We report a rare case of bilateral hearing loss and diffuse alveolar hemorrhage associated with microscopic polyangitis (MPA). A 75-year-old woman complained of hearing loss. Two months later, she was admitted due to rapidly progressing dyspnea. Chest radiography and CT scan showed diffuse bilateral consolidations. Mechanical ventilation was required for respiratory insufficiency. Laboratory data demonstrated anemia and renal failure. Steroid pulse therapy and antibiotics were initiated. On day 2, bronchoalveolar lavage showed fresh blood-like fluid, which suggested diffuse alveolar hemorrhage (DAH). Serum level of myeloperoxidase-antineutrophil cytoplasmic antibody (MPO-ANCA) was elevated. Therefore, DAH and rapidly progressive glomerulonephritis associated with MPA were diagnosed. Treatment was also performed with cyclophosphamide pulse therapy and plasmapheresis. Her pulmonary lesion improved dramatically. We emphasize the effectiveness of bronchoalveolar lavage for prompt and accurate diagnoses. Moreover, this case report also suggests that early cyclophosphamide therapy and plasmapheresis may be an effective treatment for MPA with diffuse alveolar hemorrhage. In contrast, hearing loss did not improve after therapy. Some cases reported hearing loss as a rare symptom of MPA. We also suspect that hearing loss may be a complication of MPA. We reported this case because there has been no description of hearing loss accompanying DAH associated with MPA.

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