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Fostering auditory development of a child with auditory neuropathy


Fostering auditory development of a child with auditory neuropathy. Holly Gilliam ... Pure tone audiogram becomes important to determine what the child hears. ... – PowerPoint PPT presentation

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Title: Fostering auditory development of a child with auditory neuropathy

Fostering auditory development of a child with
auditory neuropathy
  • Holly Gilliam
  • Yusnita Weirather, M.A., CCC-A
  • Deborah Gabe, M.A., CCC-A
  • March 4, 2005

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  • Auditory neuropathy
  • Auditory dyssynchrony
  • Nonperipheral hearing loss
  • Hearing loss beyond the cochlea
  • Sensori-neural hearing loss
  • Unconventional hearing loss

What is auditory dys-synchrony?
  • Hearing impairment is a disorder of auditory
    nerve function and may have, as one of its
    causes, a neuropathy of the auditory nerve,
    occurring either in isolation or as part of a
    generalized neuropathic process (Starr et al,
    Brain, 119 (3) 741)
  • A hearing disorder in which sounds enters the
    inner ear normally but the transmission of
    signals from the inner ear to the brain is
    impaired (NIDCD).
  • A type of hearing loss where the cochlea seems to
    work, but there is something wrong with how the
    auditory nerve works (Deafness and Family
    Communication Center (DFCC) at the Children's
    Hospital of Philadelphia).

Aidans mom
  • I have read that people with AN can hear in a
    number of ways. Some hear sound, but not clearly
    enough to distinguish speech. Some have moments
    of clarity and moments of dysynchrony. And Ive
    even heard of someone whose child could hear
    relatively clearly for months at a time and then
    lose that clarity again. That is part of what is
    so frustrating to me as a new AN mom - there
    isnt any definitive answer to what my child is

Audiological test results
  • Presence of cochlear hair cell activity
  • - cochlear microphonics in ABR
  • - otoacoustic emissions (may later
  • Absence of auditory nerve response
  • - no ABR wave forms observed
  • Behavioral audiometric test results vary
  • - fluctuating responses
  • - audiograms range from normal to profound

Clinical manifestations
  • Ability to detect sounds and speech cannot be
    predicted by ABR results.
  • Pure tone audiogram becomes important to
    determine what the child hears.
  • Word recognition ability is worse than predicted
    by the pure tone audiogram.
  • Functional hearing ability changes and cannot be
    predicted and occasionally, in the long term,
    hearing may improve
  • Hearing aid benefits are not uniform across cases

Zacharys audiological profile
  • Failure of bilateral newborn hearing screening
    with ABR
  • Absent auditory brainstem responses to click
    stimuli at a 90 dBeHL presentation level.
  • Robust Otoacoustic Emissions as measured by
    Transient Evoked technology. Emissions
    eventually disappear by the fourth audiological
    evaluation (6 months of age)

Normal ABR
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Zacharys medical history
  • Born at 33 weeks gestational age
  • 3 Lb 15 oz
  • Esophageal atresia type A (a congenital disorder
    of the digestive system where the esophagus does
    not fully develop). He was hospitalized for 3
    months after birth. Repaired on April 21, 2002.
  • Jaundice and possible sepsis

Audiological management
  • 9 months of age Binaural analog hearing aid
    which he disliked and did not want to retain
    longer than his parents effort to put them in.
  • FM system in conjunction with hearing aid did not
    provide much difference in his functional
  • 2 years of age Changed to binaural digital
    hearing aids which he loves and uses daily.
    Significant progress was observed in his speech
    and his excitement in hearing sounds. He would
    cup his hand to his ear and sign what is that?
  • Auditory training / aural rehabilitation once
    per week until 2 years of age and increased to
    twice a week after that.

Developmental intervention
  • 7 months
  • Cognitive / Social
  • Is more independent (not crying if mom leaves the
    room for a few minutes).
  • Explores his environment or objects by reaching
    for objects and finding hidden objects through
  • Gross motor sits independently, bangs cups
    together, rakes tiny objects.
  • Self Help Is able to finger feed himself, and
    drink from a cup with adult support.
  • Communication / speech
  • Increases eye contact communicates his needs
    through signing, gesturing or verbalizing
    responds to environmental sounds and speech by
    turning his head or vocalizing and is able to
    say mama and dada.
  • Increases vocalizations

Developmental intervention
  • 1 year and 18 months
  • Cognitive / Social
  • Finds objects and is able to play by himself
  • Points to body part
  • Increases attention span
  • Eliminates hand banging and self injurious
    behavior when frustrated
  • Gross motor stands up, holds himself.
  • Self Help Feeds himself using utensils, is able
    to drink from a cup with minimal spillage, is
    introduced to toilet training.
  • Communication / speech
  • Increases tolerance to hearing aids.
  • Uses 1 - 3 signs to express his needs, and is
    able to say mama and dada.
  • Increases vocalization
  • Parents train in communication using ASL and oral
  • Increases imitation skills

Developmental intervention
  • 2 years
  • Cognitive / Social
  • Masters pre writing skill
  • Is able to complete puzzles
  • Recognizes letters and develops pre literacy
  • Gross motor Jumps forward.
  • Self Help Sits at a table during meal, is toilet
    trained, can eat a variety of foods.
  • Communication / speech
  • Increases accurate production of age appropriate
    sounds (p, b, m, n, d, t, k, g, w, h, j)
  • Increases vocabulary
  • Use 2-3 words phrases spontaneously to request,
    comment and answer questions
  • Understands preposition

2 philosophies of how to teach infants and
children with auditory neuropathy to communicate
  • Use of sign language as the child's first
  • Use of listening skills and skills in spoken
    English together with technologies such as
    hearing aids and cochlear implants
  • A combination of these two approaches

Aural rehabilitation / auditory training
  • Age appropriate auditory and communication skills
  • Wearss hearing aids consistently during his
    waking hours
  • Discriminates speech sounds in words, phrases and
    sentences through audition only
  • Is able to perform play audiometric testing
  • Parental partnership and involvement
  • Understand issues related to auditory neuropathy,
    options in communication, early literacy skills,
    deaf mentorship, auditory development, auditory
    stimulation activities and fostering of oral
  • Increase skills in ASL and become involved in a
    shared reading program

  • Full active participation in audiological
    evaluation, management and early intervention
  • Proficiency in ASL by self study and formal
  • Participation in a deaf mentor pilot project
  • Careful monitoring of Zacharys sign and oral
    language development
  • Open mindedness to new ideas
  • Family participation in all of Zacharys first
    appointments and Saturday appointments
  • Creativity in developing new family activities to
    support Zacharys needs.

Latest performance
  • Has a desire to learn is outgoing, a risk taker,
    determined, smart, and affectionate has a great
    memory and positive behaviors best displayed in a
    structured environment
  • Is comfortable with his hearing aids thoughout
    the day
  • Uses ASL and spoken language to join 2 or 3
  • Counts to 5
  • Fingerspells a manual alphabet
  • Is age appropriate for social, motor and self
    help skills.
  • Is slightly below his age level for expressive
    language and mathematical concepts.

Family Belief
Children are apt to live up to what you believe
of them
Family - Professional Partnership
  • Tailored base of learning at home and elsewhere.
  • Periodic readjustment of management.
  • Determination of childs strength and weaknesses.
  • Creation of and dedication to the same goal

How Hard of Hearing Children Learn to Talk by
Nancy Rushmer
  • They learn to talk through listening with their
    hearing aids. Because hearing aids do not restore
    hearing, several adjustments can be used to help
  • Adults should refrain from asking the child to
    say words. Once a while, it is okay to do that.
  • Language is caught and not taught.
  • Children need time to listen and play vocal
  • They need lots of practice with nonsense
  • They self correct their own speech.
  • Children whose attempts to communicate are
    accepted feels good about himself, tries harder,
    feels free to fail and does learn to talk.

Learning conditions which will be helpful for
hard of hearing children
  • Wear hearing aids during waking hours
  • Quiet environment during conversation times
  • Use lots of descriptive language (talk about what
    child is looking at, what he is doing and what
    they both see)
  • Less directive (except when directing the child
    to carry out daily routines of eating etc)
  • Use sign language as a bridge to speech
  • Speak clearly
  • Use lots of repetition (You picked the bird. The
    bird is blue. Do you like the bird?).
  • Except childs speech attempts without correcting
    them (speech correction is okay by about 4 - 5
    years of age, but only when done by a speech
    therapist or teacher).

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