Red Ear Syndrome

  • V. RaieliEmail author
  • A. Compagno
  • M. D’Amelio
Uncommon and/or Unusual Headaches and Syndromes (J Ailani, Section Editor)
Part of the following topical collections:
  1. Topical Collection on Uncommon and/or Unusual Headaches and Syndromes


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.


Red ear Red ear syndrome Primary headaches Migraine Trigeminal autonomic cephalgias Trigeminal autonomic reflex Erythromelalgia Parasympathetic system Secondary red ear syndrome Idiopathic red ear syndrome Cranial autonomic symptoms Trigemino-vascular system 


Compliance with Ethical Standards

Conflict of Interest

V. Raieli, A. Compagno, and M. D’Amelio declare that they have no conflict of interest.

Human and Animal Rights and Informed Consent

This article does not contain any studies with human or animal subjects performed by any of the authors.


Papers of particular interest, published recently, have been highlighted as: • Of importance •• Of major importance

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Copyright information

© Springer Science+Business Media New York 2016

Authors and Affiliations

  1. 1.U.O.NPI, P.O. “G. Di Cristina” ARNAS CIVICOPalermoItaly
  2. 2.U.O.S. Servizio per l’Età Evolutiva - U.O.C. Infanzia Adolescenza Famiglia, Azienda Socio-Sanitaria ULSS7Pieve di SoligoItaly
  3. 3.Dipartimento di Biomedicina Sperimentale e Neuroscienze Cliniche (BioNeC)Università degli Studi di PalermoPalermoItaly

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