Justin Bieber’s Ramsay Hunt Syndrome

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So far, this year hasn’t gone so well for Justin Bieber and his wife Hailey. In March, Hailey was hospitalized after having transient ischemic attacks. It was later discovered that this was due to a patent foramen ovale (see our story about her here). And now, the pop star himself has canceled his current tour due to health concerns.

Bieber took to Instagram, saying: “Hey everyone. Justin here. I wanted to update you guys on what’s been going on. Obviously, as you can probably see from my face, I have this syndrome called Ramsay Hunt syndrome [RHS], and it is from this virus that attacks the nerve in my ear and my facial nerves and has caused my face to have paralysis.” He then demonstrated how he is unable to blink his right eye, wiggle his nose, or smile on the right side of his face. He added: “I’m gonna get better and I’m doing all these facial exercises to get my face back to normal and it will go back to normal. It’s just time.”

In a later Instagram story, Bieber asked fans to pray for him as it’s “been getting progressively harder to eat, which has been extremely frustrating.”

What Is Ramsay Hunt Syndrome?

RHS is a rare neurological disorder characterized by paralysis of the facial nerve and a rash affecting the ear or mouth. It is also known as herpes zoster oticus, and is a complication of varicella-zoster infection that has caused inflammation of the geniculate ganglion of cranial nerve VII (the facial nerve).

RHS is described as a triad of symptoms:

  • Ipsilateral facial paralysis
  • Vesicles near the ear and auditory canal, described as erythematous and vesicular; the rash can also affect the mouth, soft palate, and throat

The cause of RHS is the varicella-zoster virus (VZV), which is the same virus that causes chickenpox and shingles. After an acute infection (often in childhood) the virus can remain dormant in the cranial nerve or dorsal root ganglia and reactivate any time later (even many decades later) at times of physiological stress or immunocompromise.

Most commonly the reactivation presents as shingles with pain and vesicles in the dermatome of the affected nerve(s). RHS is a rarer presentation of VZV reactivation.

It is estimated that RHS occurs in five out of every 100,000 people each year in the United States. It affects males and females equally, and accounts for approximately 7% of all cases of acute facial paralysis. RHS can occur at any age, but is most common in older people, in their 60s and 70s.

Symptoms

RHS often begins with a prodrome of pain, fever, and fatigue for 1 to 3 days. This is followed by the characteristic rash and unilateral facial paralysis, which worsens over the next week.

As previously noted, the diagnosis is made with the clinical triad of vesicles, otalgia, and ipsilateral facial paralysis. However, complicated anastomoses between cranial nerves such as V, IX, XI, and XII can allow herpetic lesions to travel further, causing additional symptoms. These can manifest as changes in taste, dry eye or tearing, nasal obstruction, or dysarthria.

Involvement of the vestibulocochlear nerve can cause hearing loss, tinnitus, hyperacusis, and/or vertigo. Vagal nerve involvement can lead to hoarseness or aspiration.

Treatment

Although herpes zoster is generally self-limited, early treatment can decrease the incidence of late complications, including spastic facial paralysis and postherpetic neuralgia.

The standard treatment of RHS involves antiviral medications such as acyclovir (Zovirax), famciclovir (Famvir), or valacyclovir (Valtrex) in conjunction with corticosteroids. Prompt diagnosis and treatment seems to have the greatest benefits, and it is recommended that treatment with antivirals begin as soon as possible.

The largest retrospective study of treatment of RHS by Murakami et al. showed a statistically significant improvement in patients treated with prednisone and acyclovir within 3 days of onset. A total of 80 patients were divided into three groups depending on when treatment was started: <3 days, 3 to 7 days, and >7 days. All received the same treatment regimen and were followed for 6 to 12 months with repeated clinical exams, nerve excitability testing, and audiograms in those who reported tinnitus or hearing loss.

According to the researchers: Complete recovery was seen in 21 (75%) patients treated within the first 3 days (P<0.05), 14 (48%) patients treated at 4 to 7 days, and seven (30%) when treatment was not started until after 7 days. Further, 26 (50%) patients who were not treated in the first 3 days progressed to a complete loss of response to facial nerve stimulation.

Additional medications can be helpful for specific symptoms of RHS. For example, pain relievers (even prescription strength) for pain, carbamazepine for neuralgia, and antianxiety medications such as diazepam for vertigo.

Because some patients with RHS are unable to fully close their affected eye, they must take special care to prevent corneal injuries. Artificial tears and lubrication ointments may be helpful in this regard.

Most patients complete their recovery within 1 year and often within several weeks to a few months when the paralysis is incomplete and the patient is young and healthy. Those patients who fail to recover premorbid facial nerve function are more likely to have synkinesis as a sequela.

Synkinesis is a condition of unwanted contraction of the muscles of the face during attempted movement. It typically does not go away on its own but can be improved with specialized physical therapy (facial neuromuscular training). For those resistant to training, injections of botulinum toxin can cause temporary improvement.

Prevention may be possible with chickenpox and shingles vaccines. VZV vaccine is currently recommended for children at age 12 to 15 months with a second dose at age 4 to 6 years. Those over 13 years old who have never had chickenpox or received chickenpox vaccine should get two doses, at least 28 days apart.

Shingles vaccine is recommended for those 50 or older. Two doses are also required and should be separated by 2 to 6 months. Those with weakened immune systems can get the second dose 1 to 2 months after the first.

Michele R. Berman, MD, is a pediatrician-turned-medical journalist. She trained at Johns Hopkins, Washington University in St. Louis, and St. Louis Children’s Hospital. Her mission is both journalistic and educational: to report on common diseases affecting uncommon people and summarize the evidence-based medicine behind the headlines.

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