Minor L B
Department of Otolaryngology-Head & Neck Surgery, The Johns Hopkins University School of Medicine, Baltimore, Maryland, USA.
Am J Otol. 2000 Jan;21(1):9-19.
To present the symptoms, signs, and findings on diagnostic tests of patients with the superior canal dehiscence syndrome and to describe the surgical procedures used to treat the dehiscence in five patients.
Prospective study of a series of patients identified as having this syndrome at a tertiary care referral center.
Seventeen patients with vertigo, oscillopsia, or both evoked by intense sounds or stimuli that caused changes in middle ear and/or intracranial pressure were identified over a 4-year period. The evoked eye movements had vertical and torsional components, with the direction corresponding to the effect of the stimuli in causing excitation (Valsalva against pinched nostrils, tragal compression, sounds) or inhibition (Valsalva against a closed glottis or jugular venous compression) of the affected superior semicircular canal. Thirteen (76%) of these patients also experienced chronic dysequilibrium that was often the most debilitating symptom. Dehiscence of bone overlying the superior semicircular canal on the affected side was confirmed with computed tomographic scans in each case. Surgical procedures through the middle fossa approach to plug or resurface the superior canal were performed in five patients (canal plugging in three cases and resurfacing of the dehiscence without plugging in two). The debilitating symptoms resolved or improved after the procedures. Signs of vestibular hypofunction, without loss of hearing, were noted in one patient after plugging of the superior canal and in one other patient after resurfacing of the canal.
The superior canal dehiscence syndrome is identified based on characteristic symptoms, signs, and computed tomographic findings. The clinical presentation and findings can be understood in terms of the effect of the dehiscence on the physiology of the labyrinth. The syndrome is a treatable cause of vestibular disturbance.
阐述上半规管裂综合征患者的症状、体征及诊断检查结果，并描述用于治疗5例患者上半规管裂的手术方法。
在一家三级医疗转诊中心对一系列确诊患有该综合征的患者进行前瞻性研究。
在4年期间，共识别出17例因强烈声音或引起中耳和/或颅内压变化的刺激而诱发眩晕、视振荡或两者皆有的患者。诱发的眼球运动具有垂直和扭转成分，其方向与刺激对患侧上半规管产生兴奋（瓦尔萨尔瓦动作对抗捏紧的鼻孔、耳屏按压、声音）或抑制（瓦尔萨尔瓦动作对抗紧闭的声门或颈静脉压迫）的效果相对应。其中13例（76%）患者还经历了慢性平衡失调，这通常是最使人衰弱的症状。通过计算机断层扫描证实，每例患者患侧上半规管上方均存在骨质缺损。5例患者接受了经中颅窝入路封堵或修复上半规管的手术（3例进行了半规管封堵，2例进行了未封堵的裂孔修复）。术后，使人衰弱的症状得到缓解或改善。1例患者在封堵上半规管后，另1例患者在修复半规管后，出现了前庭功能减退但无听力丧失的体征。
上半规管裂综合征可根据特征性症状、体征及计算机断层扫描结果进行诊断。根据裂孔对迷路生理功能的影响，可以理解该综合征的临床表现及检查结果。该综合征是前庭功能障碍的可治疗病因。
