罕见耳蜗-面神经裂1例报告及文献复习

A Case Study and Literature Review of Cochlear-Facial Dehiscence

目的通过分析1例罕见耳蜗-面神经裂的临床表现、影像学特征并结合文献复习,初步探讨该类疾病的病因、发病机制及诊疗情况。方法对患者进行听觉功能、前庭功能以及颞骨CT检查并查阅相关文献。结果该患者纯音测听提示双耳低-中频(≤1000 Hz)传导性听力下降,左耳气骨导差平均为20 dB HL,右耳气骨导差平均为15 dB HL;颞骨高分辨率CT提示双侧耳蜗底转与面神经迷路段无骨性分隔,以左侧尤为明显。结论耳蜗-面神经裂为临床罕见的骨迷路裂疾病,其发病机制与"内耳第三窗"有关,常表现为低频传导性或混合性听力损失、搏动性耳鸣及自听过响等,颞骨薄扫CT对于诊断该疾病具有重要价值。

Objective To describe a rare case with cochlear-facial dehiscence(CFD).Combined with literature review,we comprehensively analyze the etiology,pathogenesis,diagnosis and treatment of CFD.Methods The audiometry,vestibular function and temporal bone HRCT were examined and related literatures were reviewed.Results The pure tone audiometry of the patient indicated that the conductive hearing of the both ears decreased at(1000 Hz).The average difference of the air bone conduction in the left ear was 20 dB HL,and 15 dB HL in the right ear.High-resolution CT of temporal bone revealed no bony margin between the superior portion of the basal turn of the cochlea and labyrinthine segment of the facial nerve bilaterally,especially for the left.Conclusion CFD is a rare otic capsule dehiscence related disease,the pathogenesis of which is related to acting as pathologic third window in the inner ear.Clinically,it is usually manifested as low frequency conductive hearing loss,pulsating tinnitus and self-audibility.The temporal bone HRCT plays an important role in the diagnosis of CFD.