The deafness gene

 
Published: Friday 15 August 1997

a deafness gene has been discovered by British scientists investigating skin disease. The faulty gene is thought to be behind the only inherited form of deafness that is not also linked to other genetic problems. Researchers from St Bartholomew's Hospital, London, and the Royal London School of Medicine and Dentistry, traced the cause of 'non-syndromic' deafness to a mutation in the gene responsible for producing the protein connexin 26. The protein appears to be an important component of the human cochlea -- the snail-shell shaped organ in the inner ear. It is here where the organ of Corti, the primary sense receptor for sound, is located. A fault in connexin 26 may cause deafness by upsetting the fluid composition in the cochlea. One of the research fellows, Howard Stevens, is the grandson of Arthur Stevens, who designed the world's first wearable electronic hearing aid. Severe deafness is the most prevalent inherited sensory disorder and affects about one in 1,000 children.

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