VEXAS syndrome with cochleovestibular involvement.

We report a case of VEXAS syndrome in a 71-year-old man presenting with auricular chondritis, scleritis, hearing loss, and vestibular dysfunction. Although the clinical picture initially suggested relapsing polychondritis, hematologic findings and UBA1 variant confirmed VEXAS syndrome. Comprehensive audiovestibular testing revealed bilateral sensorineural hearing loss and global vestibular impairment. Steroid therapy led to partial improvement. This case highlights the potential for inner ear involvement in VEXAS syndrome, underscoring the importance of systematic audiovestibular evaluation and clinical awareness to differentiate it from similar conditions such as relapsing polychondritis.