Objective To investigate the clinical features, diagnosis and treatment methods and prognosis of neurogenic tumors in the middle ear..Method The clinical data of 7 patients who were pathologically diagnosed with neurogenic tumors of the middle ear in our hospital during 2010-2022 were retrospectively analyzed.Result Among the 7 patients, 3 complained of hearing loss, 3 of them had purulent ear flow, and 1 of them had peripheral facial paralysis (H-B grade VI). The tumor invaded the external auditory canal and manifested as external auditory canal mass in 4 cases and tympanic tensor perforation in 3 cases. CT tumors in the middle ear involved the horizontal segment of the facial nerve in 2 cases, invaded the internal segment of the facial nerve and invaded the middle cranial fossa in 1 case, and the other lesions were located on the surface of the middle tympanum and promontory in 4 cases. All the 7 patients underwent tympanoplasty on the basis of complete removal of the lesions. The postoperative pathological diagnosis was as follows: 3 cases of tympanosoma, 2 cases of facial neurofibroma, 1 case of facial schwannoma, 1 case of middle ear neuroendocrine adenoma; Cholesteatoma of the middle ear and chronic suppurative otitis media were diagnosed by preoperative biopsies of facial neurememoma and neuroendocrine adenoma of the middle ear, respectively. The preoperative pathological misdiagnosis rate was 28.6%. The patients were followed up for 1-8 years after surgery, and no cases of recurrence were reported..Conclusion The incidence of neurogenic tumors in the middle ear is relatively low with different clinical manifestations, and there is a certain misdiagnosis rate. Preoperative CT and enhanced MRI examination, as well as postoperative pathology and immunohistochemistry are helpful for definite diagnosis. Operation is an effective means to treat the disease.