Home-based SSP on Individuals With PWS

Study Overview
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Brief Summary
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The Polyvagal Theory focuses on how function and structure changed in the vertebrate autonomic nervous system during evolution. The theory is named for the vagus, a major cranial nerve that regulates bodily state. As a function of evolution, humans and other mammals have a new vagal pathway that links the regulation of bodily state to the control of the muscles of the face and head including the middle ear muscles. These pathways regulating body state, facial gesture, listening (i.e., middle ear muscles), and vocal communication collectively function as a Social Engagement System (SES). Because the Social Engagement System is an integrated system, interventions influencing one component of this system (e.g., middle ear muscles) may impact on the other components. Individuals with Prader-Willi Syndrome (PWS) exhibit many behaviors that are consistent with a compromised Social Engagement System. Atypical function of the Social Engagement System results in problems associated with state regulation (e.g., impulsivity, tantrums, and difficulty with change in routine), ingestion (e.g., difficulties in sucking at birth, hyperphagia), coordination of suck/swallow/breathe, intonation of vocalizations, auditory processing and hypersensitivity, and socialization. The investigatiors propose to confirm that several features of the behavioral phenotype of PWS may be explained within the context of a dysfunctional SES, which may be partially rehabilitated via an intervention designed as a 'neural exercise' of the SES (i.e., the Safe and Sound Protocol, SSP). Specific Aims: Aim I: To demonstrate the effectiveness of the Safe and Sound Protocol (SSP) on improvement of social and regulation behaviors in individuals with PWS. Aim II: To evaluate a new methodology for collecting and evaluating vocal samples for analyses of prosody, one of the indices of the functioning of the SES.

Official Title
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Evaluating the Effectiveness of Home-based SSP on Individuals With PWS

Conditions
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Prader-Willi Syndrome

Intervention / Treatment
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* Behavioral: Safe and Sound Protocol


Participation Criteria
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Eligibility Criteria
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Inclusion Criteria: Child participants must meet criteria for Prader-Willi Syndrome (per parent membership in Prader-Willi Parent Support Group). Child participants must be between ages 3-17 years. Parent must be 18 years or older. Child participants must have normal hearing (confirmed via parental report on Qualtrics questionnaire) Exclusion Criteria: 1) Child participants who are hearing-impaired (without correction)

Ages Eligible for Study
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Minimum Age: 3 Years
Maximum Age: 17 Years

Sexes Eligible for Study
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All

Accepts Healthy Volunteers
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No

Study Plan
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How is the study designed?
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Design Details

Primary Purpose: Basic Science
Intervention Model: Single Group Assignment
Interventional Model Description: All participants will receive the auditory intervention.
Masking: None (Open Label)


Arms and Interventions

| Participant Group/Arm | Intervention/Treatment |
| --- | --- |
| Experimental: Safe and Sound Protocol<br>All child participants will participate in 1 pre-intervention assessment and 1 post-intervention assessment. The auditory intervention (i.e., Safe and Sound Protocol, SSP) will last for 1 hour per day, for 5 consecutive days. | Behavioral: Safe and Sound Protocol<br>* The auditory intervention will consist of listening to computer-altered acoustic stimulation, designed to modulate the frequency band of vocal music passed to the participant. The frequency characteristics of the acoustic stimulation are selected to emphasize the relative importance of specific frequencies in conveying the information embedded in human speech. Modulation of the acoustic energy within the frequencies of human voice, similar to an exaggerated vocal prosody, are hypothesized to recruit and modulate the neural regulation of the middle ear muscles and to functionally reduce sound hypersensitivities and improve auditory processing.<br>* Other names: Listening Project Protocol;|

What is the study measuring?
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Primary Outcome Measures

| Outcome Measure | Measure Description | Time Frame |
| --- | --- | --- |
| Change from baseline in sensory sensitivities at 1 week | Brain-Body Center Sensory Scales (BBC Sensory Scales) (parental questionnaire) | pre-intervention (within 1 week before intervention), post-intervention (within 1 week after intervention) | 

Secondary Outcome Measures

| Outcome Measure | Measure Description | Time Frame |
| --- | --- | --- |
| Change from baseline in disruptive behavior at 1 week | Developmental Behavior Checklist (DBC2) (questionnaire) | pre-intervention (within 1 week before intervention), post-intervention (within 1 week after intervention) | 
| Change from baseline in social behavior at 1 week | Safe and Sound Protocol (SSP) Parent Questionnaire | post-intervention (within 1 week after intervention) | 
| Change from baseline in Foundational Abilities (balance, gross & fine motor control, sensory, social/emotional, auditory/language and attention/organization and sleep) at 1 week | Measure of Foundational Abilities (MFA) questionnaire | pre-intervention (within 1 week before intervention), post-intervention (within 1 week after intervention) | 
| Change from baseline in prosody at 1 week | Prosody assessment of recorded speech | pre-intervention (within 1 week before intervention), post-intervention (within 1 week after intervention) | 

 Terms related to the study
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Keywords Provided by Centre Hospitalier Valida
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Polyvagal Theory, Social Behavior, Hyperacusis, Prosody, Auditory intervention