Usher's syndrome with unusual otologic manifestations

Usher's syndrome is characterized by a congenital hearing loss and retinitis pigmentosa. Ocular symptoms and signs are usually established at adolescence, and the hearing loss, the onset of which is at a young age, generally remains stable. The following case is of interest, therefore, because the hearing loss that had been present since birth progressed suddenly to total bilateral deafness in adulthood. There were no ocular symptoms at any time; the classic findings of retinitis pigmentosa were disclosed only on routine examination of the eyes.