REVIEW

Cell Research (2007): 227-239
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Mitochondrial rRNA and tRNA and hearing function

Guangqian Xing¹, Zhibin Chen¹, Xin Cao²

Correspondence: Guangqian Xing Tel: +86-25-86531424; Fax: +86-25-86611637 E-mail: xing-gq@163.com

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The human ear is a delicate sensory apparatus of hearing for normal communication, and its proper functioning is highly dependent on mitochondrial oxidative phosphorylation. The first mitochondrial point mutation for nonsyndromic and aminoglycoside-induced hearing loss was identified in 1993. Since then a number of inherited mitochondrial mutations have been implicated in hearing loss. Most of the molecular defects responsible for mitochondrial disorder-associated hearing loss are mutations in the 12S rRNA gene and tRNA genes. In this review, after a short description of normal hearing mechanisms and mitochondrial genetics, we outline the recent advances that have been made in the identification of deafness-associated mitochondrial mutations, and discuss how mitochondrial dysfunction contributes to hearing loss.

Cell Research (2007) 17: 227-239. doi: 10.1038/sj.cr.7310124; published online 2 January 2007

Keywords: mitochondrial DNA, rRNA, tRNA, gene mutation, aminoglycosides, hearing loss