BACKGROUND 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. RESULTS 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. CONCLUSIONS The categorization of voices and nonvoices is generally preserved in participants with PWS, though this may not be the case for the lowest IQ.

中文翻译：

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对Prader-Willi综合征的声音和非声音处理的研究。

背景技术Prader-Willi综合症（PWS）是一种罕见且复杂的遗传性神经发育疾病。它表现为内分泌和认知问题，包括明显的食欲亢进和严重肥胖。在许多情况下，社交和沟通技能的获取障碍会导致自闭症谱系特征，偶尔会使用特定的量表将患有该综合征的人诊断为自闭症谱系障碍（ASD）。鉴于交流技巧主要基于语音交流，因此研究PWS中的人声处理非常重要。我们能够检查从法国国家PWS和其他医院的国家参考中心招募的PWS的大量参与者（N = 61）。我们测试了他们的语音和非语音识别能力，以及他们在自由选择任务中区分声音和非声音的能力。我们将贝叶斯估计应用于分层漂移扩散模型（HDDM），以比较参与者与PWS和控件的决策。结果我们发现PWS参与者在语音和非语音处理方面均受到损害，但显示出感知语音的补偿能力。与单亲二体性的参与者相比，在15号染色体上有缺失的参与者具有较差的声音和非语音感知。HDDM使我们证明患有PWS的参与者需要积累更多信息才能做出决定，决策速度较慢，并且易于感知语音，尽管程度不及控制。