Objective The objective of this study is to investigate the clinical manifestations of the superior semicircular canal dehiscence syndrome (SSCDS) and the treatment strategies.Methods Data from 20 cases diagnosed with SSCDS from September 2004 to December 2014 were retrospectively analyzed in this study.The clinical presentations including symptoms,signs,audiological and vestibular function examination,and their imaging characteristics and treatment strategies were reviewed.Results All of the patients demonstrated variable degree of vertigo.Four patients could not tolerate the environmental noise.Autophony was noted in nine cases,among whom,two patients could feel their eye movements and heart beat,one patient could feel his footstep,one patient couldn't endure the singing by himself.Slow component vertical tortional eye movement away from the effected eye were observed in twelve patients when loud noise was given or middle ear or intracranial pressure increased.Head movement was induced by loud noise in one case.Ten patients presented with low frequency hearing loss.One case was concomitance with chronic otitis media and demonstrated severe sensorineural hearing loss.Nine patients demonstrated normal hearing.Decreased thresholds were showed by VEMP examination in six cases.Variable bone defect overlying in the SSCDS was confirmed by CT scans in all of the cases.The surgical repair of the superior semicircular canal dehiscence was performed through the middle cranial fossa approach in three cases and mastoid approach in two cases.The dizziness and the autophony were significantly alleviated after surgery.Conclusions The clinical manifestations of SSCDS mainly demonstrate dizziness,autophony and loss of low frequency hearing.Treatment strategies are mainly composed of avoidance of environmental noise and shouting voice of the patients themselves.Surgical repair of the superior semicircular canal dehiscence was proposed to conduct,either through the middle cranial fossa approach or mastoid approach,when the patient couldnt tolerate the sypmtoms.%目的 分析上半规管裂综合征患者的临床表现及干预方法,提高对该病的认识.方法 回顾性分析2004年9月至2014年12月临床诊断为上半规管裂综合征的20例患者的临床资料,总结其临床症状、体征、听力学和前庭功能检查、影像学特点以及处理策略.结果 20例患者中男性12例,女性8例;年龄26 ～ 69岁,平均46.2岁.所有患者均有不同程度的眩晕.4例患者明显不能耐受外界噪声;9例患者自诉有明显的自声增强,不能耐受自己大声叫喊的声音,其中2例能闻及眼球运动及心跳声,1例不能耐受自己唱歌,1例能感觉到自己脚步的声音.12例患者在强声或增加中耳、颅内压时能观察到慢相向健侧的垂直旋转性眼球运动,其中1例强声刺激可诱发头部运动.10例患者患耳表现为低频听力下降(主要为轻度传导性聋);1例合并中耳炎,表现为重度感音神经性听力下降;其余9例无听力下降.6例患者进行了颈肌前庭诱发肌源性电位(cVEMP)检查,均显示患侧阈值较正常侧降低.所有患者均行高分辨率颞骨CT扫描及上半规管重建,显示上半规管表面存在不同程度的骨质缺损.治疗上,15例行避免外界噪声、自己大声叫喊、避免头部外伤等保守治疗;5例患者行上半规管裂填塞修补术,其中3例经颅中窝径路、2例经乳突径路,术后眩晕、自声增强等症状明显改善,听力无下降.结论 上半规管裂综合征的临床表现主要有眩晕、自声增强、低频听力下降等.干预策略主要为避免外界噪声刺激、自己大声叫喊等保守治疗.当患者有无法忍受的临床症状时可考虑手术治疗,可经颅中窝径路或乳突径路行上半规管裂填塞修补术.
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