Ramsay Hunt Syndrome
Timothy C. Hain, MD, Chicago IL. • Page last modified: July 23, 2022
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. MRI scans often show enhancement of the superior vestibular nerve (Iwasaki et al, 2013). 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)
In 2022, Ramsay-Hunt afflicted a Pop Star, Justin Bieber. Justin wrote to Instagram saying "Hey everyone. Justin here. I wanted to update you guys on what's been going on. Obviously, as you can probably see from my face, I have this syndrome called Ramsay Hunt syndrome [RHS], and it is from this virus that attacks the nerve in my ear and my facial nerves and has caused my face to have paralysis." He then demonstrated how he was unable to blink his right eye, wiggle his nose, or smile on the right side of his face. Justin was describing the damage to the facial nerve.
Patients with Ramsay Hunt often develop very severe vestibular problems -- it is common for them to lose 100% of their vestibular function.
VEMP testing may be abnormal in Ramsay Hunt. The oVEMP which assesses the superior vestibular nerve is usually out. The cVEMP, which assesses the inferior vestibular nerve is damaged in more severe cases.
About 5/100,000 persons per year develop Ramsay Hunt. It is the second most common cause of acute peripheral facial paralysis.
Considering treatment, first one follows the usual procedures for a severe Bells palsy. Patients with zoster (Ramsay Hunt) do substantially worse than patients with idiopathic Bells Palsy (Pietersen, 2002). 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.
Additionally, prednisone treatment and antiviral treatment, namely acylovir, are now recommended (De Ru and van Benthem, 2011). 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.
Surgical treatment, including facial nerve decompression, is rarely a good idea for Ramsay Hunt.
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.