Ramsay Hunt syndrome type 2

Ramsay Hunt syndrome (RHS) type 2
Classification and external resources
Specialty	infectious disease
ICD-10	B02.2 (ILDS B02.270), G53.0
ICD-9-CM	053.11
DiseasesDB	11176
MedlinePlus	001647
eMedicine	neuro/420
MeSH	D016697

Ramsay Hunt syndrome type 2, also known as herpes zoster oticus, is a disorder that is caused by the reactivation of varicella zoster virus in the geniculate ganglion, a nerve cell bundle of the facial nerve.^[1]

Ramsay Hunt syndrome type 2 typically presents with inability to move many facial muscles, pain in the ear, taste loss on the front of the tongue, dry eyes and mouth, and a vesicular rash.

Signs and symptoms

The symptoms and signs include acute facial nerve paralysis, pain in the ear, taste loss in the front two-thirds of the tongue, dry mouth and eyes, and an erythematous vesicular rash^[2] in the ear canal, the tongue, and/or hard palate.

Since the vestibulocochlear nerve is in proximity to the geniculate ganglion, it may also be affected, and patients may also suffer from tinnitus, hearing loss, and vertigo. Involvement of the trigeminal nerve can cause numbness of the face.

Pathophysiology

Ramsay Hunt syndrome type 2 refers to shingles of the geniculate ganglion. After initial infection, varicella zoster virus lies dormant in nerve cells in the body, where it is kept in check by the immune system. Given the opportunity, for example during an illness that suppresses the immune system, the virus travels to the end of the nerve cell, where it causes the symptoms described above.^[2]

The affected ganglion is responsible for the movements of facial muscles, the touch sensation of a part of ear and ear canal, the taste function of the frontal two-thirds of the tongue, and the moisturization of the eyes and the mouth. The syndrome specifically refers to the combination of this entity with weakness of the muscles activated by the facial nerve. In isolation, the latter is called Bell's Palsy.

However, as with shingles, the lack of lesions does not definitely exclude the existence of a herpes infection. Even before the eruption of vesicles, varicella zoster virus can be detected from the skin of the ear.^[3]

Prevention

Shingles is prevented by immunizing against the causal virus, varicella zoster, for example through Zostavax, a stronger version of chickenpox vaccine.

Treatment with the steroid prednisone and the antiviral drug acyclovir is controversial, with some studies showing to achieve complete recovery in patients if started within the first three days of facial paralysis,^[4] with chances of recovery decreasing as treatment was delayed. Delay of treatment may result in permanent facial nerve paralysis. However, some studies demonstrate that even when steroids are started promptly, only 22% of all patient achieve full recovery of facial paralysis

Treatment apparently has no effect on the recovery of hearing loss.^[5]

Diazepam is sometimes used to treat the vertigo.^[6]

History

The syndrome is named for James Ramsay Hunt, the eminent neurologist who first described it.^[7][8]

References

External links

• Ramsay Hunt syndrome at GPnotebook
• NINDS Herpes Zoster Oticus Information Page National Institute of Neurological Disorders and Stroke
• Ramsay-Hunt Syndrome. Support site.
• drtbalu otolaryngology on line
• Ramsay Hunt Syndrome Causes, Symptoms, Diagnoses
• Vaccine at Centers for Disease Control and Prevention
• MR scans Imaging Zoster Oticus