What Is Ramsay Hunt Syndrome? The 'Rare' Condition That Has Left Justin Bieber With Facial Paralysis

Ramsay Hunt syndrome is a painful rash around the ear, on the face, or on the mouth, and occurs when the varicella-zoster virus infects a nerve in the head. It is a rare neurological condition.

Justin Bieber is suffering from a rare neurological condition known as Ramsay Hunt syndrome ( Image Source :Instagram/@justinbieber )

Canadian singer Justin Bieber recently announced that he is taking a break from performing because he is suffering from a condition called Ramsay Hunt syndrome. 

The 28-year-old singer explained in a video posted on his official Instagram account that Ramsay Hunt syndrome has left him unable to move half of his face and unable to take the stage. 

What Is Ramsay Hunt Syndrome?

Ramsay Hunt syndrome is a painful rash around the ear, on the face, or on the mouth, and occurs when the varicella-zoster virus infects a nerve in the head. 

The varicella-zoster which causes the Ramsay Hunt syndrome is the same virus that causes chickenpox and shingles, and is believed to infect the facial nerve near the inner in people with the syndrome, leading to irritation and swelling of the nerve. Ramsay Hunt syndrome mainly affects adults, but in rare cases, children may be affected.

Ramsay Hunt syndrome is also known as herpes zoster oticus or geniculate ganglion herpes zoster. The syndrome is a late complication of varicella-zoster virus (VZV) infection. Ramsay Hunt syndrome is a rare neurological condition, and results in inflammation of the geniculate ganglion of cranial nerve CVII.

Ramsay Hunt syndrome is named after American neurologist James Ramsay Hunt, who served as an army officer in World War I, and described three different syndromes during his lifetime. Of these, the most famous syndrome is the second, and is known as Ramsay Hunt syndrome. 

Early stages of the Varicella-zoster virus infection cause fever and diffuse vesicular rash. Though the virus often remains dormant in the body after the initial infection, subsequent reactivation of the virus causes a "zoster" or "herpes zoster" phenomenon. The Ramsay Hunt syndrome consists of pain and a vesicular rash along the involved nerve's distribution, according to the US National Institutes of Health. 

The nerve involved determines the distribution and associated symptoms of the syndrome. In less than one per cent of zoster cases, the facial nerve is involved. When this happens, it results in Ramsay Hunt syndrome.

Symptoms Of Ramsay Hunt Syndrome

Ramsay Hunt syndrome is characterised by ipsilateral (occurring on the same side of the body) facial paralysis, otalgia (ear pain), and vesicles in the auditory canal or on the auricle. People can experience a painful rash on the eardrum, ear canal, earlobe, tongue, and roof of the mouth on the side with the affected nerve.

Additional symptoms of Ramsay Hunt syndrome include a change in taste sensation, dry eye, tearing, hyperacusis, nasal obstruction, and dysarthria. Hyperacusis is a disorder in loudness perception, with patients suffering from this appearing overly sensitive to a range of sounds, finding many noises unbearable and painfully loud. Dysarthria is a motor speech disorder in which the muscles used to produce speech are damaged, paralysed or weakened.

According to the NIH, hearing loss, tinnitus, and vertigo are observed in a person in which the vestibulocochlear nerve is affected. People can experience hearing loss on one side.

A person with tinnitus experiences ringing or other noises in one or both the ears. Hoarseness in individuals with Ramsay Hunt syndrome may indicate involvement of the vagus nerve. 

After the onset of neurologic symptoms, vesicles may develop, but the first presenting symptom is usually pain. Though the vesicles typically appear on the auricle, they can be seen along the affected side of the face, scalp, palate, and tongue.

Some people may also experience a sensation of things spinning. Other symptoms include weakness on one side of the body that causes difficulty closing one eye, eating, making expressions, and making fine movements of the face, according to Mount Sinai Health System, an integrated health care system providing medical care to communities in New York, where it is based, and global communities.

How Can Ramsay Hunt Syndrome Be Diagnosed?

A healthcare provider will usually diagnose Ramsay Hunt syndrome by looking for signs of weakness in the face and a blister-like rash, and through different tests.

These tests may include blood tests for varicella-zoster virus, electromyography (EMG), Magnetic Resonance Imaging (MRI) of the head, nerve conduction, and skin tests for varicella-zoster virus. Nerve conduction will help determine the amount of damage to the facial nerve. 

How Can Ramsay Hunt Syndrome Be Treated?

Ramsay Hunt syndrome can be treated with strong anti-inflammatory drugs called steroids such as Prednisone. Other medications include antiviral medicines such as acyclovir or valacyclovir. 

If the pain continues even with steroids, strong painkillers are often given. While a person has weakness of the face, they may wear an eye patch to prevent injury to the cornea, and other damage to the eye if it does not close completely, according to Mount Sinai. A special eye lubricant may be used at night, and artificial tears could be used during the day to prevent the eye from drying out. 

The healthcare provider can advise other medicines if one has dizziness. 

Prognosis Of Ramsay Hunt Syndrome

When there is not much damage to the nerve in a person, they are likely to get better completely within a few weeks. However, if the damage is more severe, they may not fully recover, even after several months.

The chances of recovery are better if the treatment is started within three days after the symptoms begin.

What Are The Possible Complications Of Ramsay Hunt Syndrome?

The possible complications of Ramsay Hunt syndrome include changes in the appearance of the face from loss of movement, change in taste, and damage to the eye, resulting in a loss of vision. 

Also, certain nerves may grow back to the wrong structures, causing abnormal reactions to a movement. For instance, smiling causes the eye to close. 

Other complications include persistent pain, and spasm of the face muscles or eyelids. Occasionally, the varicella-zoster virus may spread to other nerves, and even to the brain and spinal cord. This can result in confusion, drowsiness, headaches, limb weakness, and nerve pain. 

A hospital stay may be needed if these symptoms occur, and a spinal tap may help determine whether other areas of the nervous system have been infected.

How To Prevent Ramsay Hunt Syndrome?

If a person loses movement in the face, or has a rash on the face and facial weakness, they must immediately reach out to a healthcare provider.  

Though there is no known way to prevent Ramsay Hunt syndrome, treating it with medicine soon after symptoms develop can improve recovery.