Tracking early emergence of sound perception impairments in FXS with multimodal fNIRS/EEG

使用多模态 fNIRS/EEG 跟踪 FXS 中早期出现的声音感知障碍

基本信息

批准号：
10505619
负责人：
Elizabeth Gayle Smith
金额：
$ 15.29万
依托单位：
CINCINNATI CHILDRENS HOSP MED CTR
依托单位国家：
美国
项目类别：
财政年份：
2022
资助国家：
美国
起止时间：
2022-09-01 至 2027-08-31
项目状态：
未结题

来源：
https://reporter.nih.gov/project-details/10505619
关键词：
10 year old Address Adolescent Adult Age Age-Months Anatomy Animal Model Area Auditory Auditory Perception Auditory system Base of the Brain Behavioral Biological Biological Markers Brain Brain imaging Caring Child Clinical Communication impairment Control Groups Data Detection Development Development Plans Diagnosis Disease Early Intervention Electroencephalography Electrophysiology (science)Ethics Etiology Evaluation Fragile X Syndrome Genetic Goals Hearing Hearing Tests Heritability Home environment Hypersensitivity Impairment Individual Infant Intellectual functioning disability Intervention K-Series Research Career Programs Language Language Delays Language Development Lead Life Linguistics Link Longevity Longitudinal Studies Measurement Measures Mediator of activation protein Mentors Mentorship Methods Modeling Nature Neurodevelopmental Disorder Outcome Pattern Phase Phenotype Premature Birth Program Development Recording of previous events Research Research Ethics Research Methodology Research Personnel Resources Risk Science Scientist Sensory Series Severities Sound Localization Specificity Speech Speech Delay Speech Development Statistical Methods Techniques Testing Time Toddler Training Treatment Effectiveness United States National Institutes of Health Visit analytical method autism spectrum disorder base behavioral phenotyping career career development early detection biomarkers experience functional near infrared spectroscopy hemodynamics high risk human model improved indexing infancy language impairment mental age multimodality neuroimaging neuromechanism neurophysiology post-doctoral training programs relating to nervous system response signal processing skills sound success therapy development tool treatment response

项目摘要

Project Summary_Abstract The purpose of this K23 is to provide the training, mentorship, and research experiences needed for the applicant to become a successful independent clinical scientist with a research program focused on understanding mechanisms linking sensory anomalies, brain dysmaturation, and speech and language impairments in neurodevelopmental disorders. The training plan focuses on developing skills required to measure and understand the behavioral, auditory, and neurophysiological indices of early speech and hearing development in Fragile X Syndrome (FXS), with three specific areas of emphasis: 1) statistical methods and signal processing techniques required for cutting edge analysis of functional neurophysiology (fNIRS/EEG), 2) development of the auditory system and speech/hearing in infancy and 3) research methods and ethics for studying infants and very young children with FXS. Dr. Craig Erickson along with co-mentors Dr. John Sweeney and Dr. Lisa Hunter will provide the mentorship, training, and resources necessary to achieve the training objectives. This research program is relevant to several objectives listed in the NIH Research Plan on FXS and Associated Disorders, including but not limited to objectives 3.1 (Develop a standard battery of functional, objective measures to better characterize the emergence of the FXS phenotype across the life span and provide precise indicators of treatment effectiveness) and 3.4 (Conduct longitudinal studies of both humans and animal models to characterize the dynamic nature of the FXS phenotype across the life span and to identify moderators and mediators of the phenotype). FXS can be diagnosed in the infant and toddler years given its genetic/heritable etiology. However, current understanding of atypical maturation of brain function in FXS and its clinical manifestations is entirely based on studies of older children (5+), adolescents, and adults, at which point impairments associated with the disorder, including delays in speech and language, have been present for several years. Thus, understanding of neural mechanisms and timing of the early brain dysmaturation that lead to early delays in FXS remains limited, which in turn limits development of interventions. The proposed career development plan provides the PI with the skills to address this gap in brain-based markers of impairment in early FXS. The research plan uses EEG/fNIRS, auditory evaluations, and speech and language assessment to investigate auditory hypersensitivity and its relation to emergence of speech and language delays in FXS. This study occurs in two phases, with the first focusing on preschoolers (2-4 years) and the second, longitudinal phase focused on the infant years (0-2 years).Thus, the aim of this early career development program is to prepare the investigator to establish an independent research program focused on determining the timing and nature of brain dysmaturation that leads to early speech and communication impairment in FXS.

项目summary_abstract 该K23的目的是提供培训，指导和研究经验申请人成为成功的独立临床科学家，研究计划的重点理解连接感觉异常，大脑不饱和以及语音和语言的机制神经发育障碍的障碍。培训计划的重点是发展所需的技能测量和理解早期语音和听力的行为，听觉和神经生理指标脆弱X综合征（FXS）的发展，具有三个特定的重点领域：1）统计方法和功能神经生理学的最先进分析（FNIRS/EEG）所需的信号处理技术，2）在婴儿期开发听觉系统以及语音/听力和3）研究方法和道德规范研究婴儿和非常年幼的FXS。克雷格·埃里克森（Craig Erickson）博士以及约翰博士 Sweeney和Lisa Hunter博士将提供实现所需的指导，培训和资源培训目标。该研究计划与NIH研究计划中列出的几个目标有关 FXS和相关疾病，包括但不限于目标3.1（开发标准电池功能性，客观的措施，以更好地表征FXS表型在整个生命周期中的出现并提供治疗有效性的精确指标）和3.4（对两者进行纵向研究人类和动物模型表征整个寿命中FXS表型的动态性质，识别表型的主持人和介体）。鉴于其遗传/可遗传的病因，可以在婴儿和幼儿的年份中诊断出FX。然而，当前对FXS中大脑功能非典型成熟及其临床表现的理解完全是基于对年龄较大的儿童（5+），青少年和成人的研究，此时与这种疾病，包括言语和语言的延迟，已经存在了几年。因此，对早期大脑不饱和症的神经机制的理解和时间导致早期延迟 FXS仍然有限，进而限制了干预措施的发展。拟议的职业发展计划在早期FXS的基于大脑的损伤标记中，PI提供了解决这一差距的技能。这研究计划使用脑电图/FNIR，听觉评估以及语音和语言评估来调查听觉过敏及其与FXS中语音和语言延迟的出现的关系。这项研究出现在两个阶段，第一次关注学龄前儿童（2 - 4年），第二个纵向阶段专注于婴儿年（0-2岁）。因此，这个早期职业发展计划的目的是准备研究人员建立一个独立研究计划，旨在确定大脑不饱和度导致FXS的早期言语和沟通障碍。