Anomalies in the development of the outer ear. Middle ear anomalies

1. Defects and damage inner ear. Congenital defects include developmental anomalies of the inner ear that have various forms. There were cases of complete absence of the labyrinth or underdevelopment of its individual parts. Most birth defects the inner ear is marked by underdevelopment of the organ of Corti, and it is the specific terminal apparatus that is undeveloped auditory nerve- hair cells.

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

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

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

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

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

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

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

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

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

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

2. Noise damage. Prolonged exposure to noise develops degenerative changes in the hair cells of the organ of Corti, extending to nerve fibers and on the cells of the spiral ganglion.

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

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

According to World Organization health care, up to 15% of children are born with clear signs various developmental anomalies. However, congenital anomalies may appear later, so in general the frequency of malformations is much higher. It has been established that in children born to older mothers, anomalies are more common, since the older the woman, the greater the volume harmful effects external environment(physical, chemical, biological) on her body. Developmental anomalies in children born to parents with developmental anomalies are 15 times more common than in children born to healthy parents.

Congenital malformations of the external and middle ear occur with a frequency of 1-2 cases per 10,000 newborns.

The inner ear appears as early as the fourth week of embryonic development. The middle ear develops later, and by the time the baby is born tympanic cavity contains a jelly-like tissue that subsequently disappears. The outer ear appears in the fifth week prenatal development.

In a newborn, the auricle can be enlarged (hypergenesis, macrotia) or reduced (hypogenesis, microtia), which is usually combined with an infection of the external auditory canal. Only some of its departments can be excessively increased or reduced (for example, earlobe). Developmental anomalies can be unilateral or bilateral and manifest as ear appendages, several auricles (polyotia). Urine splitting occurs congenital fistulas ear, atresia (absence) of the external auditory canal. The auricle may be absent, occupy an unusual place. With microtia, it can be located in the form of a rudiment on the cheek (cheek ear), sometimes only the lobe is preserved auricle or skin-cartilaginous roller with a lobe.

The auricle can be folded, flat, ingrown, corrugated, angular (macaque ear), pointed (satyr ear). The auricle can be with a transverse cleft, and the lobe with a longitudinal one. Other defects of the lobe are also known: it can be adherent, large, lagging behind. Often combined forms of defects of the outer ear. Anomalies in the development of the auricle and the external auditory canal are often combined in the form of its partial underdevelopment or complete absence. Such anomalies are described as syndromes. Yes, malformation connective tissue, in which many organs are affected, including the auricles, is called Marfan's syndrome. There are congenital deformities of both auricles in members of the same family (Potter's syndrome), bilateral microtia in members of the same family (Kessler's syndrome), ophthalmic dysplasia (Goldenhar's syndrome).

With macrotia (an increase in the size of the auricle), taking into account the variety of changes, a number of surgical interventions. If, for example, the auricle is enlarged evenly in all directions, that is, it has oval shape, excess tissue can be excised. Operations to restore the auricle in its absence are quite complicated because skin is needed, and it is necessary to create an elastic skeleton (support), around which the auricle is formed. To form the skeleton of the auricle, cartilage of the rib, cartilage of the auricle of a corpse, bone and synthetic materials are used. Ear pendants located near the auricle are removed along with the cartilage.

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

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

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

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

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

Among psychiatrists for some time, the idealistic views of Morel (Morel) dominated, who believed that a change in the auricle is a sign of mental inferiority (Morel's ear). It is currently believed that pinna anomalies do not matter in the assessment mental state personality.

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

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

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

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

At present, with increased frequency, they began to manifest birth defects development various bodies with which doctors of almost any specialty have to deal.

Moreover, complications can be both purely cosmetic and functional in nature.

Often various pathologies accompanied by damage to the hearing organs, and this, in turn, entails violations of the psychosomatic state of the child and complicates the development of the speech apparatus.

Moreover, if the lesion is bilateral, it can lead to disability of a person. Since there are many variants of combined developmental anomalies in a child, it is very often necessary to cure or alleviate the patient's condition with a complex multicomponent treatment that requires certain knowledge from specialists of different profiles.

External signs of pathologies in development hearing aid may appear in a variety of ways. The dimensions of the auricle can vary from excessively enlarged, as in macrotia, to negligible or completely absent, as in microtia or anotia. In the area of the auricle, growths may form, such as ear pendants or ear fistulas. The position of the ear shell may be incorrect. Lop-earedness, an anomaly characterized by a 90-degree angle between the surface of the head and the ear, is also considered a deviation from the norm.

To the most serious pathologies development, entailing complete or partial hearing loss, include: atresia or stenosis of the external auditory opening, developmental disorders auditory ossicles or labyrinth.

Anomaly coding according to the International Classification of Diseases

Pathologies of ear development that are congenital in nature, which lead to a deterioration in auditory function.
Other disorders in the development of the hearing organs of a congenital nature.
The classification distinguishes pathologies in the structure of the inner ear, middle and outer:

Q16.9 - pathologies in the structure of the labyrinth and the lumen of the inner ear;
Q16.3- local violations development of auditory ossicles;
Q16.I - narrowing or atresia of the external auditory canal, fistulas in the parotid region;
Q17.0 - additional auricle;
Q17.5 Protruding ear or protruding ear;
Q17.1 - enlarged ear;
Q17.2 - reduced ear.

Epidemiological factor in pathologies of development of hearing organs

According to the collected statistical data, the frequency of development congenital anomalies hearing organs is 1: 7-15 thousand babies, and in most cases located on the right. In girls, pathologies are observed 2-2.5 times less often than in boys.

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Prerequisites for the occurrence of malformations of the hearing organs

In 85% of cases, malformations of the hearing organs are episodic and do not have clear prerequisites. In other cases, these disorders are associated with hereditary predisposition.

Signs of pathologies of ear development

Inherited pathologies of the development of the hearing organs in most cases provoke such diseases as: Goldenhar syndrome, Konigsmark syndrome, Moebius syndrome, Nager syndrome, Treacher-Collins syndrome.

Konigsmark's syndrome manifests itself in such deviations as a decrease in the size of the ear shell, atresia of the external ear canal, and impaired sound passage into the inner ear. In this case, there is no external ear canal, and the auricle is a cartilaginous roller. At the same time, facial features are symmetrical, and no concomitant malformations are observed.

With Konigsmark's syndrome, it shows almost complete hearing loss - the degree of conductive hearing loss III-IV. This hereditary disease transmitted in an autosomal recessive manner.

Diagnosis of pathologies of the hearing aid

For an accurate diagnosis of ear development pathologies in newborns, it is necessary, first of all, to conduct an examination - most doctors agree on this. For such purposes are used standard methods research: acoustic impedancemetry; setting hearing thresholds by means of fixing short-latency SVPs and UAE.

To test the hearing of patients older than 4 years, tone threshold audiometry is used, and they also check the completeness of the perception of a whisper and a standard colloquial speech. If a child is diagnosed with unilateral, then checking the hearing function in the other ear, all the same, requires reliable confirmation.

With a reduced size of the auricle (microtia), it is usually diagnosed III degree conductive hearing loss, that is, about 60-70 dB. However, it is possible that sensorineural or conductive hearing loss may have indicators both greater and lesser.

Treatment of developmental pathologies of the hearing organs

(obstruction sound waves to the inner ear), developed on both sides, requires the wearing of a special hearing aid with a bone vibrator to ensure the child's ability to speak. If there is an ear canal in the ear, then a conventional hearing aid is sufficient.

The structure of the ear suggests the presence of a mucous membrane, which is located all the way from the nasopharynx through auditory tube to the middle ear and mastoid process. In this regard, sick children, as well as healthy ones, are susceptible to otitis media. In medical practice cases of mastoiditis with microtia and atresia of the external auditory canal have been reported. Such complications are treated only surgically.

- group congenital pathologies, which are characterized by deformation, underdevelopment, or the absence of the entire shell or its parts. Clinically it can be manifested by anotia, microtia, hypoplasia of the middle or upper third cartilage of the external ear, including a folded or fused ear, protruding ears, splitting of the earlobe and specific anomalies: “satire ear”, “macaque ear”, “Wildermuth ear”. Diagnosis is based on history, physical examination, assessment of sound perception, audiometry, impedance or ABR test, computed tomography. Surgical treatment.

Anomalies in the development of the auricle - relatively rare group pathologies. According to statistics, their frequency in various parts planet is in the range from 0.5 to 5.4 per 10,000 newborns. Among Caucasians, the prevalence is 1 in 7,000 to 15,000 infants. In more than 80% of cases, violations are sporadic. In 75-93% of patients, only 1 ear is affected, of which in 2/3 of cases - the right one. Approximately in a third of patients, malformations of the auricle are combined with bone defects. facial skeleton. In boys, such anomalies occur 1.3-2.6 times more often than in girls.

Causes of anomalies in the development of the auricle

Defects of the external ear are the result of violations of intrauterine development of the fetus. hereditary defects are relatively rare and are part of the genetically determined syndromes: Nager, Treacher-Collins, Konigsmark, Goldenhar. A significant part of the anomalies in the formation of the ear shell is due to the influence of teratogenic factors. The disease is provoked:

intrauterine infections. include infectious pathologies from the TORCH group, the pathogens of which are able to penetrate the hematoplacental barrier. This list includes cytomegalovirus, parvovirus, pale treponema, rubella, rubella virus, herpes virus types 1, 2 and 3, toxoplasma.
physical teratogens. Congenital anomalies of the auricle potentiates ionizing radiation during X-ray studies, prolonged exposure to high temperatures(hyperthermia). Less often in role etiological factor speaks radiation therapy at cancer, radioactive iodine.
Bad habits of the mother. Relatively often, a violation of the intrauterine development of a child provokes chronic alcohol intoxication, narcotic substances use of cigarettes and other tobacco products. Among the drugs, the most significant role playing cocaine.
Medications. A side effect of some groups pharmacological preparations is a violation of embryogenesis. These medications include tetracycline antibiotics, antihypertensives, iodine and lithium-based drugs, anticoagulants, and hormonal agents.
Mother's illnesses. Anomalies in the formation of the auricle may be due to metabolic disorders and glandular function. internal secretion mother during pregnancy. The list includes the following pathologies: decompensated diabetes mellitus, phenylketonuria, lesions thyroid gland hormone-producing tumors.

Pathogenesis

The basis of the formation of anomalies of the ear shell is a violation of the normal embryonic development mesenchymal tissue located around the ectodermal pocket - I and II gill arch. IN normal conditions precursor tissues of the external ear are formed by the end of the 7th week of intrauterine development. At 28 obstetric week appearance the outer ear corresponds to that of a newborn child. The influence of teratogenic factors during this time interval is the cause of congenital defects in the cartilage of the auricle. The earlier it was rendered negative impact the more severe its consequences. Later damage does not affect embryogenesis auditory system. Exposure to teratogens for up to 6 weeks is accompanied by severe malformations or complete absence of the shell and outer part of the ear canal.

Classification

IN clinical practice apply classifications based on clinical, morphological changes auricle and adjacent structures. The main goals of dividing the pathology into groups are to simplify the assessment of the patient's functional capabilities, the choice of treatment tactics, and the decision on the need and expediency of hearing aids. The classification of R. Tanzer is widely used, which includes 5 degrees of severity of anomalies of the auricle:

I - anotia. It is a total absence of tissues of the shell of the outer ear. As a rule, it is accompanied by atresia of the auditory canal.
II - microtia or complete hypoplasia. The auricle is present, but severely underdeveloped, deformed, or lacking certain parts. There are 2 main options:

Option A - Combination of microtia with complete atresia of the external ear canal.
Option B - microtia, in which the ear canal is preserved.

III - hypoplasia of the middle third of the auricle. It is characterized by underdevelopment of the anatomical structures located in the middle part of the cartilage of the ear.
IV - underdevelopment of the upper part of the auricle. Morphologically it is represented by three subtypes:

Subtype A - folded ear. There is an inflection of the curl forward and downward.
Subtype B - ingrown ear. It is manifested by the fusion of the upper part of the back surface of the shell with the scalp.
Subtype C - total hypoplasia of the upper third of the shell. The upper sections of the curl, the upper leg of the antihelix, the triangular and navicular fossae are completely absent.

V - protruding ears. Option congenital deformity, in which there is a passion for the angle of attachment of the auricle to the bones of the cerebral part of the skull.

The classification does not include local defects in certain parts of the shell - the curl and the earlobe. These include Darwin's tubercle, satyr's ear, bifurcation or enlargement of the earlobe. It also does not include disproportionate ear enlargement due to cartilage tissue- macrotia. The absence of these variants in the classification is due to the low prevalence of these defects compared to the above anomalies.

Symptoms of anomalies in the development of the auricle

Pathological changes can be detected already at the time of the birth of the child in the delivery room. Depending on the clinical form symptoms have characteristic differences. Anotia is manifested by agenesis of the shell and opening of the ear canal - in their place is a shapeless cartilaginous tubercle. This form is often combined with bone malformations. facial skull, more often - mandible. In microtia, the shell is represented by a vertical ridge displaced forward and upward, at the lower end of which there is a lobe. In various subtypes, the ear canal may be preserved or closed.

Hypoplasia of the middle of the auricle is accompanied by defects or underdevelopment of the helical stalk, tragus, lower antihelix peduncle, cup. Anomalies in the development of the upper third are characterized by the "bending" of the upper edge of the cartilage outward, its fusion with the tissues of the parietal region located behind. less often top part sinks are completely missing. The auditory canal in these forms is usually preserved. With protruding ears, the outer ear is almost completely formed, however, the contours of the shell and antihelix are smoothed, and the angle between the bones of the skull and cartilage is more than 30 degrees, due to which the latter somewhat “bulges” outwards.

Morphological variants of earlobe defects include abnormal enlargement compared to the entire shell, its complete absence. When split, two or more flaps are formed, between which there is a small groove ending at the level of the lower edge of the cartilage. Also, the lobe can grow to those located behind skin. An anomaly in the development of the helix in the form of Darwin's tubercle is clinically manifested by a small formation in the upper corner of the shell. With the "ear of the satyr" there is a sharpening of the upper pole in combination with a smoothing of the curl. With the "ear of the macaque", the outer edge is slightly enlarged, middle part curl is smoothed or completely absent. "Wildermuth's ear" is characterized by a pronounced protrusion of the antihelix above the level of the curl.

Complications

Complications of anomalies in the development of the auricle are associated with untimely correction of deformities of the auditory canal. Existing in such cases, severe conductive hearing loss in childhood leads to deaf-mutism or severe acquired disorders of the articulatory apparatus. Cosmetic defects negatively affect social adaptation child, which in some cases causes depression or other mental disorders. Stenosis of the lumen of the outer ear impairs the excretion of dead epithelial cells and earwax, which creates favorable conditions for life pathogenic microorganisms. As a result, recurrent and chronic otitis externa and otitis media, myringitis, mastoiditis, other bacterial or fungal infections regional structures.

Diagnostics

The diagnosis of any pathology of this group is based on an external examination of the ear area. Regardless of the variant of the anomaly, the child is referred for a consultation with an otolaryngologist to exclude or confirm violations of the sound-conducting or sound-perceiving apparatus. The diagnostic program consists of the following studies:

Assessment of auditory perception. Basic diagnostic method. It is carried out with the help of sounding toys or speech, sharp sounds. During the test, the doctor evaluates the child's reaction to sound stimuli of varying intensity in general and from each ear.
Tonal threshold audiometry. It is indicated for children older than 3-4 years, due to the need to understand the essence of the study. With isolated lesions of the outer ear or their combination with pathologies of the auditory ossicles, the audiogram shows a deterioration in sound conduction while maintaining bone conduction. With concomitant anomalies of the organ of Corti, both parameters decrease.
Acoustic impedance and ABR test. These studies can be done at any age. The purpose of impedancemetry is to study functionality tympanic membrane, auditory ossicles and to identify a malfunction of the sound-perceiving apparatus. In case of insufficient information content of the study, the ABR test is additionally used, the essence of which is to assess the reaction of the CNS structures to a sound stimulus.
CT of the temporal bone. Its use is justified in cases of suspected severe malformations. temporal bone With pathological changes sound-conducting system, cholesteatoma. CT scan performed in three planes. Also, based on the results of this study, the question of the feasibility and scope of the operation is being decided.

Treatment of anomalies in the development of the auricle

The main method of treatment is surgical. Its goal is to eliminate cosmetic imperfections, compensation of conductive hearing loss and prevention of complications. The selection of technique and scope of the operation is based on the nature and severity of the defect, the presence comorbidities. The recommended age for intervention is 5-6 years. By this time, the formation of the auricle is completed, and social integration does not yet play such a role. important role. In pediatric otolaryngology, the following surgical techniques are used:

Otoplasty. Restoration of the natural shape of the auricle is performed in two main ways - using synthetic implants or an autograft taken from the cartilage of the VI, VII or VIII ribs. The Tanzer-Brent operation is underway.
Meatotympanoplasty. The essence of the intervention is the restoration of the patency of the auditory canal and the cosmetic correction of its inlet. The most common technique is according to Lapchenko.
Hearing aid. It is advisable for severe hearing loss, bilateral lesions. Classic prostheses or cochlear implants are used. If it is impossible to compensate for conductive hearing loss with the help of meatotympanoplasty, devices with a bone vibrator are used.

Forecast and prevention

The prognosis for health and the cosmetic result depend on the severity of the defect and the timeliness of the procedure. surgical treatment. In most cases, a satisfactory cosmetic effect partially or completely eliminate conductive hearing loss. Prevention of anomalies in the development of the auricle consists of pregnancy planning, consultation with a geneticist, rational medication, refusal bad habits, prevent exposure to ionizing radiation during pregnancy, timely diagnosis and treatment of diseases from the group of TORCH infections, endocrinopathies.