Hearing Impairment Related to Age in Usher Syndrome Types 1B and 2A
Open Access
- 1 April 1999
- journal article
- research article
- Published by American Medical Association (AMA) in JAMA Otolaryngology–Head & Neck Surgery
- Vol. 125 (4) , 441-445
- https://doi.org/10.1001/archotol.125.4.441
Abstract
THE USHER syndrome was first described in 1858 and characterized as a disorder with bilateral sensorineural hearing loss and visual impairment caused by tapetoretinal degeneration.1 Several authors emphasized a high prevalence of this syndrome in certain families, and a hereditary nature was suspected. von Wibout2 suggested that the syndrome had an autosomal recessive mode of inheritance.Keywords
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