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

Lookup NU author(s): Paul Counter, Dr Theresa Wardell, Professor Robert Taylor, Emeritus Professor Doug Turnbull


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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.

Publication metadata

Author(s): Counter PR, Hilton MP, Webster D, Wardell T, Taylor RW, Besley G, Turnbull DM, Robinson PJ

Publication type: Article

Publication status: Published

Journal: Journal of Laryngology and Otology

Year: 2001

Volume: 115

Issue: 9

Pages: 730-732

ISSN (print): 00222151

ISSN (electronic):

Publisher: Cambridge University Press


DOI: 10.1258/0022215011908766

PubMed id: 11564302


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