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The deafness\u2010linked mutations often occur in the mitochondrial 12S rRNA gene and in tRNA genes. Mutations in the 12S rRNA gene account for most of the cases of aminoglycoside ototoxicity. The other hot spot for mutations associated with hearing impairment is the tRNA<jats:sup>Ser(UCN)<\/jats:sup> gene, as five deafness\u2010linked mutations have been identified in this gene. Nonsyndromic deafness\u2010linked mtDNA mutations are often homoplasmic or at high levels of heteroplasmy, indicating a high threshold for pathogenicity. 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