Introduction:
Autoimmune (when the body’s immune system attacks its healthy cells) red ear syndrome is caused by an unknown origin and results in painful, reddening episodes of one or both ears. Pain may sometimes extend up to the neck and cheeks. It is usually confined to one side. Simple physical activities and touching the ear or brushing hair can trigger pain episodes. Drug therapy is found to be beneficial in relieving pain and reducing the frequency of pain episodes.
What Is Autoimmune Red Ear Syndrome?
There are two types of syndromes based on their onset:
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Primary Red Ear Syndrome - It shows early onset, hence, more commonly affecting children and adolescents. The majority of cases show an association with migraines.
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Secondary Red Ear Syndrome - It shows late onset, hence, more commonly affecting older people. It shows an association with cluster headaches, spine problems, TMJ (temporomandibular joint) problems, and other problems associated with jaws.
It may also show an association with erythromelalgia (a rare condition that exhibits redness, warmth, and burning type of pain in the extremities) and hand-foot syndrome.
What Causes Autoimmune Red Ear Syndrome?
The pathophysiological behind this is still unclear. However, two ways are suspected to be the possible reason behind it; dysfunction in cervical spinal nerves or dysregulation of brainstem trigeminal-autonomic circuits.
Who Experiences Red Ear Syndrome?
Red ear syndrome can affect people of any age, but it is relatively rare. On an average it affects the people of age groups from 40 years. Male children are more likely affected than female children.
What Triggers Autoimmune Red Ear Syndrome?
Pain episodes of autoimmune red ear syndrome can be both spontaneous or triggered. Triggering factors include heat, light touching or rubbing the ear, physical exercise, neck movements, brushing of the hair, chewing, tooth grinding, and showering.
What Are the Symptoms of Autoimmune Red Ear Syndrome?
One may feel burning pain with redness of earlobes on one (most commonly) or both sides. The intensity and duration of pain vary from person to person. Generally, it is in the form of an annoying dull ache. However, some may feel severe pain. Also, it is characterized by short, frequent episodes of pain rather than continuous pain. The majority of patients report daily attacks ranging from one to twenty attacks per day, most commonly during the daytime.
The area of redness and burning pain is limited to the outer earlobes. However, it may extend up to the cheeks, temple, upper neck, or rarely ipsilateral (appearing on the same side) to the face.
What Are the Ways to Diagnose Red Ear Syndrome?
MRI (magnetic resonance imaging) of the cervical spine should be done to find out any secondary association of red ear syndrome associated with the spine. If it is suspected to be associated with TMJ disorders, then an orthodontic assessment is required.
- Proposed diagnostic criteria for primary red ear syndrome:
A. Minimum 20 attacks per day and also showing criteria B and E.
B. Pain episodes last up to four hours.
C. Pain showing a minimum of two of the following characteristics:
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Burning sensation.
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Unilateral location.
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Mild to moderate severity.
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Triggered by touch or heat.
D. The ear pain extends to half of the face.
E. One or more than one attack per day, although cases with lower frequency may occur.
F. Not related to other disorders.
What Is the Differential Diagnosis for Autoimmune Red Ear Syndrome?
Other conditions which may show the same symptoms as red ear syndrome are:
1. Relapsing polychondritis (a rare degenerative condition causing inflammation of cartilaginous structures like ears).
2. Cellulitis (a serious bacterial skin infection that results in redness, inflammation, and pain in the infected area).
3. Chilblains (tiny, itchy skin swellings that result when exposed to cold weather).
4. Contact dermatitis (a skin rash or blister that develops due to an allergic reaction triggered by contact with certain substances like perfumes, soap, plants).
What Is the Treatment for Autoimmune Red Ear Syndrome?
Pain-relieving medicines are used for the treatment of red ear syndrome to relieve the symptoms. Following are some of the drugs which have shown benefits in the treatment of red ear syndrome.
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Non-steroidal Anti-inflammatory Drugs: Ibuprofen and Indomethacin are used as pain-relieving drugs for red ear syndrome. However, they may show different responses in different patients.
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Tricyclic Antidepressants: Amitriptyline and Imipramine can successfully relieve the pain but show no effect on ear redness.
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Beta-Blockers: Propranolol is used in the treatment of secondary red ear syndrome if it is associated with spondylosis.
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Calcium Channel Blockers: Secondary red ear syndrome associated with chronic whiplash of TMJ can be treated with Verapamil in combination with other drugs. Primary red ear syndrome associated with migraine can be treated with Flunarizine and Nimodipine. It shows both reductions in the frequency and severity of pain episodes.
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Anticonvulsants: Gabapentin (anti-seizure medicine) is used to treat red ear syndrome and has shown benefits of reducing redness of the ear and frequency of attacks.
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Serotonergic Agonists and Antagonists: Limited benefits can also be achieved with Methysergide if red ear syndrome is associated with arachnoiditis (a rare painful condition caused by the inflammation of the arachnoid membrane that encloses the spinal cord nerves).
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Topical Application of Anesthetics or Steroids: Topical applications of Lidocaine (an anesthetic cream that numbs the area where the cream has been applied) and steroid creams may not be useful in relieving pain. However, ice packs may comfort acute pain. Icing the affected around two to three times a day for eight to ten minutes numbs the area and relieves pain. However, care should be taken that if too much icing is done, it may cause frostbite.
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Local Nerve Blockade: Blocking pain passing the nerve (greater auricular nerve) by injecting local anesthesia may show some benefit in relieving pain. The effect of which may be observed from a few hours to some days.
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Botulinum Toxin Type A: Red ear syndrome has also been reported to be successfully treated with injections of Botulinum Toxin Type A.
Conclusion:
It is an extremely rare condition, with only 101 cases reported in the literature. Further research in this field is needed to know the exact etiology and treatment options for autoimmune red ear syndrome. Though experimental treatment options are being tried on patients with this condition, they significantly provide symptomatic relief improving the patient's lifespan.
