Ramsay Hunt syndrome type II

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]

Overview

Ramsay Hunt syndrome (RHS) type 2, is a disorder that is caused by the reactivation of pre-existing herpes zoster virus in a nerve cell bundle (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.

Pathophysiology

RHS type 2 is essentially shingles of the geniculate ganglion. Briefly, the herpes zoster virus, which causes chicken pox, lies dormant in various nerve cells in the body, where it is kept in check by the patient's immune system. 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.

Disease States to Differentiate Ramsay Hunt from

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.

Differential diagnosis

Diseases	Clinical manifestations					Para-clinical findings		Gold standard	Additional findings
	Symptoms				Physical examination
						Lab Findings	Imaging
	Acute onset	Recurrency	Nystagmus	Hearing problems
Peripheral
BPPV	+	+	+/−	−	+ Dix-Hallpike maneuver	−	−	Dix-Hallpike maneuver	May be associated with nausea, vomiting, and gait instability
Vestibular neuritis	+	+/−	+ /− (unilateral)	−	+ Head thrust test	−	−	History/ Physical exam	May be associated with nausea, vomiting, gait instability and previous upper respiratory infection
HSV oticus	+	+/−	−	+/−	Taste loss in the front two-thirds of the tongue Acute facial nerve paralysis Vesicles in the ear canal, the tongue, and/or hard palate	+ VZV antibody titres	In MRI with gadolinium dye we may have enhancement of the facial nerve and cranial nerve VIII	History/ Physical exam	May be associated with otalgia, dry mouth, and dry eyes
Meniere disease	+/−	+	+/−	+ (Progressive)	Sensorineural hearing loss	−	In CT scan we may see small or invisible vestibular aqueduct	History/ Physical exam/ Rulling out other diagnoses	May be associated with nausea, vomiting, and tinnitus
Labyrinthine concussion	+	−	−	+	high frequency hearing loss	−	We may see other evidences of head trauma or temporal bone fracture	History/ Physical exam	It happens following blunt head trauma May be associated with dizziness or tinnitus
Perilymphatic fistula	+/−	+	−	+	Tullio phenomenon	−	CT scan may show fluid around the round window recess	History/ Physical exam/Imaging	Can be a complication of a stapedectomy, head injury, or heavy lifting It may be provoked by sneezing, lifting, straining, coughing, and loud sounds
Semicircular canal dehiscence syndrome	+/−	+	−	+ (air-bone gaps on audiometry)	Tullio phenomenon	−	CT scan may show defect in the arcuate eminence of the superior semicircular canal	History/ Physical exam/Imaging	It may be provoked by Valsalva maneuver, coughing, and sneezing
Vestibular paroxysmia	+	+	+/− (Induced by hyperventilation)	−	Impaired caloric testing	−	We may see evidence of vestibulocochlear nerve compression on MRI	History/ Physical exam/Imaging	It may be provoked by head turn or other action They respond well to treatment with carbamazepine or oxcarbazepine
Cogan syndrome	−	+	+/−	+	Interstitial keratitis Oscillopsia Absent vestibular function on caloric test Systemic vasculitis (Aortitis)	Increased ESR and cryoglobulins	In CT scan we may see calcification or soft tissue attenuation obliterating the intralabyrinthine fluid spaces	History/ Physical exam	It may cause Ménière-like attacks
Vestibular schwannoma	−	+	+/−	+	Sensorineural hearing loss + Rinne test Lateralization of Weber test to the normal ear	−	In CT scan we may see erosion, and widening of the internal acoustic meatus Hypointense mass on T1-weighted MRI, and hyperintense mass on T2-weighted MRI	Imaging	Gadolinium-enhanced MRI scan is definitive diagnostic test of acoutic neuroma
Otitis media	+	−	−	+/−	Fever Presence of effusion in the middle ear	Increased acute phase reactants	Opacification of the middle ear	History/ Physical exam	Patient may show other signs and symptoms of upper respiratory infection such az cough, nasal discharge, and fever
Aminoglycoside toxicity	+	−	−	+	Oscillopsia	−	−	History/ Physical exam	May be associated with nausea, vomiting, and ataxia It may be irreversible Gentamicin is the most common one
Recurrent vestibulopathy	+	−	−	−	−	−	−	History/ Physical exam	The underlying pathophysiology is unknown It may happen infrequently, every one to two years It may be associated with nausea and vomiting It may overlap with vestibular migraine
Central
Vestibular migrain	–	+	+/−	+/−	History of migraine headaches	−	They may have white-matter hyperintensities (WMHs) on MRI	ICHD-3 criteria	It may be associated with anxiety and depression
Epileptic vertigo	−	+	+/−	−	They may experience loss of consciousness and motor/sensory problems	−	−	EEG	They response well to anti-seizure drugs
Multiple sclerosis	−	+	+/−	−	Lhermitte's sign Spasticity Increased reflexes Internuclear ophthalmoplegia Optic neuritis Gait disturbance	Elevated concentration of CSF oligoclonal bands	Brain atrophy and some contrast enhancing plaques on CT scan Cerebral plaques disseminating in space and time on MRI	History and physical examination Imaging CSF analysis	MS is at least two times more common among women than men The onset of symptoms is mostly between the age of fifteen to forty years, rarely before age fifteen or after age sixty
Brain tumors	+/−	+	+	+	Papilledema Focal neurological deficits	Cerebral spinal fluid (CSF) may show cancerous cells	On CT scan most of the brain tumors appears as a hypodense mass lesions On MRI most of the brain tumors appears as a hypointense or isointense on T1-weighted scans, or hyperintense on T2-weighted MRI.	Imaging Biopsy	Patieny may experience headache, seizures, visual changes and changes in personality, mood and concentration
Cerebellar infarction/hemorrhage	+	−	++/−	−	Limb ataxia Gait disturbance Dysarthria	−	Based on the time interval between stroke and imaging we may have different presentations	Imaging	Posterior inferior cerebellar artery is the most common artery that causes vertigo
Brain stem ischemia	+	−	+/−	−	Contralateral body weakness Visual field deficits Oculomotor abnormalities Bulbar findings	−	Based on the time interval between stroke and imaging we may have different presentations For more information click here	Imaging	It may be associated with subclavian steal syndrome
Chiari malformation	−	+	+	−	Tachycardia Pupillary dilatation Impaired gag reflex Impaired coordination	−	In CT scan we may see hydrocephalus, herniated cerebellar tonsils, and a flattened spinal cord In MRI we may see cerebellar tonsillar herniation, wedge shaped tonsils, syringohydromyelia, small posterior fossa, obstructive hydrocephalus, and brainstem anomalies	Imaging	Patient may experience ringing in the ears
Parkinson	−	+	−	−	Hypomimia Cogwheel rigidity Resting tremor Gait problems Bradykinesia	−	On brain CT scan, Parkinson disease is characterized by cortical and subcortical atrophy MRI findings in Parkinson disease are reduction in T2 relaxation time and reduced iron content in putamen and GPe	History and physical examination	Patients may present with slowness of movement (bradykinesia), shaking hands while they are at rest (resting tremor) and muscle stiffness (rigidity).

ABBREVIATIONS

VZV= Varicella zoster virus, MRI= Magnetic resonance imaging, ESR= Erythrocyte sedimentation rate, EEG= Electroencephalogram, CSF= Cerebrospinal fluid, GPe= Globus pallidus externa, ICHD= International Classification of Headache Disorders

Diagnosis

Symptoms

• Pain in the ear (otalgia)
• Taste loss in the front two-thirds of the tongue
• Dry mouth
• Dry eyes
• Tinnitus. 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.

Physical Examination

HEENT

• Acute facial nerve paralysis
• Vesicles in the ear canal, the tongue, and/or hard palate

Treatment

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 prednisone and acyclovir is started within the first 3 days of onset of symptoms. 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.

External links

• Template:NINDS
• Ramsay-Hunt Syndrome Ramsay-Hunt Syndrome support site
• Template:WhoNamedIt

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