LAUSR

Abstract Background A few studies have reported transcanal endoscopic management of isolated congenital middle ear malformations (CMEMs). Objective The purpose of this study is to describe our surgical experience in endoscopic ear surgery for isolated CMEMs and evaluate the surgical effect of hearing reconstruction. Methods From January 2017 to January 2022, a retrospective study was performed on 36 patients (37 ears) with isolated CMEMs who all underwent endoscopic surgery. Demographic data, high-resolution computed tomography (HRCT) findings, intraoperative findings, surgical management and audiometric data were recorded. Results Anomalies were categorized according to the Teunissen and Cremers classification system: 8 ears were categorized as class I, 8 ears as class II, 19 ears as class III and 2 ears as class IV. The air conduction pure tone average (AC-PTA) of 37 cases was 61.5 ± 8.6 dB preoperatively and 29.6 ± 6.9 dB postoperatively (p < 0.001). The mean preoperative air-bone gap (ABG) significantly decreased from 43.1 ± 8.7 dB to 12.8 ± 5.5 dB postoperatively. 36 of 37 cases (97%) met the criteria for successful operation. Conclusion Isolated CMEMs are mainly manifested as aplasia of the stapes’ superstructure and dysplasia of the long process of the incus. Transcanal endoscopic surgery seems a safe technique for the management of isolated CMEMs.