Archway dehiscence is a rare condition. It causes a disorder of the organ of balance in humans. The condition triggers problems with hearing as well as maintaining balance.
What is arcuate dehiscence?
A very recent disorder, arcuate dehiscence was first described in America in 1998. It is a very rare disease in which there is a disturbance of the organ of balance. The disorder causes impairments in hearing as well as the sense of balance. Due to its rare occurrence and fairly recent existence, the disorder has not yet been adequately researched. As a result, physicians and researchers have not yet been able to comprehensively clarify important questions about the cause. It could be that the damage is hereditary. Further research results must be awaited. What is clear, however, are the existing symptoms. These are manifested in a disturbed sense of balance, a change in hearing and unpleasant ear pressure. Sufferers report tinnitus. These are ear noises that are perceived without an external event. Tinnitus can be experienced as very distressing. A successful treatment option for arcuate dehiscence is performed via surgery. This involves repairing or replacing the damaged arcuate duct so that the balance organ’s ability to function is restored.
Causes
Arcuate dehiscence is caused by changes in the organ of balance. This is located in the inner ear and consists of three arcades. The arcades are divided into the anterior, posterior, and external arcades. Each of them is responsible for balancing a different movement and thus establishing equilibrium. The arcades are to be understood as spatial axes on which different movements are balanced. In case of arcuate dehiscence, the structure of the anterior or superior arcuate is altered in comparison with healthy people. The cover of the arcade consists of a bony structure. This becomes thinner in the affected person or, in severe cases, disappears completely. So far, it is unclear whether arcuate dehiscence can be inherited. Evidence exists for this theory, but confirmation or localization of the affected gene is still pending. There is agreement among researchers that arcuate dehiscence can be triggered by external physical effects. If damaged in an accident, the floor gait may suffer irreparable injury.
Symptoms, complaints, and signs
Symptoms of arcuate gait dehiscence include problems with hearing and disturbances in balance. With increased concentration and exertion, the balance disturbance increases. As a result, gait unsteadiness sets in and simple locomotion becomes very problematic. Processes such as standing up or climbing stairs can hardly take place without assistance. The disease triggers hearing loss. The sound information received from the outer ear is reduced and diminished in the inner ear. Hearing loss sets in. At the same time, the patient perceives his own voice as louder when he speaks. For this reason, he automatically reduces his own volume when speaking and is hardly understood by those around him. Parallel to the hearing loss, a sensitivity to loud sounds develops. Sound measured at a volume of 70-80 decibels or more is perceived as loud by those affected. This corresponds to a volume just above the normal conversational volume. Other complaints include dizziness and ringing in the ears. There is an unpleasant ear pressure in the ear.
Diagnosis and course
The diagnosis is made by a specialist in a hospital using high-resolution imaging techniques. The changes in the structure of the affected arcuate can only be adequately detected by computed tomography. Coincidentally, the disease can also be discovered and diagnosed when performing surgery on the ear. The course of the disease can be classified as constant. The damage refers only to the upper arcade and does not spread further. Therefore, further impairment is not expected.
Complications
Arcuate dehiscence can lead to various complications. In most cases, however, the affected person’s hearing is severely impaired.Similarly, the patient can no longer keep his balance easily. The balance disorder increases especially when the patient is concentrating hard or is under stress. As a result, the affected person’s everyday life is relatively severely restricted. Ordinary walking and standing is not possible. This often also leads to dizziness and headaches. The quality of life decreases greatly due to arcuate dehiscence. Reduced hearing also has a negative effect on everyday life. However, it is possible to compensate for this symptom by using a hearing aid. In this case, no further complications occur. Due to the reduced hearing ability, the patient also speaks more quietly and for this reason is poorly understood by other people. The ear also suffers from a strong pressure, which leads to unpleasant feelings. In most cases, surgical intervention is also possible to compensate for the arcuate dehiscence. This does not cause any further discomfort. The organ of equilibrium is also fully functional after surgery and has no further damage.
When should you see a doctor?
In any case of arcuate dehiscence, a doctor should be consulted. In most cases, this disease does not heal itself, so treatment and diagnosis by a doctor is definitely necessary. The doctor should be consulted if the affected person shows insecurity in walking or in keeping balance. A doctor should be consulted especially in case of sudden dizziness and insecurity. Not infrequently, arcuate dehiscence is also manifested by the patient’s increased sensitivity to loud noises or hectic movements. If these complaints occur as well, a doctor must also be consulted. High pressure in the ears may also indicate this disease. Therefore, if this occurs over a longer period of time in the affected person, a doctor should also be consulted. As a rule, arcuate dehiscence can be diagnosed by a general practitioner or an ENT specialist. Further treatment depends on the cause and the spread of the arcuate dehiscence. In many cases, this requires surgical intervention to prevent further complications and damage.
Treatment and therapy
Treatment of archway dehiscence involves closure surgery. This can be performed in two different ways. The chosen practice depends on the damage. In the case of total loss of the archway, the missing bone structure is completely replaced with alternative material in a surgical procedure. This method is also chosen when there is uncertainty as to whether the existing archway could further reduce in structure in the coming period. Thus, there would be a risk that it could disappear completely. Another treatment option is the placement of a plug in the archway. This is then permanently glued in place. During bonding, the remaining bony layer of the archway is connected to the plug. Both measures have so far led to good results in patients. They were subsequently able to hear normally again. The organ of balance also showed no further impairment. No other therapeutic measures have been reported so far. After the recovery period of the surgery, the organ of balance is fully functional again very quickly.
Outlook and prognosis
Archway dehiscence has a progressive course with a gradual increase in symptoms. Over a prolonged period of time, there is a continuous degradation of the bone in the arcade that progresses unceasingly. Initially, the bony cover thins until it completely dissolves in the final phase. Without medical treatment, there is no chance of recovery. Relief from the symptoms can only be achieved through medical care. For a cure, there are several treatment methods available to the patient that achieve individual success. When administering medication, the goal is to minimize the pressure in the head. Beta blockers are combined with balance exercises and result in a cure for the condition in some patients. In addition, Jacobsen progressive muscle relaxation and psychotherapy are used to teach techniques that help reduce internal pressure as well as general relaxation.If the drug therapy option fails, surgical interventions are performed. A distinction is made between two proven methods with comparable success. Physicians refer to these methods as “roofing” and “plugging. In the first technique, the thinning bony layer is completely replaced. In the second procedure, a plug is inserted into the archway, which is then glued to a layer of bone. However, both surgical procedures carry the risk of deafness.
Prevention
Preventive measures do not exist at the current scientific level. In everyday life, increased care can be taken to avoid physical impact on the ear. However, accidents can never be completely ruled out. People who have arcuate dehiscence may choose not to have offspring. In these cases, no defective gene is passed on. However, since it is not certain whether the disease is a genetic cause, this is speculation.
Follow-up
The extent to which follow-up care is necessary depends on the severity of the disease. Arch dehiscence is a progressive disease. In the initial stage, doctors usually prescribe medication to stop the typical symptoms. Regular check-ups including high-resolution computed tomography are important. Some doctors recommend progressive muscle relaxation. In some patients, healing occurs. The typical complaints disappear. Further scheduled examinations thus become unnecessary. If drug treatment fails, surgery remains. Two procedures have become established for this purpose. If the balance and hearing problems could be eliminated, no follow-up care is necessary. In rare cases, none of the measures leads to a cure. The patient is left with the option of living with his or her condition. To normalize everyday life, doctors usually prescribe speech therapy. The sessions are designed to discuss communication in everyday life. Hearing can sometimes be increased by a prescribed hearing aid. Loss of the sense of balance also leads to complications. Sufferers can no longer manage their daily lives without a walker. If an operation fails, deafness sets in. Depending on the extent of the hearing loss, further therapies may be necessary. In some cases, even outpatient care is advisable.
What you can do yourself
Many patients suffer particularly from hearing impairment. In particular, the often observed noise sensitivity, which already makes everyday noises in traffic or at work unbearable, is a great burden. Noise-dampening earplugs can help here as a first aid measure. Since sufferers often also perceive their own voice to be too loud, they speak so softly that they can no longer be understood by those around them. With the help of a speech therapist, these people can relearn the correct pitch of their voice for communicating with others. In many cases, the hearing impairment can also be corrected by a hearing aid, which even young patients should not shy away from using. Affected persons who suffer particularly from the disturbance of the sense of balance and therefore have difficulty walking can at least prevent themselves from sustaining serious injuries in the event of a fall by using a walking aid or a wheelchair. Headaches, which are common, can be alleviated by autogenic training and other relaxation techniques. Preventive measures against arcuate dehiscence do not exist according to current scientific knowledge. However, people in whose families the
However, as a precautionary measure, people in whose families the disease has occurred are advised to avoid strong physical impacts on the ear, such as regular blows to the head during martial arts.
