Sudden deafness is acute onset of impaired hearing which develops within hours to few days. The commonly accepted audiometric criterion is a decrease in hearing of ≥ 30 dB, affecting at least three consecutive frequencies. Hearing loss is thought to involve several causative factors, including internal ear circulatory disturbances. We report the case of a female with an internal carotid artery (ICA) pseudoaneurysm in the distal cervical tract and unilateral sudden sensorineural hearing loss (SSNHL). As putative risk vascular factor, the patient had history of migraine since youth. Extensive screenings for autoimmune, rheumatic diseases, virological, and microbiological infections were negative. The patient denied recent cervical trauma. Furosemide and oral prednisone were given with initial benefit and withdrawn in 3 weeks. The patient experienced short-lasting episodes of headache, tinnitus, vertigo. Five weeks after first onset, she underwent magnetic resonance imaging (MRI) angiogram which revealed fusiform dilatation of left ICA in the cervical tract. It can be proposed, but it remains to be proved, that the pseudoaneurysm of the cervical ICA plays a role in the patient SSNHL in relation to turbulent flow or thromboembolism of branches to the inner ear.