Orofacial dysfunction in ectodermal dysplasias measured using the Nordic Orofacial Test-Screening protocol.
OBJECTIVE
To screen orofacial function in people with various ectodermal dysplasia (ED) syndromes and compare with a healthy reference sample.


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.
Bergendal
Birgitta
B
National Oral Disability Centre, Institute for Postgraduate Dental Education, Jönköping, Sweden. birgitta.bergendal@lj.se
McAllister
Anita
A
Stecksén-Blicks
Christina
C
D000293:Adolescent; D000328:Adult; D002648:Child; D002675:Child, Preschool; D003680:Deglutition Disorders / Q000175:diagnosis*; D004476:Ectodermal Dysplasia / Q000150:complications* / Q000503:physiopathology; D005145:Face; D005149:Facial Expression; D005260:Female; D006685:Hoarseness / Q000175:diagnosis; D006801:Humans; D008297:Male; D008334:Mandible / Q000502:physiology; D008403:Mass Screening; D008409:Mastication / Q000502:physiology*; D008410:Masticatory Muscles / Q000502:physiology; D008875:Middle Aged; D009666:Nose / Q000502:physiology; D012119:Respiration; D013064:Speech Disorders / Q000175:diagnosis*; D014831:Voice / Q000502:physiology; D014987:Xerostomia / Q000175:diagnosis*; D055815:Young Adult