Beyond shingles - Ramsay Hunt Syndrome

Many people are aware of shingles - the painful rash that can develop years later after having chickenpox. But there are other forms of this reactivation of the varicella zoster virus (also known as human alphaherpesvirus 3 [HHV3]). 

Herpes zoster activation on the nerve from shingles to postherpetic neuralgia. Source

 

Ramsay Hunt syndrome is a painful recurrence of varicella zoster infection, the viral infection that causes chicken pox. When the virus infects a facial nerve in the inner ear, it causes a painful rash around the ear, called herpes oticus, which is referred to as Ramsay Hunt, or just Hunt, syndrome. A similar recurrence in the skin is known as shingles. The re-activated viral infection causes the nerve to become irritated and swell.

Symptoms of Ramsay Hunt syndrome

The most obvious symptom is the painful rash, which can occur on the eardrum, in the ear canal, or on the earlobe, as well as the tongue and roof of the mouth on the same side as the affected ear. Other ear-related symptoms include vertigo, which is a sensation that the room is spinning (similar to extreme dizziness), and hearing loss on the affected side. Another major symptom is weakness on one side of the face, which can manifest as difficulty closing the eye on the affected side, difficulties with the corner of the mouth on that side (such as trouble closing the mouth for chewing while eating or smiling), difficulties making facial expressions and facial droop, or facial paralysis and difficulty making finer facial movements. In the presence of these symptoms, a doctor can test the blood or perform a skin test for varicella zoster virus, test conduction along the facial nerve to determine the extent of damage, and imaging tests to ensure no other cause in the head.

How Ramsay Hunt syndrome is treated

Ramsay Hunt syndrome is treated pharmaceutically to alleviate the symptoms. The best responses are achieved when treatment begins within 3 days of symptoms appearing. The cause is a virus, so antibiotics have no effect, but antiviral drugs may have some effect. The drugs that may be prescribed include acyclovir and valacyclovir, and they may be recommended to be used for a week or more. To reduce the rash, strong anti-inflammatory drugs are used, specifically steroids such as prednisone. They are usually given for about a week. Other treatments include:

-An eye patch to protect the eye on the weak side and avoid corneal damage

-Painkillers for continuing pain during steroid treatment

-Anti-emetic drugs for vertigo

Delayed treatment can increase the risk of complications, which includes disfigurement. Other reasons for timely treatment are complications from extensive nerve damage. The damaged nerve may grow back in the wrong area, known as synkinesis, which can cause tears or blinking at inappropriate stimuli, such as chewing. Other complications include postherpetic neuralgia, changes to taste, and spread of the infection to other nerves.