Abstract
Permanent childhood hearing loss occurs in 1 to 2 births per 1,000. It is believed that 50–60% of these cases are the result of mutations in the genetic code contained in the chromosomes, and it appears that several hundred genes are linked to hereditary hearing loss (Smith, Green, &Van Camp, 2005). Of these genetic hearing loss causes, approximately one-third are syndromic and two-thirds are non-syndromic. Advances made by genetics researchers over the past several years have identified the chromosomal locations of a considerable number of gene mutations that result in hearing loss. As a result, there is an increasing number of clinically available gene-based tests that allow the establishment of a definitive diagnosis for a variety of hereditary hearing losses. In cases where such diagnosis occurs, it can help guide counseling as well as appropriate and detailed recommendations for remediation.
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