Meniere's diseasePrepared by Dr. saef moniemS.H.O O.R.L-H.N.S2012

Scott-Brown's ORL-HNS7th edition

G Michael Halmagyi MD FRACP Clinical Professor Department of Neurology Royal Prince Alfred Hospital Sydney, Australia

Ian S Curthoys PhD Emeritus Professor School of Psychology University of Sydney Sydney, Australia

Cummings OtolaryngologyHead & Neck SurgeryFIFTH EDITION

Benjamin T. Crane, M.D., Ph.D.Assistant ProfessorDepartments of Otolaryngology and NeurobiologyUniversity of Rochester School of Medicine and DentistryDepartment of OtolaryngologyStrong Memorial HospitalRochester, New York

David A. Schessel, M.D.Assistant ProfessorDepartment of OtolaryngologyStony Brook University Medical Center School of MedicineStony Brook, New York

definitionMeniers disease

o It’s an idiopathic endolymphatic hydrops & a disorder of the inner ear.

o is one of the PERIPHERAL VESTIBULOPATHIES characterized by spontanous attacks of vertigo with associated SNHL ,tinitus & aural fullness.

o Meniere’s Disease was first described by Prosper Meniere in 1861

o Note :The triad of hearing loss, tinnitus, and vertigo constitutes Meniere’s syndrome. If the cause is unknown, it is defined as Meniere’s disease.

Aeteology & pathogenesis• overproduction or malabsorption of endolymph, result in endolymphatic

hypertension.

• Endolymphatic hydrops leads to distortion of membranous labyrinth

• Reisner’s membrane can be seen bulging into the scala vestibuli in some histologic studies

• Microruptures may lead to episodic attacks which resolve when the tears heal

delayed endolymphatic hydrops can occure after many years in• syphilis• mumps, • Cogan's syndrome, • trauma and even • chronic suppurative otitis media,

This produce the clinical picture of Meniere's disease - so-called Meniere's syndrome.

Sometimes the attacks of vertigo can occuer in the other, previously normal, ear this is known as contralateral delayed endolymphatic hydrops, which is thought to be due to the development of autoimmunity to inner ear antigens.

So Meniere’s disease is precipitated by a variety of events such as o autoimmune, o viral, o traumatic, or o vascular or o ischemic and may even be the result of o congenital anatomic and o molecular variations that may act as triggers for the later development of symptomatic

hydrops

Incidenceo Wide variation exists in the published rates for incidence of Meniere’s

disease. o from 17 per 100,000 in the Japanese population to 513 per 100,000 in the

population of southern Finland,o more prevalent among whites,o male-to-female ratio of approximately 1.o The peak age at onset is in the fourth and fifth decades of life, presentation

can be at almost any age.o The frequency of bilateral disease is of 19% to 24%.o The rate depends on the duration of follow-up. o Symptoms of bilateral disease may appear many years or decades after

onset of the unilateral symptoms.o Familial occurrence of Meniere’s disease has been reported in 10% to 20%

of cases.o An autosomal dominant mode of inheritance has been suggested,o Migraine is strongly associated with Meniere’s disease in familial cases. o Human leukocyte antigens (HLA) B8/DR3 and Cw7 have been associated

with Meniere’s disease.

CLINICAL MANIFESTATIONSPatients with Meniere's disease have

spontaneous vertigo (96.2%) , with associated nausea and vomiting tinnitus (91.1%) ipsilateral hearing loss (87.7%) often are preceded by an aura consisting of a sense of fullness in the ear,

o Following its onset, the vertigo typically increases in intensity over a period of minutes and then usually lasts for several hours.

o Attacks lasting longer than a day are unusual and if present should cast doubt on the diagnosis

o The hearing loss and tinnitus, on the other hand, may persist for days. Occasionally, hearing improves temporarily after an attack.

o Patients may have continuing postural imbalance and nausea that lasts for several days after the attack has abated.

o Accompanying symptoms and signs may include diarrhea, and sweating.

o The hallmark of Meniere’s disease is variability and unpredictabil ity, and this holds true for the hearing loss associated with the disease

A typical attack has three phases, each defined by the direction of the spontaneous nystagmus.

• the nystagmus, usually horizontal or horizontal-torsional, • beats towards the affected ear; • this phase usually lasts less than an hour.

The first is the irritative phase

• the nystagmus beats away from the affected ear; • this phase lasts several hours, sometimes even a day or

two.

The second is the paretic phase,

• the nystagmus again beats towards the affected side; • this phase lasts about as long as the second phase.

The third is the recovery phase,

Hearing Loss and Tinnitus

The SNHL in Meniere’s disease typically is fluctuating and progressive. It often occurs with the sensation of fullness or pressure in the ear. A pattern of low-frequency fluctuating loss and a coincident nonchanging, high-

frequency loss is described, a “peaked” or “tent-like” tracing on the audiogram is obtained. This peak classically occurs at 2 kHz. Over time, the hearing loss normalizes across frequencies and becomes less variable. In only 1% to 2% the hearing loss progress to profound deafness. Additional features include

Diplacusis (43.6%) , a difference in the perception of pitch between the ears recruitment (56%).

Tinnitus tends to be nonpulsatile described as whistling or roaring. It may be continuous or intermittent. Tinnitus often begins, gets louder, or changes pitch as an attack approaches. Frequently a period of improvement follows the attack.

Audiogram in Meniere's disease. This patient, a 14-year-old female, began to have attacks of acute spontaneous vertigo with right-sided aural fullness and then tinnitus. (a) Audiogram on presentation shows a 60 dB low-frequency Hearing loss with normal acoustic reflexes (Z), indicating a fully recruiting cochlear hearing loss. (b) Audiogram after four months of treatment with a rigorous low-sodium diet shows normal pure-tone thresholds.

o In the early stages of the disease, most patients are well between attacks.

o As the disease progresses, persistent hearing loss, tinnitus and postural imbalance persist.

o Some patients, particularly those in the later stages of the disease, develop drop attacks

(Tumarkin or otolithic crises),

o recurrent vestibulopathy and atypical Meniere’s disease describe the condition on persons

with less than the classic triad of hearing loss, vertigo, and sensation of aural fullness or tinnitus.

Tumarkin or otolithic criseso are thought to occur as a result of acute otolithic dysfunction. o The patient simply drops to the ground without warning and can sustain a fracture or other serious

injury. o The patient may describe a sensation of being pushed to the ground by some external force during

these attacks. o There is no associated vertigo or loss of consciousness. o Some patients may have only one or two of these attacks during the course of their illness, while

others have repeated attacks.

Lermoyez syndrome o described an unusual clinical presentation in which tinnitus and hearing loss precede the

onset of vertigo. o When the vertiginous episode occurs, the tinnitus and hearing loss dramatically resolve. o The temporal bone studies in a patient with such attacks noted hydrops and membrane

ruptures isolated to the basal turns of the cochlea and the saccule.

Diagnosis

No single test is available to make the diagnosis of Meniere’s disease.

the most important aspect of the diagnostic process is a complete history,

including a detailed description of the pattern of disease presentation,

supported by quantitative testing.

The most recent definition of the disease has been established by the (AAO-

HNS)

AAO-HNS Criteria for Meniere’s Disease Diagnosis

Major SymptomsVertigo• Recurrent, well-defined episodes of spinning or rotation• Duration from 20 minutes to 24 hours.• Nystagmus associated with attacks• Nausea and vomiting during vertigo• No neurologic symptoms with vertigoDeafness• Hearing deficits fluctuate• Sensorineural hearing loss• Hearing loss progressive, usually unilateralTinnitus• Variable, often low-pitched and louder during attacks• Usually unilateral• Subjective

Diagnosis

Possible Meniere’s disease• Episodic vertigo without hearing loss or• Sensorineural hearing loss, fluctuating or fixed, with dysequilibrium, but without definite episodes• Other causes excludedProbable Meniere’s disease• One definitive episode of vertigo• Hearing loss documented by audiogram at least once• Tinnitus or sense of aural fullness in the presumed affected ear• Other causes excludedDefinite Meniere’s disease• Two or more definitive spontaneous episodes of vertigo lasting at least 20 minutes• Audiometrically documented hearing loss on at least one occasion• Tinnitus or sense of aural fullness in the presumed affected ear• Other causes excluded Certain Meniere’s disease• Definite Meniere’s disease, plus histopathologic confirmation

Investigations

The caloric test The caloric test often can localize the involved ear.

o A significant caloric response reduction is found in 48% to 73.5% of patients with Meniere’s disease.

o Complete absence of caloric response is reported in 6% to 11% of patients.Electrocochleography

o The summating potential (SP) is larger and more negative. o The most commonly used value is the ratio of amplitudes of the SP and the

eighth cranial nerve action potential (AP), the SP/AP ratio .o It is not a definitive test, because ratios are elevated in 62% of patients with

Meniere’s disease and in 21% of control subjects. o It is most sensitive and specific for Meniere's disease when tone-burst and

click stimuli are usedo Giving patients 4 g of oral sodium chloride for three days prior to the

electrocochleogram may increase the sensitivity of the test.

Treatmento Therapy of Meniere’s disease is aimed at the reduction of its associated

symptoms. o Currently, almost all proven therapies are directed at relieving vertigo, which

usually is the most distressing symptom.

Dietary Modification and Diuretics• Salt restriction and diuresis may be the best initial therapy for Meniere’s disease.• Despite the popularity of these treatments, neither salt restriction nor use of

diuretics has had its efficacy confirmed by double-blind, placebo-controlled studies.

• Carbonic anhydrase inhibitors such as acetazolamide were recommended on the basis of the localization of carbonic anhydrase to the dark cells and the stria vascularis.

• However, these agents have not proved to be clinically more effective than other diuretics.

• Despite the lack of hard evidence of their efficacy, a low-salt diet and diuresis

constitute an appropriate and effective treatment for Meniere’s disease with a low risk of side effects

Vasodilatorso Betahistine is an oral preparation of histamine.o It has proved to be effective in treatment of Meniere’s disease in a placebo-

controlled study.

Symptomatic Treatmento Antivertiginous medications, antiemetics, sedatives, antidepressants, and psychiatric

treatment have been reported to be beneficial in reducing the severity of the vertigo and vegetative symptoms and in improving tolerance of Meniere’s symptoms.

Local Overpressure Therapyo A relatively recent approach to decrease hydrops by pulsing pressure in the

middle ear. o overpressure in the middle ear was reported to decrease Meniere’s symptoms

during acute vertigo attacks.o Since 2000, the Meniett device has been approved for use by the U.S. Food and

Drug Administration (FDA). o The pressure is delivered in complex pulses of up to 20 cm of water, over a 5

minute period. o A randomized controlled trial demonstrated that patients using the Meniett

device experienced a significant decrease in vertigo symptoms for the first 3 months of therapy

o Long-term treatment with the Meniett device has shown results similar to the natural course of Meniere’s disease.

o Note : simple placement of a ventilation tube with no additional therapy also has been reported to control vertigo symptoms

Intratympanic Injection

• The term chemical labyrinthectomy often is applied to intratympanic gentamicin treatment, • Gentamicin has a vestibulotoxicity that is high relative to its cochleotoxicity; • The gentamicin can be administered through either a tympanostomy tube or directly

injected through the tympanic membrane. • Peripheral vestibular deficits are evident on head thrust testing after even a single dose of

gentamicin• The concentration of the medication used and frequency of injection vary by series. • reported elmination of vertigo in 90% of 92 patients.• The risk of hearing loss with gentamicin using many current protocols is similar to that with

the natural history of Meniere’s disease.• Intratympanic injection of dexamethasone is a reasonable procedure to offer when vertigo is

intractable• The mechanism for steroid effect on vertigo symptoms is not currently clear. • the effect on hearing loss and tinnitus is minimal. The risk of hearing loss appears to be low. • Dexamethasone injections may need to be repeated every 3 months to maintain the patient

free of vertigo symptoms. • Concentrations used have varied from 2 to 24 mg/mL.

Surgical treatmentsEndolymphatic sac surgery

• may result in a reduction in the frequency, duration and intensity of vertigo attacks.

• it is not always effective in stopping the vertigo attacks • no benefits for the auditory symptoms.

Selective vestibular neurectomy • effective in stopping vertigo attacks in most patients • hearing loss and facial paralysis is potential complications.

surgical labyrinthectomy • can stop the vertigo attacks at the expense of losing any

remaining hearing on that side.

Those whose hearing is below the reach of standard hearing aids may be

helped by cochlear implantation.

OUTCOMES AND COMPLICATIONS

The natural history of Meniere's disease is variable.

Attacks may occur days, months or even years apart

Some patients have a single bout of attacks lasting only a few months and never

develop any permanent loss of auditory or vestibular function.

Others have progressive course and continue to have vertigo attacks, along with

continual tinnitus and no useful hearing in one ear.

Others still are fortunate and have no further vertigo attacks and little tinnitus

- so- called burnt-out Meniere's disease.

In most patients, only one ear is affected in the early stages of Meniere's

disease.

Unfortunately, the second ear eventually becomes involved in about half of the

patients

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