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. 2022 Nov 16:13:992512.
doi: 10.3389/fpsyg.2022.992512. eCollection 2022.

Late phonological development in Williams syndrome

Affiliations

Late phonological development in Williams syndrome

Vanesa Pérez et al. Front Psychol. .

Abstract

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.

Keywords: Williams syndrome; atypical language development; intellectual disability; neurodevelopmental genetic disorders; phonological development; phonological processes; spontaneous speech assessment.

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Conflict of interest statement

The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest.

Figures

FIGURE 1
FIGURE 1
Profiles of relative frequency of processes by classes (SYS, syllable structure; SBT, substitution; OMI, omission; ASM, assimilation; ADD, addition) for WS groups and TD groups. (A) Profiles of WS1 and WS2 groups. (B) Profiles of TD1 and TD2 groups. (C) Profiles of WS1 and TD1groups. (D) Profiles of WS2 and TD2 groups.
FIGURE 2
FIGURE 2
Profiles of relative frequency of syllable structure processes (CCR, consonant cluster reduction; FCD, final consonant deletion; VCR, vowel cluster reduction; SYD, unstressed syllable deletion; MTT, metathesis) for WS groups and TD groups. (A) Profiles of WS1 and WS2 groups. (B) Profiles of TD1 and TD2 groups. (C) Profiles of WS1 and TD1 groups. (D) Profiles of WS2 and TD2 groups.
FIGURE 3
FIGURE 3
Profiles of relative frequency of substitution processes (LIQ, liquid; VOW, vowel; FRC, fricative; VOS, voiced stop; VLS, voiceless stop; NSL, nasal) for WS groups and TD groups. (A) Profiles of WS1 and WS2 groups. (B) Profiles of TD1 and TD2 groups. (C) Profiles of WS1 and TD1 groups. (D) Profiles of WS2 and TD2 groups.
FIGURE 4
FIGURE 4
Profiles of relative frequency of omission processes (LIQ, liquid; VOW, vowel; FRC, fricative; VOS, voiced stop; VLS, voiceless stop; NSL, nasal) for WS groups and TD groups. (A) Profiles of WS1 and WS2 groups. (B) Profiles of TD1 and TD2 groups. (C) Profiles of WS1 and TD1 groups. (D) Profiles of WS2 and TD2 groups.
FIGURE 5
FIGURE 5
Cluster membership for a range of solutions (2, 3, and 4 clusters) for classes of processes. (A) Cluster membership of cases in WS1 group. (B) Cluster membership of cases in WS2 group.
FIGURE 6
FIGURE 6
Cluster membership for a range of solutions (2, 3, and 4 clusters) for syllable structures processes. (A) Cluster membership of cases in WS1 group. (B) Cluster membership of cases in WS2 group.
FIGURE 7
FIGURE 7
Cluster membership for a range of solutions (2, 3, and 4 clusters) for substitution processes. (A) Cluster membership of cases in WS1 group. (B) Cluster membership of cases in WS2 group.
FIGURE 8
FIGURE 8
Cluster membership for a range of solutions (2, 3, and 4 clusters) for omission processes. (A) Cluster membership of cases in WS1 group. (B) Cluster membership of cases in WS2 group.

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References

    1. Abbeduto L., Benson G., Short K., Dolish J. (1995). Effects of sampling context on the expressive language of children and adolescents with mental retardation. Ment. Retard. 33 279–288. - PubMed
    1. Aguilar E., Serra M. (2003). A-RE-HA: Análisis del retraso del habla: Protocolos para el análisis de la fonética y la fonología infantil. Barcelona: Universitat de Barcelona.
    1. Barnes E., Roberts J., Long S. H., Martin G. E., Berni M. C., Mandulak K. C., et al. (2009). Phonological accuracy and intelligibility in connected speech of boys with Fragile X syndrome or Down syndrome. J. Speech Lang. Hear. Res. 52 1048–1061. 10.1044/1092-4388(2009/08-0001) - DOI - PMC - PubMed
    1. Becerra A. M., Mervis C. B. (2019). Age at onset of declarative gestures and 24-month expressive vocabulary predict later language and intellectual abilities in young children with Williams syndrome. Front. Psychol. 10:2648. 10.3389/fpsyg.2019.02648 - DOI - PMC - PubMed
    1. Bellugi U., Lichtenberger L., Jones W., Lai Z., St. George M. (2000). I. The neurocognitive profile of Williams syndrome: A complex pattern of strengths and weaknesses. J. Cogn. Neurosci. 12 (Suppl. 1), 7–29. 10.1162/089892900561959 - DOI - PubMed

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