Otological Manifestations of Immunoglobulin G4-related Disease: A Review

This is the authors' abstract. We don't add key points for this paper.

The systemic autoimmune disease known as immunoglobulin G4-related disease (IgG4-RD) can affect any organ in the human body. Elevated serum IgG4 levels are usually associated with IgG4-RD and infections. The salivary glands, pancreas, lymph nodes, and inner ear are among the organs affected by this chronic fibroinflammatory disease. There have been reports of otological involvement in IgG4-RD in clinical settings. Otological involvement in IgG4-RD is accompanied by other organ manifestations. Patients with bilateral progressive or fluctuating mixed-type hearing loss, particularly if they respond to immunosuppressive therapy, are thought to have an autoimmune cause. This systemic disease can also present with otitis media, indicative of middle ear mucosal involvement. Additionally, vestibular dysfunction occurs in a nonwhirling manner in patients without a history of vertigo or imbalance. Diagnosis of this disease is based on clinical presentations, laboratory tests, and histopathological reports. Due to the substantial morbidity that IgG4-RD can cause, early identification and treatment are crucial. Chronic symptoms and a lack of response to conventional treatments should raise suspicion of IgG4-RD.