Ramsay Hunt syndrome: causes, symptoms and treatment

Ramsay Hunt syndrome is a neurological disease that causes the appearance of vesicles on the skin around the ear canal, as well as facial paralysis, ear pain, and other signs characteristic. It is associated with infection of a region of the facial nerve by the varicella-zoster virus.

In this article we will describe in detail the causes, symptoms and treatment of Ramsay Hunt syndrome. To do this, we will place special emphasis on the nervous system disorders with which this disease is related, since they are a fundamental aspect in its understanding.

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What is Ramsay Hunt syndrome?

Ramsay Hunt syndrome, also known as “herpes zoster oticus”, “geniculate neuralgia” and “nerve intermediary neuralgia”is a neurological disorder characterized by the appearance of erythematous eruptions vesicular on the skin, mainly in areas close to the ear canal, as well as other signs related.

The disease was described by the neurologist James Ramsay Hunt in 1907. This expert attributed the appearance of the distinctive eruptions to infection of the geniculate ganglion by the varicella-zoster virus.

Although it is a relatively rare diagnosis, it is estimated that Ramsay Hunt syndrome is the cause of between 16 and 18% of all unilateral facial palsies. Its association with Bell's palsy (that is, anything caused by problems with the facial nerve) is particularly significant.

Ramsay Hunt syndrome is not usually life-threatening; However, the muscular alterations that characterize it can interfere in a very marked way in the life of those who suffer from it. Likewise, less than half of the affected people recover completely from the symptoms.

Causes of this disease

This disease occurs as a result of Varicella-zoster virus reactivation in the geniculate ganglion, which is located in the facial nerve, the seventh cranial nerve. This nerve controls many of the movements of the face, such as those involved in facial expressions and chewing, as well as the perception of flavors on the tongue.

After infection, the varicella-zoster virus remains inactive in nerve cells thanks to the action of the immune system. However, if the virus manages to reach the geniculate ganglion of the facial nerve, it interferes with the functioning of this, causing the symptoms that we will describe in the following section, including vesicular eruptions.

Occasionally other cranial nerves are affected as well., in particular the fifth (trigeminal), the sixth (abducens or external oculomotor), the eighth (vestibulocochlear) and the ninth (glossopharyngeal). When this happens, signs related to the functions of the nerve in question appear.

It is important to bear in mind that the symptoms occur only in one half of the head, since the most normal thing is that the virus affects only one of the two facial nerves.

• Related article: "Cranial nerves: the 12 nerves that exit the brain"

Main symptoms and signs

One of the most characteristic and useful signs for the diagnosis of Ramsay-Hunt syndrome is the presence of a partial or complete paralysis of many muscles of the face. This is manifested through the difficulty to make movements with the mouth, such as those that are necessary to eat, or to close one of the eyelids, among other aspects.

It is also very common for them to appear eruptions consisting of fluid-filled vesicles, mainly on the palate, on the tongue and in the auditory canal, both externally and internally. Other common symptoms are loss of taste on the outside of the tongue and dry mouth and eyes.

Since neurological lesions are located near the vestibulocochlear nerve, which transmits information related to sound and balance from the inner ear to the brain cortex, it is also common for ear pain to appear, loss of hearing capacity (hearing loss), dizziness, vertigo and tinnitus (perception of sounds such as buzzing and ringing).

Sometimes the involvement of the geniculate ganglion by the varicella-zoster virus does not cause the appearance of distinctive vesicles but pain, facial muscle paralysis, and other symptoms associates. When this happens, the term “zoster sine herpete” is used.

Treatment and management

The most common way to treat the infection that causes Ramsay Hunt syndrome is by administering steroid anti-inflammatories, including prednisone. Antiviral drugs such as acyclovir are also often prescribed.

When the pain is very intense and requires specific treatment, analgesics can be administered. Sometimes very powerful drugs are prescribed since the discomfort can also have a very high intensity.

It has been found that heLoss of hearing and mobility in the muscles of the face they are largely maintained even if treatment is applied early and appropriately.

In any case, in a large number of countries vaccination against the varicella-zoster virus is mandatory; this makes Ramsay Hunt syndrome tend to be rare in much of the world.