Otological Manifestations of Systemic Diseases

A wide variety of systemic diseases can present with otologic symptoms and findings, including acute or chronic otitis media, conductive, sensorineural, and mixed hearing loss, and facial nerve palsy. Their definitive and differential diagnosis, as well as their therapeutic management, is fraught with difficulty because the recognition of unknown or rare systemic diseases requires a high index of suspicion. Therefore, a good knowledge of the otologic manifestations of systemic diseases is essential. With this in mind, we have attempted to review the many and varied systemic diseases that can cause otologic manifestations, such as granulomatous (Langerhans cell histiocytosis, granulomatosis polyangiitis, and sarcoidosis), infectious (tuberculosis, syphilis, Lyme disease, mycoses, and congenital viral diseases), rheumatic (rheumatoid arthritis, Caplan’s syndrome, Felty’s syndrome, Still’s, and Sjögren’s syndrome), malignant (multiple myeloma, leukemia, lymphoma, and neurofibromatosis), musculoskeletal (Paget’s disease, osteogenesis imperfecta, fibrous dysplasia, and osteopetrosis), metabolic (mucopolysaccharidoses, gout, ochronosis, and glutaric aciduria), autoimmune (systemic lupus erythematosus, and relapsing polychondritis), and immunodeficiency (primary or congenital immunodeficiency, and acquired immunodeficiency-AIDS) disorders.