Otosclerosis is a slowly progressive ear disease. It can lead to hearing loss or even deafness. The pathological changes are similar to inflammation and are often limited to the inner ear, which is located in the so-called petrous bone. In addition to the loss of hearing, otosclerosis can also cause ringing in the ears.
Here you will find further information and selected otosclerosis specialists and centers.
An ossicle ( stapes) is located in the petrous bone inside the ear. It transmits sound from the eardrum to the inner ear. The acoustic signals cause the stapes and the other two ossicles (malleus and incus) to vibrate. These are transmitted as impulses to the brain, which then processes them.
In otosclerosis, the temporal bone tissue dissolves and ultimately hardens. The stapes becomes increasingly unable to transmit the sound it receives to the inner ear.
At first only one ear is affected, later the second ear is also affected. For this reason, people who notice increasing hearing loss should consult an ear, nose and throat specialist as soon as possible.
Otosclerosis is an extremely rare disease. Most of those affected are between 20 and 40 years old. In isolated cases, the disease manifests itself in the form of sensorineural hearing loss.
One noticeable symptom that occurs in a large number of patients with otosclerosis is tinnitus. This technical term is used by doctors to describe noises in the ear such as
Otosclerosis sufferers only hear low-pitched tinnitus noises.
Some of them also experience dizziness. This is particularly the case if the inner ear is also involved in the disease.
Otosclerosis itself manifests itself in connective tissue-like growths. They initially surround the stapes footplate so that the stapes can no longer move unhindered (stapes ankylosis). The progression of the disease ultimately causes conductive hearing loss.
It is characteristic of the ear disease that the earlier it appears in the life of the person concerned, the faster it progresses. As the disease progresses, there may also be a short-term improvement in hearing.
The inner structure of the ear @ Henrie | AdobeStock
The ear, nose and throat specialist diagnoses ossification of the ear canal with the help of several hearing tests. This is how he finds out whether and to what extent the signal transmission to the inner ear is damaged.
In the early stages of otosclerosis, there are often no noticeable changes in the middle ear or the eardrum. This is why diagnosis in the early stages of hearing loss is extremely difficult, even for medical experts.
Some sufferers have Schwartze's sign. This is a reddish discoloration visible through the eardrum.
Imaging procedures such as
clearly reveal inflammatory processes in the temporal bone area. Current foci of inflammation can also be localized using a nuclear medicine examination (TCS).
The ENT specialist also carries out a stapedius reflex measurement: This shows the extent to which the ability of the stapes to pass on incoming acoustic signals is impaired.
Another test is the tuning fork test, in which the struck metal instrument is held against the temporal bones. The doctor can use this to compare whether the patient hears the sound transmitted in this way better than the normal acoustic stimulus.
With the help of speech audiometry (speech test), the specialist can determine whether spoken sentences and words are more difficult to understand.
The causes of inner and middle ear ossification have not yet been fully clarified scientifically. However, it is now almost certain that the disease has a genetic component. Scientists recently discovered the TGFB1 gene, which is always altered at the same location in otosclerosis patients.
Other possible triggering factors are viral infections (mumps, measles) and hormonal changes. The latter seem to have a major influence: In many patients, the disease occurs for the first time after pregnancy. In addition, existing otosclerosis worsens when oral contraceptives are taken.
According to some doctors, autoimmune reactions could also lead to otosclerosis. This is when the immune system mistakenly fights its own cells and tissue, mistaking them for foreign bodies that need to be eliminated.
Otosclerosis cannot be treated with medication. In patients with severe hearing loss, only surgery (stapedectomy) is helpful.
Surgery is always necessary if the patient can no longer understand speech at less than 30 decibels. Patients who prefer not to have an operation or for whom such an operation would not be successful (in the case of sensorineural hearing loss) can be prescribed a hearing aid.
Hearing aids improve hearing ability, but do not eliminate the cause of otosclerosis © Alexander Raths | AdobeStock
The doctor injects the local anesthetic into the external auditory canal. He then makes a tiny incision in the ear canal and folds the eardrum forward. This allows him to see the ossicles.
He removes the stapes or its base plate with a laser or surgical micro-cutlery. He then inserts a prosthesis or partial prosthesis and fixes the eardrum back in its usual position. The prosthesis/partial prosthesis is called a stapes-plasty. It ensures that the auditory ossicles vibrate better again.
Some otosclerosis sufferers still have to wear hearing aids after their operation. However, the operation is usually successful. Although hearing aids significantly improve hearing, they cannot stop the progression of the disease.
Another surgical procedure is called a stapedotomy. It has the advantage that there are fewer complications, as only the leg of the stapes is removed from the patient.
Using a sharp needle or laser, the otosclerosis specialist drills a tiny hole in the footplate of the stapes. There he places a prosthesis (piston) made of
and fixes it to the anvil.
During the procedure, the surgeon checks the patient's hearing performance several times. After the procedure, the patient should take it easy for about 2 to 3 weeks. If the operation was without complications, the patient can even fly a few months later.
The right contact for patients with otosclerosis is the ENT specialist. These specialists have completed 60 months of further training in the field of ear, nose and throat medicine after completing their medical studies. Specialist training includes all diagnostic and therapeutic treatments in the field of ear, nose and throat diseases.
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