Primary and secondary red ear syndrome: Implications for treatment

Approximately 57 patients with red ear syndrome have been reported in the literature since it was initially described in 1994. The clinical phenotype therefore continues to be defined and no consistent treatment response has been described. We report three new cases of red ear syndrome and suggest that the disorder may exist as a primary trigeminal or cervical autonomic cephalalgia, coexist with other rare trigeminal autonomic cephalalgias, or may be secondary. The pathogenesis in these different circumstances may be distinct, the triggers specific and, when secondary, the disorder may be more resistant to treatment.