SENSORINEURAL HEARING-LOSS CAUSED BY MITOCHONDRIAL-DNA MUTATIONS - SPECIAL REFERENCE TO THE A1555G MUTATION

Mutations in mitochondrial DNA, which are maternally inherited, have b een thought to be one of the causes of sensorineural hearing loss. Two mitochondrial mutational sites (A1555G, A7445G) have been reported to be responsible for non-syndromic hearing impairments. The A1555G muta tion causes increased susceptibility to aminoglycoside antibiotic-indu ced hearing loss as well as non-syndromic sensorineural hearing loss. Our wide screening study showed that there may be a great number of su bjects within the Japanese population who have the A1555G mutation. Re cent reports suggest that high-risk populations may exist throughout t he world. The aminoglycoside-induced hearing loss associated with a mi tochondrial mutation is commonly bilateral, symmetric, high frequency involved, and is sometimes associated with progressive sensorineural h earing loss. (C) 1998 by Elsevier Science Inc.