8岁儿童单侧先天性卵圆窗闭锁伴面神经走行异常的迷路开窗术：一例病例报告

文献信息

中文摘要

背景： 鼓膜正常的儿童传导性听力损失可能由先天性中耳畸形引起，这类畸形在诊断和手术方面均具有挑战性。本文报告一例8岁女孩，其单侧传导性听力损失与复杂中耳畸形相关，并成功接受了迷路开窗术治疗。
方法： 该患者因学校筛查发现的右侧听力损失就诊。耳镜检查结果无异常。纯音测听显示明显的传导性听力损失，气导（AC）阈值为72 dB HL，骨导（BC）阈值为23 dB HL。颞骨计算机断层扫描显示右侧卵圆窗闭锁，伴面神经走行异常及镫骨影异常。双侧外半规管发育不全也被发现为相关异常。右耳探查性鼓室切开术证实卵圆窗完全闭锁，并见脱离的镫骨足板漂浮于中耳腔内。使用CO2激光在推测的卵圆窗位置创建开窗，随后放置活塞式假体。
结果： 术后听力显著改善，AC阈值为20 dB HL，BC阈值为15 dB HL，并在3年随访期内保持稳定。
结论： 本病例突出表明，迷路开窗术是治疗伴面神经走行异常的先天性卵圆窗闭锁儿科患者的有效手术选择。

英文摘要

Conductive hearing loss in children with a normal tympanic membrane may result from congenital middle ear anomalies that pose diagnostic and surgical challenges. Here, we report a case of an 8-year-old girl with unilateral conductive hearing loss associated with complex middle ear malformation that was successfully treated with vestibulotomy. She presented with right-sided hearing loss that was identified during a school screening. Otoscopic examination findings were unremarkable. Pure-tone audiometry revealed significant conductive hearing loss, with an air-conduction (AC) threshold of 72 dB HL and a bone conduction (BC) threshold of 23 dB HL. Temporal bone computed tomography demonstrated right-sided oval window atresia with an aberrant facial nerve course and an abnormal stapes shadow. Bilateral lateral semicircular canal aplasia was also identified as an associated anomaly. Exploratory tympanotomy of the right ear confirmed complete oval window atresia and a detached stapes footplate floating within the middle ear cavity. A fenestration was created at the presumed oval window using a CO2 laser, followed by placement of a piston prosthesis. Postoperatively, hearing improved significantly, with an AC threshold of 20 dB HL and a BC threshold of 15 dB HL, and remained stable over the 3-year follow-up period. This case highlights vestibulotomy as an effective surgical option for pediatric patients with congenital oval window atresia and an aberrant facial nerve course.