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Red Ear Syndrome or Red Ear Symptom/Sign?

Reported in the journal this month are 6 more cases of red ear syndrome (RES), most of which are thought to be primary in origin. They all have a symptom/sign of a red ear (RE), and mostly respond to primary headache therapies, or don’t respond at all, which fits with my experience and that of others. A couple of the cases may represent secondary causes for RES, but that is not the point of their communication or this editorial. I believe we need to leave secondary RES as a syndrome, but the RE itself maybe just a symptom or sign, and as the authors suggest, “RES may be an associated feature of primary headaches.” I would agree with their assessment, and agree that one should treat the underlying primary headache disorder in hope of getting symptom relief, so that one ends up treating the RE case as migraine, trigeminal autonomic cephalalgias (TACs), or neuralgias. From my experience this approach works, as clinically over the years I had come to the same conclusion as the authors. Thus, these current reported cases are important, not to just add to the cases of RES that have now been reported many times, but because they confirm the heterogeneity of the disorders causing this “syndrome.” I suspect that RE will remain a symptom or sign among other autonomic phenomena in headache disorders. So, the real value of this paper is to point out that attempts to treat the underlying primary headache disorder may offer the best results in some cases. Of course, some baseline imaging of head and neck is necessary in all cases of RES, since secondary etiologies can be present, and appear to make this a syndrome. When I first encountered a case of RES several years ago, I found it to be unique in a patient who maintained it was her main complaint and that on exposure to cold it became her main sign. Otherwise her case had a migraine phenotype. RES was first described by Dr. James Lance, whose patients had cervical spine anomalies on imaging as did our patient. Subsequently, we published our case along with colleagues from the Mayo Clinic. Since then I have seen other cases of RE related to entities such as: “migraine,” “occipital neuralgia,” and “trigeminal neuralgia” and did a review on the subject along with another colleague, Dr. David Dodick. I have followed all cases reported in the literature in adults and children, with interest. One interesting case I had seen, but never reported, was a woman in her mid-forties with severe ear pain on one side with autonomic symptoms of tearing and lid droop on the same side, who did not complain of a RE. She had some occipital discomfort on the same side, and when her occipital nerve was palpated it reproduced the auricular pain and her ear turned bright red (Fig. 1). She did not know her ear was red, until it was pointed out to her with a mirror. She stated her ear was “on fire” and the RE sign seen was identical to the RES cases reported, and she had auricular and pinna pain at the same time. Later she also developed pain in her throat with swallowing that suggested an atypical glossopharyngeal neuralgia, which has been noted in the past with secondary RES suggested to be a cranial or cervical neuralgia. No treatments worked as well as a nonsteroidal antiinflammatory drug (NSAID), moderate dose of verapamil, and an auricular temporal nerve block. It is almost impossible to classify her case, but I would think it maybe a primary headache disorder related to the TACs and neuralgias. All imaging was normal and no secondary cause has been found.