Aortitis syndrome is known to be an autoimmune disease, which has many symptoms of occulusive thromboaortopathy. It has recently been reported that the patients with systemic autoimmune diseases may have sensorineural hearing loss and that steroid therapy should be effective not only for the original disorder but also for the associated hearing disturbance.
Eleven patients (3 males and 8 females) with aortitis syndrome and sensorineural hearing loss have been treated in Kitasato University Hospital during the 16 years since the opening of the hospital.
The onset of hearing impairment was at 32 to 48 years of age, 10 years older than the mean age of onset of the aortitis syndrome reported in the literatures.
Various types of hearing impairment were noted : slight hearing loss, total deafness of sudden onset and fluctuating type. Tinnitus was associated with hearing loss in many cases.
Several patients also had vertigo and dysequilibrium, and in some of them the diagnosis was peripheral vestibular hearing loss, although in the cases of aortitis syndrome with hearing loss reported in the literature neither vertigo nor dizziness was mentioned.
The usual treatment for acute stage sudden deafness was not effective in most of our patients. However, one female patient treated with long-term steroid therapy for systemic arteritis showed marked improvement of both her hearing and her general condition.
We cannot conclude that the origin of sensorineural hearing loss is arteritis in the inner ear in all cases of aortitis syndrome with hearing loss. However, our cases of aortitis syndrome with steroid-dependant hearing loss indicate that the possibility remains of hearing loss due to arteritis of the labyrinthine artery in aortitis syndrome.
