A rare case of aortitis syndrome with fluctuating sensorineural hearing loss.

A 40-year-old female with right hearing loss had sudden left hearing loss and tinnitus without vertigo. Both ears responded well to steroid (Rinderon) administration with improvement to 43dB on the left and 68dB on the right. We found a pulsating tumor on the right side of the neck, Raynaud's phenomenon and differences in blood pressure between the two radial arteries. The serum level γ-globulin was abnormally high. The CRP was highly positive, and the ESR was increased. Aortography showed a narrowed aortic arch with swelling of the aortic wall. The right neck tumor was biopsied, and the histopathological diagnosis was aortitis syndrome. Continuous steroid administration improved the physical findings and hearing, but mild tinnitus persisted.