Sudden Hearing Loss: Could it be Inflammatory Bowel Disease?

Abstract

Purpose: Cogan's syndrome (CS) is a rare autoimmune disorder characterized by ocular and vestibuloauditory symptoms. We describe a patient initially diagnosed with CS and was later found to have Crohn's disease (CD). Case: A 36-year-old woman with no medical history initially developed acute eye pain and photophobia. After evaluation by ophthalmology, she was treated for iritis with a topical steroid. A week later, she developed unilateral hearing loss. Rheumatology workup revealed negative antinuclear antibody, antinuclear cytoplasmic antibody, rheumatoid factor, hepatitis serologies and HLA-B27. Erythrocyte sedimentation rate and C-reactive protein were normal. Chest x-ray was negative for lymphadenopathy or other lesions. She was diagnosed with CS and refused Methotrexate due to potential side effects. A month later she developed panuveitis despite topical steroid therapy. Adalimumab was given, but she had an injection site reaction which caused her to stop the medication. She then presented to GI clinic with an 8 lb weight loss and new-onset intermittent, lower abdominal pain. She denied nausea, vomiting, diarrhea, constipation, or rectal bleeding. Colonoscopy was performed, which revealed a normal colon but multiple ulcers in the terminal ileum. Pathology showed chronic active ileitis consistent with Crohn's disease. MR enterography revealed mild wall thickening and enhancement involving a 3 cm segment of terminal ileum. The patient was diagnosed with CD five months after her initial symptoms began. Discussion: Less than 250 case reports of CS have been described. The syndrome is defined in two forms: “typical” or “atypical.” Typical CS is characterized by nonsyphilitic interstitial keratitis affecting one or both eyes. A majority of patients develop symptoms in the eye or ear alone, but rapidly involve both within three months. Atypical CS includes other inflammatory complications such as eye involvement other than interstitial keratitis. In contrast to typical CS, there is often a delay of more than two years between ocular and audiovestibular manifestations. Previous case series show an association with inflammatory bowel disease. In this population, CS symptoms often precede inflammatory bowel disease (IBD) diagnosis. On the other hand, auditory impairment is a rare extraintestinal manifestation of IBD. We use this case to stress the importance of evaluating for IBD in a patient with auditory and visual complaints.