Ulcerative colitis (UC) is characterized by an inflammatory disorder of the gastrointestinal tract. Immune-mediated extraintestinal manifestations of UC have increasingly attracted attention in the literature recently, for which UC is now considered as a systemic disease. Neurologic involvement associated with UC is probably under-reported because of the unawareness of many physicians, although early recognition and treatment are crucial in preventing major morbidity and sequel. In this case report is presented a patient newly diagnosed as UC, who developed both sensorineural hearing loss and intractable status epilepticus that we suggest to have resulted from immune-mediated mechanisms.