目的:丙二醛内神经鞘瘤(ILS)是一个罕见的发现。诊断具有挑战性,目前还没有黄金标准治疗。在这篇文章中,我们提供了两个病例系列,并回顾了最新的文献,以评估最佳的诊断和治疗方案.
方法:我们回顾了评估最常见和相关症状集的最新文献,该疾病的临床特征,诊断测试和成像,可能的治疗方法和术后听力康复技术。然后,我们将文献数据与我们自己的系列数据进行了比较。
结果:ILS的临床表现和发展可能与其他疾病重叠,更常见的耳科条件。全测听电池测试,VEMPS和增强对比的MRI的电生理研究似乎对于正确诊断这些肿瘤至关重要。存在几种治疗方法:放射学随访,放射治疗,完全或部分手术切除。听力康复主要是通过同时植入耳蜗来实现的。
结论:我们的病例系列数据与现有文献相符。ILSs是一种罕见的前庭神经鞘瘤。诊断具有挑战性,并且延迟了所有诊断测试的时间,然而敏感,不是特定于ILS。最合适的治疗方法似乎是手术切除这些肿瘤,然后同时植入耳蜗以恢复听力。
OBJECTIVE: Intralabyrinthine schwannomas (ILSs) are an uncommon finding. Diagnosis is challenging and no gold standard treatment exists yet. In this article, we present a two-cases series and review the latest available literature to assess the best diagnostic and therapeutic scheme.
METHODS: We reviewed the latest available literature assessing most frequent and relevant sets of symptoms, clinical features of the disease, diagnostic tests and imaging, possible treatments and after-surgery hearing rehabilitation techniques. We then compared literature data to our own series ones.
RESULTS: ILSs clinical presentation and development may overlap with other, more common otological conditions. Full audiometric battery test, electrophysiological study of VEMPS and MRI with contrast enhancement all appear to be critical to correctly diagnose these tumors. Several treatments exist: radiological follow-up, radiation therapy, full or partial surgical excision. Hearing rehabilitation is mostly accomplished through simultaneous cochlear implantation.
CONCLUSIONS: Our
case-series data matches the available literature. ILSs are a rare type of vestibular schwannomas. Diagnosis in challenging and delayed in time as all the diagnostic tests, yet sensitive, are not specific for ILSs. The most suitable treatment seems to be surgical excision of these tumors followed by simultaneous cochlear implantation to restore hearing.