Red Ear Syndrome

Abstract

The Red Ear syndrome (RES) is an intriguing syndrome originally described for the first time nearly 20 years ago. RES is characterized by unilateral/bilateral episodes of pain and burning sensation of the ear, associated with ipsilateral erythema. RES episodes are indeed isolated in some patients, but they can occur in association with primary headaches, including in particular migraine in the developmental age. Although the underlying pathophysiological mechanisms are still uncertain, in the recent years the described comorbidities have aroused increasing interest because of possible clinical implications. Moreover, RES seems to be more often associated with clinical features of migraine partially provoked by the involvement of the parasympathetic system. This clinical association has shed new light on the pathophysiology of RES, supporting the hypothesis of a shared pathophysiological background, for example, through the activation of the trigeminal autonomic reflex. Current therapies of RES will be also discussed. Finally, we will resume the more controversial aspects of this relatively new and probably underestimated neurological syndrome.

Pediatric red ear syndrome: A case report of an erythromelalgia type and review of the literature

“…Red ear syndrome is a disorder primarily reported in the neurologic literature due to its association with primary headache syndromes, including migraine . However, patients with this condition can present to a dermatologist, given the prominent cutaneous findings . The condition classically presents as unilateral or bilateral erythema of the ear, with additional findings of burning, pain, or increased temperature.…”

“…Idiopathic (primary) RES is typically seen in children or young adults, whereas secondary RES is more common in adults . Secondary causes also include upper cervical spine pathology and TMJ dysfunction …”

“…Red ear syndrome has been proposed to be within the spectrum of trigeminal autonomic cephalgias given its association with primary headache syndromes . It has been suggested that the erythema is mediated by the trigeminal autonomic reflex, resulting in extracranial vasodilation …”

The Mystery of “Red Ear Syndrome”: Sign or Syndrome

“…The term “red ear syndrome (RES),” first coined by Lance, defines a clinical disorder characterized by painful uni‐bilateral attacks of the region of the ear associated with auricular redness and burning sensations. RES can be idiopathic, related to primary headaches (migraine or trigeminal autonomic cephalalgias [TACs]), or secondary to several etiologic disorders . Lance used the term “Mystery” to describe this curious syndrome.…”

“…RES is actually considered a rare condition, and this opinion perhaps represents the first “mystery” as more than 100 cases together with several small clinical series have been published (eg, the primary trochlear headache, mentioned in the International Headache classification, has fewer articles) . Moreover, we reported RES in about 30% of clinical pediatric migraine cases in 2 different populations …”