Rituximab ameliorated severe hearing loss in Cogan's syndrome: a case report

Abstract

BackgroundRituximab is a monoclonal antibody inducing depletion of B lymphocytes and presently approved for the treatment of non-Hodgkin's lymphoma and rheumatoid arthritis. Here is the first report of the use of this drug in a case of Cogan's syndrome (CS).Case Presentationa 25-year-old Italian woman was referred with conjunctival hyperaemia, interstitial keratitis, moderate bilateral sensorineural hearing loss accompanied by tinnitus, dizziness, nausea and vertigo, poorly responsive to oral and topical steroidal therapy. Diagnosis of typical CS was made. The administration of a combined immunosuppressive treatment resolved ocular inflammation, dizziness, nausea, and vertigo but gave little results in controlling progressive hearing loss. A noticeable improvement in hearing function was documented by pure tone audiometry after infusion of Rituximab.Discussionin CS, hearing function is often the most difficult parameter to control with therapy. A positive effect of Rituximab on was observed in our case. The drug also allowed to significantly reduce the number of adjuvant immunosuppressive medications.

Diagnostic Difficulties of Cogan Syndrome

“…If the response is incomplete, the addition of another immunosuppressive agent (methotrexate, cyclophosphamide, azathioprine, cyclosporine) should be considered [8]. Observations have also been made on the positive effect of rituximab [16], and anti-TNF blockers [17] in the treatment of patients with CS.…”

Cogan's syndrome is a new nosological entity in the current classification of systemic vasculitides: A clinical case and a review of literature

“…В последние годы накапливаются сообщения об эф-фективности при СК ингибиторов фактора некроза опухо-ли α (этанерцепт, инфликсимаб), анти-В-клеточной тера-пии (ритуксимаб), тоцилизумаба [13,37,[41][42][43][44].…”

Cogan and Behcet Syndromes

“…39 Rituximab was effective in improving hearing in a woman with CS with IK and vestibular symptoms. 40 Cochlear implants have been shown to be beneficial in patients with end-stage hearing loss from CS. 41,42 This surgical intervention is equally as effective in CS as in non-AIED hearing loss and significantly improves the quality of life of these patients.…”