Ankylosing spondylitis (AS) is a chronic systemic inflammatory disorder that primarily affects the spine and sacroiliac joints. Recent studies described audiovestibular impairment in AS patients. The aim of this study was to evaluate the hearing and function of the cochlear system in patients with AS. Thirty-seven AS patients and 20 healthy controls were evaluated prospectively. Otorhinolaryngologic examinations were performed in all patients together with pure tone audiometry, speech discrimination test, tympanometry, and distortion product otoacoustic emission (DPOE). Disease duration, Bath Ankylosing Spondylitis Disease Activity Index (BASDAI) scores, and hematologic findings (CRP and ESR) were also collected. Pure tone audiometry findings of the patients and controls were significantly different in all frequencies (p < 0.01). Speech discrimination scores were also significantly different (p < 0.01). No significant difference was found between DPOE responses of the patients and controls (p > 0.05). There was no correlation between disease duration, BASDAI scores, hematological findings, and audiometry findings (p > 0.05). This study demonstrated that there is an association between AS and hearing loss, but the cochlea is not the main source of hearing loss.