The development and validation of a novel tool for the assessment of bulbar dysfunction in ALS

项目摘要 我们的“父母”赠款提案的总体目标是开发和验证一个临床医生管理的延髓 肌萎缩侧索硬化症（ALS）的功能障碍评估工具，称为ALS球功能障碍指数 （ALS-IBD）。由于SARS-CoV-2大流行的中断，临床实践和临床研究已经 被迫转向，同时为发展新的和 扩展现有的远程评估平台，以满足临床和研究评估需求。 作为这一转变努力的一部分，我们最近创建了VirtualSLP，这是一个支持远程在线多媒体的软件工具。 模态-高质量视频/运动学和音频/声学-数据收集，使用多平台兼容， 基于网络浏览器的音频和视频录制。与此同时，我们设计了一个自动提取的工具， 神经系统疾病的运动学和声学指标，提供临床可解释的特征/ 已被确定为对检测和跟踪oro的发病和进展重要的措施， 神经系统疾病中的运动和言语（又名延髓）障碍。我们的计划的下一步 技术开发努力是将自动度量提取软件结合到VirtualSLP中， 创建一个用户友好和直观的基于云的平台，用于研究和临床评估延髓 功能障碍为了实现VirtualSLP的快速开发和部署，我们将使用现代工程 方法论和以用户为中心的设计，以确保软件开发周期中的许多步骤。 该周期将从最终用户的参与开始开始（例如，研究人员、临床医生和患者） 记录用户当前和预期的端到端软件体验的会话， 执行现有软件组件的基线分析，并确定和实施 必要的改变。必要的组件将被纳入创建VirtualSLP在亚马逊网站上 AWS平台，以增强其可用性、互操作性、可扩展性和安全性，作为NIH的一部分 可持续发展倡议。虚拟SLP与最终用户的可用性测试将在 开发周期结束时和开发周期结束时。完成后，这项工作将产生一个 用于收集音频和视频数据的增强型软件工具以及将使用的相应的基于AI的分析 在临床研究中。VirtualSLP将推动我们在培养临床医生方面的具体工作- 延髓ALS评估和监测管理工具（ALS-IBD-Remote）通过提供一种新的云， 基于平台进行临床验证。这项工作的目的是宣传目前资助的意图， 通过支持临床语音科学家、数据科学家和软件之间的合作， 工程师加强研究软件的设计、实施和“云就绪性”。

Project Summary The overall aim of our “parent” grant proposal is to develop and validate a clinician-administered bulbar dysfunction assessment tool for Amyotrophic Lateral Sclerosis (ALS), called ALS-Index of Bulbar Dysfunction (ALS-IBD). Interrupted by the SARS-CoV-2 pandemic, clinical practices as well as clinical research have been forced to pivot, simultaneously providing an unprecedented opportunity for the development of new and expansion of existing remote assessment platforms, which can serve clinical and research assessment needs. As part of this pivoting effort, we recently created VirtualSLP, a software tool to support remote, online multi- modal - high quality video/kinematic and audio/acoustic – data collection, using a multi-platform compatible, web-browser based audio and video recordings. In parallel, we have designed a tool for automatic extraction of kinematic and acoustic metrics of neurological diseases, which provides clinically interpretable features/ measures that have been identified as important for detecting and tracking the onset and progression of oro- motor and speech (aka bulbar) impairments in neurological diseases. The next step in the progression of our technology development efforts is to incorporate the automatic metrics extraction software within VirtualSLP, creating a user-friendly and intuitive cloud-based platform for research and clinical assessment of bulbar dysfunction. To achieve rapid development and deployment of VirtualSLP, we will use modern engineering methodology and user-centered design to iterate through a number of steps in the software development cycle. The cycle will begin with the engagement of end users (e.g., researchers, clinicians and patients) in design sessions to document users' current and anticipated end-to-end experiences with the software, while performing the baseline analysis of the existing software components and determining and implementing necessary changes. The necessary components will be incorporated to create VirtualSLP on the Amazon Web Services (AWS) platform, to enhance its usability, interoperability, scalability, and security, as part of the NIH STRIDES initiative. Usability testing of Virtual SLP with end users will be performed throughout the development process and at the end of the development cycle. When completed, the work will result in an enhanced software tool for collection of audio and video data and corresponding AI-based analytics to be used in the context of clinical research. VirtualSLP will propel our specific work on developing a clinician- administered tool for bulbar ALS assessment and monitoring (ALS-IBD-Remote) by providing a novel cloud- based platform for its clinical validation. This work aims to exemplify the intent of the current funding opportunity by supporting collaborations between clinical speech scientists, data scientists, and software engineers to enhance the design, implementation, and "cloud-readiness" of research software.