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）：该项目研究威廉姆斯综合症（WS）和唐氏综合症（DS）患者语言处理的感觉和记忆基础。这两种综合征都是基于遗传的神经发育障碍，其特征是语言强项和弱点的分布不均匀。在存在轻度至中度精神发育迟滞的情况下，WS 患者在语音、言语短期记忆和表达词汇方面表现出相对优势。相比之下，患有 DS 的人表现出相对较弱的语音能力、较差的言语短期记忆和表达词汇量减少。尽管经验支持很少，当前关于 WS 中语言功能的理论常常基于听觉处理相对优势的假设。 首先，检查行为听觉测量，包括气导、鼓室导抗测试、耳声发射、同时和后向掩蔽。然后将这些测量值与（a）索引早期听觉记忆的生理反应和（b）索引早期语言处理的语音分析进行比较。这两个临床人群的优势和劣势的相反模式提供了一个机会：（1）凭经验检查 WS 个体的听觉敏感性，（2）凭经验检验短期听觉处理在 WS 个体中特别强大的假设， (3)检验当前的假设，即在两组已确诊的遗传性疾病中，听觉处理、听觉记忆和语音处理之间存在很强的相关性。重要的临床应用是调查 WS 中听力损失高于预期的可能性，并确定听觉与语言功能的相互作用。