Functioning middle ear neuroendocrine tumor: A rare case of systemic activity

Introduction: Middle ear neuroendocrine tumors (MeNETs) are rare neoplasms with dual epithelial and neuroendocrine differentiation. They typically present as indolent lesions with nonspecific symptoms, making diagnosis challenging. Case Report: We present a case of a functioning MeNET in a 59-year-old male with left-sided hearing loss, ipsilateral postauricular and occipital pain, and systemic symptoms suggestive of neuroendocrine activity. Surgical excision and histopathology confirmed a grade 1 neuroendocrine tumor. Conclusion: This case represents the first reported instance of a functioning MeNET, broadening the understanding of this tumor’s potential systemic activity. It highlights the importance of thorough clinical evaluation, surgical management, and long-term surveillance, as well as the need for further research into the pathophysiology of MeNETs