[Mitochondrial DNA mutations and aminoglycoside antibiotics and hearing loss].

Mitochondrial DNA mutations are one of the most important causes of sensorineural hearing loss. A1555G and C1494T mutations of mitochondrial 12S rRNA gene are the molecular basis for aminoglycoside hyper- sensitivity and can lead to aminoglycoside-induced hearing loss. Primary mutations in tRNA such as tRNA(Ser(UCN))7472insC are associated with syndromic hearing loss. While other mutations such as tRNA"(Se(UCN) )G7444A were considered synergy with the primary RNA mutations, modulating the phenotypic manifestation. This review de- scribes a detailed summary of hearing loss associated with mtDNA mutations and/or aminoglycoside antibiotics, and provides the possible molecular mechanisms in deafness expression.