Usher Syndrome

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A [genetic] disease marked by congenital [deaf]ness followed by the onset of [retinitis pigmentosa].

Originally described in a 1914 paper by British [ophthalmologist] C.H. Usher, the syndrome is transmitted by an autosomal [recessive] gene - all children of two affected parents will have the condition, and the children of one parent with Usher and one without will be [carrier]s. <P> While the deafness may be present at birth or within the first ten years, the [vision loss] associated with Usher Syndrome does not usually manifest itself until after the age of twenty. An individual's [balance] is generally affected as well. <P> It is believed that more than half of the estimated 16,000 [deaf-blind] Americans have Usher Syndrome. <P> <P ALIGN=right>more [eponymous syndromes]