Otosclerosis is a degenerative disease of the inner and middle ear. Due to so-called bony changes in the petrous bone, sound transmission from the eardrum to the inner ear is impeded. The result is hearing loss, which can lead to deafness as the otosclerosis progresses.
What is otosclerosis?
Because otosclerosis can lead to deafness, an ENT physician should be consulted in a timely manner. Otosclerosis is a bony change of the temporal bone. The three ossicles, the malleus, incus and stapes, are connected to the petrous bone via the inner ear. In front of this arrangement is the eardrum, which transmits sound to the auditory nerve via the ossicles and the inner ear. In a healthy inner and middle ear, the ossicles are movably connected. In otosclerosis, ossification of the stapes occurs as a result of inflammatory and degenerative processes. This restricts the mobility of the stapes, whereupon sound is no longer or only partially transmitted. Hearing problems such as tinnitus occur. In the further course, the disease leads to hearing loss and finally to deafness. Otosclerosis generally occurs in both ears simultaneously and predominantly between the ages of 20 and 45.
Causes
Otosclerosis can have several causes, although a primary cause for the ossifications has not been clearly assigned to date. Since otosclerosis is often preceded by inflammatory diseases and viral infections, measles, rubella, and mumps may be a possible trigger for the disease. Furthermore, so-called autoimmune processes can cause otosclerosis. In this case, the body’s own immune system reacts allergically to its own body and fights it. Otosclerosis may also have a hereditary component. The disease occurs more frequently in families in which other family members already have the disease. The gene involved has not yet been deciphered, but studies show that children whose parents have otosclerosis are very likely to develop the disease as well. Another cause could be hormonal balance. Since otosclerosis mainly affects women, hormonal influences could trigger the disease. In particular, because pregnant women and women who take contraceptives (“birth control pills”) are more likely to develop otosclerosis.
Typical symptoms and signs
- Hearing loss
- Deafness (deafness)
- Complaints similar to a hearing loss
- Tinnitus
Diagnosis and course
Otosclerosis is diagnosed by the otolaryngologist. However, especially in the early stages of the disease, the diagnosis proves difficult. When the disease first begins, it has no symptoms, so several years may pass during which otosclerosis remains undetected. If otosclerosis is suspected, a hearing test is performed at the beginning. Furthermore, the stapedius reflex – the function of the middle ear muscles – is tested. With this test, pathological changes can be detected. A hearing test with a tuning fork is also performed. These tests are used to determine how severe the hearing loss already is. As a rule, a conductive hearing loss is diagnosed. A speech test shows whether the affected person already understands spoken words less well than a healthy person. Imaging methods such as X-ray, CT, MRI and scintigraphy are also used to confirm the diagnosis. With these examination methods, inflammations as well as bony changes can be detected, so that the stage of otosclerosis can be determined. The course of otosclerosis depends on several factors. The earlier otosclerosis is detected and treated, the more favorable the prognosis. If surgical treatment is performed in a timely manner, hearing loss can be significantly improved. If treated in the early stages, hearing loss can sometimes even be completely eliminated. If otosclerosis is not treated in time, hearing loss is the result, which can lead to complete deafness in the further course.
Complications
Due to otosclerosis, affected individuals primarily suffer from discomfort in the ears. There is a sudden hearing loss that occurs without any particular reason.Likewise, in the worst case, complete deafness of the affected person can occur if no therapy is initiated. Especially in young people, hearing loss can lead to severe psychological discomfort or depression and thus significantly reduce the quality of life. Furthermore, it is not uncommon for tinnitus or other ear noises to occur, which significantly reduce the quality of life. This can further lead to sleep problems, which can result in a general irritability and dissatisfaction of the affected person. However, the further course of otosclerosis depends greatly on its severity and the time of diagnosis. In many cases, otosclerosis can be treated completely, with no particular complications. Hearing aids can also be used to limit the symptoms of hearing loss. The treatment itself is performed by means of a surgical procedure and does not lead to further discomfort. The patient’s life expectancy is also not affected or reduced by the disease.
When should you go to the doctor?
Decreased hearing ability is cause for concern. A visit to the doctor is necessary as soon as certain frequencies from the environment can no longer be heard or there is a general reduction in hearing. If the affected person notices that he or she can no longer perceive sounds in everyday life as usual or that he or she can hear less in direct comparison to other people, a doctor should be consulted. In principle, it is advisable to have a check-up at regular intervals throughout life in order to be able to adequately assess the quality of hearing and to react immediately in the event of changes. If one-sided hearing or the development of ringing in the ears occurs, consultation with a doctor is recommended. In the event of an increase in symptoms or deafness, medical attention must be sought as soon as possible. Otherwise, there is a risk of lifelong deafness. Changes in behavior, increased risk of accident or injury, and irritability also indicate an irregularity. A visit to the doctor is advisable as soon as there is withdrawal behavior or an aggressive demeanor. Whistling in the ear, ringing in the ears or insomnia and headaches should be examined and treated. If the complaints occur suddenly and abruptly, a doctor should be consulted immediately. This is an acute although not life-threatening condition for which medical attention is needed as soon as possible.
Treatment and therapy
There is currently no drug treatment for otosclerosis. If surgery cannot be performed, a hearing aid may improve hearing. However, if the disease has progressed to the point of deafness, hearing aids may not help or may help only to a limited extent. Otosclerosis is treated by means of surgery. A distinction is made between two procedures, stapedectomy and stapedotomy. In stapedectomy, the stapes and the adjacent part of the stapes footplate are removed. The ossicle is then replaced with a stapesplasty (also called a prosthesis). The stapesplasty takes over the function of the stapes and transmits the vibrations of the sound. Stapedectomy is usually performed under local anesthesia (local anesthesia). In this case, the doctor can already check during the operation whether hearing changes are present. In a stapedectomy, the entire stapes is not removed, but only the stapes legs. In this procedure, a small hole is drilled in the stapes footplate and a small prosthesis is inserted, which is then attached to the anvil. This prosthesis (made of platinum Teflon) transmits the vibrations of the sound and thus improves the hearing of the affected person.
Outlook and prognosis
The further course as well as the prospects for recovery of patients affected by otosclerosis depend primarily on the timing and type of treatment. In general, there is a chance of at least partial regeneration of hearing abilities. Initially, despite medical countermeasures, hearing continues to decline or stagnates at the already diagnosed level. Without appropriate therapy, the risk of significantly reduced, acoustic perception is considered very high. As a result, long-term hearing loss and, in severe cases, deafness occur. Early surgical intervention significantly increases the likelihood of recovery. Approximately 90 percent of patients achieve a noticeable or complete reduction in symptoms.Following surgery, dizziness is a common complication. These impairments usually last only a few days. Sometimes dizziness can persist for a longer period of time. Rarely, the surgery remains without a desired outcome and leads to further hearing deterioration. Familial accumulation of otosclerosis can serve as a warning signal. However, a preventive examination is also advisable in the case of frequent occurrence of tinnitus or unexplained limitations of hearing. An ear, nose and throat specialist will detect corresponding changes in the auditory canal at an early stage and thus increase the prospect of a complete elimination of the symptoms. Severe progressions can be avoided by thorough control.
Prevention
Currently, there are no preventive measures to prevent otosclerosis. If there is a family predisposition, an otolaryngologist should be consulted regularly to have the sound transmission of the hearing examined. If ear noises such as buzzing, humming, etc. occur frequently, a doctor should be consulted for a thorough examination. If tinnitus has already been diagnosed, regular check-ups should be performed so that any otosclerosis can be detected and treated in time.
Follow-up care
After surgery, pain and complications should be avoided. In the aftermath of surgery, an initial hearing test is performed. In addition, wound care and wound healing are the main focus. Antibiotics are given to prevent possible infection. As part of the aftercare for the operation, the stitches and the tamponade are removed. Aftercare is provided by an ENT specialist. Getting used to a hearing aid takes time. By using the hearing aid not only voices but also sounds and noise in the background are amplified. Spatial hearing is no longer possible. Whenever possible, the hearing aid should be inserted and used. Close contacts should be involved in helping the patient to help himself. The patient’s social environment is to be informed about this health change. They should be shown optimal ways of communicating with the patient. In addition, aftercare is to be provided by a hearing care professional. He checks the technical functionality and the fit. If the patient suffers from the hearing impairment, an accompanying psychotherapeutic treatment can reduce the suffering and positively influence the acceptance of the restriction. In addition, patients can talk to other people with otosclerosis in a self-help group about how to cope with everyday life and about problems.
What you can do yourself
People who have otosclerosis can support medical therapy by taking various measures. First, regular monitoring of symptoms is important. The patient can create a complaint diary and note in it, for example, the hearing ability or pain in the area of the ear. In addition, the progress controls suggested by the doctor should also be taken advantage of, because otosclerosis can progress relatively quickly. Hearing ability can be improved with hearing aids and other aids. If the hearing ability continues to deteriorate despite all measures, the physician must be informed. Patients who suffer greatly from ostosclerosis are best to speak with a therapist. Although the condition is not life-threatening, deafness, dizziness and other symptoms can significantly impair quality of life. This makes close monitoring of symptoms and regular adjustment of medication all the more important. Finally, possible triggers for inner ear disease must be found. In a discussion with the doctor, it can be determined when the hearing complaints first occurred and in which situations they become more severe. It is then often sufficient to adjust lifestyle habits or to correct the hormonal balance with medication.
