What Is Red Ear Syndrome (RES)?
A Rare Cause of Ear Redness and Warmth
Medically reviewed by John Carew, MD
Red ear syndrome (RES) is a very rare disorder. It causes redness with moderate to severe burning on the outside of one or both ears.
An episode of red ear syndrome can occur without a known cause or due to triggers such as rubbing your ear. Heat or cold stimulation or brushing your hair can also start the problem.
While the cause of red ear syndrome is still unclear, it may involve the peripheral or central nervous system. Treatment involves a choice of drugs, though results vary by person.
This article describes red ear syndrome, its symptoms, causes, and diagnosis. It also explains treatment and ways to cope with this problem.
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Symptoms of Red Ear Syndrome
Symptoms of red ear syndrome vary. Episodes can be unilateral (one-sided), bilateral (two-sided), or alternating, though the problem most often affects just one ear. In unilateral red ear syndrome, the left ear is more likely to be involved.
The majority of people affected by red ear syndrome have daily episodes. However, intermittent attacks that occur in clusters every month or two are possible. Episodes typically last 30 to 60 minutes, though their duration can range from seconds to four hours.
Symptoms of red ear syndrome include:
Pain in the affected ear that can be dull, sharp, or poking
Burning sensation of the affected ear
Redness at the visible portion of your outer ear (pinna), with the inclusion of your temple, cheek, upper or whole face on the same side as your affected ear
Causes and RES Triggers
While there is no known cause for red ear syndrome, there are some theories. Dysfunction of your central nervous system as well as peripheral nervous system has been suggested.
RES can be classified into primary RES and secondary RES. Primary RES has the following features:
More common in younger individuals
Variable occurrence
Onset with migraine headache either during the prodromal phase (the phase between initial symptoms and full development of a migraine) or the attacks
Dysfunction of the peripheral vertical nerves (the parts of your nervous system outside your brain and spinal cord) or the central nerves of the trigeminal-autonomic pathways (nerve pathways that carry pain sensation from your face and mouth)
Secondary RES is more common in older women without a history of migraine. It occurs in association with any of the following conditions:
Intracranial or vertical spine lesions (lesions affecting parts of the spinal cord)
Central pain syndrome (pain due to damage to the brain, brainstem, or spinal cord)
Pleomorphic adenoma of the parotid gland (a tumor on a salivary gland)
Traction injury of upper cervical roots (neck trauma affecting the roots of the spinal nerves)
Atypical neuralgias (nerve pain from nerve damage)
Toxicity due to drugs such as cytarabine (a drug used in leukemia treatment)
Some evidence also links red ear syndrome with multiple sclerosis, though its association with the disease requires further research.
Triggers
Symptoms can occur spontaneously or as a result of the following RES triggers:
Touch
Stress
Hot or cold sensation
Coughing
Sneezing
Neck movements
Chewing
Combing hair
Awakening from sleep, especially in hot weather
Ear Complications in COVID-19
COVID-19 has been linked with ear and hearing effects such as pain, hearing loss, tinnitus (ringing in the ear), otitis media (middle ear infection), and vertigo (a spinning sensation). While some of these effects may be linked to ear pain and redness, the onset of red ear syndrome is not noted.
It is inconclusive whether these other ear effects originate from the COVID-19 virus, its treatments, or the vaccinations used to prevent it.
Treatment to Manage RES
The response to treatment for RES is variable. General nondrug therapies can reduce symptoms. Strategies for managing RES without drugs include:
Avoiding triggers
Preventing/treating headaches when migraine is involved
Cooling your ear(s) during an acute attack
While several medications are used to manage symptoms of red ear syndrome, there is no cure. Reactions to medications to help with symptoms vary by individual and cause of the RES. Some drugs used to treat red ear syndrome include:
Sibelius (flunarizine)
Deseril, Sansert (methysergide)
Living With Red Ear Syndrome
Living with red ear syndrome can be challenging. The burning and pain can be severe, making daily activities difficult for people who live with this long term.
Identifying and avoiding your known triggers can help you reduce the frequency and severity of episodes. Managing an underlying condition, such as migraine, can also help you reduce the number of episodes you experience.
Talk to your healthcare provider about support groups that might be helpful as you learn how to live with this problem. It's unlikely that you'll find a support group specifically for red ear syndrome, but you may find support from people managing similar symptoms, such as the following groups:
Who Diagnoses RES?
The diagnosis of RES can be made by different healthcare providers depending on your symptoms. RES is most often diagnosed by the following specialists:
Neurologist (specialist in the brain and nervous system): Diagnosis of RES when it occurs with migraine
Otolaryngologist (ear, nose, and throat specialist): Diagnosis of idiopathic RES with painful, throbbing ear
Dermatologist: Diagnosis of RES when it presents with symptoms of erythromelalgia (a skin disease characterized by warmth and recurrent burning pain)
Diagnosing red ear syndrome is challenging for any healthcare provider since it is a relatively unknown and rarely encountered condition. It was first described in 1994. Due to the rarity of the disease, research is limited.
Red ear syndrome is a diagnosis of exclusion. This means that to make a diagnosis, your healthcare provider follows a careful step-by-step process of ruling out conditions that share your symptoms until it's obvious which one is most likely the cause of your symptoms. This process is called making a differential diagnosis.
The differential diagnosis for red ear syndrome includes ear, nose, and throat (otolaryngological) disorders and skin (dermatological) diseases. Some of these conditions include:
Conditions related to your brain and skull must be ruled out before a diagnosis of red ear syndrome is made. Your provider may require magnetic resonance imaging (MRI) of your cervical spine and brain to rule out these conditions. An orthodontic assessment of your temporomandibular joints may be needed to rule out TMJ disorder.
The following diagnostic criteria for primary red ear syndrome have been proposed, though further research is needed to determine the variables that can be involved:
Episodes of external ear pain last up to four hours.
Ear pain has at least two of the following characteristics: burning quality, one-sided location, mild to moderate severity, or triggered by touch or heat stimulation to the ear.
The ear pain is accompanied by redness of the external ear on the same side.
Episodes of pain and redness occur at least one or more times daily, though attacks can occur less frequently.
There is a history of at least 20 attacks that fulfill the above criteria.
Symptoms can't be attributed to any other disorder.
Summary
Red ear syndrome is a rare condition that is likely underreported. Its main feature involves red and throbbing outer ears. The problem can occur in one ear, both ears, or be alternating.
The severity of ear pain, the frequency of attacks, and the length of episodes vary by person. In some cases, the pain and redness can spread to your cheeks and jaw on the same side as the affected ear.
While the exact cause of red ear syndrome is not known, it has been linked to migraines or other conditions like TMJ disorder. Red ear syndrome does not involve complications, but it can be a sign of another condition that needs treatment.
Further research is needed so healthcare providers can understand the causes and best ways to treat this condition. Though there is no cure for red ear syndrome, getting an accurate diagnosis and treatment can help you manage symptoms and cope with the chronic nature of this problem.
Read the original article on Verywell Health.
