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

a 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.in 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.Rituximab is a chimeric human-mouse monoclonal antibody against lymphocyte CD20 surface antigen, and treatment induces depletion of B lymphocytes by various mechanisms. These antibodies are thought to act in vivo mostly through activation of antibody-dependent cell-mediated cytotoxicity and complement-dependent cytotoxicity, although direct growth inhibition and/or induction of apoptosis may also take place [1]. Rituximab is 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 severe Cogan's syndrome (CS).A 25-year-old Italian woman developed severe bilateral sensorineural hearing loss over the course of 12 months from her first acute inflammatory episode characterized by conjunctival hyperaemia, interstitial keratitis and tinnitus. Before the admission, the patient had already experienced a 3-week regimen of oral prednisone (50 mg/day at regressive dosage) and dexametasone eye-drops with poor response. After the patient had been screened for a possible infectious origin of her symptoms and all other known causes of interstitial keratitis had been exclu