Sensorineural hearing loss caused by the A1555G mutation in the mitochondrial DNA

Mitochondrial mutations are known to be one of the causes of non-syndromic sensorineural hearing loss (SNHL). The A1555G mitochondrial mutation is ass ociated with a susceptibility to aminoglycoside antibiotics. It is likely t hat among the hearing impaired population there are a great number of subje cts with the A1555G mutation. In our series screening hearing impaired pati ents, the AI555G mitochondrial mutation was found in 72 subjects with and w ithout bilateral sensorineural hearing loss, from 15 families. The present study was focused on the genetic and clinical features of these patients be aring the A1555G mutation. The present results confirmed the A1555G mutatio n involvement in susceptibility to aminoglycoside antibiotics. Furthermore, subjects who have this mutation exhibit a mild? high frequency, progressiv e hearing loss even without aminoglycoside injection, suggesting the A15556 mutation may play a more general role in causing hearing loss.