Pérez Vanesa, Martínez Verónica, Diez-Itza Eliseo
LOGIN Research Group, University of Oviedo, Oviedo, Spain.
Escuelas Universitarias Gimbernat, University of Cantabria, Torrelavega, Spain.
Front Psychol. 2022 Nov 16;13:992512. doi: 10.3389/fpsyg.2022.992512. eCollection 2022.
Williams syndrome is a neurodevelopmental genetic disorder characterized by a unique phenotype, including mild to moderate intellectual disability and an uneven neuropsychological profile of relative strengths and weaknesses. Language structure components (i.e., phonology, morphosyntax, and vocabulary) have been considered an area of specific ability compared to pragmatic language use. However, research on phonological development in Williams syndrome is very scarce, and it suggests atypical patterns. Therefore, the aim of the present study was to explore the profiles of late phonological development in Spanish-speaking children, adolescents, and adults with Williams syndrome, based on the analysis of five classes of processes (Syllable Structure, Substitution, Omission, Assimilation, and Addition) in spontaneous speech. The phonological profiles of seven children (aged 3-8 years), and seven adolescents and young adults (aged 14-25 years) with Williams syndrome were compared with two normative groups of typically developing (TD) children at different stages of late phonological development (aged 3 and 5 years). The frequency of phonological processes in the group of children with Williams syndrome was similar to that of 3-year-old TD children, which suggests that they would be in the first stage of late phonological development (expansion stage). The group of older individuals with Williams syndrome showed a much lower frequency of processes, similar to that of 5-year-old TD children in the last stage of phonological development (resolution stage). However, their phonological processes appeared to be persistent and independent of chronological age. Furthermore, asynchronies in quantitative and qualitative profiles (relative frequency) indicated atypical and complex trajectories in late phonological development, which cannot be described as simply delayed or protracted. Remarkable individual differences were observed, especially in the group of adolescents and adults with Williams syndrome, although the majority of cases conformed to the modal profiles of their groups. A major tendency for Omission, including final consonant deletion, may be considered atypical and specific to Williams syndrome at all ages. The results of the present study raise the need for continued and appropriate phonological assessment and treatment for people with Williams syndrome across the lifespan.
威廉姆斯综合征是一种神经发育性遗传疾病,其特征是具有独特的表型,包括轻度至中度智力障碍以及相对优势和劣势不均衡的神经心理学特征。与语用语言使用相比,语言结构成分(即语音、形态句法和词汇)一直被认为是一种特殊能力领域。然而,关于威廉姆斯综合征语音发展的研究非常稀少,且显示出非典型模式。因此,本研究的目的是通过分析自发言语中的五类过程(音节结构、替代、省略、同化和添加),探索讲西班牙语的患有威廉姆斯综合征的儿童、青少年和成人的晚期语音发展特征。将七名患有威廉姆斯综合征的儿童(3至8岁)以及七名青少年和青年成人(14至25岁)的语音特征与两组处于晚期语音发展不同阶段的典型发育(TD)儿童的规范组(3岁和5岁)进行比较。患有威廉姆斯综合征的儿童组中语音过程的频率与3岁TD儿童相似,这表明他们处于晚期语音发展的第一阶段(扩展阶段)。年龄较大的患有威廉姆斯综合征的个体组中过程频率要低得多,与处于语音发展最后阶段(解决阶段)的5岁TD儿童相似。然而,他们的语音过程似乎持续存在且与实际年龄无关。此外,数量和质量特征(相对频率)的不同步表明晚期语音发展存在非典型和复杂的轨迹,不能简单地描述为延迟或延长。观察到了显著的个体差异,特别是在患有威廉姆斯综合征的青少年和成人组中,尽管大多数病例符合其组的典型特征。包括词尾辅音删除在内的省略的主要趋势在所有年龄段都可能被认为是非典型的且是威廉姆斯综合征所特有的。本研究结果表明,需要对患有威廉姆斯综合征的人在其整个生命周期内持续进行适当的语音评估和治疗。