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June 1988

Classification of Usher's Syndrome

Author Affiliations

Burlington, Mass

Arch Otolaryngol Head Neck Surg. 1988;114(6):613. doi:10.1001/archotol.1988.01860180027018

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The relationship of auditory and vestibular abnormalities in Usher's syndrome was investigated by Patrick Brookhauser, MD, and colleagues, Boy's Town Institute, Omaha, at the Eastern Section meeting of the Triological Society in New York City. This evaluation of 25 patients was carried out to improve the diagnostic classification, estimate the prevalence of the disease, and determine the role of mental retardation in this disorder. This recessively inherited disorder generally presents with congenital hearing loss and retinitis pigmentosa. Although the incidence is 4.4 per 100000, Usher's syndrome accounts for 50% of the deaf/blind individuals in this country. By evaluating these patients, two Usher types have been identified: type I, with severe-to-profound cochlear loss and absent peripheral vestibular responses; and type II, with a moderate-to-severe cochlear loss with normal vestibular responses. There was no consistent association with mental retardation. Neither of these types appears progressive, and the severity of retinitis pigmentosa was

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