Ramsay Hunt Syndrome

Ramsay Hunt Syndrome

Timothy C. Hain, MD, Chicago IL. Page last modified: June 18, 2009

Ramsay Hunt syndrome is an acquired paralysis of the face (Bells Palsy) specifically caused by a varicella-zoster virus (VSV) infection of the facial nerve. Often the virus mainly attacks the geniculate ganglion (Wackym 1997) . The symptoms on the affected side typically include facial weakness, a painful herpes type skin eruption on the pinna of the ear, and there is frequently vestibulo-cochlear disturbance (i.e. there may be dizziness or a disturbance of hearing). The latter symptoms are due to involvement of the 8th cranial nerve, which runs with the 7th nerve and the nervus intermedius (a sensory nerve) together in the internal auditory canal. Other cranial nerves can be involved as well.(Xanthopoulos, Noussios et al. 2002) , and the virus can also affect the brainstem ( Sartoretti-Schefer, Kollias et al. 1999)

VEMP testing may be abnormal in Ramsay Hunt.

About 5/100,000 persons per year develop Ramsay Hunt. It is the second most common cause of acute peripheral facial paralysis.

Total recovery of facial movement occurs in about 50% of treated patients. If hearing is affected (about 1/3), complete hearing recovery occurs in about half.

Considering treatment, first one follows the usual procedures for a severe Bells palsy. Additionally, prednisone treatment and antiviral treatment, namely acylovir, are now recommended. Either 750 mg of intravenous acyclovir/day or 4000 mg of oral medication is equally effective. Hearing recovery tends to be best in patients treated within 3 days of onset with acyclovir/Prednisone. Most authors have found that only 5% of persons with Ramsay-Hunt have residual hearing loss.

References:

Murakami et al, Annals Neurology, 41, 3, 1997, 353-357
Sartoretti-Schefer S, Kollias S and Valavanis A (1999). "Ramsay Hunt syndrome associated with brain stem enhancement." AJNR Am J Neuroradiol 20(2): 278-80.
Wackym PA (1997). "Molecular temporal bone pathology: II. Ramsay Hunt syndrome (herpes zoster oticus)." Laryngoscope 107(9): 1165-75.
Xanthopoulos J, Noussios G, Papaioannides D, Exarchakos G and Assimakopoulos D (2002). "Ramsay Hunt syndrome presenting as a cranial polyneuropathy." Acta Otorhinolaryngol Belg 56(3): 319-23.

Additional material:

Supplemental material on the site DVD:

Movie of Bell's phenomenon -- in the first half, looking at the paralyzed side, eye blinks are weak and the eye rolls up. On the second half, looking at the normal side, blinks are brisk and no eye rolling is seen. This movie was obtained from a patient who had Ramsay Hunt syndrome.