Pulsatile tinnitus, which is uncommon in clinical practice, has a complex etiology, but most of the causes can be identified after careful examination. Since in some patients it is associated with a life-threatening pathological condition, it is essential to confirm the cause of the condition. In this article, we review the classification, the identified causes, and the clinical manifestations, investigations, and successful treatment experiences of the less common vascular pulsatile tinnitus, such as dural arteriovenous malformations and arteriovenous fistulas, for the attention of otolaryngologists. The pathophysiology of pulsatile tinnitus and its classification Pulsatile tinnitus is produced by blood vessels or other structures in the cranial cavity, head and neck, or thoracic cavity, and is felt by patients through bone structures, blood vessels, and blood flow to the cochlea. Depending on the cause, these can be classified as vascular or non-vascular. The vascular origin is a disturbance of blood flow due to accelerated blood flow or narrowing of the lumen. Depending on the type of vessel, vascularity is divided into arterial and venous. Venous disorders are not only caused by venous abnormalities, but can also be caused by increased intracranial pressure and transmission of arterial pulsations to the dural venous sinuses, and those originating from other structures than arteries or veins are called nonvascular. It can also be classified as objective, meaning that it can be heard both by the patient and by others, such as the examiner, and subjective, meaning that it can only be heard by the patient. According to statistics, dural arteriovenous malformations account for approximately one third of intracranial vascular malformations. The disease can be congenital or secondary to thrombophlebitis of the dural veins or sinuses due to trauma, infection, surgery, tumor, pregnancy or postpartum, which often involves the transverse sinus, sigmoid sinus, cavernous sinus, anterior skull base and cerebellar curtain. The related vessels are the posterior auricular artery, the occipital artery, the posterior branch of the middle meningeal artery, and the intracranial arteries and veins. Patients are usually around 40 years of age, with equal incidence in both sexes and no familial pattern. Pulsatile tinnitus is the main symptom of the disease, some patients have objective tinnitus, other symptoms include mental changes, headache, loss of vision, diplopia and facial pain. The diagnosis can be made by CT or MRI. A similar lesion to dural arteriovenous malformation is dural arteriovenous phlegm, and both have the same etiology. The main symptom is also pulsatile tinnitus. Other symptoms may include headache, nausea, vomiting, facial pain, hemiparesis, diplopia, loss of vision, and intracranial hemorrhage. Angiography can be used to diagnose this disease. Traumatic internal carotid artery and cavernous sinus sputum commonly occurs days or weeks after head trauma, transsphenoidal pituitary tumor, with protruding eyes, conjunctival edema, III, IV, VI cerebral nerve palsy, and severe throbbing tinnitus. The etiology of Paget’s disease is unknown, but it may be a viral infection. Davies reported pulsatile tinnitus in only 20 of 236 cases of Paget’s disease, which is thought to be secondary to the formation of new vessels and arteriovenous fistulas in the temporal bone.