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Arch Otolaryngol Head Neck Surg. 2003;129:405-406.

	Since this article does not have an abstract, we have provided the first 150 words of the full text and any section headings.

IN 1992, León et al¹ mapped the first nonsyndromic deafness locus to chromosome 5q31 in a large kindred from Costa Rica, segregating autosomal dominant postlingual deafness that begins as a low-frequency loss at about age 10 years and progresses to involve all frequencies by age 30 years. Five years later, Lynch et al² identified a protein-truncating mutation in the causative gene HDIA1, the human homologue of the Drosophila gene diaphanous. As of September 2002, 41 dominant (DFNA + integer), 33 recessive (DFNB + integer) and 8 X-linked (DFN + integer) deafness loci have been mapped and 29 different deafness-related genes have been cloned.³

These discoveries are truly monumental in their impact. They are changing both our understanding of the biology of hearing and deafness and the clinical management of deafness. Already, genetic screening of GJB2 is offered in many centers worldwide. Because mutations in this gene . . . [Full Text of this Article]

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