Medical

Ramsay Hunt Syndrome: What is it?

Following the cancellation of his tour, Justin Bieber revealed he is suffering from facial paralysis. His Ramsay Hunt Syndrome diagnosis has left many wondering what it is. Here is all you need to know about this syndrome.

By Ameya Arora

18 Jun 2022

An American army officer and a neurologist, James Ramsay Hunt, in 1907 coined the term Ramsay Hunt Syndrome, describing a condition in which shingles affect nerves in your face close to either one of your ears. A 2001 study published in the Journal of Neurology, Neurosurgery, and Psychiatry reports that the shingles affecting either ear is a condition caused by Varicella Zoster Virus (VZV). The virus also causes chicken pox, which is most common in children. If you’ve had chickenpox as a child, VZV can reactivate later in your life and cause shingles.

Both shingles and chickenpox lead to a rash that appears in the affected area of the body. Unlike chickenpox, a shingles rash near facial nerves around your ears can cause other complications, such as pain in the ear, or facial paralysis. These complications are the characteristics of Ramsay Hunt Syndrome.

If you notice such a rash on your face along with symptoms such as facial muscle weakness, you should consult your doctor as early as you can. Early identification of symptoms and treatment can ensure that you don’t experience any complications, which we’ll consider shortly, from Ramsay Hunt Syndrome.

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Who Is Vulnerable To Ramsay Hunt Syndrome?

The syndrome can affect anyone who has had chickenpox or shingles in the past. A 2012 study published in the Permanente Journal reports that although the Ramsay Hunt Syndrome can affect anyone from 3 months to 82 years, people in their 70s and 80s are at a higher risk. People who have low immunity are also likely to experience more severe symptoms and complications. They are also less likely to have a successful recovery.

What Are The Symptoms Of Ramsay Hunt Syndrome?

The most common and visible symptoms of Ramsay Hunt Syndrome are shingles rash near one, or both ears, and weakness, stiffness, or facial paralysis. The facial paralysis is noticeable on that side of the face which is affected by the shingles rash. When your face is paralysed, your muscles may feel harder to control, as if they’ve lost their strength.

When you have Ramsay Hunt Syndrome, you can notice a shingles rash by its red, pus-filled blisters, inside, outside, or around the ear. In some cases rashes might also appear in your mouth or throat. In other cases, rashes may not appear at all, but still have paralysis in your face. This paralysis may get reflected in the form of difficulty to express a smile or frown.

Other common symptoms of Ramsay Hunt Syndrome may include:

Pain in the affected ear
Pain in the throat
A kind of a ringing noise in the ear, known as tinnitus
Hearing loss
Difficulty in closing the eyes
Loss of taste
Blurred or unclear speech
Vertigo, the feeling that the room, or the objects around you are spinning
Increased sensitivity to sounds, known as hyperacusis
Dry mouth and eyes
Social anxiety as a result of facial abnormalities

What Are The Causes And Risk Factors Of Ramsay Hunt Syndrome?

According to a 1992 study published in Revista da Associacao Medica Brasileira, if you have had chickenpox as a child, the virus can remain inactive in your nerves for years. However, in times of low immune system functioning and physiological stress, the virus can reactivate, leading to herpes zoster, known as ‘shingles’, anywhere in the body, or ‘Ramsay Hunt Syndrome' if facial paralysis is involved.

The Ramsay Hunt Syndrome is not a contagious disease, but you can develop it if you have shingles virus in your body. If you are exposed to the varicella-zoster virus and haven’t had a previous infection, you can develop chickenpox or shingles. These shingles can further lead to Ramsay Hunt Syndrome. Thus, the causes include:

Prior exposure to chickenpox
Being older than 60 years
Having a weaker immune system

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What Is The Treatment For Ramsay Hunt Syndrome?

A 2016 study published in International Archives Of Otorhinolaryngology, shows that oral antivirals and steroids can significantly help with complications of the condition. The Ramsay Hunt Syndrome requires concrete medications that fight the virus and may also involve injections. Medications may also be provided to target specific symptoms such as vertigo or dizziness. Your doctor may also provide you with eye drops to keep your eyes lubricated and prevent cornea damage. At home, you should try to keep the rash area clean and cool. You can use a cold compress to minimise the pain.

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How Is The Ramsay Hunt Syndrome Diagnosed?

At first, you will notice red blisters or rashes on your face upon getting infected by shingles. If you notice any symptoms of the condition, you should consider seeing the doctor as soon as possible. Your doctor can use several methods to diagnose you with Ramsay Hunt Syndrome, such as:

1. Take your medical history: if you had chickenpox as a child, it is very likely that the virus has reactivated and shingles are responsible for the rashes on your face.

2. Perform a physical examination: your doctor closely examines your body for any other symptoms.

3. Ask you questions about any other symptoms: your doctor might ask if you are suffering from any other symptoms such as pain or dizziness.

4. Take a biopsy, a tissue or fluid sample: your doctor might send a sample of the rash to a lab to confirm the diagnosis.

5. Perform a blood test: your doctor may prescribe you a blood test to check for the varicella-zoster virus.

6. Perform MRI scan (Magnetic Resonance Imaging) of your head. It helps to confirm that any other condition or disorder is not causing the symptoms.

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Can There Be Any Complications of Ramsay Hunt Syndrome?

A 2022 paper published in the National Library of Medicine suggests three categories of complications caused by Ramsay Hunt Syndrome.

1. The presenting symptoms include pain, rash, hearing loss, facial paralysis, dysgeusia, tinnitus, vertigo, hoarseness, and dysarthria.

2. Short-term complications of Ramsay Hunt Syndrome reported by the paper include corneal abrasion, depression, social anxiety, exposure keratopathy, and transmission of chickenpox to unvaccinated or immunocompromised close contacts.

3. Lastly, the paper reports that long-term complications might include development of synkinesis (unwanted contractions of facial muscles). Postherpetic neuralgia (a long-lasting burning pain which persists even after the rash disappears), scarring from the vesicles (a blister which is likely to leave a mark), persistent depression, and lasting social anxiety due to facial dysfunction.

4. If the syndrome is not caught and treated early, permanent weakness of the facial muscles and some hearing loss may persist.

5. Moreover, as in some cases you may not be able to close your affected eye completely, your eye may become extremely dry. If medicated eye drops prescribed by the doctor are not used for lubrication, it is likely to damage the surface of the eye, known as cornea. The damage can cause constant irritation or permanent vision loss (although usually minor).

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