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Cochlear implantation of a patient with a previously undescribed mitochondrial DNA defect

Published online by Cambridge University Press:  08 March 2006

P. R. Counter ,
M. P. Hilton ,
D. Webster ,
T. Wardell ,
R. W. Taylor ,
G. Besley ,
D. M. Turnbull  and
P. J. Robinson
P. R. Counter
Affiliation:
University Department of Otolaryngology, Southmead Hospital, Bristol, UK
M. P. Hilton
Affiliation:
University Department of Otolaryngology, Southmead Hospital, Bristol, UK
D. Webster
Affiliation:
University Department of Otolaryngology, Southmead Hospital, Bristol, UK
T. Wardell
Affiliation:
Department of Neurology, Medical School, Newcastle upon Tyne, UK
R. W. Taylor
Affiliation:
Department of Neurology, Medical School, Newcastle upon Tyne, UK
G. Besley
Affiliation:
Willnick Biochemical Genetics Unit, Royal Manchester Children's Hospital, Manchester, UK
D. M. Turnbull
Affiliation:
University Department of Otolaryngology, Southmead Hospital, Bristol, UK
P. J. Robinson
Affiliation:
University Department of Otolaryngology, Southmead Hospital, Bristol, UK
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Abstract

Mitochondrial DNA (mtDNA) defects are responsible for symptom complexes that are characteristically heterogeneous but are typically represented by muscle weakness and neurological deficits. One common feature of mitochondrial disease is deafness. This report details the assessment and outcome of a patient with a previously undescribed mtDNA rearrangement who underwent cochlear implantation. The patient shows a marked improvement in sentence recognition tests and recognition of environmental sounds. Patients with profound sensorineural hearing loss due to mtDNA defects should be considered as candidates for cochlear implantation when they no longer benefit from conventional hearing aids.

Keywords

Cochlear ImplantationDnaMitochondria
Type
Research Article
Information
The Journal of Laryngology & Otology , Volume 115 , Issue 9 , September 2001 , pp. 730 - 732
Copyright
Royal Society of Medicine Press Limited 2001

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