GBOU "NIKIO im. L.I. Sverzhevsky" of the Moscow Department of Health

Causes Symptoms of otosclerosis Diagnosis Treatment of otosclerosis

Otosclerosis is a pathology of the middle and inner ear associated with the appearance of bone tissue in the area of ​​​​attachment of the stapes (the third, innermost auditory ossicle) to the oval window of the vestibule (the border of the middle and inner ear), as well as with the growth of the bone plates of the cochlea and semicircular canals. Otosclerosis occurs most often in women of childbearing age.

Otosclerosis in the area of ​​connection of the stapes with the oval window leads to disruption of the sequential transmission of sound waves to the cochlea - this is how sound transmission pathology develops. Bone growths in the cochlea interfere with the normal perception of sounds by the nerve endings of the auditory nerve - this is a pathology of sound perception or sensorineural hearing loss. When the semicircular canals are affected by otosclerosis, vestibular disorders develop (dizziness, nausea, unsteady gait, difficulty maintaining balance with a sudden change in body position).

Symptoms of otosclerosis

The main danger is the long asymptomatic period of otosclerosis. Imperceptible and gradual changes in the bone capsule can occur over 2-3 years, manifesting themselves only as a slight noise in the affected ear. At this stage of development of otosclerosis, changes can be detected during a routine examination by an otolaryngologist, during which audiometry is performed.

As the disease progresses and there is no treatment, the symptoms of otosclerosis appear as follows:

• Impaired perception of low frequencies while maintaining perception of high frequencies. In some cases, the sensitivity of the sound-receiving apparatus to high frequencies even increases. At this stage, patients complain of problems with understanding male speech, although the speech of children and women retains its clarity and clarity for them.
• Improving hearing acuity in noisy environments. This symptom is a subjective sensation of the patient, who mistakes the natural increase in the volume of speech of the interlocutor, who is trying to speak louder in a noisy environment, as an improvement in hearing.
• Violations of the functions of the sound-receiving apparatus leads to the fact that the simultaneous entry into the cochlea of ​​sounds from the external environment and acoustic waves transmitted through the soft tissues of the body impairs the perception of the former. Thus, a person may complain that when he moves, chews food, etc., his understanding of the interlocutor’s speech deteriorates significantly.
• As the disease progresses, the above-described symptoms “smooth out” - the perception of both low and high frequencies deteriorates, and the understanding of the interlocutor speaking in a whisper or in a full voice becomes approximately equivalent.
• One of the characteristic features of otosclerosis is the constant regression of hearing. Unlike a number of other diseases in which there is a periodic decrease or increase in hearing acuity, this does not happen with otosclerosis.
• Noise in the affected ear. This symptom is present in the overwhelming majority of patients with otosclerosis and is described by them as “rustling”, “white noise”.
• In addition to hearing disorders, patients with otosclerosis may develop neurotic disorders. The limitation in communication imposed by hearing loss leads to a decrease in a person’s social activity, which can cause him to become withdrawn and cause mood swings. Often, patients develop sleep disturbances caused by constant tinnitus.

Regardless of the causes of the disease and its manifestations, treatment of otosclerosis should begin as early as possible in order to prevent unwanted changes not only in the hearing organs, but also in the state of the nervous system.

Forms of the disease

There are several forms of otosclerosis:

• conductive - a violation of the conductivity of sound waves, amenable to surgical correction up to the restoration of hearing;
• mixed - not only the conduction function is disrupted, but also the auditory analyzer, and can be partially restored by acoustic perception;
• Cochlear otosclerosis is a complete loss of sound perception that cannot be corrected surgically.

The disease can develop at different rates - slowly, spasmodically and quickly.

Causes

The exact mechanisms of development of this disease are currently unknown. But the most likely causes of otosclerosis include the following factors:

• Hereditary predisposition. According to various estimates, from 27 to 40% of all patients diagnosed with otosclerosis have a family history of cases of this disease in relatives of the previous generation. In addition, many patients have genetic defects of various types.
• Endocrine and metabolic disorders. This hypothesis is supported by the fact that in women suffering from otosclerosis, deterioration is observed during pregnancy.
• Risk factors that increase the likelihood of developing otosclerosis include infectious diseases (in particular, measles), persistent acoustic injuries (working in conditions of industrial noise without the use of protective headphones, etc.), as well as vascular diseases, in which the blood supply to the tissues of the inner ear is disrupted.

The general state of health, as many researchers believe, is indirectly related to the development of otosclerosis. Without being the direct cause of this disease, any health disorder (a previous cold, severe stress, chronic gastrointestinal diseases, etc.) can activate the mechanisms directly responsible for the development of the disease. This theory deserves attention, especially from the point of view of patients with unfavorable heredity. So, if there are cases of otosclerosis in the family history, regular preventive examinations by an otolaryngologist and other specialists are a measure for the timely detection of the disease.

– How is the operation - stapedoplasty? How do patients tolerate the intervention?

The operation is considered microsurgical and high-tech. It is passed through a funnel under a microscope. The operation is performed under local anesthesia: we inject the ear into the ear canal - this is the only painful sensation. The surgical intervention lasts about 1 hour. Before the operation, we provide premedication: we administer sedatives and painkillers intravenously. As a rule, patients are half asleep after this. But after an operation under local anesthesia, a person can immediately evaluate the result: he suddenly begins to hear everything, which has enormous psychological significance for both the patient and the surgeon.

Classification of otosclerosis

Treatment of otosclerosis and its manifestations largely depend on the form of the disease. This pathology is usually classified depending on the type of impairment of sound perception and sound conduction:

Conductive otosclerosis.

In this form of the disease, only a violation of sound conduction is observed, which developed under the influence of the proliferation of bone tissue and a decrease in the mobility of the stapes. Treatment of this type of otosclerosis is one of the most favorable in terms of prognosis, as it allows you to restore the functions of the inner ear.

Cochlear otosclerosis.

This form of the disease is characterized by a progressive deterioration in sound perception. Surgical treatment of cochlear otosclerosis may be ineffective, especially if you consult a doctor late in the course of the disease. H3: Mixed otosclerosis.

This form of the disease combines the two previous ones - a decrease in hearing acuity is caused by both a violation of sound conduction and its perception. Treatment for this form allows the functions of the inner ear to be restored to a certain extent (to the level of bone conduction).

Treatment tactics are determined by the individual characteristics of the clinical case and the appropriateness of prescribing a particular method.

Depending on the causes of the disease (identified or suspected), reducing the rate of disease progression and treating otosclerosis can be carried out using a complex of medications.

Before and After Results

Next, we present several tone threshold audiograms of patients before and after surgery.

Audiogram of the patient before surgery on the left ear. The right ear was operated on 7 months ago. The pure-tone threshold audiogram shows conductive hearing loss of the second degree in the left ear (WHO) with the presence of a Carhart wave.

Audiogram of the same patient 15 days after piston stepoplasty

Audiogram of the patient before surgery. The tone threshold audiogram shows conductive hearing loss of the first degree in the left ear, mixed hearing loss of the fourth degree in the right ear (WHO). The first to be operated on is the worse hearing ear – the right ear.

Audiogram of the same patient 12 days after piston stepoplasty on the right ear

Treatment of otosclerosis

At the Research Clinical Institute of Otolaryngology named after. L.I. Sverzhevsky uses both innovative and classical methods of treating otosclerosis.

The survey uses a wide range of tools and methods aimed at obtaining detailed information regarding the severity of changes. Among them:

• otoscopy;
• microotoscopy;
• audiometry;
• study with a tuning fork;
• acoustic impedance measurement;
• indirect otolitometry;
• vestibulometry;
• CT scan of the brain;
• X-ray examination of skull bones, etc.

A preliminary comprehensive examination allows you to identify and evaluate all the features of the clinical picture, and create an individual treatment program that is most effective in each specific case.

To make an initial appointment and draw up a diagnostic plan, call 633-99-60.

Diagnostics.

An audiological examination serves as the foundation for diagnosing and clarifying the form of the disease. Tuning fork tests are carried out (the experience of Rinne, Weber, Jelle), tone threshold audiometry, and acoustic impedance measurements. A pure-tone threshold audiogram evaluates not only the hearing thresholds by air and bone conduction, but also the value of the bone-air interval, which is especially important for mixed hearing loss. Multislice computed tomography (MSCT) of the temporal bones (step 0.6-1 mm) is important in the diagnosis of otosclerosis. The method allows you to determine the localization, prevalence of lesions and the degree of activity of the pathological process (recording on MSCT).

– Can you remember any funny case related to otosclerosis?

I had a patient who heard virtually nothing for 10 years. The stapedoplasty operation gave a good result, and the man left the hospital in a great mood. When, a month later, he came to me for a follow-up examination, and I asked if there were any complaints about hearing, he hesitated and answered: “Rather, on the contrary, doctor! A turtle has been living at home for a long time, but I could not even imagine how noisy this animal is! She scratches with her paws, eats loudly, shuffles her shell along the floor, and before I thought that a silent creature was walking next to me.”

Source:

information project “Moscow is the capital of health”

– What should a patient be prepared for during the rehabilitation period?

Stapedoplasty surgery is associated with some limitations. Since we open the inner ear (and it contains receptors not only for the auditory, but also for the vestibular analyzers), patients may experience severe dizziness after surgery. Therefore, for a month we recommend avoiding loud noises, lifting heavy objects, driving a car or taking the subway. You can't fly on an airplane for another 3 months. A person stays in the hospital for 7-8 days, and then he is under outpatient observation for 3 weeks, during which time he is issued a sick leave certificate. Further recommendations depend on the nature of the patient’s activity.

To make an accurate diagnosis, use:

• audiometry;
• tuning fork study (identifying the difference in the sensation of sound vibrations through air and directly through tissue);
• diagnostics of sensitivity threshold to ultrasound;
• radiography (detection of pathologies of the auditory bones);
• MRI. With high detail from different projections, the condition and mobility of the auditory bones are assessed, the presence of inflammation and other changes in bone or connective tissue is revealed.

Causes and course of the disease

The causes of the disease have not yet been established.
There are many assumptions and hypotheses, among which are the influence of inflammatory and infectious effects. Some experts are inclined to the hypothesis of genetic inheritance of the disease according to an autosomal dominant type of inheritance, which is detected in individuals who are carriers of genetic defects. They also pay attention to hormonal changes in a woman’s body during pregnancy, among which hearing loss such as otosclerosis is often detected, which occurs as a result of sound or ultrasound trauma to the labyrinth. Histologically, the focus of otosclerosis in the bone capsule of the labyrinth is an irregular structure, which is penetrated by numerous vascular spaces that are activated under the influence of various endogenous (internal) and exogenous (external) factors, including surgical trauma to the “immature focus.” The lesions can be different: single and multiple, diffuse and limited, symmetrical. Most often they are found in the area of ​​the windows of the labyrinth, mainly in the window of the vestibule of the labyrinth.

If the otosclerotic lesion is located along the edge of the foot plate, and the stapes legs and annular ligaments are involved in the pathological process, then ankylosis (immobility) and conductive hearing loss (difficulty in conducting sound waves) develop. When otosclerotic lesions spread to the scalae cochlea, hearing loss with a sensorineural component develops.

Types of Hearing Damage

The outer and middle ears transmit sound; the inner ear perceives it. If there is isolated damage to the outer or middle ear, we are talking about conductive (transmission) hearing loss (hearing loss). If the disorder is associated with the inner ear, we are talking about sensorineural hearing loss, or cochlear neuritis. In cases where both the middle and inner ears are damaged, they speak of a mixed form of hearing loss.

Otosclerosis is an osteodystrophy of the temporal bone affecting the capsule of the labyrinth. More often it leads to fixation of the stapes, but can also lead to damage to the cochlea and other parts of the labyrinth. The etiology of the disease is unknown. Otosclerosis is a progressive disease that usually appears at a young age. Sometimes it is detected in very young people, more often in women. There are family cases. The risk of a child developing otosclerosis if one of the parents has the disease is 1 in 4. Hearing loss progresses to almost complete deafness; in more severe cases, sensorineural hearing loss predominates. The disease is usually bilateral.