Wednesday, June 7, 2017

Red ear syndrome

Inspired by a patient

Raieli V, Compagno A, D'Amelio M. Red Ear Syndrome. Curr Pain Headache Rep.
2016 Mar;20(3):19. 

The Red Ear syndrome (RES) is an intriguing syndrome originally described for the first time nearly 20 years ago. RES is characterized by unilateral/bilateral episodes of pain and burning sensation of the ear, associated with ipsilateral erythema. RES episodes are indeed isolated in some patients, but they can occur in association with primary headaches, including in particular migraine in the developmental age. Although the underlying pathophysiological mechanisms are still uncertain, in the recent years the described comorbidities have aroused increasing interest because of possible clinical implications. Moreover, RES seems to be more often associated with clinical features of migraine partially provoked by the involvement of the parasympathetic system. This clinical association has shed new light on the pathophysiology of RES, supporting the hypothesis of a shared pathophysiological background, for example, through the activation of the trigeminal autonomic reflex. Current therapies of RES will be also discussed. Finally, we will resume the more controversial aspects of this relatively new and probably underestimated neurological syndrome.

From the article 

Though pain and reddening are mainly localized to the ear lobe, they might extend towards the cheek, the mandible (especially in secondary cases) the occiput  or to the whole hemicranium.

RE attacks, initially described as unilateral and side-locked, show a wide variability mainly unilateral in adults cases and bilateral in the pediatric age.  Pain is usually described as burning and moderately disabilitating. The duration of episodes widely varies, with most of attacks lasting less than one hour. Frequency of attacks is also variable (described also as pluridaily attacks, especially when RES is associated with TACs [trigeminal autonomic cephalalgia]). In our population its frequency was typically related to migraine frequency, while in several studies with RES associated to TACs, frequency was similar to TACs without RES with periods of remission…

Few clear evidences are available in literature about therapy of RES. In Lance’s case series [2], both pharmacological (propanolol, flunarizine, amytriptyline, imipramine) and non-pharmacological (prescription of a dental plate, local anesthetic block, surgical section, or an application of ice-pack to the ear) treatments produced usually a partial remission of symptoms. In two patients of a paediatric series, affected by RES in association with migraine, administration of nimodipine, and flunarizine determined a reduction of intensity and frequency of both disorders. In addition, Arjona  reported five new cases of RES in adulthood, using amitriptyline in all cases, displaying a complete resolution of symptoms in 3 out of them, however they didn’t specify what was the relationship with treatments. One patient who had also a history of migraine was treated with amytryptiline and piroxicam, but we cannot state whether the treatment was effective or not because there was no follow-up. 
Indomethacin seemed to be ineffective in the treatment of RES associated with TACs, recommending therefore gabapentin as the first-line management or, possibly, verapamil.

Lambru G, Miller S, Matharu MS. The red ear syndrome. J Headache Pain. 2013
Oct 4;14:83. doi: 10.1186/1129-2377-14-83.

Red Ear Syndrome (RES) is a very rare disorder, with approximately 100 published cases in the medical literature. Red ear (RE) episodes are characterised by unilateral or bilateral attacks of paroxysmal burning sensations and reddening of the external ear. The duration of these episodes ranges from a few seconds to several hours. 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 RES seems to be associated with migraine, whereas late-onset idiopathic forms have been reported in association with trigeminal autonomic cephalalgias (TACs). Secondary forms of RES occur with upper cervical spine disorders or temporo-mandibular joint dysfunction. RES is regarded refractory to medical treatments, although some migraine preventative treatments have shown moderate benefit mainly in patients with migraine-related attacks. The pathophysiology of RES is still unclear but several hypotheses involving peripheral or central nervous system mechanisms have been proposed.

From the srticle

Gabapentin has been the most widely tried medication in RES patients to date. In a series of 12 patients with RES, 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 RES also responded to a combination of gabapentin and verapamil although no information about dosages was given. One patient failing gabapentin did obtain a slight reduction of pain using pregabalin 600 mg daily.

Lisotto C, Mainardi F, Maggioni F, Zanchin G. O003. Red ear syndrome: a new form of trigeminal autonomic cephalalgia? J Headache Pain. 2015 Dec;16(Suppl 1):A127. (no abstract)

Some authors have suggested that RES could be considered a form of trigeminal autonomic cephalalgia (TAC) on the basis that both have a similar phenotype characterized by short-lasting attacks of unilateral pain, associated with cranial autonomic features. The majority of RES described in the literature are primary but secondary RES has been reported. The underlying disorder can encompass mainly upper cervical spine lesions and temporo-mandibular joint dysfunction. Several different drugs have been tried in RES patients, but most of them seem to produce a marginal benefit. Gabapentin has been the most widely used medication in subjects with RES. This condition has been described in some patients in association with TACs, except for hemicrania continua, thereby supporting the possible nosological and pathophysiological link between RES and TACs. We propose RES to be included in the ICHD-3 beta Appendix in the TACs chapter.

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