Neuroendocrine adenoma of the middle ear is a rare tumor of the middle ear. The origins, as well as classification of the disease, are still controversial. We reported 2 cases of neuroendocrine adenoma of the middle ear separately in a 60-year-old male and a 48-year-old patient who both presented with hearing loss. This article reviewed the reported literatures and summarized the pathogenesis, clinical features, imaging manifestations, pathological features, and biological behavior of neuroendocrine adenoma of the middle ear, so as to improve the understanding of the disease.
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