- 2nd M/C cause of atraumatic peripheral facial paralysis
J Ramsay Hunt, who described various clinical presentations of facial paralysis and rash, also recognised other frequent symptoms and signs such as tinnitus, hearing loss, nausea, vomiting, vertigo, and nystagmus. He explained these eighth nerve features by the close proximity of the geniculate ganglion to the vestibulocochlear nerve within the bony facial canal. Hunt’s analysis of clinical variations of the syndrome now bearing his name led to his recognition of the general somatic sensory function of the facial nerve and his defining of the geniculate zone of the ear. It is now known that varicella zoster virus (VZV) causes Ramsay Hunt syndrome.
- Ramsay Hunt syndrome (RHS) type 1
- Rare, degenerative, neurological disorder characterized by myoclonus epilepsy, intention tremor, progressive ataxia and occasionally cognitive impairment.
- Ramsay Hunt syndrome (RHS) type 2
- Disorder that is caused by the reactivation of varicella zoster virus in the geniculate ganglion, a nerve cell bundle of the facial nerve.
57% RHS cases are misdiagnosed.
- Facial paralysis (complete > incomplete paralysis)
- Ear pain (due to zoster oticus)
- Can project into neighboring regions of the face and nape of the neck
- Herpetic eruptions in any cranial dermatome
- Cutaneous findings of herpes zoster oticus pointing to the diagnosis RHS typically manifest in Hunt’s zone (eardrum, the external auditory canal and the central portion of the ear, the cavum conchae)
Patient 1 (56y/F): The patient reported pain of increasing intensity in the left ear for one week. Two days before hospital admission weeping lesions developed on the concha of the left ear. Simultaneously the patient observed facial paralysis and a partial loss of hearing on the left side as well as vertigo with nausea and vomiting.
Patient 2 (81y/F): Three days before hospital admission the patient first noticed pruritus on the right ear. Tw o days later distinct pain developed here with projection to the right side of the face. In the further course loss of control over the right eyelid, the right corner of the mouth and the right half of the tongue. During the hospital stay the patient complained of sensitivity to noise in the right ear.
Patient 3 (76y/F): The patient developed acute severe pain on the right side of the face and occipital region, subsequently accompanied by hearing loss and cervical skin lesions on the right side. Hospital admission four days after the onset of symptoms.
- Bell’s palsy (facial paralysis without rash)
- Patients with Ramsay Hunt syndrome often have more severe paralysis at onset and are less likely to recover completely