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Otosclerosis, or otospongiosis, is a peculiar dystrophic disease of the ear, affecting mainly the bone capsule of the labyrinth, clinically manifested by ankylosis of the stapes and progressive hearing loss. This disease affects 1% of the world's population, and mostly women.

Etiology and pathogenesis. Several theories of the development of otosclerosis are proposed. It is believed that the disease is inherited in an autosomal dominant manner. Otosclerotic foci are found in 40% of individuals who are carriers of various genetic defects. Some authors believe that otosclerosis is an abnormality of the constitution, manifested in the inferiority of the mesenchyme of the body. Characteristic is the progression of otosclerosis with hormonal changes that occur in the body of a woman during pregnancy. The hormonal factor can also explain the more frequent detection of otosclerosis in them. Although otosclerosis is considered an ear disease, it can cause abnormalities in a number of body systems (bone, vascular, autonomic, endocrine), which is manifested in the corresponding clinical signs. A number of authors consider the cause of otosclerosis neuro-trophic disorders, which extend to the external auditory canal, eardrum, and pharynx. This gave reason to K.L. Khilov to call the disease otodystrophy. Among the factors contributing to the development of otosclerosis, a prolonged exposure to noise is distinguished, in which, due to the inferiority of the labyrinth capsule, chaotic movement of molecules occurs, leading to a rush of blood and the progression of changes in bone tissue. Inflammatory and tumor theories are of historical significance only.

Changes in the bone labyrinth capsule begin in the bone marrow spaces, especially in places where there are embryonic cartilage residues. As a result of the increased activity of osteoclasts around the blood vessels, the bone tissue is decalcified and a delimited focus of the spongy (spongy) bone is formed, containing an excessive amount of bone marrow spaces rich in blood vessels. This phase of otosclerosis is called active. Subsequently, the newly formed, immature spongy bone is resorbed a second time and with the help of osteoblasts is transformed into a mature plate bone.

Most often, the primary otosclerotic lesion is localized between the cochlea and the vestibule, in the region of the oval window, especially directly anterior to the base of the stapes. When the process spreads to the annular ligament and the base plate of the stapes, ankylosis of the stapes occurs, characterized by conductive hearing loss. Much less frequently, foci are localized in the region of a round window, sometimes completely blocking it. Foci of otosclerosis can also spread to the cochlear receptor formations, leading to sensorineural hearing loss. Bilateral and symmetrical localization of foci of otosclerosis is more often noted.

Before the appearance of auditory disorders, they speak of the histological stage of otoclerosis, while from the moment tinnitus and hearing loss occur, the disease goes into the clinical stage.

Clinic. When examining a patient, attention is drawn to the gradual development of the disease, often the bilateral nature of hearing loss, hearing impairment in women due to pregnancy and the presence of otosclerosis in relatives.

At otoscopy, atrophy of the skin of the external auditory canals (Hilov's symptom) and tympanic membranes (Lempert's symptom) due to thinning of their fibrous layer, decrease in the sensitivity of the skin of the auditory canals (Fresels symptom) are noted. The auditory canals are wide (a symptom of Tillo-Verkhovsky), do not contain sulfur (a symptom of Toynbee-Bing), and are easily vulnerable. Through the atrophic eardrum, the auditory ossicles are clearly visible, and sometimes there is a hyperemic mucous membrane of the promontorium (Schwartz symptom), naeaaoaeunoao? Uay i pronounced activity of the process (“e? Aniue” ioineea? Ic). Sometimes there are exostoses of the external auditory canals (Mayer's symptom). In most cases, the eardrum has a normal appearance.

Dry skin of the body, brittle nails, excessive vulnerability of blood vessels, blueness of the sclera, pathology of the thyroid and parathyroid glands, pituitary gland, and gonads can be observed. A history of brittle bones, rickets, osteomalacia and other bone system pathologies are noted.

From laboratory data, a low content of calcium and phosphorus, proteins, and blood sugar is characteristic. A decrease in serum cholinesterase activity indicates a decrease in the tone of the autonomic nervous system. The content of ACTH increases, which also negatively affects osteogenesis.

Hearing impairment is usually noted at the age of 16-20 years. A characteristic and almost constant symptom is tinnitus, which often precedes hearing loss. At the beginning of the disease with conductive hearing loss, it is low-frequency, and as sensorineural hearing loss develops, it becomes high-frequency.

Hearing loss as a result of ankylosis of stapes occurs imperceptibly, progresses for many years. There may be periods of exacerbations, manifested by a sharp deterioration in hearing and increased noise in the ears. They occur under the influence of changes in the hormonal sphere in women during pregnancy, childbirth, and severe experiences. An unfavorable form of the disease is rarely observed, characterized by the rapid progression of sensorineural hearing loss.

At the beginning of the disease, patients complain of hearing loss in one ear, but bilateral hearing loss is objectively noted. By the nature of hearing loss and the clinical course, tympanic, mixed and cochlear forms of otosclerosis are distinguished, which are associated with the spread of the otosclerotic process in the ear labyrinth.

With the tympanic form of otosclerosis, ankylosis of the foot plate of the stapes is observed. Hearing is reduced in the type of impaired sound conduction. Negative experiments of Rinne and Federichi are characteristic. On the tonal audiogram, the thresholds for bone conduction are slightly increased (up to 20 dB), and for air conduction thresholds up to 50 dB, and the curve of air conduction has an upward character since the perception of low tones is more affected. Between the curves of air and bone conduction there is a bone-air interval of more than 20-50 dB. The larger the interval with almost intact bone conduction, the better the prognosis of the increase in hearing acuity during surgery for otosclerosis. FUNG is not detected. Speech intelligibility is not broken. The lower limit of perceived sound frequencies is increased to 50-80 Hz. The localization of the sound source in the vertical plane is broken. The vertices of tympanograms are reduced (tympanograms such as A1, A2), the acoustic reflection of the stapes is not recorded. The pressure in the tympanic cavity and the ventilation function of the auditory tubes are normal.

Often there is paracusis Willisii, which consists in improving speech intelligibility during vibration and noise influences, for example when riding in a tram, metro. This phenomenon is explained by the best sound conduction conditions with the swaying effect of vibration on the auditory ossicle system. Another explanation is the natural increase in speech intensity in people in noisy environments. Symptom is observed with symmetrical hearing loss.

A reliable sign of otosclerosis, indicating ankylosis of the stapes, is the negative experience of Jelle. With otosclerosis, there is no wave-like perception of a change in the sound volume of the tuning fork installed on the mastoid process with increasing and rarefying air pressure in the external auditory canal using a Politzer balloon. More precisely, the Jelle experiment is carried out using an impedance audiometer, when a change in the perception of sound volume by air conduction is determined with a decrease or increase in pressure on the eardrum.

The spread of otosclerotic foci to the round window of the cochlea and deep into it leads to the appearance of a sensorineural component of hearing loss, characteristic of a mixed form of otosclerosis.
Hearing in this case is reduced by a mixed type. Deterioration of bone conduction can be associated both with impaired mobility of the labyrinth windows, and with the direct effect of osteosclerotic foci on the cochlear nervous system. The bone-air interval in the tonal audiogram is reduced to 15-20 dB, and the bone and air conduction curves often go horizontally or acquire a descending character in the high-frequency zone. Thresholds of bone conduction increase to 30-40 dB. FUNG is periodically noted, indicating functional impairment of the sensitive cells of the Corti's organ. With speech audiometry, 100% speech intelligibility is achieved. The lateralization of sound in Weber's experience is often indefinite due to the mixed nature of hearing loss. Normal ultrasound sensitivity is maintained.

The cochlear form of otosclerosis is diagnosed with a decrease in tonal hearing, similar to impaired sound perception. This can be observed as a result of the sequential transition of the tympanic form to a mixed one, and then the cochlear form of otosclerosis with a long course of the disease. Sometimes hearing loss develops rather quickly, masking ankylosis of stapes, which is associated with increased activity of the otosclerotic process. There is no bone-air interval on the tonal audiogram and the curves have a downward view in the high-frequency zone. Thresholds of bone conduction can become higher than 40 dB. In contrast to the primary sensorineural hearing loss with the cochlear form of otosclerosis, the Rinne, Federici, Bing, and Jellé tuning fork tests are negative, 100% speech intelligibility is noted at high thresholds of bone conduction. FUNG is often detected, but auditory sensitivity to ultrasound persists. There is a high level of uncomfortable volume. The lower limit of perceived frequencies is increased to 60-80 Hz. The changes characteristic of otosclerosis are determined with impedanometry. Despite the audiological data, the cochlear form is sometimes difficult to distinguish from primary sensorineural hearing loss. In such cases, the totality of all clinical manifestations of the disease, including changes in blood composition, is taken into account, as well as radioimmunoassay, which allows the diagnosis and determination of the activity of the otosclerotic process by the nature of the distribution of the radiopharmaceutical in the bones of the skull and parenchymal organs (Artyushkina E.A., 1990).

Spontaneous vestibular disturbances in the form of slight dizziness and mild imbalance are relatively rare and atypical for otosclerosis. Hyporeflexia of caloric nystagmus is sometimes detected, which may be associated with inhibition of receptors of the vestibular apparatus. The ventilation function of the auditory tubes is usually not impaired. When radiography of the temporal bones, their pronounced pneumatization is noted. Computer and magnetic resonance imaging can determine the otosclerotic foci in the area of ​​the oval window, semicircular canals, internal auditory canal.

Surgical treatment of tympanic and mixed forms of otosclerosis. Of crucial importance for the appropriateness of the operation is bone conduction (“? Aca? Aa” oeeoee), ioaieaaaiia ii einoii-aicaooiiio eioa? Aaeo oiiaeuiie aoaeia? Aiiu. Oi? Ioeie eaiaeaaoaie aey iia? Aoee yaey? Ony eeoa n iioa? Ae neooa in bone conduction up to 15 dB, and in air - up to 50 dB. A contraindication to surgical treatment is an active poor-quality course of the osteodystrophic process, including “e? Aniue ioineea? Ic”.

There are several ways to restore the conduct of sounds to the spiral organ. In the beginning, a radical ear operation was performed for otosclerosis with the formation of a window in the bone wall of the lateral semicircular canal - Lempert’s ear labyrinth fenestration (Lempert D., 1938). The window was closed with a non-free meat-tympanal flap. In this case, sound waves enter the ear lymph through transpoantat, a new window in the semicircular canal, bypassing the fixed stirrup (Khilov KL, 1952).

In 1952, Rosen S. proposed an operation - mobilization of the stapes (direct and indirect), which gave a temporary improvement in hearing. Then Fowler (Fowler EP, 1956), Portman (Portmann M., 1958), A.I. Kolomiychenko (1958), V.F. Nikitina (1959), N.A. Preobrazhensky (1961) and others developed hearing repair operations - stapedectomy, stapes interposition and stapedoplasty.

Currently, with otosclerosis, endaural microoperation is used - stapedoplasty with autobone, auto-cartilage or synthetic materials. The essence of the operation consists in separating the eardrum with a skin flap of the ear canal, opening the tympanic cavity, releasing the foot plate of the stapes from the otosclerotic foci, removing the legs and head of the stapes. Then fenestration of the foot of the stirrup is performed. The hole with a diameter of 0.5-0.8 mm is closed with a thin flap of vein or fat, on which a prosthesis (usually Teflon) is installed, fixed on a long process of the anvil. Sound transmission is carried out along the chain of auditory ossicles through a prosthesis, a transplant that closes the window on the threshold of the labyrinth, to the ear lymph and the spiral organ.

Subject to indications for the operation and its technical perfection, there is a significant improvement in hearing after stapedoplasty in most of the operated patients (up to 80%). In many of them, subjective tinnitus is also eliminated. With a high-frequency and mixed spectrum of tinnitus noise, stapedoplasty is combined with resection of the tympanic string and tympanic plexus (Soldatov I.B. et al., 1984).

Surgical intervention is performed on the hearing loss of the ear. An operation on the other ear is performed no earlier than 6 months with a good functional result of the first operation. K.L. Khilov (1958) explained the improvement in hearing, including in the unoperated ear, not only by the restoration of sound conduction, but also by disinhibition of the auditory zone of the cerebral cortex, which for a long time did not receive impulses from the periphery and therefore was in a state of inhibition. Patients with a cochlear stage of otosclerosis require hardware hearing aid, since surgical treatment is not very effective.

Conservative treatment of otosclerosis is indicated in order to reduce the activity of the otosclerotic process and reduce tinnitus. It is mainly used for the cochlear form of otosclerosis or its adverse course. A dairy-and-vegetable diet with a salt restriction rich in vitamins is indicated. Calcium, phosphorus and fluorine preparations (calcium chloride, calcium gluconate, calcium glycerophosphate, phytin, phosphene, sodium fluoride) are used to seal the cancellous bone in combination with long-term intake of vitamin D3. With hormonal disorders, parathyroidin and testosterone are used.

Patients with otosclerosis are under the dynamic supervision of a part physician and a garrison otolaryngologist, are examined and treated in a hospital. They are operated on in the clinic of otolaryngology of the Military Medical Academy, some district and central hospitals.

Operated patients are exempted from physical training. During the year after the operation, it is recommended to avoid heavy physical exertion and significant drops in barometric pressure (flying on an airplane, caisson work, diving into the water, etc.).

At testimony, military personnel are examined according to Art. 40 of the order of the Ministry of Defense of the Russian Federation N 315 1995
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