Ramsay Hunt syndrome, also known as herpes zoster oticus, is an acute peripheral facial neuropathy characterized by a painful rash around the ear. It is caused by an infection of the varicella zoster virus that affects the facial nerve close to the inner ear.
The syndrome was first described by James Ramsay Hunt in 1907, who noted a patient with otalgia and cutaneous and mucosal rashes that he linked to a viral infection.
Although it may affect individuals of any age, the incidence of the condition increases with age, and it most commonly affects elderly people over 60 years.
An infection with the varicella-zoster virus is responsible for the symptoms associated with Ramsay Hunt syndrome. The virus is thought to infect the facial nerve that is located near the inner ear, which leads to irritation and swelling in the area.
The symptoms of Ramsay Hunt syndrome include a painful blister-like rash in and around the ear on one side. Occasionally the tongue and the roof of the mouth may also be affected.
It is common for people to also experience tinnitus or loss of hearing on the affected side and some vertigo may also be experienced. Other possible symptoms include difficulty in closing the eye on the affected side, lack of control of the mouth leading to difficulty eating and unsymmetrical facial expressions.
If the condition is not treated adequately, complications may occur. This may include disfigurement of the face, damage to the eye, taste changes, muscle spasms and persistent pain.
Ramsay Hunt syndrome may be suspected upon observation of the presenting symptoms, such as rash and weakness of the facial muscles localized to one side of the face. Diagnosis can then be confirmed with blood tests or skin tests that detect the presence of the causative varicella zoster virus.
Early diagnosis is an important factor to promote a positive prognosis for the condition. If treatment is commenced within 3 days of the initiation of symptoms, 70% of patients are likely to make a full recovery. However, as the time to treatment increase the prognosis steadily decreases.
Other testing methods may then be required to investigate the extent of the infection and damage to the affected nerves, which will help to guide treatment decisions. This may include electromyography, lumbar puncture, magnetic resonance imaging (MRI) or nerve conduction tests.
The first-line treatment for Ramsay Hunt syndrome is usually a short course of corticosteroid medications, such as prednisolone for 5-7 days. Patients may also benefit from a course of antiviral medications, although the evidence to support this practice is less clear. Some patients also find that they require additional pain relief medications to cope with the severe pain associated with the syndrome.
For patients that have difficulty closing their eye due to facial weakness, wearing an eye patch to protect the eye and prevent corneal abrasion is recommended. Additionally, using artificial tears during the day and an eye lubricant at night can help to prevent drying out of the eye.