Orientation reaction. The meaning of the orienting reaction in the Great Soviet Encyclopedia, BSE. Forms of animal behavior

Usher's syndrome is an autosomal recessive disease that manifests itself in congenital hearing impairment of varying degrees, progressive pigmentary retinal degeneration (Fig. 123), leading to a gradual narrowing of visual fields and blindness, and sometimes loss of a sense of balance. In the world 5-6% of total number people with any hearing impairment and 50% of deaf-blind people suffer from this syndrome. Clinical polymorphism is determined by genetic heterogeneity - 12 loci are known associated with different types of this disease. The most well-known form of Usher syndrome is caused by mutations in the MY07A gene (HSA11q13.5), which encodes myosin 7A.

Cruson's syndrome (craniofacial dysostosis) is an autosomal recessive disease caused by premature fusion of the coronal and sagittal sutures of the cranium. The syndrome is characterized by retraction of the orbits and bones of the cheeks, bulging eyes, a depressed nose bridge, and a protruding jaw (Fig. 124). Most patients have hearing loss. The phenotype characteristic of this syndrome develops due to mutations in the FGFR2 (HSA10q26) gene encoding fibroblast growth factor receptor 2.

Alport's syndrome is a combination of hereditary nephritis and deafness (Fig. 126). Symptoms from the excretory system appear at the age of 5-10 years. Gradual hearing loss usually starts earlier. Initially, this is a neurosensory hearing loss of high tones, then low tones, passing from sound-conducting to sound-perceiving hearing loss. Sometimes there is myasthenia gravis, loss of memory and intelligence. The sex-linked recessive form of this syndrome is caused by mutations in the a-5 gene of the type IV collagen chain - C01.4A5 (HSAXq22.3). The autosomal recessive form of Alport's syndrome is associated with mutations in the C0L4A3 and C0L4A4 genes (HSA2q36-q37), which encode the a-3 and a-4 chains of type IV collagen. The genetic nature of the autosomal dominant form is still less studied, but its association with mutations in the C0L4A3 gene is assumed.

Pendred's syndrome is an autosomal recessive disease that manifests itself as hearing loss and thyroid pathologies - nodular goiter and hypothyroidism. Children with this disease are characterized by progressive hearing loss, which usually begins at the age of three years, slow bone growth, skeletal disproportions - a large skull, relatively short limbs (Fig. 127). The genetic basis of the disease is mutations in the PDS gene (HSA7q31), which encodes pendrin, an enzyme present in the cochlea, thyroid gland, and kidneys.

Richards-Randle syndrome is an autosomal recessive disorder characterized by ataxia, hypogonadism, and sensorineural deafness. Patients develop profound dementia, progressive muscle atrophy, gonadal hypoplasia, underdevelopment of secondary sexual characteristics, ketoaciduria, and progressive sensorineural hearing loss from early childhood. The molecular genetic basis of this disease remains unknown.

Gervell and Lange-Nielsen syndrome is an autosomal recessive disease characterized by congenital deafness and cardiac arrhythmia - a prolonged QT interval, reflecting the processes of electrical excitation and recovery of the heart muscle. Often, patients have multiple heart defects, there is a high risk of syncope and sudden death. Clinical polymorphism, which manifests itself in a wide variation in the severity of the manifestation, is determined by various mutations in the ^N01 gene (HSA11q15.5), which encodes one of the potassium channel proteins.

Multiple lentigo syndrome (leopard syndrome) is an autosomal dominant disease, the minimum diagnostic signs of which are: multiple lentigo (pigmented spots 1-5 mm in size darker than freckles), pulmonary artery stenosis, moderate hypertelorism, genital anomalies, deafness (Fig. 128 ). The etiological cause is mutations in the PTPN1 gene (HSA12q24.1), which encodes non-receptor type 1 protein tyrosine phosphatase.

Waardenburg's syndrome is a hereditary disease that has the following symptoms: telecanthus (lateral displacement of the inner corner of the eye), heterochromia of the iris, a gray strand above the forehead, and congenital deafness (Fig. 129). Deafness is caused by disorders of the spiral (Corti) organ with atrophic changes in the spinal ganglion and auditory nerve. Treatment of deafness in this syndrome is ineffective. Waardenburg syndrome types 1 and 3 are caused by dominant mutations in the PAX3 gene (HSA2q35), which encodes a transcription factor of the PAX family. Penetrance of these mutations is incomplete, expressivity varies.

Rice. 129. Waardenburg syndrome

More on the topic Hereditary forms of deafness and hearing loss in childhood:

1. Medicines used in gynecology of childhood and adolescence

hearing features.

a brief description of

Types of hearing

Relative hearing - the ability to distinguish between sounds of different pitches and intervals.

Musical ear - with the ability to assess the duration of a sound (a sense of rhythm), to determine the intensity and timbre of a sound.

Absolute hearing - the ability to accurately determine the pitch of an individual sound.

Principle of audiometry

Audiometric study of hearing consists in determining the curve of the threshold of auditory perception depending on the height and intensity of the signal. Audiometry makes it possible:

determine the acuity of hearing within the full range of auditory perception of the human ear;

have an idea of ​​the relationship between bone and air sound conduction;

evaluate the point of physiological hypersensitivity to sounds, which is at a height of 2048 Hz.

What is a scotomat?

Cattle mats - hearing loss in the form of islands of deafness.

What is the essence of the loudness leveling phenomenon?

The phenomenon of loudness leveling is as follows: if a normal ear perceives a certain auditory stimulus, the intensity of which gradually increases, then the degree of audibility increases evenly; if the signal is amplified, then its audibility improves in proportion to the amplification. The loudness leveling phenomenon is pathognomonic for damage to the hair cells of the organ of Corti and is never observed in diseases of the conductive section.

Age-related changes in the outer ear.

The external auditory meatus is short and narrow, and is vertical at first. In children of one year, the external auditory canal consists of cartilage tissue, and only in subsequent years does the base of the external auditory canal ossify. The tympanic membrane is thicker than in adults and is located almost horizontally.

Age-related changes in the middle ear.

The middle ear cavity is filled with amniotic fluid, making it difficult for the ossicles to vibrate. Gradually, this fluid is absorbed and instead of it, air enters from the nasopharynx through the auditory (Eustachian) tube. The auditory tube in children is wider and shorter than in adults, which creates special conditions for the entry of microbes, mucus and fluid during regurgitation, vomiting, runny nose into the middle ear cavity. This explains the rather frequent inflammation in children of the middle ear - otitis media.

Age-related changes in the perception of pitch.

Newborns have low auditory sensitivity, which by 7-8 days of life increases so much that conditioned reflexes to sound stimuli are formed. By the end of the 2nd and the beginning of the 3rd months, hearing becomes distinct. In the first 3 months, children react by blinking to a loud sound, older than 3 months - by blinking to speech. From 6 months old, babies listen to sounds. However, at birth, the hearing organs are not yet fully formed. The walls of the auditory canal ossify by the age of 10, and the development of the hearing organs as a whole ends completely only by the age of 12.

Age-related changes in hearing acuity.

The distinction of sounds, with a difference between them of 17 musical tones, is found in children at 3.5 months; for 13 - 14 tones - in 4.5 months; for 7 - 10 tones - at the end of the 5th month. Further, the accuracy of the analysis of sounds increases rapidly. On the 6th month, differentiations in 3-5 tones are formed, on the 6th-7th - in 1-2 tones. By 6 - 7 months, the subtlety of a child's hearing almost corresponds to the norm of an adult. The upper limit of hearing in children is higher than in adults, and reaches 22 thousand Hz, and sometimes even up to 32 thousand Hz. The maximum hearing acuity at 14 - 19 years old is determined by the smallest value of hearing thresholds; in children 7-13 years old and in young people over 20 years old, it is lower. The time threshold of the auditory analyzer decreases with age. At 8 - 10 years old it is 12 - 15 ms, at the age of 25 - 3 - 5 ms, i.e. 3-5 times less.

Hearing for words in children is lower than for tones, and lower than for adults. At 6.5 - 9.5 years old, the hearing threshold for high-frequency words is 17 - 24 dB, and for low-frequency words 19 - 24 dB, in adults for low-frequency words - 7 - 10 dB. With bone conduction of sounds, the threshold of audibility of frequencies of 10 - 12 thousand Hz almost does not change from 7 to 39 years. The upper limit of bone conduction is the largest from 11 to 15 years old (more than 25 thousand Hz), and in children 6-10 years old it is less (19 thousand Hz).

Pathology of the organ of hearing.

a brief description of

Deafness classification

Deafness is a complete loss (complete deafness) or the most severe degree of hearing damage, in which the remains of hearing remain, allowing you to perceive very loud non-speech sounds (whistle, beep, etc.) or well-known words uttered in a loud voice near the ear (partial deafness ).

Deafness can be bilateral or unilateral.

There are congenital and acquired deafness.

Hearing impairment can occur in connection with pathological changes: sound-conducting and sound-receiving departments. Depending on the localization of the pathological process in the hearing aid, there is also a third type of disorders, namely, diseases associated with impaired function of both the sound-conducting and sound-receiving departments. Thus, it is customary to distinguish between deafness (or hearing loss):

1) conductivity;

2) perception;

3) mixed.

Conduction deafness characteristic

Conduction deafness occurs when acoustic vibrations are delayed if an obstacle arises in their path or the acoustic resistance of the medium increases. Such

the phenomenon can be observed in the presence of a foreign body or sulfuric plug in the external auditory canal, with its congenital infection, with acute inflammation of the middle ear, with immobility of the auditory ossicles (otosclerosis), with increased pressure inside the labyrinth, etc.

In cases of conduction deafness, the low register of the audiometric scale decreases. The threshold audibility of the middle and high register can be at the level of the physiological norm.

Bone conduction is better than air conduction.

The defeat of the sound-conducting department of the hearing aid is manifested in a violation of the auditory perception of low frequencies and an improvement in the auditory perception of high frequencies. Low vowels s, y, o are worse for patients than high vowels and, e, a. Consonants, which include low frequency components: b, m, c, e, d, etc., patients hear worse than consonants characterized by high frequency components: s, g, w, h, u.

The auditory perception of low tones is impaired and amounts to 40 dB for 128 Hz, 50 dB for 256 Hz, and 65 dB for 512 Hz.

Characteristics of deafness of perception

Deafness of perception occurs when the organ of Corti and the endings of the fibers of the cochlear branch of the auditory nerve are damaged. Usually there is a significant hearing loss, and often complete deafness. The auditory perception of high tones is very limited or completely absent, and since the frequency components of the articulated sounds belong to the high register of the tonal scale, the auditory perception of speech sounds deteriorates sharply or is completely absent. This mainly applies to young children who have the association processes of articulated speech in higher centers. nervous system not yet fixed.

In cases of congenital deafness of syphilitic origin or associated with degenerative processes in the inner ear, the lesion of the hearing aid is often bilateral, although there are, as a rule, quantitative differences between the right and left sides. The disease process is often irreversible. Auditory perception is usually limited to three or four tones: 128, 256, 512 and sometimes 1024 cycles/sec. Bone conduction is impaired. The bone conduction curve lies below the air conduction curve (“positive Rinne”), the loudness leveling phenomenon is positive and pathognomonic for the organ of Corti lesion.

In the study of air conduction, and more often bone, islands of deafness (scotomata) are found. This symptom is pathognomonic for lesions of the organ of Corti.

on the. In children, it is sometimes necessary to observe a decrease in hearing of a functional nature in diseases localized in the sound-conducting section of the hearing aid. These types of hearing impairments are treatable. Low vowels: s, u, oh, patients hear better than high ones - and, uh. Patients do not hear well or do not hear consonants at all, characterized by high component frequencies (s, s, c, w, w, h, etc.). The consonant p is the best audible, in all likelihood, due to the sensation of vibration, the worst thing for patients to hear, in addition to those listed above, is the sound - l.

Pathology of the vestibulocochlear nerve

Hearing disorders caused by pathological changes in the trunk of the vestibulocochlear nerve. It is extremely difficult to analyze hearing impairments caused by pathological changes in the vestibulocochlear nerve, especially when they are associated with pathological changes in the organ of Corti or diseases localized in the base of the skull.

Inflammation of the vestibulocochlear nerve is observed quite often with various kinds of infectious diseases, and especially with influenza. The resulting deafness in its nature resembles deafness associated with a disease of the sound-conducting department, however, there is no symptom of loudness equalization, there is also no insular hearing impairment, the audiogram of threshold auditory perception is characterized by a drop in the curve in the high-frequency region, the bone conduction curve lies below the air conductivity. In cases of pathological changes in the vestibulocochlear nerve, treatment can achieve a significant improvement in the condition, while damage to the organ of Corti is the cause of persistent and irreversible hearing changes.

Deafness of central origin

Deafness of central origin. This form of deafness develops in diseases of the central nervous system. Hearing loss is characterized by: common features:

1) they are always double-sided;

2) the decrease in audibility concerns both low and high tones;

3) there is no loudness leveling phenomenon;

4) it is not possible to detect insular hearing loss;

5) the ability to distinguish the height of acoustic stimuli, their intensity, as well as the duration (rhythm) is lost, in connection with this, speech disorders: melodic, dynamic and rhythmic;

6) with a rather large residual hearing in relation to simple tones, as well as the sounds and noises of musical instruments, a significant decrease in the audibility of articulated sounds is noted, and in connection with this, it becomes difficult to understand colloquial speech; this applies primarily to hearing impairments of cortical origin.

Characteristics of mixed deafness

This type of deafness is especially common. Mixed deafness occurs more frequently in children than in adults. Diseases of the sound-conducting department lead to degenerative changes in the organ of Corti. Hearing impairments are initially functional in nature, but over time they become fixed, eventually leading to mixed deafness. The air conduction of all tones deteriorates, and the decrease in the threshold audibility of high tones is more pronounced. Bone conduction of low tones can improve,

however, in relation to high tones, it is sharply reduced, and in some cases, patients do not hear high tones through the bone at all.

One of the most common forms of mixed deafness, best studied by otologists and relatively easy to diagnose, is that in which the disease is localized in the organ of Corti. The phenomenon of loudness equalization is positive, and the presence of persistent islands of deafness confirms the diagnosis.

Bilateral mixed deafness is the most severe form of hearing loss. The audibility of articulated sounds, consisting of both high and low formants, is impaired. In advanced cases, patients lose the ability to understand spoken language.

Deafness due to pathological changes in the outer ear

Pathological changes in the outer ear (inflammatory conditions, bone tumors, sulfur plugs, foreign bodies) cause conduction deafness. Congenital fusion of the external auditory canal with congenital deformities of the auricle is often accompanied not only by the deformation of the tympanic cavity, but also by the underdevelopment of the inner ear. In these cases, deafness is of a mixed type.

Deafness due to pathological changes in the middle ear

A significant role in the pathology of hearing is played by inflammation of the interosseous joints, which can lead to limited mobility of the auditory ossicles and is often accompanied by impaired function of the inner ear. The immobility of the ossicular chain adversely affects the conduction of low tones; complications from the inner ear, which may be functional, cause a violation of the perception of high tones. An extremely complex picture of mixed deafness arises, which is not always easy to analyze.

Pathological processes occurring in the upper part of the tympanic cavity, especially in the posterior part, are the biggest obstacle to acoustic stimuli and cause a much greater hearing loss than processes localized in the middle and lower part of the tympanic cavity. Due to chronic inflammation of the middle ear, accompanied by necrosis and destruction of the bone wall of the tympanic cavity and auditory ossicles, there are very deep hearing impairment. Prolonged superficial inflammatory processes can lead to the development of hypertrophic changes in the mucous membrane, which manifests itself in the formation of polyps, which sometimes fill the entire lumen of the tympanic cavity and even the external auditory canal, which causes a further deepening of conduction deafness. Each form of middle ear inflammation can be complicated by diseases of the inner ear. Deafness in such cases has a mixed character, conductive-perceiving.

The reasons for the transition of the painful process to the inner ear are:

bone destruction and in particular the formation of cholesteatoma;

2. spread of infection through the blood vessels;

3. damage to the auditory cells of the organ of Corti, caused by a decrease in the conductivity of auditory stimuli in the middle ear.

Eustachian tube obstruction

Violation of the patency of the Eustachian tube is the root cause of changes in the middle ear. It may arise due to:

1) edema on allergic grounds. In this regard, the regulation of air pressure in the tympanic cavity is disrupted, which in turn limits the mobility of the tympanic membrane and auditory ossicles;

2) blockage of the lumen with liquid food. This occurs in cases of congenital malformations of the palate, as well as dysfunction of the closing pharyngeal ring;

3) the spread of the inflammatory process from the upper pharynx, which causes a narrowing of the lumen of the Eustachian tubes. This, in turn, prevents the normal regulation of air in the middle ear, as well as the outflow of the separated mucous membrane.

The degree of damage to the middle ear in such cases is different. However, weakened sound signals reach the organ of Corti, which in turn has an inhibitory effect on the cerebral cortex. delayed mental development child and language development. There is also a negative effect on the psyche.

Characteristics of otosclerosis

Otosclerosis is characterized by bilateral damage to the inner ear, and the intensity of the disease process is usually different. Noise in the ears is an extremely unpleasant and annoying moment for the patient, contributing to a deeper hearing loss. In quiet conditions, the degree of hearing impairment increases, in noise it decreases. Remission periods can last very long, sometimes several years. The reason for an unexpected deterioration in the condition can be a number of factors: fatigue, severe and prolonged illness, etc., and in women - pregnancy, childbirth, feeding a child and menopause. The highest stage of development of the disease occurs between 35 and 45 years.

Symptoms of imbalance are extremely rare.

When otoscopy fails to note significant deviations from the norm. The mobility of the tympanic membrane can be impaired in cases where the otosclerotic process extends to the auditory ossicles. The patency of the Eustachian tube is preserved, blowing is not accompanied by an improvement in the condition. On x-ray of the temporal bone, the bone structure of the mastoid process looks completely normal; his pneumatization is not disturbed. The results of the study of hearing depend on the localization of the otosclerotic process. Both conduction deafness and mixed type deafness are equally common, while perceptual deafness and loudness leveling phenomena are never observed. Air sound conductivity is usually evenly reduced, although the audibility of low tones is more impaired. Very characteristic of otosclerosis is the improvement in hearing through the bone.

Prevention of otosclerosis

The goal of prevention of otosclerosis is to delay the development of the disease, which, if state of the art medical science is incurable. Colds, overwork should be avoided, and women should avoid re-pregnancy, especially in cases where hearing loss has occurred after childbirth, or otosclerosis has manifested itself in some other symptoms. Otosclerosis is an indication for abortion. Treatment is exclusively surgical.

Traumatic deafness

The issue of trauma-induced deafness refers to mechanical damage to the hearing aid due to skull trauma, damage to the hearing aid due to acoustic trauma, and damage to the hearing aid due to sudden changes in atmospheric pressure.

Fractures of the pyramid of the temporal bone occur much more often than one might think. They can cause deafness, which often appears long after the injury.

Skull fractures can be longitudinal or transverse. In the first case, it is mainly the tympanic cavity that is damaged, in the second case, the inner ear is damaged. Often there are hemorrhages in the tympanic cavity and the inner ear, leading to hearing loss.

Deafness classification childhood

From the point of view of etiology, childhood deafness is divided into three groups:

1) hereditary,

2) congenital,

3) acquired.

Characteristics of hereditary deafness

Cases of hereditary deafness are much less common than congenital and acquired. We talk about hereditary deafness when it is observed in several members of the same family or as a pathological trait is transmitted from generation to generation. A detailed study of hearing very often reveals deafness in members of such a family, however, in some cases of a very slight degree. Often there are insular hearing defects, characteristic of degenerative processes in the cochlea. With hereditary deafness, accompanied by underdevelopment of the bony labyrinth, there is a violation of the function of the organ of Corti.

Hereditary deafness can be either dominant or recessive.

In the first case, deafness is accompanied by other hereditary diseases, such as poly- and syndactymia, etc., which is never observed in cases where deafness is a recessive trait.

Deafness of the recessive type may not appear in every generation, which is the cause of diagnostic difficulties. With this type of deafness, the amount of residual hearing is usually so small that in practice a person can be considered completely deaf. In exceptional cases, persons with hereditary deafness can hear ordinary conversational speech at a distance of 1 meter.

Characteristics of congenital deafness

Congenital deafness is caused by:

1) underdevelopment of the hearing aid during fetal life. A delay in the development of the hearing aid occurs when a negative factor begins to act already in the first half of pregnancy, i.e. around 4-5 weeks.

2) damage to the hearing apparatus of the fetus in the womb.

Hearing damage can occur in the second half of pregnancy, ie. starting from the 5th or 6th month of intrauterine development.

Congenital deafness most often refers to the type of perceptual deafness, conduction deafness is observed only in 7% of cases and is caused by congenital infection of the external auditory canal and deformation of the auricle.

Causes of congenital deafness

Causes of congenital deafness can be:

1) illness of the mother during pregnancy,

An infectious and especially viral disease that a woman suffers during pregnancy can lead to damage to the fetus, which sometimes manifests itself in a lesion

hearing aid. If the mother suffers from an infectious disease between 6 and 12 weeks of pregnancy, then the damage to the hearing aid can be expressed in the underdevelopment of the cochlea and its nerve elements. Rubella leads to the development of bilateral deafness due to pathological changes in the cochlea, as well as in the sac of the vestibule. Other infectious diseases carried by the mother, such as tonsillitis, typhoid, paratyphoid, and infectious jaundice, have a negative effect on the development of the fetal labyrinth. In cases where the mother is ill with tuberculosis, a tuberculous process may develop in the middle ear of the fetus, leading to deafness.

In cases of maternal syphilis, the fetus shows degenerative changes in the cells of the organ of Corti, as well as ruptures of the Reisner and main membranes. If a spontaneous abortion does not occur, then the child is born with a malformation of the hearing aid. The deafness observed at the same time belongs to the perceiving type. The loudness equalization phenomenon is always positive, and bone conduction disturbance is noted. Recently, toxoplasmosis is considered one of the leading causes of congenital deafness. Diabetes in the mother is the cause of spontaneous abortions in the first months of pregnancy, especially in primigravida.

2) Toxic factors that damage the fetus and have a negative impact on its hearing apparatus are closely related to the working conditions and nutrition of the mother. Medicinal substances that a woman takes during pregnancy, gases harmful to the body, salts of heavy metals that a pregnant woman encounters due to the nature of her work, can be harmful to a developing fetus. Improper nutrition, beriberi, malnutrition of a pregnant woman affect the development of the fetus. Quinine, taken as a remedy for malaria and also for abortive purposes, can cause deafness.

3) hormonal disorders are also referred to as factors harmful to the hearing aid. A typical example is deafness in cretins associated with dysfunction of the thyroid gland. Cretins often show thickening of the mucous membrane of the middle ear, as well as deformation of the auditory ossicles. In some cases, the foot plate of the stirrup is also thickened. There is no connection between bones. In the organ of balance, as a rule, it is not possible to detect deviations from the norm, while degenerative changes are repeatedly noted in the organ of Corti. The changes described above were found in newborns who died a few days after birth.

4) With incompatibility of the Rh factors of the mother and child, congenital deafness is often observed, the etiology of which in a number of cases cannot be explained by any other

reason. Under physiological conditions, 85% of white people are Rh-positive, and 15% are Rh-negative. Serological conflict, which consists in the incompatibility of Rh factors, can cause the development of severe jaundice, as well as hemolytic anemia in the newborn. Rhesus conflict

factors is the cause of an increased content of bilirubin in the blood, which has a negative effect on the state of the nervous system. Painful symptoms from the nervous system appear in connection with the toxic effect of bile pigments on the main nuclei of the brain. Jaundice of nuclei predverno-

cochlear nerve may explain the occurrence of congenital deafness.

Classification of acquired deafness

Acquired deafness is divided into:

1) deafness due to birth trauma,

2) Deafness that developed after birth,

Diagnosis of deafness in the first 2 years of a child's life is often a significant difficulty. The following points indicate violations of the function of the hearing aid:

1) the child loses the ability to determine where the sound comes from,

3) does not react at all to high-pitched sounds, for example, doorbells, telephones, etc.

3) Deafness, which appeared in a later period.

Allergic diseases in the first 2 years of a child's life are almost always accompanied by changes in the allergic nature of the nasal mucosa, nasopharynx and Eustachian tubes. Although the Eustachian tube is relatively wide, swelling of its mucosa often leads to occlusion of its lumen, which may result in inflammation of the middle ear, which is often characterized by a chronic course, without perforation of the tympanic membrane.

and discharge of pus. The so-called latent form of inflammation develops, leading to permanent damage to the hearing aid. Hearing impairments that occur in the above cases are of a progressive nature.

The deafness of conduction that arose at the beginning turns into a mixed type of deafness, which is most often detected only after 2 years of life, i.e. at an age when the child's hearing can already be examined. Functional disturbances are usually noted in the inner ear, tk. the stimuli reaching it are too weak to cause excitation in the organ of Corti. Hearing loss associated with the process occurring in the Eustachian tube and in the middle ear ranges from 30 to 60 dB, exceeding this limit is noted only when the lesion of the inner ear joins.

Deafness due to birth trauma

Deafness due to birth trauma.

Birth trauma can cause deafness if:

1) bleeding and lack of oxygen,

2) disproportions between the size of the birth canal and the size of the baby's head, when the fetus, pushed out with force by the contracting muscles of the uterus, is damaged while passing through the narrow birth canal.

3) interventions that needed to be done during childbirth, for example, the use of forceps, etc.

1. If oxygen is not supplied to the newborn for more than 5 minutes, then irreversible changes in the nuclei of the vestibulocochlear nerve and deafness of central origin develop. There is also a danger of damage to the organ of Corti, because. experimentally found that short-term

a break in the supply of oxygen is accompanied by a decrease in the electrical potential of the cochlea.

2. Birth trauma occurs when there is a disproportion between the size of the birth canal and the size of the baby's head, as well as when the fetus is in the wrong position. In such cases, without appropriate medical assistance, both the mother and the child die. If the child survives, then in the future he may experience the consequences of birth trauma. Due to the disproportion between the birth canal and the size of the fetus, foci of hemorrhage between the periosteum and the bones of the skull may occur. If such a hematoma becomes infected, then inflammation of the meninges, as well as the labyrinth, can develop as a complication.

Dysfunction after suffering inflammation of the labyrinth is discovered much later, when the mother, concerned that the child does not speak, goes to the doctor.

Deafness in such cases is characterized by the following features: damage to the perceiving department, a positive phenomenon

equalization of loudness, the presence of islands of hearing loss. Inflammation of the labyrinth in most cases develops due to the spread of infection through the hematogenous route, which entered the bloodstream during childbirth, if they occurred in non-sterile conditions.

A complication of inflammation of the meninges is deafness of central origin, in which there is no symptom of loudness equalization, and islands of hearing loss are not detected.

3. With manual assistance during childbirth, when there are narrowings or irregularities in the insertion of the birth canal, you can squeeze the brain. The child is born in a state of asphyxia, while breathing is superficial, the pulse is slow. If convulsions appear, then their cause is a subdural hemorrhage. Fractures and cracks of the skull are extremely rare. The cause of hemorrhages is the movement of the bones of the skull, which are superimposed on each other, which leads to the development of congestion in the blood vessels and their rupture.

Features of deafness that occurs in the first 2 years of life

Inflammation of the meninges in the first 2 years of a child's life is one of the most common causes acquired deafness. Hearing impairment is usually bilateral, although hearing is reduced to varying degrees on each side. After the cessation of the inflammatory process of the meninges, the hearing impairment continues to progress, so that eventually the remnants of hearing also disappear.

The results of audiometric studies performed in children between 2 and 3 years of age indicate complete deafness at all frequencies in approximately 10% of cases. Residual hearing, limited by the ability to perceive tones with a frequency of 256 and 512 cycles/sec, was noted in 75% of cases. In the remaining 15% of cases, residual hearing provided the possibility of auditory perception and tone with a frequency of 1024 cycles/sec. The audibility of vowels is usually very weak. The child hears a voice, but does not distinguish between individual vowels. Among the consonants, the child hears p, g, b best of all, l is worst of all.

Literature:

Neiman L.V., Bogomilsky M.R. Anatomy, physiology and pathology of the organs of hearing and speech: Proc. for stud. higher ped. textbook institutions / Ed. IN AND. Seliverstov. - M.: VLADOS, 2001.

Samsonov F. A., Krapukhin A. V.

Speech, as one of the most important functions of the brain, is not innate, like some elementary forms. nervous activity, but develops according to the laws of conditioned reflexes. Its development is associated with the development and improvement of the brain. Based on the signals of the first system and innate unconditioned reflexes in the cerebral cortex, conditional connections are created between various parts of the brain and the vocal apparatus. nerve impulses from the region of the speech-motor analyzer through the cranial nerves, the organs of speech are set in motion. Feedback from the periphery to the center is carried out along the kinesthetic and auditory pathways. On the basis of such a feedback system, a second signal system is formed, which is supported by the function of the first signal system (especially the function of the auditory and visual analyzers).

So, for normal speech and its development in a child, it is necessary:

a) normal structure and function of the central nervous system and speech centers;

c) normal hearing, which is necessary not only for the perception and imitation of the speech of others, but also for the control of one's own speech.

Anatomical and functional features of the nervous system, respiratory apparatus, lips, tongue, palate and other organs, their specific development is controlled by hereditary factors that form a polygenic system. Thus, it can be argued that voice and speech, as signs of an individual, are determined by a polygenic hereditary system.

To study the genetic determinism and social influence on the development of speech, the twin method is usually used.

According to N. A. Kryshova and K. M. Shteingart (1969), the temporal characteristics of speech with repeated repetition of the same word coincide in identical twins. With complicated speech tasks requiring acquired individual experience, these temporal characteristics differ in twins, but to a lesser extent than in the control group of unrelated people, that is, both elementary and more complex speech activity has an innate conditionality in twins. With a significant genotypic similarity in the timing of speech development, similarity is also determined in the characteristics of the functional activity of the speech-motor analyzer.

The extreme variants of speech formation in children are also more dependent on hereditary factors. It is known that early formation speech, as well as later (in 2-2.5 years), can be traced in individual families for several generations, for which environmental conditions were the same. This is also confirmed by studying the timing of the development of speech in twins.

With a certain probability, we can talk about the hereditary conditionality of ease and difficulty in children in mastering reading and writing. Reinhold speaks about the influence of the hereditary factor in understanding the nature of some forms of dyslexia and dysgraphia in his work “Congenital Dyslexia” (1964). He notes that the immaturity of the brain in its individual areas is inherited from parents, which manifests itself in specific functional disorders. In such cases, several family members suffer from dyslexia and dysgraphia.

Hereditary speech delay, that is, the absence of normal speech in a child aged 3 years, according to M. Zeeman, is observed in 20.6% of all cases of late development of speech. The author observed families in which a delay in speech development could be traced in three generations, most often along the father's side. A. Mitrinovich-Modzheevskaya (1965) indicates that persons with delayed speech development, as a rule, are left-handed; this is observed more often in men, and the defect is transmitted to them from the father. According to some authors, the reason for the delayed development of speech is the delay in the process of myelination of motor and associative nerve fibers in the central nervous system. This process usually begins earlier in girls than in boys. The fact of the hereditary nature of the delay in speech development is also confirmed by observations on identical twins.

Using the clinical and genealogical method, we examined children from the preparatory and first grades at the boarding school No. 96 of the Moskvoretsky district of Moscow (senior speech therapist of the school - A. V. Krapukhin; speech pathologists of the defectological faculty of the Moscow State Pedagogical Institute named after V.I. I. Lenin Zaikina V. P. and Dubovik I. E.). Among those surveyed, about 18% of children with delayed speech development had a hereditary burden for this speech defect. Often, the delayed development of speech was traced in the pedigree in combination with other speech defects (dyslalia, bradilalia, etc.). Here is the pedigree of the R. family, in which a delay in speech development was noted in several family members (see Fig. 1).

Lack of speech (dumbness) as a result of hereditary deafness, that is, deafness, as a rule, is not accompanied by organic lesions of the speech apparatus (peripheral and central sections). Therefore, the absence of speech in this case is completely due to a hereditary (congenital) pathology of the hearing organ.

Stuttering.

The whole variety of etiological factors with which the authors associate the occurrence of stuttering can be divided into two types: predisposing and causing. The first include: the pathological course of pregnancy and childbirth, heredity in terms of the transmission of a weak type of higher nervous activity; somatic weakness of the child, especially in the first three years of life; unfavorable, nervous situation in the family, as well as speech underdevelopment and dyslalia, although the latter are also the result of pathogenic factors. All predisposing factors come down to one thing - a change in the functioning of the nervous system that arose in the early stages of its formation.

Among the causing factors, undoubtedly, the first place is occupied by mental trauma, more often manifested in the form of fright. Among other reasons, an unfavorable speech environment (contact with stutterers) and craniocerebral injuries should be noted. It is known that only a certain combination of predisposing and causing factors plays a role in the ecology of each disease. It can be assumed that in the etiology of some forms of stuttering, any causing factor (or combination of factors) of the external environment can be decisive if it affects an organism with a certain genotype.

Many authors point to the role of hereditary predisposition in the occurrence of stuttering. So, M. Zeeman believes that stuttering is inherited in 1/3 of cases, and cites statistical information from other authors: Gutsman - determined the heredity of stuttering in 28.8% of cases; Tromner - in 34%; Mulder and Nadolechny at 40%; Migind - in 42%; Sedlachkov - in 30.9% of cases. The author notes that it is difficult to identify stuttering in the pedigree when interviewing relatives, because many are often embarrassed to admit that they stuttered themselves or stuttering was observed in their relatives.

When examining 100 stuttering pupils of the same school No. 98 in Moscow (a student Blagova E.V. took part in the work), we found that the majority are boys (69%). The hereditary factor in the etiology of stuttering of the examined could be noted in 17% of cases, and the transmission of the defect was traced through the father's line. As an example, we give the pedigree of Sergei S.

In the development of family stuttering, one cannot ignore the influence of the same unfavorable factors, but one should also think about the significance of a specific predisposition, which can manifest itself not only in the form of stuttering, but also in other speech defects (takhilalia, delayed speech development, dyslalia), autonomic and emotional instability etc.

Simple statistical calculations of heredity in stuttering are supplemented by data obtained from examinations of twins. It turned out that the manifestation of heredity in identical twins differs from its manifestation in fraternal twins. Of the total number of 31 pairs of identical twins, M. Zeeman (11 own observations) notes the stuttering of one twin in only one pair, in other cases both twins stuttered. Other relationships were found in fraternal twins: out of 8 pairs of twins examined, only 1 twin stuttered, although in 4 pairs one of the parents stuttered.

Dyslalia.

The pronunciation of speech sounds, mastered by imitation, depends on the properties of the stimulus - the object of imitation, on the usefulness of the apparatus that perceives it (hearing, kinesthetic sense), on the ability to reproduce the same act (M. A. Piskunov, 1962). The muscular system of the organs of articulation is involved in the act of pronunciation together with other muscles of the body, that is, speech articulation is associated with the general motor skills of the body. Motor talent as a biological predisposing factor determines the accuracy and clarity of neuromuscular activity (articulation), which develops and improves in the process of individual development under the influence of the external environment.

Articulatory disorders in children after 5 years of age are more common in boys than in girls, and are also more likely to be inherited through the male line. There are cases when in three generations of the same family there were persons with an identical feature of the pronunciation of the sound “P”. The hereditary nature of dyslalia is confirmed by cases of the same sound pronunciation defect in both identical twins.

L. N. Ilyina (1971) examined 123 families with familial forms of speech disorders (stuttering, dyslalia, underdevelopment of speech) and found during a clinical and genealogical study that in families where there are children with speech disorders, identical speech disorders are observed in parents . Interestingly, stuttering was transmitted more often through the mother's side to boys, and through the father's side - with the same frequency to both boys and girls. The sibs had the same speech defects in the form of disturbances in tempo, rhythm, phonetics, vocabulary, and contextual presentation. Identical twins (11 pairs) had similar speech disorders, as well as changes in the mental, neurological and somatic state. Concordance for speech disorders, according to the author, is 80%. When analyzing pedigrees, it was found that speech disorders begin at a certain age and are transmitted according to the dominant type.

Tahilalia.

Rapid slurred speech (takhilalia, battarism) may be in children from families where there are fast and normal speaking faces. Some authors consider accelerated speech to be organically caused by a violation of the central speech mechanism and assign a significant role in this to heredity, pointing to genetic connection accelerated speech with stuttering.

Kurshev V. A. (1967) observed 8 children who did not come into contact with fast-speaking parents at the time of development of fast speech and were surrounded by slow-speaking parents, and yet began to speak quickly. This fact indicates the connection of this speech defect with heredity. The author believes that the fast pace of speech is due to the fast pace of thinking, and that takhilalia is a mental-speech disorder.

In this article, we do not consider speech disorders in hereditary forms of mental underdevelopment. It is known that oligophrenia of hereditary and non-hereditary etiology is accompanied by various speech disorders up to dumbness, difficulty understanding addressed speech, disorders (up to impossibility) of reading and writing, stuttering, dyslexia, etc.

We also do not touch upon speech defects caused by disorders of the articulatory and vocal apparatus of hereditary etiology (cleft lip and palate, progeny and prognathia, etc.). Chromosomal syndromes and gene diseases can also have symptoms of speech and voice disorders associated with disorders of the central and peripheral parts of the speech apparatus in the clinical picture.

In addition, in the hereditary pathology of the nervous system (cortical speech centers, subcortical formations associated with speech function, and the cerebellum) and in neuromuscular diseases (myotonia, myopathy), when the muscles of the organs of articulation are involved in the process, there is often a gross violation of speech activity, which is included in the complex symptoms of a neurological disease.

So, various speech disorders - delayed speech development, stuttering, takhilalia and dyslalia, unrelated to defects in the psyche and sensory organs - in some cases have a genetic condition. Speech is a young function of the cerebral cortex, it develops under the influence of various factors, each of which is important for the development of normal speech. Since both morphological and functional signs of the organism are formed on the basis of a certain genotype (under the influence of the environment), it is impossible to exclude the role of the genetic factor in the etiology of some speech disorders. Information available in the literature on the role of heredity in the pathology of speech is still scattered and scarce. However, in last years the interest of geneticists, speech therapists, physiologists and doctors in studying and comprehending the significance of the genetic factor in the development of speech has noticeably increased. It can be hoped that the joint efforts of specialists from different branches of science in a comprehensive study of the etiology and pathogenesis of speech disorders will help speech therapists to build more effectively pedagogical process and create conditions for the successful treatment and prevention of r

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Hearing loss is a reduction in a person's ability to hear sounds. environment in part or in full. A slight decrease in the ability to perceive and understand sounds is called hearing loss, and a complete loss of the ability to hear is called deafness.

Deafness is subdivided into neurosensory, conductive and mixed. Deafness, like hearing loss, can be congenital or acquired.

Sounds are sound waves that vary in frequency and amplitude. Partial hearing impairment manifests itself in the inability to perceive certain frequencies or differentiate sounds with low amplitude.

Causes of Hearing Loss

The causes of hearing loss are of different nature:

1. Infectious diseases of an acute nature, which the mother of the child was exposed to during pregnancy, which include influenza, rubella, mumps. Increased blood pressure in the mother during the prenatal period of the child can also cause hearing loss.
2. Abuse by the mother during pregnancy of drugs (especially loop diuretics, aminoglycosides, streptomycin, gentomycin), alcoholic beverages or drugs.
3. Birth injuries of a different nature and various deviations at birth:
   • the weight of the baby is less than one and a half kilograms;
   • childbirth that began before the thirty-second week;
   • insufficient oxygen during birth or prolonged breath holding after birth;
   • damage to the baby during childbirth of a mechanical nature.
4. Hereditary (genetic) hearing disorders.
5. Infectious diseases that were transferred by the child in the early age period, namely meningitis, encephalitis, measles, rubella, mumps, influenza, and so on.
6. Certain diseases, which include Meniere's disease, otosclerosis, acoustic neuroma, Möbius syndrome, multiple congenital arthrogrypposis, can cause hearing impairment or loss.
7. Complications after chronic inflammatory processes of the ear (otitis) are hearing impairment.
8. Continuous exposure to noise for long periods of time can cause hearing loss, especially at high frequencies.
9. Acoustic injuries, namely, being in areas of sudden shots and explosions.
10. The consequences of accidents and various accidents can be hearing loss.
11. Postponed chemotherapy can also cause hearing loss.
12. Age-related changes affecting the cochlea and the central auditory system. Moreover, these processes can proceed imperceptibly after thirty years.

Acquired hearing loss

Acquired hearing loss is a dysfunction of the auditory system that can occur at any age due to the following factors:

• The consequences of diseases of an infectious nature, namely, meningitis, measles, parotitis.
• Chronic infections in the ears that cause not only hearing loss but, in some cases, life-threatening illnesses such as brain abscesses or meningitis.
• Otitis media, which causes a buildup of fluid in the middle ear.
• Applications in medicinal purposes ototoxic drugs such as antibiotics and antimalarials.
• Existing head or ear injuries.
• Exposure to loud noise of a sudden or prolonged nature - exposure to noisy equipment, loud music and other excessively loud sounds, as well as gunshots and explosions.
• Degeneration of sensory cells that occur due to age-related changes.
• Finding earwax, as well as any foreign bodies in the external auditory canal. Such hearing problems are easily corrected by cleaning the ear canal of such substances.

Hearing loss in otitis media

Otitis is an inflammatory disease of different parts of the ear, which is accompanied by manifestations of fever, intoxication and severe pain. In some cases, otitis media causes permanent or temporary hearing loss.

Chronic otitis media is the main cause of hearing problems in childhood.

Otitis in children and adults is sometimes accompanied by damage to the tissues of the outer and middle ear. With otitis externa, the infection enters through small skin lesions that form after damage to the auditory shell of the outer ear. Such changes occur due to mechanical deformations, as well as chemical and thermal burns. The causative agents of the disease in this case are staphylococci and streptococci, as well as Pseudomonas aeruginosa, Proteus, and so on. At the same time, some diseases, namely diabetes mellitus, gout, hypovitaminosis and other metabolic disorders, can also be factors provoking otitis externa.

Otitis media is caused by infectious diseases of the nasopharynx, in which pathological microorganisms enter the middle ear cavity through the auditory tube. These include influenza and parainfluenza viruses, adenovirus and rhinovirus, respiratory syncytial virus, as well as pneumococcus, pyogenic streptococcus, moraxella and Haemophilus influenzae bacteria. Young children are particularly susceptible to otitis media due to the fact that their auditory tubes are shorter and wider. Infection with otitis media can also penetrate from the outside due to mechanical damage and barotrauma of the eardrum. Provoke the occurrence of the disease and chronic infectious processes in the nasopharynx, namely, chronic tonsillitis, sinusitis and adenoid disease.

During the course of otitis externa, the inflammatory process first of all begins in the superficial layers of the auricle, and then can develop in the surrounding tissues and the eardrum.

During the course of otitis media, the mucous membrane begins to become inflamed and produce exudate (a certain fluid that is released during inflammation of the tissues). Exudate may have a serous form, which is typical for viral infections, or purulent form, due to its bacterial nature. The accumulation of fluid in the middle ear begins to protrude the eardrum outward and in some cases leads to its rupture. All of the above features of the course of otitis media are the cause of hearing impairment in people of different ages.

Severe inflammation leads to a violation of the mobility of the auditory ossicles, which causes a decrease in the perception of sound waves and is a hearing impairment.

Untreated otitis and their constant relapses cause scarring in the tissues of the middle ear, which leads to a decrease in the mobility of the auditory ossicles and the elasticity of the tympanic membrane. This causes hearing loss in people of different age categories. Inflammatory processes that are protracted can penetrate into the inner ear and affect auditory receptors. Such deformities lead to the appearance of deafness in the patient.

Symptoms of Hearing Loss

Acquired hearing impairments in the vast majority do not appear suddenly. It happens, of course, that the patient has a sudden loss of hearing, progressing over several hours. Usually, hearing loss symptoms develop over several or many years. At the same time, the first signs of hearing loss are almost invisible, and the symptoms differ in different categories of people.

The primary symptoms of hearing loss are:

1. Frequent requests of the patient to repeat the words spoken by the interlocutor again.
2. Increasing the sound volume of your computer, TV, or radio above normal volume.
3. The feeling that everyone around is talking incomprehensibly and indistinctly.
4. There was a feeling that it became more difficult to understand some voices, especially the high ones - women's and children's.
5. Constant tension of hearing during conversation and increased fatigue from talking with other people.
6. Cessation of visits to meetings of people and other collective events. This also applies to other social events that previously provided positive emotions.

Symptoms of hearing loss in childhood include:

• Lack of response to external noise, both in the normal state and when carried away by any activity - playing, drawing, and so on.
• Lack of response to loud sudden noise during sleep in the form of awakening and continued restful sleep.
• Inability to determine the source of the sound.
• No imitation of sounds.
• Frequent ear infections and inflammation.
• Noticed problems in understanding the speech of other people.
• Having a delay in the development of speech or a discrepancy between the level of speech development and age parameters.
• Lagging behind peers when participating in group games.

Congenital hearing impairment

Congenital hearing impairments are dysfunctions of the auditory system that are associated with hearing loss or hearing loss, detected at birth or shortly after birth.

Causes of congenital hearing loss include:

1. Hereditary genetic factors.
2. Non-hereditary genetic features.
3. Complications during pregnancy, which are caused by syphilis, rubella, mumps and others transferred by the mother.
4. Low weight newborn - less than one and a half kilograms.
5. Received asphyxia during birth, that is, a strong lack of oxygen.
6. Oral use by the mother during pregnancy of ototoxic drugs, namely, aminoglycosides, diuretics, antimalarial drugs, cytotoxic drugs.
7. Severe jaundice suffered by an infant in the neonatal period, which can cause damage to the auditory nerve in a child.

genetic hearing loss

Genetic hearing loss can fall into two categories:

• non-syndromic (isolated),
• syndromic.

Non-syndromic disorders include hearing loss that does not have any other associated symptoms that are inherited. Such hearing impairments occur in seventy percent of cases of deafness due to heredity.

Syndromic hearing loss is caused by hearing loss in conjunction with other factors, such as diseases of other organs and systems. Syndromic disorders occur in thirty cases of deafness due to heredity. In medical practice, more than four hundred different syndromes have been recorded, one of the manifestations of which is deafness. These syndromes include:

• Usher's syndrome - a simultaneous violation of the auditory and visual systems,
• Pendred's syndrome - in which hearing loss is combined with hyperplasia of the thyroid gland,
• Gervell-Lange-Nielson syndrome - causes a combination of deafness and cardiac arrhythmia with the appearance of a longer QT interval,
• Waardenburg syndrome - describes a violation of the auditory system in combination with the appearance of pigmentation.

If we take into account the type of inheritance of hearing dysfunctions, they can be divided into the following types:

1. Autosomal recessive, which includes seventy-eight percent of cases.
2. Autosomal dominant, which includes twenty percent of cases.
3. X-linked, which includes one percent of cases.
4. Mitochondrial, which accounts for one percent of cases.

In modern medicine, more than a hundred genes have been identified that, when mutated, are responsible for hearing impairment. Each population of people has its own specific mutations that are different from other populations. But it can be noted with certainty that in about a third of cases genetic disorders The cause of such negative transformations is a mutation in the connexin 26 (GJB2) gene. The Caucasoid race is most susceptible to the 35delG gene mutation.

Hearing loss in the elderly

The majority of people with acquired hearing loss are elderly or old age. Hearing impairment in the elderly is associated with age-related changes, as a result of which degenerative transformations occur in the hearing organs. Changes in the hearing organs due to age affect all parts of the auditory system, from the auricle to the zone of the auditory analyzer in the cerebral cortex.

Hearing loss in the elderly is a complex and complex process that experts are carefully studying. Senile hearing loss is called the term "presbycusis" and is divided into two types:

• conductive,
• sensorineural.

The conductive form of senile hearing loss is caused by degenerative changes occurring in the middle ear, as well as a decrease in the elasticity of the tissues of the skull.

The sensorineural type of senile hearing loss has four subspecies, caused by various reasons:

• sensory - caused by atrophy of the hair cells of the organ of Corti,
• neural - associated with a decrease in the number of nerve cells present in the spiral ganglion,
• metabolic - due to atrophic changes in the vascular strip, weakening the metabolic processes in the cochlea,
• mechanical - associated with atrophic transformations of the main membrane of the cochlea.

Some experts adhere to a different classification of senile hearing loss. It describes three types of hearing loss age-related nature:

• presbycusis is hearing loss caused by internal factors of a genetic and physiological nature,
• sociocusia - hearing loss, which is caused by external factors that contribute to severe wear of the organs of the auditory system,
• occupational noise hearing loss is a hearing impairment that occurs under the influence of strong industrial noise at the place of work of the individual.

Features of children with hearing impairment

Hearing-impaired children have significant differences in psychophysiological and communication development. These features of children with hearing impairment prevent them from developing at the same speed as their peers, and also cause problems in mastering knowledge, life-saving skills and abilities.

Hearing impairment in children significantly reduces the quality of speech formation and verbal thinking. At the same time, cognitive processes and cognitive activity also experience a deficit in improvement and development.

Children with hearing loss may also have other disabilities, such as:

1. Problems with the functioning of the vestibular apparatus.
2. Various visual defects.
3. Brain dysfunction of a minimal degree, which leads to inhibition of the overall development of the psyche.
4. Extensive brain lesions that cause oligophrenia.
5. Defects in the brain systems that lead to childhood cerebral palsy and other traffic disorders.
6. Violations of the functioning of the auditory and speech zones in the brain.
7. Diseases of the central nervous system and the body as a whole, which cause severe mental illness - schizophrenia, manic-depressive psychosis, and so on.
8. Diseases of the internal organs of a severe degree (heart, kidneys, lungs, digestive tract, and so on), which generally weaken the child's body.
9. Serious socio-pedagogical neglect.

There are two types of hearing impairment and children, which depend on the degree of loss of the ability to perceive and process sounds:

• hearing loss affecting children who are hard of hearing
• deafness.

With hearing loss, the perception of speech, but only spoken loudly, articulately and close to the ear, is preserved. Although, of course, the possibilities of this child for full verbal communication with others are limited. Deafness is characterized by the fact that with it there is a perception of certain tones of speech without the ability to perceive and distinguish speech itself.

Various auditory disorders in childhood lead to inhibition and distortion of the development of speech functions. But, at the same time, the researchers noted the following fact that if severe hearing loss appears at a time when the child has already mastered the skills of reading and writing to a good degree, then the disease does not lead to defects in the development of speech. Although in this case there are various violations associated with pronunciation.

The most important factors that influence the speech development of children with hearing impairments are:

• The degree of hearing loss - children with auditory impairments speak as badly as they hear poorly.
• The age period of the onset of pathological hearing impairment - the earlier hearing defects occur, the more severe the speech impairment, up to the appearance of deafness.
• Conditions for the development of the child and the availability of correct psycho-corrective measures - the earlier special measures are taken to develop and maintain correct speech, the higher the effectiveness.
• Physical development of a general nature and mental development of the child - a more complete speech is noted in children with a good physical development, good health and normal mental development. In children with poor health (passive, lethargic children) and mental disorders, significant speech defects will be observed.

Classification of hearing impairment

Hearing impairment can be divided into several types:

• Conductive - arising from blockage of any quality that appears in the outer or middle ear. In this case, the sound cannot normally penetrate the inner ear.
• Neural - arise due to the complete loss of the brain's ability to perceive and process electrical impulses. In some cases, the brain cannot correctly decipher the received signals. These disorders include "auditory dyssynchrony" or "neuropathy".
• Sensory - appear if there are violations of the functioning of the hair cells of the inner ear. As a result, the snail cannot normally pick up sounds.
• Sensorineural - combined disorders in which there are dysfunctions of hair cells, as well as large inaccuracies in the perception and processing of signals of a sound nature by the brain. These types of disorders are persistent because it is almost impossible to establish how the cochlea and the brain are involved in hearing loss. The greatest difficulty in the correct diagnosis is observed when examining young children.
• Mixed - these include the synthesis of sensorineural and conductive disorders. With this hearing loss, sound signals cannot be transmitted normally from the outer and middle ear to the inner, and there are also malfunctions of the inner ear, areas of the brain and neural parts of the hearing system.

Preobrazhensky's classification of hearing impairment

In modern practice, there are several classifications of auditory disorders. One of the most popular is the classification of hearing impairments by Professor B. S. Preobrazhensky. It is based on the level of perception of oral loud speech and speech spoken in a whisper. The study of the perception of loud speech is necessary due to the fact that it contains elements of whispered speech, namely, deaf consonants and unstressed parts of the word.

According to this classification, there are four degrees of hearing impairment: mild, moderate, significant and severe. Easy degree characterized by the perception of spoken speech at a distance of six to eight meters, and whispered - from three to six meters. A moderate degree of hearing impairment is diagnosed when conversational speech is perceived at a distance of four to six meters, whispered - from one to three meters. A significant degree of auditory impairment is determined by understanding colloquial speech at a distance of two to four meters, and whispered speech - from the auricle to one meter. A severe degree of auditory impairment is distinguished in the case of understanding colloquial speech at a distance from the auricle to two meters, and in whispering - from complete misunderstanding to discrimination at a distance of half a meter.

Conductive and sensorineural hearing loss

Conductive hearing loss is characterized by impaired conduction of sounds due to defects in the outer or middle ear. Normal conduction of sound signals is ensured only by properly functioning ear canal, tympanic membrane and ear bones. With any violation of the above organs, a partial hearing loss, called hearing loss, is observed. With partial hearing loss, there is some deterioration in the ability to perceive sound signals. Conductive hearing loss allows speech to be recognized if the individual is able to hear it.

Conductive hearing loss is caused by:

• obstruction of the ear canal,
• anomalies in the structure and functioning of the middle ear, namely the tympanic membrane and / or ear ossicles.

Sensorineural hearing impairment is caused by defects in the functioning of the inner ear (cochlea), or a violation of the proper functioning of the auditory nerve, or the inability of certain brain areas to perceive and process sounds. In the first case, there is a pathological deformation of the hair cells, which are located in the cochlea of ​​Corti. In the second and third cases, sensorineural hearing impairment occurs due to pathology of the VIII cranial nerve or parts of the brain that are responsible for the auditory system. At the same time, a violation of the functioning of exclusively auditory areas of the brain, which is called central hearing loss, is considered a rather rare phenomenon. In this case, the patient can hear normally, but with such low sound quality that he cannot understand the words of other people at all.

Sensorineural disorders of the auditory system can lead to deafness various degrees– from low to high, as well as to complete hearing loss. Experts believe that sensorineural hearing loss is most often caused by pathological changes in the functioning of the cochlear hair cells. Such degenerative changes can be either congenital or acquired. With acquired transformations, they can be caused by infectious diseases of the ears, or noise injuries, or a genetic predisposition to the appearance of anomalies in the auditory system.

Phonemic hearing disorder

Phonemic hearing is the ability to distinguish the phonemes of a language, namely, to analyze and synthesize them, which is expressed in understanding the meaning of speech spoken in a particular language. Phonemes are the smallest units that make up any language; they make up the structural components of speech - morphemes, words, sentences.

When perceiving speech and non-speech signals, there are differences in the operation of the hearing system. Non-speech hearing is a person's ability to perceive and process non-speech sounds, namely musical tones and various noises. Speech hearing is the ability of a person to perceive and process the sounds of human speech in a native or other language. In speech hearing, phonemic hearing is distinguished, with the help of which phonemes and sounds are distinguished, which are responsible for the semantic load of the language, so that a person can analyze individual speech sounds, syllables and words.

Phonemic hearing disorders are common in children and adults. The causes of phonemic hearing impairment in children are as follows:

1. The lack of formation of acoustic images of individual sounds, in which phonemes cannot differ in sound, which causes the replacement of some sounds by others when speaking. Articulation turns out to be insufficiently complete, since not all sounds can be pronounced by the child.
2. In some cases, the child can articulate all the sounds, but at the same time, he cannot distinguish which sounds he needs to pronounce. At the same time, the same word can be pronounced differently by a child, since the phonemes are mixed, which is called the mixing of phonemes or the interchange of sounds.

With impaired speech hearing, the child develops sensory dyslalia, which means the inability to correctly pronounce sounds. There are three forms of dyslalia:

• acoustic-phonemic,
• articulatory-phonemic,
• articulatory-phonetic.

Acoustic-phonemic dyslalia is characterized by the presence of defects in the design of speech with the help of sounds, which is caused by the inability to fully process the phonemes of the language due to anomalies in the sensory link of the speech perception system. At the same time, it should be noted that the child does not have disorders of the auditory system, that is, there is no hearing loss or deafness.

Violation of phonemic hearing in adults is due to local lesions of the brain, namely:

• sensory temporal aphasia,
• sensory aphasia of the nuclear zone of the sound analyzer.

Mild cases of sensory temporal aphasia are characterized by understanding single words or short phrases, especially those that are used daily and belong to the constant daily routine of the patient. In this case, there is a disorder of phonemic hearing.

Severe cases sensory aphasia are characterized by a complete misunderstanding of speech by a sick person, the words cease to have a semantic load for him and turn into a combination of incomprehensible noises.

Sensory aphasia, expressed in the defeat of the nuclear zone of the sound analyzer, not only leads to a violation of phonemic hearing, but also to a severe speech disorder. As a result, it becomes impossible to distinguish the sounds of oral speech, that is, to understand speech by ear, as well as other speech forms. Such patients do not have active spontaneous speech, it is difficult to repeat the words they hear, writing from dictation and reading is impaired. It can be concluded that as a result of a violation of phonemic hearing, the entire speech system is pathologically transformed. However, in these patients ear for music preserved, as is the articulation.

Conductive hearing loss

Conductive hearing loss is caused by the impossibility of its conduction in normal form through the ear canal due to disorders in the outer and middle ear. Features of conductive hearing loss have been described in previous sections.

Diagnosis of hearing loss

Diagnosis of hearing impairment is carried out by various specialists - audiologists, audiologists, otorhinolaryngologists.

The hearing test procedure is as follows:

• The patient, first of all, undergoes a consultation with an audiologist. The doctor performs an otoscopy, that is, an examination of the outer ear along with the eardrum. The purpose of this examination is to identify or refute mechanical damage ear canal and tympanic membrane, as well as pathological conditions of the ears. This procedure does not take much time, besides it is absolutely painless.

Very important during the examination are the patient's complaints, which can describe various symptoms of hearing impairment, namely the appearance of slurred speech when communicating with other people, the occurrence of ringing in the ears, and so on.

• Carrying out tone audiometry, in which it is necessary to recognize various sounds that differ in frequency and loudness. As a result of the examination, a tonal audiogram appears, which is only an inherent characteristic of auditory perception for this person.
• Sometimes it is necessary to conduct speech audiometry, which establishes the percentage of words that a person can distinguish at different volumes of their pronunciation.

Tone and speech audiometry are subjective diagnostic methods. There are also objective diagnostic methods, which include:

• Impendancemetry or tympanometry, which allows diagnosing disorders in the middle ear. This method allows you to fix the level of mobility of the tympanic membrane, as well as to confirm or refute the presence of pathological processes in the middle ear.
• Registration of otoacoustic emission evaluates the condition of the hair cells, which help to establish the quality of the work of the cochlea of ​​the inner ear.
• Registration of evoked electrical potentials determines the presence or absence of damage to the auditory nerve or areas of the brain responsible for hearing. In this case, a change in the electrical activity of the brain is recorded in response to the sent sound signals.

Objective methods are good for examining adults, children of any age, including newborns.

Hearing loss treatment

Hearing impairment is treated with the following methods:

1. Prescribing medications.
2. Uses certain methods audiology and speech therapy.
3. Constant exercises of auditory and speech development.
4. The use of hearing aids.
5. Using the recommendations of a psychoneurologist to stabilize the psyche and emotional sphere of the child.

Speech therapy for hearing loss

Speech therapy for hearing impairment is of great importance, since hearing-impaired children have speech disorders associated with pronunciation. Speech therapists organize classes in such a way as to improve the articulation of the child and achieve natural pronunciation of words and phrases. In this case, various speech therapy methods of a general nature are used and specially selected, taking into account the individual characteristics of the child.

Exercise therapy for hearing loss

There are a number of exercises that are used for pathologies of the auditory canal in order to improve its functioning. These include special breathing exercises, as well as with the tongue, jaws, lips, smile and puffing out of the cheeks.

To improve hearing acuity in hearing-impaired children, special exercises are used that train the quality of sound perception.

Treatment of phonemic hearing impairment

Treatment of phonemic hearing impairment in children is carried out in a complex way:

• Special speech therapy exercises which develop the quality of perception and pronunciation of speech in children.
• A special speech therapy massage is shown, which is done to the articulatory muscles. Such a massage is carried out using special probes and normalizes muscle tone for the correct pronunciation of sounds.
• Microcurrent reflexology - activates the speech zones of the cortex hemispheres, which are responsible for understanding speech, the possibility of correctly structuring sentences, for a developed vocabulary, good diction and desire for communication.
• Special logarithmic exercises are used in groups and individually.
• General musical development is shown, which includes singing, exercises to develop a sense of rhythm, musical games and learning to master musical instruments.

Parenting Children with Hearing Impairment

The upbringing of children with hearing impairment is carried out by parents, as the rehabilitation of the baby. The first three years of his life are of great importance for the formation of correct mental and other processes, as well as the personality of the child. Since at this time, babies mainly spend time with their parents, the correct behavior of adults is of great importance for correcting developmental defects in the child.

It is clear that in this case, parental care for the baby requires a lot of time and effort. But it should be noted that there are often cases when the parents of a hearing-impaired child were able to carry out the rehabilitation of the baby under the supervision of an experienced deaf teacher.

It is important for a hearing impaired child to have direct verbal contact with parents, as well as joint activities. The volume of speech signals during communication should be sufficient to ensure the understanding of speech by the child. Also important are constant and intensive auditory training, which are the basis of the rehabilitation process. It should be noted that a hearing-impaired child should be able to communicate not only with his parents, but also with other normally hearing and normally speaking people.

The upbringing of such children should take place in accordance with the recommendations and under the supervision of deaf psychologists and deaf teachers. At the same time, parents should apply corrective methods that are recommended by experts.

Teaching children with hearing loss

Education of children with hearing impairment should be carried out in special preschool and school institutions. In these institutions, competent specialists will be able to provide the correct corrective assistance, as well as apply teaching methods that are correct for these children. In public schools, the child will be assisted by specialists working in special classes or groups organized for such children.

In kindergartens for children with hearing impairment, correctional work is carried out with children from one and a half to two years old. The attention of pedagogical influence is the general development of the child, namely his intellectual, emotional-volitional and physical characteristics. At the same time, all those areas that need to be developed in ordinary, well-hearing children are involved.

The educational process with children with hearing impairment is aimed at a lot of work on the development of speech, its pronunciation components, as well as the correction of residual hearing and the development of verbal and other thinking. The educational process consists of individual and group lessons, the use of choral recitations with music accompaniment. Then classes for the development of speech are connected, in which amplifiers and hearing aids are used.

Literacy education for these children begins at the age of two. At the same time, the work takes place purposefully and constantly - kids learn to read and write with the help of block letters. Such teaching methods allow the development of speech perception at a full level, as well as normal level speech reproduction (as in healthy children) through writing.

Rehabilitation of children with hearing impairment

The main task of deaf psychologists and deaf teachers working with children with hearing impairments is to reveal their compensatory abilities and use these mental reserves to fully overcome hearing defects and receive the necessary education, full socialization, as well as inclusion in the process professional activity.

Rehabilitation work with children with hearing impairment should be carried out fully and begin as early as possible. This is due to the fact that from early and preschool age, children with these problems lag behind their peers. This is manifested both in the insufficient level of development of activity, and in the possibility of communication with adults. It can also be noted that in children with hearing impairment there is an incorrect formation of individual psychological experience, as well as inhibition in the maturation of certain mental functions and significant deviations in the formation of general mental activity.

For the successful rehabilitation of hearing-impaired children, the preservation of the intellect and cognitive sphere, as well as other sensory and regulatory systems, is of great importance.

There is a strong point of view in deaf pedagogy that the possibilities for the rehabilitation of deaf and hard of hearing children practically have no limits. This does not depend on the severity of the hearing impairment; early diagnosis of hearing impairment and the same correction of a pedagogical and psychological nature are important. The most important time for rehabilitation is the age of birth up to three years.

The main corrective point is speech development, which helps to avoid deviations in the formation of mental functions.

Working with a hearing impairment

Hearing-impaired people need a kind of professional activity that requires minimal communication with other people, reliance on sight, not hearing, and the absence of a quick reaction of behavior and speech.

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Prevention of hearing loss

Timely prevention of hearing impairment in more than half of cases can lead to the preservation of hearing in a child or adult.

Preventive measures include the following:

1. Conducting immunization measures against infectious diseases, which include measles, rubella, mumps and meningitis.
2. Immunization of adolescent girls and women of reproductive age against rubella before conception.
3. The implementation of diagnostic procedures with pregnant women in order to exclude the presence of infections in them: syphilis and others.
4. Exercising careful control of the doctor of the use of ototoxic drugs; inadmissibility of independent use of these medicines without prescription by a specialist.
5. Early hearing screening for newborns at risk:
   • having deaf close relatives,
   • born with very low birth weight
   • subjected to asphyxia at birth,
   • ill in infancy with jaundice or meningitis.
6. Early examination allows you to correctly diagnose and start proper treatment in a timely manner.
7. Elimination (or at least reduction) of exposure to prolonged loud noises, both in the workplace and at home. Preventive measures at work include the use of personal protective equipment, as well as raising employee awareness of the dangers of prolonged exposure to excessive noise and the development of laws and regulations regarding high noise exposures.
8. Severe hearing loss, as well as hearing loss due to chronic otitis media, can be prevented with timely diagnosis. Examination in the early stages of the disease will allow timely application of conservative or surgical methods of treatment, which can save the patient's hearing.
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