Abstract
Red ear syndrome is relatively new, being first described 12 years ago (1). Fifty-eight patients have been reported up to now (2). Because it is a new and rare syndrome, a consensus about its peculiarities is trying to be developed by the authors that report cases. It seems that red ears with burning pain or discomfort make up the core of the syndrome (1). With the reporting of new cases, different age groups, and primary and secondary forms, an association with other headache syndromes and various therapy options have been presented (2–5). The underlying pathophysiology of the disease is unknown.
Probably, the purest form is seen in children, while secondary forms are probably more frequent in the elderly. In this article, we report a young boy whose ears become red and painful with tactile stimulation. This patient's episodes were ‘cut like a knife’ after greater auricular nerve (GAN) injection with a combination of local anaesthetic and steroid.
Case report
A 9-year-old boy was referred with the complaint of red and burning ears. Severe burning sensation with bright red discolouration in the ears was spontaneous or triggered by contact with the earlobe. While the red colour in the ear disappears within 10 min, the burning sensation lasts for about 15–20 min.
The episodes started 2 months ago with burning and reddening in the right ear, and this was followed by the left ear within a few days. Along with the spontaneous attacks, which occurred 2–10 times a day, episodes were also triggered by contact with the earlobe. Between the attacks, the patient was anxious about any contact with the environment that could precipitate the ear burning. Pillow contact with the ears could interefe with the patient's sleep at nights, and the patient was sleepless for several nights a week.
The patient's attacks were invoked by a light touch to the earlobe with the examiner's finger. During the episode, redness was more prominent in the upper and posterior part of the earlobe and the burning sensation was limited to the ears and did not spread to any neighbouring structure. During the attack, GAN was tender and the tenderness disappeared with the cessation of the burning pain. GAN tenderness during the attacks led us to the idea of blocking the nerve. GAN was blocked with1 cc 2% prilocaine; 125 mg methyl-prednisolone was added to the injection in order to prolong the effect of local anaesthetic. Within 20 min of the injection, neither of the ears was responsive to any harsh stimuli. The patient was totally symptom free during the 8-week control examination.
The patient's physical and neurological examination, routine biochemistry study and hemogram, and cranial cervical and temporal bone MRI, were normal.
Discussion
Red ears as an outstanding feature of a phenomenon was first emphasized by Lance (1). The phenomenon was christened as ‘red ear syndrome’ in the original paper in 1996. Red ears were commonly unilateral and there was a burning pain or a discomfort. In the 12 patients first described, red ears were the primary or sole complaint in some patients, and accompanying or part of the main symptom in others (1).
If only reddening of the ears was considered, the syndrome spectrum could be widened by every red ear accompanying various painful conditions of the head and face. Like other primary headache syndromes whose criteria were composed of a combination of various symptoms (6), the same could be done for red ear syndrome. Combining red ears with the other symptom (i.e. is burning) could limit the syndrome description. Like trigeminal neuralgia, the precipitant factors of the red ear syndrome such as the tactile stimulation could be cited in a ‘description’ paragraph. As with every primary headache syndrome, red ear syndrome could have secondary forms. Therefore, structural abnormalities in the upper cervical region should be excluded (4, 7).
The presence of primary and secondary forms has been proposed and it has been suggested that primary forms generally occur in children and secondary forms in the elderly (7). The patient that we report is a relatively easy patient when compared with the literature, because the patient is very young and there are no illnesses related to age and no congenital malformations either. Precipitation of the burning episode was simple (i.e. with tactile stimulation or spontaneous). In addition to this, tenderness of the GAN during episodes invoked the idea of GAN blockade. Prilocaine was mixed with methyl-prednisolone in order to prolong the effect of the local anaesthetic blockade.
In the original paper in which red ear syndrome is described by Lance, a man aged 83 years old, whose sole complaint was burning pain and redness in his right ear triggered by touching, is presented. An attempt was made to block his posterior auricular and auriculotemporal nerves. Although the symptom combination and precipitating factor are the same and symptom location and the concept of blocking the innervating nerve parallel to ours, the symptoms were not prevented (1). However, details of the blocking procedure were not mentioned in the article. Analogy with the greater occipital nerve injection, tenderness in the greater occipital nerve which has a predictive value in the success of the injection (8). The same could be valid in GAN blockade in this case. Addition of the steroid in the injection fluid could have a further beneficial effect. Contrary to this, the steroid could have a major role in the effect rather than the local anesthetic, as has been suggested in the literature regarding the effect of steroids rather than local anesthetics in the treatment of cluster headaches (8).
Although very dramatic results have been obtained by greater occipital nerve injection in cluster headache and other primary headache syndromes, the exact mechanism of action of the greater occipital nerve blockade is not known very well. Modulation in the central nociceptive signalling is one of the possibities mentioned in previous articles about the subject (9–11).
It seems that in red ear syndrome, in the simplest form which consists of red ears and burning pain triggered with only tactile stimulation, GAN blockade could be a very simple, safe, cheap and effective way of treatment. In other cases whose clinical peculiarities are close to this form, especially with GAN tenderness, GAN injection should be tried. However, we are not able to say which of the ingredients of the injection preparation plays a major role in terminating the episodes.