Yang B Q, Yang X L, Wu Z Y, Wang L, Ren J, Wang W J, Hua Q Q
Department of Otorhinolaryngology Head and Neck Surgery, Renmin Hospital of Wuhan University, Wuhan 430060, China.
Zhonghua Er Bi Yan Hou Tou Jing Wai Ke Za Zhi. 2022 Jul 7;57(7):843-847. doi: 10.3760/cma.j.cn115330-20210809-00537.
To explore the clinical features and treatment strategy of rare tumor in the internal auditory canal(IAC). A retrospective study was carried out in 213 patients with lesion of ICA form January 2010 to December 2020. According to imaging features, surgical findings, and pathological diagnosis, there were 7 cases of non-sporadic acoustic neuroma, including 2 cases of cavernous hemangioma, 2 cases of aneurysm, 1 case of intralabyrinthical schwannoma, 1 case of meningioma, and 1 case of unilateral neurofibromatosis type 2 (NF2). The clinical manifestations, imaging data and intraoperative conditions as well as the formulation of individualized treatment strategies and prognosis were comprehensively analyzed. In addition to hearing loss, cavernous hemangioma early appeared damage of facial nerve. CT showed expansion and calcification of IAC. Patients with aneurysm appeared tinnitus and vertigo early. CT showed enlargement of ampulla shape of IAC. DSA or MRA can help confirm the diagnosis. Patients with intralabyrinthine schwannoma early appeared refractory vertigo. High resolution MRI was helpful for diagnosis. "Dural tail sign" can be seen on enhanced MRI of meningeoma. Neurofibromatosis type 2 usually presented as bilateral vestibular schwannomas,but a few patients presented only with unilateral vestibular schwannomas.. All patients underwent labyrinth approach resection except one patient with NF2 for followed-up. Their postoperative symptoms were relieved. No tumor recurrence was observed during 6-3 years of follow-up. The clinical and imaging manifestations of rare tumors of the internal auditory canal are different. The principle of treatment is also different. It is helpful to avoid the serious consequences caused by blind operation to confirm diagnosis before operation.
探讨内听道（IAC）罕见肿瘤的临床特征及治疗策略。对2010年1月至2020年12月期间213例IAC病变患者进行回顾性研究。根据影像学特征、手术所见及病理诊断，其中非散发性听神经瘤7例，包括海绵状血管瘤2例、动脉瘤2例、迷路内神经鞘瘤1例、脑膜瘤1例、单侧2型神经纤维瘤病（NF2）1例。综合分析其临床表现、影像学资料、术中情况以及个体化治疗策略的制定和预后。海绵状血管瘤除听力损失外，早期出现面神经损伤。CT显示IAC扩大及钙化。动脉瘤患者早期出现耳鸣和眩晕。CT显示IAC壶腹形扩大。DSA或MRA有助于确诊。迷路内神经鞘瘤患者早期出现难治性眩晕。高分辨率MRI有助于诊断。脑膜瘤增强MRI可见“脑膜尾征”。2型神经纤维瘤病通常表现为双侧前庭神经鞘瘤，但少数患者仅表现为单侧前庭神经鞘瘤。除1例NF2患者进行随访外，所有患者均行迷路入路切除术。术后症状均缓解。随访6至3年未见肿瘤复发。内听道罕见肿瘤的临床及影像学表现各异，治疗原则也不同。术前明确诊断有助于避免盲目手术造成的严重后果。
