Ankylosing spondylitis (AS) is a rheumatic disease characterized by chronic inflammation. The aim of this study was to evaluate the functions of the cochlea and the vestibular system in patients with AS. The study group consisted of 32 patients with AS and 30 healthy volunteers as a control group. Otorhinolaryngologic examinations were performed in all patients together with pure-tone audiometry, speech tests, impedancemetry, transient evoked otoacoustic emission (TEOAE) and electronystagmography (ENG). A significant difference was found between the 2 groups with regard to pure-tone averages at high frequencies in each ear (p < 0.05). The rates of reproducibility in TEOAE testing were significantly lower in patients with AS (p = 0.03). The signal-to-noise rates of the response values were lower at all frequencies in patients with AS, but a statistically significant difference was only observed at 2, 3 and 4 Hz (p < 0.05). ENG revealed pathologies in 11 patients with AS (34%), 8 of which were central (25%) and 3 of which were peripheral (9%). No correlation was found between cochleovestibular dysfunction and age, sex, disease duration, activity and medication taken. This study demonstrated that there is an association between AS and cochleovestibular dysfunction.