View this post on Instagram

A post shared by Justin Bieber (@justinbieber)

In the three-minute-long video, Bieber brought our attention to a disorder that many of us had never heard of. Now that we're aware of its existence, here’s all you need to know about Ramsay Hunt Syndrome.

What is Ramsay Hunt syndrome?

Ramsay Hunt syndrome, also called herpes zoster oticus, is a rare neurological disorder characterized by paralysis of the facial nerve or facial palsy, and hearing loss in the affected ear.

The disorder was named after American neurologist James Ramsay Hunt, who first described the syndrome in 1907. It is caused by the varicella-zoster virus, the same virus that causes chickenpox and shingles.

According to Mayo Clinic, the condition “occurs when a shingles outbreak affects the facial nerve near one of [the person’s] ears.”

It is caused by the varicella-zoster virus, the same virus that causes chickenpox and shingles in adults.

“The virus can remain dormant for decades in a person who has had chickenpox as a child. Reactivation of the varicella-zoster virus results in shingles and, in some cases, develops into Ramsay Hunt syndrome,” said the National Organization for Rare Disorders (NORD) on its website.

“After chickenpox clears up, the virus still lives in your nerves. Years later, it may reactivate. When it does, it can affect your facial nerves,” said Mayo Clinic.
It is, however, still unknown why the virus reactivates and affects the facial nerve.

Signs, symptoms

There are two main signs and symptoms of Ramsay Hunt syndrome, according to Mayo Clinic, which include:

• painful red rash with blisters full of fluid on, in, and around one ear
• facial weakness or paralysis—also called facial palsy—on the same side as the affected ear

Rash and facial paralysis usually occur at the same time. However, in some cases, one can appear or occur before the other. It is also possible for the rash to never happen in other cases.

“Facial muscles affected by nerve palsy may be weak or feel stiff and may result in the inability of affected individuals to smile, wrinkle the forehead or close their eye on the affected side. In some cases, speech may become slurred,” NORD explained.

Meanwhile, rash and painful blisters may also affect the mouth, soft palate, and top portion of the throat.

People who suffer from Ramsay Hunt syndrome may also experience:

• Otalgia or ear pain
• Temporary hearing loss
• Tinnitus or ringing in the ears
• Difficulty closing one eye
• Vertigo or a sensation of spinning or moving
• Changes in taste perception or loss of taste
• Dry mouth and eyes

In some cases, ear pain can be intense and can potentially spread to affect the neck. Hearing loss can also become permanent in rare cases.
Some other symptoms include nausea and vomiting.

Risk factors and complications

According to health experts and multiple studies, Ramsay Hunt syndrome can occur in any person who has had chickenpox. It is more common in older adults or people aged 60 years and above, while it is rare in children.

While the condition is not contagious, the reactivation of the virus that causes it can cause chickenpox in people who have not previously had chickenpox or those who have not received the vaccine for it.

The syndrome can also be severe for people who have immune system problems.

When left untreated or unmanaged, Ramsay Hunt syndrome may cause complications such as:

• Permanent hearing loss and facial weakness
• Eye damage, including eye pain and blurred vision

Postherpetic neuralgia is another possible complication, which occurs when a shingles infection damages nerve fibers.

“The messages sent by these nerve fibers become confused and exaggerated, causing pain that may last long after other signs and symptoms of Ramsay Hunt syndrome have faded,” Mayo Clinic said.

Treatment, prevention

Studies said that pain caused by Ramsay Hunt syndrome and long-term complications can be eased and prevented by taking certain medications, including:

• Antiviral drugs, such as acyclovir (Zovirax), famciclovir (Famvir), or valacyclovir (Valtrex), can help combat the chickenpox virus.
• Corticosteroids such as prednisone can help boost the effect of antiviral drugs.
• Anti-anxiety medications, including diazepam (Valium), help relieve vertigo.
• Pain relievers to ease intense pain associated with Ramsay Hunt syndrome.

“Most authorities agree that beginning antiviral treatment within three days of onset appears to have the greatest benefit as prompt diagnosis and management seems to improve outcomes,” NORD explained.

“Further treatment is directed towards specific symptoms that are apparent in each individual,” it added. “This includes pain medication, carbamazepine, an anti-seizure medicine which may help reduce neuralgic pain, and vertigo suppressants like antihistamines and anticholinergics.”

Special care to prevent corneal injuries—such as abnormal drying and foreign body irritation—is also recommended for individuals who experience an inability to close the affected eye. This includes taking the following steps:

• Use moisturizing eye drops throughout the day or when the eye becomes dry.
• Apply ointment to the eye and tape the eyelid shut or wear an eye patch at night.

Other ways to reduce discomfort brought by Ramsay Hunt syndrome include:

• Keeping rash-affected areas clean.
• Applying cool, wet compress to the rash.
• Taking an over-the-counter pain reliever or an anti-inflammatory drug.
• According to Mayo Clinic, the disorder can still be prevented. Children’s routine vaccination against chickenpox reduces the chances of infection with the chickenpox virus.
• For people aged 50 and above, the shingles vaccine is recommended.

Often misdiagnosed

Ramsay Hunt syndrome affects both males and females. It is the second most common cause of atraumatic peripheral facial paralysis.

According to one study, an estimated 5 out of every 100,000 people develop Ramsay Hunt syndrome each year in the United States. However, some researchers believe cases of the disease often go undiagnosed.

Doctors often identify the syndrome based on medical history, physical exam, and the disorder’s distinctive signs and symptoms.

Some studies have also pointed out that the condition can also be misdiagnosed as a disorder that can be similar to those of Ramsay Hunt syndrome.

One similar disorder is Bell’s palsy, which presents similar symptoms such as facial paralysis, pain behind the affected ear, and facial droop including difficulty in making facial expressions.