Xanthoma of the temporal bone: a unique case of this rare condition.

Xanthoma of the temporal bone is extremely rare; we describe only the fourteenth reported case. Our case is further remarkable because it is the first report of such an occurrence in a patient with familial type III hyperlipoproteinemia. Moreover, while otalgia, infection, hearing loss, and tinnitus were the most common initial symptoms in the previous 13 cases, our patient reported only diplopia, vertigo, and unstable gait. The patient underwent a simple mastoidectomy and debulking, and his diplopia, vertigo, and unstable gait resolved.