Objective: The application of molecular genetic techniques to the study of hereditary hearing impairment has contributed significantly to our understanding of auditory physiology and disease processes. This article reviews the current state of our knowledge regarding the genes associated with syndromic 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 selected.
Data extraction: Data pertaining to phenotypes, location of genes, identification of genes, and implications for hearing were extracted.
Conclusion: Significant progress has been made in understanding the molecular pathogenesis of deafness.