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Journal of Speech and Hearing Disorders Vol.48 308-314 August 1983.
© American Speech-Language-Hearing Association
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Retinitis Pigmentosa and Progressive Hearing Loss

Adrienne Karp 1 and Frances Santore 2

1 New York Association for the Blind, New York, NY
2 New York League for the Hard-of-Hearing, New York, NY

Much material has been written about the deaf-blind patient diagnosed as having Usher's syndrome, a pathologic condition involving hearing impairment and retinitis pigmentosa. Contrary to the accepted pattern of prelingual deafness in such cases, however, there are a number of patients who report a progressive, postlingual hearing loss associated with retinitis pigmentosa. These patients may suffer from a variation of classical Usher's syndrome. An attempt is made to verify this statement through presentation of case histories and audiologic findings. In addition, diagnostic and rehabilitative techniques employed in assisting patients with these dual impairments are offered.

Submitted on June 17, 1982
Accepted on May 3, 1983




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A. van Aarem, C. W. R. J. Cremers, and M. J. L. Benraad-van Rens
Usher Syndrome: A Temporal Bone Report
Arch Otolaryngol Head Neck Surg, August 1, 1995; 121(8): 916 - 921.
[Abstract] [PDF]


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Copyright © 1983 by the American Speech-Language-Hearing Association.