Sarcoidosis is a common multisystemic granulomatous disorder affecting several organs and tissues. However, the respiratory tract is the region commonly involved in more than 90% of patients, and the middle ear is a direct extension of it. In spite of this, direct middle ear and/or mastoid involvement of sarcoidosis is more rarely seen. Otological involvement may mimic a number of other diseases of the ear; sarcoidosis will probably not be considered prospectively. In addition, pulmonary symptoms of the patients often go unnoticed for some time. We report a patient presenting with hearing loss and tinnitus as the primary manifestation of sarcoidosis of the ear. © 2009 Elsevier Inc. All rights reserved.