title: Speech, eating and saliva control in rare diseases - a database study.
abstract: The aim was to study the background to and the manifestations of affected intelligibility of speech and reported difficulty with eating and saliva control in rare diseases. In Sweden, a disease or disorder is defined as rare when it affects no more than 100 individuals per million population and leads to a marked degree of disability. In 1996-2008, 1703 individuals with 169 rare diseases (3-67 years) answered a questionnaire about oral health and oro-facial function and 1614 participated in a clinical examination. A control group of 135 healthy children was included. Oromotor impairment was a frequent finding (43%) and was absent among the controls. Half the children in the youngest age group (3-6 years) had moderate/severely affected intelligibility or no speech compared with one-third in the other age groups. The most frequent eating difficulties were related to chewing and were found in approximately 20% of the individuals in the study group. Artificial nutrition was most common in children aged 3-6 years (9·2%), followed by children aged 7-12 years (4·9%), adolescents aged 13-19 years (3·3%) and adults (1·4%). Impaired saliva control was common (31·2%) and strongly and significantly correlated with oromotor dysfunction, intellectual disability, open mouth at rest and epilepsy. In conclusion, oromotor impairment and oro-facial dysfunctions, such as affected intelligibility, eating difficulties and impaired saliva control, are frequent in individuals with rare diseases. There is a strong correlation between oromotor impairment and affected intelligibility, eating difficulties and impaired saliva control in individuals with rare diseases.
authors.affiliation: Mun-H-Center Orofacial Resource Center for Rare Diseases, Public Dental Service, Gothenburg, Sweden.
authors.forename: L
authors.initials: L
authors.lastname: Sjögreen
authors.affiliation: Mun-H-Center Orofacial Resource Center for Rare Diseases, Public Dental Service, Gothenburg, Sweden.
authors.forename: Å
authors.initials: Å
authors.lastname: Mogren
authors.affiliation: Mun-H-Center Orofacial Resource Center for Rare Diseases, Public Dental Service, Gothenburg, Sweden.
authors.forename: J
authors.initials: J
authors.lastname: Andersson-Norinder
authors.affiliation: Special Care Dentistry/Clinic of Oral Medicine, Odontologen, Public Dental Service, Gothenburg, Sweden.
authors.forename: J
authors.initials: J
authors.lastname: Bratel
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D016022:Case-Control Studies; D002648:Child; D002675:Child, Preschool; D016208:Databases, Factual; D004435:Eating; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D035583:Rare Diseases / Q000453:epidemiology*; D012472:Salivation; D013065:Speech Intelligibility; D011795:Surveys and Questionnaires; D013548:Sweden / Q000453:epidemiology; D055815:Young Adult
keywords: artificial feeding; deglutition disorders; drooling; intellectual disability; intelligibility; rare diseases; speech