Ramsay Hunt syndrome is a neurological disease that causes vesicles to appear on the skin around the ear canal, as well as facial paralysis, ear pain and other characteristic signs. 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 so, we will place special emphasis on the alterations of the nervous system with which this disease is related, since they are a fundamental aspect in its understanding.

What is Ramsay Hunt syndrome?

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

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

Although this is a relatively rare diagnosis, it is estimated that Ramsay Hunt syndrome is the cause of between 16 and 18% of all unilateral facial paralyses . Its association with Bell’s palsy (i.e., any paralysis caused by problems in the facial nerve) is particularly significant.

Ramsay Hunt syndrome does not usually carry a risk of death; however, the muscular alterations that characterize it can interfere very markedly in the lives of those who suffer it. Less than half of those affected recover completely from their symptoms.

Causes of this disease

This disease occurs as a result of the reactivation of the varicella-zoster virus in the geniculated ganglion , which is located in the facial nerve, the seventh cranial pair. 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 flavours on the tongue.

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

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

It is important to note that symptoms occur in only one half of the head, since the virus most commonly affects only one of the two facial nerves.

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 in making movements with the mouth, such as those needed to eat, or to close one of the eyelids, among other aspects.

It is also very common to see rashes consisting of fluid-filled vesicles , mainly on the palate, tongue and ear canal, both externally and internally. Other common symptoms are loss of taste on the outside of the tongue and dry mouth and eyes.

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

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

Treatment and handling

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

When the pain is very intense and requires specific treatment, pain killers can be administered. Sometimes very powerful drugs are prescribed as the discomfort can also be very intense.

It has been found that l he hearing and mobility losses in the muscles of the face are largely maintained even if treatment is applied early and properly.

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