Transcanal Endoscopic Ear Surgery for Congenital Cholesteatoma: A Multi-institutional Series

Objective: To assess outcomes of transcanal endoscopic ear surgery (TEES) for congenital cholesteatoma. Study Design: Case series with chart review of children who underwent TEES for congenital cholesteatoma over a 10-year period. Setting: Three tertiary referral centers. Methods: Cholesteatoma extent was classified according to Potsic stage; cases with mastoid extension (Potsic IV) were excluded. Disease characteristics, surgical approach, and outcomes were compared among stages. Outcomes measures included residual or recurrent cholesteatoma and audiometric data. Results: Sixty-five cases of congenital cholesteatoma were included. The mean age was 6.5 years (range, 1.2-16), and the mean follow-up was 3.9 years (range, 0.75-9.1). There were 19 cases (29%) of Potsic stage I disease, 10 (15%) stage II, and 36 (55%) stage III. Overall, 24 (37%) patients underwent a second-stage procedure, including 1 with Potsic stage II disease (10%) and 21 (58%) with Potsic stage III disease. Eight cases (12%) of residual cholesteatoma occurred. One patient (2%) developed retraction-type (“recurrent”) cholesteatoma. Recidivism occurred only among Potsic stage III cases. Postoperative air conduction hearing thresholds were normal (<25 dB HL) in 93% of Potsic stage I, 88% of stage II, and 36% of stage III cases. Conclusion: TEES is feasible and effective for removal of congenital cholesteatoma not extending into the mastoid. Recidivism rates were lower with the TEES approach in this large series than in previously reported studies. Advanced-stage disease was the primary risk factor for recidivism and worse hearing result. As minimally invasive TEES is possible in the youngest cases, children benefit from early identification and intervention.