People who had chickenpox may have Ramsay Hunt syndrome later in life characterized by the Ramsay hunt triad. Ramsay hunt syndrome recovery depends on severity.

Ramsay Hunt Syndrome

Humza Khizer

Overview

Ramsay Hunt syndrome is a complication of shingles, which affects the facial nerve. The syndrome is caused by the varicella zoster virus (member of herpes virus group) that causes chickenpox. The infection results in the inflammation and irritation of the facial nerve.

People who develop Ramsay Hunt Syndrome have a history of chickenpox as a child and even though it heals, the virus remains latent in the body within the sensory nerve ganglia. However, the virus is harmless unless reactivated and symptoms appear. These symptoms are called RHS.

When shingles affect the nerves of the face, close to either side of the ear, it causes Ramsay Hunt Syndrome. The ear is affected by herpes zoster oticus virus in shingles. Moreover, the general varicella zoster also causes chickenpox, which is very common in children. For anyone who has had chickenpox in their childhood or throughout life, the virus can reactivate in the future and lead to shingles.

Shingles and chickenpox both are recognized by a rash, which appears on the body. Unlike chickenpox, the rashes of shingles are present near the facial nerve, which can also cause other complications. These complications include ear pain and facial nerve paralysis. This condition is called Ramsay Hunt Syndrome.

If anyone ever sees rashes on their face and notices any of the symptoms of facial nerve weakness, they should see their healthcare provider immediately. Early treatment can prevent complications from RHS. 
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<ul>
	<li>
	<h5>Ramsay Hunt Syndrome Type 1</h5>
	</li>
</ul>

It is a rare neurological disorder, and it can cause seizures, loss of body functions, tremors, and cognitive impairment. The other name for Ramsay Hunt Syndrome type 1 is Ramsay Hunt Cerebellar Syndrome.

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<ul>
	<li>
	<h5>Ramsay Hunt Syndrome Type 2</h5>
	</li>
</ul>

Three syndromes have been described for RHS. Ramsay Hunt Type 2 correlates with the condition the most. It is caused when the varicella-zoster virus is reactivated in the geniculate ganglion. Patients usually present with facial nerve paralysis, which leads to drooping, dry eyes (keratoconjunctivitis sicca), dry mouth (xerostomia), and hearing loss. Although the diagnosis is based on clinical symptoms, PCR of direct immunofluorescent assay analysis of the vesicular fluid can also aid in the diagnosis.&nbsp;Although Ramsay Hunt Syndrome 1 and 3 share a similar name, they are not linked with Ramsay Hunt Syndrome 2. 
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<ul>
	<li>
	<h5>Ramsay Hunt Syndrome Type 3</h5>
	</li>
</ul>

Ramsay Hunt Syndrome type 3 leads to neuropathy in the palm section of the ulnar nerve. The other names used for this condition are Hunt&rsquo;s disease and Artisan&rsquo;s palsy.

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Signs And Symptoms

The symptoms of Ramsay Hunt Syndrome are different for everyone. People who get affected usually experience facial nerve paralysis and rash on the affected side of the ear. However, people usually don&rsquo;t get both symptoms together, and only one side is affected in most cases.

Nerve palsy affects the facial muscles, which may feel stiff, resulting in a wrinkle on the forehead. The patient is unable to smile, and the face appears to develop a saggy appearance. Facial weakness is usually severe within the first week after the symptoms start. Asymmetric muscle tone is visibly evident, and some people also suffer from the drooping mouth and drool saliva. Also, irritation can occur when people are unable to close their eyes and in rare cases, the cornea can become damaged. Patients may also experience blurred vision.

Most patients who have Ramsay Hunt Syndrome have painful, erythematous (reddish), vesicular (fluid-filled) rashes along the pinna of the ear and the external ear canal. 80% of the patients with the syndrome have reported having vesicular rashes of the ear and mouth<a href="#accordion">[1]</a>. The rash and painful blisters can also affect the mouth, soft palate, eardrum, and upper portion of the throat on the side of the affected nerve. Additional symptoms include tinnitus (ringing of the ear) and otalgia (ear pain).

Furthermore, the pain is very intense in some patients, and individuals usually develop sensorineural hearing loss. In sensorineural hearing loss, the vibrations are not transmitted to the brain properly because of a defect in the inner ear or auditory nerve, which results in ipsilateral hearing loss i.e. hearing loss on the affected side.

Although hearing loss is temporary, it is seen in 47% of patients with Ramsay Hunt Syndrome<a href="#accordion">[2]</a>. Moreover, some of them may also have hyperacusis, in which the sounds feel louder than they are because of an abnormality in the stapedius muscle of the eardrum leading to tremendous discomfort. Pain spreads to the neck in some people as well.&nbsp;

Other symptoms include vomiting, nausea, and vertigo (the surroundings usually feel like they are spinning). 
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<h5>Rare Symptoms</h5>

In rare cases of Ramsay Hunt Syndrome, there is a change in the perception of taste, loss of vision because of eye damage from corneal ulcers or infections, abnormal facial movement reactions since the nerves grow back in the wrong muscles, post-herpetic neuralgia (persistent pain), and facial weakness.

It is very rare, but the virus can spread to other nerves in the brain or spinal cord, which can lead to confusion, limb weakness, drowsiness, and nerve pain.

RHS is not contagious, but if the varicella-zoster is reactivated, it can cause chickenpox in people who are not vaccinated and haven&rsquo;t gotten it before. Contact should be avoided with these people, especially for those who have a weak immune system, till the rashes start scabbing.

Facial palsy with evidence of varicella-zoster virus is also seen in some people with Ramsay Hunt Syndrome. However, there are no associated skin abnormalities, and they are referred to as zoster sine herpete.

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Causes

The cause of Ramsay Hunt Syndrome is the reactivation of the varicella-zoster virus in the facial nerve. The facial nerve is the VIIth cranial nerve, affecting the face, tongue, and inner ear. Varicella Zoster Virus (VZV) causes both shingles and chickenpox.

If someone has gotten chickenpox in their childhood, the virus remains dormant (inactive) in the nerves for years. When the previously inactive VZV reactivates, it causes shingles. The virus travels from the nerve fibers to the skin, which leads to the development of painful sores. The virus develops into RHS, affecting the facial nerve. Although, the reason why the virus involves the facial nerve is yet to be elucidated. 
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Diagnosis

Varicella zoster virus isolation is the conventional cell culture test, which also gives a definitive diagnosis. However, growing VZV in the cell culture is tough and can be a very slow process, limiting its clinical utility. The sensitivity of the culture is 30-40% and the specificity is 100%.

Healthcare providers often make a diagnosis based on the hallmark signs, for example, facial weakness, and blister-like rash. A nerve condition study may be performed for the determination of the extent of damage to the facial nerve, and the likelihood of recovery.

A sample is taken from the fluid in one of the blisters in the ear for diagnosis confirmation. They may also use a blood sample or tear sample. MRI can also reveal the swelling of the facial nerves, and scans also help in the determination of whether the virus has reached the nerves or the brain.

It is important to mention that Ramsay Hunt Syndrome is often misdiagnosed because many doctors usually do not encounter this condition in routine.&nbsp;

The diagnosis of Ramsay Hunt Syndrome is made through the patient&rsquo;s clinical history and physical exam. However, no routine testing is indicated. The rash, pain, and facial droop are pathognomonic.

Tzanck smear test of the vesicular fluid can be performed, and PCR analysis of the saliva, tears, and vesicular fluid can also be done. But they are not necessary. Moreover, MRI shows any inflammation present near the geniculate ganglia of the affected facial nerve. Although, MRI and CT both don&rsquo;t contribute to the diagnosis.&nbsp;

Vestibular testing, audiometry, and flexible fiberoptic laryngoscopy also help in the determination of whether the cranial nerve is involved. Furthermore, when the cause of acute facial paralysis is unknown clinical signs, serological studies, and imaging can help. However, in most cases, these symptoms are obvious<a href="#accordion">[1]</a>.

If available, electromyography and electroneuronography give a useful prognosis of the nerve damage, which is not very precise with a physical examination. However, it is usually predominant in cases of House-Brackmann grade VI paralysis. 
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Treatment

Immediate treatment of Ramsay Hunt Syndrome can ease the pain and risk of long-term complications. The following medications are given to patients: 
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<ul>
	<li>Antiviral Drugs -&nbsp;&nbsp;Acyclovir, famciclovir, and valacyclovir help in combating the chickenpox virus. 
	&nbsp;</li>
	<li>Corticosteroids -&nbsp;&nbsp;A regime of high doses of prednisone can boost the effects of antiviral drugs in Ramsay Hunt Syndrome. 
	&nbsp;</li>
	<li>Anti-<a href="https://www.merisehat.pk/disease/anxiety" target="_blank">Anxiety </a>Medications -&nbsp;&nbsp;Vertigo can be relieved with diazepam 
	.</li>
	<li>Pain Medications -&nbsp;&nbsp;Pain is associated with Ramsay Hunt Syndrome, and it can be severe. Patients may require prescription medications. 
	&nbsp;</li>
</ul>

The complications include the following:&nbsp; 
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Permanent Hearing Loss -&nbsp;The hearing loss that is associated with Ramsay Hunt Syndrome is temporary. However, if not treated properly, it can become permanent. 
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Eye Damage -&nbsp;Facial weakness due to RHS can cause difficulties in closing the eyelid, which can lead to damaged cornea. This damage can lead to blurred vision and pain. 
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Post-Herpetic Neuralgia -&nbsp;When shingles infection damages the nerve fibers, it can lead to post-herpetic neuralgia. The messages these nerve fibers send are hindered, which causes pain that lasts long even after other symptoms subside. 
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Anyone who has gotten chickenpox is at risk of RHS. It usually occurs in older adults and affects people over the age of sixty. Moreover, it is rare in children.

Although Ramsay Hunt Syndrome is not contagious, if the varicella-zoster virus is reactivated, it can cause chickenpox in people who have not gotten it before and are not vaccinated. In addition, the infection is serious for people who are immunocompromised. 
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What Can Be Done To Avoid It? 
If anyone in your surroundings has VZV, avoid contacting them till scabbing appears. Especially, if you&rsquo;ve never gotten vaccinated against it or gotten it in your life. People with weak immune systems, newborns, and pregnant women should also stay away. 
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The treatment of Ramsay Hunt Syndrome involves antiviral drugs and steroids. The protocol should be started within three days after the symptoms appear.&nbsp;

Varicella vaccine can be given to children, which can reduce the chance of getting chickenpox which causes the reactivation of the virus (RHS). However, if a person gets chickenpox, they are susceptible to reactivation, developing shingles, or Ramsay Hunt Syndrome.

Zostavax vaccine helps in the prevention of reactivation of the virus. This enables either prevention or reduction in the reactivation or symptoms of Ramsay Hunt Syndrome or Shingles. The vaccine is usually given to people who have had chickenpox as children, or at the age of sixty and older. CDC suggests that the vaccine should be given to people exposed to chickenpox because 20% of people are likely to get shingles without the vaccine.

People who have Ramsay Hunt Syndrome should take care to prevent corneal injury because it causes difficulty in closing their eyes. If this happens, it can expose the cornea to abnormal drying, and foreign bodies can irritate it. Healthcare providers give artificial lubricants and ointments for protecting the cornea. Moreover, some patients are also asked to wear an eye patch. 
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Key Points

<ul>
	<li>Varicella Zoster Virus causes Ramsay Hunt Syndrome.</li>
	<li>Patients with Ramsay Hunt syndrome have more severe paralysis compared to <a href="https://www.merisehat.pk/disease/bells-palsy" target="_blank">Bell&rsquo;s Palsy</a>.</li>
	<li>The two main symptoms are painful red rash with fluid filled blisters around or inside one ear.</li>
	<li>The treatment is through antivirals and steroids.</li>
	<li>Recovery takes a few weeks to several months.</li>
</ul>

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