1. Cochlear Implants & Deaf Culture Medical Breakthrough, or Cultural Genocide? Tim Bunnell Director, Nemours Center for Pediatric Auditory and Speech Sciences Alfred I. duPont Hospital for Children.

3. Ear Structures

4. Cochlea source: wikipedia

5. deafness (little ‘d’) Definition –Profound hearing Impairment (threshold > 90dB) –Unable to make use of amplifying hearing aids to understand speech Assessment –Otoacoustic Emissions (OAE) –Brainstem evoked response (ABR) –Audiogram –Funtional Hearing (Hearing In Noise Test)

6. Causes of deafness Congenital –Genetic Syndromic - E.g., Waardenberg, Pendred, Usher’s Non-syndromic - E.g., Connexin 26 –Idiopathic (unknown origin) Acquired –Presbycusis - Aging-related hearing loss –Drug-induced - E.g., chemotherapy –Injury - Especially head injury

7. Cochlear Implants External device and processor Through the skin transmission Electrode array inserts into cochlea Multiple electrodes on array Different frequency bands go to different electrodes

8. Cochlear Implant Outcomes Adults (intact auditory nerve) –Good outcome Postlingually deaf short period of deafness –Good outcome Prelingually deaf extremely rare Children (intact auditory nerve) –Good outcome Postlingually deaf short period of deafness –Good outcome Prelingually deaf 2 years or younger at time of first implant –Poor outcome Prelingually deaf 5 years or older at time of first implant

9. Prosthesis not Cure Implantee must learn to use stimulation –Significant (re)habilitation –Device must be ‘tuned’ Speech –Stimulus known –Stimulus unknown Music –Familiar instrumental Demo audio: www.hei.org//research/aip/audiodemos.htm (Bob Shannon, Qian-Jie Fu, John G. Galvin III)www.hei.org//research/aip/audiodemos.htm

10. Deafness (big ‘D’) First language is a signed/manual language Member of ‘Deaf Community’ ‘Wellness’ model –Is not handicapped –Does not need to be cured or fixed Denial or healthy adaptation?

11. ASL Derived from French sign language –Used in US & Canada –Totally unlike British sign language Moderate dialectical variation in different regions –Toronto –Louisiana –California –Washington, DC VERY unlike English in structure –Except spelling borrowed from English –simultaneous ASL & English not possible

12. Education Oral –Speech emphasis –Maybe some sign or gestures Sign –Sign emphasis –Maybe some oral/aural exposure Bilingual –Balanced mix of sign and oral/aural Schools for the deaf –Sterck School in Delaware - ‘D’ –Clarke School in MA, PA, NY, FL - ‘d’

13. Gallaudet University Land donated by Amos Kendall in 1862 in NE D.C. For Deaf, Dumb, and Blind College formed in 1864 Rubella epidemic 1960’s led to large increase in students in late 70’s - 80’s Deaf President Now 1988 (I. King Jordan, Ph.D.) http://pr.gallaudet.edu/dpn/ http://pr.gallaudet.edu/dpn/ Unity for Gallaudet - 2006 I.K.Jordan’s successor not deaf enough.

14. Controversy If all children are implanted will there still be deaf people and culture? Is deafness a cultural ‘difference’ or a physical impairment? What to do with deaf children of hearing parents? –90% of deaf children born to hearing parents –Children identified in newborn screening –Who is the initial point of contact? –Who ‘educates’ parents of newborn deaf child?

15. Interesting/Useful Links PBS Show on Deaf Culture: - http://www.pbs.org/wnet/soundandfury/culture/index.html Wikipedia article on Gallaudet University: - http://en.wikipedia.org/wiki/Gallaudet_University National Association of the Deaf (NAD) statement on CI: - http://www.nad.org/site/pp.asp?c=foINKQMBF&b=138140 Some good websites describing CIs: - http://www.utdallas.edu/~loizou/cimplants/tutorial/tutorial.htm - http://www.nidcd.nih.gov/health/hearing/coch.asp