Ramsay Hunt syndrome type II

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


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).[1] 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.


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.[2]

Differential diagnosis

Diseases Clinical manifestations Para-clinical findings Gold standard Additional findings
Symptoms Physical examination
Lab Findings Imaging
Acute onset Recurrency Nystagmus Hearing problems
+ + +/−
Vestibular neuritis
+ +/− + /−


  • + Head thrust test
HSV oticus
+ +/− +/− + VZV antibody titres
Meniere disease
+/− + +/− + (Progressive)
Labyrinthine concussion
+ +
Perilymphatic fistula
+/− + +
  • CT scan may show fluid around the round window recess
Semicircular canal

dehiscence syndrome

+/− + +

(air-bone gaps on audiometry)

Vestibular paroxysmia
+ + +/−

(Induced by hyperventilation)

Cogan syndrome
+ +/− + Increased ESR and cryoglobulins
  • In CT scan we may see calcification or soft tissue attenuation obliterating the intralabyrinthine fluid spaces
Vestibular schwannoma
+ +/− +
Otitis media
+ +/− Increased acute phase reactants
Aminoglycoside toxicity
+ +
Recurrent vestibulopathy
  • It may happen infrequently, every one to two years
  • It may be associated with nausea and vomiting
  • It may overlap with vestibular migraine
Vestibular migrain
+ +/− +/−
  • ICHD-3 criteria
Epileptic vertigo
+ +/−
  • They response well to anti-seizure drugs
Multiple sclerosis
+ +/− Elevated concentration of CSF oligoclonal bands
  • 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
+/− + + + 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.
Cerebellar infarction/hemorrhage + ++/−
  • Based on the time interval between stroke and imaging we may have different presentations
Brain stem ischemia + +/−
  • Based on the time interval between stroke and imaging we may have different presentations
  • For more information click here
Chiari malformation
+ +
  • Patient may experience ringing in the ears


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



Physical Examination



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.[45] 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.


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