1. 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

3. Terminology Auditory neuropathy Auditory neuropathy Auditory dyssynchrony Auditory dyssynchrony Nonperipheral hearing loss Nonperipheral hearing loss Hearing loss beyond the cochlea Hearing loss beyond the cochlea Sensori-neural hearing loss Sensori-neural hearing loss Unconventional hearing loss Unconventional hearing loss

4. 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).

5. www.raisingdeafkids.org/hearingloss/types/an 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 hearing 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 hearing

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

7. Clinical manifestations Ability to detect sounds and speech cannot be predicted by ABR results. Ability to detect sounds and speech cannot be predicted by ABR results. Pure tone audiogram becomes important to determine what the child hears. Pure tone audiogram becomes important to determine what the child hears. Word recognition ability is worse than predicted by the pure tone audiogram. 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 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 Hearing aid benefits are not uniform across cases

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

9. www.babyhearing.org/ Normal ABR

11. Zacharys medical history Born at 33 weeks gestational age Born at 33 weeks gestational age 3 Lb 15 oz 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. 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 Jaundice and possible sepsis

12. 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 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.

13. Developmental intervention 7 months: 7 months: Cognitive / Social: Cognitive / Social: Is more independent (not crying if mom leaves the room for a few minutes). 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 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. 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. Self Help: Is able to finger feed himself, and drink from a cup with adult support. Communication / speech: 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 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 Increases vocalizations

14. Developmental intervention 1 year and 18 months: 1 year and 18 months: Cognitive / Social: Cognitive / Social: Finds objects and is able to play by himself Finds objects and is able to play by himself Points to body part Points to body part Increases attention span Increases attention span Eliminates hand banging and self injurious behavior when frustrated Eliminates 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 minimal spillage, is introduced to toilet training. Self Help: Feeds himself using utensils, is able to drink from a cup with 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 vocalization Increases vocalization Parents train in communication using ASL and oral language Parents train in communication using ASL and oral language Increases imitation skills Increases imitation skills

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

16. 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

17. Aural rehabilitation / auditory training Age appropriate auditory and communication skills Age appropriate auditory and communication skills Wearss hearing aids consistently during his waking hours Wearss hearing aids consistently during his waking hours Discriminates speech sounds in words, phrases and sentences through audition only Discriminates speech sounds in words, phrases and sentences through audition only Is able to perform play audiometric testing Is able to perform play audiometric testing Parental partnership and involvement 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. 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 Increase skills in ASL and become involved in a shared reading program

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

19. 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 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 Is 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 5 Counts to 5 Fingerspells a manual alphabet Fingerspells 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 and mathematical concepts. Is slightly below his age level for expressive language and mathematical concepts.

20. FAMILY GOAL

21. Family Belief

22. Family - Professional Partnership COMMUNICATION: 1.Tailored base of learning at home and elsewhere. 2.Periodic readjustment of management. 3.Determination of childs strength and weaknesses. 4.Creation of and dedication to the same goal: REACHING HIS POTENTIAL

23. 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: 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. 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. 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 good about himself, tries harder, feels free to fail and does learn to talk. Children whose attempts to communicate are accepted feels good about himself, tries harder, feels free to fail and does learn to talk.

24. Learning conditions which will be helpful for hard of hearing children: Wear hearing aids during waking hours Wear hearing aids during waking hours Quiet environment during conversation times 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) 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) Less directive (except when directing the child to carry out daily routines of eating etc) Use sign language as a bridge to speech development Use sign language as a bridge to speech development Speak clearly Speak clearly Use lots of repetition (You picked the bird. The bird is blue. Do you like the bird?). 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). 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).