In recent years, an immune-mediated disorder involving IgG4 has been described, which targets multiple organs and explains a number of disorders previously regarded as "idiopathic" or of unknown origin. Furthermore, the discovery of IgG4-related disease (IgG4-RD) has placed a number of pathologies within its spectrum, linking symptoms and conditions formerly considered isolated. Reports of the manifestations of IgG4-RD in the head and neck are scarce. Otological manifestations have been reported, but only a handful of cases are available in the literature. This is the first report of recalcitrant serous otitis media secondary to IgG4-RD, confirmed by immunohistopathology. A case of IgG4-RD of the middle ear is presented, manifesting itself as recalcitrant serous otitis media. The case is presented from an otolaryngological and histopathological perspective and briefly reviews this rare disorder. The importance of the awareness of IgG4-RD resides mainly in the fact that it is a treatable condition. This can potentially improve the quality of life of a number of patients, some of whom may not have had a clear diagnosis. A favorable response to glucocorticoids has been reported. In cases of persistent symptoms, immunosuppressive therapy has been used with success.
Keywords: IgG4-related disease; autoimmune ear pathology; chronic otitis media.