Otopathology by Light Microscopy and Molecular Techniques

描述（由申请人提供）：了解疾病的病理基础是医学研究的核心，包括影响听觉和前庭系统的疾病。耳科的独特之处在于内耳在生活中是不可接近的，因此，传统的病理研究技术，如活检和手术切除是不可行的。因此，深入了解耳部疾病的病理基础只能通过对颞骨的死后研究和建立可靠的动物模型来获得。提高对影响听觉和前庭系统的疾病的病理和发病机制的理解将导致这些疾病的更合理的诊断和临床管理。人类颞骨的获取、加工和研究是一项耗时且昂贵的工作，超出了临床病理部门的范围，因此是一项在颞骨实验室进行的研究工作。本提案是根据RFA （DC-06-001）中宣布的指导方针制定的，旨在建立人类颞骨研究资源增强联盟。我们的目标包括：开发定制的方案来评估预期获得的颞骨。2. 将所有颞骨的组织病理报告和图像进行数字化存档，以提高档案和新获得标本的研究和教育价值。3. 开发用于颞骨研究的分子生物学分析的改进方案，特别是优化免疫染色和质谱为基础的蛋白质组学的适用性，同时保留处理组织的形态。4. 收集已登记颞骨供体的全面临床资料。5. 建立培养未来颞骨研究人员的计划。6. 与颞骨研究联盟合作。这项研究将提供关于听力和平衡障碍的原因的新知识，这将导致更好的诊断和治疗方法。

DESCRIPTION (provided by applicant): A 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 the RFA (DC-06-001) that is meant to establish the Human Temporal Bone Consortium for Research Resource Enhancement. Our goals include the following: 1. To develop customized protocols for assessment of prospectively acquired temporal bones. 2. To archive in digital format the histopathology reports and images for all temporal bones in order to enhance the research and educational value of archival and newly acquired specimens. 3. To develop improved protocols for applying molecular biologic assays to temporal bone research, especially protocols that will optimize the applicability of immunostaining and mass spectrometry- based proteomics while preserving the morphology of the processed tissues. 4. To collect comprehensive clinical information from registered temporal bone donors. 5. To establish a program for the training of future temporal bone researchers. 6. To cooperate with the Temporal Bone Research Consortium. This research will provide new knowledge about the causes of disorders of hearing and balance, which should lead to better methods of diagnosis and therapy.