What is red ear syndrome

Red ear syndrome is a very rare entity characterized by paroxysmal unilateral or bilateral painful attacks to the external ear that are accompanied by ear redness (erythema), burning, or warmth ¹. Swelling is rare ². Red ear syndrome episodes are generally isolated, but they can also occur with primary headaches as migraine among young patients or with trigeminal autonomic headaches among the elderly. The duration of these episodes ranges from a few seconds to several hours ³. The attacks occur with a frequency ranging from several a day to a few per year. Episodes can occur spontaneously or be triggered, most frequently by rubbing or touching the ear, heat or cold, chewing, brushing of the hair, neck movements or exertion. Early-onset idiopathic red ear syndrome seems to be associated with migraine, whereas late-onset idiopathic forms have been reported in association with trigeminal autonomic cephalalgias ³. Secondary forms of red ear syndrome occur with upper cervical spine disorders or temporo-mandibular joint dysfunction ³. Red ear syndrome is regarded refractory to medical treatments, although some migraine preventative treatments have shown moderate benefit mainly in patients with migraine-related attacks.

Red ear syndrome has a slight female preponderance (54 women, 47 men), with a male to female ratio of 1:1.25. Interestingly, though, in the two series of young migraineurs with red ear syndrome described by Raieli et al. ⁴, the majority of patients were males (75% and 68% of patients respectively). From the available data ⁴, the median age of onset of red ear syndrome is 44 years old, although a wide range of 4 to 92 years is reported.

Red ear syndrome is a very rare disorder, with approximately 100 published cases in the medical literature ³.

The exact cause of red ear syndrome remains unknown and treatment options vary considerably ⁵.

Figure 1. Red ear syndrome

Footnote: A 4-year-old boy presented with a 2-year history of unilateral recurrent red ear (generally on the left ear) that was associated with episodic ear swelling, discomfort, and a burning sensation. These episodes occurred up to three times every month; each episode lasted for approximately 1 hour and spontaneously resolved. Initially, the episodes were isolated, but during the last 6 months, they began to be associated with a migraine without aura simultaneous to ear redness. In the interval between two episodes, the patient had no problem. His perinatal history and childhood development were reportedly normal. Visual inspection results of the pinna and otoscopic examination results were bilaterally normal. The head and neck examination and allergological assessment were within normal limits as well as laboratory tests and a magnetic resonance imaging (MRI) of the brain. A neurological visit led to a diagnosis of idiopathic red ear syndrome being made. The patient started treatment with cetirizine for 2 weeks and showed a slight reduction in the frequency of attacks.

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Red ear syndrome triggers

Most patients with red ear syndrome have both spontaneous and triggered attacks. However, some patients can exhibit exclusively spontaneous attacks (25 patients) ⁶ or triggered attacks (14 patients) ⁷. Triggers most often include: heat (reported in six patients) ⁸, rubbing the ear (three patients) ⁹, physical exercise (three patients) ¹⁰ and neck movement (three patients) ¹¹. Other cutaneous stimulations able to provoke RE episodes include: light touching of the ear, brushing of the hair, chewing, tooth grinding and showering ¹².

Red ear syndrome causes

The pathophysiological mechanisms underlying red ear syndrome are currently unclear. However, several theories have been proposed and can be divided into two main groups namely, peripheral theories with mechanisms involving a dysfunction in cervical spinal nerves (predominantly C3 root) and central theories in which the underlying mechanisms would involve a dysregulation of brainstem trigemino-autonomic circuits.

In his original paper, Lance ¹³ observed that red ear syndrome was commonly associated with irritative lesions of the third cervical nerve root and this led him to suggest that, in cases of underlying cervical pathology, an antidromic discharge of impulses along C3 may occur leading to pain and vasodilatation due to release of vasodilator peptides. Support for this hypothesis appeared to come from the fact that symptoms were temporarily relieved in one patient following local anaesthetic block of the C3 root and permanently relieved in another patient after C3 section. Lance also presented two cases in which attacks were triggered but where the underlying cause was thought to be temporomandibular joint dysfunction. In these cases he 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 red ear syndrome might be an example of Angry Back-firing C-nociceptors syndrome, in which the phenomenon of ‘cross-modality threshold stimulation’ occurs, whereby temperature changes alter the threshold for the pain induced by mechanical stimulation ¹⁴. It was this hypothesis of an antidromic discharge from fibres of the third cervical root causing vasodilation in the referred pain area by discharge of vasodilator peptides, which led Lance to label red ear syndrome an auriculo-autonomic cephalgia, though the exact mechanism by which C-fibres become “angry” was not specified ¹⁵.

On the basis of the presence of red ear syndrome features in a patient with paroxysmal hemicrania, Goadsby and Lipton ¹⁶ suggested that both conditions may share common pathogenic mechanisms centred on the brainstem connections between the trigeminal nerve and the facial parasympathetic outflow. They suggested a primary role for the dysregulation of brainstem trigemino-autonomic circuits in the pathophysiology of the syndrome. The anatomical basis of this unifying hypothesis would be the convergence of processing nociceptive information at the level of the trigeminal nucleus caudalis and dorsal horn nuclei of the upper cervical spine, of both trigeminal afferents and C1-C2 spinal afferents. red ear syndrome has subsequently been described in association with cephalgia ¹⁷ and SUNA (short-lasting unilateral neuralgiform headache attacks with autonomic symptoms ¹⁸, thereby supporting the possible nosological and pathophysiological link between red ear syndrome and TACs.

Given the frequent association of red ear syndrome with migraine, Raieli et al. ¹⁹ proposed that symptoms of migraine-associated red ear syndrome may be due to the trigeminovascular activation during migraine attacks producing extracerebral vasodilatation via direct release of vasodilator substances (substance P, CGRP and nitric oxide). The activation of the trigeminovascular system would explain the ear pain that extends beyond the trigeminal distribution to the earlobe, due to the overlap between trigeminal and upper cervical spinal nerves in the trigemino-cervical complex ²⁰. The same authors showed that red ear syndrome was associated with migraine features partially provoked by parasympathetic system activation and concluded that red ear syndrome could be considered a specific sign of parasympathetic hyperactivation, via the trigemino-autonomic reflex, during migraine, in essence considering red ear syndrome as a migraine-related phenomenon, sharing activation of pathophysiological mechanisms known to be pivotal in migraine.

However, all these theories concerning a trigemino-autonomic dysregulation as the main mechanism in red ear syndrome and red ear syndrome associated with migraine and trigeminal autonomic cephalgias raise an interesting anatomical inconsistency. The trigemino-autonomic reflex is based on a brainstem connection between the trigeminal nerve and the facial parasympathetic outflow ¹⁶. According to this hypothesis, the facial nerve parasympathetic outflow fibres that appear to be responsible for the facial autonomic symptomatology in trigeminal autonomic cephalgias should also be able to produce vasodilation and therefore redness of the skin of the ear in red ear syndrome. However, unlike other parts of the face, such as the cheek and the nose, the vasodilation of the skin of the ear is mainly under sympathetic vasoconstrictor control and occurs when there is inhibition of the sympathetic vasoconstrictor fibers ²¹. During trigeminal autonomic cephalgias attacks there is marked trigemino-parasympathetic activation, along with a sympathetic deficit. It may be possible that in cases of red ear syndrome in conjunction with trigeminal autonomic cephalgias, the presence of an imbalance between parasympathetic and sympathetic systems in the latter may in turn facilitate the inhibition of the sympathetic tone of the ear giving raise to the red ear phenomenon. However, as it is the sympathetic dysregulation and not a parasympathetic activation that is the predominant mechanism for the ear reddening, it seems less likely that the trigemino-autonomic reflex plays a central role at least in isolated cases of red ear syndrome. Other mechanisms may contribute more specifically to the red ear phenomenon.

A further pathophysiological hypothesis is based on the fact that the clinical presentation of red ear syndrome is strikingly similar to the clinical presentation of erythromelalgia (EM), a dermatological condition characterised by paroxysmal episodes of burning pain and erythema often involving hands and feet. The diagnostic criteria of erythromelalgia include: burning pain, aggravated by warmth and relieved by coldness, erythema and increased skin temperature ²². The pathophysiology of erythromelalgia is unfortunately unclear but local primary damage of vascular and neuronal structures has been proposed as the main pathophysiological mechanism underlying it. In essence, it is postulated that primary vascular misdistribution leading to skin hypoxia may cause a secondary hypoxic-induced neuropathy. Conversely, primary small-fibre dysfunction may lead to a secondary vascular misdistribution and hypoxia accompanied by secondary capillary proliferation ²³. Given the clinical similarities, some authors have raised the possibility that red ear syndrome may be an auricular variant of erythromelalgia, possibly caused by similar small sensory and sympathetic nerve dysfunction ²⁴. Moreover, they stated that since a gene has been identified in hereditary erythromelalgia caused by mutations of Na 1.7 channels in sensory and sympathetic nerves ²⁵, sodium-channel blocking drugs may be a reasonable therapeutic option to explore in the management of red ear syndrome.

Secondary causes of red ear syndrome

The majority of red ear syndrome described in the medical literature are primary but secondary red ear syndrome has been reported (25 cases). Lance ¹³ originally described the first series of 12 patients with red ear syndrome. Ten out of these patients had a secondary pathology thought to be responsible for the red ear episodes. Subsequently other authors have reported cases of red ear syndrome secondary to an underlying pathology.

Secondary causes of red ear syndrome

• Upper cervical spine:
  • Hypertrophy of the ipsilateral C2-C3 facet joint
  • Degeneration of superior facet of C4
  • Cervical arachnoiditis with posterior column myelomalacia
  • Traction injury of upper cervical roots
  • Narrowing of C4 neural foramen
  • Chari I malformation
  • Chronic whiplash
  • Congenital fusion of C1-C3 vertebrae with enlargement of the cervical spinal canal
  • Neurovascular compression between vertebral artery and C3 root
• Cranial and cervical neuralgias • Atypical glossopharyngeal neuralgia
  • Atypical trigeminal neuralgia
  • C3 root neuralgia
• Temporomandibular joint (TMJ) dysfunction
• Thalamic syndrome
• Herpes zoster
• Pleomorphic adenoma of carotid body

Secondary red ear syndrome cases can be divided in two main groups: upper cervical spine lesions and temporo-mandibular joint dysfunction. Eleven patients with red ear syndrome secondary to upper cervical spine abnormalities were found in the literature. Different aetiologies have been implicated including cervical spondylosis, infections of the meninges, traction injury, narrowing of the neural foramen at the side of the pain and congenital cervical abnormalities. Eleven patients with red ear syndrome and co-existing temporo-mandibular joint dysfunction ipsilateral to the side of the pain have been described ²⁶. One patient’s red ear syndrome attacks settled with the use of a dental plate seeming to point to a causal relationship between temporo-mandibular joint dysfunction and red ear syndrome in this case ¹³.

Other secondary causes of red ear syndrome include Chiari 1 malformation, thalamic syndrome and a neurovascular compression of the C3 root by the vertebral artery ²⁷.

In summary, the primary form of red ear syndrome affects younger males and females almost equally and is characterised by short-lasting episodes occurring with a variable frequency. Attacks are usually spontaneous and most patients have a personal history of migraine. The secondary form of red ear syndrome occurs in older people, with a female preponderance, usually without a personal history of migraine and is characterised by short-lasting, daily attacks, often provoked by specific triggers. More clinical and therapeutic data is needed to ascertain if primary and secondary forms show different responses to medical treatments, as suggested by some authors ²⁶.

Red ear syndrome signs and symptoms

Site of pain

Pain is felt over the pinna of the ear, often maximal on the ear lobe. It can radiate towards the cheek and mandible ²⁸, as well as towards the occiput ²⁹ and the involvement of the V1 area or whole hemicranium has also been described ³⁰.

Laterality of attack

From the original description, red ear episodes were thought to be predominantly unilateral and side-locked ³¹. Subsequently, cases of unilateral attacks occurring on either side in the same patient ³² as well as bilateral attacks have been described ³³. In total, 62 patients (62%) had strictly unilateral attacks, 31 (31%) bilateral attacks and 6 (6%) either unilateral or bilateral attacks. Data is missing for one patient.

Among patients with unilateral red ear episodes, the pain is reported more frequently on the left (58%) than the right side (42%) (left-sided attacks: 30 patients; right-sided attacks: 22 patients; data not available for 10 patients).

Severity of pain

The degree of intensity of the pain episodes in red ear syndrome has not been properly assessed. Generally, it is reported as an annoying discomfort rather than an excruciating pain. However, authors do also report cases of patients complaining of moderate to severe attacks. Lambru et al. described a case of a patient with SUNA (Short-lasting unilateral neuralgiform headache attacks with autonomic symptoms) and red ear syndrome occurring on the same side. The severity of the red ear episodes were felt by the patient to be moderate (VRS: 5/10) compared to the excruciating intensity of the SUNA attacks (VRS: 8-10/10), suggesting a lower degree of severity of the pain of an red ear episode compared to that typical of a trigeminal autonomic cephalgia attack ¹⁸. However, some authors have reported cases of patients complaining of severe pain. Boes et al. ³⁴ described a patient with episodes of left ear pain attacks associated with reddening that the patient described as severe. The attacks were suppressed completely with indometacin 75 mg/day, supporting a diagnosis of paroxysmal hemicrania-associated red ear syndrome.

Character of pain

Patients consistently described the pain as a burning sensation. Other characters that have been less often reported include a dull ache ²⁹, a stabbing pain ³⁴, a sharp pain ²⁶ and a jabbing pain ³⁵.

Duration of the individual attacks

The duration of each red ear episode can vary widely. Generally, the attacks are short-lasting with 63 red ear syndrome patients (63%) reporting attacks lasting up to four hours. The majority had attacks lasting 30 to 60 minutes ³². However, short-lived attacks lasting seconds have been reported ¹¹. Only six patients (6%) had attacks lasting over 4 hours of whom two complained of constant pain ³⁵. Data on duration of attacks in 32 patients was not available from the case reported.

Attack frequency and remission periods

The attack frequency varies immensely both among sufferers and within individual sufferers. The majority of red ear syndrome patients report daily attacks ranging from one daily to up to 20 attacks a day ²⁷. A small minority of patients only report infrequent attacks that occur 1 to 6 times per month ¹⁹.

Red ear episodes occur most commonly during the daytime, although Lance ³⁵ reported a case in whom red ear attacks had awakened the patient from sleep.

In most patients, red ear syndrome seems to be a chronic problem without significant remission periods, although, in some cases the follow-up was too short to allow any meaningful conclusion to be drawn ³⁶. Some case reports clearly describe patients with episodic bouts of red ear syndrome attacks alternating with remission periods. Donnet and Valade ³⁷ described a 92-year-old woman who experienced attacks of burning pain and red ear associated with lacrimation following an episodic pattern. The episodes were short-lasting, occurring in bouts of daily attacks lasting 15 to 45 days every 12–18 months. Similarly, the case of a 36-year-old woman who complained of episodes of ear, temple, cheek and upper neck pain associated with ear redness and other autonomic symptoms such as conjunctival injection, tearing and nasal blockage lasting between 10–60 minutes was described. She had daily attacks occurring in “clusters”, presumably alternating with remission periods ³². Finally Boulton et al. described the case of a 66 year-old woman with a five year history of episodes of pain behind the left ear and ear redness, initially occurring in bouts of daily attacks lasting 1–2 weeks, followed by remissions lasing months but that subsequently became almost daily ²⁶.

Associated features

The key clinical feature of red ear syndrome is the presence of burning ear pain associated with marked ear reddening although Hirsch reported five patients with episodes of bright red discoloration of the ears not associated with auricular pain ³⁸. The erythema of the ear consistently follows the ear pain and usually lasts as long as the burning pain does. Rare exceptions to this pattern exist. Selekler et al. described the case of a patient in whom the discoloration of the ear would disappear within 10 min from the onset of the attack, whereas the burning pain would continue for a further 5–10 minutes ³³ and Boulton et al. ²⁶ reported a case of a 66 year-old woman in whom episodes of pain behind the left ear were accompanied by reddening of the ipsilateral ear only 50% of the time.

The area of reddening is usually limited to the external ear and is most pronounced over the earlobe ³⁹. Similarly to the pain pattern, the redness can extend beyond the ear, most frequently involving the cheek ³². Involvements of the temple, upper neck and rarely the entire face ipsilaterally to the affected ear have also been reported ³⁰. Given the complex sensory and autonomic innervation of the external ear, it would be important to obtain more precise information on the area of reddening to help clarify the pathophysiological mechanisms at play. Unfortunately, besides some descriptions of redness involving only the earlobe and one case in whom the redness involved the helices ⁴⁰, very little anatomical detail has been provided in the literature.

In the vast majority of red ear syndrome patients, redness and warmth of the ear are the only associated symptoms. However, swelling of the ear during attacks has been reported in three cases ²⁴ and other cranial autonomic symptoms such as aural fullness ²⁷, ipsilateral lacrimation ⁴¹ and ipsilateral conjunctival injection, lacrimation and nasal blockage have also featured in the literature ³². Nausea, vomiting, photophobia, phonophobia, osmophobia, motion sensitivity or restlessness are not features of red ear syndrome.

Red ear syndrome diagnosis

Due to the association of secondary red ear syndrome with upper cervical spine pathology and temporo-mandibular joint dysfunction, an MRI of the cervical spine should be carried out and, when the clinical suspicion for temporo-mandibular joint dysfunction is high, an orthodontic assessment is warranted. In addition, given the possible link between red ear syndrome and thalamic lesions ⁴⁰, an MRI scan of the brain should be included in the diagnostic work up of red ear syndrome.

Given the description of a patient with chronic paroxysmal hemicrania, presenting as red ear syndrome with unilateral short-lasting, attacks of ear pain and redness, sometimes involving the entire side of the face which responded to indometacin ⁴², all patients with short-lasting strictly unilateral side-locked attacks of ear pain and redness occurring more than once a day should have a trial of indometacin up to 225 mg a day ⁴³, to exclude an indometacin-sensitive headache disorder.

Classification and diagnostic criteria

Given the relatively limited description of red ear syndrome in terms of aetiology, pathophysiology and treatment, it is currently not included in the International Classification of Headache Disorders-II ⁴⁴ and International Classification of Headache Disorders-IIIβ ⁴⁵. Some authors have suggested that red ear syndrome could be considered a form of trigeminal autonomic cephalgia on the basis that both are a similar phenotype characterized by short-lasting attacks of unilateral pain, occurring once or more times a day associated with cranial autonomic features ¹⁶. Lance proposed that since symptoms in red ear syndrome are mostly centred over the earlobe (which derives its sensory supply from C3), red ear syndrome may be a form of autonomic cephalalgia mediated by the greater auricular nerve rather than the trigeminal nerve, and that therefore, the term “auriculo-autonomic cephalgia” rather than trigeminal autonomic cephalgia should be used ¹⁵.

Red ear syndrome often appears to occur in associated with migraine without aura. Indeed, 81% (n=60/74) of patients reported in the literature with primary red ear syndrome had a personal history of migraine ²⁷. Given this striking association, Raieli et al., suggested that red ear syndrome be encompassed into the wider clinical spectrum of migraine, with red ear syndrome episodes considered a migraine related phenomenon when occurring during a migraine attack and an “acephalgic” migraine phenomenon when occurring outside a migraine attack ¹⁹. Subsequently, the same authors studied a group of 40 young migraineurs with red ear syndrome and found that red ear syndrome was significantly associated with some of the migraine associated features, possibly explained by parasympathetic nervous system activation. They concluded that red ear syndrome could be considered a specific sign of parasympathetic hyperactivation via the trigemino-autonomic reflex, occurring during migraine ⁴⁶.

In view of its various possible aetiologies, other authors argued that primary and secondary forms of red ear syndrome be kept separated. They proposed that primary red ear syndrome be considered a migraine-related phenomenon, whereas secondary forms considered a neuralgiform radiculopathy involving the C3 root ¹¹.

Although the pathophysiological mechanisms and consequently the treatment of red ear syndrome remains unclear, the clinical description in 100 patients provides enough detail to allow us to propose the first set of diagnostic criteria (Table 1). Since secondary forms of red ear syndrome are rather frequent, physicians should carefully look for secondary causes of red ear syndrome, namely a disease of the upper cervical spine and temporo-mandibular joint dysfunction. Secondary red ear syndrome due to a disorder of the neck or temporo-mandibular joint should be classified within Chapter 11 of the International Classification of Headache Disorders-IIIβ (Headache or facial pain attributed to disorder of the cranium, neck, eyes, ears, nose, sinuses, teeth, mouth or other facial or cervical structure).

Table 1. Proposed diagnostic criteria for primary red ear syndrome

A.	At least 20 attacks fulfilling criteria B-E
B.	Episodes of external ear pain lasting up to 4 hours.
C.	The ear pain has at least two of the following characteristics:
	-Burning quality
	-Unilateral location
	-Mild to moderate severity
	-Triggered by cutaneous or thermal stimulation of the ear.
D.	The ear pain is accompanied by ipsilateral redness of the external ear.
E.	Attacks occur with a frequency of ≥1 per day, although cases with lower frequency may occur.
F.	Not attributed to another disorder.

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Red ear syndrome treatment

Currently, there are no medications with approved efficacy. A study has proposed the use of gabapentin, amitriptyline, or non-steroidal anti-inflammatory drugs, but with poor results ⁴⁷. Familiarization with red ear syndrome presentation symptoms is important to recognize this relatively new disease, thus avoiding delayed diagnosis and mistreatment.

Several of the drugs routinely used in primary headaches and other pain syndromes have been tried in cases or small series of red ear syndrome patients in an open-label fashion. Most of these drugs have been reported produce a marginal benefit only. red ear syndrome is, therefore, generally considered refractory to treatments. Medications with some reported effect are summarised in Table 2.

Table 2. Effective treatments in red ear syndrome

	Number of patients	Type of RES	Dosage	Improvement
Gabapentin	7	Primary and secondary	–	Good
Ice pack	4	Primary	–	Good
Amitriptyline	3	Primary and secondary	–	Good
Indometacin	2	Primary	50-75 mg/day	1 patient: pain free
				1 patient: good
Imipramine	1	Secondary	125 mg/day	50% reduction in pain severity
Verapamil	1	Secondary	–	–
Ibuprofen	1	Primary	400 mg	Pain free
Propranolol	1	Secondary	80 mg	Good
Flunarizine	1	Primary	–	–
Nimodipine	1	Primary	–	–
Pregabalin	1	Primary	600 mg	Mild
Methysergide	1	Secondary	–	–
Greater auricular nerve blockade	1	Primary	2% prilocaine 1 cc + 125 mg methylprednisolone	Pain free

[Source ³ ]

Non-steroidal anti-inflammatory drugs

Ibuprofen taken daily was reported to be highly effective in two patients with red ear syndrome ²⁶. In one of these cases, the benefits persisted even when the drug was discontinued due to side effects, raising the possibility of a spontaneous improvement of the condition rather than a specific effect of the medication. Conversely, ibuprofen 1200 mg for 7 days has had no effect in other cases ³⁶. Oral indometacin given at the dose of 25 mg tds, completely suppressed the pain in the patient with chronic paroxysmal hemicrania-related red ear syndrome ⁴² but when tried in a dose up to 50 mg tds in other five patients with red ear syndrome, only one reported an improvement ²⁶.

Tricyclic antidepressants

Three patients were said to respond to a trial of amitriptyline but no details of dosing were given ²⁷. Imipramine 125 mg daily was effective in one patient, reducing pain severity by 50%, but no mention of any effect on ear redness was made ³⁵.

Beta-blockers

The β-blocker propranolol was used at a dose of 40 mg twice daily to successfully treatment one case of red ear syndrome secondary to cervical spondylosis. However, in another case of secondary red ear syndrome associated with possible temporo-mandibular joint dysfunction, propranolol was not found to be helpful ³⁵.

Calcium channel blockers

Verapamil was reported to be effective in two red ear syndrome patients. In both these cases, the syndrome was associated with other conditions such as chronic whiplash and temporo-mandibular joint dysfunction. In one patient, verapamil was administered in combination with other drugs ³². Flunarizine and nimodipine have also been tried in two patients with migraine-related red ear syndrome. In both the cases, the treatments led to a reduction in frequency and severity of both the red ear syndrome and migraine episodes ¹⁹.

Anticonvulsants

Gabapentin has been the most widely tried medication in red ear syndrome patients to date. In a series of 12 patients with red ear syndrome, seven of the eight on gabapentin reported a marked improvement in terms of frequency of attacks and ear colour changes. One patient with possible cluster headache-related red ear syndrome also responded to a combination of gabapentin and verapamil although no information about dosages was given ³². One patient failing gabapentin did obtain a slight reduction of pain using pregabalin 600 mg daily ³⁰.

Serotonergic agonists and antagonists

Triptans have been tested in two patients with limited benefit reported ³². One patient with red ear syndrome secondary to cervical arachnoiditis, failed to respond to pizotifen, but did show improvement with methysergide ³⁵.

Topical lidocaine or steroidal application

Topical applications of anaesthetics and steroids are generally ineffective. Two patients failed topical application of lidocaine [12,20] and two patients topical steroids (clobetasol 0.05% cream being used in one such case) ³⁶. However, cooling of the ear by application of an ice-pack has been reported to provide significant relief in five patients during an acute attack ³⁶.

Local nerve blockade

Blocking the greater auricular nerve with 1 cc prilocaine 2% and 125 mg methylprednisolone in a patient with red ear syndrome led to a symptom free period of more than eight weeks. In this case, red ear syndrome episodes were brought on by light touching of the ear and during these attacks the patient reported greater auricular nerve tenderness ³³. Lance reported a poor outcome of C3 root blockades in two patients with red ear syndrome. The block was performed in one patient using bupivacaine 0.5%, with pain relief lasting only few hours, whereas a C2 and C3 root block in the second patient relieved the pain for several hours ³⁵.

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