Congenital Aural Stenosis With Cholesteatoma

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).

Retrospective case series.

Tertiary care hospital.

Patients with CAS (with and without cholesteatoma) and CCAA.

Surgery for CAS/CAA.

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.

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).

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.