Tinnitus synchronous with the pulse is usually caused by vascular anomalies. In our own patient group we found the most frequent cause to be highly vascularised tumours related to the pretrous bone, the most common being glomus tumours. Pulsatile tinnitus however also is a main symptom of dural arterio-venous malformations with shunts into the transverse and sigmoid sinus. Finally pulsatile tinnitus may be caused by venous deformities, possibly combined with anomalies of the bulb of the jugular vein. While clinical methods give valuable clues to the type of pathological findings, subtle radiological investigations are necessary for the final diagnosis, such as high resolution computer tomography and super-selective angiography. There has been substantial progress in recent years due to technical developments leading to improved diagnosis and treatment by interventional radiology.