Neary W J, Newton V E, Laoide-Kemp S N, Ramsden R T, Griffith G, Evans D G, Harris R, Strachan T
Centre for Audiology, Education of the Deaf and Speech Pathology (CAEDSP), University of Manchester, UK.
J Laryngol Otol. 1996 Jul;110(7):634-40.
Ninety-three patients with unilateral vestibular schwannomas were examined in a clinical, genetic and audiological study, to determine whether they had features associated with neurofibromatosis Type 1 or neurofibromatosis Type 2. In 91 families, one patient only was found to be affected with a unilateral vestibular schwannoma. Patients did have a few café-au-lait macules, but fewer than six in number. None of the patients satisfied the cutaneous diagnostic criteria for neurofibromatosis Type 1. Neither Lisch nodules nor presenile posterior subcapsular lenticular opacities or cortical opacities were a feature. Five patients with unilateral vestibular schwannomas are described where the clinical findings raised the possibility of neurofibromatosis Type 2. It is suggested that certain individuals with unilateral vestibular schwannomas are at risk of developing neurofibromatosis Type 2. Furthermore, the possibility of neurofibromatosis Type 2 should be considered if more than one individual in a family is found to be affected with a unilateral vestibular schwannoma.
对93例单侧前庭神经鞘瘤患者进行了临床、遗传学和听力学研究，以确定他们是否具有与1型神经纤维瘤病或2型神经纤维瘤病相关的特征。在91个家庭中，仅发现1例患者患有单侧前庭神经鞘瘤。患者确实有一些咖啡牛奶斑，但数量少于6个。没有患者符合1型神经纤维瘤病的皮肤诊断标准。既没有Lisch结节，也没有老年性后囊下晶状体混浊或皮质混浊。描述了5例单侧前庭神经鞘瘤患者，其临床发现增加了2型神经纤维瘤病的可能性。提示某些单侧前庭神经鞘瘤患者有发生2型神经纤维瘤病的风险。此外，如果一个家庭中发现不止一人患有单侧前庭神经鞘瘤，则应考虑2型神经纤维瘤病的可能性。
