DOI: https://doi.org/10.36347/sjmcr.2026.v14i07.016

Pages: 1675-1679

Introduction: Tympanic paraganglioma is a rare tumor that predominantly affects women. Type 4 is uncommon. It can clinically and radiologically mimic a cholesteatoma. Clinical Case: We present a case of a type 4 tympanic paraganglioma in a 63-year-old male with no significant past medical history who presented with right-sided hearing loss and tinnitus that had been progressing for 15 months, complicated by ear pain. Otoscopic examination revealed a non-pulsatile mass in the external auditory canal. The Weber and Rinne tests, as well as the audiogram, demonstrated conductive hearing loss. Computed tomography (CT) scanning revealed a mass occupying the middle ear with mild bone erosion and extension into the external auditory canal, suggesting a cholesteatoma. Laboratory tests were normal. Surgical resection revealed a firm, brownish, polypoid lesion measuring 2 x 1.5 cm. Histologically, a tumor proliferation arranged in nests was observed within a hypervascularized stroma. The tumor cells had regular nuclei with “salt-and-pepper” chromatin. Immunohistochemical analysis revealed that the tumor cells expressed synaptophysin and chromogranin A. Pancytokeratin was negative. S100 highlighted the sustentacular cells, and Ki-67 was low. The postoperative course was uneventful. The prognosis was favorable after 10 months of follow-up. Conclusion: The symptoms and erosive appearance on imaging may lead to a misdiagnosis of cholesteatoma. Pathological examination then corrects the diagnosis. Testing for germline mutations is necessary to identify familial forms.