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Case Reports
. 2025;90(3):73-78.
doi: 10.17116/otorino20259003173.

[Congenital cholesteatoma of the middle ear with total atresia of the external auditory canal: two cases report]

[Article in Russian]
I A Anikin  1 , N N Khamgushkeeva  1 , A D Knyazev  1 , A D Mamedova  1
Affiliations
Case Reports

[Congenital cholesteatoma of the middle ear with total atresia of the external auditory canal: two cases report]

[Article in Russian]
I A Anikin et al. Vestn Otorinolaringol. 2025.

Abstract
in English, Russian

Congenital stenosis and atresia of the external auditory canal are the most common developmental anomalies of the external ear. Cases of combination of these malformations with cholesteatoma of the temporal bone have been repeatedly described in the world literature. This combined pathology is more typical of congenital stenosis of the external auditory canal. Total atresia of the external auditory canal is rarely associated with cholesteatoma. Cholesteatoma found behind the atretic plate is classified as congenital, excluding other possible mechanisms of it's formation. The article presents two clinical cases of a combination of congenital cholesteatoma and complete atresia of the external auditory canal: in an 8-year old girl and a 19-year old boy without the development of intratemporal and extratemporal complications.

Врожденный стеноз и атрезия наружного слухового прохода — наиболее частые аномалии развития наружного уха. В мировой литературе неоднократно описаны случаи сочетания этих пороков развития с холестеатомой височной кости. Данная комбинированная патология больше свойственна врожденному стенозу наружного слухового прохода. Тотальная атрезия наружного слухового прохода сочетается с холестеатомой очень редко. Холестеатома, локализующаяся за атретической пластинкой, классифицируется как врожденная при исключении других возможных механизмов ее формирования. В статье описаны два собственных клинических наблюдения комбинации врожденной холестеатомы и тотальной атрезии наружного слухового прохода, у девочки 8 лет и у юноши 19 лет, без развития интратемпоральных и экстратемпоральных осложнений.

Keywords: complete atresia of the external auditory canal; complete aural atresia; congenital cholesteatoma; middle ear; total atresia of the external auditory canal.

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