Abstract
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.
Highlights
Red Ear Syndrome (RES) is a rare condition originally described by Lance in 1994 [1] and further characterized in 1996 [2]
We propose that RES be included in the 4th Chapter of the IHS Classification, “Other Primary Headache Disorders”, until further understanding regards its pathophysiology and treatment emerges
The features of RES have been reviewed in 100 patients
Summary
Red Ear Syndrome (RES) is a rare condition originally described by Lance in 1994 [1] and further characterized in 1996 [2]. 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 [16]. 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 [10].
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