Bilateral congenital cholesteatoma in branchio-oto-renal syndrome

Bilateral congenital cholesteatoma in branchio-oto-renal syndrome

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 abnor...

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Veröffentlicht in:Journal of laryngology and otology 1999-09, Vol.113 (9), p.841-843
Hauptverfasser: Worley, George A., Vats, Archana, Harcourt, Jonathan, Albert, David M.
Format: Artikel
Sprache:eng
Schlagworte:
abnormalities
bilateral
Biological and medical sciences
Branchio-Oto-Renal Syndrome - complications
Branchio-Oto-Renal Syndrome - diagnostic imaging
Cholesteatoma
Cholesteatoma, Middle Ear - complications
Cholesteatoma, Middle Ear - congenital
Cholesteatoma, Middle Ear - diagnostic imaging
Clinical Records
Ear ossicles
Ear Ossicles - abnormalities
Ear, auditive nerve, cochleovestibular tract, facial nerve: diseases, semeiology
Female
Hearing loss
Hearing Loss, Bilateral - diagnostic imaging
Hearing Loss, Bilateral - etiology
Humans
Infant
Medical sciences
middle ear
Otorhinolaryngology. Stomatology
Tomography, X-Ray Computed
Tumors
Urinary tract
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Beschreibung
Zusammenfassung: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.
ISSN:0022-2151
1748-5460
DOI:10.1017/S0022215100145359