Introduction:First described in 1976, the neuroendocrine adenoma of the middle ear (NEAME) is an extremely uncommon entity accounting for <2% of all middle and inner ear neoplasms. Because of its rarity, an overall assessment of this pathology is lacking in the current literature which is mostly limited to case reports and small case series. The dual histological differentiation with both glandular and neuroendocrine features as well as its non specific clinical manifestation with hearing loss, tinnitus and aural fullness make its diagnosis a challenge for both pathologists and otolaryngologists. The objective of this case report is to extend the current literature about this unusual pathology contributing to a better understanding of the disease. Material:We present the case of a 63-year old woman with left sided conductive hearing loss. Since the otoscopic findings were suspicious for a cholesteatoma, she was scheduled for middle ear surgery. Intraoperatively, a tumor mass was found invading the lateral semicircular canal and the ossicular chain. Results:After the NEAME diagnosis, a radical mastoidectomy including ossicles removal was performed to prevent recurrence. Discussion:Mimicking more common middle ear pathologies such as cholesteatoma, paraganglioma, chronic otitis media and acoustic neuroma, NEAME may be easily misdiagnosed. A tissue biopsy is therefore mandatory to confirm the diagnosis and promptly undergo proper surgical treatment. Although the entity is mostly low grade malignant, postoperative late metastasis as well as death are reported. Therefore, a long term follow-up is required for a better understanding of the biological behavior of the disease as well as to establish standardized treatment protocols and limit the morbidity.

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