Abstract
The clinical course of hearing loss associated with retinitis pigmentosa is outlined in four brief case summaries. This incidence of hearing loss in retinitis pigmentosa is 10% and occurs several years before clinical visual abnormalities. A battery of audiometric tests all suggest that the hearing defect is within the cochlea and that it is not rapidly progressive. Onset age has not been established. The employment of more than routine hearing tests, testing of other family members, electroretinograms, and historical information about certain features of familial vision are useful in establishing early diagnosis.
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