[IgG4-associated diseases mainly with auricular symptoms: a case report and literature review]

Summary To analyze the clinical features of IgG4-related diseases mainly with otologic manifestations and investigate diagnosis and treatment of IgG4-related diseases. We report the clinical course and diagnosis and treatment process of a case of IgG4-related disease misdiagnosed as chronic suppurative otitis media. After looking through relevant literatures, we review 22 cases of IgG4-related diseases mainly with otologic manifestations, and summarize their epidemiological characteristics, clinical manifestations, histopathologic features, radiologic features, and diagnostic and therapeutic criteria. IgG4-related diseases mainly with otologic manifestations are usually clinically characterized by atypical symptoms such as otalgia, tinnitus, aural fullness, otorrhea and progressive or fluctuating hearing loss, together with nausea, vomiting, and dizziness. The three central histopathologic features are dense lymphoplasmacytic infiltration, storiform fibrosis and obliterative phlebitis. The exact diagnosis of IgG4-related diseases requires a combination of clinical manifestations, serology, histopathology and radiologic results. Glucocorticoids are currently the first-line approach for IgG4-related diseases. Otologic manifestations involved in IgG4-related diseases are poorly specific, which is likely to result in more misdiagnosis and treatment delay. Current golden standard for diagnosing IgG4-related diseases is the identification of characteristic histopathology. In addition, immunohistochemistry can help clinicians diagnose IgG4-related diseases as early as possible. Otologists should strengthen their understanding of otologic manifestations of IgG4-related diseases. Early diagnosis and treatments are the key factors in prognosis.