Orofacial dysfunction in ectodermal dysplasias measured using the Nordic Orofacial Test-Screening protocol

Abstract

Objective. To screen orofacial function in people with various ectodermal dysplasia (ED) syndromes and compare with a healthy reference sample. Material and methods. The ED group comprised 46 individuals (30 M and 16 F; mean age 14.5 years, range 3–55). Thirty-two had hypohidrotic ED, while 14 had other ED syndromes. The reference sample comprised 52 healthy individuals (22 M and 30 F; mean age 24.9 years, range 3–55). Orofacial function was screened using the Nordic Orofacial Test-Screening (NOT-S) protocol containing 12 orofacial function domains (maximum score 12 points). Results. The total NOT-S score was higher in the ED group than in the healthy group (mean 3.5 vs. 0.4; p<0.001). The dysfunctions most frequently recorded in the subjects with ED occurred in the domains chewing and swallowing (82.6%), dryness of the mouth (45.7%), and speech (43.5%). Those with other ED syndromes scored non-significantly higher than those with hypohidrotic ED (mean 4.6 vs. 3.0; p>0.05). Conclusions. Individuals with ED scored higher than a healthy reference sample in all NOT-S domains, especially in the chewing and swallowing, dryness of the mouth, and speech domains. Orofacial function areas and treatment and training outcomes need to be more closely evaluated and monitored.