The semicircular canal dehiscence is a rare disease. It causes a disorder of the organ of equilibrium in humans. The disease causes problems with hearing and keeping one’s balance.
What is semicircular canal dehiscence?
As a very recent disease, semicircular canal dehiscence was first described in America in 1998. It is a very rare disease in which there is a disorder of the organ of equilibrium. The disorder causes impairment of hearing and a sense of balance.
Due to its rare occurrence and its relatively recent existence, the disease has not yet been adequately researched. The doctors and researchers are therefore not yet able to comprehensively clarify important questions about the cause. It could be that the damage is hereditary. Further research results must be awaited. On the other hand, there is clarity with the existing symptoms. These are expressed in a disturbed sense of balance, a change in hearing and uncomfortable pressure on the ear.
Sick people report tinnitus. These are noises in the ear that are perceived without an external event. Tinnitus can be experienced as very stressful. A successful treatment option for semicircular canal dehiscence is performed through an operative intervention. The damaged semicircular canal is repaired or replaced so that the functionality of the organ of equilibrium is restored.
Causes
Abbreviated as SSCD by AbbreviationFinder.org, the semicircular canal dehiscence is triggered by changes in the equilibrium organ. This is located in the inner ear and consists of three semicircular canals. The semicircular canals are divided into the anterior, posterior and outer semicircular canals. Each of them is responsible for balancing out a different movement and thus for establishing equilibrium.
The semicircular canals are to be understood as spatial axes on which different movements are balanced. In the case of semicircular canal dehiscence, the structure of the anterior or upper semicircular canal is different compared to that of healthy people. The covering of the semicircular canal consists of a bony structure. This becomes thinner in the sick person or disappears completely in severe cases. So far it is unclear whether semicircular canal dehiscence can be inherited.
There is evidence for this theory, a confirmation or localization of the affected gene is still pending. There is agreement among the researchers that semicircular canal dehiscence can be triggered by external physical influences. If damaged in an accident, the floor corridor can suffer irreparable injuries.
Symptoms, ailments & signs
Symptoms of semicircular canal dehiscence include hearing problems and disorders of balance. With increasing concentration and exertion, the imbalance increases. As a result, unsteady gait occurs and simple locomotion becomes very problematic.
Processes like getting up or climbing stairs can hardly take place without help. The disease triggers hearing loss. The sound information recorded by the outer ear is reduced and passed on in a reduced manner in the inner ear. A hearing loss arises. At the same time, the patient perceives his / her own voice as being louder when speaking.
For this reason, it automatically reduces its own volume when speaking and is barely understood by other people. In parallel to the hearing loss, a sensitivity to loud noises develops. Sound that is measured at a volume of 70-80 decibels is perceived as loud by those affected. This corresponds to a volume just above the normal conversation volume. Other complaints include dizziness and ringing in the ears. There is an uncomfortable pressure in the ear.
Diagnosis & course
The diagnosis is made by a specialist in a hospital using high-resolution imaging procedures. The changes in the structure of the affected semicircular canal can only be adequately identified using computed tomography. Coincidentally, the disease can also be discovered and diagnosed while performing an operation on the ear.
The course of the disease can be classified as constant. The damage only affects the superior semicircular canal and does not spread any further. Further impairments are therefore not to be expected.
Complications
The semicircular canal dehiscence can lead to various complications. In most cases, however, the affected person’s hearing is severely impaired. Likewise, the patient can no longer easily keep himself in balance. The imbalance especially increases when the patient is very concentrated or under stress.
This restricts the affected person’s everyday life to a relatively large extent. Ordinary walking and standing are not possible. This often leads to dizziness and headaches as well. The quality of life decreases sharply due to the semicircular canal dehiscence. The reduced hearing ability also has a negative effect on everyday life.
However, it is possible to compensate for this symptom by using a hearing aid. There are no further complications. Due to the impaired hearing, the patient also speaks more quietly and is therefore poorly understood by other people. The ear also suffers from strong pressure, which leads to uncomfortable feelings.
In most cases, surgery is also possible to compensate for the semicircular canal dehiscence. There were no further complaints. The organ of equilibrium is also fully functional after the operation and shows no further damage.
When should you go to the doctor?
In the event of semicircular canal dehiscence, a doctor should always be consulted. In most cases, this disease does not self-heal, so treatment and diagnosis by a doctor is definitely necessary. The doctor should then be consulted if the person concerned is unsure about walking or maintaining balance. Consult a doctor, especially if you suddenly feel dizzy or feel insecure.
It is not uncommon for semicircular canal dehiscence to manifest itself in the patient’s increased sensitivity to loud noises or hectic movements. If these symptoms also occur, a doctor must also be consulted. High pressure in the ears can also indicate this disease.
If this occurs over a longer period of time in the affected person, a doctor should also be consulted. As a rule, semicircular canal dehiscence can be diagnosed by a general practitioner or an ENT specialist. Further treatment depends on the cause and extent of the semicircular canal dehiscence. In many cases, surgery is necessary to avoid further complications and damage.
Treatment & Therapy
The therapy of semicircular canal dehiscence takes place via an occlusion operation. This can be done in two different ways. The chosen practice depends on the damage. In the event of a total loss of the semicircular canal, the missing bone structure is completely replaced by alternative material in a surgical procedure.
This method is also chosen if there is uncertainty as to whether the existing semicircular canal could further diminish in its structure in the coming period. This would run the risk of it disappearing completely. Another treatment option is to place a plug in the semicircular canal.
This is then permanently glued. When gluing, the remaining bony layer of the semicircular canal is connected to the plug. Both measures have so far led to good results with patients. You were then able to hear normally again.
The organ of equilibrium also showed no further impairments. Further therapeutic measures are not yet known. After the recovery period of the operation, the organ of equilibrium is fully functional again very quickly.
Outlook & forecast
The semicircular canal dehiscence is progressive with a gradual increase in symptoms. Over a longer period of time there is a continuous breakdown of the bones in the semicircular canal, which progresses incessantly. Initially, the bony cover thins until it dissolves completely in the final phase. Without medical treatment, there are no prospects of a cure.
The symptoms can only be alleviated through medical care. There are several treatment methods for healing that are available to the patient and achieve individual success. When giving medication, the goal is to minimize pressure in the head. Beta blockers are combined with balance exercises and lead to a cure for the disease in some patients.
In addition, with progressive muscle relaxation according to Jacobsen and psychotherapy, techniques are taught that contribute to a reduction in internal pressure and general relaxation.
If the drug therapy option fails, surgical interventions are carried out. A distinction is made between two tried and tested procedures with comparably good results. Doctors refer to the methods as “roofing” and “plugging”. The first technique completely replaces the thinning bony layer. In the second procedure, a plug is installed in the semicircular canal, which is then glued to a layer of bone. However, both surgical interventions carry the risk of numbness.
Prevention
With the current state of science, there are no preventive measures. In everyday life, greater care can be taken to ensure that there is no physical impact on the ear. However, accidents can never be completely ruled out.
People with semicircular canal dehiscence can choose not to have any offspring. In these cases, no faulty gene is passed on. However, since it cannot be determined with certainty whether the disease is a genetic cause, this is a matter of speculation.
Aftercare
The extent to which follow-up care is necessary depends on the severity of the disease. Semicircular canal dehiscence is a progressive disease. In the initial stage, doctors usually prescribe medication to remedy the typical symptoms. Regular check-ups, including high-resolution computed tomography, are important.
Some doctors recommend progressive muscle relaxation. Some patients heal. The typical complaints disappear. This means that further scheduled examinations are unnecessary. If the drug treatment fails, the operation remains. Two procedures have been established for this.
If the balance and hearing problems have been resolved, no follow-up care is necessary. In rare cases, none of the measures leads to a cure. The only option left for the patient is to live with his illness. In order to normalize everyday life, doctors usually prescribe speech therapy. Everyday communication should be discussed in the sessions.
Hearing ability can sometimes be improved with a prescribed hearing aid. The loss of balance also leads to complications. Sick people can no longer cope with their everyday lives without an assistant. If an operation fails, numbness sets in. Depending on the severity of the hearing loss, further therapies may be necessary. In some cases, outpatient care is even advisable.
You can do that yourself
Many patients suffer particularly from hearing impairment. In particular, the noise sensitivity that can often be observed, which makes everyday noises in traffic or at work unbearable, is a major burden. Noise-dampening earplugs can help here as a first aid measure.
Since those affected often perceive their own voice 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 voice pitch for communicating with third parties. In many cases, the hearing impairment can also be corrected with a hearing aid, which even young patients should not hesitate to use.
Those affected, who particularly suffer from impaired sense of balance and therefore have difficulty walking, can at least prevent serious injuries if they fall by using an assistant or a wheelchair. The common headaches can be alleviated by autogenic training and other relaxation techniques.
According to current scientific knowledge, there are no preventive measures against semicircular canal dehiscence. However, as a precautionary measure, people with families with the disease are advised to avoid strong physical impacts on the ear, such as regular blows to the head in martial arts.