Case report: Azathioprine - rescue of the only hearing ear in recurrent hearing loss?

Introduction The sympathetic hearing loss, similar to the sympathetic ophthalmia can occur after damage to the contralateral sensory organ. It is believed that damage to the cochlea leads to exposure of antigens that induce autoimmune triggered antibody mediated inflammation of the contralateral ear.

Medical history A 57-year-old patient presented with left unilateral deafness since the age of 10 and the question of CI implantation. The deafness on the left ear was questionably caused by a mumps infection. Since the age of 50, the patient has also reported about recurrent hearing loss on the right ear. Repeated therapies with intravenous and oral glucocorticoids each led to a recovery of the right hearing. However, after discontinuation of cortisone, the right auditory threshold has dropped again after a short time.

Results Neither instrumental diagnostics nor the long duration of deafness indicated a satisfactory rehabilitation result with CI-implantation. A probatory immunosuppressive therapy was started. The therapy regimen included Prednisolon 250mg and acetazolamide 250mg as maintenance dose each with reduction regime in combination with azathioprine 100 mg daily, which usually achieves its effect after 2-5 months. The hearing threshold was stable and consequently the glucocorticoid treatment could be completely stopped after 1 month and the acetazolamide after 9 months. Currently, the patient shows a constant hearing ability subjectively as well as in audiometric testing under the therapy of azathioprine 100mg daily.

Conclusions During the immunosuppressive therapy, no further hearing loss occurred on the right side, which could be an indication for a sympathetic hearing loss. In cortisone dependent hearing loss, long-term immunosuppressive therapy should be considered.

Publication History

Article published online:
10 June 2020