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Role of mitochondrial variation in maternally inherited diabetes and deafness syndrome

Published online by Cambridge University Press:  24 October 2008

T Howes ,
C Madden ,
S Dasgupta ,
S Saeed  and
V Das
T Howes
Affiliation:
University Department of Audiological Medicine, Manchester Royal Infirmary, UK
C Madden
Affiliation:
University Department of Audiological Medicine, Manchester Royal Infirmary, UK
S Dasgupta
Affiliation:
University Department of Audiological Medicine, Manchester Royal Infirmary, UK
S Saeed
Affiliation:
University Department of Otolaryngology – Head and Neck Surgery, Manchester Royal Infirmary, UK
V Das*
Affiliation:
University Department of Audiological Medicine, Manchester Royal Infirmary, UK
*
Address for correspondence: Dr Vijay Das, University Department of Audiological Medicine, Manchester Royal Infirmary, Manchester M13 9WL, UK. Fax: +44 (0)161 276 5953 E-mail: Vijay.das@cmmc.nhs.uk
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Abstract

Maternally inherited diabetes and deafness syndrome is caused by the mitochondrial deoxyribonucleic acid mutation 3243 A>G (where A = adenine and G = guanine). The degree of heteroplasmy of the mitochondrial deoxyribonucleic acid may correlate with the rate of progression of the hearing loss. This has important implications for counselling patients with this pathology. Cochlear implantation is a successful method of rehabilitation for patients with hearing loss as part of this syndrome.

Keywords

Sensorineural Hearing LossCochlear ImplantationMitochondrial DNAMIDDHeteroplasmy
Type
Clinical Records
Information
The Journal of Laryngology & Otology , Volume 122 , Issue 11 , November 2008 , pp. 1249 - 1252
Copyright
Copyright © JLO (1984) Limited 2008

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