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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

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implications of auditory neuropathy for ehdi programs
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
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) or

provider(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 be

discussing unapproved or "off-label" uses of pharmaceuticals or devices.

contributing researchers
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
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 affected
  • AN may lend towards not considering cochlear implants as an option
what is an ad
What is AN/AD?
  • Abnormal auditory brainstem responses
  • Normal otoacoustic emissions
  • Normal outer hair cell function
  • Abnormal neural function
  • No acoustic reflexes
  • Large cochlear microphonic
what is an ad6
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.
  • Progressive loss of peripheral auditory function (e.g. loss of OAE’s, CM)
  • Stable
  • Worsen
  • Partial recovery
historical perspective
Historical Perspective
  • 1980’s first published accounts of ‘normal’ hearing and absent ABR’s
    • Davis and Hirsh, Worthington and Peters
  • 1984 Kraus reported 1.3% of the children with hearing loss had absent ABR’s
  • Addition of OAE increased the diagnostic capabilities
  • Davis and Hirsh – .5%
  • Berlin – 12%
  • Kraus – 1.3%
  • Rance - 12-14% of the severe to profound
nicu vs well baby
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
  • 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
risk factors
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
Associated Risk Factors
  • Anoxia
  • Hyperbilirubinemia
  • Infectious 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
Recommendations for Screening
  • Use AABR in the NICU
  • Recognize 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
audiologic diagnostic assessment
  • ABR
  • Otoscopic
  • Acoustic immittance (high frequency probe)
  • TEOAE &/or DPOAE
  • BOA
abr assessment
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
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
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
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
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 testing27
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
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
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
Medical testing
  • Genetic testing
  • Ophthalmologic evaluation by 12 months of age
  • CMV titers- test ASAP after birth
  • EKG (Jervell and Lange-Neilson Syndrome)
  • CT/MRI
    • Cochlear dysplasia/large vestibular aqueduct syndrome
    • Cochlear ossification following meningitis
developing a treatment program for children with auditory neuropathy

Developing a Treatment Program for Children with Auditory Neuropathy

Arlene Stredler Brown, CCC-SLP, CED

what do we know
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
What is difficult for parents?
  • Feeling helpless
  • Waiting to reach a definitive diagnosis
  • Variability in skills among children
  • Identifying a communication method
  • Finding comfort in making choices that may change
developing an action plan
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 plan35
Developing an Action Plan..
  • Developing a unique intervention program
    • Identify the functional profile of the child
    • Assessment in a variety of developmental domains
      • Communication
      • Language
      • Functional auditory skills
      • Speech
      • Cognition
developing an action plan36
Developing an Action Plan..
  • Assess at regular intervals to monitor achievement
    • Baserate data
    • Rate of progress
    • Maintain development commensurate with cognitive age
trends in successful treatment
Trends in Successful Treatment
  • Visual communication
    • Speechreading
    • English-based signs
    • Cued Speech (receptive vs. expressive)
  • Cochlear Implants
cautions in treatment methods
Cautions in Treatment Methods
  • Amplification (according to some)
  • American Sign Language (ASL)
  • Auditory-Verbal therapy
creating a profile of functional auditory skill development
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
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
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
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 profile43
Creating a Functional Developmental Profile
  • Types of assessment
    • Parent/caregiver report
    • direct 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
Developmental Domains to Assess
  • Cognitive skills
  • Functional Auditory Skills
  • Communication Skills
    • Gesture
    • communication intention
    • facial expression
    • turn-taking
    • Vocalizations
developmental domains to assess45
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 assess46
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
Considerations When Choosing a Method
  • Options
  • Purpose/goals
    • Develop language
    • Develop English
    • Potential to develop speech
  • Evaluate what is available in the schools
    • Do not limit choice based on availability
    • Prepare local school district to offer instruction using the method you have chosen
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
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
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 Speech
    • Sign language/s: MCE, CASE, PSE, ASL
    • Speechreading
final note
Final Note!
  • Reminder….
    • Do not change method randomly – even after CI