Steroid-responsive sensorineural hearing loss associated with aortitis syndrome

Five cases of sensorineural hearing loss associated with aortitis syndrome are presented, and their clinical features are discussed in detail. All patients were middle-aged females. Pure-tone audiometry revealed a high-tone gradual-loss type of configuration, and the recruitment phenomenon was proved to be positive in most cases. The degree of hearing loss correlated well with the erythrocyte sedimentation rate. However, the most remarkable clinical feature was that the hearing loss showed steroid responsiveness in all cases. Based on these clinical features, it was suggested that the steroid-responsive sensorineural hearing loss associated with this syndrome might not be an incidental accompanying symptom but rather one of the local manifestations of the disease arising from similar mechanisms as the systemic inflammatory process. Associated conductive disturbance is also discussed.