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Abstract

Background

Red ear syndrome is characterised by episodic unilateral erythema of the ear associated with pain and burning sensation.

Patients and methods

We describe the case of a young woman with daily, frequent, recurrent episodes of red ear syndrome in the absence of any structural lesions, primary headache disorder or obvious triggers. We review all previously described cases and discuss postulated mechanisms for this syndrome.

Conclusions

The exact cause of this rare headache disorder remains unknown and treatment options vary considerably.

Keywords

Headache red ear syndrome erythromelalgia trigeminal-autonomic reflex

Introduction

Red ear syndrome (RES) is characterised by unilateral erythema of the ear with associated pain and burning sensation. This phenomenon presents more commonly in association with primary headache disorders or conditions of the temporo-mandibular joint (TMJ) or cervical spine, but is rare as a primary complaint. Since it was first described by Lance in 1996 (1), approximately 60 cases with varying presentations have been reported in the literature, accompanied by much speculation on possible aetiological causes (Table 1).

Table 1.

Previously described cases of red ear syndrome (1996–2010).

Author(s)	Case	Age/sex	Ear(s) affected	Onset/frequency/duration	Headache history?	Precipitant(s)	Treatment(s)	Idiopathic (I)/ secondary (S)/mixed (M)	Secondary cause
Lance (1996) (1)	1	83 M	R	16 years	No	Touch	Methysergide	S	Cervical arachnoiditis
	2	73 F	L	8 months; 2–3 per week	No	Coughing; sneezing; neck; neck movement	Indomethacin	S	Cervical spondylosis
	3	62 M	L	15 years	Yes			S	Cervical spondylosis
	4	37 M	L	15 months	No	Physical activity; heat	Cooling; propranolol;C2–C3 root block	S	Cervical spondylosis
	5	33 F	R	3 years; seven episodes lasting 15 mins	No	Neck movement; stress		S	Possible traction injury of upper cervical roots?
	6	50 F	L	18 years	Yes, migraine	Touch	Flunarizine; C3 root block; C3 section	S	Atypical glossopharyngeal and C3 neuralgia
	7	35 M	L		No	Jaw movement; cold	Surgical section of L glosso-pharyngeal n.	S	Atypical glossopharyngeal and C3 neuralgia
	8	12 M	LR	5 years; daily, lasting up to 60 mins	No	Jaw movement	Dental plate	S	TMJ dysfunction
	9	42 F	L	3 years; up to 2 per week, lasting 30–60 mins	Yes, migraine	Menstrual period; emotional upset	Amitriptyline	S	Atypical trigeminal neuralgia; possible TMJ dysfunction
	10	42 F	L	5 years	Yes, migraine		Imipramine	S	Thalamic syndrome
	11	44 F	R	Since childhood, worse in last 4 years; 2-4 per day, lasting hours	Yes	Heat	Cooling	I
	12	14 F	R	7 years; 1 per week lasting 10–30 mins	Yes	Touch; heat; jaw movement		I
Dodick (1998) (13)	1	59 F	L	7 years; occurring 3–5 times per day for 3 days, lasting 15–20 mins, followed by 2 weeks remission	Yes		Indomethacin	S	Extratrigeminal EPH
Boes et al. (1998) (18)	1	42 F	L	10 years; 5 per day, lasting 2–60 mins	Yes		Indomethacin	S	CPH
	2	49 F	R	3 years; 4–15 per day, lasting 3–10 mins	Yes		Indomethacin	S	CPH
Lee et al. (2000) (19)	1	9 M	LR	1–2 per day, lasting 60 mins	Yes			S	SLE: thalamic lesion?
Raieli et al. (2002) (14)	1	10 M	LR	5 per month, lasting 30–60 mins	Yes, migraine		Nimodipine	I
	2	8 M	LR	40 days (post-head injury); 3–4 per month, lasting 30 mins	Yes, migraine			M	Cervical irritation after injury?
	3	9 M	L	6 per month, lasting 60 mins	Yes, migraine			I
	4	12 F	L	1 year; 2–3 per month, lasting 30-45 mins	Yes, migraine			I
	5	5 M	LR	6 months; 2–4 per month, lasting <30 mins	Yes, migraine			I
	6	13 M	R	1 year; 4 per month, lasting 30 mins	Yes, migraine	Stress		I
	7	11 M	R	8 months; 3–5 per month, lasting <30 mins	Yes, migraine		Flunarizine	I
	8	36 F	L	A few years; 1–2 per month, lasting 30-180 mins	Yes, migraine			I
Donnet and Valade (2004) (2)	1	22 M	LR	12 years; 3–5 per month, lasting 2 hours	Yes, migraine			I
	2	92 F	L	18 years; daily for 15–45 days every 12–18 months, lasting 20–120 mins	No			S	TAC
Kumar and Swanson (2004) (16)	1	66 M	L	4 weeks; every 2–3 hours, lasting 20–30 secs	No	Touch		I
	2	66 M	R	4 years	No	Head movement		I
Ramirez and Kirsner (2004) (10)	1	53 M	LR	3 years; initially several episodes per month, gradually became constant	No	Heat; physical activity	Cooling	S	Primary erythromelalgia
Evans and Lance (2004) (15)	1	54 F	L	10 years; 5 per month every 2 years, lasting 30–120 mins	Yes, migraine			I
Arjona et al. (2005) (20)	1	70 F	L	4 weeks; continuous	No			S	Herpes zoster
	2	23 F	R	1 month; 20 per day, lasting secs–mins	Yes			I
	3	41 M	L	2 episodes lasting hours	Yes	Physical activity (inc. sexual intercourse)		S	Chiari I malformation
	4	26 F	R	Countless episodes, lasting mins	Yes			I
	5	48 F	L	4 months; daily, lasting hours	No			S	TMJ dysfunction
Al-Din et al. (2005) (3)	1	42 F	Unilateral	2–3 per week, lasting 30–90 mins	No		Gabapentin	I
	2	58 F	Unilateral	<1 per month, lasting >10 mins	No	Lying on affected side	Gabapentin	S	TMJ dysfunction
	3	51 F	Unilateral	>1 per day, lasting >30 mins	Yes, migraine		Gabapentin	I
	4	39 F	Unilateral	1–2 per week, lasting 30–60 mins	No	Lying on affected side	Gabapentin	S	TMJ dysfunction
	5	49 F	Unilateral	2–4 per day, lasting 15–30 mins	Yes, migraine		Gabapentin	I
	6	45 F	Unilateral	2–3 per day, lasting 5–10 mins	Yes, migraine	Lying on affected side	Gabapentin	S	TMJ dysfunction
	7	37 F	Unilateral	1–2 per day, lasting 60–90 mins	No	Lying on affected side	Gabapentin	S	Pleomorphic adenoma of carotid body; TMJ dysfunction
	8	76 F	Unilateral	Daily, lasting 60–90 mins	No	Lying on affected side	Gabapentin	S	TMJ dysfunction
	9	54 F	R	1–2 per day, lasting >30 mins	Yes, migraine		Gabapentin	I
	10	57 F	Unilateral	>1 per day, lasting >20 mins	No	Cold	Indomethacin	S	TMJ dysfunction
	11	29 F	L	Daily, lasting >4 hours	Yes, migraine		Gabapentin	I
	12	60 M	Unilateral	2–3 per day, lasting <30 mins	No		Gabapentin	I
	13	62 F	Unilateral	1–2 per day, lasting <60 mins	Yes, migraine		NSAID	S	TAC
	14	36 F	Unilateral	1–6 per day, lasting 10–60 mins	No		Gabapentin	I
	15	56 F	Unilateral	1–5 per day, lasting >30 mins	Yes, migraine		Verapamil; gabapentin	I
	16	25 F	Unilateral	Daily, lasting >60 mins	No		Verapamil	S	TMJ dysfunction
Boulton et al. (2007) (17)	1	49 F	L	2 years; daily, erythema lasting 10–15 mins, pain up to 9 hours	Yes, migraine	Cold	Manual compression; ibuprofen	I
	2	66 F	L	5 years; daily, with periods of remission for months	No		Head movements; ibuprofen	I
	3	58 F	L	2 years; daily, 30 mins–48 hours	Yes, migraine	Touch; heat	Indomethacin	S	TMJ dysfunction
Staatsen and Koehler (2008) (7)	1	47 F	LR	4 years; 2 per week, lasting 1–10 mins	Yes, migraine	Heat	Sumatriptan	I
	2	51 M	R	4 years; 2 per month, lasting 1–10 days	No	Stress	NSAIDs	S	Trigeminovascular/cervical pathology
	3	45 F	LR	20 years, more frequent in last 3 years; 1–2 per week, lasting up to 15 mins	No		Triptans; NSAIDs	I
Brill et al. (2009) (4)	1	7 M	LR	3 years; 1–3 per week, lasting 10–45 min	Yes	Heat	Cooling	I
Selekeler et al. (2009) (21)	1	9 M	LR	2 months; 2–10 per day, lasting 10-20 mins	No	Touch	Greater auricular n. LA block	I
Creach et al. (2010) (22)	1	44 F	R	14 months; 1–3 per day, lasting 90–120 mins	Yes	Head movement; physical activity	Neurovascular decompression of R vertebral artery	S	C3 root and vertebral artery compression

M: male; F: female; R: right; L: left; TMJ: temporo-mandibular joint; EPH: episodic paroxysmal hemicrania; CPH: chronic paroxysmal hemicrania; SLE: systemic lupus erythematosus; NSAIDs: nonsteroidal anti-inflammatory drugs; TAC: trigeminal autonomic cephalalgia; LA: local anaesthetic.

Donnet and Valade (2) proposed a distinction between two different types of RES; the first being an idiopathic form, more commonly seen in young people and associated with migraine, and the secondary RES occurring more frequently in adults in association with cervical disorders. However, cases have also been reported of the red ear phenomenon occurring in isolation (3,4). In some instances it appears to be precipitated by touch, movements of the head or neck or changes in temperature, but may also occur spontaneously. This relatively newly described condition is rare and is not well understood. Debate continues regarding the underlying pathology as new cases come to the fore. Here we describe the case of a young female with RES and review the literature on this condition.

Case report

A 23-year-old Caucasian female presented to the neurology clinic with a five-year history of recurrent attacks of severe burning pain associated with reddening of the left ear. In early childhood she underwent tonsillectomy, adenoidectomy and bilateral myringotomy, but there was no prior history of migraine, episodic headaches or neck injury. Initially her symptoms were infrequent but for the past 12 months she was having up to three attacks per day. There were no obvious triggers and they occurred at any time of the day. Typically, episodes were characterised by intense burning pain in the left ear pinna, which reached maximum intensity after 5–10 minutes, and lasted 15–25 minutes before slowly dissipating (Figure 1). Occasionally the pain radiated over the adjacent scalp, but at no time was this or the ear tender to touch. Attacks were always associated with reddening of the ear (Figure 2), which started a few minutes after the pain and then normalised as the pain resolved. There were no other migrainous or associated symptoms and she was completely asymptomatic between episodes. On examination she had no focal neurological deficits. In the past she had been trialled unsuccessfully on a number of abortive (paracetamol, ibuprofen, sumatriptan (including the subcutaneous formulation)) and prophylactic (amitriptyline, pregabalin, verapamil) drugs. As part of her work-up she had normal magnetic resonance imaging (MRI) brain and cervical spine scans, blood panel (inflammatory markers, autoimmune screen) and ear, nose and throat (ENT) review.

Figure 1.

Pain intensity during an attack of red ear syndrome. During 12 hours of observation the patient had two attacks of red ear syndrome and pain intensity was recorded using a visual analogue scale (0–5). Five minutes into the second attack (broken line), she received high-flow oxygen, which appeared to improve her symptoms.

Figure 2.

Skin changes during acute attack of red ear syndrome. (A) Normal ear between attacks. (B) Approximately five minutes after onset of pain, corresponding to maximum pain intensity, we observed distinct reddening of the superior margin of the ear pinna, with relative sparing of the inferior lobe.

Discussion

Anatomy and physiology

An understanding of the anatomy and physiology of the ear is essential in trying to determine the underlying mechanisms in RES. Given its relatively small size, the ear is unusual in terms of the number of inputs it receives from multiple sensory nerves derived from different neural segments including two superior cervical plexus nerves (C2 and C3) and a combination of four cranial nerves (V, VII, IX and X)(5). The ear lobe itself is supplied by the greater auricular nerve, derived from the second and third cervical root, whilst the anterosuperior aspect is innervated by the auriculotemporal nerve, derived from the mandibular branch of the trigeminal nerve (V3) (Figure 3A). The sensation of pain in the ear can therefore arise directly from a source within the ear itself, e.g. external or middle ear infection, or can be secondary (non-otogenic) pain originating elsewhere, but referred to the ear (5). The most common source of this referred otalgia is dental pathology (6), but non-otogenic ear pain can be referred from a range of structures including the mouth, teeth, larynx or thyroid gland as well as neural, vascular and lymphatic structures in the neck and oesophagus. Further to this, cervical spine degenerative disease is also a significant source of referred otalgia in older patients.

Figure 3.

Innervation and blood supply of the outer ear. (A) Innervation is derived from the greater auricular nerve (C2, C3) and the mandibular branch of the trigeminal nerve (V3), which during a typical attack appeared to be the focus of redness in our patient. (B) Blood is supplied via the middle temporal artery (MTA) and posterior auricular artery (PAA), which both branch from the external carotid artery.

Also of relevance in the aetiology of RES is the blood supply to the ear, which comes from an anastomosis between branches of the middle temporal and posterior auricular arteries, as part of the external carotid circulation (Figure 3B), with sensory innervation of the blood supply arising from the trigeminal nerve (7). Facial skin has both sympathetic vasoconstrictor and parasympathetic vasodilator fibres (8), and increased temperature and erythema of the ear can therefore occur either via inhibition of sympathetic vasoconstrictor fibres or activation of autonomic vasodilatation (3). Vasodilatation, causing burning pain and erythema, can occur via the release of calcitonin gene-related peptide (CGRP) and substance P from sensory neurons in the trigeminal nucleus; these act as vasodilators by stimulating the production of endothelium-derived relaxing factor (nitric oxide) in cutaneous blood vessels (7).

Pathophysiology

In his original paper describing 12 cases, Lance (1) observed that RES was commonly associated with irritative lesions of the third cervical root; this led him to suggest that an antidromic discharge of impulses along C2/C3 may occur, leading to pain and vasodilatation by release of vasodilator peptides, in cases involving an underlying cervical pathology. Support for this hypothesis came from the fact that symptoms were temporarily relieved in one patient following local anaesthetic block of the C3 root and permanently relieved in a second patient after C3 section. Lance also presented two cases where the underlying cause was thought to be TMJ dysfunction, one of whom was apparently cured by the use of a dental plate. In such cases Lance proposed that a local axon reflex, triggered by non-noxious stimuli such as heat, touch or chewing, might precipitate the antidromic discharge. He postulated that RES might be an example of Angry Back-firing C-nociceptor (ABC) syndrome, in which the phenomenon of ‘cross-modality threshold stimulation’ (9) occurs, whereby temperature changes alter the threshold for pain induced by mechanical stimulation. The mechanism by which the C-fibres become ‘angry’ in RES, however, was not precisely specified. The third disorder associated with RES in Lance’s original paper was thalamic syndrome, and the proposed explanation in this case was via disinhibition of trigemino-thalamic neurons leading to continuous burning pain, perhaps in combination with disinhibition of the C3 afferent fibres and thus concomitant vasodilatation.

In the 60 cases presented in the literature so far, including the original 12 cases described by Lance (1) (Table 1), 10 cited cervical pathology as a potential explanation for the symptoms seen. Eleven cases were explained by TMJ dysfunction, and a possible thalamic lesion was cited in two cases. Five of the cases might be categorised under the trigeminal-autonomic cephalalgia (TAC) category (including episodic and chronic paroxysmal hemicrania). The majority of the remaining cases were thought to be idiopathic (29 cases), most of which were associated with migraines (16 out of 29 cases). Rarer associations included herpes zoster virus, Chiari I malformation and primary erythromelalgia. Interestingly, erythromelalgia has similar features to RES including burning pain, erythema and increased skin temperature, but typically affects the feet, or sometimes the hands, very rarely affecting the ears, and with a defined histology (10). This similarity between the two conditions led Brill et al. (4) to suggest that RES may in fact be an auricular variant of erythromelalgia.

Two distinct types of RES were proposed by Donnet and Valade (2), an idiopathic (or primary) form that occurs more often in younger people, is spontaneous and associated with migraine, and a secondary form seen more frequently in older patients, associated with upper cervical pathology or TAC, with specific triggers and occasionally associated with migraine. A variety of precipitants have been described for those cases in the literature so far, including touch, heat, cold, physical activity, neck, head or jaw movements (chewing, tooth grinding, eating), emotion, stress, coughing, sneezing, and lying on the affected side. Specific triggers were cited by patients as precipitating the attacks in both those cases that were presumed to be idiopathic in nature (nine out of 29) and those with a potential underlying secondary cause (19 out of 30).

Regardless of whether the underlying cause is primary or secondary in nature, a common pathophysiological mechanism has been suggested, in which activation of the trigeminovascular system is the final common pathway (2). This so-called trigeminal-autonomic reflex (Figure 4), which consists of a brainstem connection between the trigeminal nerve and facial nerve parasympathetic outflow and acts as a mediator for the vasodilator response, was first proposed as an explanation for a group of short-lasting primary headache syndromes with autonomic features, known as TACs (11). It was suggested that in TACs there may be a pain threshold above which autonomic symptoms (including conjunctival injection, lacrimation, nasal congestion, rhinorrhoea, ptosis, eyelid oedema and Horner’s syndrome) develop.

Figure 4.

Trigeminal-autonomic reflex. Nociceptive sensory innervation of the dura mater is provided by the ophthalmic division of the trigeminal nerve, which projects to second-order neurons in the trigeminocervical complex (trigeminal nucleus caudalis (TNC) and dorsal horns of C1 and C2), before ascending to pain centres in the thalamus. The effector limb of this reflex consists of relay neurons from the trigeminocervical complex, which connect to the superior salivatory nucleus (SSN). Preganglionic parasympathetic neurons then project via the facial nerve to the pterygopalatine ganglion (PPG) and then onto the cranial vessels and dura mater. Trigeminal ganglion (Vg), trigeminal nucleus caudalis (TNC), superior salivatory nucleus (SSN), pterygopalatine ganglion (PPG). (Adapted from May and Goadbsy, 1999) (12).

It remains unclear why selective activation of the trigeminal-autonomic reflex, resulting in vasodilation of vessels primarily limited to the ear, occurs in this syndrome. The answer may reside within the complex anatomical relationship between this reflex arc and the upper cervical roots. Further to this, the ‘cervical-autonomic reflex’ (13) has been proposed as an apparent functional connection between cervical somatic afferents and parasympathetic brainstem afferents, in which stimulation of the greater occipital nerve leads to local release of vasodilator substances.

Al-Din et al. (3) suggest that the neural mechanisms in migraine are not only capable of stimulating the trigeminal-autonomic reflex (causing cranial autonomic features) but also the cervical autonomic reflex, responsible for RES. In cases with a secondary cause, e.g. TMJ dysfunction, an exacerbation in TMJ pain may exceed the threshold required to trigger autonomic dysfunction. A unifying hypothesis was therefore put forward to explain both primary and secondary cases of RES in which antidromic discharge of impulses along the C3 root induces autonomic changes by stimulating the presumed cervical autonomic reflex. Primary cases are caused by either centrally or peripherally generated stimulation, which can also involve the trigeminal-autonomic reflex.

Raieli et al. (14) described eight cases of idiopathic RES associated with migraine and suggested that in such idiopathic cases, the attacks might be due to an intrinsic dysregulation of brainstem trigeminal-autonomic circuits rather than a local axon reflex as previously suggested by Lance (1).

Where does red ear syndrome fit in?

TACs include: short-lasting unilateral, neuralgiform headache with conjunctival injection (SUNCT), hemicrania continua, chronic paroxysmal hemicrania (CPH), episodic paroxysmal hemicrania (EPH) and cluster headache. Goadsby and Lipton (11) proposed that a common mechanism, namely the trigeminal-autonomic reflex, could be responsible for both the paroxysmal hemicranias and RES.

The International Headache Society (IHS) classification system instituted in 1988 comprises 13 major categories of headache, separated into primary and secondary disorder. Within the secondary group is a subdivision of ‘not classifiable headaches’, and it was suggested by Raieli et al. (14) that this would be a reasonable place for RES to be classified. This suggests therefore that RES may be a form of TAC mediated by the auriculo-temporal nerve (a branch of V3), although Evans and Lance (15) prefer the term ‘auriculo-autonomic cephalgia’ to TAC, to reflect the fact that the greater auricular nerve, which supplies the relevant part of the earlobe, is derived from C3.

The apparent association with migraine in the idiopathic cases described by Raieli et al. (14) was supported by their duration and the fact that, like migraine, they were more commonly unilateral. They proposed that red ear episodes that occurred in the absence of migraine in these patients could represent ‘atypical’ or ‘acephalgic’ migraine attacks.

In contrast, Kumar and Swanson (16) do not believe that RES can be regarded as a form of TAC or have a place in the IHS classification of secondary headaches. Due to its varied aetiology, they instead argue that RES is best viewed as an entity which itself has primary and secondary forms, the primary being migrainous and the secondary a neuralgiform radiculopathy involving the C3 root with release of vasodilator peptides or nitric oxide. Similarly, Al-Din et al. (3) also argue that RES is a separate syndrome from TACs, since whereas in cases of CPH and EPH there is an absolute response to indomethacin, they did not observe such a response in their patients when indomethacin was tried.

Staatsen and Koehler (7) suggest that RES could be considered as an extracranial version of other primary headaches, such as migraine or TAC.

Treatment

So far, no consistent treatment response for RES has been described (16). In the 60 cases described in the literature (Table 1) a wide variety of treatments have been utilised (Table 2) with varying degrees of success.

Table 2.

Treatment options for red ear syndrome.

Treatment		Frequency	Idiopathic	Secondary
Drugs	Gabapentin	13	8	5
	NSAIDs (ibuprofen, indomethacin)	11	3	8
	Calcium channel blockers (flunarizine, verapamil, nimodipine)	5	2	3
	Tricyclic antidepressants (imipramine, amitriptyline)	2		2
	Triptans (including sumatriptan)	2	2
	Methysergide	1		1
	Propranolol	1		1
Root/nerve block/section	C2–C3 root block	2		2
	Greater auricular nerve block	1	1
	C3 root section	1		1
	Glossopharyngeal nerve section	1		1
	Neurovascular decompression	1		1
Other	Cooling	4	2	2
	Dental plate	1		1
	Manual compression	1	1
	Head movements	1	1

NSAIDs: nonsteroidal anti-inflammatory drugs.

The multiplicity of potential causes for RES has been mooted as an explanation for the absence of a single effective treatment choice and for the fact that many patients have proven to be treatment resistant, especially those with an underlying secondary pathology as a possible cause (17). It has therefore been argued (7) that as in the treatment of headache in general, a trial-and-error approach may be necessary as multiple drugs may be effective and/or required in different cases. Where possible, treatment should be in terms of the underlying pathology, for example C3 section or placement of a dental plate have been shown to be successful in some secondary cases of RES (1).

Idiopathic versus secondary forms of red ear syndrome

All previously described cases (Table 3) were subtyped into those with idiopathic disease (n = 29), those with secondary disease (n = 30) and cases with a mixed picture (n = 1). A number of observations can be made regarding idiopathic and secondary forms of RES: Patients with idiopathic disease were younger on average than those with secondary forms (34.8 ± 20.5 versus 49.5 ± 18.6), they had less-frequent episodes (50% daily, 32% weekly, 18% monthly versus 70% daily, 20% weekly, 10% monthly) of very similar duration (approx. 25% lasting <15 minutes and approximately 75% lasting >15 minutes in both cases). A history of migraine was more common in idiopathic than secondary cases (55% versus 20%) and the gender ratios were similar (idiopathic: 38% male; secondary 30% male). Interestingly the presence of a specific precipitant, such as touch, heat or neck, head or jaw movements, were more common in the secondary compared to idiopathic forms of RES (63% versus 31%), which might be attributable to the underlying pathology. Secondary cases also appeared to have a greater response to treatments than idiopathic cases, both for drugs (67% versus 52%) and more invasive procedures such as nerve or root block and/or section (17% versus 3%), which might also be related to the proposed pathology.

Table 3.

Characteristics of idiopathic and secondary forms of red ear syndrome.

		Idiopathic	Secondary
n		29	30
Average age		34.8 ± 20.5	49.5 ± 18.6
Sex	Males %	38	30
	Females %	62	70
Migraine	Yes %	55	20
Frequency	Daily %	50	70
	Weekly %	32	20
	Monthly %	18	10
Duration	<15 minutes %	26	24
	>15 minutes %	74	76
Precipitant	Yes %	31	63
Response to treatment	Drugs %	52	67
	Nerve block/section	3	17
	Other	14	10

Conclusion

In the absence of any identifiable structural lesion, our patient, who is young, appears to have the idiopathic form of RES, although she does not complain of migraine and her episodes are very frequent, which is more in keeping with secondary forms. She did have a number of ENT procedures as a young girl, and despite her symptoms developing 10 years later, one might speculate that neuronal pathways within the trigeminal-autonomic reflex may have been sensitised following surgery. She failed on a number of analgesics but did benefit from high-flow oxygen while being monitored.

Clinical implications

Red ear syndrome is a rare headache disorder characterised by episodic unilateral erythema of the ear associated with pain and a burning sensation.

Two forms have been identified: Idiopathic disease is more commonly seen in young people and associated with migraine, and secondary disease occurs more frequently in adults in association with cervical disorders.

The exact cause remains unknown but activation of the trigeminal-autonomic reflex has been postulated as a final common pathway.

Treatment options vary considerably.

Footnotes

Funding

This research received no specific grant from any funding agency in the public, commercial, or not-for-profit sectors.

Conflict of interest

None declared.

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Red ear syndrome: A review of all published cases (1996–2010)

Abstract

Background

Patients and methods

Conclusions

Keywords

Introduction

Case report

Discussion

Anatomy and physiology

Pathophysiology

Where does red ear syndrome fit in?

Treatment

Idiopathic versus secondary forms of red ear syndrome

Conclusion

Clinical implications

Footnotes

Funding

Conflict of interest

References