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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.
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.
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.
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.
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