Clinical experience with the diagnosis and treatment of middle ear adenomatous neuroendocrine tumors.

Middle ear adenoma, also known as middle ear adenomatous neuroendocrine tumors (MEANTs), is a rare neoplasm, accounting for < 2% of all middle ear tumors. It is characterized by slow growth, non-invasive behavior, and nonspecific symptoms, which often complicate preoperative diagnosis. This review summarizes our clinical experience in the diagnosis and treatment of 11 cases of MEANT. In this cohort, four cases were preoperatively diagnosed as cholesterol granuloma, five as cholesteatoma, and two as middle ear tumors. The median age at diagnosis was 36.7 years, with all patients (100%) experienced progressive conductive or mixed hearing loss. Additional symptoms included tonal tinnitus (9/11, 81.8%), aural fullness (6/11, 54.5%), otalgia (4/11, 36.4%), pulsatile tinnitus (2/11, 18.2%), otorrhea (2/11, 18.2%), and facial nerve paresis (1/11, 9%). None of the patients exhibited carcinoid syndrome or bloody otorrhea. All patients underwent surgical treatment, and postoperative histopathology confirmed the diagnosis of MEANTs. Immunohistochemical analysis showed positivity for CK and SYN in all cases, S-100 was positive in 91% (10/11) of patients. All patients were followed up for 1.5 to 10 years postoperatively. These findings highlight the nonspecific presentation of middle ear adenoma, which may lead to misdiagnosis as chronic inflammatory disease or other middle ear neoplasms. For differential diagnosis of MEANTs, high-resolution computed tomography (HRCT) of the ear or MRI with non-echo-planar diffusion-weighted imaging (non-EPI DWI) are valuable. Complete surgical resection remains the mainstay of treatment, and ossicular chain reconstruction should be considered when appropriate.