2025 Volume 84 Issue 4 Pages 204-212
Relapsing polychondritis (RP) is a rare, progressive systemic inflammatory disorder that targets the cartilage and proteoglycan. While occurrence of audiovestibular symptoms has been reported in 20%–50% of cases of RP, reports of detailed vestibular assessments remain limited. This study was aimed at clarifying the characteristics of audiovestibular dysfunction in patients with RP through comprehensive neuro-otological evaluations.
We retrospectively reviewed the data of five patients diagnosed as having RP based on the clinical symptoms and results of cartilage biopsy between 2009 and 2025. All the patients were evaluated by pure-tone audiometry and vestibular testing, including caloric testing, cervical vestibular evoked myogenic potential (cVEMP), and ocular VEMP (oVEMP), and their clinical omit outcomes were evaluated.
All five patients exhibited bilateral sensorineural hearing loss. Although only two patients reported dizziness or imbalance, vestibular function testing revealed moderate to severe bilateral canal paresis and VEMP abnormalities in all five cases. All the patients received steroid therapy, and while the hearing improved in two cases, vestibular dysfunction persisted in all three cases in whom the treatment outcomes were evaluated.
This study showed that detailed neuro-otologic evaluation frequently reveals the presence of asymptomatic vestibular dysfunction in patients with RP, which tends to be refractory to treatment. Therefore, potential balance impairments should be taken into consideration when managing patients with RP.
