Abstract: Objective To explore the clinical characteristics, laboratory examination, pathological features, diagnosis, and treatment of a rare case of granulomatosis with polyangiitis(GPA)with initial otological symptoms combined with a nasopharynx lesion. Methods This article retrospectively analyzed the clinical case, summarized its characteristics, and reviewed related literature at home and abroad. Results This patient's earliest clinical symptoms included otitis media, labyrinthitis, and a nasopharynx lesion. Then came the facial paralysis, and the trigeminal nerve stimulation symptoms worsened gradually. Only acute and chronic infiltrating inflammatory cells were found in the middle ear and nasopharynx lesion biopsies. The antibiotics were then modified, and the mastoidectomy surgery was performed to avoid petrosal bone and cerebral invasion. Despite improvements in the local inflammation of the middle ear, the earache, facial palsy, and other symptoms did not improve. Glucocorticoid therapy, on the other hand, appeared to be helpful. As a result, we completed appropriate autoimmune disease examinations and had another nasopharynx biopsy. Finally, the GPA diagnosis was confirmed. Glucocorticoid and immunosuppressive treatments alleviated symptoms. For three months following surgery, there was no obvious evidence of lung or renal invasion. Conclusion In clinical practice, the diagnosis of GPA should be considered when certain atypical otitis media is repeatedly unhealed after conventional treatment but sensitive to glucocorticoids, along with nasopharynx lesions, sinusitis, lung or kidney involvement, and gradual invasion of the inner ear and cranial nerve manifesting as vertigo attacks and facial palsy. When repeated pathological biopsies fail to reveal specific diagnostic evidence, dynamic monitoring of anti-neutrophil cytoplasmic antibody, erythrocyte sedimentation rate, urine occult blood, chest CT, and serum creatinine are also important.

Key words: Granulomatosis with polyangiitis(Wegener's granulomatosis), Otitis media, Labyrinthitis, Nasopharynx occupied lesion, Anti-neutrophil cytoplasmic antibody