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UsherNet.org > What is Usher-Syndrome > Usher Study by Mary Guest
Retinitis Pigmentosa

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Summary

Usher Study by Mary Guest

A Condition which affects Hearing and Sight.
by Mary Guest, Head of Usher Services at Sense.

What is it?
Usher syndrome is an inherited condition which results in hearing loss and a progressive loss of vision from Retinitis pigmentosa (RP). The hearing loss is thought to be congenital, and ranges from moderate to profound. RP can occur without hearing loss. The condition is named after a British ophthalmologist, C.H. Usher, who in a paper in 1914 described several cases in which the link between congenital deafness and RP was stressed. However, as far back as 1860 workers such as von Graef and Liebreich in Berlin were aware of the link between congenital deafness and RP, especially in consanguinous marriages.

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