An Unusual Headache: Red Ear Syndrome

A 4-year-old boy presented with a 2-year history of unilateral recurrent ear erythema (generally on the left ear) that was associated with episodic ear swelling, discomfort, and a burning sensation (Figure 1). These episodes occurred up to three times every month; each episode lasted for approximately 1 h 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 (RES) being made. The patient started treatment with cetirizine for 2 weeks and showed a slight reduction in the frequency of attacks.

Figure 1.

Red and slightly swollen left ear during an episode

Red ear syndrome is a rare entity characterized by paroxysmal unilateral or bilateral painful attacks to the external ear that are accompanied by ear redness, burning, or warmth. Swelling is rare [1]. RES 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. 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 [2]. Familiarization with RES presentation symptoms is important to recognize this relatively new disease, thus avoiding delayed diagnosis and mistreatment.