Ramsay Hunt Syndrome

Author: Augusto A Miravalle, MD; Chief Editor: Karen L Roos, MD more...

Updated: Feb 6, 2012

What would you like to print?

Background
Pathophysiology
Epidemiology
Show All

Background

Ramsay Hunt syndrome is defined as an acute peripheral facial neuropathy associated with erythematous vesicular rash of the skin of the ear canal, auricle (also termed herpes zoster oticus), and/or mucous membrane of the oropharynx.

Herpes zoster oticus, day 6. Image courtesy of Manolette Roque, MD, Ophthalmic Consultants Philippines Co, EYE REPUBLIC Ophthalmology Clinic.

This syndrome is also known as geniculate neuralgia or nervus intermedius neuralgia. Ramsay Hunt syndrome can also occur in the absence of a skin rash, condition known as zoster sine herpete.^[1]

Ramsay Hunt syndrome was first described in 1907 by James Ramsay Hunt in a patient who had otalgia associated with cutaneous and mucosal rashes, which he ascribed to infection of the geniculate ganglion by human herpesvirus 3 (ie, varicella-zoster virus [VZV]).^[2]

The following may be observed:

VZV auricularis
VZV in any of the zoster zones of the head and neck (herpes auricularis, herpes facialis, and herpes occipito-collairs) with facial palsy
VZV in any of the zoster zones with facial palsy and auditory symptoms (eg, tinnitus, deafness, vertigo, nystagmus, ataxia)

Pathophysiology

Ramsay Hunt syndrome is defined as VZV infection of the head and neck that involves the facial nerve, often the seventh cranial nerve (CN VII). Other cranial nerves (CN) might be also involved, including CN VIII, IX, V, and VI (in order of frequency). This infection gives rise to vesiculation and ulceration of the external ear and ipsilateral anterior two thirds of the tongue and soft palate, as well as ipsilateral facial neuropathy (in CN VII), radiculoneuropathy, or geniculate ganglionopathy.

VZV infection causes 2 distinct clinical syndromes. Primary infection, also known as varicella or chickenpox, is a common pediatric erythematous disease characterized by a highly contagious generalized vesicular rash. The annual incidence of varicella infection has significantly declined after the introduction of mass vaccination programs in most countries of the world.^[3]After chickenpox, VZV remain latent in neurons of cranial nerve and dorsal root ganglia. Subsequent reactivation of latent VZV can result in localized vesicular rash, known as herpes zoster. VZV infection or reactivation involving the geniculate ganglion of CN VII within the temporal bone is the main pathophysiological mechanism of Ramsay Hunt syndrome.

Frequency

United States

Ramsay Hunt syndrome is a rare complication of latent VZV infection.^[4]As stated above, Ramsay Hunt syndrome might occur in the absence of cutaneous rash (zoster sine herpete). Interestingly, VZV has been detected by polymerase chain reaction (PCR) in the tear fluid of patients diagnosed with Bell palsy.^[5]Ramsay Hunt syndrome is estimated to account for 16% of all causes of unilateral facial palsies in children, and 18% of facial palsies in adults. Ramsay Hunt syndrome is rare in children younger than 6 years.^[4]

Ramsay Hunt syndrome is thought to be the cause of as many as 20% of clinically diagnosed cases of Bell palsy.^[5]

The incidence of Ramsay Hunt syndrome among patients with HIV infection is unknown. However, it may occur at a higher rate than in the general population because individuals with HIV infection have a higher risk of VZV infection.^[2]

International

Similar frequency has been reported in other countries.

Mortality/Morbidity

Ramsay Hunt syndrome is not usually associated with mortality. It is a self-limiting disease; the primary morbidity results from facial weakness. Unlike Bell palsy, this syndrome has a complete recovery rate of less than 50%.

Proceed to Clinical Presentation

Contributor Information and Disclosures

Author

Augusto A Miravalle, MD Fellow, Department of Neurology, Beth Israel Deaconess Medical Center, Harvard Medical School

Augusto A Miravalle, MD is a member of the following medical societies: American Academy of Neurology

Disclosure: Nothing to disclose.

Specialty Editor Board

Marion Priscilla Short, MD Assistant Professor, Departments of Neurology, Pediatrics, and Pathology, University of Chicago Hospitals and Clinics

Marion Priscilla Short, MD is a member of the following medical societies: American Academy of Neurology, American Association of Neuropathologists, American College of Medical Genetics, American Medical Association, and American Society of Human Genetics

Disclosure: Nothing to disclose.

Francisco Talavera, PharmD, PhD Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Medscape Salary Employment

Florian P Thomas, MD, MA, PhD, Drmed Director, Regional MS Center of Excellence, St Louis Veterans Affairs Medical Center; Director, National MS Society Multiple Sclerosis Center; Director, Neuropathy Association Center of Excellence, Professor, Department of Neurology and Psychiatry, Associate Professor, Institute for Molecular Virology, St Louis University School of Medicine

Florian P Thomas, MD, MA, PhD, Drmed is a member of the following medical societies: American Academy of Neurology, American Neurological Association, American Paraplegia Society, Consortium of Multiple Sclerosis Centers, and National Multiple Sclerosis Society

Disclosure: Nothing to disclose.

Chief Editor

Karen L Roos, MD John and Nancy Nelson Professor of Neurology, Professor of Neurological Surgery, Department of Neurology, Indiana University School of Medicine

Karen L Roos, MD is a member of the following medical societies: American Academy of Neurology and American Neurological Association

Disclosure: Nothing to disclose.

Additional Contributors

The authors and editors of eMedicine gratefully acknowledge the contributions of previous authors Deepak Awasthi, MD, and Augusto A Miravalle, MD, to the development and writing of this article.

References

Bhupal HK. Ramsay Hunt syndrome presenting in primary care. Practitioner. Mar 2010;254(1727):33-5, 3. [Medline].
Goldani LZ, Ferreira da Silva LF, Dora JM. Ramsay Hunt syndrome in patients infected with human immunodeficiency virus. Clin Exp Dermatol. Jun 1 2009;[Medline].
Kleinschmidt-DeMasters BK, Gilden DH. The expanding spectrum of herpesvirus infections of the nervous system. Brain Pathol. Oct 2001;11(4):440-51. [Medline].
Sandoval C C, Nunez F A, Lizama C M, Margarit S C, Abarca V K, Escobar H R. [Ramsay Hunt syndrome in children: four cases and review]. Rev Chilena Infectol. Dec 2008;25(6):458-64. [Medline].
[Guideline] Gilchrist JM. Seventh cranial neuropathy. Semin Neurol. Feb 2009;29(1):5-13. [Medline].
Ryu EW, Lee HY, Lee SY, Park MS, Yeo SG. Clinical manifestations and prognosis of patients with Ramsay Hunt syndrome. Am J Otolaryngol. Nov 8 2011;[Medline].
Coffin SE, Hodinka RL. Utility of direct immunofluorescence and virus culture for detection of varicella-zoster virus in skin lesions. J Clin Microbiol. Oct 1995;33(10):2792-5. [Medline].
Naganawa S, Nakashima T. Cutting edge of inner ear MRI. Acta Otolaryngol Suppl. Feb 2009;15-21. [Medline].
Uscategui T, Doree C, Chamberlain IJ, Burton MJ. Antiviral therapy for Ramsay Hunt syndrome (herpes zoster oticus with facial palsy) in adults. Cochrane Database Syst Rev. Oct 8 2008;CD006851. [Medline].
[Best Evidence] Dworkin RH, Barbano RL, Tyring SK, et al. A randomized, placebo-controlled trial of oxycodone and of gabapentin for acute pain in herpes zoster. Pain. Apr 2009;142(3):209-17. [Medline].
Aizawa H, Ohtani F, Furuta Y, et al. Variable patterns of varicella-zoster virus reactivation in Ramsay Hunt syndrome. J Med Virol. Oct 2004;74(2):355-60. [Medline].
Aviel A, Marshak G. Ramsay Hunt syndrome: a cranial polyneuropathy. Am J Otolaryngol. Jan-Feb 1982;3(1):61-6. [Medline].
Byl FM, Adour KK. Auditory symptoms associated with herpes zoster or idiopathic facial paralysis. Laryngoscope. 1976;86:372-9.
Furuta Y, Aizawa H, Ohtani F, et al. Varicella-zoster virus DNA level and facial paralysis in Ramsay Hunt syndrome. Ann Otol Rhinol Laryngol. Sep 2004;113(9):700-5. [Medline].
Furuta Y, Ohtani F, Kawabata H, et al. High prevalence of varicella-zoster virus reactivation in herpes simplex virus-seronegative patients with acute peripheral facial palsy. Clin Infect Dis. Mar 2000;30(3):529-33. [Medline].
Gnann JW Jr, Whitley RJ. Clinical practice. Herpes zoster. N Engl J Med. Aug 1 2002;347(5):340-6. [Medline].
Hunt JR. On herpetic inflammations of the geniculate ganglion: a new syndrome of its complications. J Nerv Ment Dis. 1907;34:73-96.
Kohler A, Chofflon M, Sztajzel R, Magistris MR. Cerebrospinal fluid in acute peripheral facial palsy. J Neurol. Mar 1999;246(3):165-9. [Medline].
McKenna MJ, Rauch SD. Practical neurotology. In: The Practice of Neurosurgery. Vol. 1996:199.
Pitkaranta A, Piiparinen H, Mannonen L, Vesaluoma M, Vaheri A. Detection of human herpesvirus 6 and varicella-zoster virus in tear fluid of patients with Bell's palsy by PCR. J Clin Microbiol. Jul 2000;38(7):2753-5. [Medline].
Robillard RB, Hilsinger RL, Adour KK. Ramsay Hunt facial paralysis: clinical analyses of 185 patients. Otolaryngol Head Neck Surg. Oct 1986;95(3 Pt 1):292-7. [Medline].
Whitley RJ. A 70-year-old woman with shingles: review of herpes zoster. JAMA. Jul 1 2009;302(1):73-80. [Medline].