Objective:To analyze the etiology of repeatedly attacks of intractable vertigo and some types of sensorineural deafness whose clinical manifestation were not in conformity with the known spectrum diseases,and explore the screening method to prevent missed diagnosis or misdiagnosis, then provide references for clinical diagnosis and treatment for rare etiology. Method:The authors retrospectively analyzed the clinical manifestations, diagnosis, treatment and prognosis from 4 cases of vertigo sufferers and 2 cases of hearing impairment sufferers whose serological tests were positive for syphilis. All these 6 cases were treated with large doses of penicillin aqueous solutions (24 million U/d), multi-times intravenous infusion, the course of the treatment was 14 d. Result:The clinical manifestations of these 6 patients were lack of characteristic, as well as the results of hearing and vestibular function, imaging diagnosis. Positive syphilis detection of serology and cerebrospinal fluid tests were the main diagnostic basis. After anti-syphilis treatment, 5 cases got satisfied clinical symptoms improvement, 1 case suffered from low-tone sensorineural hearing loss, whose hearing fluctuated recurrently. Conclusion:Syphilis infection may damage the Ⅷ cranial nerve and then lead to vertigo and hearing loss, through chronic syphilitic osteitis of temporal bone, atrophy of organ of corti, osteolytic lesion surrounding the endolymphatic duct, and neurosyphilis. For patients presented with intractable vertigo, and those whose clinical manifestations are not in conformity with the known diseases of unilateral ear or bilateral ears rapidly progressive deafness, syphilis serology screening and validation tests are recommended in case of missed diagnosis or misdiagnosis.