Objective: To investigate the clinical classification and surgical outcomes of congenital middle ear malformations accompanied by facial nerve anomalies. Methods: A retrospective cohort study was conducted to analyze clinical data of patients with congenital middle ear malformations and concomitant facial nerve anomalies who received treatment in the Department of Otolaryngology, Zhujiang Hospital, Southern Medical University, between January 2021 and December 2024. Facial nerve anomalies were categorized into four distinct types: partial obstruction of the oval window, complete obstruction of the oval window, aberrant course over the promontory, and branching anomalies. Ossicular chain malformations were classified in accordance with the Teunissen classification system. Individualized hearing reconstruction strategies were implemented based on the specific anatomical characteristics of the anomalies. All patients were followed up for 3 to 24 months. Postoperative therapeutic efficacy was evaluated by comparing preoperative and postoperative mean air conduction thresholds and air-bone gaps (ABG), while monitoring surgical complications. Results: Thirteen patients (17 ears) aged [M (Q1, Q3)] 15.0 (11.5, 21.0) years were included, with eight males and five females. Facial nerve anomalies were categorized as follows: partial obstruction of the oval window in six ears (three cases of Type Ⅰ, three cases of Type Ⅲ), complete obstruction of the oval window in three ears (one case of Type Ⅰ, two cases of Type Ⅲ), aberrant course over the promontory in six ears (all Type Ⅳ), and branching anomalies in two ears (both Type Ⅳ). Hearing reconstruction modalities employed included: incus-stapedial piston placement following vestibular fenestration in nine ears, malleus-stapedial piston placement following vestibular fenestration in two ears, partial ossicular replacement prosthesis (PORP) implantation in three ears, total ossicular replacement prosthesis (TORP) implantation in one ear, stapes mobilization in one ear, and laser-assisted ossicular chain release in one ear.Statistical analysis revealed that postoperative mean air conduction thresholds [(33.8±15.2) dB HL vs (60.1±13.8) dB HL, P<0.001] and mean ABG [(14.6±6.9) dB vs (37.2±9.2) dB, P<0.001] were significantly reduced compared with preoperative values. Postoperative ABG≤20 dB was achieved in 15 ears (88.2%). Postoperative complications were limited to transient vertigo in five cases and taste disturbance in two cases. No cases of facial paralysis, sensorineural hearing loss, tympanic membrane perforation, or tinnitus were observed. The median follow-up duration was 6.0 (5.5, 20.0) months. Conclusion: Individualized hearing reconstruction strategies based on the classification of facial nerve and ossicular chain anomalies can effectively protect the facial nerve and achieve significant hearing improvement, demonstrating favorable clinical outcomes.
目的： 探讨先天性中耳畸形伴面神经畸形的临床分类及手术疗效。 方法： 回顾性分析2021年1月至2024年12月于南方医科大学珠江医院耳鼻咽喉头颈外科行手术治疗的先天性中耳畸形伴面神经畸形患者临床资料，将面神经畸形分为：部分遮窗、完全遮窗、走行鼓岬表面及分支畸形四类，听骨链畸形根据Teunissen分型，按不同策略重建听力，术后随访3~24个月，比较手术前后气导平均阈值、气骨导差（ABG）变化，观察手术并发症，从而评估手术疗效。 结果： 纳入13例（17耳）患者，男8例，女5例，年龄［M（Q1，Q3）］15.0（11.5，21.0）岁。面神经部分遮窗6耳（Ⅰ型3耳、Ⅲ型3耳），完全遮窗3耳（Ⅰ型1耳、Ⅲ型2耳），走行鼓岬表面6耳（Ⅳ型6耳），分支畸形2耳（Ⅳ型2耳）。9耳前庭开窗Piston连接砧骨，2耳前庭开窗Piston连接锤骨，3耳部分听骨赝复物（PORP），1耳全听骨赝复物（TORP），1耳镫骨撼动术，1耳激光听骨链松解术。与术前相比，术后气导平均听阈［（33.8±15.2）分贝听力级（dB HL）比（60.1±13.8）dB HL，P<0.001］和ABG［（14.6±6.9）dB比（37.2±9.2）dB，P<0.001］均降低。15耳（88.2%）术后ABG≤20 dB。术后5例出现短暂眩晕，2例出现味觉异常。随访时间6.0（5.5，20.0）个月，所有患者无面瘫、感音神经性耳聋、鼓膜破损、耳鸣并发症。 结论： 先天性中耳畸形伴面神经畸形根据面神经畸形和听骨链畸形分类采取不同听力重建策略，可有效保护面神经，显著改善听力，具有良好的临床疗效。.