ABSTRACT
Cogan syndrome is a rare autoimmune disease that is characterized by acute with Audiovestibular dysfunction. Bilateral and progressive profound sensorineural hearing loss is the most important sign of the disease. Delay diagnosis leads to deafness or death and atypical form has not complete response to therapy. The purpose of this case report is presenting the sign and symptom of syndrome and advancing knowledge for early diagnosis and reporting an exceptional case. The patients was a 51-y male with chief complaints of eye redness and then hearing loss and typical vertigo. Clinical and Para clinical data rule out is the main differential diagnosis of syndrome [sarcoidosis, syphilis] and finally the Cogan syndrome was the diagnosis. Treatment with early high dose corticosteroid was used and fortunately good response was observed. The diagnosis of Cogan syndrome is based on classic finding and rule out of differential. The main differential diagnosis of Cogan syndrome includes syphilis, sarcoidosis, [PAN] all causes of peripheral vertigo. Clinical and Para clinical findings lead to diagnosis of Cogan syndrome. Exceptional items in this case: 1- Cogan syndrome is the disease of young adults, but this case was 51 year old 2- Atypical from of Cogan has not good response to drugs. But our patient completely recovered by using high dose prednisolone. At end we have to say that complete knowledge about the symptom of inner ear autoimmune disease and early treatment led to better response even in the case of atypical form