The syndrome is classically caused by the spread of an infection into the petrous apex of the temporal bone.

The constellation of symptoms was first described as a consequence of severe, advanced ear infection which has spread to a central portion of the temporal bone of the skull.

[citation needed] In persons with longstanding ear infection and typical symptoms, medical imaging such as CT or MRI of the head may show changes that confirm disease involvement of the petrous apex of temporal bone.

[citation needed] The medical treatment is done with antibiotics: ceftriaxone plus metronidazole (which covers anaerobic bacteria).

Due to critical structures that block surgical access, it is not possible to completely remove the petrous apex.