Red ear syndrome (RES) is characterized by recurrent unilateral or bilateral painful attacks of the external ear, accompanied by ear redness, burning, or warmth. Proposed etiologies of this rare condition include dysregulation of sympathetic outflow, upper cervical pathology, glossopharyngeal and trigeminal neuralgia, TMJ dysfunction, thalamic syndrome, and primary headache syndromes. Idiopathic cases also exist in the literature. Pediatric cases are particularly rare and more commonly associated with migraine. Given the various potential etiologies, no single treatment is effective in all cases. This paper summarizes the current understanding and management of RES, and describes a case of idiopathic pediatric RES.
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