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© 1994 Oxford University Press
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A gene responsible for a dominant form of neurosensory non-syndromic deafness maps to the NSRD1 recessive deafness gene interval
Unité de Génétique Moléculaire Humane, URA CNRS 1445, Instrtut Pasteur 25 rue du Dr Roux, 75724 Paris Cedex 15 1Laboratoire de Physjotogie Sensorieile, URA CNRS 1447 and Université Claude Bernard Lyon I, Département dORL, Hospital E.Herriot 69437 Lyon Cedex 03 2Service de Génée, Hospital E.Herrot, 69437 Lyon Cedex 03 France
*To whom correspondence should be addressed
Received August 24, 1994; Accepted October 4, 1994
The first localization of a gene responsible for autosomal, neurosensory, recessive deafness recently assigned NSRD1 to the centromeric region of human chromosome 13. We now report on a dominant form of neurosensory deafness found In a family of French origin. The deafness is moderate to severe, has a prellngual onset and affects predominantly the high frequencies. The gene responsible for this form of deafness was found by linkage analysis to map to the same region of chromosome 13 as NSRD1. A multipoint analysis gave a maximum lod score of 4.66 with a most likely location close to locus D13S175. This suggests that different mutations in NSRD1 may cause both dominant and recessive neurosensory deafness.
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