To date, there have only been a few studies on the oral health-related quality of life (OHRQoL) of people with Ehlers-Danlos syndromes (EDS) and the oral symptoms. The aim of this study was, therefore, to analyse the OHRQoL of people with EDS from their own point of view as well as obtain information about their age at the time of the diagnosis, the period of time until diagnosis, and the presence of oral symptoms (if any) and their association with oral health quality.
The study was designed as an anonymous questionnaire-based cross-sectional study. We conducted a descriptive analysis of the Oral Health Impact Profile-14 (OHIP-14) scores, age of the participants, age at diagnosis, and the time period between the first signs of the disease and the diagnosis of EDS. To verify the differences in OHIP-14 scores between patients with and without oral symptoms, a Mann-Whitney U test was performed.
A multivariate quantile (median) regression analysis was performed to evaluate the effect of different general characteristics (gender, age, and the presence of oral symptoms) on the OHIP‑14 scores. Furthermore, using a Mann-Whitney U test, the influence of different oral symptoms was verified by testing the differences between patients without any oral symptoms and patients with a specific diagnosis.
A total of 79 evaluable questionnaires from 66 female (83.5%) and 13 male (16.5%) participants were analysed. On average, after the first symptom, it takes 18.36 years before EDS are correctly diagnosed.
Oral symptoms were described by 69.6% of the participants. The OHIP-14 score was 8 (10) points for patients without oral symptoms and 19 (15) for patients with oral symptoms. The quantile regression for OHIP-14 scores, depending on oral symptoms, was highly significant (p = 0.0007). OHIP-14 scores for dysgnathia, periodontitis, TMD, a high-arched palate, malocclusion, and the anomaly of tooth formation were significantly different between the participants with and the participants without oral symptoms.
Long diagnostic pathways seem to be a typical problem in patients with EDS. Oral symptoms associated with the underlying disease occurred regularly and showed a negative correlation with OHRQoL.