Auditory Memory Processing in Williams Syndrome

威廉姆斯综合症的听觉记忆处理

• 批准号: 6985255
• 负责人: JEFFREY A MARLER
• 金额: $ 5.27万
• 依托单位: JAMES MADISON UNIVERSITY
• 依托单位国家: 美国
• 财政年份: 2003
• 资助国家: 美国
• 起止时间: 2003-07-01 至 2007-05-31
• 项目状态: 已结题
• 来源: https://reporter.nih.gov/project-details/6985255
• 关键词: Downs syndrome; Williams syndrome; adolescence (12-20); auditory discrimination; clinical research; developmental neurobiology; electrophysiology; hearing; human subject; language; memory; neurophysiology; otoacoustic emission; phonology; psychological tests; psychophysics; psychophysiology; sensory mechanism; stimulus /response; tympanum; verbal behavior; vocabulary

DESCRIPTION (provided by applicant): This project investigates the sensory and memory underpinnings of language processing in individuals with Williams syndrome (WS) and Down's syndrome (DS). Both syndromes are genetically-based neurodevelopmental disorders characterized by an uneven profile of language strengths and weaknesses. In the presence of mild-to-moderate mental retardation, individuals with WS show relative strengths in phonology, verbal short-term memory, and expressive vocabulary. In contrast, individuals with DS show relatively weak phonological abilities, poor verbal short-term memory, and diminished expressive vocabulary. In spite of minimal empirical support, current theories about language function in WS are frequently grounded upon an assumption of relative strengths in auditory processing. First, behavioral auditory measurements are examined including air conduction, tympanometry, otoacoustic emissions, simultaneous and backward masking. These measures are then compared with both (a) physiological responses indexing early auditory memory and (b) phonological analyses indexing early language processing. The inverse pattern of strengths and weaknesses in these two clinical populations provides an opportunity to: (1) empirically examine auditory sensitivity in individuals with WS, (2) empirically test the hypothesis that short-term auditory processing is particularly robust in individuals with WS, and (3) test a current hypothesis that there is a strong correlation between auditory processing, auditory memory, and phonological processing in two groups with confirmed genetic disorders. Important clinical applications are to investigate the possibility of higher-than-expected instances of hearing loss in WS and to identify auditory interactions with language function.

描述（由申请人提供）：该项目调查了威廉姆斯综合症（WS）和唐氏综合症（DS）的个人语言处理的感官和记忆基础。这两种综合征都是基于遗传的神经发育障碍，其特征是语言优势和劣势的不平衡。在存在轻度至中度的智力障碍的情况下，具有WS的个体在语音学，言语短期记忆和表达词汇方面表现出相对优势。相比之下，DS的个体表现出相对较弱的语音能力，言语短期记忆不佳以及表达词汇的降低。尽管经验支持最少，但WS中有关语言功能的当前理论经常以听觉处理相对强度的假设为基础。 首先，检查了行为听觉测量值，包括空气传导，鼓声，耳声排放，同时和后退掩蔽。然后将这些措施与（a）生理反应索引早期听觉记忆和（b）语音分析索引早期语言处理的（b）。这两个临床人群的优势和劣势的逆模式为：（1）经验检查WS患者的听觉敏感性，（2）经验测试以下假说，即短期听觉处理在WS的个体中尤为强大，并且（3）当前的假设确认了在听觉过程中进行了强烈的校正，并且在听觉上进行了两次恢复，并进行了两次听觉，并进行了两种遗传性的处理，并进行了两种遗传学的处理。重要的临床应用是调查WS中听力损失高于预期的可能性，并确定与语言功能的听觉相互作用。