Hereditary pathologies of hearing. Hearing pathology in children

The organs of hearing in humans have a complex structure, they allow not only to perceive sounds, but are also responsible for balance. Ear diseases are accompanied by various unpleasant symptoms, if you do not start treatment in a timely manner, you can completely or partially lose your hearing.

Ear pain is the first sign of the development of the disease

Types of ear diseases

The ear is made up of the outer ear canal, auricle and the inner ear, diseases can begin by different reasons, sometimes affect several departments at once.

The main types of ear diseases:

1. Pathologies of a non-inflammatory nature - otosclerosis, Meniere's disease, vestibular neuritis, most often have a genetic origin, chronic.
2. Infectious diseases - these diseases are leading among ear pathologies, they are most often diagnosed in children, because their ear canal is shorter than in adults, the infection spreads quickly. This group includes all types of otitis media.
3. Fungal infections (otomycosis) - opportunistic fungi can affect any part of the hearing organs, the disease most often develops against the background of injuries, reduced immunity, oncological diseases, after surgery, with metabolic disorders.
4. Ear injuries are most common in children and athletes. This group includes damage to the shells during a blow, bruise, bite, the presence of foreign bodies in the ear canal, burns, barotrauma. A separate place is occupied by hematoma - a hemorrhage between the periosteum and cartilage, in which the process of tissue suppuration begins.

Names of diseases of the ears

Most ear diseases have a similar clinical picture, manifesting themselves in the form of pain, itching, burning, redness of the skin, discharge, and hearing loss. With a strong inflammatory process, there is a deterioration in the functioning of the vestibular apparatus - impaired coordination, dizziness, nausea, vomiting.

Infectious pathology, in which the auditory canal is damaged, the ventilation process in the tympanic cavity develops otitis media. The cause of the disease is the penetration of pathogenic microorganisms from the nasopharynx and upper respiratory tract.

Eustachitis - damage to the auditory canal

Symptoms:

• pain, sensation of the presence of water inside the ear, discomfort increases with movement;
• decrease in auditory perception;
• an increase in temperature indicates the development of a purulent process.

The most dangerous ear pathology is deafness. The congenital form occurs in the womb due to viral infections in the mother, the acquired form develops in children before three years, as a complication of other diseases, when exposed to certain drugs.

Infectious pathology, characterized by inflammation of the mastoid process of the temporal bone, the presence of a purulent process, occurs when the infection spreads from the middle ear. The main pathogens are influenza bacillus, pneumococci, staphylococci, streptococci.

Mastoiditis is an infectious disease

Signs of the disease:

• heat;
• signs of severe intoxication;
• deterioration in auditory perception;
• throbbing pain;
• the auricle swells, slightly protrudes;
• appear purulent discharge from the ear.

A sudden attack of dizziness often indicates the onset of an inflammatory process in the inner ear.

Against the background of the disease, narrowing and damage to blood vessels occurs, blood supply is disturbed, fluid accumulates near the ear cavity. The exact causes of the development of pathology have not yet been identified, some experts believe that the disease is of viral origin, other doctors adhere to the hereditary theory. Trigger factors - violations in the work of blood vessels, low level estrogen, changes in water-salt balance.

Meniere's disease - accumulation of fluid in the ear labyrinth

Clinical picture:

• tinnitus, congestion;
• dizziness, nausea;
• balance worsens;
• loud noises are irritating.

Minier's disease is considered incurable, therapy is aimed at prolonging the remission stage. Patients should adhere to a salt-free diet, give up addictions, and avoid exposure to ultraviolet radiation.

Acoustic neuritis (cochlear neuritis)

The disease refers to pathologies of a neurological nature, the causes of the disease are inflammatory processes in the nasopharynx, injuries, cervical osteochondrosis, cardiovascular and endocrine diseases, atherosclerosis, brain injury.

Cochlear neuritis - inflammation of the auditory nerve

Symptoms:

• hearing loss;
• flickering black spots before the eyes;
• dull headaches;
• noise in ears;
• bouts of dizziness.

If you start the disease, necrosis of the tissues of the auditory nerve will begin, which will lead to complete irreversible hearing loss.

Otitis and tympanitis

The inflammatory process in various parts of the hearing organs, the most common ear disease, develops as a complication of influenza, colds, sore throats, sinusitis, and ear injuries. Most often diagnosed in children and the elderly.

Types and symptoms of otitis:

1. With an organic form, boils appear on the outer part of the ear canal, which develop in sebaceous glands, hair follicles, the disease is accompanied by severe pain, an increase in the parotid lymph nodes, ulcers form at the site of bursting abscesses. Diffuse otitis media develops when the ear is damaged by viruses, bacteria, fungi, purulent discharge appears, the ear turns red, itches, the touch is accompanied by pain, discomfort increases when the mouth is opened.
2. Otitis media - develops when pathogenic microorganisms penetrate into the ear cavity, which irritate the Eustachian tube. At the initial stage of the disease, which gives off to the head, the temperature rises, auditory perception worsens. The second stage is accompanied by purulent discharge, while the pain disappears, the temperature decreases. The disappearance of suppuration against the background of severe hearing loss indicates the beginning of the third stage of the disease.
3. Labyrinth - inflammation of the inner ear, accompanied by bouts of dizziness, poor balance, nausea, vomiting, tinnitus. As the pathology develops, the color of the skin changes, and discomfort occurs in the region of the heart.
4. Mesotympanitis is a type of purulent otitis media, the symptoms are similar to inflammation of the middle ear, pus is released periodically.
5. Epitympanitis is a severe form of otitis media, in which the bone rots, the walls of the middle ear are destroyed, purulent discharge has a sharp unpleasant odor, pain is strong and prolonged.

Warming up the ear with otitis can be carried out only in the absence of temperature and purulent discharge.

With otitis externa, the parotid lymph nodes are enlarged

A fungal infection that affects the membranes and the ear canal, the causative agents of the disease are yeast-like and mold fungi.

Otomycosis - fungal infection of the ear

Otosclerosis is a hereditary disease

Main symptoms:

• dizziness;
• noise and ringing in the ears;
• hearing loss.

Otosclerosis is inherited only through the female line, an effective method of treatment is prosthetics.

The disease begins when the inflammatory process from the middle ear spreads to the vessels and sinuses located in the temporal bone, it is diagnosed more often in young people. With pathology, symptoms appear that are unusual for ear diseases.

In otogenic sepsis, the middle ear becomes inflamed

Signs of otogenic sepsis:

• feverish conditions, chills;
• tachycardia;
• dyspnea;
• increased fatigue;
• loss of appetite and sleep.

Before starting drug therapy, drainage is performed to remove purulent masses.

Excessive accumulation of sulfur is observed when improperly carried out hygiene procedures, excessive synthesis of ear secretion - the cork clogs the ear canal, gradually hardens.

Earplug blocking the ear canal

Symptoms:

• autotomy;
• hearing loss;
• ear congestion and;
• coughing and dizziness appear if the clot irritates the walls of the ear canal.

Often signs ear plug appear after water procedures - a clot of sulfur swells, obscures the entire lumen.

Ear injury

Most often, damage occurs against the background of mechanical damage, one or more parts of the hearing organs may be affected, if the integrity of the membrane of a person is disturbed, nausea is disturbed, and the head is very dizzy.

Ear injuries often cause dizziness

Barotrauma occurs with pressure drops, the disease manifests itself in explosives, high-altitude workers, people who work at great depths. First, a person feels a blow, then a pain syndrome occurs, when the membrane ruptures, blood flows.

Ear neoplasms benign formed at the site of scars, burns, dermatitis, localized on the outer or middle ear. Malignant tumors may appear after purulent otitis, with metaplasia.

Neoplasms near the ears

Which doctor should I contact?

He deals with the diagnosis and treatment of ear diseases. If necessary, consultation may be required.

Diagnostic methods

Most ear diseases can be diagnosed by an experienced specialist during examination and questioning of the patient. But if the clinical picture is not completely clear to the doctor, he will prescribe other diagnostic methods. Devices for the study of the hearing organs can be seen in the photo.

Special tube for examining the auditory canal

Methods for detecting ear diseases:

• otoscopy - examination of the ear canal and eardrum using a special tube;
• audiometry - measurement of hearing acuity, determination of auditory sensitivity to waves of various frequencies;
• tympanometry - a probe is inserted into the ear canal, after which the specialist measures the volume of the ear canal, periodically changing the pressure inside the ear;
• x-ray - allows you to assess the state of the structure of all parts of the organ of hearing;
• CT - the method allows you to see injuries, displacement of bones, identify inflammatory and infectious pathologies, tumors, abscesses;
• Ultrasound - performed to detect neoplasms, foci of infection, size and characteristics of the ear canal;
• bacterial culture to determine effective antibacterial drugs;
• clinical, biochemical and serological studies blood to detect infectious diseases.

All research methods are painless, special preparation is required only for blood tests - they need to be taken on an empty stomach, the last meal should be 10-12 hours before the study.

Possible Complications

The main consequence of ear diseases is complete or partial hearing loss, which can be reversible and irreversible. If you do not start treatment in a timely manner, the infection will begin to spread to the lymph nodes, brain tissue.

The consequences of ear diseases:

• brain sepsis, meningitis;
• violation of the integrity of the eardrum;
• abscess of the ear and brain;
• malignant and benign neoplasms;
• paralysis of the facial nerve;
• hearing loss.

Advanced forms of diseases almost always lead to disability, in some cases death is possible.

If ear diseases are not treated in time, facial paralysis may develop.

Treatment of ear diseases

The list of ear pathologies and symptoms of their manifestation is very large, so only a doctor can correctly diagnose the disease and draw up a treatment regimen. Always spend complex therapy, which is aimed at eliminating the inflammatory process, pain syndrome and other unpleasant symptoms, preventing the development of complications, prolonging the remission stage in chronic forms of the disease.

Main groups medicines:

• painkillers - Nurofen;
• antibacterial drops and tablets - Normax, Otofa, Flemoxin Solutab;
• antiseptics - Miramistin, Furacilin, Dioxidin;
• systemic drugs for the treatment of otomycosis - Nystatin, Levorin;
• hormonal drugs - Hydrocortisone;
• enzymes - Lidaza, Chymotrypsin;
• painkillers and anti-inflammatory ear drops - Otizol, Otipaks;
• vasoconstrictor drops to eliminate swelling of the nasopharynx - Pinosol, Sinuforte, Vibrocil;
• means for softening sulfur - Remo-vaks.

Otipax - anti-inflammatory drops for the ears

In the treatment of perforated and purulent otitis media, steroidal anti-inflammatory drugs should not be used; antibacterial agents from the group of fluoroquinolones - Normaks, Tsipromed - are used in therapy. Additionally, mucolytics are prescribed for the speedy elimination of pus - Sinupret, Erespal.

In the treatment of non-inflammatory diseases drug therapy ineffective, laser, radio waves, ultrasound, endoscopy, cryosurgery are used to eliminate dysfunction.

Prevention

To avoid ear diseases, it is necessary to follow the rules of hygiene, protect the hearing organs from the negative effects external factors, especially during therapy and after illness, timely treat diseases of the nose, throat, fungal pathologies.

How to prevent ear diseases:

• do not clean the ears with hard, foreign objects;
• clean only the outer edge of the ear with cotton swabs - the hearing organs are capable of self-cleaning, so there is no need to try to remove sulfur inside the ear canal;
• protect ears from cold, wind;
• when swimming and diving, wear a protective cap to prevent water from entering the ear;
• flu, tonsillitis, sinusitis most often cause complications in the ears, so these diseases should be treated immediately;
• try to use headphones less often;
• visit an ENT doctor 1-2 times a year for a preventive examination.

A simple exercise will help you quickly get rid of the ear plug - you need to chew chewing gum for several minutes, then gently pull the earlobe down several times. This method is suitable for small traffic jams, otherwise you will need special medicines, or the help of an ENT.

The best prevention of inflammatory diseases - strong immunity, hardening, a balanced diet, an active and healthy lifestyle will help to avoid not only ear infections, but also other serious diseases.

Biology and genetics

Hereditary hearing disorders: Hereditary hearing disorders occur under the influence of genetic factors, including as a result of congenital defects. Some researchers in a special group of factors of hearing loss distinguish factors of pathological effects on the organ of hearing of the fetus that are not related to the genetic background. Non-syndromic form of hearing loss is a form of hearing loss in which hearing loss is not accompanied by other signs or diseases of other organs and systems that would be inherited along with ...

29. Hereditary diseases of the hearing organs:

Hereditary hearing loss occurs under the influence of genetic factors, including as a result of congenital defects. Some researchers in a special group of factors of hearing loss distinguish factors of pathological effects on the organ of hearing of the fetus, not related to the genetic background. The result of such exposure, as in the case of a hereditary disease, is congenital hearing loss.

According to recent studies, more than 50% of all cases of congenital and early childhood hearing loss are associated with hereditary causes. It is believed that every eighth inhabitant of the Earth is a carrier of one of the genes that cause recessive hearing loss.

Connexin 26 gene (GJB2) was found to be the most significant for the development of hearing loss. Only one change in this gene, which is designated as the 35delG mutation, is responsible for 51% of all births of early childhood hearing loss. Other changes in this gene are also known.

Thanks to the research, it is known that in our country every 46 inhabitants are carriers of the 35delG mutation. Therefore, sadly, the probability of meeting carriers of the altered gene is quite high.

Among all cases of congenital hearing loss and / or deafness, syndromic pathology is 20-30%, non-syndromic up to 70-80%.

Non-syndromic form of hearing loss - a form of hearing loss in which hearing loss is not accompanied by other signs or diseases of other organs and systems that would be inherited along with hearing loss.

Syndromic form hearing loss accompanied (for example, Pendred's syndrome is a syndrome characterized by a combination of hearing loss and thyroid dysfunction).

The combination of hearing loss with pathology of other organs and systems, considered within the framework of known syndromes, was not found in the group with the deletion.

(bradiacusia or hypoacusia) is a deterioration in hearing of varying severity (from slight to deep), occurring suddenly or developing gradually, and due to a disorder in the functioning of the sound-perceiving or sound-conducting structures of the auditory analyzer (ear). With hearing loss, a person has difficulty hearing various sounds, including speech, as a result of which normal communication and any communication with other people is difficult, which leads to his desocialization.

Deafness is a kind of final stage of hearing loss and represents an almost complete loss of the ability to hear various sounds. With deafness, a person cannot hear even very loud sounds, which normally cause ear pain.

Deafness and hearing loss may affect only one or both ears. Moreover, hearing loss of different ears can have a different degree of severity. That is, a person can hear better with one ear and worse with the other.

Deafness and hearing loss - a brief description

Hearing loss or deafness can affect one or both ears, and the degree of its severity may be different in the right and left ear. Since the mechanisms of development, causes, as well as methods of treating hearing loss and deafness are the same, they are combined into one nosology, considering them as successive stages of one pathological process of hearing loss in a person.

Hearing loss or deafness can be caused by damage to the sound-conducting structures (organs of the middle and outer ear) or the sound-receiving apparatus (organs of the inner ear and brain structures). In some cases, hearing loss or deafness can be caused by simultaneous damage to both the sound-conducting structures and the sound-receiving apparatus of the auditory analyzer. In order to clearly understand what the defeat of one or another apparatus of the auditory analyzer means, it is necessary to know its structure and functions.

So, the auditory analyzer consists of the ear, the auditory nerve and the auditory cortex. With the help of the ears, a person perceives sounds, which are then transmitted encoded along the auditory nerve to the brain, where the received signal is processed and the sound is “recognized”. Due to the complex structure, the ear not only picks up sounds, but also "recodes" them into nerve impulses that are transmitted to the brain via the auditory nerve. The perception of sounds and their "transcoding" into nerve impulses are produced by various structures of the ear.

So, the structures of the outer and middle ear, such as the tympanic membrane and auditory ossicles (hammer, anvil and stirrup), are responsible for the perception of sounds. It is these parts of the ear that receive sound and conduct it to the structures of the inner ear (cochlea, vestibule, and semicircular canals). And in the inner ear, the structures of which are located in the temporal bone of the skull, there is a "recoding" of sound waves into electrical nerve impulses, which are then transmitted to the brain through the corresponding nerve fibers. In the brain, processing and "recognition" of sounds takes place.

Accordingly, the structures of the outer and middle ear are sound-conducting, and the organs of the inner ear, auditory nerve and cerebral cortex are sound-receiving. Therefore, the entire set of hearing loss options is divided into two large groups - those associated with damage to the sound-conducting structures of the ear or the sound-receiving apparatus of the auditory analyzer.

Hearing loss or deafness can be acquired or congenital, and depending on the time of occurrence - early or late. Early hearing loss is considered to be acquired before the child reaches the age of 3-5 years. If hearing loss or deafness appeared after the age of 5, then it refers to late.

Acquired hearing loss or deafness is usually associated with the negative impact of various external factors, such as ear injuries, past infections complicated by damage to the auditory analyzer, constant noise exposure, etc. Acquired hearing loss should be noted separately, due to age-related changes in the structure of the auditory analyzer, which not associated with any negative effects on the organ of hearing. Congenital hearing loss is usually caused by malformations, genetic abnormalities of the fetus or some infectious diseases suffered by the mother during pregnancy (rubella, syphilis, etc.).

The specific causative factor in hearing loss is determined during a special otoscopic examination conducted by an ENT doctor, audiologist or neuropathologist. In order to choose the optimal method of therapy for hearing loss, it is imperative to find out what causes hearing loss - damage to the sound-conducting or sound-perceiving apparatus.

Treatment of hearing loss and deafness is carried out by various methods, among which there are both conservative and surgical. Conservative methods are usually used to restore a sharply deteriorating hearing against the background of a known causative factor(for example, with hearing loss after taking antibiotics, after a traumatic brain injury, etc.). In such cases, with timely therapy, hearing can be restored by 90%. If conservative therapy was not carried out as soon as possible after hearing loss, then its effectiveness is extremely low. In such situations, conservative treatment methods are considered and used solely as ancillary.

Operative methods of treatment are variable and allow you to restore a person's hearing in the vast majority of cases. Most of the surgical treatment of hearing loss involves the selection, installation and adjustment of hearing aids that allow a person to perceive sounds, hear speech and interact normally with others. Another large group of methods of surgical treatment of hearing loss is to carry out very complex operations to install cochlear implants to restore the ability to perceive sounds to people who cannot use hearing aids.

The problem of hearing loss and deafness is very important, since a hard of hearing person is isolated from society, he has sharply limited opportunities for employment and self-realization, which, of course, leaves a negative imprint on the whole life of a hearing impaired person. The consequences of hearing loss in children are the most severe, as their poor hearing can lead to muteness. After all, the child has not yet mastered speech very well, he needs constant practice and further development of the speech apparatus, which are achieved only with the help of constant listening to new phrases, words, etc. And when the child does not hear speech, he can completely lose even already the existing ability to speak, becoming not only deaf, but also dumb.

It must be remembered that about 50% of cases of hearing loss can be prevented with proper observance of preventive measures. Thus, effective preventive measures are the vaccination of children, adolescents and women of childbearing age against dangerous infections, such as measles, rubella, meningitis, mumps, whooping cough, etc., which can cause complications in the form of otitis media and other ear diseases. Also effective preventive measures to prevent hearing loss are high-quality obstetric care for pregnant women and women in childbirth, proper hygiene of the auricles, timely and adequate treatment of diseases of the ENT organs, avoiding the use of drugs toxic to the auditory analyzer, as well as minimizing noise exposure to the ears in industrial and other premises (for example, , when working in noisy environments, wear ear plugs, noise canceling headphones, etc.).

Deafness and dumbness

Especially susceptible to the secondary development of dumbness are children who become deaf at the age of less than 5 years. Such children gradually lose the already learned speech skills, and they become mute due to the fact that they cannot hear speech. Children who are deaf from birth are almost always mute because they cannot acquire speech without hearing it. After all, a child learns to speak by listening to other people and trying to pronounce imitative sounds on his own. And a deaf baby does not hear sounds, as a result of which he simply cannot even try to pronounce something, imitating others. It is because of the inability to hear that deaf children from birth remain mute.

Adults who have acquired hearing loss, in very rare cases, become dumb, because their speech skills are well developed and are lost very slowly. A deaf or hard-of-hearing adult may speak strangely, drawing out words or speaking very loudly, but the ability to reproduce speech is almost never completely lost.

Deafness in one ear

Deafness in one ear according to the mechanisms of development, causes and methods of treatment is no different from any variant of acquired hearing loss.

With congenital deafness, the pathological process usually affects both ears, since it is associated with systemic disorders in the entire auditory analyzer.

Classification

Depending on which structure of the auditory analyzer is affected - sound-conducting or sound-perceiving, the whole set of different types of hearing loss and deafness is divided into three large groups:
1. Sensorineural (sensorineural) hearing loss or deafness.
2. Conductive hearing loss or deafness.
3. Mixed hearing loss or deafness.

Sensorineural (sensoneural) hearing loss and deafness

Sensorineural hearing loss is not one disease, but a whole group of various pathologies that lead to impaired functioning of the auditory nerve, inner ear, or auditory area of ​​the cerebral cortex. But since all these pathologies affect the sound-receiving apparatus of the auditory analyzer, and therefore have a similar pathogenesis, they are combined into one large group of sensorineural hearing loss. Morphologically, sensorineural deafness and hearing loss can be caused by impaired functioning of the auditory nerve and cerebral cortex, as well as anomalies in the structure of the inner ear (for example, atrophy of the sensory apparatus of the cochlea, changes in the structure of the vascular cavity, spiral ganglion, etc.) arising from genetic disorders or as a result of past illnesses and injuries.

That is, if hearing loss is associated with impaired functioning of the structures of the inner ear (cochlea, vestibule or semicircular canals), the auditory nerve ( VIII pair cranial nerves) or areas of the cerebral cortex responsible for the perception and recognition of sounds, from this it is the neurosensory variants of hearing loss.

By origin, sensorineural hearing loss and deafness can be congenital or acquired. Moreover, congenital cases of sensorineural hearing loss account for 20%, and acquired, respectively - 80%.

Cases of congenital hearing loss can be caused either by genetic disorders in the fetus, or by anomalies in the development of the auditory analyzer, arising from the adverse effects of factors environment during the period of intrauterine development. Genetic disorders in the fetus are present initially, that is, they are transmitted from parents at the time of fertilization of the egg by the sperm. If at the same time the sperm or egg have any genetic abnormalities, then the fetus will not form a full-fledged auditory analyzer during fetal development, which will lead to congenital sensorineural hearing loss. But anomalies in the development of the auditory analyzer in the fetus, which can also cause congenital hearing loss, occur during the period of bearing a child with initially normal genes. That is, the fetus received normal genes from its parents, but during the period of intrauterine growth, it was affected by any unfavorable factors (for example, infectious diseases or poisoning, suffered by a woman, etc.), which disrupted the course of his normal development, resulting in an abnormal formation of the auditory analyzer, manifested by congenital hearing loss.

In most cases, congenital hearing loss is one of the symptoms of a genetic disease (for example, Treacher-Collins, Alport, Klippel-Feil, Pendred, etc.) syndromes caused by mutations in genes. Congenital hearing loss, as the only disorder that is not combined with any other disorders of the functions of various organs and systems and is caused by developmental anomalies, is relatively rare, in no more than 20% of cases.

The causes of congenital sensorineural hearing loss, which is formed as a developmental anomaly, can be severe infectious diseases (rubella, typhoid, meningitis, etc.) suffered by a woman during pregnancy (especially during 3-4 months of gestation), intrauterine infection of the fetus with various infections (for example, toxoplasmosis, herpes, HIV, etc.), as well as poisoning of the mother with toxic substances (alcohol, drugs, industrial emissions, etc.). The causes of congenital hearing loss due to genetic disorders are the presence of genetic abnormalities in one or both parents, consanguineous marriage, etc.

Acquired hearing loss always occurs against the background of initially normal hearing, which is reduced due to the negative impact of any environmental factors. Sensorineural hearing loss of acquired genesis can be provoked by brain damage (traumatic brain injury, hemorrhage, birth trauma in a child, etc.), diseases of the inner ear (Ménière's disease, labyrinthitis, complications of mumps, otitis media, measles, syphilis, herpes, etc.). etc.), acoustic neuroma, prolonged exposure to noise on the ears, as well as taking medications that are toxic to the structures of the auditory analyzer (for example, Levomycetin, Gentamicin, Kanamycin, Furosemide, etc.).

Separately, it is necessary to highlight a variant of sensorineural hearing loss, which is called presbycusis, and consists in a gradual decrease in hearing as you grow older or age. With presbycusis, hearing is lost slowly, and at first the child or adult stops hearing high frequencies (birdsong, squeaking, telephone ringing, etc.), but perceives low tones well (the sound of a hammer, a passing truck, etc.). Gradually, the spectrum of perceived frequencies of sounds narrows due to an increasing deterioration in hearing to higher tones, and, ultimately, a person stops hearing at all.

Conductive hearing loss and deafness

It must be understood that conductive hearing loss and deafness are not one pathology, but a whole group of the most various diseases and states united by the fact that they affect the sound-conducting system of the auditory analyzer.

With conductive hearing loss and deafness, the sounds of the surrounding world do not reach the inner ear, where they are "recoded" into nerve impulses and from where they enter the brain. Thus, a person does not hear because the sound does not reach the organ that can transmit it to the brain.

As a rule, all cases of conductive hearing loss are acquired and are caused by various diseases and injuries that disrupt the structure of the outer and middle ear (for example, sulfur plugs, tumors, otitis media, otosclerosis, damage to the eardrum, etc.). Congenital conductive hearing loss is rare and is usually one of the manifestations of a genetic disease caused by gene abnormalities. Congenital conductive hearing loss is always associated with anomalies in the structure of the outer and middle ear.

Mixed hearing loss and deafness

Depending on the period in a person's life, hearing loss appeared, congenital, hereditary and acquired hearing loss or deafness are distinguished.

Hereditary hearing loss and deafness

Hereditary hearing loss can manifest itself at different ages, i.e. it is not necessarily congenital. So, with hereditary hearing loss, only 20% of children are born already deaf, 40% begin to lose their hearing in childhood and the remaining 40% note a sudden and causeless hearing loss only in adulthood.

Hereditary hearing loss is caused by certain genes, which are usually recessive. This means that a child will only have hearing loss if he or she receives recessive deafness genes from both parents. If a child receives a dominant gene for normal hearing from one of the parents, and a recessive gene for deafness from the second, then he will hear normally.

Since the genes for hereditary deafness are recessive, this type of hearing impairment, as a rule, occurs in closely related marriages, as well as in unions of people whose relatives or they themselves suffered from hereditary hearing loss.

The morphological substrate of hereditary deafness can be various disorders of the structure of the inner ear, which arise due to defective genes passed on to the child by the parents.

Hereditary deafness, as a rule, is not the only health disorder that a person has, but in the vast majority of cases it is combined with other pathologies that are also of a genetic nature. That is, usually hereditary deafness is combined with other pathologies that also developed as a result of anomalies in the genes passed on to the child by the parents. The most common hereditary deafness is one of the symptoms genetic diseases, which are manifested by a whole complex of signs.

Currently, hereditary deafness, as one of the symptoms of a genetic anomaly, occurs in the following diseases associated with abnormalities in the genes:

• Treacher-Collins Syndrome(deformation of the bones of the skull);
• Alport syndrome(glomerulonephritis, hearing loss, reduced functional activity of the vestibular apparatus);
• Pendred syndrome(violation of thyroid hormone metabolism, large head, short arms and legs, enlarged tongue, disorder of the vestibular apparatus, deafness and dumbness);
• LEOPARD syndrome(cardiopulmonary insufficiency, anomalies in the structure of the genital organs, freckles and age spots all over the body, deafness or hearing loss);
• Klippel-Feil syndrome(violation of the structure of the spine, arms and legs, incompletely formed external auditory canal, hearing loss).

Deafness genes

The most common genes for deafness are:

• OTOF(the gene is located on chromosome 2 and, if present, a person suffers from hearing loss);
• GJB2(with a mutation in this gene, called 35 del G, a person develops hearing loss).

Congenital hearing loss and deafness

Congenital hearing loss can occur when the body of a pregnant woman is exposed to the following adverse factors:

• Damage to the central nervous system child due to birth trauma (for example, hypoxia due to entanglement of the umbilical cord, compression of the bones of the skull due to the imposition of obstetric forceps, etc.) or anesthesia. In these situations, hemorrhages occur in the structures of the auditory analyzer, as a result of which the latter is damaged and the child develops hearing loss.
• Infectious diseases suffered by a woman during pregnancy , especially at 3-4 months of gestation, that can disrupt the normal formation of the fetal hearing apparatus (for example, influenza, measles, chickenpox, mumps, meningitis, cytomegalovirus infection, rubella, syphilis, herpes, encephalitis, typhoid fever, otitis media, toxoplasmosis, scarlet fever, HIV). The causative agents of these infections are able to penetrate the fetus through the placenta and disrupt the normal course of the formation of the ear and auditory nerve, resulting in hearing loss in a newborn child.
• Hemolytic disease of the newborn. With this pathology, hearing loss occurs due to a violation of the blood supply to the central nervous system of the fetus.
• Severe somatic diseases of a pregnant woman, accompanied by vascular damage (e.g. diabetes mellitus, nephritis, thyrotoxicosis, cardiovascular diseases). With these diseases, hearing loss occurs due to insufficient blood supply to the fetus during pregnancy.
• Smoking and alcohol consumption during pregnancy.
• Constant exposure to the body of a pregnant woman of various industrial poisons and toxic substances (for example, when living in a region with unfavorable environmental conditions or working in hazardous industries).
• The use of drugs during pregnancy that are toxic to the auditory analyzer (for example, Streptomycin, Gentamycin, Monomycin, Neomycin, Kanamycin, Levomycetin, Furosemide, Tobramycin, Cisplastin, Endoxan, Quinine, Lasix, Uregit, Aspirin, ethacrynic acid, etc.).

Acquired hearing loss and deafness

So, the possible causes of acquired hearing loss or deafness are any factors that lead to a violation of the structure of the ear, auditory nerve or cerebral cortex. These factors include severe or chronic diseases ENT organs, complications of infections (for example, meningitis, typhoid, herpes, mumps, toxoplasmosis, etc.), head trauma, contusion (for example, a kiss or a loud scream directly into the ear), swelling and inflammation of the auditory nerve, prolonged exposure to noise , circulatory disorders in the vertebrobasilar basin (for example, strokes, hematomas, etc.), as well as taking medications that are toxic to the auditory analyzer.

According to the nature and duration of the course of the pathological process, hearing loss is divided into acute, subacute and chronic.

Acute hearing loss

Acute hearing loss does not develop all at once, but gradually, and at the initial stage, a person feels congestion in the ear or tinnitus, and not hearing impairment. A feeling of fullness or tinnitus may come and go intermittently as early warning signs of upcoming hearing loss. And only some time after the appearance of a feeling of congestion or tinnitus, a person experiences a persistent hearing loss.

The causes of acute hearing loss are various factors that damage the structures of the ear and the area of ​​the cerebral cortex responsible for the recognition of sounds. Acute hearing loss can occur after a head injury, after infectious diseases (eg, otitis media, measles, rubella, mumps, etc.), after hemorrhages or circulatory disorders in the structures of the inner ear or brain, and after taking toxic to the ear drugs (for example, Furosemide, Quinine, Gentamicin), etc.

Acute hearing loss is amenable to conservative therapy, and the success of treatment depends on how quickly it is started relative to the appearance of the first signs of the disease. That is, the earlier the treatment of hearing loss is started, the greater the likelihood of hearing normalization. It must be remembered that successful treatment of acute hearing loss is most likely when therapy is started within the first month after hearing loss. If more than a month has passed since the hearing loss, then conservative therapy, as a rule, turns out to be ineffective and only allows you to maintain hearing at the current level, preventing it from worsening even more.

Among cases of acute hearing loss, sudden deafness is also distinguished into a separate group, in which a person experiences a sharp deterioration in hearing within 12 hours. Sudden deafness appears abruptly, without any preliminary signs, against the background of complete well-being, when a person simply stops hearing sounds.

As a rule, sudden deafness is unilateral, that is, the ability to hear sounds is reduced in only one ear, while the other remains normal. In addition, sudden deafness is characterized by severe hearing loss. This form of hearing loss is caused by viral infections, and therefore prognostically more favorable compared to other types of deafness. Sudden hearing loss responds well conservative treatment, thanks to which you can fully restore hearing in more than 95% of cases.

Subacute hearing loss

Chronic hearing loss

With chronic hearing loss, hearing loss is combined with constant noise or ringing in the ears, which is not heard by others, but is very difficult to bear by the person himself.

Deafness and hearing loss in a child

The course, mechanisms of development and treatment of deafness and hearing loss in children are the same as in adults. However, the treatment of hearing loss in children is given more importance than in adults, since for this age group, hearing is critical for mastering and maintaining speech skills, without which the child will become not only deaf, but also dumb. Otherwise, there are no fundamental differences in the course, causes and treatment of hearing loss in children and adults.

The reasons

The causative factors of congenital hearing loss are various negative effects on a pregnant woman, which, in turn, lead to disruption of the normal growth and development of the gestating fetus. Therefore, the causes of congenital hearing loss are factors that affect not so much the fetus itself, but the pregnant woman. So, Possible causes of congenital and genetic hearing loss are the following factors:

• Damage to the central nervous system of the child due to birth trauma (for example, hypoxia against the background of entanglement of the umbilical cord, compression of the bones of the skull when applying obstetric forceps, etc.);
• Damage to the central nervous system of a child with drugs for anesthesia administered to a woman during childbirth;
• Infections experienced by a woman during pregnancy that may interfere with the normal development of the fetal hearing apparatus (eg, influenza, measles, chickenpox, mumps, meningitis, cytomegalovirus infection, rubella, syphilis, herpes, encephalitis, typhoid fever, otitis media, toxoplasmosis, scarlet fever , HIV);
• Hemolytic disease of the newborn;
• Pregnancy proceeding against the background of severe somatic diseases in a woman, accompanied by vascular damage (for example, diabetes, nephritis, thyrotoxicosis, cardiovascular diseases);
• Smoking, alcohol or drug use during pregnancy;
• Constant exposure to the body of a pregnant woman of various industrial poisons (for example, a permanent stay in a region with an unfavorable environmental situation or work in hazardous industries);
• Use during pregnancy of drugs that are toxic to the auditory analyzer (for example, Streptomycin, Gentamicin, Monomycin, Neomycin, Kanamycin, Levomycetin, Furosemide, Tobramycin, Cisplastin, Endoxan, Quinine, Lasix, Uregit, Aspirin, ethacrynic acid, etc.);
• Pathological heredity (transmission of deafness genes to a child);
• closely related marriages;
• The birth of a child premature or with low body weight.

• Birth trauma (a child during childbirth may receive an injury to the central nervous system, which will subsequently lead to hearing loss or deafness);
• Bleeding or bruising in the middle or inner ear or in the cerebral cortex;
• Violation of blood circulation in the vertebrobasilar basin (a set of vessels that supply blood to all structures of the skull);
• Any damage to the central nervous system (for example, traumatic brain injury, brain tumors, etc.);
• Operations on the organs of hearing or brain;
• Complications on the structures of the ear after suffering inflammatory diseases, such as, for example, labyrinthitis, otitis media, measles, scarlet fever, syphilis, mumps, herpes, Meniere's disease, etc.;
• Acoustic neuroma;
• Prolonged effect of noise on the ears (for example, frequent listening to loud music, working in noisy workshops, etc.);
• Chronic inflammatory diseases of the ears, throat and nose (for example, sinusitis, otitis media, eustachitis, etc.);
• Chronic ear pathologies (Ménière's disease, otosclerosis, etc.);
• Hypothyroidism (deficiency of thyroid hormones in the blood);
• Taking medications that are toxic to the auditory analyzer (for example, Streptomycin, Gentamicin, Monomycin, Neomycin, Kanamycin, Levomycetin, Furosemide, Tobramycin, Cisplastin, Endoxan, Quinine, Lasix, Uregit, Aspirin, ethacrynic acid, etc.);
• Sulfur plugs;
• Damage to the eardrums;
• Age-related hearing loss (presbycusis) associated with atrophic processes in the body.

Signs (symptoms) of deafness and hearing loss

You need to know that with hearing loss various degrees gravity, a person loses the ability to perceive certain spectra of sounds. So, with mild hearing loss, the ability to hear high and quiet sounds, such as whispers, squeaks, phone calls, birdsong, is lost. When the hearing loss worsens, the ability to hear the following sound spectra in height, that is, quiet speech, the rustle of the wind, etc., disappears. As the hearing loss progresses, the ability to hear sounds belonging to the upper spectrum of perceived tones disappears, and discrimination of low sound vibrations remains, such like the rumble of a truck, etc.

A person, especially in childhood, does not always understand that he has a hearing loss, since the perception of a wide range of sounds remains. That's why To identify hearing loss, it is necessary to take into account the following indirect signs of this pathology:

• Frequent asking;
• The absolute lack of reaction to the sounds of high tones (for example, the trills of birds, the squeak of a bell or telephone, etc.);
• Monotonous speech, incorrect placement of stresses;
• Too loud speech;
• shuffling gait;
• Difficulties in maintaining balance (noted with sensorineural hearing loss due to partial damage to the vestibular apparatus);
• Lack of reaction to sounds, voices, music, etc. (normally, a person instinctively turns towards the sound source);
• Complaints of discomfort, noise or ringing in the ears;
• The complete absence of any emitted sounds in infants (with congenital hearing loss).

Degrees of deafness (hard of hearing)

• I degree - mild (hearing loss 1)- a person does not hear sounds, the volume of which is less than 20 - 40 dB. With this degree of hearing loss, a person hears a whisper from a distance of 1 - 3 meters, and ordinary speech - from 4 - 6 meters;
• II degree - medium (hearing loss 2)- a person does not hear sounds whose volume is less than 41 - 55 dB. With moderate hearing loss, a person hears speech at normal volume from a distance of 1-4 meters, and a whisper - from a maximum of 1 meter;
• III degree - severe (hearing loss 3)- a person does not hear sounds whose volume is less than 56 - 70 dB. With moderate hearing loss, a person hears speech at normal volume from a distance of no more than 1 meter, and no longer hears a whisper at all;
• IV degree - very severe (hearing loss 4)- a person does not hear sounds whose volume is less than 71 - 90 dB. With moderate hearing loss, a person does not hear speech at normal volume;
• V degree - deafness (hard of hearing 5)– a person cannot hear sounds whose volume is less than 91 dB. In this case, a person hears only a loud cry, which normally can be painful for the ears.

How to define deafness?

To determine the type, degree and specific characteristics of hearing loss, the following methods are used:

• Audiometry(the ability of a person to hear sounds of different heights is being studied);
• Tympanometry(the bone and air conduction of the middle ear is examined);
• Weber test(allows you to identify whether one or both ears are involved in the pathological process);
• Tuning fork test - Schwabach test(allows you to identify the type of hearing loss - conductive or neurosensory);
• Impedancemetry(allows to identify the localization of the pathological process that led to hearing loss);
• Otoscopy(examination of ear structures with special instruments in order to identify defects in the structure of the tympanic membrane, external auditory canal, etc.);
• MRI or CT (the cause of the hearing loss is revealed).

The biggest problem is the detection of hearing loss in infants, since they, in principle, still do not speak. Applied to children infancy they use adapted audiometry, the essence of which is that the child must respond to sounds with head turns, various movements, etc. If the baby does not respond to sounds, then he suffers from hearing loss. In addition to audiometry, impedancemetry, tympanometry, and otoscopy are used to detect hearing loss in young children.

Treatment

General principles of therapy

• Medical therapy(used for detoxification, improving blood circulation in brain and ear structures, eliminating the causative factor);
• Physiotherapy methods(used to improve hearing, detoxification);
• Auditory exercises(used to maintain the level of hearing and improve speech skills);
• Surgical treatment(operations to restore the normal structure of the middle and outer ear, as well as to install a hearing aid or cochlear implant).

Sensorineural hearing loss is much more difficult to treat, and therefore all possible methods and their combinations. Moreover, there are some differences in the tactics of treating acute and chronic sensorineural hearing loss. So, in case of acute hearing loss, a person needs to be hospitalized as soon as possible in the specialized department of the hospital and carried out drug treatment and physiotherapy to restore the normal structure of the inner ear and thereby restore hearing. Specific methods of treatment are chosen depending on the nature of the causative factor (viral infection, intoxication, etc.) of acute neurosensory hearing loss. With chronic hearing loss, a person periodically undergoes treatment courses aimed at maintaining the existing level of sound perception and preventing possible hearing loss. That is, in acute hearing loss, treatment is aimed at restoring hearing, and in chronic hearing loss, it is aimed at maintaining the existing level of sound recognition and preventing hearing loss.

Therapy for acute hearing loss is carried out depending on the nature of the causative factor that provoked it. So, today there are four types of acute neurosensory hearing loss, depending on the nature of the causative factor:

• Vascular hearing loss- provoked by a violation of blood circulation in the vessels of the skull (as a rule, these violations are associated with vertebrobasilar insufficiency, hypertension, strokes, cerebral atherosclerosis, diabetes mellitus, diseases of the cervical spine);
• Viral hearing loss- provoked by viral infections (infection causes inflammation in the inner ear, auditory nerve, cerebral cortex, etc.);
• Toxic hearing loss- provoked by poisoning with various toxic substances (alcohol, industrial emissions, etc.);
• Traumatic hearing loss- provoked by trauma to the skull.

Chronic neurosensory hearing loss is treated comprehensively, periodically conducting courses of medication and physiotherapy. If conservative methods are ineffective, and the hearing loss has reached III-V degree, then surgical treatment is performed, which consists in installing a hearing aid or cochlear implant. Of the medicines for the treatment of chronic neurosensory hearing loss, B vitamins are used (Milgamma, Neuromultivit, etc.), aloe extract, as well as agents that improve metabolism in brain tissues (Solcoseryl, Actovegin, Preductal, Riboxin, Nootropil, Cerebrolysin, Pantocalcin, etc. ). Periodically, in addition to specified drugs, for the treatment of chronic hearing loss and deafness, Prozerin and Galantamine are used, as well as homeopathic remedies (for example, Cerebrum Compositum, Spascuprel, etc.).

Among the physiotherapeutic methods for the treatment of chronic hearing loss, the following are used:

• Laser irradiation of blood (helium-neon laser);
• Stimulation by fluctuating currents;
• Quantum hemotherapy;
• Phonoelectrophoresis endural.

Surgical treatment of deafness (hard of hearing)

Operations for the treatment of conductive deafness consist in restoring the normal structure and organs of the middle and outer ear, due to which the person regains hearing. Depending on which structure is being restored, the operations are named accordingly. For example, myringoplasty is an operation to restore the eardrum, tympanoplasty is the restoration of the auditory ossicles of the middle ear (stapes, hammer and incus), etc. After such operations, as a rule, hearing is restored in 100% of cases.

There are only two operations for the treatment of neurosensory deafness - these are hearing aid or cochlear implant placement. Both options for surgical intervention are performed only with the ineffectiveness of conservative therapy and with severe hearing loss, when a person cannot hear normal speech even at close range.

Fitting a hearing aid is a relatively simple operation, but unfortunately it will not restore hearing to those who have damage to the sensitive cells in the cochlea of ​​the inner ear. In such cases effective method hearing restoration is the installation of a cochlear implant. The operation to install an implant is technically very complicated, therefore it is carried out in a limited number of medical institutions and, accordingly, is expensive, as a result of which it is not accessible to everyone.

The essence of a cochlear prosthesis is as follows: mini-electrodes are introduced into the structures of the inner ear, which will recode sounds into nerve impulses and transmit them to the auditory nerve. These electrodes are connected to a mini-microphone placed in the temporal bone, which picks up sounds. After installing such a system, the microphone picks up sounds and transmits them to electrodes, which, in turn, recode them into nerve impulses and output them to the auditory nerve, which transmits signals to the brain, where sounds are recognized. That is, cochlear implantation is, in fact, the formation of new structures that perform the functions of all ear structures.

Hearing aids for the treatment of hearing loss

Analogue hearing aids are well-known devices that are seen behind the ear in older people. They are quite easy to use, but bulky, not very convenient and quite rude in amplifying the audio signal. An analog hearing aid can be purchased and used independently without special setup by a specialist, since the device has only a few modes of operation, which are switched by a special lever. Thanks to this lever, a person can independently determine the optimal mode of operation of the hearing aid and use it in the future. However, analog hearing aids often create interference, amplify different frequencies, and not just those that a person does not hear well, as a result of which its use is not very comfortable.

A digital hearing aid, unlike analog, is adjusted exclusively by a hearing care professional, thereby amplifying only those sounds that a person cannot hear well. Thanks to the precision of tuning, the digital hearing aid allows a person to hear perfectly without interference and noise, restoring sensitivity to the lost spectrum of sounds and without affecting all other tones. Therefore, in terms of comfort, convenience and accuracy of correction, digital hearing aids are superior to analog ones. Unfortunately, in order to select and adjust a digital device, it is necessary to visit a hearing aid center, which is not available to everyone. There are various models of digital hearing aids available today, so you can choose best option for each individual person.

Treatment of deafness with cochlear implantation: the device and principle of operation of a cochlear implant, a commentary by a surgeon - video

Sensorineural hearing loss: causes, symptoms, diagnosis (audiometry), treatment, advice from an otorhinolaryngologist - video

Sensorineural and conductive hearing loss: causes, diagnosis (audiometry, endoscopy), treatment and prevention, hearing aids (opinion of an ENT doctor and an audiologist) - video

Hearing loss and deafness: how the auditory analyzer works, causes and symptoms of hearing loss, hearing aids (hearing aids, cochlear implantation in children) - video

Hearing loss and deafness: exercises to improve hearing and eliminate tinnitus - video

Hearing impairment is a reduction in a person's ability to pick up the sounds of the 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.

Hearing loss 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 arterial 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 carried by the child at an early age 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 infectious processes in the ears, which cause not only hearing loss, but also, in some cases, life-threatening diseases such as brain abscesses or meningitis.
• Otitis media, which causes a buildup of fluid in the middle ear.
• Therapeutic uses of 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 inflammatory disease 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.

Otitis media chronic form is main reason 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 external 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). The exudate may have a serous form, which is typical for viral infections, or a 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, which are of a protracted nature, can penetrate into inner ear and damage 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, transferred by the mother, mumps and others.
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 medicines.
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 various syndromes, 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 of genetic hearing impairment, 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

Most people with acquired hearing loss are elderly or old. 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 communicative 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. Wherein cognitive processes and cognitive activity are also deficient 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 cerebral palsy and other movement 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 internal organs severe (heart, kidney, lung, 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 this child to 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 speech development defects. 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.
• The 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 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 sound signals 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 type of hearing loss, sound signals cannot be transmitted normally from the outer and middle ear to the inner, and there are also disturbances in the functioning 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 hearing impairment. 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. An easy degree is characterized by the perception of spoken language at a distance of six to eight meters, and a whispered one - 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 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 correct 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, it is enough a rare occurrence It is considered a violation of the functioning of exclusively auditory areas of the brain, which is called central hearing loss. 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 of varying degrees - from low to high, as well as complete hearing loss. Experts believe that sensorineural hearing loss is most often caused by pathological changes functioning of 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 certain 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 the understanding of single words or short phrases, especially those that are used everyday and are part of the patient's daily routine. In this case, there is a disorder of phonemic hearing.

Severe cases of sensory aphasia are characterized by a complete incomprehension of speech by a sick person, 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. At the same time, in these patients, musical ear is preserved, as well as articulation.

Conductive hearing loss

Conductive hearing impairment is caused by the impossibility of its normal conduction 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 to the ear canal and tympanic membrane, as well as pathological conditions 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 hearing impairment, namely the appearance of illegible 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 tone audiogram appears, which is only this person inherent characteristic of auditory perception.
• 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. The use of certain methods of 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

Logopedic work with hearing impairment has great importance because hearing-impaired children have speech problems 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 individual characteristics 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 are used that 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 cerebral cortex, which are responsible for understanding speech, the possibility of correctly structuring sentences, for developed vocabulary, good diction and desire for communicative contact.
• 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 features. 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 - the 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 impaired hearing 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 systems and control systems.

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 moment 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.

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. Implementation 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 individual means protection, as well as raising employee awareness of the dangers of long-term excessive noise and developing 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 for early stages diseases will allow timely application of conservative or surgical methods treatment that can save the patient's hearing.
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The results of epidemiological studies conducted in Russia in recent years indicate that about 1 million children and adolescents in our country have a hearing pathology, and the All-Union Society of the Deaf and Dumb has more than 1.5 million hearing impaired. Calculations show that for every 1,000 physiological births, one deaf child is born. In addition, during the first 2-3 years of life, another 2-3 children lose their hearing. 14% of people aged 45 to 64 and 30% of people over 65 have hearing loss. According to experts, by 2020 more than 30% of the entire population of the globe will have hearing impairments.

Good hearing is a prerequisite for the normal psycho-speech development of the baby. Hearing-impaired child often lags behind peers in mental development, it is difficult for him to study at school, inevitable difficulties in communication, choice future profession. Hearing loss in early childhood, before the child develops speech, leads to muteness and disability.

Hearing impairment in most cases in the form of a decrease in its severity can be due to various causes (congenital and acquired), among which heredity, prematurity, neonatal jaundice are of great importance, cerebral paralysis, side effect certain drugs (thalidomide), primarily antibiotics (streptomycin, kanamycin, monomycin, especially in the first 3 months of pregnancy) and diuretics, quinine poisoning, alcohol consumption during pregnancy, malformations, infectious (syphilis), including viral (rubella, chickenpox, measles, influenza) diseases, head injuries, noise exposure, age-related changes.

The decrease in hearing acuity in such cases can reach significant severity, up to deafness. The severity of the disturbances and the difficulty (often impossible) of restoring auditory function in this case are mainly associated with damage to the sound-perceiving (sensory) formations of the inner ear and auditory nerve (sensory hearing loss).

Absolute deafness is rare. There are usually remnants of hearing that allow the perception of very intense sounds, including some speech sounds uttered in a loud voice above the ear. Legible perception of speech in deafness is not achieved; this is different from hearing loss, in which sufficient amplification of sound provides the possibility of verbal communication. A strict distinction between deafness and hearing loss presents a certain difficulty, depends on the method of hearing research and to some extent is conditional.

Usually deafness called a pronounced persistent hearing loss that prevents speech communication in any conditions, even with the use of sound amplifying equipment. However, such patients can hear some very loud sounds. The absolute impossibility of perceiving any sounds is extremely rare.

hearing loss called a decrease in hearing acuity of varying severity, in which the perception of speech is difficult, but still possible when certain conditions are created (approaching the speaker to the deaf, the use of a hearing aid).

Hearing impairment, depending on the pathogenesis, can be neurosensory or conductive in nature. With a neurosensory disorder, there is a pathology of the sound-perceiving apparatus (sensitive cells of the cochlea of ​​the auditory nerve and brain structures). With conductive deafness (hearing loss), the disturbance covers the sound-conducting system (outer and middle ear, middle ear fluid).

For an approximate assessment of the state of hearing, you can use whispered and colloquial speech (speech audiometry). With a mild degree of hearing loss, whispered speech is perceived by the patient from a distance of 1 - 3 m, conversational speech - from a distance of 4 m or more. At medium degree T. whispered speech is perceived from a distance of less than 1 m, colloquial speech - from a distance of 2 - 4 m. With a severe degree of hearing loss, whispered speech, as a rule, is not perceived at all, colloquial speech is perceived from a distance of less than 1 m. A more accurate determination of the degree of hearing loss is carried out using tone audiometry. Mild degree of hearing loss refers to hearing loss in the tones of the speech range within 40 dB, moderate - about 60 dB, severe - about 80 dB. Major hearing loss is referred to as deafness.

In the diagnosis of hearing loss, tuning forks have not lost their significance. They are used mainly in the conditions of the clinic, and especially if it is necessary to examine the patient at home. To determine the auditory threshold (the minimum sound intensity still perceived by the subject's ear during air conduction), which characterizes hearing acuity, the tuning fork is placed at the entrance to the external auditory canal so that the tuning fork axis (the transverse line between its branches) is in line with the axis of the auditory passage in the immediate vicinity; while the tuning fork should not touch the tragus and hair.

Identification of hearing loss in young children presents significant difficulties, since the child cannot give an answer about the presence or absence of auditory sensation. Recently, the study of hearing in children has been carried out using computer technology by recording auditory evoked potentials, which are an electrical response to sound stimulation, which varies depending on the location of the potential tap and its parameters (computer audiometry).

According to auditory evoked potentials, one can judge the presence of an auditory reaction in a child of any age, and, if necessary, in the fetus. In addition, this study allows you to make an objective conclusion about the degree of hearing loss, the location of the lesion of the auditory pathway, as well as assess the prospects rehabilitation measures. The method of objective assessment of hearing in children according to the measurement of acoustic resistance (impedance) of the middle ear has become more widespread in audiological practice.

Hearing loss can be genetically determined. Both dominant and recessive genes are responsible for hereditary deafness. Genetic disorders are perhaps the main cause of sensorineural hearing loss in children. They explain about half of the cases of profound deafness in children.

The most common genetic disorders are: Usher's syndrome, which occurs in 3-10% of patients with congenital deafness, 1 out of 70 people is a carrier of the recessive gene for Usher's syndrome; Waardenburg syndrome, fixed in 1 - 2% of cases; Elport syndrome - 1%. In total, more than 400 different syndromes are known with various types inheritance.

At Usher's syndrome deafness, vestibular disorders and pigmentary degeneration retina leading to blindness. Most people with Usher syndrome are born with severe hearing loss. One of the first noticeable symptoms of visual impairment is poor eyesight at night or in insufficiently consulted places - a violation of dark adaptation (night blindness).

Night ("night") blindness in most cases manifests itself in adolescence. Later, there is a gradual loss of lateral (peripheral) vision to the so-called "tunnel", although central vision can be quite high for a long time, practically without suffering.

The symptoms of Usher's syndrome usually progress over the years. Many people with Usher syndrome also have some balance problems. Usher's syndrome is inherited in a classical recessive manner. When two heterozygous gene carriers marry, there is a 1:4 chance that the child born will suffer from Usher syndrome. There is currently no way to recognize carriers of the gene.

Waardenburg syndrome described by Waardenburg in 1951. Among children with congenital deafness, patients with Waanrenburg syndrome make up about 3%. Variable gene expression is an important reason for the wide clinical polymorphism of this syndrome. This disease is inherited in an autosomal dominant manner. With Waardenburg syndrome, there is:

1) outward displacement of the inner corners of the eyes and lacrimal openings with a normal distance between the pupils and the outer corners of the eyes (99%);

2) high wide nose bridge (75%), hypoplasia of the nostrils and fused eyebrows (50%); "profile of a Roman legionnaire";

3) violation of pigmentation, manifested in the form of a gray or piebald strand of hair on the head, usually above the forehead (17-45%), heterochromia of the iris (50%), depigmented areas on the skin and fundus;

4) congenital bilateral sensorineural deafness (20%) or hearing loss due to hypoplasia of the organ of Corti.

In addition to these symptoms, there are often mental retardation, high palate, sometimes with a cleft, small skeletal anomalies and heart defects. The most severe of all manifestations of Waardenburg syndrome are hearing impairments, occurring in 20% of cases.

The degree of hearing impairment can vary from subclinical to deep complete deafness. Sound perception disorders are often bilateral, symmetrical. characteristic feature disease is hypotrophy of the vestibular apparatus. With the help of caloric and rotational tests, vestibular disorders are detected in almost 75% of patients, that is, even more often than hearing impairment.

Pathomorphological data suggest that patients with Waardenburg syndrome may experience severe morphological changes in the inner ear, for example, aplasia of the Scheibe type or even complete absence spiral organ and neurons in the spiral node. These changes result from a hereditary degenerative process that begins in utero and destroys a normally formed ear. Apparently, the dominant gene that causes this syndrome causes defective development of the primordium, from which cells are subsequently formed that have different functions, namely, cells of the sensory epithelium of the spiral organ, neurons of the spiral node, pigment cells, and certain groups of mesenchymal cells. Defective formation of the rudiment leads to disruption of the processes of migration and differentiation of the cells formed from it, and in the future - to the inferiority of the corresponding systems.

At Alport syndrome deafness is combined with glomerulonephritis, leading to renal failure. One of the symptoms of neurofibromatosis type II is also deafness due to the formation of bilateral schwannoma of the vestibulocochlear nerve.

Pendred syndrome described in 1896. Pendred. The disease is characterized by a combination of congenital or manifested in early childhood sensorineural deafness with goiter, which develops due to a violation of the biosynthesis of thyroid hormones. A genetic lesion of the inner ear occurs with Pendred syndrome in utero, after birth, degenerative changes in the cochlea only continue to progress. They have progressive hearing loss in the first year of life. In about half of the cases, children with Pendred syndrome have complete deafness, the rest have hearing loss of II-III degree. The perception of high frequencies is more sharply disturbed. The hearing loss is usually bilateral and symmetrical. Almost all patients have vestibular disorders.

To diagnose Pendred's syndrome, a perchlorate test is used to confirm thyroid dysfunction. In children with Pendred's syndrome, the radioactivity of the thyroid gland is reduced. hormone therapy, although it does not affect the hearing defect, it stops the development of goiter and normalizes the function of the thyroid gland.

The disease is inherited in an autosomal recessive manner, so the parents of affected children are usually healthy. The frequency of Pendred's syndrome, for example, among the population of England reaches 1: 13,000.

In addition to syndromic forms of hereditary deafness, non-syndromic (isolated) forms are distinguished, of which several have been discovered to date. The first gene for isolated hereditary deafness was mapped on the long arm of the 5th chromosome in members of a family from Costa Rica. The mode of inheritance was autosomal dominant. The mapped gene for isolated autosomal recessive deafness includes the GJB2/connexin26 gene, which transmits non-syndromic sensorineural DFNB1 deafness, which occurs in the Caucasoid population.

The frequency of congenital severe hearing loss or deafness is 1:1000 newborns. At least half of these cases are genetically determined. More often, hereditary hearing impairments are not congenital, but develop in childhood or even later. In 80% of cases, these disorders are inherited autosomal recessively, in 15-20% - autosomal dominantly, and in less than 1% - recessively X-linked. Autosomal dominant deafness is bilateral and is divided into 2 types: childhood, occurring before the age of 15 and adult, developing at a later age. X-linked deafness was discovered in 1965. Fraser Y.R. boys had a sharp impairment of perception at high frequencies with a threshold of 80 dB to 100 dB.