Acute hearing loss (AHL) has traditionally been considered to be a neglected and underestimated symptom of stroke. However, because the blood supply to the auditory system originates from the vertebrobasilar system, stroke in the distribution of the vertebrobasilar circulation can present with acute hearing loss (AHL) and/or tinnitus. Approximately one-tenth of vertebrobasilar ischemic stroke (VBIS) is accompanied by AHL. Sometimes, AHL is a warning symptom of impending VBIS (mainly in the anterior inferior cerebellar artery). In this case, the MRI is normal, and the clinician must rely on other clinical features to make the diagnosis. This review summarizes the current advances in the clinical syndromes and signs of AHL due to VBIS.