Acquired Ear Canal Cholesteatoma in Congenital Aural Atresia/Stenosis

Objectives: To estimate the prevalence of external auditory canal (EAC) stenosis in patients with congenital aural atresia (CAA) and the prevalence of acquired ear canal cholesteatoma in patients with EAC stenosis and to identify risk factors that may predict the presence of ear canal cholesteatoma in those patients. Study Design: Retrospective chart review. Setting: University tertiary referral center. Materials and Methods: Patients with EAC stenosis with and without acquired ear canal cholesteatoma were identified from the medical records of 673 patients (770 ears) with CAA. Demographic data, symptoms at presentation, and audiometric data were compared between those with and those without cholesteatoma to identify the risk factors for the presence of ear canal cholesteatoma. Results: Of the 770 ears evaluated, 101 (13.1%) were found to have at least 1 stenotic ear canal. Of this group of 101 ears with canal stenosis, 18 of 94 ears (7 ears were missing data; 19.1%) had a concurrent cholesteatoma, with 1 patient having bilateral cholesteatomas. Demographic, clinical, and audiometric parameters showed that only female sex was associated with a higher rate of ear canal cholesteatoma. Within the cholesteatoma group, right ears in female patients and left ears in male patients predominated. Conclusion: Approximately 1 in 5 patients with congenital aural stenosis were found to have ear canal cholesteatoma. Female sex is a risk factor; basic audiometric parameters provide no diagnostic utility in distinguishing ears with cholesteatoma from those without cholesteatoma.