Abstract
Background/Objectives: An aberrant internal carotid artery (aICA) in the middle ear is a rare vascular anomaly with potentially catastrophic consequences if injured during otologic procedures. Given its rarity, standardized treatment recommendations are lacking. This study aims to present four cases of aICA bleeding, systematically review the literature, and evaluate the outcomes of conservative and interventional management. Methods: A retrospective review of four patients treated for intraoperative aICA hemorrhage at two tertiary referral centers was performed. A systematic review was conducted following PRISMA guidelines. Neurologic and otologic outcomes, hemostasis, and complications were analyzed. Results: Two patients were treated conservatively with external auditory canal packing, while two required endovascular coil embolization due to pseudoaneurysm formation or persistent bleeding. One patient suffered a stroke due to traumatic ICA occlusion. The systematic review identified 20 additional cases. Conservative treatment alone sufficed in 37.5% of cases, whereas 62.5% required vessel occlusion via coiling, balloon occlusion, or stenting. Neurologic complications occurred in 25% of patients, while otologic outcomes varied widely and were inconsistently reported. Conclusions: Initial external auditory canal packing and a CT angiogram should be recommended for all patients. Initial conservative management may be appropriate for cases with early hemostasis if close monitoring is ensured. Endovascular treatment is often necessary, particularly in cases of pseudoaneurysm or rebleeding.