Objective. To study the effect of specific clinical, laboratory, and imaging parameters on the course of severe (type 1) malignant external otitis (MEO). Study Design. Case series with chart review. Setting. Tertiary, university-affiliated medical center. Subjects and Methods. Fifty-seven patients hospitalized with severe MEO were followed for disease course and survival in a tertiary center between 1990 and 2008. Results. In 20% of patients, disease was persistent and/or aggressive despite prolonged and extensive treatment. Of this subgroup, 45% died of the disease. Prognostic factors of persistent/aggressive disease were facial nerve paralysis, bilateral disease, and significant major computed tomography findings (temporomandibular joint destruction, infratemporal fossa or nasopharyngeal soft tissue involvement). Cultures grew fungi in 5 patients, and follow-up imaging revealed disease progression. The overall 5-year survival was 55% for patients with short-term disease and 40% for patients with persistent/aggressive disease (P = .086). By age, 5-year survival was 75% in patients younger than 70 years old and 44% in older patients (P = .029). Conclusions. A significant subset of patients with MEO has a prolonged, aggressive, and highly fatal disease that needs to be identified early. These patients more frequently have bilateral disease, cranial nerve paralysis, and positive computed tomography findings. Their follow-up should routinely include imaging studies to evaluate disease progression, and every effort should be made to identify and treat underlying fungal infection.