双侧听神经瘤

Bilateral acoustic neuromas

目的探讨双侧听神经瘤的临床表现、生长特性及治疗。方法回顾性分析１９８７年６月～１９９７年５月间收治５例双侧听神经瘤的临床资料。结果本病约占全部听神经瘤患者的５％（５／９２）。５例的首发症状均为耳鸣伴听力减退。来诊时除位听神经症状外，有多个颅神经和脑干受累的临床表现。２例有孤立的浅褐色皮肤色素斑，５例有数量不等、分布不一的其它神经纤维瘤。５例中４例行一侧听神经瘤切除术，其中１例术后因脑水肿、高颅压、脑疝死亡。对１例术前、术后颅脑冠状位和轴位ＭＲＩ检查表明，其肿瘤平均每年朝上下、左右方向各生长１．１ｃｍ和０．７ｃｍ。结论双侧听神经瘤的治疗与单侧听神经瘤有不同。

Objective To investigate the clinical course, growth character and treatment of bilateral acoustic neuromas (BANs).Methods Five cases of BANs were reported.Results From 1986 to 1996, 92 patients with acoustic tumors were managed. Of them, 5(5%) bilateral acoustic neuromas were observed. All patients were males, the age at the time of testing ranged from 16 to 31 years (mean 23.8 years). In all patients, progressive hearing loss and tinnitus were the most common initial symptoms. In addition, signs of other cranial nerve deficits were detected in all patients. Two cases had cafe au lait spots and all cases had neurofibromas of varying sizes and shapes in subcutaneous tissues. Four had unilateral acoustic tumors operated on, one by the translabyrinthine approach, 3 by the suboccipital approach. One patient died due to encephaledema and hernia of brain after operation. According to the MRI data in one case, the average growth rate of acoustic tumor was 1.1cm (superior/inferior) and 0.7cm (medial/lateral) per year.Conclusion The management of BANs patients presents challenging problems different from the unilateral acoustic tumors.