Objective To better understand superior semicircular canal dehiscence(SSCD)syndrome. Methods A retrospective study was conducted on 6 patients who were diagnosed with SSCD syndrome in the Otology and Skull Base Surgery group of Fudan University. The clinical presentations including symptoms,signs,auditory tests and high resolution temporal bone computed tomography were reviewed. Results Four patients presented with low frequency heating loss while acoustic reflex responses were intact. Another patient was concomitance with chronic otitis media demonstrated profound sensorineural hearing loss. The sixth patient demonstrated normal hearing. Two patients also complained of autophony,but they were unable to tolerate their own voice. Five patients presented with vertigo while 2 patients were unable to tolerate the environmental noise. All patients showed slow component vertical tortional eye movement away from the effected eye which was induced by the presence of loud sound or pressure in the middle ear or valsalva maneuver. Four patients also demonstrated vertigo induced by the loud sound,1 patient was induced head movement by 110 dB tone. All patients were revealed variable bone defect overlying on the SSC using high resolution temporal bone CT scan with SSC reformation. Conclusions The diagnosis of SSCD syndrome was established on both the presence of bone defect overlying superior semicircular canal which was demonstrated using high resolution temporal bone CT scan,and the presence of associated vestibular and auditory symptoms and signs.%目的 探讨上半规管裂综合征的临床诊断学特点,提高对该病的认识.方法 回顾性分析近期诊断的6例上半规管裂综合征患者,总结其临床症状、体征、听力学和影像学特点.结果 6例患者中4例患耳表现为低频听力下降,声反射均能引出;1例合并有中耳炎表现为极重度感音神经性听力下降;另1例听力正常.2例次患者有明显的自声增强,不能耐受自己说话的声音.5例次患者有不同程度的眩晕,眩晕持续时间数小时不等,程度不一.2例次患者主诉不能耐受外界噪声.4例次患者利用强声能诱导眩晕.所有患者在强声刺激或增加中耳压力、捏鼻鼓气时能观察到慢相向健侧的垂直旋转性眼球运动,其中1例患者强声刺激能诱发头部运动.所有患者均行颞骨CT检查,并行上半规管重建显示上半规管表面不同程度的骨质缺损.2例患者全身麻醉下行上半规管裂填塞修补术,术后症状明显改善.结论 上半规管裂隙综合征的诊断主要依据颞骨高分辨率CT提示上半规管表面覆盖的骨质缺损,同时患者具有相关的前庭耳蜗症状.
展开▼
