He Ramsay-Hunt syndrome Is a peripheral facial palsy (PFP), caused by the herpes zoster virus (Arana-Alonso, et al., 2011).

In addition, it is associated with an involvement of the external auditory canal and the tympanic membrane (Gómez-Torres et al., 2011).

Ramsay-Hunt syndrome is a medical condition of infectious nature and is also the second leading cause of peripheral facial paralysis without a traumatic origin (Boemo et al., 2010).

The clinical presentation of this pathology is very varied, being frequently found in four stages (Arana-Alonso, et al., 2011).

However, some of the symptoms and medical complications that occur as a result of the Ramsay-Hunt may include: facial paralysis, Headache , Nausea, elevation of body temperature, hearing loss, Tinnitus , Vertigo, among others (Boemo et al., 2010).

On the other hand, in the case of diagnosis of this pathology, clinical history and physical examination Are essential (de Peña Ortiz et al., 2007). In addition, it is also possible to carry out Specify the clinical findings (Boemo et al., 2010).

Treatment of Ramsay-Hunt syndrome usually involves the administration of Corticosteroids And antivirals. The essential aim of medical interventions is to avoid secondary medical sequelae (Boemo et al. 2010).

Characteristics of Ramsay-Hunt syndrome

It is a rare neurological disorder characterized by facial paralysis, involvement of the external auditory canal and 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 infection by Herpes zoster (Facial Palsy UK, 2016).

This medical condition was first described by physician James Ramsay Hunt in 1907. In one of his clinical reports, A case characterized by facial paralysis and herpetic lesions in the auditory canal (de Peña Ortiz et al., 2007).

This pathology can also be called optic herpes zoster and is considered a Polyneuropathy Caused by a neurotrope virus (Plaza Mayor et al., 2016).

The term polyneuropathy is used to refer to the presence of a lesion one or several nerves, regardless of the type Of injury and anatomical area affected (Colmer Oferil, 2008).

On the other hand, neurotropos viruses are pathological agents that attack the nervous system (SN).

Thus, Ramsay-Hunt syndrome specifically affects the facial nerve.

The facial nerve or the cranial nerve VII, is a nervous structure that has the function of controlling much of the functions of the facial area (Devéze et al., 2013).

In addition, the facial nerve is a structure in pairs that runs through a bone channel, in the skull, below the ear area, towards the muscles (National Institute of Neurological Disorders and Stroke, 2010).

When any pathological event (trauma, degeneration, infection, etc.) results in the development of lesions or inflammation in the facial nerve, the muscles responsible for controlling facial expression may become paralyzed or weakened (American Academy of Ophthalmology, 2016).

When the herpes zoster virus reaches the facial nerve and adjacent regions, a wide variety of cutaneous, muscular, sensory, and other signs and symptoms may appear.

The most important clinical features of Ramsay-Hunt syndrome are:

• Paralysis of the facial nerves.

• External auditory canal involvement (rash).

• Tympanic affection.

Frequency

Ramsay-Hunt syndrome is the second cause of atraumatic peripheral facial palsy (PFP).

Statistical studies estimate that Ramsay-Hunt syndrome comprises 12% of facial paralysis, approximately 5 cases per 100,000 inhabitants per year (Boemo et al., 2010).

As for sex, there are no significant differences, therefore, it affects men and women equally (Boemo et al. 2010).

Even though anyone who has suffered chickenpox Can develop this pathology, is more common among adults (Mayo Clinic, 2014).

Specifically, it can be observed more frequently in the second and third decade of life (Boemo et al., 2010).

Ramsay-Hunt syndrome is an uncommon or rare disease in children (Mayo Clinic, 2014).

Signs and symptoms

The clinical manifestations of Ramsay-Hunt syndrome are varied; symptoms can be grouped into several categories (Boemo et al., 2010):

• General Symptomatology : Fever, headache, nausea, anorexy , asthenia .

• Sensitive symptoms : Rash, hearing loss, etc.

• Peripheral facial paralysis

• Associated Symptomatology: instability, Vertigo , Tinnitus, etc...

Apart from this grouping, the characteristic symptoms of Ramsay-Hunt syndrome usually vary from case to case.

In most cases, there may be a prodromal phase characterized by the presence of (Peña Ortiz et al., 2007):

• Headache : Severe and persistent headache.

• Asthenia : Muscle weakness, fatigue, persistent tiredness, physical and psychological exhaustion.

• Adinamia : Reduced physical initiative due to the presence of significant muscle weakness.

• Anorexy : Anorexia as a symptom is used to denote the presence of a lack of appetite or inappetence that accompanies a wide Variety of medical conditions.

• Fever : Abnormal increase or increase in body temperature.

• Nausea and vomiting.

• Intense otalgia : This is a type of earache that appears as a consequence of the pressure that the accumulation of fluid in the Areas of the middle ear over the eardrum and other adjacent structures.

On the other hand, several cutaneous lesions also appear in the external auditory canopy and in the posterior areas of the tympanic membrane (de Peña Ortiz et al., 2007):

• Erythematous spots : Erythema are red spots on the skin that occurs as a consequence of immunological processes, such as
  inflammation.

• Vesicles: They are small blisters on the skin that are composed in its interior by liquid. They usually appear about 12 or 24 hours After the development of the infectious process and usually become pustules.

In addition, of 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 Finds paralyzed or"fallen"(American Academy of Ophthalmology, 2016).

Therefore, many people often have several deficits associated with muscles that control facial expression: inability to close the Eyes, smile, frown, raise eyebrows, talk and / or eat (Benítez et al., 2016).

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

The vestibulocochlear nerve has an essential function in the control of additive function and balance. Thus, when some of its branches (cochlear or vestibular) are affected, several sensorial symptoms may appear (Boemo et al., 2010).

• Cochlear branch injury : Hearing loss and tinnitus.

• Vestibular branch injury : Dizziness, nausea, nystagmus.

Specifically, the symptoms of the involvement of the vestibulocochlear nerve are:

• Hearing loss : Total or partial reduction of the hearing capacity.

• Tinnitus : Presence of a buzzing, rumor or auditory whistle.

• Vertigo : Is a specific type of dizziness that we often describe as a sensation of movement and twists.

• Sickness : Feeling of gastric discomfort, urgency of vomiting.

• 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 four stages (clinical classification of ramsay-Hunt) (Erana-Alonso et al., 2011):

• Stadium I : Development of skin eruptions (vesicles in the territory of the facial nerve), infectious syndrome (fever, headache, etc...) And otalgia.

• Stage II : The symptoms of stage I are present and, in addition, facial paralysis begins to develop.

• Stage III : All symptoms at In addition to Hearing loss And vertigo.

• Stage IV : Increased involvement of cranial nerves and development of secondary medical symptoms.

Causes

Ramsay-Hunt syndrome is produced by the Varicella-Zoster virus (VZV) (Boemo et al., 2010). This virus is the cause of chickenpox and shingles.

Several experimental studies indicate that, once chickenpox has contracted, the virus may remain dormant for decades. However, due to some conditions (stress, fever, tissue damage, radiotherapy, immunosuppression), it can be reactivated and, in some cases, lead to the development of Ramsay-hunt syndrome (National Organization for Rare Disorders, 2011).

Diagnosis

The diagnosis of Ramsay-Hunt syndrome is usually confirmed through history and clinical examination, complementary tests and neuroimaging techniques (Gómez-Torres et al., 2013).

The clinical history of the patient should include the family and personal medical history, the registry of the symptomatology, Of presentation and evolution of the pathology, besides other aspects.

The clinical examination should be based on a thorough examination of the symptoms present. In addition, it is also essential to A neurological examination, to determine the presence of nerve damage (de Peña Ortiz et al., 2007).

As for the complementary tests that are usually used, the cytology or the serological study of the virus, are essential for To determine the presence of an infection caused by varicella-zoster virus (de Peña Ortiz et al., 2007).

In the case of imaging tests, magnetic resonance imaging or Computed tomography , Are useful for Confirm the presence of neurological damage.

In addition to these, other complementary tests are also used, such as audiometries, brainstem evoked potentials, or Facial nerve electroneurography to assess the degree of auditory involvement and degree of facial nerve involvement (Boemo et al., 2010).

Treatments

The treatment used in Ramsay-Hunt syndrome focuses on curbing the infectious process, reducing symptoms and pain and, in addition, reducing the risk of development of 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 medicines that medical specialists Are the Zovirax, Famvir or Valtrex.

• Corticosteroids : They are often used in high doses for short periods of time to increase the effect of antiviral drugs. One of the most used is the Prednisone . In addition, steroids are also often used to reduce inflammation and thus the likelihood of medical sequelae.

• Analgesics: It is possible that the pain associated with the auditory hall is serious, reason why some specialists usually use drugs that contain Oxycodone or Hydrocodone , For analgesic treatment.

On the other hand, other non-pharmacological therapeutic interventions, such as surgical decompression (by Peña Ortiz et al., 2007), may also be used.

This type of surgical approach is still very controversial, it is usually restricted to cases that do not respond to pharmacological treatment in at least a period of more than 6 weeks (de Peña Ortiz et al., 2007).

Generally, the treatment of choice for most cases of Ramsay-Hunt syndrome are antiviral drugs and corticosteroids (Boemo et al., 2010).

Forecast

The clinical evolution of Ramsay-Hunt syndrome is usually worse than expected in other facial paralysis. Approximately, a set of cases ranging from 24-90% of the total, usually show significant medical sequelae (Boemo et al., 2010).

Although with good medical intervention, both facial paralysis and hearing loss are temporary, in some cases it may become permanent (Mayo Clinic, 2014).

In addition, muscle weakness resulting from facial paralysis prevents efficient closure of the eyelid and, therefore, external agents can cause eye injury. Eye pain or blurred vision may appear as one of the medical sequelae (Mayo Clinic, 2014).

In addition, severe involvement of the cranial nerves may also cause persistent pain, long after the resolution of the remaining signs and Symptoms (Mayo Clinic, 2014).

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. Of Peña Ortiz, A. L., Gutiérrez Oliveros, T., Guarneros Campos, A., & Sotomayor López, D. (2007). Ramsay Hunt syndrome. Rev Mex Dermatology , 190-195.
4. Facial Palsy UK. (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 . Retrieved from the National Organization for Rare Disorders.
8. Plaza-Mayor, G., López Estebaranz, J., López Lafuente, J., & Santos Granados, G. (2016). Ramsay-Hunt Syndrome . Retrieved from"Revista Clínica Española".
9. Image Source 1