Paraganglion bodies exist in the region of the middle ear as well as in other regions of the body. Tumors of this tissue occur in the ear and adjacent tissues. The symptoms and the extent of the tumor at diagnosis are largely dependent on the site of the paraganglion from which the tumor arises. The tumor in the ear has been recognized as an entity only recently. Much remains to be known of its natural history and of its response to therapy, and there exists diversity of opinion concerning the optimal method of treatment. Five patients showed varied response to irradiation therapy. The clinical course in one of these patients indicates cessation of growth after inadequate therapy. In contrast, two patients give evidence of continued tumor growth after irradiation therapy.