Information on the following diseases can be found in the Related Disorders section of this report:

• None

The symptoms of Ramsay-Hunt syndrome vary from case to case. Affected individuals usually experience paralysis (palsy) of certain facial nerves and a rash affecting the ear (herpes zoster oticus). These two symptoms do not always occur simultaneously. In most cases, only one side of the face is affected (unilateral).

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 of the face. In some cases, speech may become slurred.

Most cases of Ramsay-Hunt syndrome have a reddish (erythematous), painful, blistering 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. These cases may be referred to as Ramsay-Hunt syndrome zoster sine herpete.

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. Some affected individuals develop sensorineural hearing loss, a condition in which sound vibrations are not properly transmitted to the brain due to a defect of the inner ear or the auditory nerve, resulting in hearing loss. Hearing loss is usually temporary (transient), however, in rare cases it may become permanent.

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.

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.

Ramsay-Hunt syndrome affects males and females in equal numbers. According to one estimate, 5 out of every 100,000 people develop Ramsay-Hunt syndrome each year in the United States. The disorder is the second most common cause of atraumatic peripheral facial paralysis.

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

Bell's palsy is a nonprogressive neurological disorder of one of the facial nerves (7th cranial 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. Paralysis results from decreased blood supply (ischemia) and/or compression of the 7th cranial nerve. The exact cause of Bell's palsy is not known. Viral and immune disorders are frequently implicated as a cause for this disorder. There may also be an inherited tendency toward developing Bell's palsy. (For more information on this disorder, choose "Bell’s palsy" as your search term in the Rare Disease Database.)

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 (7th cranial nerve) may produce muscle weakness; pressure on the trigeminal nerve (5th cranial 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. (For more information in this disorder, choose "acoustic neuroma" as your search term on the Rare Disease Database.)

Trigeminal neuralgia, also known as tic douloureux, is a disorder of the fifth cranial nerve (trigeminal nerve) characterized by attacks of intense, stabbing pain affecting the mouth, cheek, nose, and/or other areas on one side of the face. The exact cause of trigeminal neuralgia is not fully understood. (For more information on this disorder, choose "trigeminal neuralgia" as your search term in the Rare Disease Database.)

Diagnosis
A diagnosis of Ramsay-Hunt syndrome is suspected based upon a thorough clinical evaluation, a detailed patient history and identification of characteristic symptoms (i.e., facial palsy and rash). Blood tests that detect herpes zoster viral DNA and/or increasing levels of antibodies to herpes zoster virus can confirm a diagnosis of Ramsay-Hunt syndrome.

Treatment
The treatment of Ramsay-Hunt syndrome is directed toward the specific symptoms that are apparent in each individual. Some individuals may not require any treatment. Others may receive antiviral medications such as acyclovir or famciclovir in conjunction with corticosteriods (e.g., prednisone). These antiviral drugs have become the standard therapy for varicella-zoster. Most authorities agree that beginning antiviral treatment within three to five days appears to be beneficial.

Additional therapy may include anti-seizure medicine (anticonvulsants) such as carbamazepine, which may help reduce neuralgic pain.

Information on current clinical trials is posted on the Internet at www.clinicaltrials.gov. All studies receiving U.S. government funding, and some supported by private industry, are posted on this government web site.

For information about clinical trials being conducted at the NIH Clinical Center in Bethesda, MD, contact the NIH Patient Recruitment Office:

Tollfree: (800) 411-1222
TTY: (866) 411-1010
Email: prpl@cc.nih.gov

TEXTBOOKS
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Rowland LP, ed. Merritt’s Neurology. 10th ed. Lippincott Williams & Wilkins. Philadelphia, PA. 2000:441.

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Kuhweide R, Van de Steene V, Vlaminck S, Casselman JW. Ramsay Hunt syndrome: pathophysiology of cochleovestibular symptoms. J Laryngol Otol. 2002;116:844-8.

Xanthopoulos J, Noussios G, Papaioannides D, Exarchakos G, Assimakipoulos D. Ramsay Hunt syndrome presenting as a cranial polyneuropathy. Acta Otorhinolargyngol Belg. 2002;56:319-23.

Grose C, Bonthius D, Afifi AK. Chickenpox in the geniculate ganglion: facial nerve palsy, Ramsay-Hunt syndrome and acyclovir treatment. Pediatr Infect Dis J. 2002;21:615-7.

Kaberos A, Balatsouras DG, Korres SG, Kandiloros D, Economou C. Audiological assessment in Ramsay Hunt syndrome. Ann Otol Rhinol Laryngol. 2002;111:68-76.

Sweeney CJ, Gilden DH. Ramsay Hunt syndrome. J Neurol Neurosurg Psychiatry. 2001;71:149-54.

Kinishi M, Amatsu M, Mohri M, et al., Acyclovir improves recovery rate of facial nerve palsy in patients with Ramsay Hunt syndrome. Auris Nasus Larynx. 2001;28:223-6.

Morrow MJ. Bell’s palsy and herpes zoster oticus. Curr Treat Options Neurol. 2000;2:407-16.

Aframian D, Ben-Oliel R, Sharav Y. Ramsay Hunt syndrome – differential diagnosis, pathogenesis, and therapy. Harefuah. 1999;136:278-80, 339.

Murakami S, Hato N, Horiuchi J, et al., Clinical features and prognosis of facial palsy and hearing loss in patients with Ramsay Hunt syndrome. Nippon Jibiinkoko Gakkai Kaiho. 1996;99:1772-9.

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FROM THE INTERNET
National Institute of Neurological Disorders and Stoke. Ramsay-Hunt syndrome Information Page. February 9, 2005. Available at: http://www.ninds.nih.gov/disorders/ramsay2/ramsay2.htm Accessed On: May 27, 2005.

Awasthi D. Ramsay Hunt Syndrome. eMedicine Journal. 2005;3:9pp. Available at: http://www.emedicine.com/neuro/topic420.htm Accessed On: May 27, 2005.

Hain TC. Ramsay-Hunt syndrome. Neurology. Northwestern University Medical School. Available at: http://www.neuro.nwu.edu/meded/CRANIAL/ramseyhunt.html Accessed On: May 27, 2005.