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
Sign-in/Register to access full text options 
Talk to us
Join us for a 30 min session where you can share your feedback and ask us any queries you have
Schedule a callMore From: Archives of Otolaryngology - Head and Neck Surgery
Disclaimer: All third-party content on this website/platform is and will remain the property of their respective owners and is provided on "as is" basis without any warranties, express or implied. Use of third-party content does not indicate any affiliation, sponsorship with or endorsement by them. Any references to third-party content is to identify the corresponding services and shall be considered fair use under The CopyrightLaw.
