Variety of Audiologic Manifestations in Patients With Superior Semicircular Canal Dehiscence
Variety of Audiologic Manifestations in Patients With Superior Semicircular Canal Dehiscence
复制标题
DOI：
10.1097/mao.0b013e3181bc35ce
复制
发表时间：
2010-01-01
影响因子：
2.1
通讯作者：
Dai, Chun-Fu
中科院分区：
文献类型：
--
作者：
Chi, Fang-Lu;Ren, Dong-Dong;Dai, Chun-Fu
目的：介绍11例上级半规管裂开（SSCD）综合征患者的不同症状、体征和诊断试验结果，以及3例患者的手术治疗。研究设计：回顾性病例分析。设置：三级神经耳科和听力学中心。患者：通过病史、体格检查、前庭功能测试和高分辨率计算机断层扫描记录的SSCD患者。干预：2例行颅中窝入路手术，1例行经乳突修补术。结果：11例患者确诊为SSCD，其余均行保守治疗。临床表现多样，包括1）听力表现：混合性听力损失（4/11），传导性听力损失（3/11），极重度感音神经性听力损失（2/11），听力正常（2/11）; 2）前庭表现：慢性失衡（8/11）、Tullio现象（7/11）、Hennebert征（8/11）、耳鸣（3/11）和自鸣（3/11）;和3）伴随疾病：脑脊膜膨出（1/11）。3例患者出现异常眼震。所有病例均经高分辨率计算机断层扫描证实上级半规管上方的骨裂开。3例患者接受手术治疗。结论：上级半规管裂的临床表现多样，复杂，手术方法简单，易于掌握。盖骨或岩骨稀疏或轻度裂开可能是发展为SSCD的先兆或侵袭性原因。未观察到裂开的大小与前庭症状的严重程度或听力损失的程度之间存在相关性。手术修复裂开可以缓解症状，发病率低。
Objective: 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.Study Design: Retrospective case review.Setting: Tertiary neurotologic and audiologic center.Patients: Patients with SSCD documented by history, physical examination, vestibular function testing, and high-resolution computed tomographic scans.Intervention: Two patients underwent surgical procedures through middle fossa approach, and 1 patient underwent transmastoid repair. The rest underwent conservative treatments.Results: 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).Conclusion: 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.