How is Cogans syndrome treated? What is the prognosis?
Treatment includes topical steroids for anterior ocular manifestations and high-dose glucocorticoids for posterior ocular, audiovestibular, and systemic disease. Failure to taper prednisone and treatment-resistant disease is treated with immunosuppressives (methotrexate, azathioprine, and mycophenolate mofetil). Cyclophosphamide is used to treat large- and medium-vessel vasculitis. Patients who fail to respond have been treated with cyclosporine. TNFα antagonists and tocilizumab have been used in case reports.
Some patients recover after a single episode. Most patients have exacerbations with over 50% sustaining permanent hearing loss. Cochlear implants can be beneficial for these patients. Vascular surgery may be necessary for aortic valve replacement or aneurysm repair.
