Hearing and Balance Function and Preservation in Inner Ear Disease

神经耳科已将其活动重点放在 内耳生物学和医学的两个主要领域。个人 受冯·希佩尔·林道病(VHL)的影响， 原发性肺腺癌所致听力和平衡障碍 内耳的内淋巴囊。一个协作性的IRB 批准的方案用于调查听力和平衡功能以及 通过手术进行保存正在进行中。对这些人的研究，在 与NCI的泌尿外科肿瘤科和 NINDS的外科神经病学分支，提供了一个独特的 内耳生物学的病理模型。分子遗传学研究 已经完成，以定义是否存在某些突变 VHL基因导致了这些罕见的肿瘤。听力学和临床 神经学评估正在进行，以定义自然的 听力损失、平衡障碍和相关耳鸣病史 与肿瘤有关。这些调查可以提供更好的 对内耳生物学的理解，并可作为 内耳疾病，如梅尼埃病。协作努力 与NIAID一起致力于描述 受影响的个体的神经耳病学和听力学特征 慢性神经疏螺旋体病。以类似于在 梅毒中枢神经系统疾病，慢性感染的 由莱姆病原体伯氏疏螺旋体感染中枢神经系统， 与感觉神经性听力障碍的发病机制有关 失落、眩晕或其他前庭障碍，以及耳鸣 莱姆病患者。特殊疾病的发病率 听觉前庭症状，中枢与中枢神经的特征。 外周听觉系统异常及相关因素的影响 针对这些参数的抗菌治疗正在接受 NIDCD神经耳科。

The Neuro-otology Branch has focused its activities on two major areas of inner ear biology and medicine. Individuals affected by von Hippel Lindau disease (VHL) are predisposed to hearing and balance impairment due to primary adenocarcinomas of the endolymphatic sac of the inner ear. A collaborative IRB approved protocol to investigate hearing and balance function and preservation by surgery is underway. Study of these individuals, in collaboration with the urologic oncology branch of the NCI and the surgical neurology branch of the NINDS, has provided a unique pathologic model of inner ear biology. Molecular genetic studies have been completed to define whether certain mutations of the VHL gene result in these rare tumors. Audiologic and clinical neurotologic evaluations are being conducted to define the natural history of hearing loss, balance dysfunction and tinnitus associated with the tumors. These investigations can provide better understanding of inner ear biology and may serve as a model for inner ear diseases such as Meniere's disease. Collaborative efforts with the NIAID have been directed at characterizing the neuro-otologic and audiologic features of individuals affected by chronic neuroborreliosis. In a manner analagous to that seen in syphilitic central nervous system disease, chronic infection of the central nervous system by the lyme pathogen, Borrelia burgdorferi, has been implicated in the pathogenesis of sensorineural hearing loss, vertigo or other vestibular disturbances, as well as tinnitus in individuals with Lyme disease. The incidence of specific audiovestibular symptoms, the characterization of central vs. peripheral auditory system abnormalities, and the effects of antimicrobial therapy on these parameters are being examined by the NIDCD Neuro-otology Branch.