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Objective To improve early diagnosis of patients who have basilar artery occlusion (BAO) and to provide evidence for treatment decisions based on clinical characteristics, treatment strategies, and prognostic outcomes of cases of bilateral hearing loss as a prodromal symptom.Methods We conducted a retrospective analysis of six patients who had BAO with an initial symptom of hearing loss. This analysis encompassed demographic data, clinical symptoms, examination findings, treatment approaches, and prognostic outcomes.Results Six patients (mean age 62±16.5 years) presented with bilateral sudden hearing loss and were diagnosed with proximal BAO. All had subtle initial symptoms, leading to diagnostic delays (mean 13 ± 5.4 hours). Five underwent endovascular treatment (EVT), and all patients required rescue balloon angioplasty because of underlying atherosclerotic stenosis. Intravenous tirofiban was administered perioperatively. At discharge, hearing had normalized in four patients; one patient (patient 3) had persistent profound bilateral sensorineural hearing loss. The patient who did not undergo EVT (patient 6) died on day 5 of hospitalization. At 90 days, five EVT-treated patients achieved favorable functional outcomes (modified Rankin Scale score 0–1). Univariate analysis did not show statistically significant differences between outcome groups in baseline National Institutes of Health Stroke Scale (NIHSS) score, posterior circulation Alberta Stroke Program Early CT score, onset-to-arrival time, or age, although patients with unfavorable outcomes tended to be older and have higher NIHSS scores.Conclusions Sudden bilateral hearing loss should serve as a critical warning sign of BAO, a potentially life-threatening condition. Without rapid intervention, such as intravenous thrombolysis or EVT to re-establish perfusion, the occlusion may progress, leading to severe and potentially fatal outcomes.