Ramsay Hunt Syndrome: a rare presentation of shingles

Caused by reactivation of the virus that causes chickenpox, Varicella Zoster Virus

Jun 16, 2022

Last week the world learned about another rare condition - Ramsay Hunt Syndrome - after Justin Bieber announced on his social media that he has been diagnosed with it. But before a ton of misinformation starts to spread (might already be too late, oops), we wanted to present some information about the condition.

Ramsay Hunt Syndrome, also known as herpes zoster oticus, is a rare condition caused by the reactivation of Varicella-Zoster Virus (VZV) in people who have previously had chickenpox. VZV is the virus that causes chickenpox in (mostly) children and shingles (a.k.a. herpes zoster) in adults. In order to develop shingles later in life, you must have been infected with VZV previously.

VZV is in the Herpesvirus family, which are a large family of DNA viruses. One of the unique features of this family of viruses is they can establish latency in our bodies. That means that after primary infection and illness, the virus is not cleared from our bodies by our immune system, but rather is able to hide out within specific cell types in our bodies and exist within our cells. In the case of VZV, these cells happen to be peripheral neurons. In the case of another herpesvirus, such as Herpes Simplex Virus, those cells are epithelial cells (a type of skin cell).

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When these viruses become reactivated after primary illness, symptoms present according to the cell types the virus lives inside. Reactivation of VZV can cause shingles (secondary infection) later in life (only if you previously had chickenpox, the primary infection). Since VZV resides in peripheral neurons, this is why shingles typically presents as a hemispherical blistering rash on the torso, arms, or face, and can be quite painful and debilitating, and potentially even fatal.

In the case of Ramsay Hunt Syndrome, this is a rare neurological complication of shingles. It affects less than 1% of those with herpes zoster (shingles). It occurs when a shingles outbreak affects the facial nerve near one of your ears and can cause facial paralysis, ear pain, hearing impairment, vertigo, tinnitus, and rash in and around the ear, mouth, and face. However, it is the second most common cause of facial paralysis (with Bell’s palsy being #1), and typically affected about 5/100,000 people every year. It is most commonly observed in individuals 60 and older, and is extremely rare in children.

COVID-19 vaccines, or other vaccines, are not the cause of shingles. Previous infection with VZV is the cause of shingles/VZV reactivation, but certain stressors on the immune system can lead to reactivation of VZV. These include stress, weakened immune system, and use of immunosuppressive drugs (e.g. cancer patients, organ transplant recipients, autoimmune diseases, untreated HIV infection).

Ramsay Hunt Syndrome (RHS) can be challenging to diagnose because the symptoms don’t always occur simultaneously. Some cases do not require treatment, but if needed, antivirals and corticosteroids are prescribed and should be taken within 3 days of symptom onset for the best outcome. Everyone recovers from RHS, but to varying degrees. Most people make a full recovery, although in rare instances, some degree of facial paralysis and/or hearing loss can be permanent. It’s important to note that Bell’s palsy has a poorer prognosis than RHS.

The good news is that we can prevent this through vaccination. Indeed, if we prevent initial infection with VZV, you prevent recurrence and reactivation as shingles later in later. A vaccine has been available since 1995 that significantly reduces the chances of getting chickenpox. It has over 90% effectiveness in preventing VZV infection.

Even if you have previously gotten infected with VZV and had chickenpox when you were younger, there is a shingles vaccine as well. The shingles vaccine is recommended for adults ages 50+ (and those ages 19+ who have compromised immune system) to prevent shingles and its complications. Even if you already had shingles or received the chickenpox vaccine, you should still get the shingles vaccine, as this prevents and reduces your chance of having a recurrence of VZV later in life.

Additional contributors:

Ilze Abersone, MS

Veda Bojar