Otopathology 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-06-001)中宣布的指导方针制定的，该指导方针旨在建立人类颞骨研究资源增强联盟。我们的目标包括以下几个方面：1.开发用于评估预期获得的颞骨的定制方案。2.将所有颞骨的组织病理学报告和图像以数字格式存档，以提高档案和新获得的标本的研究和教育价值。3.开发将分子生物学分析应用于颞骨研究的改进方案，特别是将优化免疫染色和基于质谱学的蛋白质组学的适用性的方案，同时保留处理后组织的形态。4.从注册的颞骨捐赠者那里收集全面的临床信息。5.制定培养未来颞骨研究人员的计划。6.与颞骨研究联盟合作。这项研究将对听力和平衡障碍的原因提供新的认识，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.