Implications of Auditory Neuropathy for EHDI Programs Vickie Thomson MA Sandra Gabbard, PhD Arlene...
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Transcript of Implications of Auditory Neuropathy for EHDI Programs Vickie Thomson MA Sandra Gabbard, PhD Arlene...
Implications of Auditory Neuropathy for EHDI Programs
Vickie Thomson MA
Sandra Gabbard, PhD
Arlene Stredler Brown, MA
Marion Downs Hearing Center
Denver, CO
Faculty Disclosure Information
In the past 12 months, I have not had a significant financial interest
or other relationship with the manufacturer(s) of the product(s) orprovider(s) of the service(s) that will be discussed in my
presentation.
This presentation will (not) include discussion of pharmaceuticals or
devices that have not been approved by the FDA or if you will bediscussing unapproved or "off-label" uses of pharmaceuticals or
devices.
Contributing Researchers
Yvonne Sininger, Ph.D.Arnold Starr, M.D.Linda Hood, Ph.D.Charles Berlin, Ph.D.Lazlo Stein, Ph.D.Jon Shallop, Ph.D.Gary Rance, MSc.
Auditory Neuropathy vs Auditory Dys-synchrony
Berlin, Hood and Rose coined the term “dys-synchrony” to provide a more comprehensive view of auditory neuropathy
The auditory nerve may not be affectedAN may lend towards not considering
cochlear implants as an option
What is AN/AD?
Abnormal auditory brainstem responsesNormal otoacoustic emissionsNormal outer hair cell functionAbnormal neural functionNo acoustic reflexesLarge cochlear microphonic
What is AN/AD?
Speech perception worse than expected based on the audiogram
Inconsistent responses to sound Worse in noise Fluctuating hearing loss If a maturational problem recovery may return
by 12-18 months Perceptual ability may improve although ABR
remains abnormal.
Variability
Progressive loss of peripheral auditory function (e.g. loss of OAE’s, CM)
StableWorsen Partial recovery
Historical Perspective
1980’s first published accounts of ‘normal’ hearing and absent ABR’s• Davis and Hirsh, Worthington and Peters
• 1984 Kraus et.al reported 1.3% of the children with hearing loss had absent ABR’s
• Addition of OAE increased the diagnostic capabilities
Incidence?
Davis and Hirsh – .5%Berlin – 12%Kraus – 1.3%Rance - 12-14% of the severe to
profound
NICU vs. Well Baby
Rance reported that 85% were NICU graduates
Berg et al reported 24% of a NICU cohort had AN/AD profiles (23% were bilateral)39% > 38 weeks gestation
Colorado study
Colorado Screening Rates
Year # of Births
# Screened
# Referred
Confirmed hearing loss
2003 68957 66567 (97%)
2997/4.5% 139
2004 69801 67697 (97%)
2368/3.3% 143
Methods
Data from both the Colorado Department of Public Health and Environment and the Colorado Home Intervention Program was analyzed.
All hearing losses were identified by local audiologists and reported to these agencies
Increasing Identification Rate for AN/AD
AN/AD Incidence
2003 2004 Combined (2003&2004)
Cases Identified
8 9 17
Total Screened
67,778 66, 567 134,345
Incidence rate
1.18:10,000 1.35:10,000 1.27:10,000
Hispanic Incidence Rates
2003 2004 Combined (2003&2004)
Cases Identified
3 3 6
Total Screened
21,533 21,582 43,115
Incidence rate
1.39:10,000 1.39:10,000 1.39:10,000
Age of Identification
Risk Factors
15 of 20 (75%) bilateral AN/AD cases were admitted to the NICU
7 of 20 (35%) bilateral AN/AD cases were premature births
Associated Risk Factors
AnoxiaHyperbilirubinemiaInfectious diseases (e.g. mumps)Immune disorders (e.g. Guillain-Barre
syndrome)Genetic Syndromes (e.g. Charcot-Marie-
Tooth and Fredreich’s Ataxia)Hereditary recessive and dominate
Recommendations for Screening
Use AABR in the NICURecognize that AN/AD is present in ‘well
baby infants’Educate medical homes about the
importance for referring whenever there is a concern regardless of the screening outcome
RECOMMENDED PROTOCOL FOR INFANT AUDIOLOGIC
ASSESSMENT
THE COLORADO INFANT AUDIOLOGIC ASSESSMENT TASK FORCE
AUDIOLOGIC DIAGNOSTIC ASSESSMENT
ABROtoscopic Acoustic immittance (high
frequency probe)TEOAE &/or DPOAEBOA
ABR Assessment
• Threshold search to clicks in 10 dB steps
• If NR, compare rarefaction & condensation click response (auditory neuropathy)
• Threshold search to 500 & 3000 Hz tone pip (or ASSR)
• Threshold search to clicks by bone conduction
Issues in Infant ABR Assessment
Always look for cochlear microphonic when neural response is abnormal or absent (Auditory Neuropathy)
Must have frequency specific thresholds (tones or ASSR)
Bone Conduction may be useful Placement of oscillator Calibration Head band versus hand held position
ASSR and Behavioral Thresholds
In general, ASSR thresholds are within 20 dB of behavioral thresholds
Largest discrepancies when hearing is normal
Best correlated for severe to profound hearing losses
Differences greatest in the low frequencies
Aoyagi et al, 1994, Levi et al, 1995, Rance et al, 1995, Lins et al, 1996, Picton et al, 1998
Otoacoustic Emissions
Sound produced by Outer Hair Cell movement in response to a stimulus
Evoked Emissions Distortion Product (DPOAE) Transient Evoked (TEOAE)
Spontaneous (SOAE) Present for hearing better than
approximately 35 dB with normal middle ear function.
Why Behavioral Testing?
• Behavioral tests are the only “true” tests of hearing (Sininger, 1993 cited in Hicks,Tharpe & Ashmead, 2000 )
• permits observation of the infant’s auditory development
• demonstrates auditory behaviors to parents and caregivers
Why Behavioral Testing?
• Behavioral tests serve as “cross-checks” of physiologic measures (Jerger & Hayes, 1976)• confirms audiometric configuration
(OAE; ABR)• determines presence of conductive
component (ABR; immittance measures)• confirms threshold predictions (ABR)
Recommendation for Behavioral Assessment
• Use age appropriate techniques and
use child's developmental level. • Use insert phones when possible.• Use audiologist in room with child.• Use quiet distracting toys.• Use multiple reinforces to keep
attention.• Use a variety of interesting stimuli.• Always include as part of test battery!!!
Recommendations for Middle Ear Assessment
• Do not rely of 226-Hz tympanometry in infant under 6 months of age.
• Between 4-6 months, it appears that 226-Hz tympanograms begin to be effective for detection of MEE.
• For ages birth to 6 months, use a higher probe frequency (800-1000 Hz), with criteria of any discernable peak within normal range.
• Correlate results with other diagnostic measures.
Medical testing
Genetic testing Ophthalmologic evaluation by 12 months of
age CMV titers- test ASAP after birth FTA-ABS EKG (Jervell and Lange-Neilson Syndrome) CT/MRI
Cochlear dysplasia/large vestibular aqueduct syndrome
Cochlear ossification following meningitis
Developing a Treatment Program Developing a Treatment Program for Children with Auditory for Children with Auditory NeuropathyNeuropathy
Arlene Stredler Brown, CCC-SLP, CED
What do we know?
Diagnosis is difficult for parents to understand
Course of the condition is unpredictable
The greatest need is to monitor language development and auditory development in order to develop an appropriate treatment plan
What is difficult for parents?
Feeling helplessWaiting to reach a definitive diagnosis Variability in skills among childrenIdentifying a communication method Finding comfort in making choices
that may change
Developing an Action Plan.. Helping parents during the diagnostic
process Specific audiologic battery
Helping parents to locate treatment Information, support, navigating the Part C
system, the EHDI system, and other early childhood initiatives
Developing an Action Plan..
Developing a unique intervention program Identify the functional profile of the childAssessment in a variety of developmental
domainsCommunicationLanguageFunctional auditory skillsSpeechCognition
Developing an Action Plan..
Assess at regular intervals to monitor achievementBaserate dataRate of progressMaintain development commensurate with
cognitive age
Trends in Successful Treatment
Visual communicationSpeechreadingEnglish-based signsCued Speech (receptive vs. expressive)
Cochlear Implants
Cautions in Treatment Methods
Amplification (according to some)American Sign Language (ASL)Auditory-Verbal therapy
Creating a Profile of Functional Auditory Skill Development
Expect auditory behaviors that are not hierarchical
Monitor for changes in auditory behavior (may become more systematic)
Document listening in a variety of conditions Quality of responses to auditory stimuli Identify conditions when the child responds Identify consistency of responses Look for variability Aided vs. unaided
Auditory Skill Development
Monitor with trial amplification Awareness vs. speech discrimination Parents’ desire to be pro-active Caution regarding power of amplification
With a cochlear implant, expect hierarchical auditory skill development Allow time for spontaneous recovery Monitor development of speech & language Identify auditory discrimination skills vs. pure tone
hearing levels Determine site of lesion
Tools to Measure Functional Auditory Skill Development
Functional Auditory Performance Indicators (FAPI) – Stredler-Brown & Johnson
Auditory-Verbal Ages & Stages of Development - Estabrooks
The Developmental Approach to Successful Listening II (DASL) – Stout & Windle
The Development of Listening Function - Razack
Creating a Functional Developmental Profile
Assess at regular intervals IFSP recommends every six months
Expect developmental gains at a rate that is commensurate with that child’s cognitive skills
Creating a Functional Developmental Profile
Types of assessment Parent/caregiver reportdirect observation of the child Observation of child’s interaction with a
parent videotaped interaction Clinician-administered assessments
Multi-disciplinary – all developmental domains
Developmental Domains to Assess
Cognitive skillsFunctional Auditory SkillsCommunication Skills
Gesturecommunication intention facial expression turn-takingVocalizations
Developmental Domains to Assess
Language Skills (receptive & expressive) Language areas
Semantics Syntax Pragmatics
Skill areas: Imitation Initiation of communication Production of sounds, words, sentences
Modalities Visual: Speechreading, sign language Auditory Multiple modes
Developmental Domains to Assess
Speech Development number of utterances quality of utterances
Spontaneous condition Spontaneous imitation Prompted imitation
inventory of specific phonemes Vowels Consonants
Non-true words and true words Speech intelligibility for true words
Subjective Objective (e.g., LIPP)
Considerations When Choosing a Method Options Purpose/goals
Develop languageDevelop language Develop EnglishDevelop English Potential to develop speechPotential to develop speech
Evaluate what is available in the schools Do Do notnot limit choice based on availability limit choice based on availability Prepare local school district to offer Prepare local school district to offer
instruction using the method you have instruction using the method you have chosenchosen
A TEAM EFFORT…A TEAM EFFORT…
It is to be hoped that, in the future, intervention and education for children with auditory
neuropathy will be more prescriptive. At this time, however, professionals have the
responsibility to work as a team, to identify the developmental profile of each child in an effort to identify appropriate intervention strategies. And, as professionals, we have a responsibility to remain committed to the method or methodology that works for each child.
Identifying the Team Audiologist
diagnosing the condition monitoring the course of the condition monitoring the use of amplification recommending candidacy for a cochlear implant
ENT/Otologist Early Interventionist: Educator of the D/HH,
SLP, Educational Audiologist Experienced parents Other physicians
Qualities to look for in an Interventionist/Therapist
Skilled in a variety of modes/communication methods
supports options knows parent-centered intervention paradigm knowledgeable about auditory training
techniques knows specific visual communication
techniques; advantages & challenges Cued SpeechCued Speech Sign language/s: MCE, CASE, PSE, ASLSign language/s: MCE, CASE, PSE, ASL SpeechreadingSpeechreading
Final Note!
Reminder….Do not change method randomly – even
after CI