Sensorineural hearing loss caused by mitochondrial dna mutations: Special reference to the A1555G mutation

Shin Ichi Usami, Satoko Abe, Hideichi Shinkawa, William J. Kimberling

Research output: Contribution to journalArticle

28 Scopus citations


Mutations in mitochondrial DNA, which are maternally inherited, have been thought to be one of the causes of sensorineural hearing loss. Two mitochondrial mutational sites (A1555G, A7445G) have been reported to be responsible for non-syndromic hearing impairments. The A 1555G mutation causes increased susceptibility to aminoglycoside antibiotic-induced hearing loss as well as non-syndromic sensorineural hearing loss. Our wide screening study showed that there may be a great number of subjects within the Japanese population who have the A1555G mutation. Recent reports suggest that high- risk populations may exist throughout the world. The aminoglycoside-induced hearing loss associated with a mitochondrial mutation is commonly bilateral, symmetric, high frequency involved, and is sometimes associated with progressive sensorineural hearing loss.

Original languageEnglish (US)
Pages (from-to)423-435
Number of pages13
JournalJournal of Communication Disorders
Issue number5
Publication statusPublished - Jan 1 1998


ASJC Scopus subject areas

  • Experimental and Cognitive Psychology
  • Linguistics and Language
  • Cognitive Neuroscience
  • LPN and LVN
  • Speech and Hearing

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