Cogan's Syndrome

Core Messages • Cogan’s syndrome (CS) is a rare disease, probably of organ-specifi c autoimmunity, likely triggered by an infection. • Typical CS is characterized by nonsyphilitic interstitial keratitis associated with middle ear symptoms with progressive hearing loss. • CS becomes atypical in case of different type of eye and/or ear involvement or when the interval between the initial manifestations is longer than 2 years. • Systemic corticosteroids therapy is the most effective treatment especially in the presence of compromised auditory acuity. • The immediate steroid treatment can prevent hearing loss. • In case of failure or insuffi cient response to corticosteroids, immunomodulatory agents can be useful.