A Case of Ramsay Hunt Syndrome without Facial Nerve Palsy (Haymann Type IV)

Abstract

The three cardinal signs of the complete form of Ramsay Hunt syndrome are facial nerve palsy, herpes zoster oticus, and 8^th nerve dysfunction.

We encountered a patient with Ramsay Hunt syndrome who exhibited herpes zoster oticus without facial nerve palsy (Haymann type IV). A 54-year old male complaining of vertigo and hearing loss on the left side associated with pain and a skin rash in the left ear and headache was referred to our hospital. Examination of the hearing and vestibular functions revealed left-sided sensorineural hearing loss and canal paresis with spontaneous horizontal and rotary nystagmus, but no evidence of facial palsy. Reactivation of varicella zoster virus (VZV) was concluded from the elevated VZV-specific serum IgM and IgG antibody levels and result of analysis of saliva by PCR assay. Cerebrospinal fluid examination revealed findings consistent with viral meningitis. Finally, the patient was diagnosed as having Ramsay Hunt syndrome of the incomplete type (Haymann type IV) with viral meningitis, and was initiated on treatment with acyclovir and prednisolone. Both the headache and herpetic vesicles responded well to the treatment. However, the left-sided total sensorineural hearing loss, positional nystagmus to the right, and canal paresis on the left persisted for eleven months.

Cochleovestibular symptoms with Haymann type IV Ramsay Hunt syndrome are presumably associated with VZV reactivation in the spiral and/or vestibular ganglion, but not in the geniculate ganglion. Previous reports have revealed the poor prognosis of 8^th nerve dysfunction and risk of central nervous complications in patients with Haymann type IV Ramsay Hunt syndrome. Therefore, attention should be paid to the presence of the central nervous symptoms in such patients.