Red Ear Syndrome

Red Ear Syndrome is a rare disorder of unknown etiology which was first described in 1994. The defining symptom of Red Ear Syndrome is - yes, you guessed it correctly - redness of one or both external ears, accompanied by a burning sensation[1].

Attacks of skin redness and burning sensation or pain in one or both external ears are the only common symptoms. Pain is often most pronounced at the ear lobe, and sometimes radiates to the jawbone and cheek. The pain is normally mild, but has occasionally been described as severe, as a dull ache, as a stabbing pain, as a sharp pain, and as a jabbing pain. The attacks can last seconds or hours, with 30 minutes to an hour being typical. Most patients have daily attacks, ranging from 20 a day to a few a year.

It is believed this syndrome may represent an auriculo-autonomic headache or be part of the group of disorders known as trigeminal autonomic cephalgias, which includes cluster headaches. It is more often associated with migraine in younger people, while late-onset Red Ear Syndrome may result from pathology of the upper cervical spine or trigeminal autonomic cephalgia.

Most patients with Red Ear Syndrome have both spontaneous and triggered attacks. Triggers most often include heat, rubbing the ear, physical exercise, and neck movement. Other stimulations able to provoke episodes of Red Ear Syndrome include light touching of the ear, brushing of the hair, chewing, tooth grinding and showering.

Red Ear Syndrome is considered rare, but the exact prevalence is unknown. There are only a bit more than 100 cases described in the medical literature. A variety of treatments have been tried with a limited success.

[1] Lambru et al: The red ear syndrome in Journal of Headache and Pain – 2013