Implications%20of%20Auditory%20Neuropathy%20for%20EHDI%20Programs

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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

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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.

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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.

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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

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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

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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.

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Variability

• Progressive loss of peripheral auditory function
  (e.g. loss of OAEs, CM)
• Stable
• Worsen
• Partial recovery

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Historical Perspective

• 1980s first published accounts of normal
  hearing and absent ABRs
• Davis and Hirsh, Worthington and Peters
• 1984 Kraus et.al reported 1.3 of the children
  with hearing loss had absent ABRs
• Addition of OAE increased the diagnostic
  capabilities

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Incidence?

• Davis and Hirsh .5
• Berlin 12
• Kraus 1.3
• Rance - 12-14 of the severe to profound

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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 gt 38 weeks gestation
• Colorado study

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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
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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

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Increasing Identification Rate for AN/AD
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AN/AD Incidence
2003 2004 Combined (20032004)
Cases Identified 8 9 17
Total Screened 67,778 66, 567 134,345
Incidence rate 1.1810,000 1.3510,000 1.2710,000
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Hispanic Incidence Rates
2003 2004 Combined (20032004)
Cases Identified 3 3 6
Total Screened 21,533 21,582 43,115
Incidence rate 1.3910,000 1.3910,000 1.3910,000
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Age of Identification
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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

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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
  Fredreichs Ataxia)
• Hereditary recessive and dominate

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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

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RECOMMENDED PROTOCOL FOR INFANT AUDIOLOGIC
ASSESSMENTTHE COLORADO INFANT AUDIOLOGIC
ASSESSMENT TASK FORCE
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AUDIOLOGIC DIAGNOSTIC ASSESSMENT

• ABR
• Otoscopic
• Acoustic immittance (high frequency probe)
• TEOAE /or DPOAE
• BOA

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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

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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

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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

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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.

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Why Behavioral Testing?

• Behavioral tests are the only true tests of
  hearing (Sininger, 1993 cited in Hicks,Tharpe
  Ashmead, 2000 )
• permits observation of the infants auditory
  development
• demonstrates auditory behaviors to parents and
  caregivers

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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)

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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!!!

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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.

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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

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Developing a Treatment Program for Children with
Auditory Neuropathy

• Arlene Stredler Brown, CCC-SLP, CED

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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

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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
  

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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

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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

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Developing an Action Plan..

• Assess at regular intervals to monitor
  achievement
• Baserate data
• Rate of progress
• Maintain development commensurate with cognitive
  age

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Trends in Successful Treatment

• Visual communication
• Speechreading
• English-based signs
• Cued Speech (receptive vs. expressive)
• Cochlear Implants

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Cautions in Treatment Methods

• Amplification (according to some)
• American Sign Language (ASL)
• Auditory-Verbal therapy

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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

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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

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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

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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 childs cognitive skills

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Creating a Functional Developmental Profile

• Types of assessment
• Parent/caregiver report
• direct observation of the child
• Observation of childs interaction with a parent
• videotaped interaction
• Clinician-administered assessments
• Multi-disciplinary all developmental domains

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Developmental Domains to Assess

• Cognitive skills
• Functional Auditory Skills
• Communication Skills
• Gesture
• communication intention
• facial expression
• turn-taking
• Vocalizations

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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

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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)

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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

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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.

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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

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Qualities to look for in an Interventionist/Therap
ist

• 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

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Final Note!

• Reminder.
• Do not change method randomly even after CI