Optopathology by Light Microscopy and Molecular Techniques

描述(由申请人提供)：疾病的病理基础知识是医学研究的核心，包括影响听觉和前庭系统的障碍。耳科的独特之处在于，在生活中无法接触到内耳，因此，常规的病理研究技术，如活检和手术切除是不可行的。因此，只有通过对颞骨的尸检研究和开发可信的动物模型，才能深入了解耳病的病理基础。对影响听力和前庭系统的疾病的病理和发病机制的更好的了解将导致对这些疾病的更合理的诊断和临床处理。人类颞骨的获取、加工和研究是一项耗时和昂贵的工作，超出了临床病理学部门的范围，因此是一项在颞骨实验室进行的研究工作。这项建议是根据RFA-DC-11-003中宣布的旨在建立NIDCD耳部病理学研究合作网络的指导方针制定的。我们的目标包括以下几个方面：1.研究耳蜗前庭神经鞘瘤所致听力损失的病理机制。2.探讨耳硬化症的放射学-组织学相关性，以及耳硬化症累及耳蜗时感音神经性聋的发病机制。3.研究人内耳感觉和非感觉细胞周转的动力学。4.建立培养未来颞骨研究人员的计划。5.与NIDCD耳科病理学研究协作网络合作。这项拟议的研究旨在为耳硬化症、听神经瘤和内耳细胞更替的病理学和病理生理学提供新的知识和见解，进而带来更好的诊断和治疗方法。

DESCRIPTION (provided by applicant): Knowledge of the pathologic basis of disease is central to the study of medicine, including disorders affecting the auditory and vestibular systems. Otology is unique in that the inner ear is inaccessible during life and so, conventional techniques of pathologic study such as biopsy and surgical excision are not feasible. Consequently, insight into the pathologic basis of ear disease can only be obtained by postmortem study of temporal bones and by developing credible animal models. An improved understanding of the pathology and pathogenesis of disorders affecting the auditory and vestibular systems should lead to more rational diagnosis and clinical management of these diseases. The procurement, processing and study of human temporal bones is a time-consuming and costly endeavor which lies outside the purview of clinical departments of pathology, and is therefore a research endeavor that is performed in temporal bone laboratories. This proposal was developed in keeping with the guidelines announced in RFA-DC-11-003 that is meant to establish the NIDCD Otopathology Research Collaboration Network. Our goals include the following: 1. Investigate pathology of hearing loss caused by cochleovestibular schwannoma. 2. Investigate radiologic-histologic correlations in otosclerosis, and the pathogenetic mechanism of sensorineural hearing loss when otosclerosis involves the cochlea. 3. Investigate dynamics of sensory and non-sensory cell turnover in the human inner ear. 4. Establish a program for the training of future temporal bone researchers. 5. Cooperate with the NIDCD Otopathology Research Collaboration Network. The proposed research is designed to provide new knowledge and insight into the pathology and pathophysiology of otosclerosis, acoustic tumors and cell turnover in the inner ear, which, in turn, should lead to better methods of diagnosis and therapy.