Ramsay Hunt syndrome

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Figure 1. Vesicles in the ear canal in a patient with Ramsay Hunt syndrome
Figure 2. Another case with vesicles in the ear canal. Photo courtesy of Dr. Stephen Reich
Ramsay Hunt syndrome (geniculate herpes) is a peripheral facial palsy due to a varicella zoster virus (VZV) attack involving the facial nerve and geniculate ganglion. (1-4) Due to the very proximal involvement, the facial weakness is often accompanied by taste impairment, hyperacusis, and diminution of salivary and lacrimal secretion, all markers of a very proximal facial nerve lesion. Pain in and behind the ear may be prominent, more than the mild mastoid aching in the typical case of Bell’s palsy. Vesicles may appear on the TM, in the EAC, on the lateral surface of the pinna and in the cleft between the ear and mastoid process (Figures 1 and 2). Occasionally, the herpetic eruption may also involve the palate or the neck. Hunt described two types: an otalgic form with pain in the ear and a prosopalgic form with pain deep in the face.

Some patients develop facial paralysis without ear or mouth rash but associated with serologic or DNA evidence of VZV infection (zoster sine herpete, zoster sine zoster). Vesicles may develop only after the onset of facial weakness. It has been estimated that up to one-third of Bell’s palsy cases may be due to zoster sine herpete. Compared with Bell’s palsy, patients with Ramsay Hunt syndrome often have more severe paralysis at onset and are less likely to recover completely.

References

1. Bhupal HK. Ramsay Hunt syndrome presenting in primary care. Practitioner 2010; 254:33-53. 2. Gupta NM, Parikh MP, Panginikkod S, Gopalakrishnan V. Ramsay Hunt syndrome. QJM. 2016 Oct;109(10):693. 3. Serinken M, Eken C, Dal O, Kutlu M. Man With Facial Nerve Palsy and Ear Pain. Ramsay Hunt Syndrome. Ann Emerg Med. 2016 Jan;67(1):141, 148. 4. Sweeney CJ, Gilden DH. Ramsay Hunt syndrome. J Neurol Neurosurg Psychiatry 2001;71:149-154.