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

BACKGROUND AND OBJECTIVE: The A1555G mutation in the mitochondrial genome causes sensorineural hearing loss and familial aminoglycoside ototoxicity. PATIENTS AND METHOD: Screening for the A1555G mutation was performed on 72 patients with nonsyndromic sensorineural hearing loss. RESULTS: 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. CONCLUSIONS: The A1555G mutation should be screened in individuals with maternal relatives with hearing loss before administering aminoglycosides.