Medical Excellence - Issue 2

Various triggers of RES have been identified; however, we found no reports of dietary triggers that provoke RES. Alcohol and spicy foods are known to cause bilateral facial flushing. Gustatory flushing is mediated by an autonomic neural reflex involving the trigeminal nerve. The presence of a dietary trigger that causes neurological symptoms suggests a migrainous etiolo- gy, as such triggers inmigraine are well-known and avoidance is a therapeutic mainstay. The non-concentrate orange juice that our patient consumed is a well-known brand in the United Kingdom. There is a possibility that ethyl butyrate (also known as butyric acid ethyl ester), which is often used in flavoring extracts, could be the culprit rather than the orange juice itself. In our patient, dietary, stressor and lifestyle modifications were sufficient to relieve her physical symptoms and psychological distress. We ad- vocate examining a patient’s lifestyle and encouraging the patient to keep a symptom diary to identify environmental factors that provoke RES. Clinicians should be made aware of the likeli- hood of migraine pathogenesis in primary RES and the range of management options available (non-pharmacological lifestyle changes as well as prophylaxis). Red ear syndrome is a little known condition with much variation in individual patients’ symptoms and variable respons- es to proposed treatments. It may go unrecognized and can cause the patient undue anxiety. Often patients feel ignored without a firm diagnosis or management. Patients may present repeatedly to emergency departments, general practices or various special- ist departments (ear, nose and throat; dermatology; neurology; audiovestibular medicine; and/or audiology) before being diag- nosed. Raised awareness of this disorder with prompt diagnosis has cost benefits by reducing the number of primary and sec- ondary care presentations, decreasing psychological distress and speeding return to usual daily activities. Conclusions Red ear syndrome is a rare syndrome of diverse pathophysiology which is difficult to treat. To our knowledge, our present report is the first to describe a dietary trigger of RES. Successful man- agement with lifestyle modifications and avoidance of migraine triggers gives insight into the pathogenesis of primary migraine- associated RES. Consent Written informed consent was obtained from the patient for the publication of this report and any accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal. Competing interests The authors declare that they have no competing interests. Authors ’ contributions SG reviewed the literature and wrote the initial manuscript drafts. CC managed the patient, reviewed the literature and completed the manuscript. Both authors read and approved the final manuscript. Acknowledgements We thank the reviewers for their valuable comments and suggestions. References 1. Lambru G, Miller S, Matharu MS: The red ear syndrome. J Headache Pain 2013, 14:83. 2. Lance JW: The red ear syndrome. Neurology 1996, 47:617–620. 3. Boes J, Swanson JW, Dodick DW: Chronic paroxysmal hemicrania presenting as otalgia with a sensation of external acoustic meatus obstruction: two cases and a pathophysiologic hypothesis. Head- ache 1998, 38:787–791. 4. Raieli V, Pandolfi E, La Vecchia M, Puma D, Calò A, Celauro A, Ragusa D: The prevalence of allodynia, osmophobia and red ear syndrome in the juvenile headache: preliminary data. J Headache Pain 2005, 6:271–273. 5. Kumar N, Swanson JW: The ‘red ear’ syndrome revisited: two cases and a review of literature. Cephalalgia 2004, 24:305–308. 6. Donnet A, Valade D: The red ear syndrome. J Neurol Neurosurg Psy- chiatry 2004, 75:1077. 7. Brill TJ, Funk B, Thaçi D, Kaufmann R: Red ear syndrome and auricular erythromelalgia: the same condition? Clin Exp Dermatol 2009, 34:e626–e628. 8. Al-Din AS, Mir R, Davey R, Lily O, Ghaus N: Trigeminal cephalgias and facial pain syndromes associated with autonomic dysfunction. Cephalalgia 2005, 25:605–611. 9. Hirsch AR: Red ear syndrome. Neurology 1997, 49:1190. 10. Purdy RA, Dodick DW: Red ear syndrome. Curr Pain Headache Rep 2007,11:313–316. 11. Förderreuther S, Straube A: [A rare headache syndrome: SUNCT syndrome, hemicrania continua and red ear syndrome] [Article in German]. Nervenarzt 1999, 70:754–758. 12. Boulton P, Purdy RA, Bosch EP, Dodick DW: Primary and secondary red ear syndrome: implications for treatment. Cephalalgia 2007, 27:107–110. 13. Selekler M, Kutlu A, Uçar S, Almaç A: Immediate response to greater auricular nerve blockade in red ear syndrome. Cephalalgia 2009, 29:478–479. 14. Bender SD: Primary and secondary red ear syndrome: implications for treatment. Cephalalgia 2007, 27:1286–1287. 15. Ryan S, Wakerley BR, Davies P: Red ear syndrome: a review of all published cases 1996–2010. Cephalalgia 2013, 33:190–201. 16. Dodick DW: Extratrigeminal episodic paroxysmal hemicrania: further clinical evidence of functionally relevant brain stem connections. Headache 1998, 38:794–798. Source: Chan, C.C. & Ghosh, S. J Med Case Reports (2014) 8: 338. https://doi.org/10.1186/1752-1947-8-338. © Chan and Ghosh; licensee BioMed Central Ltd. 2014. ORT Open Access syndrome precipitated by a dietary a case report 1,2* and Susmita Ghosh 3 ed ear syndrome is a rare condition characterized by episodic attacks of erythema of e ear y burning ear pain. Symptoms are brought on by touch, exertion, heat or cold, stress, neck washing or brushing of hair. Diagnosis and treatment of this condition are challenging. The case involves a woman whose symptoms were brought on by a dietary trigger: orange juice as well as ignificant physical and psychological morbidity. Avoidance of triggers resulted in sy ptomatic ion: A 22-year-old Caucasian woman who was a student presented twice to our departme t with oms, the first time with hyperacusis (abnormal sound sensitivity arising from within the auditory ds of moderate volume), intermittent right tinnitus and subjective hearing difficulties. She presented ith highly distressing episod s of eryth matous ears, which were associated with burning pain and temporal are s, and i olera ce to noise. After keeping a symptom diary, she identified orange as triggers of er symptoms. No local head and neck pathology was present. Investigations and egative. Avoidance of triggers led to great symptomatic improvement. To the best of our knowledge, have not previously been reported as a trigger for this syndrome. This case shows a direct temporal link er and supports a primary pathogenesis. Recognition and management of primary headache disorder ry and lifestyle changes brought about symptomatic relief. d ear syndrome is a little-known clinical syndrome of unknown etiology and management. To the best e, our present case report is the first to describe primary red ear syndrome triggered by orange juice. erived from avoidance of this trigger, which is already known to precipitate migraines, gives some pathogenesis of red ear syndrome. ary trigger, Erythema, Lifestyle modifications, Migraine, Red ear syndrome e (RES) is a rare condition characterized ema of the ear accompanied by burning lgia. One or, less commonly, both ears nd erythema may extend beyond the ear ptoms may be spontaneous or triggered n, heat or cold, stress, neck movements, g, chewing an /or brushing of hai [1]. Recognition of this condition is important but difficult because of its rarity. Case presentation A 22-year-old Caucasian woman who was a student pre- sented to our neuro-otology clinic on two separate occa- sions five years apart. Her initial symptoms were a six- onth history of intermittent right-sided tinnitus and bilateral hype acu is (abn mal sound s nsitivity arising from within the auditory system to n rmal or moderate- level ambient noise which would not trouble other people). She also reported right ear fullness and signifi- cant difficulty hearing in background noise when stressed. Otoscopy, a neuro-otological examin tion, pure-tone audiometry, tympanometry, stapedial reflexes, oto-acoustic ng.chan@nhs.net estibular Medicine, St Ann ’ s Hospital, St Ann ’ s Road, Audiovestibular Medicine, Royal National Throat, Nose rays Inn Road, London WC1X 8DA, UK ation is available at the end of the article © 2014 Chan and Ghosh; licensee BioMed Central Ltd. This is an Open Access article distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/4.0 ), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly credited. The Creative Commons Public Domain Dedication waiver (http://creativecommons.org/publicdomain/zero/1.0/ ) applies to the data made available in this article, unless otherwise stated. 4  |  Issue-2 MEDICAL EXCELLENCE