HEARING AND BALANCE FUNCTION AND PRESERVATION IN INNER EAR DISEASE

神经耳科的活动主要集中在内耳生物学和医学两个主要领域。受von Hippel Lindau病（VHL）影响的个体由于内耳内淋巴囊的原发性腺癌而易患听力和平衡障碍。一项经IRB批准的合作方案正在进行中，以调查听力和平衡功能以及手术保护。与NCI泌尿肿瘤学分会和nds外科神经病学分会合作，对这些个体的研究提供了一种独特的内耳生物学病理模型。分子遗传学研究已经完成，以确定VHL基因的某些突变是否导致这些罕见的肿瘤。正在进行听力学和临床神经学评估，以确定与肿瘤相关的听力损失，平衡功能障碍和耳鸣的自然史。这些研究可以更好地了解内耳生物学，并可能作为内耳疾病如梅尼埃尔病的模型。与NIAID的合作努力一直针对慢性神经螺旋体病患者的神经-耳科和听力学特征。与梅毒中枢神经系统疾病类似，莱姆病病原体伯氏疏螺旋体对中枢神经系统的慢性感染与莱姆病患者的感音神经性听力丧失、眩晕或其他前庭障碍以及耳鸣的发病机制有关。NIDCD神经耳科正在研究特定听庭症状的发生率，中枢与外周听觉系统异常的特征，以及抗菌治疗对这些参数的影响。-内耳，内淋巴囊，莱姆病，伯氏疏螺旋体，耳鸣，眩晕-人类受试者和人类受试者：访谈，问卷调查或仅调查

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 Menieres 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. - inner ear, endolymphatic sac, Lyme Disease, Borrelia, tinnitus, vertigo - Human Subjects & Human Subjects: Interview, Questionaires, or Surveys Only