Genetic and Rare Diseases Information Center (GARD)


Other names people use for this condition
  • Deafness-retinitis pigmentosa syndrome
  • Dystrophia retinae pigmentosa-dysostosis syndrome
  • Graefe-Usher syndrome
  • Hallgren syndrome
  • Usher's syndrome

Usher syndrome
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Usher syndrome is a genetic condition characterized by hearing loss or deafness and progressive vision loss due to retinitis pigmentosa.  Three major types of Usher syndrome have been described - types I, II, and III.  The different types are distinguished by their severity and the age when signs and symptoms appear. All three types are inherited in an autosomal recessive manner, which means both copies of the gene in each cell have mutations. [1]

References
  1. Usher syndrome. Genetic Home Reference (GHR). February 2007 Available at: http://ghr.nlm.nih.gov/condition=ushersyndrome. Accessed May 26, 2008.

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