Listening and Spoken Language Outcomes for a Child with an Auditory Brainstem Implant

1. Listening and Spoken Language Outcomes for a Child with an Auditory Brainstem Implant Susan G. Allen, M.E.D., CED., M.Ed., CCC-SLP, LSLS Cert. AVEd Director, Clarke School for Hearing and Speech / Jacksonville Campus

2. Welcome! A.G. Bell Convention 2010 Little is known about speech perception and spoken language outcomes in children with an Auditory Brainstem Implant who are prelingually deaf.

3. FDA Approval The only approval for the ABI device in the United States is for children over 12 and adults with Neurofibromatosis-2. Colletti V. 2005

4. Cochlear Implants Cochlear Implants are for treatment of patients with severe to profound hearing loss due to cochlear defects with an intact auditory nerve Colletti V 2005.

5. Auditory Brainstem Implants-ABI ABI: This device is used for patients with severe to profound hearing loss caused by severe impairment of the cochlear nerve or cochlear abnormalities. Colletti V 2005.

6. Main differences: ABI vs. CI Patient has a debilitating disease that is life-threatening Surgery is more complex and much longer Precise placement of the array more difficult Side effects during activation to be expected Stimulation levels to reach audibility generally higher Outcomes generally poorer Cochlear Corporation READ SLIDEREAD SLIDE

7. ABI24M Electrode Array 21 contacts. There is no Electrode 1. Stimulation between contacts can be monopolar or bipolar.21 contacts. There is no Electrode 1. Stimulation between contacts can be monopolar or bipolar.

8. Known Outcomes ABI recipients should experience: Detection of medium to loud environmental sounds at comfortable listening levels Detection of conversational speech at comfortable listening levels Cochlear Corporation The ABI can help patients communicate by improving lipreading and hearing environmental sounds. A small number of patients experience open set understanding. True benefit often underestimated Helps recipient cope with their illness ABI can reduce isolation and improve their quality of life The ABI can help patients communicate by improving lipreading and hearing environmental sounds. A small number of patients experience open set understanding. True benefit often underestimated Helps recipient cope with their illness ABI can reduce isolation and improve their quality of life

9. Known Outcomes Most ABI recipients should also experience: Improved perception of the rhythm and volume of speech resulting in limited improvement in speech recognition and communication ability with lip reading Limited improvement in the recognition of environmental sounds A small number of ABI recipients will experience: Improved speech recognition without lip-reading Cochlear Corporation The ABI can help patients communicate by improving lipreading and hearing environmental sounds. A small number of patients experience open set understanding. True benefit often underestimated Helps recipient cope with their illness ABI can reduce isolation and improve their quality of life The ABI can help patients communicate by improving lipreading and hearing environmental sounds. A small number of patients experience open set understanding. True benefit often underestimated Helps recipient cope with their illness ABI can reduce isolation and improve their quality of life

10. Case History A twin born premature at 30 weeks Hospitalized for 7 months in NICU Severe profound mixed hearing loss Craniofacial outer, middle and inner ear abnormalities presented with Goldenhar Syndrome

11. Case History Fitted with BaHA Softband at 4 months No auditory benefit MRI at 1 year revealed absence of 7th and 8th cranial nerves and both internal and external auditory canals Not a cochlear implant candidate

12. Case History Fitted with vibrotactile aid Child�s audiological team recommended that the family investigate the Auditory Brainstem Implant

13. Case History Auditory Brainstem Implant not approved in the United States Consulted with Dr. Vittorio Colleti in Verona, Italy Implanted with ABI at 3 years, 3 months of age

14. Case History Device activated in Italy in January 2006 No follow up until June 2006 Evaluated at House Ear Institute in June 2006 with consultation from Children�s Hospital in Birmingham, AL No behavioral responses

15. Case History Behavioral responses improved to 25 dB with consistent mapping on June 19, 2006 Received mapping and auditory habilitation at Children�s Hospital in Birmingham Total communication classroom in the Public School System in Alabama

16. Case History Due to limited progress professionals recommended the family look at other educational options Visited Clarke Jacksonville in Spring 2007 Enrolled in Diagnostic Summer Program in June 2007

17. Mapping Jorden receives ongoing mapping from Mandy Lutz Mahalak, AuD, CCC-A Assistant Director, Pediatric Cochlear Implant Audiologist Children's HEAR CenterChildren's Health System Birmingham, Alabama

18. Therapy Data July 2007: Baseline October 2007 October 2008 October 2009 May 2010 In the initial evaluation at the beginning of July 2007 (Summer Session) The above are the results except for APT/HI On the ESP: he DID discriminate between /ah/ and hop, hop and 1 vs. 3 syllables He DID NOT discriminate 2 vs 3 (baby vs. ice-cream cone) or 1 vs. trochee ( ball vs. baby) NO PATTERN PERCEPTION cause stopped with training� APT/HI was given at the end of the summer session AFTER intervention. He passed awareness tasks, duration of a single sound short/long, dog vs. hamburger, airplane vs. hamburger but nothing longer, he understood the vowel task of /a/, /u/, /i/ but not diphthongs or consonants. He was on his way!In the initial evaluation at the beginning of July 2007 (Summer Session) The above are the results except for APT/HI On the ESP: he DID discriminate between /ah/ and hop, hop and 1 vs. 3 syllables He DID NOT discriminate 2 vs 3 (baby vs. ice-cream cone) or 1 vs. trochee ( ball vs. baby) NO PATTERN PERCEPTION cause stopped with training� APT/HI was given at the end of the summer session AFTER intervention. He passed awareness tasks, duration of a single sound short/long, dog vs. hamburger, airplane vs. hamburger but nothing longer, he understood the vowel task of /a/, /u/, /i/ but not diphthongs or consonants. He was on his way!

19. Classroom Data July 2007: Baseline October 2007 October 2008 October 2009 May 2010

20. Baseline Data Movie of Jorden

21. Components of the Educational Program Classroom that used a �Bottoms up� approach that required remedial instruction with a didactic approach 4 levels of auditory development using the �Circle of Listening� Association Method The Dubard �Association Method� Susan G. Allen �Circle of Listening�

22. Association Method Techniques Used to �jump start� his phonemic awareness skills, articulation and auditory memory Production of sounds in isolation Sound segmenting and blending (�boo�) Segmenting and blending of 3 sounds (boot)

23. What we did differently with Jorden vs. other CI kids Addition of �Association Method" Rapid drilling with CVC word blends Rewrites of words and/or phrases Helped build automaticity of the production of oral language Auditory Memory Skills Visual Memory Skills for reading

24. Therapy Techniques Video Demonstrations with Susan and Alisa

25. Classroom Techniques Video Demonstrations with Lynn

26. Sound Progress

27. Sound Progress

28. Sound Progress

29. Sound Progress

30. Sound Progress

31. Jorden Now!Classroom 5.10

32. Conclusions Progress was similar to that of a child with a cochlear implant who was implanted at a later age Surprised the staff with better, steady progress sustained over time than anticipated given available written information on the ABI device Currently expected to continue to function well in the hearing world with continued auditory-oral education!

33. FAME! Video