Bilateral congenital cholesteatoma in branchio-oto-renal syndrome

G A Worley; A Vats; J Harcourt; D M Albert

doi:10.1017/s0022215100145359

Bilateral congenital cholesteatoma in branchio-oto-renal syndrome

J Laryngol Otol. 1999 Sep;113(9):841-3. doi: 10.1017/s0022215100145359.

Authors

G A Worley¹, A Vats, J Harcourt, D M Albert

Affiliation

¹ Department of Otolaryngology, Head and Neck Surgery, Great Ormond Street Hospital for Children, London, UK.

PMID: 10664690
DOI: 10.1017/s0022215100145359

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.

Publication types

Case Reports
Review

MeSH terms

Branchio-Oto-Renal Syndrome / complications*
Branchio-Oto-Renal Syndrome / diagnostic imaging
Cholesteatoma, Middle Ear / complications*
Cholesteatoma, Middle Ear / congenital*
Cholesteatoma, Middle Ear / diagnostic imaging
Ear Ossicles / abnormalities*
Female
Hearing Loss, Bilateral / diagnostic imaging
Hearing Loss, Bilateral / etiology*
Humans
Infant
Tomography, X-Ray Computed