Gosch A, Städing G, Pankau R
Department of Pediatrics, University of Kiel, Germany.
Am J Med Genet. 1994 Sep 1;52(3):291-6. doi: 10.1002/ajmg.1320520308.
In recent studies children with Williams-Beuren syndrome (WBS) have been characterized as having a distinct neuropsychological profile with verbal abilities being superior to visuo-spatial and motor skills. An unusual command of language, including excessive use of verbal stereotypes, social phrases, and clichés has been noticed. The aim of this study is to establish whether the quality and quantity of verbal behavior, and the articulation and tonal quality of the voices of children with WBS differ from other children with nonspecific developmental disabilities. A group of 25 children with WBS and a control group of 25 children matched for age (4-10 years), sex (12 girls; 13 boys), and non-verbal reasoning abilities (mean IQ = 79) were investigated. The Heidelberg Language Development Test and a picture story were administered. The mothers were asked to answer a questionnaire to assess the articulation and the vocal characteristics of their children. The results show that children with WB syndrome do not differ in most qualitative and quantitative tasks with regard to verbal competence. They produce significantly more correct plural-singular formations than the control children (t = 2.49, P < 0.01) on a primitive level of grammatical competence. In general, their articulation was reported to be more exact and clear (t = -2.73, P < 0.006). More mothers of children with WBS noticed a production of stereotypes, the use of social phrases, and clichés than did mothers of the control children (Chi square = -6.67 P < 0.005). Children with WBS were less likely to lisp as compared to the control children (Chi square = 2.08, P = 0.074).(ABSTRACT TRUNCATED AT 250 WORDS)
在最近的研究中,患有威廉姆斯-贝伦综合征(WBS)的儿童被描述为具有独特的神经心理学特征,其语言能力优于视觉空间和运动技能。人们已经注意到他们对语言有不寻常的掌握,包括过度使用语言陈词滥调、社交用语和套话。本研究的目的是确定患有WBS的儿童的言语行为的质量和数量,以及其语音清晰度和音质是否与其他非特异性发育障碍儿童不同。对一组25名患有WBS的儿童和一组25名年龄(4至10岁)、性别(12名女孩;13名男孩)及非语言推理能力相匹配(平均智商=79)的对照组儿童进行了调查。实施了海德堡语言发展测试和一个图片故事。要求母亲们回答一份问卷,以评估其孩子的语音清晰度和嗓音特征。结果表明,患有WBS综合征的儿童在大多数关于语言能力的定性和定量任务中没有差异。在语法能力的初级水平上,他们产生的正确复数-单数形式明显多于对照组儿童(t=2.49,P<0.01)。总体而言,据报告他们的语音清晰度更高、更清晰(t=-2.73,P<0.006)。与对照组儿童的母亲相比,更多患有WBS的儿童的母亲注意到孩子有陈词滥调、使用社交用语和套话的情况(卡方=-6.67,P<0.005)。与对照组儿童相比,患有WBS的儿童口齿不清的可能性较小(卡方=2.08,P=0.074)。(摘要截选至250字)