Majerus S, Barisnikov K, Vuillemin I, Poncelet M, van der Linden M
Department of Cognitive Sciences, University of Liège, Liege, Belgium.
Neurocase. 2003 Oct;9(5):390-401. doi: 10.1076/neur.9.5.390.16558.
Abstract Although phonological processing is generally considered to be a proficiency in Williams syndrome (WS), there are very few studies which have extensively explored phonological processing abilities in WS. In this study, we re-assessed phonological processing in WS by exploring verbal STM and phonological awareness abilities in 4 children with WS (CA: 10-12 years) and two control groups, one matched for chronological age (CA) and the other matched for verbal mental age (VA). Our results confirm and extend previous claims of preserved phonological STM in WS by showing specifically preserved STM performance for non-words, compared to both VA and CA control groups. However, we observed that this was the case only for non-words where support of phonological and lexico-semantic knowledge was minimized, with reduced phonological and lexico-semantic effects on STM performance. Furthermore, a more direct assessment of phonological processing abilities through phonological awareness tasks showed impaired performance for the 4 WS children. Our data confirm that STM for non-words represents a real strength in WS but they do not support previous assumptions of a more general preservation of phonological processing abilities in WS. Implications for impaired and preserved cognitive processes underlying verbal STM and phonological awareness abilities in WS are discussed.
摘要 尽管语音加工通常被认为是威廉姆斯综合征（WS）患者的一项能力，但很少有研究广泛探讨过WS患者的语音加工能力。在本研究中，我们通过探究4名WS儿童（年龄：10 - 12岁）以及两个对照组（一个按实际年龄匹配，另一个按言语心理年龄匹配）的言语短时记忆（STM）和语音意识能力，重新评估了WS患者的语音加工情况。我们的结果证实并扩展了先前关于WS患者语音STM得以保留的说法，具体表现为与言语心理年龄和实际年龄对照组相比，非单词的STM表现特别得以保留。然而，我们观察到只有在语音和词汇语义知识支持最小化的非单词情况下才是如此，语音和词汇语义对STM表现的影响有所降低。此外，通过语音意识任务对语音加工能力进行的更直接评估显示，4名WS儿童的表现受损。我们的数据证实，非单词的STM是WS患者的一项真正优势，但并不支持先前关于WS患者语音加工能力更普遍得以保留的假设。本文讨论了WS患者言语STM和语音意识能力背后认知过程受损和保留的影响。
