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Bulbar muscles are affected at a\u00a0later stage. The aim of the present study was to test whether oral function tests are able to better discriminate motor function than established scores and to replicate known associations between disease-related altered craniofacial anatomy and oral dysfunction in SMA.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods<\/jats:title>\n                    <jats:p>A\u00a0total of 43\u00a0adult individuals with SMA (mean age 39.7\u202f\u00b1\u200912; 25\u00a0men, 18\u00a0women) were included in this prospective, cross-sectional study. Oral function was measured using a\u00a0piezoelectric sensor system and an Iowa Oral Performance Instrument (IOPI) device. Data from oral function tests and established motor scores were analyzed with regard to a\u00a0possible floor or ceiling effect. It was tested to what extent SMA patients with different malocclusions presented with variable scores.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results<\/jats:title>\n                    <jats:p>Patients differed in ambulatory and treatment status (15\u00a0ambulatory vs.\u00a028 nonambulatory; 35\u00a0treated vs.\u00a08 nontreated) and orthodontic findings (22\u00a0with a\u00a0class\u00a0II molar relationship and increased overjet, 35\u00a0with posterior crossbite). In contrast to the oral function tests, some of the established motor scores showed a\u00a0clear floor effect. Statistically significant associations were identified between reduced oral function values and an enlarged overjet, a\u00a0class\u00a0II molar relationship, and a\u00a0posterior crossbite. This should be taken into account in neuromuscular evaluations.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusion<\/jats:title>\n                    <jats:p>In severely affected patients, oral function tests appear to be superior to established motor scores and fill a\u00a0diagnostic gap in research and clinical practice.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1007\/s00056-025-00597-8","type":"journal-article","created":{"date-parts":[[2025,6,24]],"date-time":"2025-06-24T08:50:00Z","timestamp":1750755000000},"page":"407-421","update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":0,"title":["Oral function tests in spinal muscular atrophy: closing the diagnostic gap in severely affected adult patients","Wie orale Funktionstests die diagnostische L\u00fccke bei schwer Betroffenen mit spinaler Muskelatrophie schlie\u00dfen"],"prefix":"10.1007","volume":"87","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-3431-7749","authenticated-orcid":false,"given":"Teresa","family":"Kruse","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sara","family":"Portegys","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Diana","family":"Leflerov\u00e0","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Annette","family":"Cap","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Brunhilde","family":"Wirth","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Raoul","family":"Heller","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Svenja","family":"Neuhoff","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Tim","family":"Hagenacker","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Bert","family":"Braumann","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Gilbert","family":"Wunderlich","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"297","published-online":{"date-parts":[[2025,6,24]]},"reference":[{"key":"597_CR1","doi-asserted-by":"publisher","first-page":"109","DOI":"10.3233\/JND-200475","volume":"7","author":"W Muller-Felber","year":"2020","unstructured":"Muller-Felber\u00a0W, Vill\u00a0K, Schwartz\u00a0O, Glaser\u00a0D, Nennstiel\u00a0U, Wirth\u00a0B et\u00a0al (2020) Infants Diagnosed with Spinal Muscular Atrophy and 4 SMN2 Copies through Newborn Screening\u2014Opportunity or Burden? 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B.\u00a0Wirth received honoraria for lectures, workshops and interviews from Biogen and AveXis\/Novartis. T.\u00a0Hagenacker received advisory and lecture honoraria and research support from Biogen, Roche and Novartis. G.\u00a0Wunderlich has received advisory board honoraria and speaker fees from Alnylam, Biogen, Hormosan, Pfizer and Sobi outside of the submitted work. D.\u00a0Leflerov\u00e0, A.\u00a0Cap, R.\u00a0Heller, S.\u00a0Neuhoff and B.\u00a0Braumann declare that they have no competing interests.","order":2,"name":"Ethics","group":{"name":"EthicsHeading","label":"Conflict of interest"}},{"value":"The study was approved by the Ethics Committee of the Medical Faculty of the University of Cologne (Reference Number: 19-1137) and by the Ethics Committee of the Medical Faculty of the University of Essen (Reference Number: 21-9851-BO). Written informed consent was obtained from the patients participating in the study.","order":3,"name":"Ethics","group":{"name":"EthicsHeading","label":"Ethical standards"}}]}}