An exclusive endoscopic transcanal treatment of congenital cholesteatoma: A multi-institutional study.

Purpose: To present surgical and audiological outcomes of the pure endoscopic transcanal approach in the treatment of congenital cholesteatoma (CC).

Methods: The present retrospective multicentre study included 34 patients who underwent exclusive transcanal endoscopic ear surgery (ETEES) for CC treatment by senior surgeons with expertise in endoscopic ear surgery. The extent of the CC was classified according to the Potsic stage. A comprehensive investigation was conducted, encompassing a multitude of parameters, including patients' demographics, CC characteristics, ossicular chain status, ossiculoplasty techniques, operative time, complications, residual or recurrent cholesteatoma, and audiological outcomes.

Results: Of the 34 CC patients enrolled in the study, 19 (55.9%) were male and 15 (44.1%) were female; their mean age was 15.2 ± 6.1 years, and their average follow-up period was 36.4 ± 10.2 months. There were 11 cases (32.4%) of Potsic stage I CC, 7 (20.6%) stage II, 10 (29.4%) stage III, and 6 (17.6%) stage IV. The average operating time was 86.2 ± 16.9 min (ranging 40-180 min). Overall, 9 (26.5%) patients underwent a second-look and revision surgery via ETEES approach, due to conductive hearing loss and recurrence, in 4 (11.8%) cases with Potsic stage III and in 2 (5.8%) cases with Potsic stage IV iatrogenic and residual CC were observed. The preoperative air-bone gap (ABG) values demonstrated a notable postoperative improvement, declining from a mean of 31.4 ± 8.1 dB preoperatively to 13.5 ± 3.6 dB (p < 0.001).

Conclusion: ETEES, with the advantage of being able to visualize concealed areas of the middle ear with minimal bone work, is a feasible, safe, and effective surgical technique in the treatment of CC, even when CC extends into the mastoid, offering comparable audiological outcomes with lower recurrence ratios relative to conventional surgical techniques present in the current literature.