompt compensation thru central cervico-ocular visual and proprioceptive pathways
bull Unilateral loss may require several weeks for compensation to occur and activity especially head movements hastens compensation
bull Vestibular suppressant medication prolongs compensation
Sixth Annual Intensive Update in Neurology 0915-162016
74
Vestibular Rehabilitation
bull Performed by certified physical therapists many sites (Epic order vestibular PT) or check Vestibular Disorders of America website for providers
bull Assessment and treatment of benign positional vertigo
bull Directed rehab programs for central andor peripheral vestibular compensationndash Unilateral vestibulopathies
ndash Bilateral vestibular hypofunction
ndash Mutifactorial balance deficits and fall risk assessment
Sixth Annual Intensive Update in Neurology 0915-162016
75
Summary of whatrsquos new in peripheral vestibular disorders
bull Lateral semicircular canal benign paroxysmal positional vertigo presents with horizontal positioning nystagmus
bull Oral steroids appear to improve outcome in acute vestibular neuritis
bull Intratympanic steroids offer promise in Menierersquos syndrome
bull Superior semicircular canal dehiscence causes pressure and noise induced dizziness VEMP offers diagnostic information
Sixth Annual Intensive Update in Neurology 0915-162016
76
References
BPPVndash White J Benign paroxysmal positional vertigo Cleveland Clinic Journal of
Medicine 71(9)722-728 2004
ndash White J Coale K Catalano P Oas J Diagnosis and management of lateral semicircular canal benign positional vertigo Otolaryngology-Head and Neck Surgery 133 278-284 2005
ndash White J Savvides P Cherian N Oas J Canalith repositioning for benign paroxysmal positional vertigo Otology and Neurotology 26 704-710 2005
ndash White J Oas J Diagnosis and management of lateral semicircular canal conversions during particle repositioning therapy Laryngoscope 115(10) 1895-1897 2005
ndash Acute Vestibular Syndrome Hotson JR Baloh RW N Engl J Med 1998 Sep 3339(10)680-5
ndash Methylprednisolone valacyclovir or the combination for vestibular neuritis Strupp M Zingler VC Arbusow V Niklas D Maag KP Dieterich M Bense S Theil D Jahn K Brandt T N Engl J Med 2004 Jul 22351(4)354-61
ndash Intratympanic steroids for Meacuteniegraveres disease or syndrome Phillips JS Westerberg BCochrane Database Syst Rev 2011 Jul 6(7)CD008514 Review
ndash Sound- andor pressure-induced vertigo due to bone dehiscence of the superior semicircular canal Minor LB Solomon D Zinreich JS Zee DSArch Otolaryngol Head Neck Surg 1998 Mar124(3)249-58
Sixth Annual Intensive Update in Neurology 0915-162016
57
Reconstructed left superior semicircular canal
Sixth Annual Intensive Update in Neurology 0915-162016
58
Sixth Annual Intensive Update in Neurology 0915-162016
59
SCHEMATIC DRAWING OF AN OTOLITHIC MACULA
Sixth Annual Intensive Update in Neurology 0915-162016
60
MACULAE OF THE UTRICLESACCULE
Sixth Annual Intensive Update in Neurology 0915-162016
61
HAIR CELLS
Sixth Annual Intensive Update in Neurology 0915-162016
62
SCANNING ELECTRON MICROGRAPH OF OTOCONIA
Sixth Annual Intensive Update in Neurology 0915-162016
63
Ocular VEMP
bull Response to loud clicks (80-110 dB) or forehead tap (Halmagyi and Curthoys 2003)
bull Contralateral oculomotor nucleus (CN III)
bull Inferior oblique muscle contraction (8 msec) detected by 5sec event triggered averaging
bull Noninverting electrode inferior to eye
bull Inverting below non-inverting electrode
bull Ground to forehead
bull Normal subjects have very low response
Sixth Annual Intensive Update in Neurology 0915-162016
64
Clinical applications
bull The o-VEMP is a sensitive test of otolithutricular
function
bull Complements vestibular test procedures that assess
semicircular canals
bull Abnormally robust responses are associated with third
window abnormalities such as semicircular canal
dehiscence
bull Serves as an excellent screening test for semicircular
canal dehiscence
Sixth Annual Intensive Update in Neurology 0915-162016
65
Question 5
bull Superior semicircular canal dehiscence
1) Causes increased ability to hear vibratory sounds
2) Improves hearing
3) Causes meningitis
4) Always needs surgical repair
Sixth Annual Intensive Update in Neurology 0915-162016
66
Question 5
bull Superior semicircular canal dehiscence
1) Causes increased ability to hear vibratory sounds
2) Improves hearing
3) Causes meningitis
4) Always needs surgical repair
Answer 1 References at end of lecture
Sixth Annual Intensive Update in Neurology 0915-162016
67
Enlarged vestibular aqueduct
bull Axial temporal bone CT showing right temporal bone with enlarged vestibular aqueduct (arrow)
bull Vestibular symptoms accompany progressive sensorineural hearing loss in 4-50 of patients in selected series
Sixth Annual Intensive Update in Neurology 0915-162016
68
Migraine
bull Family or personal history of migraine
bull 25 of migraine patients experience spontaneous vertigo (Baloh 1997)
bull Neuhauser criteria(2001) have been used to construct structured diagnostic interviews (Furman 2003)
bull Not always associated with headache
bull Motion sensitivity
bull Medication responsive
Sixth Annual Intensive Update in Neurology 0915-162016
69
ICHD-III beta Criteria for Vestibular Migraine
bull A At least five attacks fulfilling criteria C and D
bull B A current or past history of 11 migraine without aura or 12 migraine with aura
bull C Vestibular symptoms of moderate or severe intensity lasting between 5 minutes and 72 hours
bull D At least 50 associated with one of the following migrainous features
ndash 1 Headaches
ndash 2 Photophobia and phonophobia
ndash 3 Visual Aura
bull E Not better accounted for by another vestibular disorder
Sixth Annual Intensive Update in Neurology 0915-162016
70
Incidence of vertigo in MS
bull True vertigo (the perception of environmental
movement typically rotational) occurs in about
20 of MS patients and is the presenting
manifestation in 5
bull Up to 78 of MS patients report abnormalities
in balance at some point in their illness
Frohman et al Multiple Sclerosis 2003 9250
Sixth Annual Intensive Update in Neurology 0915-162016
71
Vertigo in MS due to demyelination
bull The most common neuroanatomic locations are the
root entry zone of CN VIII (giving absent calorics but
normal hearing and fixation suppression) and the
medial vestibular nucleus
bull Usually treated with steroids and vestibular
suppressants
Brandt 1993 Gass 1998 Thomke 1999
Sixth Annual Intensive Update in Neurology 0915-162016
72
Vertigo in MS
Frohmanrsquos large series (1153) reported that 52 of MS patients with acute vertigo had benign paroxysmal positional vertigo while 32 had new brainstem lesions
To avoid misdiagnoses and inappropriate treatment interventions alternative etiologies should be considered in all acute vertigo episodes in MS
E M Frohman Neurology 2000 and Multiple Sclerosis 2003
Sixth Annual Intensive Update in Neurology 0915-162016
73
Vestibular compensation
bull Acute unilateral or bilateral vestibular loss will prompt compensation thru central cervico-ocular visual and proprioceptive pathways
bull Unilateral loss may require several weeks for compensation to occur and activity especially head movements hastens compensation
bull Vestibular suppressant medication prolongs compensation
Sixth Annual Intensive Update in Neurology 0915-162016
74
Vestibular Rehabilitation
bull Performed by certified physical therapists many sites (Epic order vestibular PT) or check Vestibular Disorders of America website for providers
bull Assessment and treatment of benign positional vertigo
bull Directed rehab programs for central andor peripheral vestibular compensationndash Unilateral vestibulopathies
ndash Bilateral vestibular hypofunction
ndash Mutifactorial balance deficits and fall risk assessment
Sixth Annual Intensive Update in Neurology 0915-162016
75
Summary of whatrsquos new in peripheral vestibular disorders
bull Lateral semicircular canal benign paroxysmal positional vertigo presents with horizontal positioning nystagmus
bull Oral steroids appear to improve outcome in acute vestibular neuritis
bull Intratympanic steroids offer promise in Menierersquos syndrome
bull Superior semicircular canal dehiscence causes pressure and noise induced dizziness VEMP offers diagnostic information
Sixth Annual Intensive Update in Neurology 0915-162016
76
References
BPPVndash White J Benign paroxysmal positional vertigo Cleveland Clinic Journal of
Medicine 71(9)722-728 2004
ndash White J Coale K Catalano P Oas J Diagnosis and management of lateral semicircular canal benign positional vertigo Otolaryngology-Head and Neck Surgery 133 278-284 2005
ndash White J Savvides P Cherian N Oas J Canalith repositioning for benign paroxysmal positional vertigo Otology and Neurotology 26 704-710 2005
ndash White J Oas J Diagnosis and management of lateral semicircular canal conversions during particle repositioning therapy Laryngoscope 115(10) 1895-1897 2005
ndash Acute Vestibular Syndrome Hotson JR Baloh RW N Engl J Med 1998 Sep 3339(10)680-5
ndash Methylprednisolone valacyclovir or the combination for vestibular neuritis Strupp M Zingler VC Arbusow V Niklas D Maag KP Dieterich M Bense S Theil D Jahn K Brandt T N Engl J Med 2004 Jul 22351(4)354-61
ndash Intratympanic steroids for Meacuteniegraveres disease or syndrome Phillips JS Westerberg BCochrane Database Syst Rev 2011 Jul 6(7)CD008514 Review
ndash Sound- andor pressure-induced vertigo due to bone dehiscence of the superior semicircular canal Minor LB Solomon D Zinreich JS Zee DSArch Otolaryngol Head Neck Surg 1998 Mar124(3)249-58
Sixth Annual Intensive Update in Neurology 0915-162016
58
Sixth Annual Intensive Update in Neurology 0915-162016
59
SCHEMATIC DRAWING OF AN OTOLITHIC MACULA
Sixth Annual Intensive Update in Neurology 0915-162016
60
MACULAE OF THE UTRICLESACCULE
Sixth Annual Intensive Update in Neurology 0915-162016
61
HAIR CELLS
Sixth Annual Intensive Update in Neurology 0915-162016
62
SCANNING ELECTRON MICROGRAPH OF OTOCONIA
Sixth Annual Intensive Update in Neurology 0915-162016
63
Ocular VEMP
bull Response to loud clicks (80-110 dB) or forehead tap (Halmagyi and Curthoys 2003)
bull Contralateral oculomotor nucleus (CN III)
bull Inferior oblique muscle contraction (8 msec) detected by 5sec event triggered averaging
bull Noninverting electrode inferior to eye
bull Inverting below non-inverting electrode
bull Ground to forehead
bull Normal subjects have very low response
Sixth Annual Intensive Update in Neurology 0915-162016
64
Clinical applications
bull The o-VEMP is a sensitive test of otolithutricular
function
bull Complements vestibular test procedures that assess
semicircular canals
bull Abnormally robust responses are associated with third
window abnormalities such as semicircular canal
dehiscence
bull Serves as an excellent screening test for semicircular
canal dehiscence
Sixth Annual Intensive Update in Neurology 0915-162016
65
Question 5
bull Superior semicircular canal dehiscence
1) Causes increased ability to hear vibratory sounds
2) Improves hearing
3) Causes meningitis
4) Always needs surgical repair
Sixth Annual Intensive Update in Neurology 0915-162016
66
Question 5
bull Superior semicircular canal dehiscence
1) Causes increased ability to hear vibratory sounds
2) Improves hearing
3) Causes meningitis
4) Always needs surgical repair
Answer 1 References at end of lecture
Sixth Annual Intensive Update in Neurology 0915-162016
67
Enlarged vestibular aqueduct
bull Axial temporal bone CT showing right temporal bone with enlarged vestibular aqueduct (arrow)
bull Vestibular symptoms accompany progressive sensorineural hearing loss in 4-50 of patients in selected series
Sixth Annual Intensive Update in Neurology 0915-162016
68
Migraine
bull Family or personal history of migraine
bull 25 of migraine patients experience spontaneous vertigo (Baloh 1997)
bull Neuhauser criteria(2001) have been used to construct structured diagnostic interviews (Furman 2003)
bull Not always associated with headache
bull Motion sensitivity
bull Medication responsive
Sixth Annual Intensive Update in Neurology 0915-162016
69
ICHD-III beta Criteria for Vestibular Migraine
bull A At least five attacks fulfilling criteria C and D
bull B A current or past history of 11 migraine without aura or 12 migraine with aura
bull C Vestibular symptoms of moderate or severe intensity lasting between 5 minutes and 72 hours
bull D At least 50 associated with one of the following migrainous features
ndash 1 Headaches
ndash 2 Photophobia and phonophobia
ndash 3 Visual Aura
bull E Not better accounted for by another vestibular disorder
Sixth Annual Intensive Update in Neurology 0915-162016
70
Incidence of vertigo in MS
bull True vertigo (the perception of environmental
movement typically rotational) occurs in about
20 of MS patients and is the presenting
manifestation in 5
bull Up to 78 of MS patients report abnormalities
in balance at some point in their illness
Frohman et al Multiple Sclerosis 2003 9250
Sixth Annual Intensive Update in Neurology 0915-162016
71
Vertigo in MS due to demyelination
bull The most common neuroanatomic locations are the
root entry zone of CN VIII (giving absent calorics but
normal hearing and fixation suppression) and the
medial vestibular nucleus
bull Usually treated with steroids and vestibular
suppressants
Brandt 1993 Gass 1998 Thomke 1999
Sixth Annual Intensive Update in Neurology 0915-162016
72
Vertigo in MS
Frohmanrsquos large series (1153) reported that 52 of MS patients with acute vertigo had benign paroxysmal positional vertigo while 32 had new brainstem lesions
To avoid misdiagnoses and inappropriate treatment interventions alternative etiologies should be considered in all acute vertigo episodes in MS
E M Frohman Neurology 2000 and Multiple Sclerosis 2003
Sixth Annual Intensive Update in Neurology 0915-162016
73
Vestibular compensation
bull Acute unilateral or bilateral vestibular loss will prompt compensation thru central cervico-ocular visual and proprioceptive pathways
bull Unilateral loss may require several weeks for compensation to occur and activity especially head movements hastens compensation
bull Vestibular suppressant medication prolongs compensation
Sixth Annual Intensive Update in Neurology 0915-162016
74
Vestibular Rehabilitation
bull Performed by certified physical therapists many sites (Epic order vestibular PT) or check Vestibular Disorders of America website for providers
bull Assessment and treatment of benign positional vertigo
bull Directed rehab programs for central andor peripheral vestibular compensationndash Unilateral vestibulopathies
ndash Bilateral vestibular hypofunction
ndash Mutifactorial balance deficits and fall risk assessment
Sixth Annual Intensive Update in Neurology 0915-162016
75
Summary of whatrsquos new in peripheral vestibular disorders
bull Lateral semicircular canal benign paroxysmal positional vertigo presents with horizontal positioning nystagmus
bull Oral steroids appear to improve outcome in acute vestibular neuritis
bull Intratympanic steroids offer promise in Menierersquos syndrome
bull Superior semicircular canal dehiscence causes pressure and noise induced dizziness VEMP offers diagnostic information
Sixth Annual Intensive Update in Neurology 0915-162016
76
References
BPPVndash White J Benign paroxysmal positional vertigo Cleveland Clinic Journal of
Medicine 71(9)722-728 2004
ndash White J Coale K Catalano P Oas J Diagnosis and management of lateral semicircular canal benign positional vertigo Otolaryngology-Head and Neck Surgery 133 278-284 2005
ndash White J Savvides P Cherian N Oas J Canalith repositioning for benign paroxysmal positional vertigo Otology and Neurotology 26 704-710 2005
ndash White J Oas J Diagnosis and management of lateral semicircular canal conversions during particle repositioning therapy Laryngoscope 115(10) 1895-1897 2005
ndash Acute Vestibular Syndrome Hotson JR Baloh RW N Engl J Med 1998 Sep 3339(10)680-5
ndash Methylprednisolone valacyclovir or the combination for vestibular neuritis Strupp M Zingler VC Arbusow V Niklas D Maag KP Dieterich M Bense S Theil D Jahn K Brandt T N Engl J Med 2004 Jul 22351(4)354-61
ndash Intratympanic steroids for Meacuteniegraveres disease or syndrome Phillips JS Westerberg BCochrane Database Syst Rev 2011 Jul 6(7)CD008514 Review
ndash Sound- andor pressure-induced vertigo due to bone dehiscence of the superior semicircular canal Minor LB Solomon D Zinreich JS Zee DSArch Otolaryngol Head Neck Surg 1998 Mar124(3)249-58
Sixth Annual Intensive Update in Neurology 0915-162016
59
SCHEMATIC DRAWING OF AN OTOLITHIC MACULA
Sixth Annual Intensive Update in Neurology 0915-162016
60
MACULAE OF THE UTRICLESACCULE
Sixth Annual Intensive Update in Neurology 0915-162016
61
HAIR CELLS
Sixth Annual Intensive Update in Neurology 0915-162016
62
SCANNING ELECTRON MICROGRAPH OF OTOCONIA
Sixth Annual Intensive Update in Neurology 0915-162016
63
Ocular VEMP
bull Response to loud clicks (80-110 dB) or forehead tap (Halmagyi and Curthoys 2003)
bull Contralateral oculomotor nucleus (CN III)
bull Inferior oblique muscle contraction (8 msec) detected by 5sec event triggered averaging
bull Noninverting electrode inferior to eye
bull Inverting below non-inverting electrode
bull Ground to forehead
bull Normal subjects have very low response
Sixth Annual Intensive Update in Neurology 0915-162016
64
Clinical applications
bull The o-VEMP is a sensitive test of otolithutricular
function
bull Complements vestibular test procedures that assess
semicircular canals
bull Abnormally robust responses are associated with third
window abnormalities such as semicircular canal
dehiscence
bull Serves as an excellent screening test for semicircular
canal dehiscence
Sixth Annual Intensive Update in Neurology 0915-162016
65
Question 5
bull Superior semicircular canal dehiscence
1) Causes increased ability to hear vibratory sounds
2) Improves hearing
3) Causes meningitis
4) Always needs surgical repair
Sixth Annual Intensive Update in Neurology 0915-162016
66
Question 5
bull Superior semicircular canal dehiscence
1) Causes increased ability to hear vibratory sounds
2) Improves hearing
3) Causes meningitis
4) Always needs surgical repair
Answer 1 References at end of lecture
Sixth Annual Intensive Update in Neurology 0915-162016
67
Enlarged vestibular aqueduct
bull Axial temporal bone CT showing right temporal bone with enlarged vestibular aqueduct (arrow)
bull Vestibular symptoms accompany progressive sensorineural hearing loss in 4-50 of patients in selected series
Sixth Annual Intensive Update in Neurology 0915-162016
68
Migraine
bull Family or personal history of migraine
bull 25 of migraine patients experience spontaneous vertigo (Baloh 1997)
bull Neuhauser criteria(2001) have been used to construct structured diagnostic interviews (Furman 2003)
bull Not always associated with headache
bull Motion sensitivity
bull Medication responsive
Sixth Annual Intensive Update in Neurology 0915-162016
69
ICHD-III beta Criteria for Vestibular Migraine
bull A At least five attacks fulfilling criteria C and D
bull B A current or past history of 11 migraine without aura or 12 migraine with aura
bull C Vestibular symptoms of moderate or severe intensity lasting between 5 minutes and 72 hours
bull D At least 50 associated with one of the following migrainous features
ndash 1 Headaches
ndash 2 Photophobia and phonophobia
ndash 3 Visual Aura
bull E Not better accounted for by another vestibular disorder
Sixth Annual Intensive Update in Neurology 0915-162016
70
Incidence of vertigo in MS
bull True vertigo (the perception of environmental
movement typically rotational) occurs in about
20 of MS patients and is the presenting
manifestation in 5
bull Up to 78 of MS patients report abnormalities
in balance at some point in their illness
Frohman et al Multiple Sclerosis 2003 9250
Sixth Annual Intensive Update in Neurology 0915-162016
71
Vertigo in MS due to demyelination
bull The most common neuroanatomic locations are the
root entry zone of CN VIII (giving absent calorics but
normal hearing and fixation suppression) and the
medial vestibular nucleus
bull Usually treated with steroids and vestibular
suppressants
Brandt 1993 Gass 1998 Thomke 1999
Sixth Annual Intensive Update in Neurology 0915-162016
72
Vertigo in MS
Frohmanrsquos large series (1153) reported that 52 of MS patients with acute vertigo had benign paroxysmal positional vertigo while 32 had new brainstem lesions
To avoid misdiagnoses and inappropriate treatment interventions alternative etiologies should be considered in all acute vertigo episodes in MS
E M Frohman Neurology 2000 and Multiple Sclerosis 2003
Sixth Annual Intensive Update in Neurology 0915-162016
73
Vestibular compensation
bull Acute unilateral or bilateral vestibular loss will prompt compensation thru central cervico-ocular visual and proprioceptive pathways
bull Unilateral loss may require several weeks for compensation to occur and activity especially head movements hastens compensation
bull Vestibular suppressant medication prolongs compensation
Sixth Annual Intensive Update in Neurology 0915-162016
74
Vestibular Rehabilitation
bull Performed by certified physical therapists many sites (Epic order vestibular PT) or check Vestibular Disorders of America website for providers
bull Assessment and treatment of benign positional vertigo
bull Directed rehab programs for central andor peripheral vestibular compensationndash Unilateral vestibulopathies
ndash Bilateral vestibular hypofunction
ndash Mutifactorial balance deficits and fall risk assessment
Sixth Annual Intensive Update in Neurology 0915-162016
75
Summary of whatrsquos new in peripheral vestibular disorders
bull Lateral semicircular canal benign paroxysmal positional vertigo presents with horizontal positioning nystagmus
bull Oral steroids appear to improve outcome in acute vestibular neuritis
bull Intratympanic steroids offer promise in Menierersquos syndrome
bull Superior semicircular canal dehiscence causes pressure and noise induced dizziness VEMP offers diagnostic information
Sixth Annual Intensive Update in Neurology 0915-162016
76
References
BPPVndash White J Benign paroxysmal positional vertigo Cleveland Clinic Journal of
Medicine 71(9)722-728 2004
ndash White J Coale K Catalano P Oas J Diagnosis and management of lateral semicircular canal benign positional vertigo Otolaryngology-Head and Neck Surgery 133 278-284 2005
ndash White J Savvides P Cherian N Oas J Canalith repositioning for benign paroxysmal positional vertigo Otology and Neurotology 26 704-710 2005
ndash White J Oas J Diagnosis and management of lateral semicircular canal conversions during particle repositioning therapy Laryngoscope 115(10) 1895-1897 2005
ndash Acute Vestibular Syndrome Hotson JR Baloh RW N Engl J Med 1998 Sep 3339(10)680-5
ndash Methylprednisolone valacyclovir or the combination for vestibular neuritis Strupp M Zingler VC Arbusow V Niklas D Maag KP Dieterich M Bense S Theil D Jahn K Brandt T N Engl J Med 2004 Jul 22351(4)354-61
ndash Intratympanic steroids for Meacuteniegraveres disease or syndrome Phillips JS Westerberg BCochrane Database Syst Rev 2011 Jul 6(7)CD008514 Review
ndash Sound- andor pressure-induced vertigo due to bone dehiscence of the superior semicircular canal Minor LB Solomon D Zinreich JS Zee DSArch Otolaryngol Head Neck Surg 1998 Mar124(3)249-58
Sixth Annual Intensive Update in Neurology 0915-162016
60
MACULAE OF THE UTRICLESACCULE
Sixth Annual Intensive Update in Neurology 0915-162016
61
HAIR CELLS
Sixth Annual Intensive Update in Neurology 0915-162016
62
SCANNING ELECTRON MICROGRAPH OF OTOCONIA
Sixth Annual Intensive Update in Neurology 0915-162016
63
Ocular VEMP
bull Response to loud clicks (80-110 dB) or forehead tap (Halmagyi and Curthoys 2003)
bull Contralateral oculomotor nucleus (CN III)
bull Inferior oblique muscle contraction (8 msec) detected by 5sec event triggered averaging
bull Noninverting electrode inferior to eye
bull Inverting below non-inverting electrode
bull Ground to forehead
bull Normal subjects have very low response
Sixth Annual Intensive Update in Neurology 0915-162016
64
Clinical applications
bull The o-VEMP is a sensitive test of otolithutricular
function
bull Complements vestibular test procedures that assess
semicircular canals
bull Abnormally robust responses are associated with third
window abnormalities such as semicircular canal
dehiscence
bull Serves as an excellent screening test for semicircular
canal dehiscence
Sixth Annual Intensive Update in Neurology 0915-162016
65
Question 5
bull Superior semicircular canal dehiscence
1) Causes increased ability to hear vibratory sounds
2) Improves hearing
3) Causes meningitis
4) Always needs surgical repair
Sixth Annual Intensive Update in Neurology 0915-162016
66
Question 5
bull Superior semicircular canal dehiscence
1) Causes increased ability to hear vibratory sounds
2) Improves hearing
3) Causes meningitis
4) Always needs surgical repair
Answer 1 References at end of lecture
Sixth Annual Intensive Update in Neurology 0915-162016
67
Enlarged vestibular aqueduct
bull Axial temporal bone CT showing right temporal bone with enlarged vestibular aqueduct (arrow)
bull Vestibular symptoms accompany progressive sensorineural hearing loss in 4-50 of patients in selected series
Sixth Annual Intensive Update in Neurology 0915-162016
68
Migraine
bull Family or personal history of migraine
bull 25 of migraine patients experience spontaneous vertigo (Baloh 1997)
bull Neuhauser criteria(2001) have been used to construct structured diagnostic interviews (Furman 2003)
bull Not always associated with headache
bull Motion sensitivity
bull Medication responsive
Sixth Annual Intensive Update in Neurology 0915-162016
69
ICHD-III beta Criteria for Vestibular Migraine
bull A At least five attacks fulfilling criteria C and D
bull B A current or past history of 11 migraine without aura or 12 migraine with aura
bull C Vestibular symptoms of moderate or severe intensity lasting between 5 minutes and 72 hours
bull D At least 50 associated with one of the following migrainous features
ndash 1 Headaches
ndash 2 Photophobia and phonophobia
ndash 3 Visual Aura
bull E Not better accounted for by another vestibular disorder
Sixth Annual Intensive Update in Neurology 0915-162016
70
Incidence of vertigo in MS
bull True vertigo (the perception of environmental
movement typically rotational) occurs in about
20 of MS patients and is the presenting
manifestation in 5
bull Up to 78 of MS patients report abnormalities
in balance at some point in their illness
Frohman et al Multiple Sclerosis 2003 9250
Sixth Annual Intensive Update in Neurology 0915-162016
71
Vertigo in MS due to demyelination
bull The most common neuroanatomic locations are the
root entry zone of CN VIII (giving absent calorics but
normal hearing and fixation suppression) and the
medial vestibular nucleus
bull Usually treated with steroids and vestibular
suppressants
Brandt 1993 Gass 1998 Thomke 1999
Sixth Annual Intensive Update in Neurology 0915-162016
72
Vertigo in MS
Frohmanrsquos large series (1153) reported that 52 of MS patients with acute vertigo had benign paroxysmal positional vertigo while 32 had new brainstem lesions
To avoid misdiagnoses and inappropriate treatment interventions alternative etiologies should be considered in all acute vertigo episodes in MS
E M Frohman Neurology 2000 and Multiple Sclerosis 2003
Sixth Annual Intensive Update in Neurology 0915-162016
73
Vestibular compensation
bull Acute unilateral or bilateral vestibular loss will prompt compensation thru central cervico-ocular visual and proprioceptive pathways
bull Unilateral loss may require several weeks for compensation to occur and activity especially head movements hastens compensation
bull Vestibular suppressant medication prolongs compensation
Sixth Annual Intensive Update in Neurology 0915-162016
74
Vestibular Rehabilitation
bull Performed by certified physical therapists many sites (Epic order vestibular PT) or check Vestibular Disorders of America website for providers
bull Assessment and treatment of benign positional vertigo
bull Directed rehab programs for central andor peripheral vestibular compensationndash Unilateral vestibulopathies
ndash Bilateral vestibular hypofunction
ndash Mutifactorial balance deficits and fall risk assessment
Sixth Annual Intensive Update in Neurology 0915-162016
75
Summary of whatrsquos new in peripheral vestibular disorders
bull Lateral semicircular canal benign paroxysmal positional vertigo presents with horizontal positioning nystagmus
bull Oral steroids appear to improve outcome in acute vestibular neuritis
bull Intratympanic steroids offer promise in Menierersquos syndrome
bull Superior semicircular canal dehiscence causes pressure and noise induced dizziness VEMP offers diagnostic information
Sixth Annual Intensive Update in Neurology 0915-162016
76
References
BPPVndash White J Benign paroxysmal positional vertigo Cleveland Clinic Journal of
Medicine 71(9)722-728 2004
ndash White J Coale K Catalano P Oas J Diagnosis and management of lateral semicircular canal benign positional vertigo Otolaryngology-Head and Neck Surgery 133 278-284 2005
ndash White J Savvides P Cherian N Oas J Canalith repositioning for benign paroxysmal positional vertigo Otology and Neurotology 26 704-710 2005
ndash White J Oas J Diagnosis and management of lateral semicircular canal conversions during particle repositioning therapy Laryngoscope 115(10) 1895-1897 2005
ndash Acute Vestibular Syndrome Hotson JR Baloh RW N Engl J Med 1998 Sep 3339(10)680-5
ndash Methylprednisolone valacyclovir or the combination for vestibular neuritis Strupp M Zingler VC Arbusow V Niklas D Maag KP Dieterich M Bense S Theil D Jahn K Brandt T N Engl J Med 2004 Jul 22351(4)354-61
ndash Intratympanic steroids for Meacuteniegraveres disease or syndrome Phillips JS Westerberg BCochrane Database Syst Rev 2011 Jul 6(7)CD008514 Review
ndash Sound- andor pressure-induced vertigo due to bone dehiscence of the superior semicircular canal Minor LB Solomon D Zinreich JS Zee DSArch Otolaryngol Head Neck Surg 1998 Mar124(3)249-58
Sixth Annual Intensive Update in Neurology 0915-162016
61
HAIR CELLS
Sixth Annual Intensive Update in Neurology 0915-162016
62
SCANNING ELECTRON MICROGRAPH OF OTOCONIA
Sixth Annual Intensive Update in Neurology 0915-162016
63
Ocular VEMP
bull Response to loud clicks (80-110 dB) or forehead tap (Halmagyi and Curthoys 2003)
bull Contralateral oculomotor nucleus (CN III)
bull Inferior oblique muscle contraction (8 msec) detected by 5sec event triggered averaging
bull Noninverting electrode inferior to eye
bull Inverting below non-inverting electrode
bull Ground to forehead
bull Normal subjects have very low response
Sixth Annual Intensive Update in Neurology 0915-162016
64
Clinical applications
bull The o-VEMP is a sensitive test of otolithutricular
function
bull Complements vestibular test procedures that assess
semicircular canals
bull Abnormally robust responses are associated with third
window abnormalities such as semicircular canal
dehiscence
bull Serves as an excellent screening test for semicircular
canal dehiscence
Sixth Annual Intensive Update in Neurology 0915-162016
65
Question 5
bull Superior semicircular canal dehiscence
1) Causes increased ability to hear vibratory sounds
2) Improves hearing
3) Causes meningitis
4) Always needs surgical repair
Sixth Annual Intensive Update in Neurology 0915-162016
66
Question 5
bull Superior semicircular canal dehiscence
1) Causes increased ability to hear vibratory sounds
2) Improves hearing
3) Causes meningitis
4) Always needs surgical repair
Answer 1 References at end of lecture
Sixth Annual Intensive Update in Neurology 0915-162016
67
Enlarged vestibular aqueduct
bull Axial temporal bone CT showing right temporal bone with enlarged vestibular aqueduct (arrow)
bull Vestibular symptoms accompany progressive sensorineural hearing loss in 4-50 of patients in selected series
Sixth Annual Intensive Update in Neurology 0915-162016
68
Migraine
bull Family or personal history of migraine
bull 25 of migraine patients experience spontaneous vertigo (Baloh 1997)
bull Neuhauser criteria(2001) have been used to construct structured diagnostic interviews (Furman 2003)
bull Not always associated with headache
bull Motion sensitivity
bull Medication responsive
Sixth Annual Intensive Update in Neurology 0915-162016
69
ICHD-III beta Criteria for Vestibular Migraine
bull A At least five attacks fulfilling criteria C and D
bull B A current or past history of 11 migraine without aura or 12 migraine with aura
bull C Vestibular symptoms of moderate or severe intensity lasting between 5 minutes and 72 hours
bull D At least 50 associated with one of the following migrainous features
ndash 1 Headaches
ndash 2 Photophobia and phonophobia
ndash 3 Visual Aura
bull E Not better accounted for by another vestibular disorder
Sixth Annual Intensive Update in Neurology 0915-162016
70
Incidence of vertigo in MS
bull True vertigo (the perception of environmental
movement typically rotational) occurs in about
20 of MS patients and is the presenting
manifestation in 5
bull Up to 78 of MS patients report abnormalities
in balance at some point in their illness
Frohman et al Multiple Sclerosis 2003 9250
Sixth Annual Intensive Update in Neurology 0915-162016
71
Vertigo in MS due to demyelination
bull The most common neuroanatomic locations are the
root entry zone of CN VIII (giving absent calorics but
normal hearing and fixation suppression) and the
medial vestibular nucleus
bull Usually treated with steroids and vestibular
suppressants
Brandt 1993 Gass 1998 Thomke 1999
Sixth Annual Intensive Update in Neurology 0915-162016
72
Vertigo in MS
Frohmanrsquos large series (1153) reported that 52 of MS patients with acute vertigo had benign paroxysmal positional vertigo while 32 had new brainstem lesions
To avoid misdiagnoses and inappropriate treatment interventions alternative etiologies should be considered in all acute vertigo episodes in MS
E M Frohman Neurology 2000 and Multiple Sclerosis 2003
Sixth Annual Intensive Update in Neurology 0915-162016
73
Vestibular compensation
bull Acute unilateral or bilateral vestibular loss will prompt compensation thru central cervico-ocular visual and proprioceptive pathways
bull Unilateral loss may require several weeks for compensation to occur and activity especially head movements hastens compensation
bull Vestibular suppressant medication prolongs compensation
Sixth Annual Intensive Update in Neurology 0915-162016
74
Vestibular Rehabilitation
bull Performed by certified physical therapists many sites (Epic order vestibular PT) or check Vestibular Disorders of America website for providers
bull Assessment and treatment of benign positional vertigo
bull Directed rehab programs for central andor peripheral vestibular compensationndash Unilateral vestibulopathies
ndash Bilateral vestibular hypofunction
ndash Mutifactorial balance deficits and fall risk assessment
Sixth Annual Intensive Update in Neurology 0915-162016
75
Summary of whatrsquos new in peripheral vestibular disorders
bull Lateral semicircular canal benign paroxysmal positional vertigo presents with horizontal positioning nystagmus
bull Oral steroids appear to improve outcome in acute vestibular neuritis
bull Intratympanic steroids offer promise in Menierersquos syndrome
bull Superior semicircular canal dehiscence causes pressure and noise induced dizziness VEMP offers diagnostic information
Sixth Annual Intensive Update in Neurology 0915-162016
76
References
BPPVndash White J Benign paroxysmal positional vertigo Cleveland Clinic Journal of
Medicine 71(9)722-728 2004
ndash White J Coale K Catalano P Oas J Diagnosis and management of lateral semicircular canal benign positional vertigo Otolaryngology-Head and Neck Surgery 133 278-284 2005
ndash White J Savvides P Cherian N Oas J Canalith repositioning for benign paroxysmal positional vertigo Otology and Neurotology 26 704-710 2005
ndash White J Oas J Diagnosis and management of lateral semicircular canal conversions during particle repositioning therapy Laryngoscope 115(10) 1895-1897 2005
ndash Acute Vestibular Syndrome Hotson JR Baloh RW N Engl J Med 1998 Sep 3339(10)680-5
ndash Methylprednisolone valacyclovir or the combination for vestibular neuritis Strupp M Zingler VC Arbusow V Niklas D Maag KP Dieterich M Bense S Theil D Jahn K Brandt T N Engl J Med 2004 Jul 22351(4)354-61
ndash Intratympanic steroids for Meacuteniegraveres disease or syndrome Phillips JS Westerberg BCochrane Database Syst Rev 2011 Jul 6(7)CD008514 Review
ndash Sound- andor pressure-induced vertigo due to bone dehiscence of the superior semicircular canal Minor LB Solomon D Zinreich JS Zee DSArch Otolaryngol Head Neck Surg 1998 Mar124(3)249-58
Sixth Annual Intensive Update in Neurology 0915-162016
62
SCANNING ELECTRON MICROGRAPH OF OTOCONIA
Sixth Annual Intensive Update in Neurology 0915-162016
63
Ocular VEMP
bull Response to loud clicks (80-110 dB) or forehead tap (Halmagyi and Curthoys 2003)
bull Contralateral oculomotor nucleus (CN III)
bull Inferior oblique muscle contraction (8 msec) detected by 5sec event triggered averaging
bull Noninverting electrode inferior to eye
bull Inverting below non-inverting electrode
bull Ground to forehead
bull Normal subjects have very low response
Sixth Annual Intensive Update in Neurology 0915-162016
64
Clinical applications
bull The o-VEMP is a sensitive test of otolithutricular
function
bull Complements vestibular test procedures that assess
semicircular canals
bull Abnormally robust responses are associated with third
window abnormalities such as semicircular canal
dehiscence
bull Serves as an excellent screening test for semicircular
canal dehiscence
Sixth Annual Intensive Update in Neurology 0915-162016
65
Question 5
bull Superior semicircular canal dehiscence
1) Causes increased ability to hear vibratory sounds
2) Improves hearing
3) Causes meningitis
4) Always needs surgical repair
Sixth Annual Intensive Update in Neurology 0915-162016
66
Question 5
bull Superior semicircular canal dehiscence
1) Causes increased ability to hear vibratory sounds
2) Improves hearing
3) Causes meningitis
4) Always needs surgical repair
Answer 1 References at end of lecture
Sixth Annual Intensive Update in Neurology 0915-162016
67
Enlarged vestibular aqueduct
bull Axial temporal bone CT showing right temporal bone with enlarged vestibular aqueduct (arrow)
bull Vestibular symptoms accompany progressive sensorineural hearing loss in 4-50 of patients in selected series
Sixth Annual Intensive Update in Neurology 0915-162016
68
Migraine
bull Family or personal history of migraine
bull 25 of migraine patients experience spontaneous vertigo (Baloh 1997)
bull Neuhauser criteria(2001) have been used to construct structured diagnostic interviews (Furman 2003)
bull Not always associated with headache
bull Motion sensitivity
bull Medication responsive
Sixth Annual Intensive Update in Neurology 0915-162016
69
ICHD-III beta Criteria for Vestibular Migraine
bull A At least five attacks fulfilling criteria C and D
bull B A current or past history of 11 migraine without aura or 12 migraine with aura
bull C Vestibular symptoms of moderate or severe intensity lasting between 5 minutes and 72 hours
bull D At least 50 associated with one of the following migrainous features
ndash 1 Headaches
ndash 2 Photophobia and phonophobia
ndash 3 Visual Aura
bull E Not better accounted for by another vestibular disorder
Sixth Annual Intensive Update in Neurology 0915-162016
70
Incidence of vertigo in MS
bull True vertigo (the perception of environmental
movement typically rotational) occurs in about
20 of MS patients and is the presenting
manifestation in 5
bull Up to 78 of MS patients report abnormalities
in balance at some point in their illness
Frohman et al Multiple Sclerosis 2003 9250
Sixth Annual Intensive Update in Neurology 0915-162016
71
Vertigo in MS due to demyelination
bull The most common neuroanatomic locations are the
root entry zone of CN VIII (giving absent calorics but
normal hearing and fixation suppression) and the
medial vestibular nucleus
bull Usually treated with steroids and vestibular
suppressants
Brandt 1993 Gass 1998 Thomke 1999
Sixth Annual Intensive Update in Neurology 0915-162016
72
Vertigo in MS
Frohmanrsquos large series (1153) reported that 52 of MS patients with acute vertigo had benign paroxysmal positional vertigo while 32 had new brainstem lesions
To avoid misdiagnoses and inappropriate treatment interventions alternative etiologies should be considered in all acute vertigo episodes in MS
E M Frohman Neurology 2000 and Multiple Sclerosis 2003
Sixth Annual Intensive Update in Neurology 0915-162016
73
Vestibular compensation
bull Acute unilateral or bilateral vestibular loss will prompt compensation thru central cervico-ocular visual and proprioceptive pathways
bull Unilateral loss may require several weeks for compensation to occur and activity especially head movements hastens compensation
bull Vestibular suppressant medication prolongs compensation
Sixth Annual Intensive Update in Neurology 0915-162016
74
Vestibular Rehabilitation
bull Performed by certified physical therapists many sites (Epic order vestibular PT) or check Vestibular Disorders of America website for providers
bull Assessment and treatment of benign positional vertigo
bull Directed rehab programs for central andor peripheral vestibular compensationndash Unilateral vestibulopathies
ndash Bilateral vestibular hypofunction
ndash Mutifactorial balance deficits and fall risk assessment
Sixth Annual Intensive Update in Neurology 0915-162016
75
Summary of whatrsquos new in peripheral vestibular disorders
bull Lateral semicircular canal benign paroxysmal positional vertigo presents with horizontal positioning nystagmus
bull Oral steroids appear to improve outcome in acute vestibular neuritis
bull Intratympanic steroids offer promise in Menierersquos syndrome
bull Superior semicircular canal dehiscence causes pressure and noise induced dizziness VEMP offers diagnostic information
Sixth Annual Intensive Update in Neurology 0915-162016
76
References
BPPVndash White J Benign paroxysmal positional vertigo Cleveland Clinic Journal of
Medicine 71(9)722-728 2004
ndash White J Coale K Catalano P Oas J Diagnosis and management of lateral semicircular canal benign positional vertigo Otolaryngology-Head and Neck Surgery 133 278-284 2005
ndash White J Savvides P Cherian N Oas J Canalith repositioning for benign paroxysmal positional vertigo Otology and Neurotology 26 704-710 2005
ndash White J Oas J Diagnosis and management of lateral semicircular canal conversions during particle repositioning therapy Laryngoscope 115(10) 1895-1897 2005
ndash Acute Vestibular Syndrome Hotson JR Baloh RW N Engl J Med 1998 Sep 3339(10)680-5
ndash Methylprednisolone valacyclovir or the combination for vestibular neuritis Strupp M Zingler VC Arbusow V Niklas D Maag KP Dieterich M Bense S Theil D Jahn K Brandt T N Engl J Med 2004 Jul 22351(4)354-61
ndash Intratympanic steroids for Meacuteniegraveres disease or syndrome Phillips JS Westerberg BCochrane Database Syst Rev 2011 Jul 6(7)CD008514 Review
ndash Sound- andor pressure-induced vertigo due to bone dehiscence of the superior semicircular canal Minor LB Solomon D Zinreich JS Zee DSArch Otolaryngol Head Neck Surg 1998 Mar124(3)249-58
Sixth Annual Intensive Update in Neurology 0915-162016
63
Ocular VEMP
bull Response to loud clicks (80-110 dB) or forehead tap (Halmagyi and Curthoys 2003)
bull Contralateral oculomotor nucleus (CN III)
bull Inferior oblique muscle contraction (8 msec) detected by 5sec event triggered averaging
bull Noninverting electrode inferior to eye
bull Inverting below non-inverting electrode
bull Ground to forehead
bull Normal subjects have very low response
Sixth Annual Intensive Update in Neurology 0915-162016
64
Clinical applications
bull The o-VEMP is a sensitive test of otolithutricular
function
bull Complements vestibular test procedures that assess
semicircular canals
bull Abnormally robust responses are associated with third
window abnormalities such as semicircular canal
dehiscence
bull Serves as an excellent screening test for semicircular
canal dehiscence
Sixth Annual Intensive Update in Neurology 0915-162016
65
Question 5
bull Superior semicircular canal dehiscence
1) Causes increased ability to hear vibratory sounds
2) Improves hearing
3) Causes meningitis
4) Always needs surgical repair
Sixth Annual Intensive Update in Neurology 0915-162016
66
Question 5
bull Superior semicircular canal dehiscence
1) Causes increased ability to hear vibratory sounds
2) Improves hearing
3) Causes meningitis
4) Always needs surgical repair
Answer 1 References at end of lecture
Sixth Annual Intensive Update in Neurology 0915-162016
67
Enlarged vestibular aqueduct
bull Axial temporal bone CT showing right temporal bone with enlarged vestibular aqueduct (arrow)
bull Vestibular symptoms accompany progressive sensorineural hearing loss in 4-50 of patients in selected series
Sixth Annual Intensive Update in Neurology 0915-162016
68
Migraine
bull Family or personal history of migraine
bull 25 of migraine patients experience spontaneous vertigo (Baloh 1997)
bull Neuhauser criteria(2001) have been used to construct structured diagnostic interviews (Furman 2003)
bull Not always associated with headache
bull Motion sensitivity
bull Medication responsive
Sixth Annual Intensive Update in Neurology 0915-162016
69
ICHD-III beta Criteria for Vestibular Migraine
bull A At least five attacks fulfilling criteria C and D
bull B A current or past history of 11 migraine without aura or 12 migraine with aura
bull C Vestibular symptoms of moderate or severe intensity lasting between 5 minutes and 72 hours
bull D At least 50 associated with one of the following migrainous features
ndash 1 Headaches
ndash 2 Photophobia and phonophobia
ndash 3 Visual Aura
bull E Not better accounted for by another vestibular disorder
Sixth Annual Intensive Update in Neurology 0915-162016
70
Incidence of vertigo in MS
bull True vertigo (the perception of environmental
movement typically rotational) occurs in about
20 of MS patients and is the presenting
manifestation in 5
bull Up to 78 of MS patients report abnormalities
in balance at some point in their illness
Frohman et al Multiple Sclerosis 2003 9250
Sixth Annual Intensive Update in Neurology 0915-162016
71
Vertigo in MS due to demyelination
bull The most common neuroanatomic locations are the
root entry zone of CN VIII (giving absent calorics but
normal hearing and fixation suppression) and the
medial vestibular nucleus
bull Usually treated with steroids and vestibular
suppressants
Brandt 1993 Gass 1998 Thomke 1999
Sixth Annual Intensive Update in Neurology 0915-162016
72
Vertigo in MS
Frohmanrsquos large series (1153) reported that 52 of MS patients with acute vertigo had benign paroxysmal positional vertigo while 32 had new brainstem lesions
To avoid misdiagnoses and inappropriate treatment interventions alternative etiologies should be considered in all acute vertigo episodes in MS
E M Frohman Neurology 2000 and Multiple Sclerosis 2003
Sixth Annual Intensive Update in Neurology 0915-162016
73
Vestibular compensation
bull Acute unilateral or bilateral vestibular loss will prompt compensation thru central cervico-ocular visual and proprioceptive pathways
bull Unilateral loss may require several weeks for compensation to occur and activity especially head movements hastens compensation
bull Vestibular suppressant medication prolongs compensation
Sixth Annual Intensive Update in Neurology 0915-162016
74
Vestibular Rehabilitation
bull Performed by certified physical therapists many sites (Epic order vestibular PT) or check Vestibular Disorders of America website for providers
bull Assessment and treatment of benign positional vertigo
bull Directed rehab programs for central andor peripheral vestibular compensationndash Unilateral vestibulopathies
ndash Bilateral vestibular hypofunction
ndash Mutifactorial balance deficits and fall risk assessment
Sixth Annual Intensive Update in Neurology 0915-162016
75
Summary of whatrsquos new in peripheral vestibular disorders
bull Lateral semicircular canal benign paroxysmal positional vertigo presents with horizontal positioning nystagmus
bull Oral steroids appear to improve outcome in acute vestibular neuritis
bull Intratympanic steroids offer promise in Menierersquos syndrome
bull Superior semicircular canal dehiscence causes pressure and noise induced dizziness VEMP offers diagnostic information
Sixth Annual Intensive Update in Neurology 0915-162016
76
References
BPPVndash White J Benign paroxysmal positional vertigo Cleveland Clinic Journal of
Medicine 71(9)722-728 2004
ndash White J Coale K Catalano P Oas J Diagnosis and management of lateral semicircular canal benign positional vertigo Otolaryngology-Head and Neck Surgery 133 278-284 2005
ndash White J Savvides P Cherian N Oas J Canalith repositioning for benign paroxysmal positional vertigo Otology and Neurotology 26 704-710 2005
ndash White J Oas J Diagnosis and management of lateral semicircular canal conversions during particle repositioning therapy Laryngoscope 115(10) 1895-1897 2005
ndash Acute Vestibular Syndrome Hotson JR Baloh RW N Engl J Med 1998 Sep 3339(10)680-5
ndash Methylprednisolone valacyclovir or the combination for vestibular neuritis Strupp M Zingler VC Arbusow V Niklas D Maag KP Dieterich M Bense S Theil D Jahn K Brandt T N Engl J Med 2004 Jul 22351(4)354-61
ndash Intratympanic steroids for Meacuteniegraveres disease or syndrome Phillips JS Westerberg BCochrane Database Syst Rev 2011 Jul 6(7)CD008514 Review
ndash Sound- andor pressure-induced vertigo due to bone dehiscence of the superior semicircular canal Minor LB Solomon D Zinreich JS Zee DSArch Otolaryngol Head Neck Surg 1998 Mar124(3)249-58
Sixth Annual Intensive Update in Neurology 0915-162016
64
Clinical applications
bull The o-VEMP is a sensitive test of otolithutricular
function
bull Complements vestibular test procedures that assess
semicircular canals
bull Abnormally robust responses are associated with third
window abnormalities such as semicircular canal
dehiscence
bull Serves as an excellent screening test for semicircular
canal dehiscence
Sixth Annual Intensive Update in Neurology 0915-162016
65
Question 5
bull Superior semicircular canal dehiscence
1) Causes increased ability to hear vibratory sounds
2) Improves hearing
3) Causes meningitis
4) Always needs surgical repair
Sixth Annual Intensive Update in Neurology 0915-162016
66
Question 5
bull Superior semicircular canal dehiscence
1) Causes increased ability to hear vibratory sounds
2) Improves hearing
3) Causes meningitis
4) Always needs surgical repair
Answer 1 References at end of lecture
Sixth Annual Intensive Update in Neurology 0915-162016
67
Enlarged vestibular aqueduct
bull Axial temporal bone CT showing right temporal bone with enlarged vestibular aqueduct (arrow)
bull Vestibular symptoms accompany progressive sensorineural hearing loss in 4-50 of patients in selected series
Sixth Annual Intensive Update in Neurology 0915-162016
68
Migraine
bull Family or personal history of migraine
bull 25 of migraine patients experience spontaneous vertigo (Baloh 1997)
bull Neuhauser criteria(2001) have been used to construct structured diagnostic interviews (Furman 2003)
bull Not always associated with headache
bull Motion sensitivity
bull Medication responsive
Sixth Annual Intensive Update in Neurology 0915-162016
69
ICHD-III beta Criteria for Vestibular Migraine
bull A At least five attacks fulfilling criteria C and D
bull B A current or past history of 11 migraine without aura or 12 migraine with aura
bull C Vestibular symptoms of moderate or severe intensity lasting between 5 minutes and 72 hours
bull D At least 50 associated with one of the following migrainous features
ndash 1 Headaches
ndash 2 Photophobia and phonophobia
ndash 3 Visual Aura
bull E Not better accounted for by another vestibular disorder
Sixth Annual Intensive Update in Neurology 0915-162016
70
Incidence of vertigo in MS
bull True vertigo (the perception of environmental
movement typically rotational) occurs in about
20 of MS patients and is the presenting
manifestation in 5
bull Up to 78 of MS patients report abnormalities
in balance at some point in their illness
Frohman et al Multiple Sclerosis 2003 9250
Sixth Annual Intensive Update in Neurology 0915-162016
71
Vertigo in MS due to demyelination
bull The most common neuroanatomic locations are the
root entry zone of CN VIII (giving absent calorics but
normal hearing and fixation suppression) and the
medial vestibular nucleus
bull Usually treated with steroids and vestibular
suppressants
Brandt 1993 Gass 1998 Thomke 1999
Sixth Annual Intensive Update in Neurology 0915-162016
72
Vertigo in MS
Frohmanrsquos large series (1153) reported that 52 of MS patients with acute vertigo had benign paroxysmal positional vertigo while 32 had new brainstem lesions
To avoid misdiagnoses and inappropriate treatment interventions alternative etiologies should be considered in all acute vertigo episodes in MS
E M Frohman Neurology 2000 and Multiple Sclerosis 2003
Sixth Annual Intensive Update in Neurology 0915-162016
73
Vestibular compensation
bull Acute unilateral or bilateral vestibular loss will prompt compensation thru central cervico-ocular visual and proprioceptive pathways
bull Unilateral loss may require several weeks for compensation to occur and activity especially head movements hastens compensation
bull Vestibular suppressant medication prolongs compensation
Sixth Annual Intensive Update in Neurology 0915-162016
74
Vestibular Rehabilitation
bull Performed by certified physical therapists many sites (Epic order vestibular PT) or check Vestibular Disorders of America website for providers
bull Assessment and treatment of benign positional vertigo
bull Directed rehab programs for central andor peripheral vestibular compensationndash Unilateral vestibulopathies
ndash Bilateral vestibular hypofunction
ndash Mutifactorial balance deficits and fall risk assessment
Sixth Annual Intensive Update in Neurology 0915-162016
75
Summary of whatrsquos new in peripheral vestibular disorders
bull Lateral semicircular canal benign paroxysmal positional vertigo presents with horizontal positioning nystagmus
bull Oral steroids appear to improve outcome in acute vestibular neuritis
bull Intratympanic steroids offer promise in Menierersquos syndrome
bull Superior semicircular canal dehiscence causes pressure and noise induced dizziness VEMP offers diagnostic information
Sixth Annual Intensive Update in Neurology 0915-162016
76
References
BPPVndash White J Benign paroxysmal positional vertigo Cleveland Clinic Journal of
Medicine 71(9)722-728 2004
ndash White J Coale K Catalano P Oas J Diagnosis and management of lateral semicircular canal benign positional vertigo Otolaryngology-Head and Neck Surgery 133 278-284 2005
ndash White J Savvides P Cherian N Oas J Canalith repositioning for benign paroxysmal positional vertigo Otology and Neurotology 26 704-710 2005
ndash White J Oas J Diagnosis and management of lateral semicircular canal conversions during particle repositioning therapy Laryngoscope 115(10) 1895-1897 2005
ndash Acute Vestibular Syndrome Hotson JR Baloh RW N Engl J Med 1998 Sep 3339(10)680-5
ndash Methylprednisolone valacyclovir or the combination for vestibular neuritis Strupp M Zingler VC Arbusow V Niklas D Maag KP Dieterich M Bense S Theil D Jahn K Brandt T N Engl J Med 2004 Jul 22351(4)354-61
ndash Intratympanic steroids for Meacuteniegraveres disease or syndrome Phillips JS Westerberg BCochrane Database Syst Rev 2011 Jul 6(7)CD008514 Review
ndash Sound- andor pressure-induced vertigo due to bone dehiscence of the superior semicircular canal Minor LB Solomon D Zinreich JS Zee DSArch Otolaryngol Head Neck Surg 1998 Mar124(3)249-58
Sixth Annual Intensive Update in Neurology 0915-162016
65
Question 5
bull Superior semicircular canal dehiscence
1) Causes increased ability to hear vibratory sounds
2) Improves hearing
3) Causes meningitis
4) Always needs surgical repair
Sixth Annual Intensive Update in Neurology 0915-162016
66
Question 5
bull Superior semicircular canal dehiscence
1) Causes increased ability to hear vibratory sounds
2) Improves hearing
3) Causes meningitis
4) Always needs surgical repair
Answer 1 References at end of lecture
Sixth Annual Intensive Update in Neurology 0915-162016
67
Enlarged vestibular aqueduct
bull Axial temporal bone CT showing right temporal bone with enlarged vestibular aqueduct (arrow)
bull Vestibular symptoms accompany progressive sensorineural hearing loss in 4-50 of patients in selected series
Sixth Annual Intensive Update in Neurology 0915-162016
68
Migraine
bull Family or personal history of migraine
bull 25 of migraine patients experience spontaneous vertigo (Baloh 1997)
bull Neuhauser criteria(2001) have been used to construct structured diagnostic interviews (Furman 2003)
bull Not always associated with headache
bull Motion sensitivity
bull Medication responsive
Sixth Annual Intensive Update in Neurology 0915-162016
69
ICHD-III beta Criteria for Vestibular Migraine
bull A At least five attacks fulfilling criteria C and D
bull B A current or past history of 11 migraine without aura or 12 migraine with aura
bull C Vestibular symptoms of moderate or severe intensity lasting between 5 minutes and 72 hours
bull D At least 50 associated with one of the following migrainous features
ndash 1 Headaches
ndash 2 Photophobia and phonophobia
ndash 3 Visual Aura
bull E Not better accounted for by another vestibular disorder
Sixth Annual Intensive Update in Neurology 0915-162016
70
Incidence of vertigo in MS
bull True vertigo (the perception of environmental
movement typically rotational) occurs in about
20 of MS patients and is the presenting
manifestation in 5
bull Up to 78 of MS patients report abnormalities
in balance at some point in their illness
Frohman et al Multiple Sclerosis 2003 9250
Sixth Annual Intensive Update in Neurology 0915-162016
71
Vertigo in MS due to demyelination
bull The most common neuroanatomic locations are the
root entry zone of CN VIII (giving absent calorics but
normal hearing and fixation suppression) and the
medial vestibular nucleus
bull Usually treated with steroids and vestibular
suppressants
Brandt 1993 Gass 1998 Thomke 1999
Sixth Annual Intensive Update in Neurology 0915-162016
72
Vertigo in MS
Frohmanrsquos large series (1153) reported that 52 of MS patients with acute vertigo had benign paroxysmal positional vertigo while 32 had new brainstem lesions
To avoid misdiagnoses and inappropriate treatment interventions alternative etiologies should be considered in all acute vertigo episodes in MS
E M Frohman Neurology 2000 and Multiple Sclerosis 2003
Sixth Annual Intensive Update in Neurology 0915-162016
73
Vestibular compensation
bull Acute unilateral or bilateral vestibular loss will prompt compensation thru central cervico-ocular visual and proprioceptive pathways
bull Unilateral loss may require several weeks for compensation to occur and activity especially head movements hastens compensation
bull Vestibular suppressant medication prolongs compensation
Sixth Annual Intensive Update in Neurology 0915-162016
74
Vestibular Rehabilitation
bull Performed by certified physical therapists many sites (Epic order vestibular PT) or check Vestibular Disorders of America website for providers
bull Assessment and treatment of benign positional vertigo
bull Directed rehab programs for central andor peripheral vestibular compensationndash Unilateral vestibulopathies
ndash Bilateral vestibular hypofunction
ndash Mutifactorial balance deficits and fall risk assessment
Sixth Annual Intensive Update in Neurology 0915-162016
75
Summary of whatrsquos new in peripheral vestibular disorders
bull Lateral semicircular canal benign paroxysmal positional vertigo presents with horizontal positioning nystagmus
bull Oral steroids appear to improve outcome in acute vestibular neuritis
bull Intratympanic steroids offer promise in Menierersquos syndrome
bull Superior semicircular canal dehiscence causes pressure and noise induced dizziness VEMP offers diagnostic information
Sixth Annual Intensive Update in Neurology 0915-162016
76
References
BPPVndash White J Benign paroxysmal positional vertigo Cleveland Clinic Journal of
Medicine 71(9)722-728 2004
ndash White J Coale K Catalano P Oas J Diagnosis and management of lateral semicircular canal benign positional vertigo Otolaryngology-Head and Neck Surgery 133 278-284 2005
ndash White J Savvides P Cherian N Oas J Canalith repositioning for benign paroxysmal positional vertigo Otology and Neurotology 26 704-710 2005
ndash White J Oas J Diagnosis and management of lateral semicircular canal conversions during particle repositioning therapy Laryngoscope 115(10) 1895-1897 2005
ndash Acute Vestibular Syndrome Hotson JR Baloh RW N Engl J Med 1998 Sep 3339(10)680-5
ndash Methylprednisolone valacyclovir or the combination for vestibular neuritis Strupp M Zingler VC Arbusow V Niklas D Maag KP Dieterich M Bense S Theil D Jahn K Brandt T N Engl J Med 2004 Jul 22351(4)354-61
ndash Intratympanic steroids for Meacuteniegraveres disease or syndrome Phillips JS Westerberg BCochrane Database Syst Rev 2011 Jul 6(7)CD008514 Review
ndash Sound- andor pressure-induced vertigo due to bone dehiscence of the superior semicircular canal Minor LB Solomon D Zinreich JS Zee DSArch Otolaryngol Head Neck Surg 1998 Mar124(3)249-58
Sixth Annual Intensive Update in Neurology 0915-162016
66
Question 5
bull Superior semicircular canal dehiscence
1) Causes increased ability to hear vibratory sounds
2) Improves hearing
3) Causes meningitis
4) Always needs surgical repair
Answer 1 References at end of lecture
Sixth Annual Intensive Update in Neurology 0915-162016
67
Enlarged vestibular aqueduct
bull Axial temporal bone CT showing right temporal bone with enlarged vestibular aqueduct (arrow)
bull Vestibular symptoms accompany progressive sensorineural hearing loss in 4-50 of patients in selected series
Sixth Annual Intensive Update in Neurology 0915-162016
68
Migraine
bull Family or personal history of migraine
bull 25 of migraine patients experience spontaneous vertigo (Baloh 1997)
bull Neuhauser criteria(2001) have been used to construct structured diagnostic interviews (Furman 2003)
bull Not always associated with headache
bull Motion sensitivity
bull Medication responsive
Sixth Annual Intensive Update in Neurology 0915-162016
69
ICHD-III beta Criteria for Vestibular Migraine
bull A At least five attacks fulfilling criteria C and D
bull B A current or past history of 11 migraine without aura or 12 migraine with aura
bull C Vestibular symptoms of moderate or severe intensity lasting between 5 minutes and 72 hours
bull D At least 50 associated with one of the following migrainous features
ndash 1 Headaches
ndash 2 Photophobia and phonophobia
ndash 3 Visual Aura
bull E Not better accounted for by another vestibular disorder
Sixth Annual Intensive Update in Neurology 0915-162016
70
Incidence of vertigo in MS
bull True vertigo (the perception of environmental
movement typically rotational) occurs in about
20 of MS patients and is the presenting
manifestation in 5
bull Up to 78 of MS patients report abnormalities
in balance at some point in their illness
Frohman et al Multiple Sclerosis 2003 9250
Sixth Annual Intensive Update in Neurology 0915-162016
71
Vertigo in MS due to demyelination
bull The most common neuroanatomic locations are the
root entry zone of CN VIII (giving absent calorics but
normal hearing and fixation suppression) and the
medial vestibular nucleus
bull Usually treated with steroids and vestibular
suppressants
Brandt 1993 Gass 1998 Thomke 1999
Sixth Annual Intensive Update in Neurology 0915-162016
72
Vertigo in MS
Frohmanrsquos large series (1153) reported that 52 of MS patients with acute vertigo had benign paroxysmal positional vertigo while 32 had new brainstem lesions
To avoid misdiagnoses and inappropriate treatment interventions alternative etiologies should be considered in all acute vertigo episodes in MS
E M Frohman Neurology 2000 and Multiple Sclerosis 2003
Sixth Annual Intensive Update in Neurology 0915-162016
73
Vestibular compensation
bull Acute unilateral or bilateral vestibular loss will prompt compensation thru central cervico-ocular visual and proprioceptive pathways
bull Unilateral loss may require several weeks for compensation to occur and activity especially head movements hastens compensation
bull Vestibular suppressant medication prolongs compensation
Sixth Annual Intensive Update in Neurology 0915-162016
74
Vestibular Rehabilitation
bull Performed by certified physical therapists many sites (Epic order vestibular PT) or check Vestibular Disorders of America website for providers
bull Assessment and treatment of benign positional vertigo
bull Directed rehab programs for central andor peripheral vestibular compensationndash Unilateral vestibulopathies
ndash Bilateral vestibular hypofunction
ndash Mutifactorial balance deficits and fall risk assessment
Sixth Annual Intensive Update in Neurology 0915-162016
75
Summary of whatrsquos new in peripheral vestibular disorders
bull Lateral semicircular canal benign paroxysmal positional vertigo presents with horizontal positioning nystagmus
bull Oral steroids appear to improve outcome in acute vestibular neuritis
bull Intratympanic steroids offer promise in Menierersquos syndrome
bull Superior semicircular canal dehiscence causes pressure and noise induced dizziness VEMP offers diagnostic information
Sixth Annual Intensive Update in Neurology 0915-162016
76
References
BPPVndash White J Benign paroxysmal positional vertigo Cleveland Clinic Journal of
Medicine 71(9)722-728 2004
ndash White J Coale K Catalano P Oas J Diagnosis and management of lateral semicircular canal benign positional vertigo Otolaryngology-Head and Neck Surgery 133 278-284 2005
ndash White J Savvides P Cherian N Oas J Canalith repositioning for benign paroxysmal positional vertigo Otology and Neurotology 26 704-710 2005
ndash White J Oas J Diagnosis and management of lateral semicircular canal conversions during particle repositioning therapy Laryngoscope 115(10) 1895-1897 2005
ndash Acute Vestibular Syndrome Hotson JR Baloh RW N Engl J Med 1998 Sep 3339(10)680-5
ndash Methylprednisolone valacyclovir or the combination for vestibular neuritis Strupp M Zingler VC Arbusow V Niklas D Maag KP Dieterich M Bense S Theil D Jahn K Brandt T N Engl J Med 2004 Jul 22351(4)354-61
ndash Intratympanic steroids for Meacuteniegraveres disease or syndrome Phillips JS Westerberg BCochrane Database Syst Rev 2011 Jul 6(7)CD008514 Review
ndash Sound- andor pressure-induced vertigo due to bone dehiscence of the superior semicircular canal Minor LB Solomon D Zinreich JS Zee DSArch Otolaryngol Head Neck Surg 1998 Mar124(3)249-58
Sixth Annual Intensive Update in Neurology 0915-162016
67
Enlarged vestibular aqueduct
bull Axial temporal bone CT showing right temporal bone with enlarged vestibular aqueduct (arrow)
bull Vestibular symptoms accompany progressive sensorineural hearing loss in 4-50 of patients in selected series
Sixth Annual Intensive Update in Neurology 0915-162016
68
Migraine
bull Family or personal history of migraine
bull 25 of migraine patients experience spontaneous vertigo (Baloh 1997)
bull Neuhauser criteria(2001) have been used to construct structured diagnostic interviews (Furman 2003)
bull Not always associated with headache
bull Motion sensitivity
bull Medication responsive
Sixth Annual Intensive Update in Neurology 0915-162016
69
ICHD-III beta Criteria for Vestibular Migraine
bull A At least five attacks fulfilling criteria C and D
bull B A current or past history of 11 migraine without aura or 12 migraine with aura
bull C Vestibular symptoms of moderate or severe intensity lasting between 5 minutes and 72 hours
bull D At least 50 associated with one of the following migrainous features
ndash 1 Headaches
ndash 2 Photophobia and phonophobia
ndash 3 Visual Aura
bull E Not better accounted for by another vestibular disorder
Sixth Annual Intensive Update in Neurology 0915-162016
70
Incidence of vertigo in MS
bull True vertigo (the perception of environmental
movement typically rotational) occurs in about
20 of MS patients and is the presenting
manifestation in 5
bull Up to 78 of MS patients report abnormalities
in balance at some point in their illness
Frohman et al Multiple Sclerosis 2003 9250
Sixth Annual Intensive Update in Neurology 0915-162016
71
Vertigo in MS due to demyelination
bull The most common neuroanatomic locations are the
root entry zone of CN VIII (giving absent calorics but
normal hearing and fixation suppression) and the
medial vestibular nucleus
bull Usually treated with steroids and vestibular
suppressants
Brandt 1993 Gass 1998 Thomke 1999
Sixth Annual Intensive Update in Neurology 0915-162016
72
Vertigo in MS
Frohmanrsquos large series (1153) reported that 52 of MS patients with acute vertigo had benign paroxysmal positional vertigo while 32 had new brainstem lesions
To avoid misdiagnoses and inappropriate treatment interventions alternative etiologies should be considered in all acute vertigo episodes in MS
E M Frohman Neurology 2000 and Multiple Sclerosis 2003
Sixth Annual Intensive Update in Neurology 0915-162016
73
Vestibular compensation
bull Acute unilateral or bilateral vestibular loss will prompt compensation thru central cervico-ocular visual and proprioceptive pathways
bull Unilateral loss may require several weeks for compensation to occur and activity especially head movements hastens compensation
bull Vestibular suppressant medication prolongs compensation
Sixth Annual Intensive Update in Neurology 0915-162016
74
Vestibular Rehabilitation
bull Performed by certified physical therapists many sites (Epic order vestibular PT) or check Vestibular Disorders of America website for providers
bull Assessment and treatment of benign positional vertigo
bull Directed rehab programs for central andor peripheral vestibular compensationndash Unilateral vestibulopathies
ndash Bilateral vestibular hypofunction
ndash Mutifactorial balance deficits and fall risk assessment
Sixth Annual Intensive Update in Neurology 0915-162016
75
Summary of whatrsquos new in peripheral vestibular disorders
bull Lateral semicircular canal benign paroxysmal positional vertigo presents with horizontal positioning nystagmus
bull Oral steroids appear to improve outcome in acute vestibular neuritis
bull Intratympanic steroids offer promise in Menierersquos syndrome
bull Superior semicircular canal dehiscence causes pressure and noise induced dizziness VEMP offers diagnostic information
Sixth Annual Intensive Update in Neurology 0915-162016
76
References
BPPVndash White J Benign paroxysmal positional vertigo Cleveland Clinic Journal of
Medicine 71(9)722-728 2004
ndash White J Coale K Catalano P Oas J Diagnosis and management of lateral semicircular canal benign positional vertigo Otolaryngology-Head and Neck Surgery 133 278-284 2005
ndash White J Savvides P Cherian N Oas J Canalith repositioning for benign paroxysmal positional vertigo Otology and Neurotology 26 704-710 2005
ndash White J Oas J Diagnosis and management of lateral semicircular canal conversions during particle repositioning therapy Laryngoscope 115(10) 1895-1897 2005
ndash Acute Vestibular Syndrome Hotson JR Baloh RW N Engl J Med 1998 Sep 3339(10)680-5
ndash Methylprednisolone valacyclovir or the combination for vestibular neuritis Strupp M Zingler VC Arbusow V Niklas D Maag KP Dieterich M Bense S Theil D Jahn K Brandt T N Engl J Med 2004 Jul 22351(4)354-61
ndash Intratympanic steroids for Meacuteniegraveres disease or syndrome Phillips JS Westerberg BCochrane Database Syst Rev 2011 Jul 6(7)CD008514 Review
ndash Sound- andor pressure-induced vertigo due to bone dehiscence of the superior semicircular canal Minor LB Solomon D Zinreich JS Zee DSArch Otolaryngol Head Neck Surg 1998 Mar124(3)249-58
Sixth Annual Intensive Update in Neurology 0915-162016
68
Migraine
bull Family or personal history of migraine
bull 25 of migraine patients experience spontaneous vertigo (Baloh 1997)
bull Neuhauser criteria(2001) have been used to construct structured diagnostic interviews (Furman 2003)
bull Not always associated with headache
bull Motion sensitivity
bull Medication responsive
Sixth Annual Intensive Update in Neurology 0915-162016
69
ICHD-III beta Criteria for Vestibular Migraine
bull A At least five attacks fulfilling criteria C and D
bull B A current or past history of 11 migraine without aura or 12 migraine with aura
bull C Vestibular symptoms of moderate or severe intensity lasting between 5 minutes and 72 hours
bull D At least 50 associated with one of the following migrainous features
ndash 1 Headaches
ndash 2 Photophobia and phonophobia
ndash 3 Visual Aura
bull E Not better accounted for by another vestibular disorder
Sixth Annual Intensive Update in Neurology 0915-162016
70
Incidence of vertigo in MS
bull True vertigo (the perception of environmental
movement typically rotational) occurs in about
20 of MS patients and is the presenting
manifestation in 5
bull Up to 78 of MS patients report abnormalities
in balance at some point in their illness
Frohman et al Multiple Sclerosis 2003 9250
Sixth Annual Intensive Update in Neurology 0915-162016
71
Vertigo in MS due to demyelination
bull The most common neuroanatomic locations are the
root entry zone of CN VIII (giving absent calorics but
normal hearing and fixation suppression) and the
medial vestibular nucleus
bull Usually treated with steroids and vestibular
suppressants
Brandt 1993 Gass 1998 Thomke 1999
Sixth Annual Intensive Update in Neurology 0915-162016
72
Vertigo in MS
Frohmanrsquos large series (1153) reported that 52 of MS patients with acute vertigo had benign paroxysmal positional vertigo while 32 had new brainstem lesions
To avoid misdiagnoses and inappropriate treatment interventions alternative etiologies should be considered in all acute vertigo episodes in MS
E M Frohman Neurology 2000 and Multiple Sclerosis 2003
Sixth Annual Intensive Update in Neurology 0915-162016
73
Vestibular compensation
bull Acute unilateral or bilateral vestibular loss will prompt compensation thru central cervico-ocular visual and proprioceptive pathways
bull Unilateral loss may require several weeks for compensation to occur and activity especially head movements hastens compensation
bull Vestibular suppressant medication prolongs compensation
Sixth Annual Intensive Update in Neurology 0915-162016
74
Vestibular Rehabilitation
bull Performed by certified physical therapists many sites (Epic order vestibular PT) or check Vestibular Disorders of America website for providers
bull Assessment and treatment of benign positional vertigo
bull Directed rehab programs for central andor peripheral vestibular compensationndash Unilateral vestibulopathies
ndash Bilateral vestibular hypofunction
ndash Mutifactorial balance deficits and fall risk assessment
Sixth Annual Intensive Update in Neurology 0915-162016
75
Summary of whatrsquos new in peripheral vestibular disorders
bull Lateral semicircular canal benign paroxysmal positional vertigo presents with horizontal positioning nystagmus
bull Oral steroids appear to improve outcome in acute vestibular neuritis
bull Intratympanic steroids offer promise in Menierersquos syndrome
bull Superior semicircular canal dehiscence causes pressure and noise induced dizziness VEMP offers diagnostic information
Sixth Annual Intensive Update in Neurology 0915-162016
76
References
BPPVndash White J Benign paroxysmal positional vertigo Cleveland Clinic Journal of
Medicine 71(9)722-728 2004
ndash White J Coale K Catalano P Oas J Diagnosis and management of lateral semicircular canal benign positional vertigo Otolaryngology-Head and Neck Surgery 133 278-284 2005
ndash White J Savvides P Cherian N Oas J Canalith repositioning for benign paroxysmal positional vertigo Otology and Neurotology 26 704-710 2005
ndash White J Oas J Diagnosis and management of lateral semicircular canal conversions during particle repositioning therapy Laryngoscope 115(10) 1895-1897 2005
ndash Acute Vestibular Syndrome Hotson JR Baloh RW N Engl J Med 1998 Sep 3339(10)680-5
ndash Methylprednisolone valacyclovir or the combination for vestibular neuritis Strupp M Zingler VC Arbusow V Niklas D Maag KP Dieterich M Bense S Theil D Jahn K Brandt T N Engl J Med 2004 Jul 22351(4)354-61
ndash Intratympanic steroids for Meacuteniegraveres disease or syndrome Phillips JS Westerberg BCochrane Database Syst Rev 2011 Jul 6(7)CD008514 Review
ndash Sound- andor pressure-induced vertigo due to bone dehiscence of the superior semicircular canal Minor LB Solomon D Zinreich JS Zee DSArch Otolaryngol Head Neck Surg 1998 Mar124(3)249-58
Sixth Annual Intensive Update in Neurology 0915-162016
69
ICHD-III beta Criteria for Vestibular Migraine
bull A At least five attacks fulfilling criteria C and D
bull B A current or past history of 11 migraine without aura or 12 migraine with aura
bull C Vestibular symptoms of moderate or severe intensity lasting between 5 minutes and 72 hours
bull D At least 50 associated with one of the following migrainous features
ndash 1 Headaches
ndash 2 Photophobia and phonophobia
ndash 3 Visual Aura
bull E Not better accounted for by another vestibular disorder
Sixth Annual Intensive Update in Neurology 0915-162016
70
Incidence of vertigo in MS
bull True vertigo (the perception of environmental
movement typically rotational) occurs in about
20 of MS patients and is the presenting
manifestation in 5
bull Up to 78 of MS patients report abnormalities
in balance at some point in their illness
Frohman et al Multiple Sclerosis 2003 9250
Sixth Annual Intensive Update in Neurology 0915-162016
71
Vertigo in MS due to demyelination
bull The most common neuroanatomic locations are the
root entry zone of CN VIII (giving absent calorics but
normal hearing and fixation suppression) and the
medial vestibular nucleus
bull Usually treated with steroids and vestibular
suppressants
Brandt 1993 Gass 1998 Thomke 1999
Sixth Annual Intensive Update in Neurology 0915-162016
72
Vertigo in MS
Frohmanrsquos large series (1153) reported that 52 of MS patients with acute vertigo had benign paroxysmal positional vertigo while 32 had new brainstem lesions
To avoid misdiagnoses and inappropriate treatment interventions alternative etiologies should be considered in all acute vertigo episodes in MS
E M Frohman Neurology 2000 and Multiple Sclerosis 2003
Sixth Annual Intensive Update in Neurology 0915-162016
73
Vestibular compensation
bull Acute unilateral or bilateral vestibular loss will prompt compensation thru central cervico-ocular visual and proprioceptive pathways
bull Unilateral loss may require several weeks for compensation to occur and activity especially head movements hastens compensation
bull Vestibular suppressant medication prolongs compensation
Sixth Annual Intensive Update in Neurology 0915-162016
74
Vestibular Rehabilitation
bull Performed by certified physical therapists many sites (Epic order vestibular PT) or check Vestibular Disorders of America website for providers
bull Assessment and treatment of benign positional vertigo
bull Directed rehab programs for central andor peripheral vestibular compensationndash Unilateral vestibulopathies
ndash Bilateral vestibular hypofunction
ndash Mutifactorial balance deficits and fall risk assessment
Sixth Annual Intensive Update in Neurology 0915-162016
75
Summary of whatrsquos new in peripheral vestibular disorders
bull Lateral semicircular canal benign paroxysmal positional vertigo presents with horizontal positioning nystagmus
bull Oral steroids appear to improve outcome in acute vestibular neuritis
bull Intratympanic steroids offer promise in Menierersquos syndrome
bull Superior semicircular canal dehiscence causes pressure and noise induced dizziness VEMP offers diagnostic information
Sixth Annual Intensive Update in Neurology 0915-162016
76
References
BPPVndash White J Benign paroxysmal positional vertigo Cleveland Clinic Journal of
Medicine 71(9)722-728 2004
ndash White J Coale K Catalano P Oas J Diagnosis and management of lateral semicircular canal benign positional vertigo Otolaryngology-Head and Neck Surgery 133 278-284 2005
ndash White J Savvides P Cherian N Oas J Canalith repositioning for benign paroxysmal positional vertigo Otology and Neurotology 26 704-710 2005
ndash White J Oas J Diagnosis and management of lateral semicircular canal conversions during particle repositioning therapy Laryngoscope 115(10) 1895-1897 2005
ndash Acute Vestibular Syndrome Hotson JR Baloh RW N Engl J Med 1998 Sep 3339(10)680-5
ndash Methylprednisolone valacyclovir or the combination for vestibular neuritis Strupp M Zingler VC Arbusow V Niklas D Maag KP Dieterich M Bense S Theil D Jahn K Brandt T N Engl J Med 2004 Jul 22351(4)354-61
ndash Intratympanic steroids for Meacuteniegraveres disease or syndrome Phillips JS Westerberg BCochrane Database Syst Rev 2011 Jul 6(7)CD008514 Review
ndash Sound- andor pressure-induced vertigo due to bone dehiscence of the superior semicircular canal Minor LB Solomon D Zinreich JS Zee DSArch Otolaryngol Head Neck Surg 1998 Mar124(3)249-58
Sixth Annual Intensive Update in Neurology 0915-162016
70
Incidence of vertigo in MS
bull True vertigo (the perception of environmental
movement typically rotational) occurs in about
20 of MS patients and is the presenting
manifestation in 5
bull Up to 78 of MS patients report abnormalities
in balance at some point in their illness
Frohman et al Multiple Sclerosis 2003 9250
Sixth Annual Intensive Update in Neurology 0915-162016
71
Vertigo in MS due to demyelination
bull The most common neuroanatomic locations are the
root entry zone of CN VIII (giving absent calorics but
normal hearing and fixation suppression) and the
medial vestibular nucleus
bull Usually treated with steroids and vestibular
suppressants
Brandt 1993 Gass 1998 Thomke 1999
Sixth Annual Intensive Update in Neurology 0915-162016
72
Vertigo in MS
Frohmanrsquos large series (1153) reported that 52 of MS patients with acute vertigo had benign paroxysmal positional vertigo while 32 had new brainstem lesions
To avoid misdiagnoses and inappropriate treatment interventions alternative etiologies should be considered in all acute vertigo episodes in MS
E M Frohman Neurology 2000 and Multiple Sclerosis 2003
Sixth Annual Intensive Update in Neurology 0915-162016
73
Vestibular compensation
bull Acute unilateral or bilateral vestibular loss will prompt compensation thru central cervico-ocular visual and proprioceptive pathways
bull Unilateral loss may require several weeks for compensation to occur and activity especially head movements hastens compensation
bull Vestibular suppressant medication prolongs compensation
Sixth Annual Intensive Update in Neurology 0915-162016
74
Vestibular Rehabilitation
bull Performed by certified physical therapists many sites (Epic order vestibular PT) or check Vestibular Disorders of America website for providers
bull Assessment and treatment of benign positional vertigo
bull Directed rehab programs for central andor peripheral vestibular compensationndash Unilateral vestibulopathies
ndash Bilateral vestibular hypofunction
ndash Mutifactorial balance deficits and fall risk assessment
Sixth Annual Intensive Update in Neurology 0915-162016
75
Summary of whatrsquos new in peripheral vestibular disorders
bull Lateral semicircular canal benign paroxysmal positional vertigo presents with horizontal positioning nystagmus
bull Oral steroids appear to improve outcome in acute vestibular neuritis
bull Intratympanic steroids offer promise in Menierersquos syndrome
bull Superior semicircular canal dehiscence causes pressure and noise induced dizziness VEMP offers diagnostic information
Sixth Annual Intensive Update in Neurology 0915-162016
76
References
BPPVndash White J Benign paroxysmal positional vertigo Cleveland Clinic Journal of
Medicine 71(9)722-728 2004
ndash White J Coale K Catalano P Oas J Diagnosis and management of lateral semicircular canal benign positional vertigo Otolaryngology-Head and Neck Surgery 133 278-284 2005
ndash White J Savvides P Cherian N Oas J Canalith repositioning for benign paroxysmal positional vertigo Otology and Neurotology 26 704-710 2005
ndash White J Oas J Diagnosis and management of lateral semicircular canal conversions during particle repositioning therapy Laryngoscope 115(10) 1895-1897 2005
ndash Acute Vestibular Syndrome Hotson JR Baloh RW N Engl J Med 1998 Sep 3339(10)680-5
ndash Methylprednisolone valacyclovir or the combination for vestibular neuritis Strupp M Zingler VC Arbusow V Niklas D Maag KP Dieterich M Bense S Theil D Jahn K Brandt T N Engl J Med 2004 Jul 22351(4)354-61
ndash Intratympanic steroids for Meacuteniegraveres disease or syndrome Phillips JS Westerberg BCochrane Database Syst Rev 2011 Jul 6(7)CD008514 Review
ndash Sound- andor pressure-induced vertigo due to bone dehiscence of the superior semicircular canal Minor LB Solomon D Zinreich JS Zee DSArch Otolaryngol Head Neck Surg 1998 Mar124(3)249-58
Sixth Annual Intensive Update in Neurology 0915-162016
71
Vertigo in MS due to demyelination
bull The most common neuroanatomic locations are the
root entry zone of CN VIII (giving absent calorics but
normal hearing and fixation suppression) and the
medial vestibular nucleus
bull Usually treated with steroids and vestibular
suppressants
Brandt 1993 Gass 1998 Thomke 1999
Sixth Annual Intensive Update in Neurology 0915-162016
72
Vertigo in MS
Frohmanrsquos large series (1153) reported that 52 of MS patients with acute vertigo had benign paroxysmal positional vertigo while 32 had new brainstem lesions
To avoid misdiagnoses and inappropriate treatment interventions alternative etiologies should be considered in all acute vertigo episodes in MS
E M Frohman Neurology 2000 and Multiple Sclerosis 2003
Sixth Annual Intensive Update in Neurology 0915-162016
73
Vestibular compensation
bull Acute unilateral or bilateral vestibular loss will prompt compensation thru central cervico-ocular visual and proprioceptive pathways
bull Unilateral loss may require several weeks for compensation to occur and activity especially head movements hastens compensation
bull Vestibular suppressant medication prolongs compensation
Sixth Annual Intensive Update in Neurology 0915-162016
74
Vestibular Rehabilitation
bull Performed by certified physical therapists many sites (Epic order vestibular PT) or check Vestibular Disorders of America website for providers
bull Assessment and treatment of benign positional vertigo
bull Directed rehab programs for central andor peripheral vestibular compensationndash Unilateral vestibulopathies
ndash Bilateral vestibular hypofunction
ndash Mutifactorial balance deficits and fall risk assessment
Sixth Annual Intensive Update in Neurology 0915-162016
75
Summary of whatrsquos new in peripheral vestibular disorders
bull Lateral semicircular canal benign paroxysmal positional vertigo presents with horizontal positioning nystagmus
bull Oral steroids appear to improve outcome in acute vestibular neuritis
bull Intratympanic steroids offer promise in Menierersquos syndrome
bull Superior semicircular canal dehiscence causes pressure and noise induced dizziness VEMP offers diagnostic information
Sixth Annual Intensive Update in Neurology 0915-162016
76
References
BPPVndash White J Benign paroxysmal positional vertigo Cleveland Clinic Journal of
Medicine 71(9)722-728 2004
ndash White J Coale K Catalano P Oas J Diagnosis and management of lateral semicircular canal benign positional vertigo Otolaryngology-Head and Neck Surgery 133 278-284 2005
ndash White J Savvides P Cherian N Oas J Canalith repositioning for benign paroxysmal positional vertigo Otology and Neurotology 26 704-710 2005
ndash White J Oas J Diagnosis and management of lateral semicircular canal conversions during particle repositioning therapy Laryngoscope 115(10) 1895-1897 2005
ndash Acute Vestibular Syndrome Hotson JR Baloh RW N Engl J Med 1998 Sep 3339(10)680-5
ndash Methylprednisolone valacyclovir or the combination for vestibular neuritis Strupp M Zingler VC Arbusow V Niklas D Maag KP Dieterich M Bense S Theil D Jahn K Brandt T N Engl J Med 2004 Jul 22351(4)354-61
ndash Intratympanic steroids for Meacuteniegraveres disease or syndrome Phillips JS Westerberg BCochrane Database Syst Rev 2011 Jul 6(7)CD008514 Review
ndash Sound- andor pressure-induced vertigo due to bone dehiscence of the superior semicircular canal Minor LB Solomon D Zinreich JS Zee DSArch Otolaryngol Head Neck Surg 1998 Mar124(3)249-58
Sixth Annual Intensive Update in Neurology 0915-162016
72
Vertigo in MS
Frohmanrsquos large series (1153) reported that 52 of MS patients with acute vertigo had benign paroxysmal positional vertigo while 32 had new brainstem lesions
To avoid misdiagnoses and inappropriate treatment interventions alternative etiologies should be considered in all acute vertigo episodes in MS
E M Frohman Neurology 2000 and Multiple Sclerosis 2003
Sixth Annual Intensive Update in Neurology 0915-162016
73
Vestibular compensation
bull Acute unilateral or bilateral vestibular loss will prompt compensation thru central cervico-ocular visual and proprioceptive pathways
bull Unilateral loss may require several weeks for compensation to occur and activity especially head movements hastens compensation
bull Vestibular suppressant medication prolongs compensation
Sixth Annual Intensive Update in Neurology 0915-162016
74
Vestibular Rehabilitation
bull Performed by certified physical therapists many sites (Epic order vestibular PT) or check Vestibular Disorders of America website for providers
bull Assessment and treatment of benign positional vertigo
bull Directed rehab programs for central andor peripheral vestibular compensationndash Unilateral vestibulopathies
ndash Bilateral vestibular hypofunction
ndash Mutifactorial balance deficits and fall risk assessment
Sixth Annual Intensive Update in Neurology 0915-162016
75
Summary of whatrsquos new in peripheral vestibular disorders
bull Lateral semicircular canal benign paroxysmal positional vertigo presents with horizontal positioning nystagmus
bull Oral steroids appear to improve outcome in acute vestibular neuritis
bull Intratympanic steroids offer promise in Menierersquos syndrome
bull Superior semicircular canal dehiscence causes pressure and noise induced dizziness VEMP offers diagnostic information
Sixth Annual Intensive Update in Neurology 0915-162016
76
References
BPPVndash White J Benign paroxysmal positional vertigo Cleveland Clinic Journal of
Medicine 71(9)722-728 2004
ndash White J Coale K Catalano P Oas J Diagnosis and management of lateral semicircular canal benign positional vertigo Otolaryngology-Head and Neck Surgery 133 278-284 2005
ndash White J Savvides P Cherian N Oas J Canalith repositioning for benign paroxysmal positional vertigo Otology and Neurotology 26 704-710 2005
ndash White J Oas J Diagnosis and management of lateral semicircular canal conversions during particle repositioning therapy Laryngoscope 115(10) 1895-1897 2005
ndash Acute Vestibular Syndrome Hotson JR Baloh RW N Engl J Med 1998 Sep 3339(10)680-5
ndash Methylprednisolone valacyclovir or the combination for vestibular neuritis Strupp M Zingler VC Arbusow V Niklas D Maag KP Dieterich M Bense S Theil D Jahn K Brandt T N Engl J Med 2004 Jul 22351(4)354-61
ndash Intratympanic steroids for Meacuteniegraveres disease or syndrome Phillips JS Westerberg BCochrane Database Syst Rev 2011 Jul 6(7)CD008514 Review
ndash Sound- andor pressure-induced vertigo due to bone dehiscence of the superior semicircular canal Minor LB Solomon D Zinreich JS Zee DSArch Otolaryngol Head Neck Surg 1998 Mar124(3)249-58
Sixth Annual Intensive Update in Neurology 0915-162016
73
Vestibular compensation
bull Acute unilateral or bilateral vestibular loss will prompt compensation thru central cervico-ocular visual and proprioceptive pathways
bull Unilateral loss may require several weeks for compensation to occur and activity especially head movements hastens compensation
bull Vestibular suppressant medication prolongs compensation
Sixth Annual Intensive Update in Neurology 0915-162016
74
Vestibular Rehabilitation
bull Performed by certified physical therapists many sites (Epic order vestibular PT) or check Vestibular Disorders of America website for providers
bull Assessment and treatment of benign positional vertigo
bull Directed rehab programs for central andor peripheral vestibular compensationndash Unilateral vestibulopathies
ndash Bilateral vestibular hypofunction
ndash Mutifactorial balance deficits and fall risk assessment
Sixth Annual Intensive Update in Neurology 0915-162016
75
Summary of whatrsquos new in peripheral vestibular disorders
bull Lateral semicircular canal benign paroxysmal positional vertigo presents with horizontal positioning nystagmus
bull Oral steroids appear to improve outcome in acute vestibular neuritis
bull Intratympanic steroids offer promise in Menierersquos syndrome
bull Superior semicircular canal dehiscence causes pressure and noise induced dizziness VEMP offers diagnostic information
Sixth Annual Intensive Update in Neurology 0915-162016
76
References
BPPVndash White J Benign paroxysmal positional vertigo Cleveland Clinic Journal of
Medicine 71(9)722-728 2004
ndash White J Coale K Catalano P Oas J Diagnosis and management of lateral semicircular canal benign positional vertigo Otolaryngology-Head and Neck Surgery 133 278-284 2005
ndash White J Savvides P Cherian N Oas J Canalith repositioning for benign paroxysmal positional vertigo Otology and Neurotology 26 704-710 2005
ndash White J Oas J Diagnosis and management of lateral semicircular canal conversions during particle repositioning therapy Laryngoscope 115(10) 1895-1897 2005
ndash Acute Vestibular Syndrome Hotson JR Baloh RW N Engl J Med 1998 Sep 3339(10)680-5
ndash Methylprednisolone valacyclovir or the combination for vestibular neuritis Strupp M Zingler VC Arbusow V Niklas D Maag KP Dieterich M Bense S Theil D Jahn K Brandt T N Engl J Med 2004 Jul 22351(4)354-61
ndash Intratympanic steroids for Meacuteniegraveres disease or syndrome Phillips JS Westerberg BCochrane Database Syst Rev 2011 Jul 6(7)CD008514 Review
ndash Sound- andor pressure-induced vertigo due to bone dehiscence of the superior semicircular canal Minor LB Solomon D Zinreich JS Zee DSArch Otolaryngol Head Neck Surg 1998 Mar124(3)249-58
Sixth Annual Intensive Update in Neurology 0915-162016
74
Vestibular Rehabilitation
bull Performed by certified physical therapists many sites (Epic order vestibular PT) or check Vestibular Disorders of America website for providers
bull Assessment and treatment of benign positional vertigo
bull Directed rehab programs for central andor peripheral vestibular compensationndash Unilateral vestibulopathies
ndash Bilateral vestibular hypofunction
ndash Mutifactorial balance deficits and fall risk assessment
Sixth Annual Intensive Update in Neurology 0915-162016
75
Summary of whatrsquos new in peripheral vestibular disorders
bull Lateral semicircular canal benign paroxysmal positional vertigo presents with horizontal positioning nystagmus
bull Oral steroids appear to improve outcome in acute vestibular neuritis
bull Intratympanic steroids offer promise in Menierersquos syndrome
bull Superior semicircular canal dehiscence causes pressure and noise induced dizziness VEMP offers diagnostic information
Sixth Annual Intensive Update in Neurology 0915-162016
76
References
BPPVndash White J Benign paroxysmal positional vertigo Cleveland Clinic Journal of
Medicine 71(9)722-728 2004
ndash White J Coale K Catalano P Oas J Diagnosis and management of lateral semicircular canal benign positional vertigo Otolaryngology-Head and Neck Surgery 133 278-284 2005
ndash White J Savvides P Cherian N Oas J Canalith repositioning for benign paroxysmal positional vertigo Otology and Neurotology 26 704-710 2005
ndash White J Oas J Diagnosis and management of lateral semicircular canal conversions during particle repositioning therapy Laryngoscope 115(10) 1895-1897 2005
ndash Acute Vestibular Syndrome Hotson JR Baloh RW N Engl J Med 1998 Sep 3339(10)680-5
ndash Methylprednisolone valacyclovir or the combination for vestibular neuritis Strupp M Zingler VC Arbusow V Niklas D Maag KP Dieterich M Bense S Theil D Jahn K Brandt T N Engl J Med 2004 Jul 22351(4)354-61
ndash Intratympanic steroids for Meacuteniegraveres disease or syndrome Phillips JS Westerberg BCochrane Database Syst Rev 2011 Jul 6(7)CD008514 Review
ndash Sound- andor pressure-induced vertigo due to bone dehiscence of the superior semicircular canal Minor LB Solomon D Zinreich JS Zee DSArch Otolaryngol Head Neck Surg 1998 Mar124(3)249-58
Sixth Annual Intensive Update in Neurology 0915-162016
75
Summary of whatrsquos new in peripheral vestibular disorders
bull Lateral semicircular canal benign paroxysmal positional vertigo presents with horizontal positioning nystagmus
bull Oral steroids appear to improve outcome in acute vestibular neuritis
bull Intratympanic steroids offer promise in Menierersquos syndrome
bull Superior semicircular canal dehiscence causes pressure and noise induced dizziness VEMP offers diagnostic information
Sixth Annual Intensive Update in Neurology 0915-162016
76
References
BPPVndash White J Benign paroxysmal positional vertigo Cleveland Clinic Journal of
Medicine 71(9)722-728 2004
ndash White J Coale K Catalano P Oas J Diagnosis and management of lateral semicircular canal benign positional vertigo Otolaryngology-Head and Neck Surgery 133 278-284 2005
ndash White J Savvides P Cherian N Oas J Canalith repositioning for benign paroxysmal positional vertigo Otology and Neurotology 26 704-710 2005
ndash White J Oas J Diagnosis and management of lateral semicircular canal conversions during particle repositioning therapy Laryngoscope 115(10) 1895-1897 2005
ndash Acute Vestibular Syndrome Hotson JR Baloh RW N Engl J Med 1998 Sep 3339(10)680-5
ndash Methylprednisolone valacyclovir or the combination for vestibular neuritis Strupp M Zingler VC Arbusow V Niklas D Maag KP Dieterich M Bense S Theil D Jahn K Brandt T N Engl J Med 2004 Jul 22351(4)354-61
ndash Intratympanic steroids for Meacuteniegraveres disease or syndrome Phillips JS Westerberg BCochrane Database Syst Rev 2011 Jul 6(7)CD008514 Review
ndash Sound- andor pressure-induced vertigo due to bone dehiscence of the superior semicircular canal Minor LB Solomon D Zinreich JS Zee DSArch Otolaryngol Head Neck Surg 1998 Mar124(3)249-58
Sixth Annual Intensive Update in Neurology 0915-162016
76
References
BPPVndash White J Benign paroxysmal positional vertigo Cleveland Clinic Journal of
Medicine 71(9)722-728 2004
ndash White J Coale K Catalano P Oas J Diagnosis and management of lateral semicircular canal benign positional vertigo Otolaryngology-Head and Neck Surgery 133 278-284 2005
ndash White J Savvides P Cherian N Oas J Canalith repositioning for benign paroxysmal positional vertigo Otology and Neurotology 26 704-710 2005
ndash White J Oas J Diagnosis and management of lateral semicircular canal conversions during particle repositioning therapy Laryngoscope 115(10) 1895-1897 2005
ndash Acute Vestibular Syndrome Hotson JR Baloh RW N Engl J Med 1998 Sep 3339(10)680-5
ndash Methylprednisolone valacyclovir or the combination for vestibular neuritis Strupp M Zingler VC Arbusow V Niklas D Maag KP Dieterich M Bense S Theil D Jahn K Brandt T N Engl J Med 2004 Jul 22351(4)354-61
ndash Intratympanic steroids for Meacuteniegraveres disease or syndrome Phillips JS Westerberg BCochrane Database Syst Rev 2011 Jul 6(7)CD008514 Review
ndash Sound- andor pressure-induced vertigo due to bone dehiscence of the superior semicircular canal Minor LB Solomon D Zinreich JS Zee DSArch Otolaryngol Head Neck Surg 1998 Mar124(3)249-58