Sensorineural hearing loss caused by mitochondrial dna mutations: Special reference to the a1555g mutation

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 A1555G mutation causes increase...

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Bibliographic Details
Published in:Journal of communication disorders 1998-09, Vol.31 (5), p.423-435
Main Authors: Usami, Shin-Ichi, Abe, Satoko, Shinkawa, Hideichi, Kimberling, William J.
Format: Article
Language:English
Subjects:
Adult
Child
DNA Mutational Analysis
DNA, Mitochondrial - genetics
Female
Genetic Predisposition to Disease - genetics
Genetic Testing
Hearing Loss, Sensorineural - genetics
Humans
Japan
Male
Pedigree
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Summary: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 A1555G 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.
ISSN:0021-9924
1873-7994
DOI:10.1016/S0021-9924(98)00014-8