Association between Cogan's syndrome and inflammatory bowel disease: a case series.

BACKGROUND AND AIMS Cogan's syndrome (CS) is a rare autoimmune disease with less than 250 cases reported. It mainly affects the audiovestibular system and the eyes frequently resulting in deafness. Inflammatory bowel disease (IBD) consists of two subtypes, Crohn's disease (CD) and ulcerative colitis (UC), and represents a common form of chronic intestinal inflammation. Here, we report an association between CS and IBD in four patients. METHODS Patient data were collected using a questionnaire that was sent to one of our outpatients and three of the 13 members of the German CS self-help group. RESULTS In all Cogan patients with IBD (3 female with UC, 1 male with CD), intestinal disease was diagnosed years before the onset of CS. After suffering from a complicated IBD disease course, they suddenly developed CS-related symptoms, such as hearing loss, tinnitus or eye inflammation. Three of them went deaf within a few years after diagnosis. Although all of them had been on immunosuppressive IBD therapy, these treatment regimens did not prevent the onset of CS. CONCLUSIONS Our data suggest a strong association of IBD and CS. Since CS rapidly leads to bilateral deafness, it seems to be a rare, but nevertheless important disease that can occur in association with IBD. However, neither an early diagnosis nor an immunosuppressive therapy seems to efficaciously prevent disease progression.