Ramsay Hunt syndrome type 2

For other uses, see Ramsay Hunt syndrome.
Ramsay Hunt syndrome type 2
Other names: Herpes zoster oticus
Drawing of blisters in the ear with facial weakness
SpecialtyENT surgery
SymptomsOne sided facial paralysis, ear pain, small blisters near or in the external ear canal[1]
ComplicationsPostherpetic neuralgia[1]
Usual onset>60 years[2]
CausesVaricella zoster virus
Diagnostic methodBased on symptoms[1]
Differential diagnosisBell palsy, stroke[1]
PreventionShingles vaccine[1]
TreatmentValacyclovir, prednisone, eye protection[1]
Prognosis70% mostly recover[1]
Frequency5 per 100,000 per year[1]

Ramsay Hunt syndrome type 2, also known as herpes zoster oticus, typically presents with one sided facial paralysis, ear pain, and small blisters near or in the external ear canal.[1] In some, no or only blisters in the mouth occur.[1][2] This may be preceded by tiredness and fever.[1] Other symptoms may include dry eye, change in taste, trouble speaking, world spinning, or hearing loss.[1]

It is caused by a reactivation of varicella zoster virus (chickenpox) in the geniculate ganglion, a nerve cell bundle of the facial nerve.[1] Triggers may include physiological stress or an immunocompromised state.[1] Diagnosis is generally based on symptoms; though testing the blisters may be supportive.[1] It is a type of shingles.[1]

Treatment generally includes valacyclovir and prednisone.[1] Efforts to protect the eye from drying out include using eye lubricant during the day and taping the eye closed at night.[1] The shingles vaccine may help prevent the condition.[1] About 70% of people regain near normal facial function, though recovery may require months.[1] Other complications may include postherpetic neuralgia.[1]

Ramsay Hunt syndrome type 2 affects about 5 per 100,000 people per year.[1] While it occurs most commonly in peoples 70s and 80s, any age can be affected.[1] The condition was first described in 1907 by James Ramsay Hunt, after who it is named.[3][4]

Signs and symptoms

Small blisters in and around the ear in Ramsay Hunt syndrome type 2

Symptoms 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 red small blistering rash[5] in the ear canal, tongue, or mouth.[2] About 8% of cases do not have any blisters.[2]

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.[5]

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.[6]

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.[7]

Diagnosis

Ramsay Hunt syndrome type 2 can be diagnosed based on its appearance, however, in ambiguous cases PCR or direct immunofluorescent assay of vesicular fluid can help.

On physical exam small blisters and redness is seen in the external auditory canal, concha and or pinna. Dry eyes with possible lower cornea epithelium damage due to incomplete closure of eyelids may occur.

Lab tests

For unclear presentations, varicella zoster virus can be isolated from vesicle fluid. Tear culture PCR can have positive varicella zoster virus. However 25-35% of people with Bell's palsy can have false positive varicellar zoster virus detected in tears. If central nervous system complications such as meningitis, ventriculitis or meningoencephalitis are suspected, lumbar puncture with spinal fluid analysis and imaging (CT head) are recommended.

Prevention

See also: Varicella vaccine

Shingles is prevented by immunizing against the virus, varicella zoster, with the shingles vaccine.[8]

Treatment

Treatment is often with prednisone and the antiviral drug acyclovir or valacyclovir.[2] Best outcomes are if treatment is started within 3 days of the onset.[2] Valacyclovir may be used at a dose of 1000 mg three times per day for 7 days and prednisone may be used at a dose of 60 mg per day for two weeks followed by a taper over 1 week.[2]

Some studies demonstrate that even when steroids are started promptly, only 22% achieve full recovery of facial paralysis.[9] Treatment has no effect on the recovery of hearing loss.[10] Diazepam is sometimes used to treat the vertigo.[11]

History

The syndrome is named for James Ramsay Hunt, the neurologist who first described it.[3][12]

References

  1. 1 2 3 4 5 6 7 8 9 10 11 12 13 14 15 16 17 18 19 20 21 22 23 Crouch, AE; Hohman, MH; Andaloro, C (January 2022). "Ramsay Hunt Syndrome". PMID 32491341. {{cite journal}}: Cite journal requires |journal= (help)
  2. 1 2 3 4 5 6 7 Dorsch, JN (March 2014). "Neurologic syndromes of the head and neck". Primary care. 41 (1): 133–49. doi:10.1016/j.pop.2013.10.012. PMID 24439887.
  3. 1 2 "Ramsay Hunt's syndrome II". www.whonamedit.com. Archived from the original on 21 January 2022. Retrieved 24 July 2022.
  4. ↑ Ramsay Hunt, J.R. (1907). "On herpetic inflammations of the geniculate ganglion: a new syndrome and its complications". Journal of Nervous and Mental Disease. 34 (2): 73–96. doi:10.1097/00005053-190702000-00001. Archived from the original on 2019-12-13. Retrieved 2021-08-11.
  5. 1 2 Sweeney, C.J.; Gilden, D.H. (August 2001). "Ramsay Hunt syndrome". Journal of Neurology, Neurosurgery, and Psychiatry. 71 (2): 149–54. doi:10.1136/jnnp.71.2.149. PMC 1737523. PMID 11459884.
  6. ↑ Kim, In Sup; Shin, Seung-Ho; Kim, Jinn; Lee, Won-Sang; Lee, Ho-Ki (2007). "Correlation between MRI and Operative Findings in Bell's Palsy and Ramsay Hunt Syndrome". Yonsei Medical Journal. 48 (6): 963–968. doi:10.3349/ymj.2007.48.6.963. PMC 2628199. PMID 18159587.
  7. ↑ Murakami, S.; Honda, N.; Mizobuchi, M.; Nakashiro, Y.; Hato, N.; Gyo, K. (1998). "Rapid diagnosis of varicella zoster virus infection in acute facial palsy". Neurology. 51 (4): 1202–1205. doi:10.1212/wnl.51.4.1202. PMID 9781562. S2CID 10029189.
  8. ↑ "Fact sheet: Get the new shingles vaccine if you are 50 or older | Herpes Zoster | CDC". www.cdc.gov. 2021-03-30. Archived from the original on 2021-03-18. Retrieved 2021-03-30.
  9. ↑ Finsterer, Josef (2008). "Management of peripheral facial nerve palsy". European Archives of Oto-Rhino-Laryngology. 265 (7): 743–752. doi:10.1007/s00405-008-0646-4. PMC 2440925. PMID 18368417.
  10. ↑ Kim, Y.H.; Chang, M.Y.; Jung, H.H.; Park, Y.S.; Lee, S.H.; Lee, J.H.; Oh, S.H.; Chang, S.O.; Koo, J.W. (2010). "Prognosis of Ramsay Hunt syndrome presenting as cranial polyneuropathy". Laryngoscope. 120 (11): 2270–2276. doi:10.1002/lary.21108. PMID 20824789. S2CID 264059.
  11. ↑ NINDS Herpes Zoster Oticus Information Page at NINDS
  12. ↑ "The Ramsay Hunt syndrome". Proceedings of the Royal Society of Medicine. 47 (5): 371–384. May 1954. doi:10.1177/003591575404700517. PMC 1918846. PMID 13167057.
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