As already mentioned, the labyrinth fluid and the main membrane belong to the sound-conducting apparatus. However, isolated diseases of the labyrinthine fluid or the main membrane are almost never found, and are usually accompanied by a violation of the function of the organ of Corti as well; therefore, almost all diseases of the inner ear can be attributed to the defeat of the sound-perceiving apparatus.

Defects and damage to the inner ear. To Birth defects include developmental anomalies of the inner ear, which can vary. There were cases of complete absence of the labyrinth or underdevelopment of its individual parts. In most congenital defects of the inner ear, underdevelopment of the organ of Corti is noted, and it is precisely the specific terminal apparatus of the auditory nerve, the hair cells, that is not developed. In place of the organ of Corti, in these cases, a tubercle is formed, consisting of nonspecific epithelial cells, and sometimes this tubercle does not exist, and the main membrane turns out to be completely smooth. In some cases, underdevelopment of hair cells is noted only in certain parts of the organ of Corti, and in the rest of the length it suffers relatively little. In such cases, it may be partially preserved auditory function in the form of islands of hearing.

In the occurrence of congenital defects in the development of the auditory organ, all kinds of factors that disrupt the normal course of development of the embryo are important. These factors include the pathological effect on the fetus from the mother's body (intoxication, infection, trauma to the fetus). A certain role can be played by hereditary predisposition.

Damage to the inner ear, which sometimes occurs during childbirth, should be distinguished from congenital developmental defects. Such injuries may be the result of compression of the fetal head by narrow birth canals or a consequence of the imposition of obstetric forceps during pathological childbirth.

Damage to the inner ear is sometimes observed in young children with head bruises (falling from a height); at the same time, hemorrhages into the labyrinth and displacement of individual sections of its contents are observed. Sometimes in these cases, both the middle ear and the auditory nerve can also be damaged at the same time. The degree of hearing impairment in the case of injuries of the inner ear depends on the extent of the damage and can vary from partial hearing loss in one ear to complete bilateral deafness.

Inflammation of the inner ear (labyrinthitis) occurs in three ways: 1) due to the transition of the inflammatory process from the middle ear; 2) due to the spread of inflammation from the meninges and 3) due to the introduction of infection by blood flow (with common infectious diseases).

With purulent inflammation of the middle ear, the infection can enter the inner ear through a round or oval window as a result of damage to their membranous formations (secondary tympanic membrane or annular ligament). In chronic purulent otitis media, the infection can pass into the inner ear through the bone wall destroyed by the inflammatory process, which separates the tympanic cavity from the labyrinth.

From the side of the meninges, the infection enters the labyrinth, usually through the internal auditory meatus along the sheaths of the auditory nerve. Such a labyrinthitis is called meningogenic and is observed most often in early childhood with epidemic cerebrospinal meningitis (purulent inflammation of the meninges). It is necessary to distinguish cerebrospinal meningitis from meningitis of ear origin, or the so-called otogenic meningitis. The first is an acute infectious disease and gives frequent complications in the form of damage to the inner ear, and the second itself is a complication of purulent inflammation of the middle or inner ear.

According to the prevalence of the inflammatory process, diffuse (diffuse) and limited labyrinthitis are distinguished. As a result of diffuse purulent labyrinthitis, the organ of Corti dies and the cochlea is filled with fibrous connective tissue.

With limited labyrinthitis, the purulent process does not capture the entire cochlea, but only part of it, sometimes only one curl or even part of the curl.

In some cases, with inflammation of the middle ear and meningitis, it is not the microbes themselves that enter the labyrinth, but their toxins (poisons). The inflammatory process that develops in these cases proceeds without suppuration (serous labyrinthitis) and usually does not lead to the death of the nerve elements of the inner ear.

Therefore, after a serous labyrinthitis, complete deafness usually does not occur, however, a significant decrease in hearing is often observed due to the formation of scars and adhesions in the inner ear.

Diffuse purulent labyrinthitis leads to complete deafness; the result of a limited labyrinthitis is a partial loss of hearing for certain tones, depending on the location of the lesion in the cochlea. Since the dead nerve cells of the organ of Corti are not restored, deafness, complete or partial, that arose after a purulent labyrinthitis, is persistent.

In cases where, with labyrinthitis, the vestibular part of the inner ear is also involved in the inflammatory process, in addition to impaired auditory function, symptoms of damage to the vestibular apparatus are also noted: dizziness, nausea, vomiting, loss of balance. These phenomena are gradually subsiding. With serous labyrinthitis, the vestibular function is restored to one degree or another, and with purulent labyrinthitis, as a result of the death of receptor cells, the function of the vestibular analyzer completely drops out, and therefore the patient remains unsure of walking for a long time or forever, a slight imbalance.

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Congenital malformations of the ear are found primarily in its outer and middle sections. This is because the elements of the inner and middle ear develop at different times and in different places, so with severe congenital anomalies of the outer or middle ear, the inner ear may be quite normal.

According to domestic and foreign experts, there are 1-2 cases of congenital anomalies in the development of the outer and middle ear per 10,000 people (S.N. Lapchenko, 1972). Teratogenic factors are divided into endogenous (genetic) and exogenous (ionizing radiation, drugs, beriberi A, viral infections - measles rubella, measles, chicken pox, influenza).

Damage is possible: 1) auricle; 2) auricle, external auditory canal, tympanic cavity; 3) external, middle ear and defect of the bones of the face.

The following malformations of the auricle are observed: macrotia (macrotia) - a large auricle; microtia (microtia) - a small deformed auricle; anotia (anotia) - the absence of the auricle; protruding auricles; appendages of the auricle (single or numerous) - small skin formations located in front of the auricle and consisting of skin, subcutaneous adipose tissue and cartilage; parotid (paraauricular) fistulas - a violation of the processes of closing ectodermal pockets (2-3 cases per 1000 newborns), typical localization is the base of the helical stalk, and atypical placement of the paraauricular fistula is also possible.

Anomalies of the auricle lead to a cosmetic defect of the face, often combined with underdevelopment or absence of the external auditory canal (Fig. 51, 52, 53). Microtia and underdevelopment of the external auditory canal can be combined with hypoplasia of the entire middle ear. There are a variety of options for underdevelopment of the auditory ossicles, the lack of connection between them, most often between the hammer and the anvil.

Rice. 51. Protruding ears

Rice. 52. Microtia and agenesis of the external auditory canal

Rice. 53. Microtia and ear appendages

Anomalies in the development of the external auditory canal and middle ear cause conductive hearing loss.

Treatment of congenital anomalies of the outer and middle ear is surgical and is aimed at eliminating a cosmetic defect and reconstructing the sound-conducting system of the outer and middle ear. Restoration of the external auditory canal is carried out in children under the age of 7 years, and the correction of a cosmetic defect of the auricle is closer to 14 years.

Surgical treatment of duck appendages. They are cut off at the base.

Paraauricular fistulas in themselves do not cause any discomfort (Fig. 54). Only infection and suppuration indicate their presence and require surgical intervention. After opening the abscess and eliminating the purulent process, the epidermal passage is completely removed. The opening of the abscess is only a temporary help, since relapses of suppuration are possible in the future.

Anomalies in the development of the auricle are relatively rare. Under the ugliness of the shell is meant a change in its shape, which, according to Marchand, depends on disorders of the "first formation", since in humans the normal formation of organs ends in the third month of uterine life.

It is possible that inflammatory processes in the genesis of deformities play a certain role; there are cases of deformation of the auricles and atresia of the external auditory canal, clearly resulting from intrauterine changes on the basis of congenital syphilis (I. A. Romashev, 1928) or other diseases

Because development of the human body continues after birth, then it is more appropriate to define the concept of "ugliness" as any developmental disorder. The deformities have nothing to do with individual variations of the auricle, which are usually common and therefore do not draw our attention.

Deformities right away rush in the eyes by cosmetic insufficiency that they create either by excessive size, or distance from the head, or a decrease in the size of the auricle, the presence of outgrowths, additional formations, underdevelopment of individual parts or the complete absence of an organ, splitting of the shell, etc.

Marx(Marx, 1926) divides all deformities of the auricle into two groups: ear deformities in normally developed individuals; these are primary deformities; deformities in persons having a general or local character; these are secondary deformities.

Among psychiatrists for some time, the idealistic views of Morel (Morel) dominated, who believed that a change in the auricle is a sign of mental inferiority (Morel's ear). Currently, it is believed that the anomalies of the auricle do not matter in assessing the mental state of the individual.

According to Vali, ear anomalies observed more often in men than in women; bilateral predominate over unilateral, and among the latter, left-sided. At present, it is considered proven that anomalies in the development of the auricle can also be observed in mentally healthy people.

According to research Fraser(Fraser, 1931), Richards (1933), and Van Alyea (1944), the narcotic, middle, and inner ear develop from different bases. The inner ear develops first. appearing as a result of invagination of the ectoderm, which separates from the epithelium to form a vesicle called the otocyst. The cochlea and the vestibular section (labyrinth) are formed from it.

In view of that the inner ear develops earlier than the middle and outer, its congenital defects usually occur without accompanying defects of the last two departments. Such a deformation is a labyrinth aplasia, which causes congenital deafness of the child. The external ear and Eustachian tubes develop from the posterior segment of the first branchial fissure.

Development of the auricle up to a certain period occurs regardless of the development of the external auditory canal and middle ear; therefore, an isolated malformation of the auricle can sometimes occur. However, more often underdevelopment extends to the posterior segments of the first branchial fissure, to the mandibular and hyoid gill arches, and then deformities of both the external auditory canal and the middle ear (tympanic membrane, auditory ossicles) are observed.

According to medical statistics, from 7 to 20 percent of people in the world have anomalies and malformations of the ear, which are less often called ear deformities when it comes to the auricle. Doctors note the fact that men predominate in the number of patients with similar disorders. Anomalies and malformations of the ear are congenital, resulting from intrauterine pathologies, and acquired as a result of injuries, slowing or accelerating the growth of this organ. Violations in the anatomical structure and physiological development of the middle and inner ear lead to deterioration or complete loss of hearing. In the field of surgical treatment of anomalies and malformations of the ear, the largest number of operations are named after doctors whose method has not undergone any new improvements throughout the history of the treatment of this type of pathology. Below are considered anomalies and malformations of the ear according to their localization.

auricle or outer ear

The anatomical structure of the auricle is so individual that it can be compared with fingerprints - no two are the same. The normal physiological structure of the auricle is when its length approximately coincides with the size of the nose and its position does not exceed 30 degrees in relation to the skull. Protruding ears are considered when this angle is or exceeds 90 degrees. The anomaly manifests itself in the form of macrotia of the auricle or its parts in case of accelerated growth - for example, the earlobes or one ear, as well as its upper part, may increase. Less common is polyotia, which manifests itself in the presence of ear appendages to a completely normal auricle. Microtia is the underdevelopment of the shell, up to its absence. The “sharp ear” of Darwin, who was the first to attribute it to the elements of atavism, is also considered an anomaly. Another manifestation of it is observed in the ear of a faun or the ear of a satyr, which is one and the same. The cat's ear is the most pronounced deformation of the auricle, when the upper tubercle is highly developed and at the same time bent forward and downward. Coloboma or splitting of the auricle or earlobe also refers to anomalies and malformations of development and growth. In all cases, the functionality of the organ of hearing is not impaired, and the surgical intervention is more of an aesthetic and cosmetic nature, as, in fact, with injuries and amputation of the auricle.

Back in the first half of the last century, studying the development of the embryo, doctors came to the conclusion that earlier than the middle and outer ear, the inner ear develops, its parts are formed - the cochlea and the labyrinth (vestibular apparatus). It was found that congenital deafness is due to underdevelopment or deformation of these parts - labyrinth aplasia. Atresia or occlusion of the ear canal is a congenital anomaly and is often observed together with other defects of the ear, and is also accompanied by microtia of the auricle, disorders in the tympanic membrane, auditory ossicles. Defects in the membranous labyrinth are called diffuse anomalies and are associated with intrauterine infections as well as fetal meningitis. For the same reason, a congenital preauricular fistula appears - a channel of several millimeters that goes inside the ear from the tragus. In many cases, surgery using modern medical technology can help improve hearing in middle and inner ear anomalies. Cochlear prosthetics and implantation are very effective.

1. Defects and damage to the inner ear. Congenital defects include anomalies in the development of the inner ear, which have various forms. There were cases of complete absence of the labyrinth or underdevelopment of its individual parts. In most congenital defects of the inner ear, underdevelopment of the organ of Corti is noted, and it is precisely the specific terminal apparatus of the auditory nerve, the hair cells, that is not developed.

Pathogenic factors include: effects on the fetus, intoxication of the mother's body, infection, fetal injury, hereditary predisposition. Damage to the inner ear, which sometimes occurs during childbirth, should be distinguished from congenital developmental defects. Such damage may be the result of squeezing the fetal head by narrow birth canals or a consequence of the imposition of obstetric forceps. Damage to the inner ear is sometimes observed in young children with head bruises (falling from a height); at the same time, hemorrhages into the labyrinth and displacement of individual sections of its contents are observed. In these cases, the mean ear and auditory nerve. The degree of hearing impairment in the case of injuries of the inner ear depends on the extent of the damage and can vary from partial hearing loss in one ear to complete bilateral deafness.

2. Inflammation of the inner ear (labyrinthitis). Inflammation of the inner ear occurs due to: 1) the transition of the inflammatory process from the middle ear; 2) the spread of inflammation from the meninges; 3) the introduction of infection by blood flow.

With serous labyrinthitis, the vestibular function is restored to one degree or another, and with purulent labyrinthitis, as a result of the death of receptor cells, the function of the vestibular analyzer completely drops out, and therefore the patient remains uncertain about walking for a long time or forever, a slight imbalance.

Diseases of the auditory nerve, pathways and auditory centers in the brain

1. Acoustic neuritis. This group includes not only diseases of the auditory nerve trunk, but also lesions of the nerve cells that make up the spiral ganglion, as well as some pathological processes in the cells of the organ of Corti.

Intoxication of the cells of the spiral ganglion occurs not only when poisoned with chemical poisons, but also when exposed to toxins circulating in the blood in many diseases (for example, meningitis, scarlet fever, influenza, typhoid, mumps). As a result of intoxication with both chemical poisons and bacterial poisons, all or part of the cells of the spiral node die, followed by complete or partial loss of auditory function.

Diseases of the auditory nerve trunk also occur as a result of the transition of the inflammatory process from the meninges to the nerve sheath during meningitis. As a result of the inflammatory process, the death of all or part of the fibers of the auditory nerve occurs and, accordingly, complete or partial hearing loss occurs.

The nature of the violation of the auditory function depends on the location of the lesion. In cases where the process develops in one half of the brain and captures the auditory pathways before they cross, hearing is impaired in the corresponding ear; if at the same time all the auditory fibers die, then there is a complete loss of hearing in this ear;

with partial death of the auditory tract - a greater or lesser hearing loss, but again in the corresponding ear.

Diseases of the auditory region of the cerebral cortex, as well as diseases of the pathways, can occur with hemorrhages, tumors, encephalitis. Unilateral lesions lead to a decrease in hearing in both ears, more - in the opposite.

2. Noise damage. With prolonged exposure to noise, degenerative changes develop in the hair cells of the organ of Corti, spreading to the nerve fibers and to the cells of the spiral ganglion.

3. Air contusion. The action of the blast wave, i.e. a sudden sharp fluctuation in atmospheric pressure, usually combined with the influence of a strong sound irritation. As a result of the simultaneous action of both of these factors, pathological changes can occur in all parts of the auditory analyzer. There are ruptures of the tympanic membrane, hemorrhages in the middle and inner ear, displacement and destruction of the cells of Corti's organ. The result of this kind of damage is a permanent impairment of auditory function.

4. Functional hearing impairment - temporary disorders of auditory function, sometimes combined with speech disorders. Among the functional hearing impairments is also hysterical deafness, which develops in people with a weak nervous system under the influence of strong stimuli (fear, fear). Cases of hysterical deafness are observed more often in children.