Phonological Constraints on Language Development in Individuals With Williams Syndrome

ConditionsWilliams Syndrome Language Phonological Short-Term Memory EEG-NIRS

Trial statusRecruiting

Trial phaseNot applicable

Trial typeInterventional

Biological sexAll

Age6+

SponsorCentre Hospitalier Universitaire, Amiens

About this trial

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.

Eligibility criteria

This trial accepts healthy volunteers

Qualifiers

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).

Disqualifiers

Autistic Associated Disorders,

West syndrome

Trial design

Design model

Parallel

Treatments tested in this trial

EEG-NIRS protocol
Other intervention
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
Questionnaire
Other intervention
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

Treatment groups

130 Participants

are divided into 4 treatment groups

Group A: Patients with Williams syndromeExperimental treatment 2 interventions

Group B: Patients with Down syndromeActive comparator 2 interventions

Group C: Healthy childrenSham comparator 2 interventions

Group D: Healthy adultsActive comparator 2 interventions

Trial outcomes

Primary outcomes

questionnaire score of patients with WS caracterisation

questionnaire score for cognitive profile of patients with WS caracterisation

Time frame

2 years

Secondary outcomes

Impact of hyperacusis on questionnaire score

Time frame

2 years

Sponsors and contacts

Click on the lead sponsor to view all of their trials.

Centre Hospitalier Universitaire, Amiens

Lead sponsor

Source ClinicalTrials.gov

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