Department of Otology & Skull Base Surgery, Eye and ENT Hospital, Fudan University, Shanghai, People's Republic of China.
Otol Neurotol. 2010 Jan;31(1):2-10. doi: 10.1097/mao.0b013e3181bc35ce.
To present diverse symptoms, signs, and findings on diagnostic tests of 11 patients with superior semicircular canal dehiscence (SSCD) syndrome and surgical treatments for dehiscence of 3 patients.
Retrospective case review.
Tertiary neurotologic and audiologic center.
Patients with SSCD documented by history, physical examination, vestibular function testing, and high-resolution computed tomographic scans.
Two patients underwent surgical procedures through middle fossa approach, and 1 patient underwent transmastoid repair. The rest underwent conservative treatments.
Eleven patients were identified as SSCD. The variety of clinical manifestations, including 1) hearing presentations: mix hearing loss (4 of 11), conductive hearing loss (3 of 11), profound sensorineural hearing loss (2 of 11), and normal hearing (2 of 11); 2) vestibular manifestations: chronic disequilibrium (8 of 11), Tullio phenomenon (7 of 11), Hennebert sign (8 of 11), tinnitus (3 of 11), and autophony (3 of 11); and 3) accompanying disorders: encephalomeningocele (1 of 11). Abnormal nystagmus was identified in 3 patients. Dehiscence of bone overlying superior semicircular canal was confirmed by high-resolution computed tomographic scan in all cases. Three patients underwent operative management. (2 through the middle fossa approach and 1 through a transmastoid repair).
Superior semicircular canal dehiscence demonstrates diverse and complex clinical features. Tegmental or petrosal bone rarefaction or mild dehiscences can be a harbinger or an aggressive cause of developing SSCD. No correlation was observed between the size of dehiscence and the severity of vestibular symptoms or the degree of hearing loss. Surgical repair of dehiscence can relieve symptoms with low morbidity.
介绍 11 例上半规管裂（SSCD）综合征患者的不同症状、体征和诊断性检查结果，并介绍 3 例患者的裂孔修补术治疗。
回顾性病例分析。
三级神经耳科和听力中心。
病史、体格检查、前庭功能检查和高分辨率计算机断层扫描证实为 SSCD 的患者。
2 例患者采用经中颅窝入路手术，1 例患者采用经乳突修补术。其余患者采用保守治疗。
共确诊 11 例 SSCD 患者。临床表现多样，包括 1）听力表现：混合性听力损失（11 例中的 4 例）、传导性听力损失（11 例中的 3 例）、重度感音神经性听力损失（11 例中的 2 例）和正常听力（11 例中的 2 例）；2）前庭表现：慢性平衡障碍（11 例中的 8 例）、Tullio 现象（11 例中的 7 例）、Hennebert 征（11 例中的 8 例）、耳鸣（11 例中的 3 例）和自声增强（11 例中的 3 例）；3）伴随疾病：脑脊髓膨出（11 例中的 1 例）。3 例患者存在异常眼震。所有患者的高分辨率计算机断层扫描均证实上半规管上方骨缺损。3 例患者接受了手术治疗（2 例经中颅窝入路，1 例经乳突修补术）。
上半规管裂表现出多样而复杂的临床特征。桥脑或岩骨稀疏或轻度裂孔可能是 SSCD 发展的先兆或侵袭性病因。裂孔大小与前庭症状严重程度或听力损失程度之间无相关性。裂孔修补术可缓解症状，且发病率低。
