Multichannel cochlear implantation in Usher's Syndrome
AuthorDettman, Shani J.; Dowell, Richard C.; Brown, A. M.; Clark, Graeme M.
Source TitleProceedings 5th International Retinitis Pigmentosa Congress: scientific papers
PublisherKelleher & Associates
Document TypeConference Paper
CitationsDettman, S. J., Dowell, R. C., Brown, A. M., & Clark, G. M. (1990). Multichannel cochlear implantation in Usher's Syndrome. In Proceedings 5th International Retinitis Pigmentosa Congress: scientific papers.
Access StatusOpen Access
Four patients with Usher's syndrome have been implanted with the multichannel cochlear prosthesis developed by the University of Melbourne and Cochlear Pty. Ltd. All four patients have made good progress with the implant and use the device during most waking hours. One subject, an adult who developed speech and language before developing profound deafness, has learned to use the cochlear implant for auditory alone communication. Her results on speech perception testing compare favourably with other implanted adults. The other subjects were adolescents or young adults when implanted and had congenital profound or total hearing losses. These subjects retained adequate vision for lipreading but had lost hearing before the development of speech and language. Results for these subjects have indicated that the cochlear implant significantly aids lipreading and improves the detection and recognition of environmental sounds. However, they have not, as yet, been able to use the device for communication in the auditory alone condition. The ability to use the device in this condition would become important should vision deteriorate further. These preliminary results suggest that for patients with Usher's syndrome where deafness is progressive and speech and language have developed normally, the multichannel cochlear implant can provide an alternative auditory communication system when vision is no longer functional. When Usher's syndrome has caused a congenital profound deafness, it maybe necessary for the cochlear implant to be integrated into the child's normal speech and language development from an early age to obtain optimal results.
Keywordsretinitis pigmentosa; Usher' syndrome; multichannel cochlear prosthesis; Cochlear Pty. Ltd.
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