Cracking the auditory genetic code: Part II. Syndromic hereditary hearing impairment

Objective: The application of molecular genetic techniques to the study of hereditary hearing impairment has contributed significantly to our understa nding of auditory physiology and disease processes. This article reviews th e current state of our knowledge regarding the genes associated with syndro mic hereditary hearing impairment. Data Sources: Data were obtained from the Medline database and the internet . Study Selection: Articles relevant to genetics of syndromic deafness were s elected. Data Extraction: Data pertaining to phenotypes, location of genes, identifi cation of genes, and implications for hearing were extracted. Conclusion: Significant progress has been made in understanding the molecul ar pathogenesis of deafness.