The red ear syndrome
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《神经病学神经外科学杂志》
1 Service de Neurochirurgie, H?pital la Timone, Marseille, France
2 Centre d’Urgence Céphalée, H?pital Lariboisière, Paris, France
Correspondence to:
Dr A Donnet
Service de Neurochirurgie, H?pital la Timone, 264 bd Saint Pierre, 13385 Marseille Cedex 05, France; adonnet@AP-HM.fr
Keywords: trigeminovascular system; trigeminal-autonomic reflex; upper cervical disorders
The red ear syndrome (RES) was described by Lance,1 who suggested associations with upper cervical disorders and atypical trigeminal and glossopharyngeal neuralgias. Recently, Raieli et al2 underlined the close temporal relationship between RES and migraine.
Patient 1
A 22 year old man, with a 12 year history of migraine without and with aura, experienced acute onset of burning and painful ear without other autonomic symptoms. These symptoms were always homolateral to the hemicrania and persisted for about two hours. The RES could be preceded by a headache. He also described sudden attacks of isolated burning ear without headache or autonomic symptoms. This isolated RES was limited to one side and could occur on either side with no preference for one side or the other. The attacks were not related to any particular stimulus. They occurred three or five times a month; approximately half of the episodes were followed by a migraine attack without aura.
Patient 2
A 92 year old woman experienced, 18 years ago, attacks of burning and red left ear associated with autonomic signs, such as left lacrimation. The attacks lasted for 20 minutes to two hours and could occur every day for 15–45 days every 12–18 months. No precipitating factor was found, and the attacks were resistant to non-steroidal anti-inflammatory drugs (indometacin). Subcutaneous sumatriptan was not given because of the age of the patient.
Neurological examination and brain magnetic resonance imaging (MRI) of both patients were normal.
Discussion
Patient 1 appeared to fit the criteria for RES as described by Raieli et al.2 This type of RES occurs more frequently in children than in adults and is associated with a history of migraine with or without aura and of painful and red ear, unilateral or alternating, in isolation or associated with migraine attacks. This hypothesis was previously suggested by Hirsch3 who reported unilateral and bilateral RES episodes in patients with "vascular headaches". Patient 2 was thought to have trigeminal autonomic cephalalgia (TAC).
Despite common elements, the two patients with RES described here differed in age, associated disorders, as well as the response to therapy.
Two different types of RES can be described: the first type occurs in children or young people and is clearly correlated with migraine.2 These cases can be considered to be idiopathic. The second type occurs in adults and is associated with upper cervical disorders1 or with TAC. RES has been described in association with diverse etiologies: migraine,2 upper cervical disorders and temporomandibular joint dysfunction,1 and TAC, in particular short acting, unilateral headache attacks with conjunctival injection and tearing (SUNCT), and hemicrania continua.4–6 These associations suggest a common pathophysiological mechanism with activation of the trigeminovascular system. This variability occurs despite the belief that the final common pathway (the trigeminal–autonomic reflex) is presumably the same as in cluster headache.7
The trigeminal–autonomic reflex pathway consists of a brainstem connection between the trigeminal nerve and facial parasympathetic outflow.7 RES ear episodes can be mediated by a cervico–autonomic reflex due to either an upper cervical disorder, or directly by trigemino-autonomic stimulation via the auriculotemporal nerve.2 Trigeminovascular activation may produce pain that extends beyond the trigeminal territory. Thus the innervation of the earlobe, which is predominantly from the second and third cervical roots, can explain the association with upper cervical disorders.
References
Lance JW. The red ear syndrome. Neurology 1996;47:617–20.
Raieli V , Monastero R, Santangelo G, et al. Red ear syndrome and migraine: report of eight cases. Headache 2002;42:147–51.
Hirsch AR. Red ear syndrome. Neurology 1997;49:1190.
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.
F?rderreuther S , Straube A. Seltene Kopfschmerz-syndrome. Sunct-Syndrom, Hemicrania continua und das Syndrom von roten Ohr. Nervenartz 1999;70:754–8.
Dodick DW. Extratrigeminal episodic paroxysmal hemicrania. Further clinical evidence of functionally relevant brain stem connections. Headache 1998;38:794–8.
Goadsby PJ, Lipton RB. A review of paroxysmal hemicranias, SUNCT syndrome and other short-lasting headaches with autonomic feature, including new cases. Brain 1997;120:193–209.(A Donnet1 and D Valade2)
2 Centre d’Urgence Céphalée, H?pital Lariboisière, Paris, France
Correspondence to:
Dr A Donnet
Service de Neurochirurgie, H?pital la Timone, 264 bd Saint Pierre, 13385 Marseille Cedex 05, France; adonnet@AP-HM.fr
Keywords: trigeminovascular system; trigeminal-autonomic reflex; upper cervical disorders
The red ear syndrome (RES) was described by Lance,1 who suggested associations with upper cervical disorders and atypical trigeminal and glossopharyngeal neuralgias. Recently, Raieli et al2 underlined the close temporal relationship between RES and migraine.
Patient 1
A 22 year old man, with a 12 year history of migraine without and with aura, experienced acute onset of burning and painful ear without other autonomic symptoms. These symptoms were always homolateral to the hemicrania and persisted for about two hours. The RES could be preceded by a headache. He also described sudden attacks of isolated burning ear without headache or autonomic symptoms. This isolated RES was limited to one side and could occur on either side with no preference for one side or the other. The attacks were not related to any particular stimulus. They occurred three or five times a month; approximately half of the episodes were followed by a migraine attack without aura.
Patient 2
A 92 year old woman experienced, 18 years ago, attacks of burning and red left ear associated with autonomic signs, such as left lacrimation. The attacks lasted for 20 minutes to two hours and could occur every day for 15–45 days every 12–18 months. No precipitating factor was found, and the attacks were resistant to non-steroidal anti-inflammatory drugs (indometacin). Subcutaneous sumatriptan was not given because of the age of the patient.
Neurological examination and brain magnetic resonance imaging (MRI) of both patients were normal.
Discussion
Patient 1 appeared to fit the criteria for RES as described by Raieli et al.2 This type of RES occurs more frequently in children than in adults and is associated with a history of migraine with or without aura and of painful and red ear, unilateral or alternating, in isolation or associated with migraine attacks. This hypothesis was previously suggested by Hirsch3 who reported unilateral and bilateral RES episodes in patients with "vascular headaches". Patient 2 was thought to have trigeminal autonomic cephalalgia (TAC).
Despite common elements, the two patients with RES described here differed in age, associated disorders, as well as the response to therapy.
Two different types of RES can be described: the first type occurs in children or young people and is clearly correlated with migraine.2 These cases can be considered to be idiopathic. The second type occurs in adults and is associated with upper cervical disorders1 or with TAC. RES has been described in association with diverse etiologies: migraine,2 upper cervical disorders and temporomandibular joint dysfunction,1 and TAC, in particular short acting, unilateral headache attacks with conjunctival injection and tearing (SUNCT), and hemicrania continua.4–6 These associations suggest a common pathophysiological mechanism with activation of the trigeminovascular system. This variability occurs despite the belief that the final common pathway (the trigeminal–autonomic reflex) is presumably the same as in cluster headache.7
The trigeminal–autonomic reflex pathway consists of a brainstem connection between the trigeminal nerve and facial parasympathetic outflow.7 RES ear episodes can be mediated by a cervico–autonomic reflex due to either an upper cervical disorder, or directly by trigemino-autonomic stimulation via the auriculotemporal nerve.2 Trigeminovascular activation may produce pain that extends beyond the trigeminal territory. Thus the innervation of the earlobe, which is predominantly from the second and third cervical roots, can explain the association with upper cervical disorders.
References
Lance JW. The red ear syndrome. Neurology 1996;47:617–20.
Raieli V , Monastero R, Santangelo G, et al. Red ear syndrome and migraine: report of eight cases. Headache 2002;42:147–51.
Hirsch AR. Red ear syndrome. Neurology 1997;49:1190.
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.
F?rderreuther S , Straube A. Seltene Kopfschmerz-syndrome. Sunct-Syndrom, Hemicrania continua und das Syndrom von roten Ohr. Nervenartz 1999;70:754–8.
Dodick DW. Extratrigeminal episodic paroxysmal hemicrania. Further clinical evidence of functionally relevant brain stem connections. Headache 1998;38:794–8.
Goadsby PJ, Lipton RB. A review of paroxysmal hemicranias, SUNCT syndrome and other short-lasting headaches with autonomic feature, including new cases. Brain 1997;120:193–209.(A Donnet1 and D Valade2)