OBJECTIVE:

We report an extremely rare case of an aberrant internal carotid artery in the middle ear, together with a dehiscent high jugular bulb, a combination never previously reported.

METHODS:

Case report with a review of the literature.

RESULTS:

A 24-year-old man presented with a five-year history of aural fullness, pulsatile tinnitus and mild hearing impairment in his right ear. Otoscopy revealed a retro-tympanic mass. Computed tomography of the temporal bone revealed protrusion of the right internal carotid artery into the middle-ear cavity, with a dehiscent high jugular bulb. Magnetic resonance angiography showed a reduced diameter and lateralisation of the right internal carotid artery. A diagnosis of an aberrant internal carotid artery with a dehiscent high jugular bulb was made, and the patient was managed with conservative treatment.

CONCLUSION:

The otologist should be aware of the possibility of an aberrant internal carotid artery when the patient presents with a retro-tympanic mass, hearing loss and pulsatile tinnitus. Radiological investigation is required to make the differential diagnosis. When an aberrant internal carotid artery presents with a dehiscent high jugular bulb, the risk of serious bleeding is elevated. We recommend a conservative approach for cases presenting without bleeding complications.