Ramsay Hunt Syndrome Type 2

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 by Bayan Al Othman, MD on June 30, 2020.



Ramsay Hunt syndrome type 2 is caused by reactivation of varicella zoster virus in the geniculate ganglion. Patient often presents with facial nerve paralysis leading to facial droop, dry eyes, mouth and hearing loss. Diagnostic is largely clinical but PCR of direct immunofluorescent assay analysis of the vesicular fluid can be helpful in aiding diagnosis. Treatment includes methylprednisolone and acyclovir to achieve high rate of complete recovery.

Disease Entity

Ramsay Hunt syndrome type 2 is also known as herpes zoster oticus because reactivation of varicella zoster virus in the geniculate ganglion is what causes this disease. The geniculate ganglion is a nerve cell bundle for facial nerve (CN VII), the inflammatory response caused by reactivated virus can lead to lower motor neuron lesions of the facial nerve, leading to paralysis of facial muscles that can cause dry mouth, eyes and hearing loss.

Ramsay Hunt Syndrome Type 2
DiseaseDB 11176    
ICD-10 B02.2 (ILDS B02.270_, G53.0
ICD-9-CM 053.11
OMIM 159700
MeSH D016697
MedlinePlus 001647

Disease

Ramsay Hunt syndrome type 2 is also known as herpes zoster oticus because reactivation of varicella zoster virus in the geniculate ganglion is what causes this disease. Ramsay Hunt Syndrome type 2 can account for 12% of all facial palsies and have a worse symptom and prognosis when compared to Bell’s palsy.[1] The geniculate ganglion is a nerve cell bundle for facial nerve (CN VII), the inflammatory response caused by reactivated virus can lead to lower motor neuron lesions of the facial nerve, leading to paralysis of facial muscles that can cause dry mouth, eyes and hearing loss.

Etiology

Ramsay Hunt syndrome type 2 is caused by reactivation of varicella zoster virus in the geniculate ganglion.

Risk Factors

Immunocompromised state, lack of varicella vaccination.

Pathophysiology

Reactivation of varicella-zoster virus involving the geniculate ganglion is the main cause of Ramsay Hunt Syndrome Type 2. There are three theories on the pathophysiology of the polyneuropathy nature of Ramsay Hunt Syndrome. The hearing loss and vertigo are attributed to the close proximity of Vestibulocochlear nerve (CN VIII) to Facial nerve (CN VII) at the cerebellopontine angle. Alternatively, the virus could travel via vasa vasorum to nearby cranial nerves. Finally, varicella-zoster virus could spread anterograde through the intersynaptic transmission of the brainstem reflex pathway.[2]

Primary prevention

Varicella vaccination with 2 doses of varicella vaccine 4 to 8 weeks apart. If previously vaccinated, the second dose should be 4 weeks after the initial injection.

Diagnosis

Ramsay Hunt Syndrome Type 2 can be diagnosed based on clinical features, however, in ambiguous cases PCR or direct immunofluorescent assay of vesicular fluid can help with the diagnosis. Laboratory studies such as WBC count, ERS, and electrolytes should be obtained to distinguish infectious versus inflammatory etiologies.

History

Patients often complain of severe otalgia with painful blisters on face around ear, mouth, or tongue. Other symptoms include vertigo, nausea, vomiting, hearing loss, hyperacusis, tinnitus, eye pain and lacrimation.

Physical examination

Vesicular exanthema on the external auditory canal, concha and or pinna. Dry eyes with possible lower cornea epithelium damage due to incomplete closure of eye lids.

Signs

  • Vertigo
  • Sensorineural hearing loss and paralysis of facial nerve
  • Inability to raise ipsilateral eyebrows
  • Dysgeusia
  • Inability to close ipsilateral eye leading to drying and irritation of cornea.


The degree of nerve palsy can be graded based on House-Brackmann facial nerve grading scale.

House-Brackmann Facial Nerve Grading Scale
Grade I Normal
Grade II Mild Dysfunction
Grade III Moderate Dysfunction
Grade IV Moderately Severe dysfunction
Grade V Severe Dysfunction
Grade VI Total Paralysis

Symptoms

  • severe otalgia with painful blisters on face around ear, mouth, or tongue
  • vertigo
  • nausea
  • vomiting
  • hearing loss
  • hyperacusis
  • tinnitus
  • eye pain and lacrimation

Clinical diagnosis

On physical exam look for vesicular exanthema on the external auditory canal, concha and or pinna. Dry eyes with possible lower cornea epithelium damage due to incomplete closure of eyelids.

Diagnostic procedures

Ramsay Hunt Syndrome type 2 can usually be diagnosed based on clinical features. However, for suspected cases with unclear presentation, varicella zoster virus can be isolated from vesicle fluid.

Tear culture PCR can have positive varicella zoster virus. However, 25-35% of patients with Bell palsy can have false positive varicellar zoster virus detected in tears.

If central nervous system complications such as meningitis, ventriculitis or meningoencephalitis are suspected, prompt lumbar puncture with spinal fluid analysis and imaging (CT head) are recommended.[3]

Laboratory test

WBC count, erythrocyte sedimentation rate and serum electrolytes to differentiate infectious and inflammation etiologies.

Direct immunofluorescence assay against varicella zoster antigen has been shown with sensitivity of 90% and specificity of 99%.[4]

Differential diagnosis

  • Bell Palsy
  • Postherpetic neuralgia
  • Temporomandibular joint pain dysfunction syndrome
  • Trigeminal Neuralgia
  • Migraine
  • Benign Paroxysmal Positional Vertigo
  • Persistent idiopathic facial pain

Management

Prompt diagnosis and initiation of steroids and antiviral are the current recommendations.

General treatment

Early diagnosis and early initiation of steroids and antivirals are key to complete recovery. Retrospective studies have shown that when treatment is initiated within 3 days, complete resolution of facial nerve paralysis for 70% of the patients. However, when therapies start 7 days after symptom onset, only 30% of the patients achieve complete resolution of facial paralysis using the House–Brackmann score to grade the degree of nerve damage in a facial nerve palsy.[5]

However, no effective treatment has been identified for hearing loss.[6]

Vertigo can be treated with diazepam.[7]

Medical therapy

Oral corticosteroids and acyclovirs are recommended for treatment of Ramsay Hunt Syndrome. In addition, carbamazepine can be used as a vestibular suppressant to alleviate the symptoms.[8]

Medical follow up

Monitor degree of facial nerve damage using the House–Brackmann score. From initiation of medical therapy, follow-up on patients in 2 weeks, 6 weeks and 3 months.

Surgical Treatments

If lagophthalmos persists and corneal damage is not amenable to medical management, patients could seek surgical treatments such as tarsorrhaphy, gold weight implantation, or lateral tarsal strip.

Complications

Long term facial nerve damage leading to hearing loss and facial paralysis. Patients could also develop synkinesia, or abnormal movement with voluntary facial movements. Up to 45% of the patients with Ramsay Hunt Syndrome can experience synkinesia during recovery and can be predicted by having severe facial weakness as measured by electroneurography.[9]

In addition, patients could develop postherpetic neuralgia, unresolved facial paralysis, or herpes zoster encephalitis.[10]

Prognosis

Ramsay Hunt syndrome cause more server symptoms compared to Bell Palsy and has a worst prognosis. The degree of facial nerve paralysis resolution can be predicted by severity of initial paralysis. Studies show the rate of complete resolution to range from 10-22% to 66%.[11][12]

Sequelae of Ramsay Hunt include permanent unilateral facial nerve paralysis, polycranial neuropathy, hearing loss, vertigo, and incomplete eye closure with dry eyes.

Patients without hypertension, diabetes and with a initial House-Brackann grade II or better presentation have higher chance of complete recovery (84.6%).[13]

Additional Resources

Mayo Clinic

https://www.mayoclinic.org/diseases-conditions/ramsay-hunt-syndrome/symptoms-causes/syc-20351783

National Organization of Rare Disorders

https://rarediseases.org/rare-diseases/ramsay-hunt-syndrome/


References

  1. Bauer CA, Coker NJ. Update on facial nerve disorders. Otolaryngol Clin North Am. 1996 Jun. 29(3):445-54.
  2. Alicandri-Ciufelli M, Aggazzotti-Cavazza E, Genovese E, Monzani D, Presutti L. Herpes zoster oticus: a clinical model for a transynaptic, reflex pathways, viral transmission hypotheses. Neurosci Res. 2012 Sep. 74 (1):7-9.
  3. Lindström J, Grahn A, Zetterberg H, Studahl M. Cerebrospinal fluid viral load and biomarkers of neuronal and glial cells in Ramsay Hunt syndrome. Eur J Neurosci. 2016 Sep 19.
  4. Coffin SE, Hodinka RL. Utility of direct immunofluorescence and virus culture for detection of varicella-zoster virus in skin lesions. J Clin Microbiol. 1995 Oct. 33(10):2792-5.
  5. Murakami S, Hato N, Horiuchi J, Honda N, Gyo K, Yanagihara N. Treatment of Ramsay Hunt syndrome with acyclovir‐prednisone: significance of early diagnosis and treatment. Annals of neurology. 1997 Mar;41(3):353-7.
  6. Kim YH, Chang MY, Jung HH, Park YS, Lee SH, Lee JH, Oh SH, Chang SO, Koo JW. Prognosis of Ramsay Hunt syndrome presenting as cranial polyneuropathy. The Laryngoscope. 2010 Nov;120(11):2270-6.
  7. Stafford FW, Welch AR. The use of acyclovir in Ramsay Hunt syndrome. The Journal of Laryngology & Otology. 1986 Mar;100(3):337-40.
  8. da Costa Monsanto R, Bittencourt AG, Neto NJ, Beilke SC, Lorenzetti FT, Salomone R. Treatment and prognosis of facial palsy on ramsay hunt syndrome: results based on a review of the literature. International archives of otorhinolaryngology. 2016 Oct;20(04):394-400.
  9. Morishima N, Yagi R, Shimizu K, Ota S. Prognostic factors of synkinesis after Bell's palsy and Ramsay Hunt syndrome. Auris Nasus Larynx. 2013 Oct 1;40(5):431-4.
  10. Eskiizmir G, Uz U, Taskiran E, Unlü H. Herpes zoster oticus associated with varicella zoster virus encephalitis. Laryngoscope. 2009 Apr. 119(4):628-30.
  11. Murakami S, Hato N, Horiuchi J, Honda N, Gyo K, Yanagihara N. Treatment of Ramsay Hunt syndrome with acyclovir-prednisone: significance of early diagnosis and treatment. Ann Neurol. 1997 Mar. 41(3):353-7.
  12. Sweeney CJ, Gilden DH. Ramsay Hunt syndrome. J Neurol Neurosurg Psychiatry. 2001 Aug. 71(2):149-54.
  13. Yeo SW, Lee DH, Jun BC, Chang KH, Park YS. Analysis of prognostic factors in Bell's palsy and Ramsay Hunt syndrome. Auris Nasus Larynx. 2007 Jun. 34(2):159-64.