Syphilis is a multisystemic disease that may also affect the inner ear. The objective of this study was to investigate the frequency, clinical presentation and outcome of otosyphilis. A retrospective analysis of inpatients seen by a team of ENT specialists and dermatologists at a teaching hospital in Dresden, Germany, during 1986–2000. Six cases of otosyphilis had been diagnosed. None was related to risk factors such as HIV infection or drug abuse. We identified four female and two male patients, age range 25–58 years. Four patients suffered from syphilis stage II, one from syphilis stage and another from congential syphilis. ENT complaints included loss or impairment of hearing, tinnitus and nystagmus. All were treated with penicillin G. Adjuvant therapy included systemic corticosteroids oral pentoxifillin in two patients each. Improvement of ENT symptoms was achieved in all but one patient, who suffered from congential syphilis. This study confirms that otosyphilis is still a complication of syphilis in developed countries. The first line therapy is systemic penicillin G. There is a lack of controlled trials for adjuvant treatments such as corticosteroids and pentoxifillin. Clinical cooperation between dermatologists/venereologists and ENT specialists supports the best outcome.