Glasscock M E, Woods C I, Jackson C G, Welling D B
Department of Neurosurgery, Vanderbilt University School of Medicine, Nashville, Tenn 37203.
Laryngoscope. 1989 May;99(5):475-84. doi: 10.1288/00005537-198905000-00002.
Bilateral acoustic tumors (Neurofibromatosis 2) have now been shown to arise in patients with gene abnormalities separate from those with von Recklinghausen's disease (Neurofibromatosis 1). The management of patients with this disease has long posed a particularly challenging problem for the neurotologist. This retrospective study reviews our series of 38 patients and updates a previous report. Thirty-four patients underwent surgical excision of tumors on one or both sides. Hearing conservation was attempted in 20 with hearing preservation possible in eight. Recent advances in understanding the hereditary etiology of this disease, changing methods for diagnosis, preferred surgical techniques, and guidelines for appropriate patient management are discussed.
双侧听神经瘤(神经纤维瘤病2型)现已证实,其发病的患者存在与冯雷克林霍增氏病(神经纤维瘤病1型)患者不同的基因异常。长期以来,对于耳神经科医生而言,这种疾病患者的治疗一直是个极具挑战性的难题。这项回顾性研究对我们收治的38例患者进行了分析,并更新了之前的报告。34例患者接受了一侧或双侧肿瘤的手术切除。20例患者尝试了听力保留,其中8例听力得以保留。本文还讨论了在该疾病遗传病因认识方面的最新进展、不断变化的诊断方法、首选的手术技术以及合适的患者管理指南。