Definition

Herpes zoster oticus, also known as Ramsay Hunt Syndrome, is a manifestation of shingles that impact the ear and facial nerves. This condition affects approximately 5 per 100,000 people annually and is responsible for around 7% of cases of acute facial nerve paralysis. Although it can affect individuals of all ages, it is most commonly observed in those aged 70 to 80.

Causes

Herpes zoster oticus is caused by the Varicella zoster virus (VZV), which is responsible for varicella or chickenpox. Initial infection with VZV results in chickenpox, characterized by widespread skin blistering. Following the resolution of chickenpox, the virus migrates to the spinal cord via sensory nerves, where it remains dormant within nerve roots, including those governing facial muscle movement and auditory function. Years later, the virus may reactivate, attacking the nerve roots emanating from the spinal cord. In cases of herpes zoster oticus, the virus targets the facial and vestibulocochlear (ear) nerves, leading to unilateral facial paralysis and hearing loss.

Risk factor

Herpes zoster oticus can develop in anyone with a history of chickenpox, with a higher incidence in the elderly (over 60 years old). This disease is uncommon in children. Although the condition is not contagious, individuals with active shingles can transmit the virus to those who have never had chickenpox, potentially spreading the chickenpox in these individuals. This can lead to severe symptoms, particularly in those who are unvaccinated or immunocompromised.

Symptoms 

The symptoms of herpes zoster oticus typically include intense ear pain. Additional symptoms may involve painful blisters or vesicles and a burning sensation near the ears, face, mouth, and/or tongue. Involvement of the vestibulocochlear nerves can result in auditory symptoms such as hearing loss, tinnitus, hyperacusis (sensitivity to loud noises), and vertigo that could cause dizziness, nausea, and vomiting. Facial nerve involvement can cause unilateral facial paralysis, difficulty closing the eyes, eye pain, and excessive tearing. Pain associated with shingles may precede the appearance of the rashes and sores by several days.

Diagnosis

The diagnosis of herpes zoster oticus is established through a combination of the patient's medical history, symptoms, and physical examination. Patients with this condition often recall having had chickenpox during childhood. The physical examination involves observing the distribution of blisters or vesicles and performing tests to assess hearing and balance function symptoms. This may include listening to specific sounds produced by a tuning fork or computer and performing various movements. Additionally, the physician will check for unilateral facial paralysis, which frequently accompanies hearing loss.

Treatment typically involves antiviral medications, necessitating laboratory tests such as blood urea nitrogen (BUN), creatinine, complete blood count, and electrolyte levels to evaluate the patient's capacity to process and eliminate the medication from their body. An antibody test may be conducted for immunocompromised individuals to determine prior infection with the varicella-zoster virus.

Imaging tests are not routinely employed for this condition, as they induce inflammation of the peripheral nerves.

Management

Therapeutic approaches for herpes zoster oticus can include both pharmacological and non-pharmacological methods. Commonly prescribed antivirals are most effective when administered within 72 hours of the appearance of the last blisters and should be taken for seven days. Anti-inflammatory drugs and analgesics also alleviate pain and reduce inflammation in the affected area. The infection typically resolves within several weeks to months, although residual nerve pain may persist even after the blisters have healed. In such cases, painkillers and antidepressants can be used to manage the pain. Surgical procedures may be considered to alleviate pressure on the facial nerve.

For patients experiencing difficulty closing their eyelids, protective measures such as eye patches can help prevent eye injuries. Artificial tears, applied six times daily, can also help prevent eye injuries by increasing lubrication and protecting against corneal abrasion or ulceration.

If blistering involves the eye or eye damage occurs due to the inability to close the eyelids, the patient will be referred to an ophthalmologist for further management.

Complications

Complications of herpes zoster oticus can be categorized into short-term and long-term issues. Short-term complications may include corneal damage, depression, social anxiety, and transmission of the virus to unvaccinated or immunocompromised individuals. Long-term complications can include:

• Permanent hearing loss and unilateral facial weakness. While many individuals experience temporary hearing loss and facial paralysis, in some cases, these effects can become permanent.
• Ocular problems. Facial muscle weakness can impede eyelid closure, leading to potential corneal damage. This can result in eye pain and blurred vision, and if untreated, corneal damage can become permanent, reducing visual acuity.
• Postherpetic neuralgia. This occurs when nerve fibers are damaged by the viral infection, resulting in pain. The excessive electrical signals transmitted through these damaged nerves can cause chronic pain, persisting even after other symptoms of herpes zoster oticus have resolved.

Prevention

The prevention of herpes zoster oticus is primarily done through immunization with the varicella vaccine, which can be administered during childhood. According to the Indonesian Pediatrician Association (IDAI) guidelines from 2020, immunization can be given at 12 months of age. The vaccination is given in two doses, 6 weeks to 3 months apart. The vaccine is also recommended for individuals over 50 years old to boost immunity against the virus. This vaccination significantly reduces the incidence of varicella-zoster infections and mitigates the severity of the disease. Improved immunity also lowers the risk of the virus reactivating as shingles.

When to see a doctor?

It is crucial to seek medical attention if you experience unilateral facial paralysis or clusters of blisters on your face. Prompt treatment with antiviral and anti-inflammatory therapy within 72 hours of symptom onset can help prevent long-term complications of herpes zoster oticus, potentially preserving your ability to perform daily activities.

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