Fostering auditory development of a child with

auditory neuropathy

Holly GilliamYusnita Weirather, M.A., CCC-

ADeborah Gabe, M.A., CCC-A

March 4, 2005

TerminologyTerminology

Auditory neuropathyAuditory neuropathy Auditory dyssynchronyAuditory dyssynchrony Nonperipheral hearing lossNonperipheral hearing loss Hearing loss beyond the cochleaHearing loss beyond the cochlea Sensori-neural hearing lossSensori-neural hearing loss Unconventional hearing lossUnconventional hearing loss

What is auditory dys-synchrony?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).

www.raisingdeafkids.org/hearingloss/types/anwww.raisingdeafkids.org/hearingloss/types/an

Aidan’s momAidan’s mom

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

Audiological test resultsAudiological test results

• Presence of cochlear hair cell activityPresence of cochlear hair cell activity- cochlear microphonics in ABR- cochlear microphonics in ABR- otoacoustic emissions (may later - otoacoustic emissions (may later disappear)disappear)

• Absence of auditory nerve responseAbsence of auditory nerve response- no ABR wave forms observed- no ABR wave forms observed

• Behavioral audiometric test results varyBehavioral audiometric test results vary- fluctuating responses- fluctuating responses- audiograms range from normal to profound- audiograms range from normal to profound

Clinical manifestationsClinical manifestations

Ability to detect sounds and speech cannot be Ability to detect sounds and speech cannot be predicted by ABR results.predicted by ABR results.

Pure tone audiogram becomes important to Pure tone audiogram becomes important to determine what the child hears.determine what the child hears.

Word recognition ability is worse than predicted Word recognition ability is worse than predicted by the pure tone audiogram.by the pure tone audiogram.

Functional hearing ability changes and cannot Functional hearing ability changes and cannot be predicted and occasionally, in the long term, be predicted and occasionally, in the long term, hearing may improvehearing may improve

Hearing aid benefits are not uniform across Hearing aid benefits are not uniform across casescases

Zachary’s audiological profileZachary’s audiological profile

Failure of bilateral newborn hearing screening Failure of bilateral newborn hearing screening with ABRwith ABR

Absent auditory brainstem responses to click Absent auditory brainstem responses to click stimuli at a 90 dBeHL presentation level.stimuli at a 90 dBeHL presentation level.

Robust Otoacoustic Emissions as measured by Robust Otoacoustic Emissions as measured by Transient Evoked technology. Emissions Transient Evoked technology. Emissions eventually disappear by the fourth audiological eventually disappear by the fourth audiological evaluation (6 months of age)evaluation (6 months of age)

www.babyhearing.org/www.babyhearing.org/

Normal ABR Normal ABR

Zachary’s medical historyZachary’s medical history

Born at 33 weeks gestational ageBorn at 33 weeks gestational age 3 Lb 15 oz3 Lb 15 oz Esophageal atresia type A (a congenital Esophageal atresia type A (a congenital

disorder of the digestive system where the disorder of the digestive system where the esophagus does not fully develop). He esophagus does not fully develop). He was hospitalized for 3 months after birth. was hospitalized for 3 months after birth. Repaired on April 21, 2002.Repaired on April 21, 2002.

Jaundice and possible sepsisJaundice and possible sepsis

Audiological managementAudiological 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 interventionDevelopmental intervention

7 months:7 months: Cognitive / Social:Cognitive / Social:

Is more independent (not crying if mom leaves the room for a few Is more independent (not crying if mom leaves the room for a few minutes).minutes).

Explores his environment or objects by reaching for objects and finding Explores his environment or objects by reaching for objects and finding hidden objects through locomotionhidden objects through locomotion

Gross motor: sits independently, bangs cups together, rakes tiny Gross motor: sits independently, bangs cups together, rakes tiny objects.objects.

Self Help: Is able to finger feed himself, and drink from a cup with Self Help: Is able to finger feed himself, and drink from a cup with adult support.adult support.

Communication / speech:Communication / speech: Increases eye contact; communicates his needs through signing, Increases eye contact; communicates his needs through signing,

gesturing or verbalizing; responds to environmental sounds and speech gesturing or verbalizing; responds to environmental sounds and speech by turning his head or vocalizing; and is able to say “mama” and “dada.”by turning his head or vocalizing; and is able to say “mama” and “dada.”

Increases vocalizationsIncreases vocalizations

Developmental interventionDevelopmental intervention 1 year and 18 months:1 year and 18 months:

Cognitive / Social:Cognitive / Social: Finds objects and is able to play by himselfFinds objects and is able to play by himself Points to body partPoints to body part Increases attention spanIncreases attention span Eliminates hand banging and self injurious behavior when frustratedEliminates hand banging and self injurious behavior when frustrated

Gross motor: stands up, holds himself.Gross motor: stands up, holds himself. Self Help: Feeds himself using utensils, is able to drink from a cup with Self Help: Feeds himself using utensils, is able to drink from a cup with

minimal spillage, is introduced to toilet training.minimal spillage, is introduced to toilet training. Communication / speech:Communication / speech:

Increases tolerance to hearing aids.Increases tolerance to hearing aids. Uses 1 - 3 signs to express his needs, and is able to say “mama” and “dada.”Uses 1 - 3 signs to express his needs, and is able to say “mama” and “dada.” Increases vocalizationIncreases vocalization Parents train in communication using ASL and oral languageParents train in communication using ASL and oral language Increases imitation skillsIncreases imitation skills

Developmental interventionDevelopmental intervention

2 years:2 years: Cognitive / Social:Cognitive / Social:

Masters pre writing skillMasters pre writing skill Is able to complete puzzlesIs able to complete puzzles Recognizes letters and develops pre literacy skillsRecognizes letters and develops pre literacy skills

Gross motor: Jumps forward.Gross motor: Jumps forward. Self Help: Sits at a table during meal, is toilet trained, can eat a variety of Self Help: Sits at a table during meal, is toilet trained, can eat a variety of

foods. foods. Communication / speech:Communication / speech:

Increases accurate production of age appropriate sounds (p, b, m, n, d, t, k, g, Increases accurate production of age appropriate sounds (p, b, m, n, d, t, k, g, w, h, j)w, h, j)

Increases vocabulary Increases vocabulary Use 2-3 words phrases spontaneously to request, comment and answer Use 2-3 words phrases spontaneously to request, comment and answer

questionsquestions Understands prepositionUnderstands 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 Aural rehabilitation / auditory trainingtraining

Age appropriate auditory and communication Age appropriate auditory and communication skillsskills Wearss hearing aids consistently during his waking hoursWearss hearing aids consistently during his waking hours Discriminates speech sounds in words, phrases and sentences Discriminates speech sounds in words, phrases and sentences

through audition onlythrough audition only Is able to perform play audiometric testingIs able to perform play audiometric testing

Parental partnership and involvementParental partnership and involvement Understand issues related to auditory neuropathy, options in Understand issues related to auditory neuropathy, options in

communication, early literacy skills, deaf mentorship, auditory communication, early literacy skills, deaf mentorship, auditory development, auditory stimulation activities and fostering of oral development, auditory stimulation activities and fostering of oral production.production.

Increase skills in ASL and become involved in a “shared reading Increase skills in ASL and become involved in a “shared reading program” program”

FAMILY CONTRIBUTIONFAMILY CONTRIBUTION

Full active participation in audiological evaluation, Full active participation in audiological evaluation, management and early interventionmanagement and early intervention

Proficiency in ASL by self study and formal classesProficiency in ASL by self study and formal classes Participation in a deaf mentor pilot projectParticipation in a deaf mentor pilot project Careful monitoring of Zachary’s sign and oral language Careful monitoring of Zachary’s sign and oral language

developmentdevelopment Open mindedness to new ideasOpen mindedness to new ideas Family participation in all of Zachary’s first appointments Family participation in all of Zachary’s first appointments

and Saturday appointmentsand Saturday appointments Creativity in developing new family activities to support Creativity in developing new family activities to support

Zachary’s needs.Zachary’s needs.

Latest performanceLatest performance

Has a desire to learn; is outgoing, a risk taker, Has a desire to learn; is outgoing, a risk taker, determined, smart, and affectionate; has a great memory determined, smart, and affectionate; has a great memory and positive behaviors best displayed in a structured and positive behaviors best displayed in a structured environmentenvironment

Is comfortable with his hearing aids thoughout the dayIs comfortable with his hearing aids thoughout the day Uses ASL and spoken language to join 2 or 3 words.Uses ASL and spoken language to join 2 or 3 words. Counts to 5Counts to 5 Fingerspells a manual alphabetFingerspells a manual alphabet Is age appropriate for social, motor and self help skills.Is age appropriate for social, motor and self help skills. Is slightly below his age level for expressive language Is slightly below his age level for expressive language

and mathematical concepts.and mathematical concepts.

FAMILY GOALFAMILY GOAL

Family BeliefFamily Belief

Family - Professional Family - Professional PartnershipPartnership

COMMUNICATION:COMMUNICATION:

1.1. Tailored base of learning at home and elsewhere.Tailored base of learning at home and elsewhere.2.2. Periodic readjustment of management.Periodic readjustment of management.3.3. Determination of child’s strength and Determination of child’s strength and

weaknesses.weaknesses.4.4. Creation of and dedication to the same goal: Creation of and dedication to the same goal:

REACHING HIS POTENTIALREACHING HIS POTENTIAL

How Hard of Hearing Children How Hard of Hearing Children Learn to TalkLearn to Talk by Nancy Rushmerby Nancy Rushmer

They learn to talk through listening with their hearing They learn to talk through listening with their hearing aids. Because hearing aids do not restore hearing, aids. Because hearing aids do not restore hearing, several adjustments can be used to help them:several adjustments can be used to help them: Adults should refrain from asking the child to say words. Once a Adults should refrain from asking the child to say words. Once a

while, it is okay to do that.while, it is okay to do that. Language is caught and not taught.Language is caught and not taught. Children need time to listen and play vocal games.Children need time to listen and play vocal games. They need lots of practice with nonsense syllables.They need lots of practice with nonsense syllables. They self correct their own speech.They self correct their own speech. Children whose attempts to communicate are accepted feels Children whose attempts to communicate are accepted feels

good about himself, tries harder, feels free to “fail” and does good about himself, tries harder, feels free to “fail” and does learn to talk.learn to talk.

Learning conditions which will be Learning conditions which will be helpful for hard of hearing children:helpful for hard of hearing children:

Wear hearing aids during waking hoursWear hearing aids during waking hours Quiet environment during conversation timesQuiet environment during conversation times Use lots of descriptive language (talk about what child is looking at, Use lots of descriptive language (talk about what child is looking at,

what he is doing and what they both see)what he is doing and what they both see) Less directive (except when directing the child to carry out daily Less directive (except when directing the child to carry out daily

routines of eating etc)routines of eating etc) Use sign language as a bridge to speech developmentUse sign language as a bridge to speech development Speak clearlySpeak clearly Use lots of repetition (You picked the Use lots of repetition (You picked the birdbird. The . The birdbird is blue. Do you is blue. Do you

like the like the birdbird?).?). Except child’s speech attempts without correcting them (speech Except child’s speech attempts without correcting them (speech

correction is okay by about 4 - 5 years of age, but only when done correction is okay by about 4 - 5 years of age, but only when done by a speech therapist or teacher).by a speech therapist or teacher).