Endoscopic Surgery for a Case of Congenital Cholesteatoma with Ossicular Chain Anomaly.

The simultaneous occurrence of congenital ossicular anomalies and congenital cholesteatoma (CC) is exceedingly rare. This report describes the case of an 11-year-old boy who presented with conductive hearing loss, characterized by an average air conduction threshold of 50 dB in the left ear, without any prior otologic history. Preoperative assessments suggested the presence of CC, with suspicion of ossicular chain malformation. The patient underwent ear endoscopic surgery, during which the lesion was excised, and the ossicular chain was reconstructed using a titanium ossicular prosthesis. Postoperative follow-up indicated successful healing, with a significant improvement in hearing, as demonstrated by a reduction in the air conduction threshold to 15 and a hearing gain of 35 dB.