Abstract：
Objective: To explore the clinical features and treatment strategy of rare tumor in the internal auditory canal(IAC). Methods: 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. Results: 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. Conclusions: 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.
目的： 探讨内听道少见肿瘤的临床特征及治疗策略。 方法： 收集2010年1月至2020年12月武汉大学人民医院收治的213例内听道肿瘤的临床资料，依据影像学、术中所见及病理检查，共发现内听道少见肿瘤7例，其中海绵状血管瘤2例，内听道小脑前下动脉瘤2例，迷路内神经鞘瘤1例，脑膜瘤1例，单侧神经纤维瘤病Ⅱ型（neurofibromatosis type 2，NF2）1例。综合分析其临床表现、影像学资料、治疗措施及预后。 结果： 除听力下降外，海绵状血管瘤早期即出现面神经麻痹，CT上多可见内听道扩大、点状钙化；动脉瘤早期出现耳鸣、眩晕，CT上可见内听道“壶腹样”扩大，数字减影血管造影（DSA）或磁共振血管造影（MRA）有助于确诊；迷路内神经鞘瘤早期常出现难治性眩晕，高分辨率MRI有助于确诊；脑膜瘤增强MRI上可见“脑膜尾征”；神经纤维瘤病2型多为双侧前庭神经鞘瘤，有少数患者仅表现为单侧前庭神经鞘瘤。除1例NF2患者随访观察外，其余患者均行迷路入路病变切除，术后症状均有缓解。随访6个月~3年，未见肿瘤复发。 结论： 内听道少见肿瘤的临床症状和影像学表现各有特点，治疗原则也各不相同，术前明确诊断能够避免盲目手术造成的不良后果。.
目的： 探讨内听道少见肿瘤的临床特征及治疗策略。 方法： 收集2010年1月至2020年12月武汉大学人民医院收治的213例内听道肿瘤的临床资料，依据影像学、术中所见及病理检查，共发现内听道少见肿瘤7例，其中海绵状血管瘤2例，内听道小脑前下动脉瘤2例，迷路内神经鞘瘤1例，脑膜瘤1例，单侧神经纤维瘤病Ⅱ型（neurofibromatosis type 2，NF2）1例。综合分析其临床表现、影像学资料、治疗措施及预后。 结果： 除听力下降外，海绵状血管瘤早期即出现面神经麻痹，CT上多可见内听道扩大、点状钙化；动脉瘤早期出现耳鸣、眩晕，CT上可见内听道“壶腹样”扩大，数字减影血管造影（DSA）或磁共振血管造影（MRA）有助于确诊；迷路内神经鞘瘤早期常出现难治性眩晕，高分辨率MRI有助于确诊；脑膜瘤增强MRI上可见“脑膜尾征”；神经纤维瘤病2型多为双侧前庭神经鞘瘤，有少数患者仅表现为单侧前庭神经鞘瘤。除1例NF2患者随访观察外，其余患者均行迷路入路病变切除，术后症状均有缓解。随访6个月~3年，未见肿瘤复发。 结论： 内听道少见肿瘤的临床症状和影像学表现各有特点，治疗原则也各不相同，术前明确诊断能够避免盲目手术造成的不良后果。.
摘要：
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