A 17-year-old female with right deafness developed sudden left hearing loss without vertigo. She admitted to our hospital with an idiopathic fever that persisted for 3 months. Sensory hearing loss on both sides and inflammation shown on immunological blood examination were noted on admission. We pointed out differences in blood pressure between the radial artery and femoral artery. Magnetic resonance imaging and computed tomography showed complete occlusion of the left carotid artery with some calcification. We considered that hearing loss in this case might be associated with aortitis syndrome and continuous steroid therapy was administered. Physical findings and hearing were improved gradually.