The progress in the research of genetic hearing loss has advanced our understanding of the molecular mechanisms that govern inner ear development, function and response to injury and aging. In the developed world, over 50% of childhood deafness is attributable to genetic causes and even age-related hearing loss has been associated with genetic mechanisms. AIM: The objective of this review is to summarize recent knowledge in genetic hearing loss. STUDY DESIGN: Sistematic review. MATERIAL AND METHODS: The literature review included articles indexed at MEDLINE (The National Library of Medicine, The National Institute of Health - USA) focusing on publications from the past 3 years plus the information available at the Hereditary Hearing Loss Home Page. CONCLUSION: Advances in the genetics of hearing loss have enhanced our comprehension of auditory function and have enabled more accurate diagnosis. Hopefully, as we further understand the molecular elements of the auditory system, this knowledge will help in the development of new therapies for the treatment of the underlying genetic defects.
genetics; hearing loss; connexin 26; counseling