Chung Lawrance K, Lagman Carlito, Nagasawa Daniel T, Gopen Quinton, Yang Isaac
Neurosurgery, David Geffen School of Medicine, University of California, Los Angeles.
Department of Head and Neck Surgery, David Geffen School of Medicine, University of California, Los Angeles.
Cureus. 2017 Apr 6;9(4):e1141. doi: 10.7759/cureus.1141.
Superior semicircular canal dehiscence (SSCD) is a bony defect in the middle cranial fossa floor that results in an abnormal connection between the inner ear and cranial vault. Although the etiology of SSCD remains unclear, an inappropriately thin or fragile temporal bone likely predisposes an individual towards developing SSCD. Ehlers-Danlos syndrome (EDS) constitutes a group of genetic connective tissue disorders caused by a defect in the production, processing, or structure of collagen, or its associated proteins. The possible association between SSCD and EDS has not been previously described in the literature. We herein report a case of a 50-year-old female with EDS-hypermobility type who presented with a 15-year history of migraines, vertigo, and tinnitus. The patient was subsequently diagnosed with bilateral SSCD and underwent a right middle fossa (pre-auricular infratemporal) craniotomy for SSCD repair. She reported significant improvement in her auditory and vestibular symptoms, with the exception of continued mild dizziness and disequilibrium at the 3-month follow-up. Due to the rare reports of auditory symptoms in EDS, this case study highlights the importance of considering an otological consultation for auditory manifestations in a patient with EDS and illustrates a potential association between EDS and SSCD.
上半规管裂（SSCD）是颅中窝底的一种骨质缺损，导致内耳与颅顶之间出现异常连接。尽管SSCD的病因尚不清楚，但颞骨过薄或脆弱可能使个体易患SSCD。埃勒斯-当洛综合征（EDS）是一组由胶原蛋白或其相关蛋白的产生、加工或结构缺陷引起的遗传性结缔组织疾病。SSCD与EDS之间可能存在的关联此前在文献中尚未有描述。我们在此报告一例50岁女性，患有活动过度型EDS，有15年偏头痛、眩晕和耳鸣病史。该患者随后被诊断为双侧SSCD，并接受了右侧颅中窝（耳前颞下）开颅手术以修复SSCD。她报告听觉和前庭症状有显著改善，但在3个月随访时仍有持续的轻度头晕和平衡失调。由于EDS中听觉症状的报告罕见，本病例研究强调了对EDS患者的听觉表现进行耳科会诊的重要性，并说明了EDS与SSCD之间的潜在关联。
