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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. QuickTime™ and a decompressor are needed to see this picture. Tim Bunnell Center Director Center for Pediatric Auditory and Speech Sciences Thierry Morlet Lab Head Auditory Physiology and Psychoacoustics Linda Vallino Lab Head Craniofacial Outcomes Research Rob O'Reilly Lab Head Balance and Vestibular Disorders Tim Bunnell Lab Head Speech Research Ear Structures Cochlea source: wikipedia 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) 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 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 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 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) 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? 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 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’ 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/ Unity for Gallaudet - 2006 I.K.Jordan’s successor not deaf enough. 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? 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