Phonological Constraints on Language Development in Individuals With Williams Syndrome (SOUNDS)

Centre Hospitalier Universitaire, Amiens

Status: Enrolling

Conditions: Phonological Short-Term Memory; Williams Syndrome; EEG-NIRS; Language

Treatments: Other: Questionnaire; Other: EEG-NIRS protocol

Study type: Interventional

Funder types: Other

Identifiers

NCT07285720

PI2022_843_0021

Details and patient eligibility

About

Williams syndrome (WS) is a neurodevelopmental disease characterized by mild to moderate intellectual disability and an extremely heterogeneous cognitive profile. Research has outlined two main features of WS: an atypical social behavior associated with surprising language abilities, and a hyperacusis. The purpose of this project is to investigate the relationships between both these abilities, and particularly the role of the hyperacusis on language abilities. The hyperacusis would be crucial in developing language skills: the apparent strength in the verbal domain could rely on Phonological Short Term Memory (PSTM) more in individuals with WS than in typically developing individuals. In addition, the investigators will compare individuals with WS to individuals with Down syndrome (DS): DS is often associated to strong limitations in the PSTM with poor language abilities. To this end, the investigators will use a highly innovative approach including physiological assessments (EEG-NIRS protocol) and questionnaires. The performance of people with WS will be compared to those of participants with DS and TD participants of same DA and cognitive assessments.

Enrollment: 130 estimated patients

Sex: All

Ages: 6+ years old

Volunteers: Accepts Healthy Volunteers

Inclusion criteria

• For SW and SD: genetic diagnosis and aged between 6- and 17 years old.
• For typical participants: any child, adolescent or young adult of the same developmental or chronological age (between 6 and 17 years of age)
• adult group (between 20 and 60 years).

Exclusion criteria

• Autistic Associated Disorders,
• West syndrome

Trial design

Primary purpose: Basic Science

Allocation: N/A

Interventional model: Parallel Assignment

Masking: None (Open label)

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130 participants in 4 patient groups

Patients with Williams syndrome

Experimental group

Treatment:

Other: EEG-NIRS protocol

Other: Questionnaire

Patients with Down syndrome

Active Comparator group

Treatment:

Other: EEG-NIRS protocol

Other: Questionnaire

Healthy children

Sham Comparator group

Treatment:

Other: EEG-NIRS protocol

Other: Questionnaire

Healthy adults

Active Comparator group

Treatment:

Other: EEG-NIRS protocol

Other: Questionnaire