All You Need to Know about Ramsay Hunt Syndrome

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With a half smile and an unblinking eye, Canadian pop star Justin Bieber shared with his followers a few days ago a new video in which he announced that he was diagnosed with Ramsay Hunt Syndrome, caused by a virus that attacked his ear nerve, inducing facial paralysis. What is Ramsay Hunt Syndrome? What is the virus causing it?

Ramsay Hunt Syndrome (RHS) is a viral infection caused by the Varicella-zoster virus (VZV); the same virus that causes the common childhood illness Varicella (chickenpox). The virus remains in the body even after the illness is cured, and may reactivate during adulthood targeting a group of sensory neurons of the facial nerve near the ear known as the geniculate ganglion.

Some researchers believe that this condition is often misdiagnosed, making it difficult to determine the extent of the disease. A report indicates that the disease affects five out of every 100,000 persons in the USA only annually. Anyone who has had chickenpox can get infected by RHS, which affects both males and females; the syndrome typically affects adults over 60 years old and can rarely affect children.

The name comes from the American neurologist James Ramsay Hunt, who is best known for describing three neurological syndromes that bear his name too; he first described Bieber's disorder, type 2, in 1907.

Source: researchgate.net

The most prominent symptoms include a red rash with inflamed blisters on, in, or around one ear, as well as facial weakness or paralysis on the same side as the affected ear. These symptoms do not always develop at the same time; one may appear before the other, and sometimes a rash may not occur. Less common symptoms include earache—painful blisters around the ear and inside the auditory canal, the eardrum, and sometimes the tongue and the roof of the mouth—hearing loss, tinnitus, dizziness, dry mouth and eyes, difficulty in closing one eye, and a change in or loss of taste sensation.

In most cases, the symptoms are temporary, but they may become permanent. Immediate treatment of RHS within three days of early indications and symptoms can reduce long-term hazardous complications that may include: permanent weakness of facial muscles, hearing loss, eye pain, and trouble seeing due to corneal infections as closing the eyelid becomes difficult. This may damage the nerve fibers, disrupting the nerve messages that the nerve cells exchange, causing pain that may linger very long. The infection can also be more serious if the patient has a weakened immune system.

RHS cannot be transmitted from one person to another; it is not contagious. However, the infected person can infect other people who have never had chickenpox, or those who were not vaccinated against the disease, as children. Patients, thus, should avoid contact with these people and with anyone who has a weakened immune system, until the rash clears.

There is no specific vaccine for this condition, and the most common treatments are generic antiviral drugs and symptomatic medications, such as painkillers and medications that reduce dizziness, as well as corneal protection ointments.

References

instagram.com

mayoclinic.org

mountsinai.org

rarediseases.org

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