Highlights • Dissecting basilar artery aneurysm (DBAA) is relatively rare. • We report the first case of a DBAA manifesting as sudden sensorineural hearing loss. • This case report adds to the symptom spectrum of DBAA., Dissecting aneurysms of the basilar artery are rare and severe entities with high hemorrhage and mortality rates. Common clinical symptoms of this condition include cerebral ischemia, subarachnoid hemorrhage, and neurological deficits induced by space-occupying effects. Vertigo, headache, and cranial nerve paralysis are also frequent symptoms. We report a case of dissecting basilar artery aneurysm manifesting as sudden sensorineural hearing loss. A 53-year-old man presented with vertigo, left-sided tinnitus, and sudden sensorineural hearing loss for 1 hour. A physical examination showed left-sided hearing loss and bilateral horizontal nystagmus, and a radiological examination showed a dissecting aneurysm originating from the basilar artery. A diagnosis of a dissecting aneurysm of the basilar artery with sudden sensorineural hearing loss was made. The aneurysm was treated using stent-assisted coil embolization. Postoperatively, the vertigo and tinnitus, as well as the left hearing loss, greatly improved. Follow-up digital subtraction angiography 10 months after the operation showed complete disappearance of the dissecting aneurysm of the basilar artery. In patients with sudden sensorineural hearing loss, clinicians should be aware of the potential of a dissecting basilar artery aneurysm. An interdisciplinary approach is important in diagnosis and treatment of this rare condition.