Impact of Child and Maternal Gestures on Word Learning in Fragile X Syndrome

项目摘要 脆性X综合征（FXS）是智力残疾的主要遗传性原因， 自闭症谱系障碍（ASD）的常见已知单基因原因。脆性X综合征（Fragile X Syndrome） (FXS)在整个生命过程中与口语斗争的特征。第一个单词不出现 在FXS中直到大约26个月，与通常发育中的儿童相比， 他们说第一句话的时间明显提前了大约12个月。语言前的沟通技巧-眼睛的使用- 凝视、发声和手势来传达需要和想要另一个人--在说话之前 语言，但FXS现有的研究还没有前瞻性地研究这种联系，因为第一个单词正在出现。 对于患有FXS的儿童来说，手势使用是一种特别重要的语言前技能。现有研究 FXS已经揭示了儿童手势使用的障碍，但迄今为止还没有研究探讨儿童手势使用与儿童行为之间的联系。 手势的使用和第一个单词的出现。当孩子们对着物体做手势时， 给物体贴上标签，为单词学习创造丰富的机会。儿童词汇学习也得到了 母亲的一个手势，这提供了一个明确的联系之间的话，它的指称。一 支持单词学习的特别有效的策略可能是母亲的手势使用与跟随相结合 评论，由孩子的注意力焦点引导的高质量输入。然而，在多大程度上， 母亲的手势改善FXS幼儿的口语词汇量增长尚不清楚。因此，我们建议 (1)用FXS确定幼儿中儿童手势使用和母亲语言输入之间的关联，（2） 用FXS确定幼儿中母亲手势和语言输入与儿童词语使用的关联，以及 (3)确定儿童手势和母亲手势以及回应性母亲语言的独特贡献 在1年的时间里，对患有FXS的幼儿的儿童口语词汇增长的输入。患有FXS的幼儿及其 亲生母亲将在一年内完成三次评估，从孩子18岁开始， 24个月。既有结构化的考官管理的观察，又有自然主义的母子自由游戏 将管理测量儿童手势使用，儿童词汇，母亲手势使用和语言 输入.长期目标是为这些儿童制定早期语言干预措施。生成的数据 将为制定干预建议提供经验支持， 为父母提供指导，指导他们如何（a）增加手势的频率，以及（B）调整手势 使用语言输入的方式，促进儿童的单词学习，生产实践，和口语 词汇增长最终，这些知识将积极影响长期的沟通成果， 个人FXS

PROJECT ABSTRACT Fragile X syndrome (FXS) is the leading inherited genetic cause of intellectual disability and the most common known single-gene cause of autism spectrum disorder (ASD). Individuals with fragile X syndrome (FXS) characteristically struggle with spoken language throughout the life course. First words do not appear until around 26 months in FXS, a substantial delay in comparison to typically developing children who produce their first words significantly earlier around 12 months. Pre-linguistic communication skills—the use of eye- gaze, vocalizations, and gestures to communicate needs and wants to another person—precede spoken language, but the extant studies in FXS have not examined this link prospectively, as first words are emerging. For children with FXS, gesture use is a particularly important pre-linguistic skill to explore. Existing research in FXS has revealed impairments in child gesture use, but no studies to date have examined the link between gesture use and the onset of first words prospectively. When children gesture at objects, adults commonly label the objects, creating rich opportunities for word learning. Child word learning is also supported by mother’s inclusion of a gesture, which provides a clear connection between the word and its referent. One particularly effective strategy to support word learning may be maternal gesture use combined with follow-in commenting, high quality input guided by the child’s attentional focus. However, the extent to which child and maternal gestures improve spoken vocabulary growth in toddlers with FXS is not known. Thus, we propose to (1) determine the association between child gesture use and maternal language input in toddlers with FXS, (2) determine the association of maternal gesture and language input with child word use in toddler with FXS, and (3) determine the unique contribution of child gesture and maternal gesture and responsive maternal language input on child spoken vocabulary growth in toddlers with FXS over a 1-year period. Toddlers with FXS and their biological mothers will complete three assessments over a 1-year period starting when the child is between 18 and 24 months. Both a structured examiner-administered observation and a naturalistic mother-child free play will be administered to measure child gesture use, child vocabulary, and maternal gesture use and language input. The long-term goal is to develop early language interventions for these children. The data generated from this project will provide the empirical support for the development of an intervention proposal that will provide parents with instruction on how to (a) increase the frequency of their gestures, and (b) align gesture use with language input in ways that promote children’s word learning, production practice, and spoken vocabulary growth. Ultimately, this knowledge will positively impact long-term communication outcomes for individuals with FXS.