A study of voice and non-voice processing in Prader-Willi syndrome

Kuzma Strelnikov (1, 2) , Jimmy Debladis (2) , Juliette Salles (2, 1) , Marion Valette (1) , Carine Mantoulan (1) , Denise Thuilleaux (3) , Virginie Laurier (3) , Catherine Molinas (1) , Pascal Barone (2) , Maïthé Tauber (4, 1)

1 CHU Toulouse - Centre Hospitalier Universitaire de Toulouse
2 CERCO - Centre de recherche cerveau et cognition
3 Hôpital Marin d'Hendaye
4 CPTP - Centre de Physiopathologie Toulouse Purpan

Prader-Willi syndrome (PWS) is a rare and complex neurodevelopmental disorder of genetic origin. It manifests itself in endocrine and cognitive problems, including highly pronounced hyperphagia and severe obesity. In many cases, impaired acquisition of social and communication skills leads to autism spectrum features, and individuals with this syndrome are occasionally diagnosed with autism spectrum disorder (ASD) using specific scales. Given that communicational skills are largely based on vocal communication, it is important to study human voice processing in PWS. We were able to examine a large number of participants with PWS (N = 61) recruited from France’s national reference center for PWS and other hospitals. We tested their voice and nonvoice recognition abilities, as well as their ability to distinguish between voices and nonvoices in a free choice task. We applied the hierarchical drift diffusion model (HDDM) with Bayesian estimation to compare decision-making in participants with PWS and controls. We found that PWS participants were impaired on both voice and nonvoice processing, but displayed a compensatory ability to perceive voices. Participants with uniparental disomy had poorer voice and nonvoice perception than participants with a deletion on chromosome 15. The HDDM allowed us to demonstrate that participants with PWS need to accumulate more information in order to make a decision, are slower at decision- making, and are predisposed to voice perception, albeit to a lesser extent than controls. The categorization of voices and nonvoices is generally preserved in participants with PWS, though this may not be the case for the lowest IQ

Mots clés: Prader-Willi syndrome; Voice processing; Social interactions; Autism spectrum disorder

Domaines: Psychologie

Kuzma Strelnikov, Jimmy Debladis, Juliette Salles, Marion Valette, Carine Mantoulan, et al.. A study of voice and non-voice processing in Prader-Willi syndrome. Orphanet Journal of Rare Diseases, 2020, 15, pp.22. ⟨10.1186/s13023-020-1298-8⟩. ⟨hal-03093987⟩