Ramsay Hunt’s hypothesis that herpes zoster oticus results from reactivation of the varicella zoster virus (VZV) in the geniculate ganglion is supported by the detection of viral genome in archival temporal bones of normals and Ramsay Hunt patients by the polymerase chain reaction. Ramsay Hunt syndrome is characterized by the presence of cochleovestibular symptoms in association with facial paralysis. VZV has also been demonstrated in the spiral and/or vestibular ganglion. Two cases are reported in which cochleovestibular symptoms outweighed the facial nerve symptoms, presumably representing VZV reactivation in the spiral and/or vestibular ganglion. From these observations and the known dormancy of VZV in non-neuronal satellite cells, it is argued that the cochleovestibular symptoms in Ramsay Hunt syndrome may result from VZV transmission across the nerves inside the internal auditory canal and that prompt treatment with an antiviral-corticosteroid combination might be justified in the management of any acute non-hydropic cochleovestibular syndrome.

Viral reactivation is thought to be an important cause of post-operative facial palsy of delayed onset. We present an unusual case of Ramsay-Hunt syndrome that occurred as a consequence of middle-ear surgery by triggering varicella-zoster virus reactivation. As a pathognomonic auricular eruption was not seen, the patient was initially misdiagnosed as iatrogenic facial palsy. Clinical features, diagnosis and management are discussed.

Ramsay Hunt syndrome is characterized by facial nerve paralysis, herpetic vesicles in or around the ear and pain often associated with vestibulocochlear nerve involvement. It is thought to be a cranial polyneuropathy caused by the herpes zoster virus. We present an extreme and unusual variant of this disease with involvement of VIIth, VIIIth, Xth, XIth and XIIth cranial nerves as well as C2–4 sensory dermatomes and profound systemic upset which caused some diagnostic uncertainty.

The possible involvement of depression on cellular immunity in reactivation of varicella-zoster virus (VZV) in herpes zoster oticus was investigated. The subjects comprised 59 cases of herpes zoster oticus, 33 cases of herpes zoster sine herpete (ZSH) with facial paralysis, and 205 cases of Bell's palsy. The transformation rate of lymphocytes to phytohaemagglutinin in herpes zoster oticus tended to be lower than that in Bell's palsy. In skin tests with purified protein derivatives of tuberculin, the positivity rate in herpes zoster oticus was significantly lower than that in Bell's palsy (p<0.015). In skin tests using VZV antigen, the positivity rate in herpes zoster oticus and ZSH were significantly lower than that of Bell's palsy (p<0.001 and p<0.015, respectively). Thus, it was noted that cellular immunity, especially specific cellular immunity against VZV, was significantly depressed in herpes zoster oticus and ZSH. We consider that depression of specific cellular immunity plays an important role in triggering reactivation of VZV and onset of these diseases.

The authors present a case of Ramsay Hunt syndrome in whish the MRI appearance mimicked that of an intracanalicular acoustic neuroma.

We present the case of a 47-year-old woman with left otalgia, rotatory vertigo, sensorineural hearing loss and acute facial nerve palsy. An enhanced magnetic resonance imaging (MRI) scan showed discrete enhancement of the facial and vestibulocochlear nerves in the left internal auditory canal as well as of the labyrinth. This appearance was compatible with that in Ramsay Hunt syndrome and acyclovir was started prior to the appearance of any vesicular eruption. The diagnosis was subsequently confirmed serologically. She regained full facial function but the sensorineural hearing loss persisted. The literature pertaining to the role of the MRI in acute facial palsies is reviewed.