Objectives <p>This study aims to present a rare case of unilateral pulsatile tinnitus (PT) induced by a contralateral dural arteriovenous fistula (DAVF) in a patient with ipsilateral otosclerosis-induced mixed hearing loss, highlighting the diagnostic challenges and management implications.</p> Methods <p>A 71-year-old female presented with persistent right-sided PT and hearing loss. Clinical examination, audiological assessments, contrast-enhanced computed tomography, magnetic resonance (MR) angiography, hearing tests, and transcanal recording were performed.</p> Results <p>The MR angiography revealed a left occipital artery-transverse sinus DAVF, contralateral to the subjective perception of right-sided PT. The average pure tone audiometry for the right ear showed air conduction/bone conduction thresholds of 80/48 dB, while the left ones were 26/20 dB. The subject declined all surgical interventions and opted for follow-up care by the first author.</p> Conclusions <p>Contralateral conductive hearing loss can obscure the true laterality of PT induced by DAVF. Clinicians should consider comprehensive imaging and objective assessments to accurately diagnose and manage vascular PT in patients with otosclerosis/conductive hearing loss. This report underscores the importance of a thorough otologic evaluation in patients with PT.</p>