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目的 探讨神经纤维瘤病Ⅱ型 (双侧听神经瘤 )的临床特征及治疗决策。方法 回顾性分析 1990年 1月～ 1998年 12月收治的 7例双侧听神经瘤的临床资料。结果 双侧听神经瘤占全部听神经瘤的 5 .8% (7/ 12 2 )。 7例中 ,男 6例 ,女 1例 ;年龄 13～ 6 0岁 (年龄中位数为 2 9 1岁 )。首发症状听力下降及耳鸣 4例 ,斜视和上睑下垂、头痛、步态不稳各 1例。 6例合并有中枢和 /或其它部位神经瘤 ,5例合并皮肤咖啡色斑 ,1例合并晶状体后囊混浊斑。重型 5例 ,轻型 2例。 4例行单侧听神经瘤切除术 ,术后 1例因中枢性呼吸衰竭死亡 ;2例间隔 3周行双侧听神经瘤切除术 ,术后 1例因脑水肿颅高压死亡。手术径路 1例 (1耳 )经迷路后 ,术后无听力受损及面瘫 ;5例 (7耳 )经乙状窦后 ,术后成活的 3例 (4耳 )中 ,4耳术后听力受损 ,3侧有面神经部分或全部麻痹。结论 双侧听神经瘤即神经纤维瘤病Ⅱ型的临床特征和治疗不同于单侧听神经瘤 ,治疗决策对每人及不同阶段应酌情而定。为了避免手术致听力受损和面瘫 ,建议术中监护第Ⅶ、Ⅷ颅神经功能
Objective To investigate the clinical features and treatment decisions of type 2 neurofibromatosis (bilateral acoustic neuroma). Methods The clinical data of 7 cases of bilateral acoustic neuroma treated from January 1990 to December 1998 were retrospectively analyzed. Results Bilateral acoustic neuroma accounted for 5.8% (7/12 2) of all acoustic neuromas. In 7 cases, there were 6 males and 1 female; the age ranged from 13 to 60 years (the median age was 291). The first symptoms were hearing loss and tinnitus in 4 cases, strabismus and ptosis, headache, and gait instability in 1 case. Six patients had central and/or other sites of neuroma, 5 patients had skin caffeine, and 1 had posterior capsular opacities. Heavy in 5 cases, light in 2 cases. Four patients underwent unilateral auditory neuroma tumor resection. One patient died of central respiratory failure after surgery. Two patients underwent bilateral acoustic neuromaectomy at 3 weeks intervals. One patient died of cerebral edema due to intracranial hypertension. In 1 case (1 ear) after operation, there was no hearing impairment and facial paralysis after operation. In 5 cases (7 ears), 3 cases (4 ears) survived after sigmoid sinus, and 4 ears had hearing after operation. Impaired. Partial or total numbness of the facial nerve on the 3 side. Conclusion The clinical features and treatment of bilateral acoustic neuroma, namely neurofibromatosis type II, are different from those of unilateral acoustic neuroma. Treatment decisions should be made for each person and at different stages as appropriate. In order to avoid surgery-induced hearing impairment and facial paralysis, intraoperative ul