All You Need To Know About Ramsay Hunt Syndrome

The Ramsay Hunt syndrome has been in the news lately after some high profile cases. This article outline All You Need To Know About Ramsay Hunt Syndrome.

Ramsay Hunt syndrome (RHS) is a rare neurological disorder characterized by paralysis of the facial nerve (facial palsy) and a rash affecting the ear or mouth. Ear abnormalities such as ringing in the ears (tinnitus) and hearing loss may also be present.

Ramsay Hunt syndrome is caused by the varicella zoster virus (VZV), the same virus that causes chickenpox in children and shingles (herpes zoster) in adults.

The disorder is named after James Ramsay Hunt, a physician who first described the disorder in the 1900s. However, some physicians use herpes zostic oticus only for the ear rash and Ramsay Hunt syndrome for the combination of ear rash and facial paralysis.


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Signs & Symptoms

The symptoms of Ramsay Hunt syndrome vary from case to case. Affected individuals usually experience paralysis (palsy) of the facial nerve and a rash affecting the ear. These two symptoms do not always occur simultaneously.

Facial muscles affected by nerve palsy may be weak or feel stiff and may result in the inability of affected individuals to smile, wrinkle the forehead or close their eye on the affected side. In some cases, speech may become slurred.

Most cases of Ramsay Hunt syndrome have a reddish (erythematous), painful, blistering (vesicular) rash that affects the outer portion of the ear (pinna) and often the external ear canal. In some cases, the rash, including painful blisters, may also affect the mouth, soft palate, and top portion of the throat.

Some individuals with Ramsay Hunt syndrome may have facial palsy with evidence of varicella-zoster virus through testing (e.g., blood tests), but without the associated skin abnormalities.

Additional symptoms affecting the ear include ringing in the ear (tinnitus) and ear pain (otalgia). In some cases, ear pain may be intense. Pain may spread to affect the neck. Hearing loss is usually temporary (transient), however, in rare cases it may become permanent.


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In some cases, affected individuals may experience hyperacusis, a condition in which sounds appear louder (often dramatically) than normal. This can cause tremendous discomfort for affected individuals.

Additional symptoms that may be present include nausea, vomiting, and a sensation that one’s surroundings are spinning (vertigo). In rare cases, loss of taste, dry mouth, and dry eyes may also occur.

Diagnosis

A diagnosis of Ramsay Hunt syndrome is based on thorough clinical evaluation and a detailed patient history and identification of characteristic symptoms. Viral studies can detect varicella-zoster virus in saliva, tears, and blood but are not necessary to establish a diagnosis of Ramsay Hunt syndrome.

The diagnosis of Ramsay Hunt syndrome can be difficult because the specific symptoms of the disorder (otalgia, facial paralysis and the distinctive rash) do not always develop at the same time.

Causes

Ramsay Hunt syndrome is caused by the varicella-zoster virus, which is the same virus that causes chickenpox and shingles. The virus can remain dormant for decades in a person who has had chickenpox as a child.

Reactivation of the varicella-zoster virus results in shingles and, in some cases, develops into Ramsay Hunt syndrome. The reason why the virus reactivates and affects the facial nerve in Ramsay Hunt syndrome is unknown.

Affected Populations

Ramsay Hunt syndrome affects males and females in equal numbers. According to one estimate, 5 out of every 100,000 people develop the syndrome each year in the United States.

The disorder is the second most common cause of atraumatic peripheral facial paralysis. Some researchers believe cases of the syndrome go undiagnosed or misdiagnosed making it difficult to determine the disorder’s true frequency in the general population.

Anyone who has had chickenpox can potentially develop Ramsay Hunt syndrome. However, most cases affect older adults, especially those over 60. Cases affecting children is extremely rare.

Related Disorders

Symptoms of the following disorders can be similar to those of Ramsay Hunt syndrome.

Bell’s palsy is a non-progressive neurological disorder of the facial nerve .This disorder is characterized by the sudden onset of facial paralysis that may be preceded by a slight fever, pain behind the ear on the affected side, a stiff neck, and weakness and/or stiffness on one side of the face.  There may also be an inherited tendency toward developing Bell’s palsy.

Acoustic neuroma is a benign tumor of the 8th cranial nerve. This nerve lies within the internal ear canal. Pressure on this nerve results in the early symptoms of acoustic neuroma; a ringing sound in the ear (tinnitus), and/or hearing loss may occur.

An associated compression of the facial nerve may produce muscle weakness; pressure on the trigeminal nerve  may lead to facial numbness.

The expansion of the tumor into different areas may result in impaired ability to coordinate movement of the legs and arms (ataxia), numbness in the mouth, slurred speech (dysphagia), and/or hoarseness.

 

Treatment

The treatment of Ramsay Hunt syndrome commonly involves antiviral medications, like acyclovir or famciclovir, in conjunction with corticosteroids, like prednisone.

Most authorities agree that beginning antiviral treatment within three days of onset appears to have the greatest benefit as prompt diagnosis and management seems to improve outcomes. Despite therapy, some degree of facial paralysis and hearing loss may become permanent in some cases.

This includes pain medication, carbamazepine, an anti-seizure medicine which may help reduce neuralgic pain, and vertigo suppressants like antihistamines and anticholinergics.

Individuals with the syndrome need to take special care to prevent corneal injury. The inability to properly close the eye can expose the cornea to abnormal drying and foreign body irritation.

Artificial tears and lubricating ointments may be prescribed to protect the cornea. Intake of fruits, vegetables and herbs can also help boost the immune system in combating the virus.

This article has outlined the diagnosis, symptoms and treatment for the rare syndrome, to give readers a more in depth understanding and to create awareness.