Ramsay Hunt syndromes
Encyclopedia
Ramsay Hunt syndrome type 2 also known as herpes zoster oticus is a disorder that is caused by the reactivation of pre-existing herpes zoster
virus in a nerve cell bundle in the head (the geniculate ganglion
). The neurons in this ganglion are 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 entity would be called Bell's Palsy
.
, pain in the ear, taste loss in the front two-thirds of the tongue, dry mouth and eyes, and eruption of a erythematous vesicular rash
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
.
. Given the opportunity, for example during an illness that suppresses the immune system, the virus is reactivated and travels to the end of the nerve cell, where it causes the symptoms described above.
Like shingles, however, lack of lesions does not definitely exclude the existence of a herpes infection. The virus can be detected, even before the eruption of vesicles, from the skin of the ear.
and acyclovir is started within the first 3 days of onset of facial paralysis. Chances of complete recovery decrease as treatment is delayed. Studies have shown that half of all patients whose treatment was delayed had complete loss of response to facial nerve stimulation.
Treatment apparently has no effect on the recovery of hearing loss.
Diazepam
is sometimes used to treat the vertigo
.
Herpes zoster
Herpes zoster , commonly known as shingles and also known as zona, is a viral disease characterized by a painful skin rash with blisters in a limited area on one side of the body, often in a stripe...
virus in a nerve cell bundle in the head (the geniculate ganglion
Geniculate ganglion
The geniculate ganglion is an L-shaped collection of fibers and sensory neurons of the facial nerve located in the facial canal of the head...
). The neurons in this ganglion are 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
Facial weakness
Facial weakness is a medical sign associated with a variety of medical conditions.Some specific conditions associated with facial weakness include:* stroke* neurofibromatosis* Bell's palsy* Ramsay Hunt syndrome* Spontaneous cerebrospinal fluid leak...
. In isolation the latter entity would be called Bell's Palsy
Bell's palsy
Bell's palsy is a form of facial paralysis resulting from a dysfunction of the cranial nerve VII that results in the inability to control facial muscles on the affected side. Several conditions can cause facial paralysis, e.g., brain tumor, stroke, and Lyme disease. However, if no specific cause...
.
Symptoms and signs
The symptoms and signs include acute facial nerve paralysisAcute facial nerve paralysis
Facial nerve paralysis is a common problem that involves the paralysis of any structures innervated by the facial nerve. The pathway of the facial nerve is long and relatively convoluted, and so there are a number of causes that may result in facial nerve paralysis...
, pain in the ear, taste loss in the front two-thirds of the tongue, dry mouth and eyes, and eruption of a erythematous vesicular rash
Rash
A rash is a change of the skin which affects its color, appearance or texture. A rash may be localized in one part of the body, or affect all the skin. Rashes may cause the skin to change color, itch, become warm, bumpy, chapped, dry, cracked or blistered, swell and may be painful. The causes, and...
in the ear canal, the tongue, and/or hard palate
Hard palate
The hard palate is a thin horizontal bony plate of the skull, located in the roof of the mouth. It spans the arch formed by the upper teeth.It is formed by the palatine process of the maxilla and horizontal plate of palatine bone....
.
Since the vestibulocochlear nerve
Vestibulocochlear nerve
The vestibulocochlear nerve is the eighth of twelve cranial nerves, and is responsible for transmitting sound and equilibrium information from the inner ear to the brain...
is in proximity to the geniculate ganglion, it may also be affected, and patients may also suffer from tinnitus
Tinnitus
Tinnitus |ringing]]") is the perception of sound within the human ear in the absence of corresponding external sound.Tinnitus is not a disease, but a symptom that can result from a wide range of underlying causes: abnormally loud sounds in the ear canal for even the briefest period , ear...
, hearing loss, and vertigo
Vertigo (medical)
Vertigo is a type of dizziness, where there is a feeling of motion when one is stationary. The symptoms are due to a dysfunction of the vestibular system in the inner ear...
.
Pathophysiology
RHS type 2 is essentially shingles of the geniculate ganglion. Briefly, the herpes zoster virus lies dormant in various nerve cells in the body, where it is kept in check by the patient's immune systemImmune system
An immune system is a system of biological structures and processes within an organism that protects against disease by identifying and killing pathogens and tumor cells. It detects a wide variety of agents, from viruses to parasitic worms, and needs to distinguish them from the organism's own...
. Given the opportunity, for example during an illness that suppresses the immune system, the virus is reactivated and travels to the end of the nerve cell, where it causes the symptoms described above.
Like shingles, however, lack of lesions does not definitely exclude the existence of a herpes infection. The virus can be detected, even before the eruption of vesicles, from the skin of the ear.
Prognosis
The largest study on the treatment of RHS type 2 has shown that complete recovery can be achieved in 75% of patients if treatment with prednisonePrednisone
Prednisone is a synthetic corticosteroid drug that is particularly effective as an immunosuppressant drug. It is used to treat certain inflammatory diseases and some types of cancer, but has significant adverse effects...
and acyclovir is started within the first 3 days of onset of facial paralysis. Chances of complete recovery decrease as treatment is delayed. Studies have shown that half of all patients whose treatment was delayed had complete loss of response to facial nerve stimulation.
Treatment apparently has no effect on the recovery of hearing loss.
Diazepam
Diazepam
Diazepam , first marketed as Valium by Hoffmann-La Roche is a benzodiazepine drug. Diazepam is also marketed in Australia as Antenex. It is commonly used for treating anxiety, insomnia, seizures including status epilepticus, muscle spasms , restless legs syndrome, alcohol withdrawal,...
is sometimes used to treat the vertigo
Vertigo (medical)
Vertigo is a type of dizziness, where there is a feeling of motion when one is stationary. The symptoms are due to a dysfunction of the vestibular system in the inner ear...
.
External links
- 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