Abstract: IgG4-related disease (IgG4-RD) is a systemic autoimmune fibroinflammatory disease characterized by multiorgan infiltration of IgG4-positive plasma cells, fibrosis and vasculopathy that determine dysfunction of the affected organ. This review aims to characterize the otologic manifestations of the disease. We have conducted a systematic review of the biomedical literature published describing cases of IgG4-RD with otologic manifestations. For the review, the bibliographic databases utilized were Pubmed, Web of Science and Scopus. We selected 48 cases from which we extracted several data collections. About 52% of the patients were male between the ages of 19 and 79 years. Otologic findings were characterized by pseudotumoral lesions that most often caused a clinical presentation similar to otitis media with effusion, with cochlear involvement or sensorineural hearing loss (uni- o bilateral). Less frequent presentations included auricular chondritis, eosinophilic otitis, or hypertrophic pachymeningitis. In 32 patients (67%) the otologic manifestation was the first symptom of the ER-IgG4. Sixteen patients (33%) were treated with mastoidectomies as a result of delayed diagnosis and lack of knowledge about this entity. Two patients needed a cochlear implant. Although the otologic manifestations of IgG4-RD are unusual, it is important for the otorhinolaryngologist to know the otologic manifestations of this entity as it can be the onset of the disease, in order to allow early diagnosis and adequate treatment, avoiding permanent sequelae.

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