Rumsay Hunt syndrome
The zoster oticus is a second disease caused by the varicella-zoster virus in the ears. He is a special form of shingles (herpes zoster).
An infection with the varicella-zoster virus is the first disease that causes chickenpox.
As the virus remains in the body of the infected person for a lifetime, a reactivation of the virus in about 20% of the infected persons can lead to a second disease called shingles.
The shingles occur predominantly in the area of the upper body in appearance. Occasionally, the pronounced pain and blistering characteristic of shingles may also occur in the pinna and / or external ear canal. In this case one speaks of the so-called Zoster oticus.
The trigger for a Zoster oticus is like the shingles the varicella-zoster virus, which belongs to the group of herpesviruses. Since the zoster oticus is only a reactivation of the nerve cells fixed in the nerve cells, the risk of infection is not so high.
In Zoster oticus, the viruses are only in the vesicle contents of the rash. Nevertheless, since the virus-containing vesicular fluid is contagious, avoid direct contact with it. Although vaccinated against the virus, vaccinated people can also develop a zoster oticus.
More than 90% of the population are infected with the varicella-zoster virus and contract first chickenpox (varicella). After that, they have a lifetime immunity to chickenpox. Up to 20% of the partial immune system, usually over 40-year-old adults, later develop shingles, which may be associated with a zoster oticus. In about 2/3 of the zoster oticus sufferers, the virus spreads to the facial nerve, a cranial nerve, which leads to paralysis of the mimic muscles ( facial nerve palsy). There are no gender differences.
The varicella-zoster virus is also referred to as human herpes virus-3 and belongs to the group of herpes viruses that can cause disease. It has a double-stranded DNA with lipid envelope and is found worldwide. It prefers nerve cells and can survive in nerve vertebrae in the spinal canal ( spinal ganglion ) for years. It is transmitted by droplet infection.
The first infection with the varicella-zoster virus leads to chickenpox. The Zoster oticus develops in a reinfection with the virus or by a reactivation of the actually inactivated virus in the body. These viruses migrate along nerve fibers in dorsal root ganglia, where they survive and can only be activated again after years or decades. Reactivation destroys a major portion of the spinal ganglia, causing acute pain, also known as zoster pain. The reactivation can be triggered by fluctuations or decrease in immunity to the virus due to age, suppression of the body's immune system ( immunosuppression ), for example, in transplant patients or immune system ( immune insufficiency ), for example, in HIV-infected (AIDS). Possible causes include violent vibrations of certain body parts, X-rays, UV rays, contact with toxic ( toxic ) substances or other infectious diseases such as rabies.
The virus can be transmitted from those suffering from shingles to the unprotected, who then get chickenpox. Similarly, a child suffering from chicken pox can transmit the virus to an adult, who then gets shingles.
Most of the first symptoms of zoster oticus are indefinite symptoms such as fatigue and tiredness.
The characteristic of the herpes zoster bubbles are found in zoster oticus on the auricle, on the earlobe, in the depth of the external auditory meatus and on the eardrum. They can also occur laterally on the neck, on the tongue and on the soft palate. The bubbles are pinhead to rice grain size, water clear, taut and pearly. They form on a previously developed, slightly raised and sharply circumscribed skin erythema ( erythema ) within two to three days. After two to seven days the contents of the blisters become purulent yellowish and the redness stops. After a week, the bubbles dry out and it forms a brownish / yellowish crust. After about two to three weeks the zoster heals again. However, scarring is common, leading to dark or light spots on the skin where the blisters were previously ( hyper- and hypo-pigmentation ).
Note: The reactivation of the varicella-zoster virus can also take place without any symptoms of the skin, which is referred to as herpes zoster sine herpete.
The so-called zoster pain has already been described above. This can occur in the zoster oticus in the ear, on the side of the face or on the neck. In 2/3 of the patients suffering from Zoster oticus, peripheral facial paralysis occurs in the first or second week of the disease, which is caused by the infection of the facial nerve by the virus. This results in a weak or complete paralysis of the facial muscles on one side of the face. The patients can not frown on the affected side, the eyelid closure is not or only partially possible and the corners of the mouth hang down. Often the facial paresis is accompanied by a half-sided taste disorder and impairment of salivary secretion.
Facial palsy can also occur in zoster colli.
Early signs of developing zoster oticus may be nonspecific general symptoms, such as:
Even dull, pulling pain usually burden the patient even before the visible skin symptoms.
These come in the course of about three days and appear at the beginning as a simple uplifting redness. Subsequently, the bubbles fill with a clear liquid. Sooner or later the bubbles will open, dry out and crust. Within two to three weeks, the skin symptoms should have subsided, in the event they have occurred at all.
The pain and blisters characteristic of shingles (herpes zoster) usually occur in the upper body area.
Occasionally, however, this complaint can also be found in the area of the head.
If the above-average pain and blisters in the area of the auricle and / or the external auditory canal occur, this is called a zoster oticus.
Scarring or pigmentation of the skin may be left behind if a secondary infection of the area has occurred, ie an additional infection has occurred, for example with a bacterium.
Even a so-called zoster gangrenosus can cause scarring, as in the course of the zoster gangrenous a skin (necrosis) takes place.
After more or less successful healing of the skin symptoms, the pain can persist as a zoster oticus symptom. This is called postherpetic neuralgia. Even ear pain, a hearing loss or a morbid hypersensitivity to sounds of normal volume ( hyperacusis ) may accompany the symptoms described above as symptoms of a zoster oticus. In addition, the sense of equilibrium may be affected. This can manifest itself, for example, with vomiting and vertigo.
Important for the diagnosis is the observation of the clinical symptoms of the patient, as the blisters and the redness in the area of the ear are characteristic of zoster oticus. Further diagnostics is usually necessary only in case of problems. It can be a pathogen detection. Direct virus detection is done by polymerase chain reaction (PCR) from affected tissue or vesicle contents. The PCR is a method of amplification of the viral DNA, if it should be included in the samples. Specific antibodies to the virus can also be detected in the body. However, this is not very meaningful that Zoster oticus is a reactivation of a virus already present in the body, ie the antibodies may have already been formed during the initial infection with chickenpox from the body's defense system.
The cerebrospinal fluid ( cerebrospinal fluid ) is inflammatory altered, that is, there are many more cells of the defense system and proteins than normally found in the fluid. Let's get the cerebrospinal fluid through a lumbar puncture. A hollow needle is introduced into the spinal canal in the spinal column and CSF is removed. In the blood, multinucleated giant cells can be found under the microscope ( blood smear ).
The herpes zoster typical blisters on the skin may also be an atypical sign of infection with herpes simplex virus. This form of herpes simplex mimicking the zoster is also called "Zosteriform herpes simplex". Conversely, an irregularly located zoster as a "herpetiform zoster" can also mimic a herpes simplex infection. The clinical picture of eczema herpeticatum, like the zoster oticus, contains vesicles arranged in groups, but these burst very soon. The eczema herpeticatum is caused by an additional herpes simplex infection in already existing chronic skin disease. Blistering also occurs in Hailey-Hailey's disease. In this skin disease, the blisters form due to an innate keratinization disorder of the skin.
Acute zoster pain can be similar to pain in a pulmonary blood clot (pulmonary embolism), heart attack, appendicitis or migraine.
The so-called guidelines are principles or courses of action on which physicians can orient themselves. The clinical picture, diagnostics and recommended therapies are included.
As the zoster oticus is a special form of shingles and can develop severe courses, the guidelines can help to identify and treat it correctly.
The guidelines strongly recommend that every patient undergo antiviral therapy in a zoster oticus. Therapy is necessary here because the zoster oticus affects the nerves that are responsible for hearing so the cranial nerves VII and VIII. So patients with zoster oticus can be deaf or deaf due to a lack of therapy. Therefore, it is important to correctly diagnose a zoster oticus.
According to the guidelines, the diagnosis is mainly based on the clinical picture. For specific diagnosis to check the hearing a so-called threshold audiometry can be done.
After the bubbles have dried, disinfectant ointments are applied to kill the pathogens ( antiseptic ).
The zoster oticus must be treated antivirally. The therapy should be started no later than 72 hours after the appearance of the skin symptoms. Usually aciclovir is used, especially if it is a heavy, complicated form of zoster oticus. This can be administered orally, ie in tablet form, or intravenously, by infusion. Brivudine, famciclovir or valaciclovir can also be used as oral therapy. The drugs mentioned belong to the group of antivirals . These are therefore active substances that specifically counteract the growth and proliferation of herpes viruses. They inhibit the buildup of viral DNA. The antiviral therapy often leads within hours to freedom from pain and improvement of the skin symptoms.
Painkillers ( analgesics ), antidepressants or medicines for epilepsy ( antiepileptic drugs ) such as gabapentin can be used against the zoster pain. For zoster neuralgia, tricyclic antidepressants such as amitriptyline are the best remedy.
For the treatment so-called antiviral drugs are used. These are medicines that stop the multiplication of viruses.
Preparations that can be considered for systemic therapy in people with an intact immune system ( immunocompetent patients ) are:
Famciclovir, valaciclovir and brivudine are administered orally, ie via the mouth, whereas aciclovir is either the possibility of oral ingestion or the possibility of intravenous administration, ie the administration into a vein.
Intravenous administration is preferable to oral administration because of higher levels of activity that can build up in the organism.
In terms of the effect on skin symptoms, the above medicines are equivalent. Pain in the course of the zoster oticus, however, are fought faster by famciclovir, valaciclovir and brivudine, according to studies.
In childhood and adolescence only acyclovir may be used. In people whose immune system is weakened or suppressed ( immunosuppressed patients ), only acyclovir is also administered into the vein. In immunosuppressed patients older than 25 years, oral famciclovir may also be considered.
In addition, the patient should be treated with local anesthetic ear drops due to the usually pronounced pain in order to prevent the permanent presence of pain in the future. Also, antibiotic ear drops should be considered as part of a local treatment, as there is the possibility of a bacterial superinfection, so an additional infection with a bacterium.
In a Zoster oticus there are many different homeopathic remedies that are recommended in certain phases. So the person affected should take Sulfur at the beginning, so even before the bubbles occur. However, as a zoster oticus usually only by a general malaise or pain in the area of the future rash noticeable, this phase is rather difficult to grasp.
If the bubbles are there and rather pus-filled, the intake of Anagallis arvensis is recommended.
In case of a clear vesicular fluid, Rhus toxicodendron should be taken.
In severe rashes and severe pain Rhus vernix is taken. In the aftercare, especially if the zoster oticus causes pain in retrospect, although the rash has healed, it is advised to take Kalmia latifolia.
Due to the serious irreparable damage that is to be feared, the Zoster oticus treatment should begin on time. The best course of treatment is within the first three days after the first symptoms have appeared.
The zoster oticus is a diagnosis that justifies inpatient hospitalization. In other words, the zoster oticus treatment described below should be done in the hospital.
If the symptoms of the Zoster oticus are not taken seriously and the illness is not treated adequately, then a hearing loss, which can lead to total deafness, threatens.
Peripheral fascia palsy is also a scared complication of zoster oticus. As part of a peripheral Fasfachisparese (nerve paralysis) is the muscles responsible for the facial expressions, on one half of the face weak or even completely paralyzed. The frowning of the forehead is also no longer possible on the side on which the VII. Cranial nerve ( facial nerve ) is impaired. In addition, the following is an expression of peripheral facial palsy:
The inner ear or the vestibulochochlear nerve may also be affected. This can lead to tinnitus, hearing loss to deafness, vertigo, nausea and vomiting. Other affected cranial nerves may be: trigeminal nerve, abducens nerve, hypoglossal nerve, and vagus nerve . The irritating symptoms of the vagus nerve are hiccups ( singultus ) and difficulty sleeping. Symptoms of infestation of the trigeminal nerve are sensitivity disorders ( sensibility disorders ) and there is a risk of damage to the conjunctiva, cornea ( cornea ) as well as the optic nerve ( optic nerve ), which can lead to vision problems. The special form in which the first branch of the trigeminal nerve is affected is called zoster ophtalmicus because of the involvement of the eye.
In addition, post-herpetic neuralgia can occur. It is a chronic pain syndrome that occurs in 10-15% of patients. In the area of the Zoster's spread, the patient has persistent or recurrent pain for over four weeks. The duration is indefinite, the pain is agonizing, it can even come to suicide. The likelihood that the neuralgia persists for a long time increases with age. It can be months to years.
Meningitis, which causes varicella-zoster virus ( zoster encephalitis ), or generalization ( zoster generalisatus ) is also a serious complication. The zoster generalisatus refers to an entire affliction of the nervous system. This type of zoster is life-threatening, but usually occurs only in people who have a weakened defense system (eg in AIDS patients). In addition, the infection with the varicella-zoster virus can lead to a death of the embryo during pregnancy.
Facial paresis is a facial paralysis and may have various causes, but often it is based on a previous zoster oticus.
As in a zoster oticus the facial nerve of the facial nerve may be affected by the reinfection by the virus, it can lead to damage to the nerve and thus facial paralysis.
The Facialisparese falls through a drooping corner of the mouth, lack of eyelid closure and lack of frown on the affected side. But it can also be weak, so you only see a change when you look closely.
Facial nerve palsy usually occurs only temporarily or can be reduced in size. In 80% of cases, however, it comes to an annealing. The risk that facial nerve palsy does not heal increases with age. Therefore, it is important to quickly identify and treat a zoster oticus in order to prevent such sequelae as facial nerve palsy.
If facial nerve palsy occurs, it can be treated with glucocorticoids and antivirals. In addition, a physiotherapy should be done in retrospect, so that the affected muscles are trained again.
Since the zoster oticus can only break out if an infection with the varicella-zoster virus has already taken place, a vaccination against chickenpox is very useful. The vaccine is one of the standard vaccines for children and adolescents. Women of childbearing potential should always be vaccinated, as any infection during pregnancy will harm the child. However, vaccination during pregnancy should be avoided.
The vaccine is a live vaccine ( Zostavax ) containing attenuated viruses. The body's defense system makes antibodies against the pathogens, kills them and becomes immune to the disease.
Patients who have a weakened immune system, especially bone marrow transplant recipients, should take acyclovir as prophylaxis during the period of severe immunodeficiency.
The prognosis of the zoster oticus is generally favorable, while its complications are poor. 2/3 of the infections heal without consequences and relapse of the disease is rare. Zoster neuralgia occurs in 10% to 15% of cases, and in more than 60 years, even in every other case, and lasts for months or even years. The paralysis of the facial nerve and the hearing disorders are often incomplete.
In immunocompromised persons, the course of the disease is severe and the prognosis bad. Deadly courses and complications occur.
An early or timely antiviral therapy significantly improves the prognosis.