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Bilateral congenital cholesteatoma in branchio-oto-renal syndrome

Published online by Cambridge University Press:  29 June 2007

George A. Worley ,
Archana Vats ,
Jonathan Harcourt  and
David M. Albert
George A. Worley*
Affiliation:
Department of Otolaryngology, Head and Neck Surgery, Great Ormond Street Hospital for Children, London, UK.
Archana Vats
Affiliation:
Department of Otolaryngology, Head and Neck Surgery, Great Ormond Street Hospital for Children, London, UK.
Jonathan Harcourt
Affiliation:
Department of Otolaryngology, Head and Neck Surgery, Great Ormond Street Hospital for Children, London, UK.
David M. Albert
Affiliation:
Department of Otolaryngology, Head and Neck Surgery, Great Ormond Street Hospital for Children, London, UK.
*
Address for correspondence: Mr George A. Worley, F.R.C.S., 43 St Peter's Lane, Canterbury, Kent CT1 2BP.
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Abstract

Branchio-oto-renal syndrome is a rare autosomal dominant condition characterized by hearing loss, branchial arch abnormalities and renal tract malformations. We present the first reported case of branchio-oto-renal syndrome associated with bilateral congenital cholesteatoma and ossicular chain abnormalities. The pathogenesis of this syndrome is described and the literature is reviewed.

Keywords

Urinary tractCholesteatoma, middle earEar ossicles, abnormalitiesHearing loss, bilateral
Type
Clinical Records
Information
The Journal of Laryngology & Otology , Volume 113 , Issue 9 , September 1999 , pp. 841 - 843
Copyright
Copyright © JLO (1984) Limited 1999

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