Ramsay Hunt syndrome is defined as an acute peripheral facial neuropathy associated with erythematous vesicular rash of the skin of the ear canal, auricle (also termed herpes zoster oticus), and/or mucous membrane of the oropharynx.
This syndrome is also known as geniculate neuralgia or nervus intermedius neuralgia. Ramsay Hunt syndrome can also occur in the absence of a skin rash, condition known as zoster sine herpete.
Ramsay Hunt syndrome was first described in 1907 by James Ramsay Hunt in a patient who had otalgia associated with cutaneous and mucosal rashes, which he ascribed to infection of the geniculate ganglion by human herpesvirus 3 (ie,varicella-zoster virus [VZV]).
The following may be observed:
VZV in any of the zoster zones of the head and neck (herpes auricularis, herpes facialis, and herpes occipito-collairs) with facial palsy,
VZV in any of the zoster zones with facial palsy and auditory symptoms (eg, tinnitus, deafness, vertigo, nystagmus, ataxia).
Weakness on one side of the face that causes difficulty closing one eye, eating (food falls out of the weak corner of the mouth), making expressions, and making fine movements of the face, as well as facial droop and paralysis on one side of the face.
Infection by herpes zoster of facial nerve near ear is believed to be the main cause.
This leads to irritation and swelling of facial nerve and hence the symptoms.
At times our immune system becomes depressed and is less able to fight off infection. The body then becomes vulnerable to reactivation of the chickenpox virus.
Stress is often a trigger. Many studies have shown that stress can weaken the immune system, and that people under significant stress are more likely to suffer from infections than those who are not. For this reason, it is believed that stress can be linked to outbreaks of shingles, and thus RHS could result.
4 Making a Diagnosis
A diagnosis of Ramsay Hunt syndrome is often difficult because symptoms vary from person to person and symptoms of RHS are similar to other causes of facial weakness.
The following examinations and investigations may be used in the diagnosis of RHS:
Thorough history taking by your GP or doctor.
Examination of the facial nerve functions (such as eye closure).
A full physical examination including the ear.
Blood tests to measure the presence of the antibodies to the varicella zoster virus responsible for causing RHS.
Magnetic Resonance Imaging (MRI) to identify areas of inflammation along the track of the facial nerve and exclude any other cause of your symptoms.
Nerve conduction studies can assess facial nerve function. It is not diagnostic but indicates the degree of facial nerve damage.
Electromyography can also show if the facial nerve is damaged but again this cannot give a specific diagnosis or reason for the damage.
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