Ramsay Hunt Syndrome Clinical Presentation

  • Author: Augusto A Miravalle, MD; Chief Editor: Karen L Roos, MD   more...
 
Updated: Feb 6, 2012
 

History

A careful history must be obtained in patients with suspected Ramsay Hunt syndrome.

  • Patients usually present with paroxysmal pain deep within the ear. The pain often radiates outward into the pinna of the ear and may be associated with a more constant, diffuse, and dull background pain.
  • The onset of pain usually precedes the rash by several hours and even days.
  • Classic Ramsay Hunt syndrome can be associated with the following:
    • Vesicular rash of the ear or mouth (as many as 80% of cases)
    • The rash might precede the onset of facial paresis/palsy (involvement of the seventh cranial nerve [CN VII])
    • Ipsilateral lower motor neuron facial paresis/palsy (CN VII)
    • Vertigo and ipsilateral hearing loss (CN VII)
    • Tinnitus
    • Otalgia
    • Headaches
    • Dysarthria
    • Gait ataxia
    • Fever
    • Cervical adenopathy
  • Facial weakness usually reaches maximum severity by 1 week after the onset of symptoms.
  • Other cranial neuropathies might be present and may involve cranial nerves (CNs) VIII, IX, X, V, and VI.
  • Ipsilateral hearing loss has been reported in as many as 50% of cases.
  • Blisters of the skin of the ear canal, auricle, or both may become secondarily infected, causing cellulitis.
  • Poor prognostic factors for good functional recovery include the following:
    • Age older than 50 years
    • Complete facial paralysis
    • Lack of CN VII nerve excitability
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Physical

  • The primary physical findings in classic Ramsay Hunt syndrome include peripheral facial nerve paresis with associated rash or herpetic blisters in the distribution of the nervus intermedius.[6]
  • The location of the accompanying rash varies from patient to patient, as does the area innervated by the nervus intermedius. It may include the following:
    • Anterior two thirds of the tongue
    • Soft palate
    • External auditory canal
    • Pinna
  • The patient may have associated ipsilateral hearing loss and balance problems.
  • A thorough physical examination must be performed, including neuro-otologic and audiometric assessment.
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Causes

  • Classic Ramsay Hunt syndrome is ascribed to infection of the geniculate ganglion by herpesvirus 3 (varicella-zoster virus [VZV]).
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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

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