Download 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
Terminology
 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).
Aidan’s 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 I’ve 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 isn’t any definitive answer
to what my child is hearing
www.raisingdeafkids.org/hearingloss/types/an
Audiological test results
• Presence of cochlear hair cell activity
- cochlear microphonics in ABR
- otoacoustic emissions (may later
disappear)
• 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
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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
Zachary’s 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
www.babyhearing.org/
Zachary’s 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
parent’s effort to put them in.
 FM system in conjunction with hearing aid did not
provide much difference in his functional hearing.
 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 locomotion
 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 language
 Increases imitation skills
Developmental intervention
 2 years:
 Cognitive / Social:
 Masters pre writing skill
 Is able to complete puzzles
 Recognizes letters and develops pre literacy skills
 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
language
 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
production.
 Increase skills in ASL and become involved in a “shared reading
program”
FAMILY CONTRIBUTION
 Full active participation in audiological evaluation,
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management and early intervention
Proficiency in ASL by self study and formal classes
Participation in a deaf mentor pilot project
Careful monitoring of Zachary’s sign and oral language
development
Open mindedness to new ideas
Family participation in all of Zachary’s first appointments
and Saturday appointments
Creativity in developing new family activities to support
Zachary’s needs.
Latest performance
 Has a desire to learn; is outgoing, a risk taker,
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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 words.
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 GOAL
Family Belief
Family - Professional
Partnership
COMMUNICATION:
1. Tailored base of learning at home and elsewhere.
2. Periodic readjustment of management.
3. Determination of child’s strength and
weaknesses.
4. Creation of and dedication to the same goal:
REACHING HIS POTENTIAL
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 them:
 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 games.
 They need lots of practice with nonsense syllables.
 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,
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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 development
Speak clearly
Use lots of repetition (You picked the bird. The bird is blue. Do you
like the bird?).
Except child’s 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).