[Neuroendocrine adenoma of the middle ear]

Neuroendocrine adenomas of the middle ear are rare, low-grade neoplasms with potential for recurrence and metastasis. The nonspecific symptoms and preliminary clinical and radiological findings are misleading and often fail to provide the right diagnosis.

We analyzed the findings of 3 adult patients, who were treated between 2001 and June /2012 at the Luzerner Kantonsspital in Switzerland.

The 3 patients reported on hearing loss, ear pressure and/or tinnitus. Otoscopy showed a thickened, intact tympanic membrane with a whitish-grayish prolapsing mass. All the patients had conductive hearing loss. Computer tomography showed an unspecified well-circumscribed soft-tissue mass. Definitive histology with immunostaining after radical tumor removal led to the proper diagnosis.

The typical constellation of nonspecific clinical and radiological findings leads to the right diagnosis of neuroendocrine adenoma of the middle ear. After reviewing the literature, we illustrate the differential diagnosis as well as the relevant diagnostic and therapeutic procedures, and remind ENT physicians about this rare disease entity.