Skip to main content

Advertisement

Log in

Red Ear Syndrome

  • Uncommon and/or Unusual Headaches and Syndromes (J Ailani, Section Editor)
  • Published:
Current Pain and Headache Reports Aims and scope Submit manuscript

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.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Similar content being viewed by others

References

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

  1. Lance JW. The mystery of one red ear. Clin Exp Neurol. 1995;31:13–8.

    Google Scholar 

  2. Lance JW. The red ear syndrome. Neurology. 1996;47:617–20.

    Article  CAS  PubMed  Google Scholar 

  3. Hirsch AR. Red ear syndrome [comment]. Neurology. 1997;49:1190.

    Article  CAS  PubMed  Google Scholar 

  4. Dodick DW. Extratrigeminal episodic paroxysmal hemicrania. Further clinical evidence of functionally relevant brain stem connections. Headache. 1998;38:794–8.

    Article  CAS  PubMed  Google Scholar 

  5. Boes CJ, Swanson JW, Dodick DW. Chronic paroxysmal hemicrania presenting as otalgia with a sensation of external acoustic meatus obstruction: two cases and a pathophysiologic hypothesis. Headache. 1998;38:787–91.

    Article  CAS  PubMed  Google Scholar 

  6. Forderreuther S, Straube A. A rare headache syndrome. SUNCT syndrome, hemicrania continua and red ear syndrome. Nervenarzt. 1999;70:754–8.

    Article  CAS  PubMed  Google Scholar 

  7. Arjona A, Fernández-Romero E. Red ear syndrome: a new case. Rev Clin Esp. 2000;200:704–5.

    Article  CAS  PubMed  Google Scholar 

  8. Lee S, Bae SC, Uhm WS, Jun JB, Lee IH, Kim SY. Red ear(s) syndrome associated with child neuropsychiatric systemic lupus erythematosus. Lupus. 2000;9:301–3.

    Article  CAS  PubMed  Google Scholar 

  9. Raieli V, Monastero R, Santangelo G, et al. Red ear syndrome and migraine: report of eight cases. Headache. 2002;42:147–51.

    Article  PubMed  Google Scholar 

  10. Donnet A, Valade D. The red ear syndrome. J Neurol Neurosurg Psychiatry. 2004;75:1076–9.

    Article  Google Scholar 

  11. Evans RW, Lance JW. The red ear syndrome: an auriculo-autonomic cephalgia. Headache. 2004;44:835–6.

    Article  PubMed  Google Scholar 

  12. Kumar N, Swanson JW. The 'red ear syndrome' revisited: two cases and a review of literature. Cephalalgia. 2004;24:305–8.

    Article  CAS  PubMed  Google Scholar 

  13. Ramirez CC, Kirsner RS. A refractory case of erythromelalgia involving the ears. Am J Otolaryngol. 2004;25:251–4.

    Article  PubMed  Google Scholar 

  14. Al-Din AS, Mir R, Davey R, et al. Trigeminal cephalgias and facial pain syndromes associated with autonomic dysfunction. Cephalalgia. 2005;25:605–11.

    Article  CAS  PubMed  Google Scholar 

  15. Arjona A, Serrano-Castro PJ, Fernández-Romero E, Rubí J. The red ear syndrome: five new cases. Cephalalgia. 2005;25:479–80.

    Article  CAS  PubMed  Google Scholar 

  16. Boulton P, Purdy RA, Bosch EP, Dodick DW. Primary and secondary red ear syndrome: implications for treatment. Cephalalgia. 2007;27:107–10.

    Article  CAS  PubMed  Google Scholar 

  17. Selekler M, Kutlu A, Uçar S, et al. Immediate response to greater auricular nerve blockade in red ear syndrome. Cephalalgia. 2008;29:478–9.

    Article  Google Scholar 

  18. Brill TJ, Funk B, Thaçi D, Kaufmann R. Red ear syndrome and auricular erythromelalgia: the same condition? Clin Exp Dermatol. 2009;34:626–8.

    Article  Google Scholar 

  19. Staatsen CC, Koehler PJ. The red ear syndrome. In: Couturier EGM, Sorbi MJ, van Brockhurst J, editors. Headache and migraine 10. Anglo-Dutch Migraine Association; 2008. p. 225–231.

  20. Grimaldi D, Sónia B, Alice P, Hanne-Laure V, Fernando V-S. The Almirall European Headache Awards 2009. J Headache Pain. 2010;11:207–14.

    Article  PubMed Central  PubMed  Google Scholar 

  21. Stępień A, Brodacki B, Staszewski J. Red ear syndrome—a case report. Neurol Neurochir Pol. 2010;44:412–4.

    PubMed  Google Scholar 

  22. Sklebar D, Sklebar I, Vrabec-Matković D, Cengić L. Red ear syndrome—chronic pain syndrome of low prevalence. Acta Clin Croat. 2010;49:327–9.

    PubMed  Google Scholar 

  23. Créac'h C, Duthel R, Barral F, Nuti C, Navez M, Demarquay G, et al. Positional cluster-like headache. A case report of a neurovascular compression between the third cervical root and the vertebral artery. Cephalalgia. 2010;30:1509–13.

    Article  PubMed  Google Scholar 

  24. Raieli V, Compagno A, Brighina F, La Franca G, Puma D, Ragusa D, et al. Prevalence of red ear syndrome in juvenile primary headaches. Cephalalgia. 2011;31:597–602. This paper shows a large sample of juvenile migraineurs with RES and relates it to migraine clinical features, supporting autonomic pathogenesis.

    Article  CAS  PubMed  Google Scholar 

  25. Eismann R, Gaul C, Wohlrab J, Marsch WC, Fiedler E. Red ear syndrome: case report and review of the literature. Dermatology. 2011;223:196–9.

    Article  PubMed  Google Scholar 

  26. Maranhão-Filho PA, Vincent MB. The mystery of two red ears: a bilateral simultaneous case. Cephalalgia. 2011;31:1587.

    Article  PubMed  Google Scholar 

  27. Grandy K, Corsten G, Hong P. Pediatric-isolated auricular erythromelalgia: a case report. Case Rep Pediatr. 2012. doi:10.1155/2012/854081.

    PubMed Central  PubMed  Google Scholar 

  28. Lambru G, Bakar NA, Matharu M. SUNA and red ear syndrome: a new association and pathophysiological considerations. J Headache Pain. 2013;14:32.

    Article  PubMed Central  PubMed  Google Scholar 

  29. Picco PP, D’Alessandro M, Leoni M, Doglio M, Martini A. Primary red ear syndrome associated with 8ochlea-vestibular symptomatology: a paediatric case report. Cephalalgia. 2013;33:1277–80.

    Article  PubMed  Google Scholar 

  30. Ryan S, Wakerley BR, Davies P. Red ear syndrome: a review of all published cases (1996–2010). Cephalalgia. 2013;33:190–201.

    Article  PubMed  Google Scholar 

  31. Chen MC, Xu QF, Luo DQ, Li X, He DY. Erythema associated with pain and warmth on face and ears: a variant of erythermalgia or red ear syndrome? J Headache Pain. 2014;15:18.

    Article  PubMed Central  PubMed  Google Scholar 

  32. Lai TH, Cheng YC. Red ear syndrome. Acta Neurol Taiwan. 2014;23:78–9.

    PubMed  Google Scholar 

  33. Chan CC, Ghosh S. Red ear syndrome precipitated by a dietary trigger: a case report. J Med Case Rep. 2014;8:338.

    Article  PubMed Central  PubMed  Google Scholar 

  34. Flicinski J, Wigowska-Sowinska J, Winczewska-Wiktor A, Steinborn B. Red ear syndrome—case report and review of literature. Neurol Neurochir Pol. 2015;49:74–7.

    PubMed  Google Scholar 

  35. Moitri MO, Banglawala SM, Archibald J. Red ear syndrome: literature review and a pediatric case report. Int J Pediatr Otorhinolaryngol. 2015;79:281–5.

    Article  PubMed  Google Scholar 

  36. Barragan-Martinez D, Urbanos-Nunez A, Martinez-Salio A. Red ear syndrome: a case report with good therapeutic response. Rev Neurol. 2015;61:239–40.

    CAS  PubMed  Google Scholar 

  37. Velasco EM, Luis Lopez Mesonero L, Pedraza Hueso MI, Ruiz Pi ñero M, de Lera Alfonso M, Guerrero Peral AL. Red ear and more: facial and extrafacial erythema accompanying migraine attacks. Headache. 2016;56:174–75.

    Article  PubMed  Google Scholar 

  38. Purdy RA, Dodick DW. Red ear syndrome. Curr Pain Headache Rep. 2007;11:313–6.

    Article  PubMed  Google Scholar 

  39. Raieli V, Giordano G, Spitaleri C, Consolo F, Buffa D, Santangelo G, et al. Migraine and cranial autonomic symptoms in children and adolescents: a clinical study. J Child Neurol. 2015;30:182–6.

    Article  CAS  PubMed  Google Scholar 

  40. Bender SD. Primary and secondary red ear syndrome: implications for treatment. Cephalalgia. 2007;27:1286–7.

    Article  PubMed  Google Scholar 

  41. Gelfand AA, Reider AC, Goadsby PJ. Cranial autonomic symptoms in pediatric migraine are the rule, not the exception. Neurology. 2013;81:431–6.

    Article  PubMed Central  PubMed  Google Scholar 

  42. Lai TH, Fuh JL, Wang SJ. Cranial autonomic symptoms in migraine:characteristics and comparison with cluster headache. J Neurol Neurosurg Psychiatry. 2009;80:1116–9.

    Article  PubMed  Google Scholar 

  43. International Classification of Headache Disorders. 3rd edition (beta version). Cephalalgia. 2013;33:629–808.

    Article  Google Scholar 

  44. Lambru G, Miller S, Matharu MS. The red ear syndrome. J Headache Pain. 2013;14:83. A rewiew with an excellent discussion of some important aspects of RES, suggesting diagnostic criteria to include in the IHS classification.

    Article  PubMed Central  PubMed  Google Scholar 

  45. May A, Goadsby PJ. The trigeminovascular system in humans: pathophysiologic implications for primary headache syndromes of the neural influences on the cerebral circulation. J Cereb Blood Flow Metab. 1999;19:115–27.

    Article  CAS  PubMed  Google Scholar 

  46. Moskowitz MA, Henrikson BM, Beyerl BD. Trigeminovascular connections and mechanisms of vascular headache. In: Clifford Rose F, editor. Handbook of Clinical Neurology, Vol.4: Headache. Amsterdam: Elsevier Science Publishers; 1986. p. 107–15.

    Google Scholar 

  47. Goadsby PJ. The autonomic nervous systemic control of the cerebral circulation. In: Buijs RM, Swaab DF, editors. Handbook of Clinical Neurology, Vol. 117 (3rd series) Autonomic Nervous System. Elsevier B.V.; 2013. p. 193–202

  48. Kelman L. Osmophobia and taste abnormality in migraineurs: a tertiary care study. Headache. 2004;10:1019–23.

    Article  Google Scholar 

  49. Burstein R, Yarnitsky D, Goor-Aygh I, et al. An association between migraine and cutaneous allodynia. Ann Neurol. 2000;47:614–24.

    Article  CAS  PubMed  Google Scholar 

Download references

Author information

Authors and Affiliations

Authors

Corresponding author

Correspondence to V. Raieli.

Ethics declarations

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.

Additional information

This article is part of the Topical Collection on Uncommon and/or Unusual Headaches and Syndromes

V. Raieli, A. Compagno and M. D’Amelio contributed equally to this work.

Rights and permissions

Reprints and permissions

About this article

Check for updates. Verify currency and authenticity via CrossMark

Cite this article

Raieli, V., Compagno, A. & D’Amelio, M. Red Ear Syndrome. Curr Pain Headache Rep 20, 19 (2016). https://doi.org/10.1007/s11916-016-0547-y

Download citation

  • Published:

  • DOI: https://doi.org/10.1007/s11916-016-0547-y

Keywords

Navigation