Early Communicative Development in Williams Syndrome: A Longitudinal Case Study.

Individuals with Williams Syndrome (WS) have a specific and atypical neuropsychological profile, where language is above what is expected for their mental age, although it shows a late onset. There exists only one longitudinal study in infants younger than 20 months old with WS about early language precursors (joint attention, referential and instrumental behaviors, pointing gesture, verbal tags). The aim of this investigation is to evaluate these precursors in a baby with WS (8 to 18 months). Seven sessions of systematic observation were performed (six at baby's home, one at the Early Childhood Assistance center). The Battelle Developmental Inventory was used to evaluate the baby's development in two occasions (12 and 18 months). The results show an atypical development, and he is 5-6 months under what is expected for his chronological age. Attention towards objects prevails over preference for faces, but this one tends to increase. The pointing gesture does not emerge at the end of the observation period and therefore follows the first words that appear. The implications for the comprehension of the early linguistic profile in WS are discussed, as well as the implications for specific intervention strategies in the context of early childhood care.

Late phonological development in Williams syndrome.

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.

Complexity and sequence constraints in a Granada Spanish-speaking four-year-old with protracted phonological development.

This article describes the phonology of a Granada Spanish-speaking 4-year-old boy with Protracted Phonological Development (PPD) from the perspective of constraint-based nonlinear phonology. Although he had acquired basic word structure and a near-complete repertoire of vowels and consonants, he had difficulties producing more complex word structures (multisyllabic words, clusters, diphthongs) and producing sequences of consonant manner and place features across vowels. The analysis outlines his strengths and needs in phonological development, and proposes an intervention plan to address constraints on complexity and sequences.

Pragmatic Profiles of Adults with Fragile X Syndrome and Williams Syndrome.

Linguistic phenotypes of individuals with Fragile X (FXS) and Williams (WS) syndromes exhibit various degrees of pragmatic impairment, involving difficulties in social communication and in adapting to conversational principles. The goal of the present study was to explore syndrome-specific pragmatic profiles of adults with FXS and WS based on the assessment of the observance of Gricean maxims of conversation. The participants were 12 Spanish-speaking adults (6 FXS/6 WS), without a diagnosis of ASD, whose extensive naturalistic conversations (71,859 words) were transcribed and coded with the CHILDES/TALKBANK tools and the PREP-CORP pragmatic protocol. Violations of the maxims of conversation were analyzed, and indexes of cooperation and conversational response were obtained. Both groups showed reduced verbal production and repetitive dysfluencies; prominent features in the FXS profile were higher proportion of non-contingent language, perseverations of topic and form, and impulsive conversational responses; in the WS profile, salient characteristics were higher proportion of tangential utterances, reformulations, and conversational responses reflecting overly literal interpretation. Pragmatic profiles of violation of conversational maxims reflect specific communication skills impaired in adults with FXS and WS and raise the need for assessment and intervention methods that specifically address their social communication abilities.

Assessing Phonological Profiles in Children and Adolescents With Down Syndrome: The Effect of Elicitation Methods.

In the context of comparing linguistic profiles across neurodevelopmental disorders, Down syndrome (DS) has captured growing attention for its uneven profile. Although specific weaknesses in grammatical and phonological processing have been reported, research evidence on phonological development remains scarce, particularly beyond early childhood. The purpose of this study was to explore the phonological profiles of children and adolescents with Down syndrome. The profiles were based on the frequency and relative proportion of the processes observed by classes, and they were compared to those of typically developing preschool children of similar verbal age. A complementary goal was to assess the effect of two different methods of elicitation: a test of articulation and spontaneous speech sampling. Finally, intergroup and intragroup differences in full match percentages between three positions at syllable-level (complex onset, medial coda, and final coda) were assessed. The results of the present study confirmed that the frequency of phonological processes in children and adolescents with DS is atypically high and is above what is expected for lexical age and at the same level as grammatical age. Highly increased frequency of processes, consistent in all kinds of processes and positions at the syllable-level, and asynchronous with verbal age and mental age suggest atypical developmental trajectories of phonological development in the Down syndrome population.

Consonant cluster acquisition in Chilean children with typical and protracted phonological development.

The current study addresses the acquisition of tautosyllabic consonant clusters (CCs) in Chilean preschoolers with typical (TD) versus protracted phonological development (PPD). The objectives were to analyze accuracy of CCs and mismatch (error) patterns as a function of age (4/ 5 years) and TD/PPD group, examining effects of sonority, stress, place of articulation and development of /l/ and /ɾ/ as singletons. Participants included 20 Chilean Spanish-speaking children with TD and 20 with PPD (ages 4 and 5 years). The TD group showed higher accuracy and an age effect. For both developmental groups, timing unit match was higher than full segmental match. CCs with labial and voiceless C1s in stressed syllables were most accurate. In the PPD group, deletions predominated over substitutions, deletions of C1 were significantly higher and the typology of substitutions was more differentiated. Results are evaluated in light of previous research on Chilean preschoolers, and clinical implications are discussed.

Explicit Oral Narrative Intervention for Students with Williams Syndrome.

Narrative skills play a crucial role in organizing experience, facilitating social interaction and building academic discourse and literacy. They are at the interface of cognitive, social, and linguistic abilities related to school engagement. Despite their relative strengths in social and grammatical skills, students with Williams syndrome (WS) do not show parallel cognitive and pragmatic performance in narrative generation tasks. The aim of the present study was to assess retelling of a TV cartoon tale and the effect of an individualized explicit instruction of the narrative structure. Participants included eight students with WS who attended different special education levels. Narratives were elicited in two sessions (pre and post intervention), and were transcribed, coded and analyzed using the tools of the CHILDES Project. Narratives were coded for productivity and complexity at the microstructure and macrostructure levels. Microstructure productivity (i.e., length of narratives) included number of utterances, clauses, and tokens. Microstructure complexity included mean length of utterances, lexical diversity and use of discourse markers as cohesive devices. Narrative macrostructure was assessed for textual coherence through the Pragmatic Evaluation Protocol for Speech Corpora (PREP-CORP). Macrostructure productivity and complexity included, respectively, the recall and sequential order of scenarios, episodes, events and characters. A total of four intervention sessions, lasting approximately 20 min, were delivered individually once a week. This brief intervention addressed explicit instruction about the narrative structure and the use of specific discourse markers to improve cohesion of story retellings. Intervention strategies included verbal scaffolding and modeling, conversational context for retelling the story and visual support with pictures printed from the cartoon. Results showed significant changes in WS students' retelling of the story, both at macro- and microstructure levels, when assessed following a 2-week interval. Outcomes were better in microstructure than in macrostructure, where sequential order (i.e., complexity) did not show significant improvement. These findings are consistent with previous research supporting the use of explicit oral narrative intervention with participants who are at risk of school failure due to communication impairments. Discussion focuses on how assessment and explicit instruction of narrative skills might contribute to effective intervention programs enhancing school engagement in WS students.

Narrative competence in Spanish-speaking adults with Williams syndrome.

BACKGROUND: Williams syndrome (WS) is a genetic disorder associated with intellectual disability and characterised by displaying an atypical neuropsychological profile, with peaks and valleys, where language skills seem better preserved than non-verbal intelligence. METHOD: This study researches the narrative competence of nine Spanish-speaking adults with WS. Oral narratives were elicited from a silent film, and narrative coherence was analysed as a function of sequential order of the events narrated at three structure levels, while narrative cohesion was assessed through the frequency of use and type of discourse markers. RESULTS: WS subjects were able to remember a significant proportion of the events from the film, but coherence of narratives, i.e., sequential order of events, was more impaired. Consistently with their linguistic abilities, cohesion of narratives was better preserved, as they used discourse markers to introduce a high proportion of events. CONCLUSIONS: Construction of mental models of the narratives may be constrained in WS by non-verbal cognitive abilities, but narrative competence is also determined by textual pragmatic abilities to organize discourse, which should be addressed by specific intervention in narrative competence.

[Assimilation processes in the late stages of phonological development]. / Procesos de asimilación en las etapas tardías del desarrollo fonológico.

This article reports a research on late phonological assimilation processes in child language in order to determine the possible stages in their evolution and the variations in relative frequency as a function of directionality (progressive vs. regressive), distance (contiguous vs. non-contiguous), and type of phonemes involved in assimilations (consonants vs. vowels). The corpus analyzed was collected from 240 children ranging in age between three and six years old. The speech samples were obtained in naturalistic settings of spontaneous conversation with the researchers, and were transcribed and analyzed with the tools provided by the CHILDES Project. Results describe a profile with three stages, reflecting the general dynamics of phonological development. Around the age of four, a general reorganization of the phonological system takes place, and the least frequent assimilation processes (progressive, contiguous and intervocalic) tend to disappear earlier. The most frequent assimilation processes (regressive, non-contiguous and inter-consonant) tend to persist as processing errors in the transition to adult speech. These findings show the functional complexity of late assimilation processes, as well as the need for taking it into account when planning clinical intervention.