Congenital Aural Stenosis With Cholesteatoma

Objective: Compare surgical and audiological outcomes of patients with congenital aural stenosis (CAS) with cholesteatoma to patients with CAS without cholesteatoma and patients with complete congenital aural atresia (CCAA). Study Design: Retrospective case series. Setting: Tertiary care hospital. Patients: Patients with CAS (with and without cholesteatoma) and CCAA. Intervention: Surgery for CAS/CAA. Main Outcome Measures: Patients with CAS and CAA undergoing surgical repair from June 2004 to July 2020 were identified from an institutional database. Included patients were divided by presence of a canal cholesteatoma. Clinical history, pre- and postoperative audiometric data, and clinical outcomes were compared. Results: Of the 283 patients (300 ears), 18 (19 ears) had a canal cholesteatoma. When compared to ears without cholesteatoma (CCAA ears plus CAS ears without cholesteatoma), ears with cholesteatoma were more likely to be younger (9.2 +/- 6.6 vs. 11.5 +/- 9.2; p = 0.015), female (66.7% vs. 38.1%; p = 0.02; OR 3.2, 95% CI 1.18-8.9), and have normal/Grade I microtia (47.4% vs. 9.6%; p < 0.0001; OR 0.12, 95% CI 0.044-0.32), but not a history of draining ear (5.3% vs. 0%; p = 0.05; OR 0.06, 95% CI 0.004-0.999). Preoperative audiometric data demonstrated a lower mean air-bone gap (45.8 dB vs. 52.3 dB; p = 0.009) and better speech reception threshold (48.7 dB vs. 57.4 dB; p = 0.0004) in cholesteatoma ears. Postoperatively, ears with cholesteatoma were more likely to close the ABG within 20 dB (p = 0.001; OR 0.19, 95% CI 0.072-0.52). No patient in the cholesteatoma group developed post-operative bony/soft-tissue stenosis (0% vs. 9.7%; p = 0.65; OR 1.61; 0.21-12.6) or required revision surgery (0% vs. 11%; p = 0.38; OR 2.46, 0.32-19). Conclusions: Patients with CAS and cholesteatoma have better audiometric outcomes and likely a more durable repair with a decreased need for revision possibly secondary to greater embryologic development of the meatus, ear canal, and middle ear space despite the cholesteatoma.