Embryonically derived variant of the internal carotid artery. May have enlarged collaterals in the middle ear and be seen as a retrotympanic vascular mass. Characterized by choking or globus sensation, otalgia, hearing loss, aural fullness, vertigo, pulsatile tinnitus, and mass behind the tympanic membrane. May be associated with obstructive sleep apnea. May be discovered inadvertently upon imaging or surgery for another disorder. Complications may be fatal due to hemorrhage, particularly if surgeon is unaware of the aberrant carotid in the vicinity of the temporal bone or tonsils.

Conservative management by observation is called for, generally avoiding surgery due to the high risk for bleeding or death. For severe and troublesome symptoms (hearing loss, pulsatile tinnitus, chronic middle ear infection), surgical treatment may be indicated with careful balancing of the risks. The aberrant artery may be covered with silicone or fascia and bone grafts to reposition and protect it from unintended damage during ear surgery.