Multidisciplinary Approaches to Patient-Oriented Studies of Auditory Neuropathy

描述(由申请人提供)：许多临床听力和语言障碍将受益于以患者为中心的合作研究。这个项目的重点是听神经病(AN)，这是一种神经类型的听力损失，最近才被发现和表征。其主要特征是耳声发射保留，听性脑干反应异常或缺失，言语知觉不良。这一面向患者的多学科项目将为AN制定临床定义，调查AN的潜在发病率，开展一系列关于AN的研讨会，包括网络广播，定义和支持试点项目，确定针对AN的全面患者导向研究的资源需求，并准备一项或多项终点拨款申请。一个具有广泛专业知识的小组已经组建，对一个有兴趣。作为一个整体，该项目将成为发展协作性、多学科、以患者为导向的研究的典范。R24的正当性。作为该项目合作伙伴的临床医生和研究人员需要这种机制，以便制定共同的语言、目标和有重点的研究计划。与公共卫生的相关性。虽然是一种新发现的疾病，但我们怀疑听神经病相当常见，但很少被诊断：外周反应良好的患者抱怨言语交流困难的发生频率很高，无法受益于助听器的患者也是如此。这些人在各个年龄段都可以看到。他们在生活和职业上遇到了很大的障碍。他们的沟通障碍对他们的情绪健康和幸福有负面影响。如果AN是适合这些人的诊断，那么他们在听力和耳科患者群体中占了相当大的比例。向这些患者提供更有效的卫生保健的潜力是巨大的。该项目还将在改善科学家与医生之间的互动以发展和开展研究方面产生影响。当临床和研究企业变得过于截然不同时，就会失去重要的机会，我们将致力于开发促进实验室和临床之间全面合作的机制。

DESCRIPTION (provided by applicant): Many clinical disorders of hearing and language would benefit from collaborative patient-oriented research. This project focuses on auditory neuropathy (AN), a neural type of hearing loss that has only recently been identified and characterized. Its primary characteristics are preservation of otoacoustic emissions, abnormal or absent auditory brainstem responses, and poor speech perception. This multidisciplinary patient-oriented project will develop clinical definitions for AN, survey the potential incidence of AN, undertake a series of seminars on AN, including webcasting, define and support pilot projects, identify resources needs for full- scale patient-oriented research on AN, and prepare one or more endpoint grant applications. A group with wide expertise has been assembled with interest in AN. As a whole, the project will be a model for developing collaborative, multidisciplinary patient-oriented research. JUSTIFICATION FOR THIS R24. The clinicians and researchers who are partners in this project need this mechanism in order to develop a common language, goals, and a focused research program. RELEVANCE TO PUBLIC HEALTH. Although a newly identified disorder, we suspect that auditory neuropathy is fairly common yet rarely diagnosed: Patients with good peripheral responses who complain of difficulty with speech communication occur with great frequency, as are patients who are unable to benefit from hearing aids. These individuals are seen throughout the age range. They experience significant handicap in their lives and their vocations. Their communication handicaps have negative implications for their emotional health and well-being. If AN is an appropriate diagnosis for these individuals, they represent a sizeable proportion of the audiological and otological patient population. The potential to deliver more effective health care to these patients is significant. This project also will have an impact in the area of improving scientist-physician interactions for developing and carrying out research. Important opportunities are lost when the clinical and research enterprises become too distinct from each other, and we will undertake to develop mechanisms that promote full partnership between the lab and the clinic.