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Previous Volume 348:1664-1670 April 24, 2003 Number 17 Next

	Since this article has no abstract, we have provided an extract of the first 100 words of the full text and any section headings.

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The Usher syndrome is an autosomal recessive disorder characterized by bilateral sensorineural deafness and progressive loss of vision due to retinitis pigmentosa. It is the most frequent cause of deafness and concurrent blindness,¹ with a prevalence of 1 in 16,000 to 1 in 50,000.² The majority of cases of the Usher syndrome can be classified into one of three clinical subtypes, the most severe of which is the type 1 Usher syndrome, characterized by profound prelingual hearing loss, vestibular areflexia, and prepubertal onset of retinitis pigmentosa.² Seven loci for the type 1 Usher syndrome (USH1A to USH1G) have . . . [Full Text of this Article]

Methods

Subjects

Detection of Mutations

Results

Discussion

Source Information

From the Laboratory of Molecular Genetics, National Institute on Deafness and Other Communication Disorders, National Institutes of Health, Rockville, Md. (T.B.-Y., A.C.M., J.H.W., Z.M.A., A.J.G., T.B.F.); the Department of Human Genetics, Mount Sinai School of Medicine, New York (S.L.N., A.B.-L., R.J.D., J.P.W.); the Department of Human Genetics and Molecular Medicine, Sackler School of Medicine, Tel Aviv University, Tel Aviv, Israel (K.B.A.); and the Human Genetics Program, New York University School of Medicine, New York (H.O., C.O.).

Address reprint requests to Dr. Friedman at the Laboratory of Molecular Genetics, NIDCD, 5 Research Ct., Rm. 2A15, Rockville, MD 20850, or at friedman@nidcd.nih.gov.

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