Orofacial dysfunction in individuals with Prader-Willi syndrome assessed with NOT-S

Abstract

Objective. The objective of the study was to evaluate orofacial dysfunction in individuals with Prader-Willi syndrome (PWS) and compare it with a healthy reference group. Methods and materials . The Nordic Orofacial Test-Screening (NOT-S) protocol was used for evaluation of orofacial function in 45 (23 Male, 22 Female) individuals with PWS, aged 19.8 ± 9.5 years, and a reference group of 40 (18 M, 22 F) healthy individuals, aged 24.0 ± 16.3 years. Results . The NOT-S score was markedly higher for the individuals with PWS than for the healthy reference group (3.9 ± 2.1 vs 0.3 ± 0.5, p < 0.001). The most common domains of dysfunction in individuals with PWS were Oral motor function (60.0%), Habits (55.6%), Face at rest (53.3%), Speech (44.4%), Drooling (44.4%) and Breathing (42.2%). Conclusions . Eighty-seven per cent of the participants with PWS demonstrated dysfunction in two or more domains, particularly in the domains Oral motor function, Habits and Face at rest.

Key Words::

• NOT-S
• oral dysfunction
• Prader-Willi syndrome

Acknowledgements

Thanks to the Norwegian Prader-Willi Syndrome Association for distributing information and invitations for participation in the study. Thanks to all patients and families who participated. The study was supported by a grant from the Norwegian Foundation of Health and Rehabilitation, through the Norwegian Prader-Willi Syndrome Association.

Declaration of interest: The authors report no conflicts of interest. The authors alone are responsible for the content and writing of the paper.