Arteriovenous fistulae of the internal maxillary artery are exceedingly rare, with less than 30 cases reported in the literature. Most of these lesions are congenital, iatrogenic, or posttraumatic. The most common presentation of internal maxillary artery fistulae is pulsatile tinnitus and headache. Because these lesions are single-hole fistulae, they can be easily cured with endovascular techniques. The authors present a case of a patient who presented to their institution with a several-year history of pulsatile tinnitus who was found to have an internal maxillary artery arteriovenous fistula, which was treated endovascularly with transarterial coil and Onyx embolization.
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