[Familial susceptibility to aminoglycoside ototoxicity due to the A1555G mutation in the mitochondrial DNA]

The A1555G mutation in the mitochondrial genome causes sensorineural hearing loss and familial aminoglycoside ototoxicity.Screening for the A1555G mutation was performed on 72 patients with nonsyndromic sensorineural hearing loss.The A1555G mutation was identified in 15 patients (20.8%). All of them presented maternal relatives with deafness. Individuals with the A1555G mutation that had been treated with aminoglycosides developed more severe hearing loss.The A1555G mutation should be screened in individuals with maternal relatives with hearing loss before administering aminoglycosides.