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Abstract: Objective: To summarize the advances in the field of molecular genetics of hearing loss, as well as to prepare the pediatrician for the new advances that have been taken place over the years, with great repercussions to the diagnosis, treatment and prevention of deafness. Methods: Review of several original and review papers published during the last decade dealing with lhe díscoveries in the field of molecular genetics of hereditary deafness, with emphazis in nonsyndromic type of hearing loss. Results: Since 1992, 15 nonsyndromic autossomic dominant hearing loss forms (DFNA),19 autossomic recessive forros (DFNB), 6 X-linked forros and 2 mitochondrial forros have been published. At least 9 different genes have been identified for these forms, 8 of them already published. Conclusion: The progress in the identification of genes responsible for the different forros of hearing loss has impact in the clinical practice, as well as in the study of the mechanisms of normal hearing. The increasing identiflcatíon of genes for each case of hearing loss opens perspectives to better and more specific treatments for genetic and environmental hearing loss.
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