Red Ear: Syndrome or Symptom?

Red ear syndrome (RES) is a rare disorder characterized by attacks of unilateral ear pain during which the ear becomes red. Episodes can occur spontaneously, or be triggered, in most cases, by rubbing or touching the ear. Both duration and frequency are variable. RES has been explained by a dysfunction of cervical spinal nerves (C3 root) and a dysregulation with disinhibition of brainstem trigemino-autonomic circuits, leading to sympathetic inhibition and parasympathetic hyperactivity producing vasodilation. We describe 6 new cases of RES with different characteristics. Although all presented the cardinal symptom of red ear, the headache patterns were suggestive of other primary headaches (migraine or cluster headache). Therapeutic response was obtained when directed to the associated primary headache phenotype, suggesting that RES may be a phenomena associated with different headaches, rather than a syndrome in itself.