RAMSAY-HUNT SYNDROME: SYMPTOMS, CAUSES, AND TREATMENT - NEUROPSYCHOLOGY - 2025

The syndrome Ramsay-Hunt is a type peripheral facial paralysis (PFP), caused by the herpes zoster virus. In addition, it is associated with an involvement of the external auditory canal and the tympanic membrane.

Ramsay-Hunt syndrome is an infectious medical condition that is also the second cause of peripheral facial paralysis without traumatic origin. The clinical presentation of this pathology is very varied, being frequent to find it classified into four stages.

However, some of the symptoms and medical complications that occur as a result of Ramsay-Hunt syndrome may include: facial paralysis, headache, nausea, elevated body temperature, hearing loss, tinnitus, vertigo, among others.

On the other hand, in the case of the diagnosis of this pathology, the clinical history and physical examination are essential. In addition, it is also possible to perform various complementary tests to specify the clinical findings.

Treatment of Ramsay-Hunt syndrome usually includes the administration of corticosteroids and antivirals. The essential objective of medical interventions is to avoid secondary medical sequelae.

Ramsay-Hunt syndrome characteristics

It is a rare neurological disorder characterized by facial paralysis, involvement of the external auditory canal and the tympanic membrane.

Different names have been used in the medical literature to designate this pathology. Currently, Ramsay-Hunt syndrome (SRH) is the term used to refer to the set of symptoms that occur as a sequence of facial nerve involvement due to a herpes zoster infection.

This medical condition was initially described by the physician James Ramsay Hunt in 1907. In one of his clinical reports they presented a case characterized by facial paralysis and herpetic lesions in the ear canal.

This pathology can also be called otic herpes zoster and is considered a polyneuropathy caused by a neurotrophic virus.

The term polyneuropathy is used to refer to the presence of an injury to one or more nerves, regardless of the type of injury and the affected anatomical area. On the other hand, neurotropic viruses are pathological agents that essentially attack the nervous system (SN).

Affected facial nerve

Thus, Ramsay-Hunt syndrome specifically affects the facial nerve. The facial nerve or cranial nerve VII, is a nerve structure that has the function of controlling a large part of the functions of the facial area.

In addition, the facial nerve is a paired structure that runs through a bony canal in the skull, below the ear area, to the facial muscles.

When any pathological event (trauma, degeneration, infection, etc.) leads to the development of lesions or inflammation in the facial nerve, the muscles responsible for controlling facial expression can become paralyzed or weakened.

When the herpes zoster virus reaches the facial nerve and adjacent regions, a wide variety of signs and symptoms may appear skin, muscle, sensory, etc.

Frequency

Ramsay-Hunt syndrome is the second leading cause of atraumatic peripheral facial palsy (PFP). Statistical studies estimate that Ramsay-Hunt syndrome covers 12% of facial paralysis, approximately 5 cases per 100,000 inhabitants per year.

Regarding sex, there are no significant differences, therefore, it affects men and women equally.

Although anyone who has had chickenpox can develop this pathology, it is more common among adults. Specifically, it can be seen more frequently in the second and third decades of life.

Ramsay-Hunt syndrome is an infrequent or rare pathology in children.

Signs and symptoms

The clinical manifestations of Ramsay-Hunt syndrome are varied, the symptoms can be grouped into several categories:

• General symptoms: fever, headache, nausea, anorexia, asthenia.
• Sensory symptoms: skin rashes, hearing loss, etc.
• Peripheral facial paralysis
• Associated symptoms: instability, vertigo, tinnitus, etc.

Apart from this grouping, the characteristic symptoms of Ramsay-Hunt syndrome usually vary from one case to another. In most cases, there may be a prodromal phase characterized by the presence of:

• Headache: severe and persistent headache.
• Asthenia: muscle weakness, fatigue, persistent tiredness, physical and psychological exhaustion.
• Adynamia: reduced physical initiative due to the presence of significant muscle weakness.
• Anorexia: Anorexia as a symptom is used to designate the presence of a lack of appetite or loss of appetite that accompanies a wide variety of medical conditions.
• Fever: abnormal increase or elevation of body temperature.
• Nausea and vomiting
• Intense ear pain: this is a type of earache that appears as a result of the pressure that the accumulation of fluid in the areas of the middle ear exerts on the eardrum and other adjacent structures.

On the other hand, various skin lesions also appear on the external auditory pin and in the posterior areas of the tympanic membrane:

• Erythematous spots: Erythemas are reddish spots on the skin that occur as a result of immune processes, such as inflammation.
• Vesicles: these are small blisters on the skin that are made up of fluid inside. They usually appear about 12 or 24 hours after the development of the infectious process and usually turn into pustules.

Facial paralysis

In addition to the signs and symptoms detailed above, facial paralysis is one of the most serious and notable.

In the patient affected by Ramsay-Hunt syndrome, it is possible to observe a decrease or absence of facial mobility, half of the face is paralyzed or “drooping”.

Therefore, many people tend to have various deficits associated with the muscles that control facial expression: inability to close the eyes, smile, frown, raise the eyebrows, speak and / or eat.

On the other hand, the herpes zoster virus, in addition to the facial nerve or cranial nerve VII, can also affect the vestibulocochlear nerve, cranial nerve VIII.

The vestibulocochlear nerve plays an essential role in controlling additive function and balance. Thus, when some of its two branches (cochlear or vestibular) are affected, various sensory symptoms may appear:

• Cochlear branch injury: hearing loss and tinnitus.
• Vestibular branch injury: vertigo, nausea, nystagmus.

Specifically, the symptoms caused by vestibulocochlear nerve involvement are:

• Hearing loss: total or partial reduction in hearing capacity.
• Tinnitus: presence of an auditory buzzing, buzzing, or whistling.
• Vertigo: It is a specific type of dizziness that we often describe as a sensation of movement and spinning.
• Nausea: feeling of gastric discomfort, urge to vomit.
• Nystagmus: arrhythmic and involuntary movements of one or both eyes.

Classification

Because the clinical presentation of Ramsay-Hunt syndrome is very varied, it is usually classified into 4 stages (clinical ramsay-Hunt classification):

• Stage I: development of skin eruptions (vesicles in the territory of the facial nerve), infectious syndrome (fever, headache, etc.) and earache.
• Stage II: Stage I symptoms are present, and facial paralysis begins to develop.
• Stage III: all of the above symptoms, plus hearing loss and vertigo.
• Stage IV: increased cranial nerve involvement and development of secondary medical symptoms.

Causes

Ramsay-Hunt syndrome is caused by the Varicella-Zoster virus (VZV). This virus is the cause of chickenpox and herpes zoster.

Several experimental studies indicate that once chickenpox has been contracted, the virus can remain dormant for decades. However, due to some conditions (stress, fever, tissue damage, radiation therapy, immunosuppression), it can reactivate and, in some cases, lead to the development of Ramsay-hunt syndrome.

Diagnosis

The diagnosis of Ramsay-Hunt syndrome is usually confirmed through history and clinical examination, complementary tests, and neuroimaging techniques.

The patient's medical history must include the family and personal medical history, the symptomatology record, the moment of presentation and evolution of the pathology, in addition to other aspects.

The clinical examination should be based on a thorough examination of the symptoms present. In addition, a neurological examination is also essential to determine the presence of nerve damage.

As for the complementary tests that are usually used, cytodiagnosis or serological study of the virus, they are essential to determine the presence of an infection caused by the varicella-zoster virus.

In the case of imaging tests, magnetic resonance imaging or computed tomography, they are useful to confirm the presence of neurological damage.

In addition to these, other types of complementary tests are also used, such as audiometries, brain stem evoked potentials, or electroneurography of the facial nerve, to assess the degree of hearing impairment and the degree of facial nerve involvement.

Treatments

The treatment used in Ramsay-Hunt syndrome focuses on stopping the infectious process, reducing symptoms and pain, and also reducing the risk of developing neurological and physical sequelae in the long term.

Pharmacological interventions usually include:

• Antiviral drugs: the goal is to slow the progression of the viral agent. Some of the drugs that medical specialists are the Zovirax, Famvir or Valtrex.
• Corticosteroids: These are usually used in high doses for short periods of time to increase the effect of antiviral drugs. One of the most used is forgiveness. In addition, steroids are also often used to reduce inflammation and thus the likelihood of medical consequences.
• Analgesics: it is possible that the pain associated with the auditory pin is severe, so some specialists often use drugs that contain oxycodone or hydrocodone, for analgesic treatment.

On the other hand, other non-pharmacological therapeutic interventions can also be used, such as surgical decompression.

This type of surgical approach is still very controversial, it is usually restricted to cases that do not respond to drug treatment in at least a time greater than 6 weeks.

Generally, the treatment of choice for most cases of Ramsay-Hunt syndrome is antiviral drugs and corticosteroids.

Forecast

The clinical evolution of Ramsay-Hunt syndrome is usually worse than expected in other facial paralyzes. Approximately, a set of cases that range from 24-90% of the total, usually show significant medical sequelae.

Although, with good medical intervention, both facial paralysis and hearing loss are temporary, in some cases it can become permanent.

In addition, the muscle weakness resulting from facial paralysis, prevents the efficient closing of the eyelid and, therefore, external agents can cause eye injuries. Eye pain or blurred vision may appear as one of the medical sequelae.

In addition, severe cranial nerve involvement can also cause persistent pain, long after the other signs and symptoms resolve.

References

1. Arana-Alonso, E., Contín-Pescacen, M., Guillermo-Ruberte, A., & Morea Colmenares, E. (2011). Ramsay-Hunt syndrome: what treatment do you need? Semergen, 436-440.
2. Boemo, R., Navarrete, M., García-Aurmí, A., Lareo Copa, S., Graterol, D., & Parelló Scherdel, E. (2010). Ramsay Hunt syndrome: our experience. Acta Otorrinolaringol Esp, 418-421.
3. de Peña Ortiz, AL, Gutiérrez Oliveros, T., Guarneros Campos, A., & Sotomayor López, D. (2007). Ramsay Hunt syndrome. Dermatology Rev Mex, 190-195.
4. Palsy UK Facial. (2016). Ramsay Hunt Syndrome. Obtained from Facial Palsy.UK.
5. Gómez-Torres, A., Medinilla Vallejo, A., Abrante Jiménez, A., & Esteban Ortega, f. (2013). Ramsay-Hunt syndrome causing laryngeal paralysis. Acta Otorrinolaringol Esp, 72-74.
6. Mayo Clinic. (2014). Ramsay Hunt syndrome. Obtained from Mayo Clinic.
7. NORD. (2011). Ramsay Hunt Syndrome. Obtained from National Organization for Rare Disorders.
8. Plaza-Mayor, G., López Estebaranz, J., López Lafuente, J., & de los Santos Granados, G. (2016). Ramsay-Hunt syndrome. Obtained from the Spanish Clinical Journal.
9. Source image 1