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Sensorineural hearing loss in MELAS syndrome

Published online by Cambridge University Press:  29 June 2007

P. D. Warrick ,
P. Wardrop  and
D. W. Sim
P. D. Warrick*
Affiliation:
Department of Otolaryngology, Royal Infirmary, Edinburgh, UK.
P. Wardrop
Affiliation:
Department of Otolaryngology, Royal Infirmary, Edinburgh, UK.
D. W. Sim
Affiliation:
Department of Otolaryngology, Royal Infirmary, Edinburgh, UK.
*
Address for correspondence: Dr P. Wardrop, F.R.C.S., Otolargynology Unit, Lauriston Building, Royal Infirmary, EdinburghEH3 9EN.
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Abstract

A case of sensorineural hearing loss (SNHL) in MELAS syndrome, a variety of mitochondrial cytopathy, is presented. Mitochondrial cytopathies have gone almost unreported in the otolaryngology literature, despite evidence from a recent review that about 60 per cent of such patients suffer from SNHL (Gold and Rapin, 1994). The same review revealed that only one of 117 case reports in the period 1984–1993 contained an audiogram (Swift and Singh, 1988), and none presented sequential audiograms. However, audiometry has since been published on 23 members of a family with a mitochondrial point mutation causing only sensorineural hearing loss with no other symptoms (Vernham et al., 1994). We present a case of mitochondrial cytopathy three years after diagnosis with two sequential audiograms.

Keywords

Cytopathy, mitochondrialHearing loss, sensorineural
Type
Clinical Records
Information
The Journal of Laryngology & Otology , Volume 111 , Issue 3 , March 1997 , pp. 279 - 281
Copyright
Copyright © JLO (1984) Limited 1997

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