Cholesteatoma in Congenital Aural Atresia and External Auditory Canal Stenosis: A Systematic Review

Otolaryngol Head Neck Surg. 2022 Apr 19;1945998221094230. doi: 10.1177/01945998221094230. Online ahead of print.


Objective: Patients with congenital external auditory canal (EAC) abnormalities are at risk of developing cholesteatoma and often undergo surveillance imaging to detect it. The aims of this systematic review are to determine the incidence of cholesteatoma in patients with congenital aural atresia (CAA) and patients with congenital EAC stenosis and to investigate the most common age of cholesteatoma diagnosis. This information will help clinicians decide which patients require surveillance scanning, as well as the timing of imaging.

Data sources: Ovid MEDLINE, Embase, CENTRAL, and Web of Science databases.

Review methods: A systematic literature review following the PRISMA guidelines was performed. The data sources were searched by 2 independent reviewers, and articles were included that reported on CAA or congenital EAC stenosis with a confirmed diagnosis of cholesteatoma. The selected articles were screened separately by 3 reviewers before reaching a consensus on the final articles to include. Data collection on the number of patients with cholesteatoma and the age of diagnosis was performed for these articles.

Results: Eight articles met the inclusion criteria. The incidence of cholesteatoma was 1.7% (4/238) in CAA and 43.0% (203/473) in congenital EAC stenosis. The majority of patients with congenital EAC stenosis that developed cholesteatoma were diagnosed at age <12 years.

Conclusion: CAA is associated with a low risk of cholesteatoma formation, and surveillance imaging is unnecessary in asymptomatic patients. EAC stenosis is strongly associated with cholesteatoma, and a surveillance scan for these patients is recommended prior to 12 years of age with close follow-up into adulthood.

Keywords: cholesteatoma; congenital aural atresia; congenital external auditory canal stenosis; microtia; review.