Phonological Constraints on Language Development in Individuals With Williams Syndrome

NCT07285720 | Recruiting

• 1 other identifier: PI2022_843_0021
• Study Type: interventional
• Target: 130
• Locations: 1 country
• Sites: 1

Brief Summary

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.

Conditions

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

Keywords

Williams syndrome; Language; Phonological Short-Term Memory; EEG-NIRS

Outcome Measures

Primary Outcomes (1)

• questionnaire score of patients with WS caracterisation

  questionnaire score for cognitive profile of patients with WS caracterisation

  2 years

Secondary Outcomes (1)

• Impact of hyperacusis on questionnaire score

  2 years

Study Arms (4)

Patients with Williams syndrome

EXPERIMENTAL

Other: EEG-NIRS protocol; Other: Questionnaire

Patients with Down syndrome

ACTIVE COMPARATOR

Other: EEG-NIRS protocol; Other: Questionnaire

Healthy children

SHAM COMPARATOR

Other: EEG-NIRS protocol; Other: Questionnaire

Healthy adults

ACTIVE COMPARATOR

Other: EEG-NIRS protocol; Other: Questionnaire

Interventions

EEG-NIRS protocol OTHER

Each participant will be tested individually in a quiet room at the GRAMFC (Groupe de Recherches sur l'Analyse Multimodale de la Fonction Cérébrale, INSERM-UMR1105, Université Picardie Jules Verne and CHU Amiens-Picardie). Duration of subject's participation in the study is 120 minutes

Healthy adults; Healthy children; Patients with Down syndrome; Patients with Williams syndrome

Questionnaire OTHER

Each participant will be tested individually in a quiet room at the GRAMFC (Groupe de Recherches sur l'Analyse Multimodale de la Fonction Cérébrale, INSERM-UMR1105, Université Picardie Jules Verne and CHU Amiens-Picardie). Duration of subject's participation in the study is 120 minutes

Healthy adults; Healthy children; Patients with Down syndrome; Patients with Williams syndrome

Eligibility Criteria

• Age: 6 Years+
• Sex: all
• Healthy Volunteers: Yes
• Age Groups: Child (0-17), Adult (18-64), Older Adult (65+)

You may qualify if:

• 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).

You may not qualify if:

• Autistic Associated Disorders,
• West syndrome

Sponsors & Collaborators

• Centre Hospitalier Universitaire, Amiens: lead

Study Sites (1)

CHU Amiens

Amiens, 80054, France

RECRUITING

MeSH Terms

Conditions

Williams Syndrome; Language

Interventions

Surveys and Questionnaires

Condition Hierarchy (Ancestors)

Intellectual Disability; Neurobehavioral Manifestations; Neurologic Manifestations; Nervous System Diseases; Aortic Stenosis, Supravalvular; Aortic Valve Stenosis; Aortic Valve Disease; Heart Valve Diseases; Heart Diseases; Cardiovascular Diseases; Chromosome Disorders; Congenital Abnormalities; Congenital, Hereditary, and Neonatal Diseases and Abnormalities; Genetic Diseases, Inborn; Communication; Behavior

Intervention Hierarchy (Ancestors)

Data Collection; Epidemiologic Methods; Investigative Techniques; Health Care Evaluation Mechanisms; Quality of Health Care; Health Care Quality, Access, and Evaluation; Public Health; Environment and Public Health

Central Study Contacts

Fabrice Wallois, Pr

CONTACT

33+3 22 08 77 75; fabrice.wallois@u-picardie.fr

Study Design

• Study Type: interventional
• Phase: not applicable
• Allocation: NA
• Masking: NONE
• Purpose: BASIC SCIENCE
• Intervention Model: PARALLEL
• Sponsor Type: OTHER
• Responsible Party: SPONSOR

Study Record Dates

• First Submitted: November 17, 2025
• First Posted: December 16, 2025
• Study Start: July 5, 2024
• Primary Completion (Estimated): July 1, 2026
• Study Completion (Estimated): July 1, 2026
• Last Updated: December 16, 2025

Record last verified: 2025-11

Data Sharing

• IPD Sharing: Will not share

Locations

FR (1)