Behavioral Correlates of Caregiver-Reported Oral Health of Individuals With Joubert Syndrome: A Cross-Sectional Observational Study.

Aim: To identify the behavioral correlates of caregiver-reported oral health of individuals with Joubert Syndrome (JS).

Methods: This cross-sectional study included 302 caregivers of individuals with JS from the JS registry who responded to a 56-item REDCap survey, and a purposive subset of 30 participants with JS for which a dental screening was completed. The primary outcome was caregiver-reported oral health of individuals with JS (good/very good vs. fair/poor). Logistic regression models were used to generate confounder-adjusted odds ratios (OR) and evaluate associations between oral health behaviors and caregiver-reported oral health of individuals with JS (p < 0.05).

Results: The mean age of individuals with JS was 14.6 years (SD: 10.3). Most caregivers were female (84.1%), White (87.4%), non-Hispanic (91.1%), and reported annual household incomes above $100 000 (53.7%). Behaviors significantly associated with poorer oral health included not eating or drinking by mouth (OR = 4.52, p = 0.005), not toothbrushing daily (OR = 5.29, p < 0.001), and infrequent toothpaste use (OR = 3.68, p = 0.003). Screenings showed 40.0% had poor hygiene, 23.3% had untreated caries, and 46.7% had gingivitis.

Conclusions: Future efforts should focus on improving oral health behaviors to prevent dental disease in individuals with JS.