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Classification of Usher's Syndrome

Classification of Usher's Syndrome This article is only available in the PDF format. Download the PDF to view the article, as well as its associated figures and tables. Abstract 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 http://www.deepdyve.com/assets/images/DeepDyve-Logo-lg.png Archives of Otolaryngology - Head & Neck Surgery American Medical Association

Classification of Usher's Syndrome

Abstract

This article is only available in the PDF format. Download the PDF to view the article, as well as its associated figures and tables. Abstract 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...
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Publisher
American Medical Association
Copyright
Copyright © 1988 American Medical Association. All Rights Reserved.
ISSN
0886-4470
eISSN
1538-361X
DOI
10.1001/archotol.1988.01860180027018
Publisher site
See Article on Publisher Site

Abstract

This article is only available in the PDF format. Download the PDF to view the article, as well as its associated figures and tables. Abstract 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

Journal

Archives of Otolaryngology - Head & Neck SurgeryAmerican Medical Association

Published: Jun 1, 1988

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