Tocilizumab Successfully Treating Refractory Hearing Impairment in a Patient With Cogan Syndrome: A Case Report and Review of the Literature.

ABSTRACT

Cogan syndrome (CS) is a rare systemic vasculitis characterized primarily by nonsyphilitic interstitial keratitis and vestibular and auditory dysfunction. In this article, we report the case of a 31-year-old male diagnosed with CS for 1 year. He was admitted to the hospital with fever, dizziness, headache, tinnitus, and hearing loss. After being treated with glucocorticoids, cellular immunosuppressants, and infliximab therapy, his symptoms were greatly relieved except for hearing loss. Then, he attempted to use tocilizumab (TCZ) which was ultimately effective in controlling the auditory dysfunction. In addition, we found 4 cases of TCZ for CS through a literature review and compared them with our patient. Although glucocorticoids are still the first-line treatment for CS, TCZ therapy provides fresh hope for patients who have refractory hearing impairment with hormone resistance, or whose hormone dosages cannot be lowered to maintenance levels.