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Correspondence
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Volume 330:714-715 March 10, 1994 Number 10
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Hearing Loss

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 by Nadol, J. B.
To the Editor: In his excellent, comprehensive review of hearing loss (Oct. 7 issue),1 Dr. Nadol referred specifically to genetic-linkage analyses in Usher's syndrome and Waardenburg's syndrome, but made no mention of the precise genetic basis of Alport's syndrome (hereditary nephritis and sensorineural hearing loss). There is strong genetic linkage of this syndrome to markers near Xq22,2 and the gene responsible at this locus is known to be COL4A5 in at least 50 percent of affected families3,4. More than 50 causative mutations have now been described4,5. In a minority of cases, perhaps 20 percent, transmission is autosomal.

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