Developmental anomalies of the inner ear and cochlear implantation. Causes of developmental abnormalities of the auricle. Other diagnostic methods

- group congenital pathologies, which are characterized by deformation, underdevelopment or absence of the entire shell or its parts. Clinically, it can manifest itself as anotia, microtia, hypoplasia of the middle or upper third of the cartilage of the outer ear, including a rolled or fused ear, protruding ears, splitting of the lobe and specific anomalies: “satyr’s ear”, “macaque ear”, “Wildermuth’s ear”. Diagnosis is based on medical history, physical examination, sound perception assessment, audiometry, impedance measurement or ABR test, computed tomography. Treatment is surgical.

General information

Anomalies in the development of the auricle - relatively rare group pathologies. According to statistics, their frequency in different parts of the planet ranges from 0.5 to 5.4 per 10,000 births. Among Caucasians, the prevalence rate 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 ear is affected. In about a third of patients, malformations of the auricle are combined with bone defects of the facial skeleton. In boys, such anomalies occur 1.3-2.6 times more often than in girls.

Causes of abnormal development of the auricle

External ear defects are the result of disorders intrauterine development 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 concha is caused by the influence of teratogenic factors. The disease is provoked by:

• Intrauterine infections. Includes infectious pathologies from the TORCH group, the pathogens of which are able to penetrate the hematoplacental barrier. This list includes cytomegalovirus, parvovirus, treponema pallidum, rubella, rubella virus, types 1, 2 and 3 of herpes virus, toxoplasma.
• Physical teratogens. Congenital anomalies of the auricle are potentiated by ionizing radiation during X-ray studies, long stay at high temperatures (hyperthermia). Less often in the role etiological factor radiation therapy is used for cancer diseases, radioactive iodine.
• Mother's bad habits. Relatively often, disruption of the intrauterine development of a child is provoked by chronic alcohol intoxication, narcotic substances, use of cigarettes and other tobacco products. Among the drugs, the most significant role Cocaine plays.
• Medications. Side effect of some groups pharmacological drugs is a violation of embryogenesis. Such medications include antibiotics from the tetracycline group, antihypertensives, drugs based on iodine and lithium, anticoagulants and hormonal agents.
• Mother's diseases. Anomalies in the formation of the auricle can be caused by metabolic and gland disorders internal secretion mothers during pregnancy. The list includes the following pathologies: decompensated diabetes mellitus, phenylketonuria, lesions thyroid gland, hormone-producing tumors.

Pathogenesis

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

Classification

In clinical practice, classifications are used that are based on clinical and morphological changes in the auricle and adjacent structures. The main goals of dividing pathology into groups are to simplify the assessment of the patient’s functional capabilities, choose treatment tactics, and resolve the issue of the need and advisability of hearing aids. R. Tanzer's classification is widely used, which includes 5 degrees of severity of ear abnormalities:

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

1. Option A - combination of microtia with complete atresia of the external ear canal.
2. 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 ear cartilage.
• IV – underdevelopment of the upper part of the auricle. Morphologically represented by three subtypes:

1. Subtype A – rolled ear. There is an inflection of the curl forward and downward.
2. Subtype B – ingrown ear. It manifests itself by fusion of the upper part of the posterior surface of the shell with the scalp.
3. Subtype C – total hypoplasia of the upper third of the shell. The upper sections of the helix, the upper leg of the antihelix, the triangular and scaphoid fossae are completely absent.

• V – protruding ears. A variant of congenital deformity, in which there is a deviation in the angle of the auricle to the bones of the brain part of the skull.

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

Symptoms of ear abnormalities

Pathological changes can be detected already at the time of birth of the child in birth hall. Depending on the clinical form, symptoms have characteristic differences. Anotia is manifested by agenesis of the concha and the opening of the auditory canal - in their place is a shapeless cartilaginous tubercle. This form is often combined with malformations of the bones of the facial skull, most often - lower jaw. With microtia, the shell is represented by a vertical ridge displaced forward and upward, at the lower end of which there is a lobe. With different subtypes, the ear canal may persist or be closed.

Hypoplasia of the middle of the auricle is accompanied by defects or underdevelopment of the pedicle of the helix, tragus, lower pedicle of the antihelix, and cup. Developmental anomalies of the upper third are characterized by “bending” of the upper edge of the cartilage outward, its fusion with the tissues of the parietal region located behind. Less often top part shells are completely missing. The auditory canal in these forms is usually preserved. With protruding ears, the outer ear is almost completely formed, but the contours of the concha and antihelix are smoothed, and the angle between the bones of the skull and the cartilage is more than 30 degrees, which is why the latter “protrudes” outward somewhat.

Morphological variants of earlobe defects include an abnormal increase in comparison with the entire concha and its complete absence. When bifurcated, 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 abnormal 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 “satyr ear,” a sharpening of the upper pole is observed in combination with a smoothing of the helix. With “macaque ear” the outer edge is slightly enlarged, middle part the curl is smoothed out or completely absent. “Wildermuth’s ear” is characterized by a pronounced protrusion of the antihelix above the level of the helix.

Complications

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

Diagnostics

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

• Assessment of auditory perception. Basic diagnostic method. It is carried out using sounding toys or speech, sharp sounds. During the test, the doctor evaluates the child's reaction to sound stimuli. different intensity in general and from each ear.
• Pure tone threshold audiometry. Indicated for children over 3-4 years of age, due to the need to understand the essence of the study. For isolated lesions of the outer ear or their combination with pathologies 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 measurement and ABR test. These studies can be carried out at any age. The purpose of impedansometry is to study the functionality of the eardrum, auditory ossicles and to identify dysfunction of the sound-receiving apparatus. If the information content of the study is insufficient, the ABR test is additionally used, the essence of which is to assess the reaction of the central nervous system structures to a sound stimulus.
• CT scan of the temporal bone. Its use is justified in cases of suspected severe malformations of the temporal bone with pathological changes in the sound-conducting system, cholesteatoma. Computed tomography is performed in three planes. Also, based on the results of this study, the question of the feasibility and scope of the operation is decided.

Treatment of developmental anomalies of the auricle

The main method of treatment is surgery. Its goals are to eliminate cosmetic defects, compensate for conductive hearing loss and prevent complications. The selection of the technique and scope of the operation is based on the nature and severity of the defect, the presence accompanying pathologies. 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 an important role. The following are used in pediatric otolaryngology: surgical techniques:

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

Prognosis and prevention

Health prognosis and cosmetic result depend on the severity of the defect and the timeliness of the surgical treatment. In most cases, it is possible to achieve a satisfactory cosmetic effect and 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 use of medications, giving up bad habits, preventing exposure to ionizing radiation during pregnancy, timely diagnosis and treatment of diseases from the group of TORCH infections, endocrinopathies.

V.E. Kuzovkov, Yu.K. Yanov, S.V. Levin
St. Petersburg Research Institute of Ear, Throat, Nose and Speech

Cochlear implantation (CI) is currently generally recognized in world practice and the most promising direction for the rehabilitation of persons suffering from high-grade sensorineural hearing loss and deafness, with their subsequent integration into the hearing environment. In modern literature, the issues of classification of anomalies of the development of the inner ear are widely covered, including in relation to CI, and surgical techniques for performing CI for this pathology are described. The world experience of CI in persons with developmental anomalies of the inner ear spans more than 10 years. At the same time, there are no works on this topic in the domestic literature.
At the St. Petersburg Research Institute of Ear, Throat, Nose and Speech, for the first time in Russia, CI began to be performed on people with developmental anomalies of the inner ear. Three years of experience in such operations, the presence of successful results of such interventions, as well as an insufficient amount of literature on this issue, served as the reason for carrying out this work.

Classification of developmental anomalies of the inner ear. Current state of the issue.

With the advent of the late 80's - early 90's. high-resolution computed tomography (CT) and magnetic resonance imaging (MRI), these techniques have become widely used for diagnosing hereditary hearing loss and deafness, especially when determining indications for CI. With the help of these progressive and highly accurate techniques, new anomalies were identified that did not fit into the existing classifications of F. Siebenmann and K. Terrahe. As a result, R.K. Jackler were offered new classification, expanded and modified by N. Marangos and L. Sennaroglu. However, it should be noted that MRI in particular currently reveals such fine detail that the malformations detected can be difficult to classify.
In his classification of developmental anomalies of the inner ear, based on conventional radiography and early CT data, R.K. Jackler took into account the separate development of the vestibular semicircular and vestibular cochlear parts of a single system. The author suggested that various types of anomalies appear as a result of a delay or disruption of development at a certain stage of the latter. Thus, the types of malformations detected are correlated with the time of disruption. Later, the author recommended classifying combined anomalies as category A, and suggested a connection between such anomalies and the presence of an expanded aqueduct in the vestibule (Table 1).

Table 1
Classification of developmental anomalies of the inner ear according to R.K.Jackler

The N. marangos classification includes incomplete or aberrant development of the labyrinth (Table 2, item 5).

table 2
Classification of developmental anomalies of the inner ear according to N. Marangos

L. Sennaroglu differentiates 5 main groups (Table 3): anomalies of the development of the cochlea, vestibule, semicircular canals, internal auditory canal and aqueduct of the vestibule or cochlea.

Table 3

Main groups and configurations of cochleovestibular anomalies according to L. Sennaroglu

Cochlear malformations (Table 4) were divided by the author into six categories based on the degree of severity, depending on the time of disruption of the normal course of embryonic development. This classification of cochlear malformations includes an incomplete separation of types I and II.

Table 4
Classification of cochlear anomalies according to the time of disruption of intrauterine development according to L. Sennaroglu

Taking into account the small number of patients operated on, one case of successful CI for an inner ear anomaly is presented below.

Case from practice.

In March 2007, the parents of patient K., born in 2005, came to the St. Petersburg Research Institute of ENT with complaints about the child’s lack of reaction to sounds and lack of speech. During the examination, a diagnosis was made: Chronic bilateral sensorineural hearing loss of IV degree, congenital etiology. Secondary receptive disorder expressive speech. Consequences of intrauterine pregnancy cytomegalovirus infection, intrauterine lesions of the central nervous system. Residual organic damage to the central nervous system. Left-sided spastic upper monoparesis. Aplasia of the first finger of the left hand. Hip dysplasia. Spasmodic torticollis. Pelvic dystopia of the hypoplastic right kidney. Delayed psychomotor development.

By conclusion child psychologist – cognitive abilities the child is within the age norm, intelligence is preserved.

The child received binaural hearing aids with heavy-duty hearing aids, without effect. According to the audiological examination, short-latency auditory evoked potentials were not recorded at a maximum signal level of 103 dB, and otoacoustic emissions were not recorded on both sides.

When conducting game audiometry in hearing aids, reactions to sounds with an intensity of 80-95 dB in the frequency range from 250 to 1000 Hz were revealed.
CT scan of the temporal bones revealed the presence of a bilateral anomaly of the cochlea in the form of incomplete division of type I (Table 4). Moreover, this statement is true for both the left and right ears, despite the seemingly different picture (Fig. 1).

Rice. 1. CT data of patient K.

Note: So, on the right (1) the cochlea is represented by a small cavity, the maximum size of which is no more than 5 mm, the vestibule and semicircular canals are absent. On the left (2) the cochlea is represented by a single cavity without internal architecture, the presence of an expanded vestibule is noted (Fig. 2, arrow); expanded system of semicircular canals (Fig. 3, arrow).

Fig.2. Extended vestibule on the left

Rice. 3. Enlarged and deformed system of semicircular canals on the left

After the examination, the patient underwent CI on the left ear using the classical approach through anthromastoidotomy and posterior tympanotomy, with the introduction of an electrode through a cochleostomy. For the operation, a special shortened electrode (Med-El, Austria) was used, having a working length of the active electrode of about 12 mm, specially designed for use in cases of anomaly or ossification of the cochlea.

Despite the intact auditory ossicles and stapedius muscle tendon, acoustic reflexes from the stapedius muscle were not recorded during the operation. However, when performing neural response telemetry, clear responses were obtained when 7 out of 12 electrodes were stimulated.

Postoperative transorbital radiography of the cochlea revealed that active electrode the implant is in common cavity(Fig. 4, arrow), taking the shape of an ideal circle.

Rice. 4. Transorbital radiography. Shortened electrode in a common cavity.

During a control audiological examination one year after surgery, the patient was found to have reactions in the free sound field to sounds with an intensity of 15-20 dB in the frequency range from 250 to 4000 Hz. The patient’s speech is represented by one- and two-syllable words (“mother”, “give”, “drink”, “kitty”, etc.), a simple phrase of no more than two one- or two-syllable words. Considering that the patient’s age at the time of the re-examination was less than 3 years, the results of auditory-speech rehabilitation in this case should be considered excellent.

Conclusion

The modern classification of developmental anomalies of the inner ear not only gives an idea of ​​the diversity of such pathology and the time of occurrence of the defect during intrauterine development, but is also useful in determining the indications for cochlear implantation and in the process of choosing tactics for intervention. The observation presented in the work allows us to evaluate the possibilities of cochlear implantation as a means of rehabilitation in difficult cases, expands the understanding of indications for implantation.

Literature

• Jackler R.K. Congenital malformations of the inner ear: a classification based on embryogenesis//R.K. Jackler, W. M. Luxford, W.F. House/ Laryngoscope. – 1987. – Vol. 97, no. 1. – P. 1 – 14.
• Jackler R.K. The large vestibular aqueduct syndrome//R.K. Jackler, A. De La Cruz/ Laryngoscope. – 1989. – Vol. 99, No. 10. – P. 1238 – 1243.
• Marangos N. Dysplasien des Innenohres und inneren Gehörganges//N. Marangos/HNO. – 2002. – Vol. 50, no. 9. - P. 866 – 881.
• Sennaroglu L. A new classification for cochleovestibular malformations//L. Sennaroglu, I. Saatci/Laryngoscope. – 2002. – Vol. 112, No. 12. – P. 2230 – 2241.
• Siebenmann F. Grundzüge der Anatomie und Pathogenese der Taubstummheit// F. Siebenmann/Wiesbaden: J. F. Bergmann; 1904. – 76s.
• Stellenwert der MRT bei Verdacht auf Innenohrmissbildung//S. Kösling, S. Jüttemann, B. Amaya et al. / Fortschr Röntgenstr. – 2003. – Vol. 175, No. 11. – S. 1639 – 1646.
• Terrahe K. Missbildungen des Innen- und Mittelohres als Folge der halidomidembryopathie: Ergebnisse von Röntgenschichtuntersuchungen//K. Terrahe/Fortschr Röntgenstr. – 1965. – Vol. 102, No. 1. – P. 14.

Missing earlobe or entire ear may be a congenital or acquired anomaly in the development of the auricle during life. Congenital aplasia of the auricle is called anotia and occurs in 1 out of 18 thousand newborns. Congenital rudimentary, underdeveloped earlobes are often combined with deformation of the entire auricle and are the result of a violation of the processes of embryogenesis. Loss of the lobe or pinna as a result of injuries (mechanical, thermal, chemical) is an acquired defect of the outer ear.

The auricle (auricula) consists of an elastic C-shaped cartilage covered with skin and a lobe. The degree of development of the cartilage determines the shape of the ear and its protrusions: the free curved edge - the helix (helix) and the antihelix located parallel to it (anthelix); the anterior projection - the tragus (tragus) and the antitragus lying behind it (antitragus). The lower part of the auricle is called the lobula or lobula and is a progressive, characteristic feature of a person. The earlobe is devoid of cartilage tissue and consists of skin and fatty tissue. Normally, the C-shaped cartilage is slightly more than 2/3, and Bottom part– lobe – slightly less than 1/3 of the total height of the auricle.

Underdevelopment or complete absence of the auricle is one of the most severe ear developmental anomalies. The absence of a lobe, part or whole ear can be unilateral or bilateral and is often associated with other congenital anomalies of the face: underdevelopment of the lower jaw, soft tissues of the cheek and zygomatic bones, transverse cleft of the mouth - macrostomy, syndrome of the 1st-2nd branchial arches. Complete aplasia of the auricle, characterized by the presence of only the earlobe or a small skin-cartilaginous ridge. In this case, there may be a narrowing or fusion of the ear canal, the presence of parotid cutaneous-cartilaginous appendages, parotid fistulas, etc. The absence of the external ear can be an independent defect not associated with other organs, or occur simultaneously with independent malformations of the kidneys, heart, limbs, etc. d.

Congenital absence of the external ear is usually associated with underdevelopment of the cartilaginous frame of the auricle and, to one degree or another, is accompanied by developmental disorders internal cavities ear, providing the function of sound conduction. However, the absence of an external ear does not in any way affect the intellectual and physical abilities of children.

Classification of developmental anomalies of the external ear

Existing classification options congenital anomalies development of the auricles is built taking into account the degree of underdevelopment of the outer ear.

The Tanzer grading system for underdevelopment of the auricles proposes to classify variants of congenital defects from stage I (complete anotia) to stage IV (protruding ear).

The classification according to the Aguilar system considers the following options for the development of the auricles: Stage I - normal development of the auricles; Stage II – deformation of the ears; Stage III – microtia or anotia.

Weyerd's three-stage classification is the most complete and distinguishes the stages of auricular defect depending on the degree of need for their plastic reconstruction.

Stages of underdevelopment (dysplasia) of the ears according to Weyerd:

• Dysplasia I degree– most of the anatomical structures of the auricle can be recognized. When performing reconstructive operations, there is no need for additional cartilage tissue and skin. Grade I dysplasia includes macrotia, protruding ears, and mild and moderate deformities of the ear cup.
• Dysplasia II degree– only certain parts of the auricle are recognizable. To carry out partial reconstruction using plastic surgery additional skin and cartilage implants are required. Grade II dysplasia includes severe ear deformities and microtia (small ear sizes).
• Dysplasia III degree – it is impossible to recognize the structures that make up the normal auricle; an undeveloped ear resembles a wrinkled lump. At this grade, a complete reconstruction using significant skin and cartilage implants is necessary. Variants of grade III dysplasia are microtia and anotia.

Reconstructive otoplasty in the absence of a lobe or ear

Defects in the development of the auricles, such as the absence of a lobe or outer ear, require complex reconstructive plastic surgery. This is the most time-consuming and labor-intensive option of otoplasty, which places high demands on qualifications. plastic surgeon and carried out in several stages.

Particularly difficult is the complete reconstruction of the outer ear in the case of its congenital absence (anotia) or loss due to injury. The process of recreating the missing ear is carried out in 3-4 stages and takes about a year.

The first stage includes the formation of the cartilaginous frame of the future ear from the patient’s costal cartilages. At the second stage, the automaterial (cartilaginous base) is placed in a specially formed subcutaneous pocket in place of the missing ear. The implant should take root in its new location within 2-6 months. During the third stage, the cartilaginous base of the future ear is disconnected from the adjacent tissues of the head, moved to the required position and fixed in the correct position. The wound in the area behind the ear is covered with a skin graft taken from the patient himself (from the arm, leg or abdomen). At the last stage, the natural recesses of the auricle and tragus are formed. Thus, in the newly recreated ear all the anatomical elements inherent in the normal auricle are present.

And although reconstructive otoplasty cannot restore hearing, the new ear created by surgeons allows patients to experience themselves and the world around them in a new way. The shape of the ear created in the process of reconstructive otoplasty is practically no different from the natural one.

Carrying out reconstructive otoplasty in children who have no external ear is possible no earlier than 6-7 years of age. In case of bilateral hearing loss, early hearing aid is indicated (wearing hearing aid) so that there is no delay in mental and speech development. In some cases, with bilateral hearing defects, surgical intervention is performed on the inner ear. Alternative way solutions cosmetic problem A solution to the absence of an external ear, which is widespread abroad, is to wear a specially made removable prosthesis of the auricle.

In the absence of an earlobe, operations to restore it are also performed. For this purpose, skin grafts taken from the behind-the-ear space or neck area are used. With competent and technical correct execution similar operation postoperative scars practically invisible.

Despite the successes achieved by reconstructive plastic surgery in solving the problem of the absence of the lobe and outer ear, the search for new materials and methods of otoplasty continues at present for the most natural reconstruction of such an organ as complex in shape and function as Auricle.

For atresia of the external auditory canal the tympanic bone (os tympanicum) may be absent. Middle ear deformities are associated with this deficiency. varying degrees. In mild deformities, the eardrum is preserved, but, as a rule, it is always incorrectly formed. In other, more severe cases, there is only a bone plate in place of the eardrum.

Wherein tympanic cavity can be reduced by thickening the walls, especially due to lower section. Sometimes the cavity is so narrowed that it takes on a slit-like shape, and with high degrees of deformity it may even be completely absent, in its place there is only spongy bone.

Auditory ossicles, especially the malleus and incus, in most cases are formed incorrectly. The hammer handle is especially deformed; Sometimes there is no connection between the malleus and the eardrum.

For severe degrees deformities bones may be completely absent, and muscles tympanic cavity exist and are even well developed. However, in the absence of a hammer, the tensing eardrum the muscle is attached to the lateral wall. The facial nerve always exists, but the course can be changed. The Eustachian tube almost always exists, but occasionally partial or complete atresia occurs.

Haruzek(Charousek, 1923) observed isolated underdevelopment of the lateral wall of the attic and the shrapnel membrane, the auditory ossicles were deformed. At the same time, the same patient had microtia with atresia of the external auditory canal and deafness with normal excitability of the vestibular apparatus in the other ear. This rare case underdevelopment of all three departments.
Microsurgical operations for anomalies of the middle ear, removal of abnormally formed auditory ossicles, especially the malleus, can lead to improved hearing.

Developmental abnormalities of the inner ear

(labyrinth) are very rarely expressed in the form of organ aplasia; the latter leads to deafness in that ear.
Usually anomalies the development of the labyrinth is limited (partial) in nature and concerns only the organ of hearing, auditory nerve or the brain part of the latter, but there are also more different changes that involve the entire pyramid of the temporal bone, the middle and outer ear, facial nerve. According to Siebenmann, with atresia of the external auditory canal, only one third of cases are found pathological changes in the inner ear.

The bulk deaf and dumb, having more or less gross changes on the part of the inner ear or auditory nerve, usually does not have those on the part of the outer and middle ear (B. S. Preobrazhensky), which is due to the peculiarities of the embryonic development of the auditory organ.

Developmental abnormalities of the inner ear, which are limited in nature without the presence of simultaneous malformations of the surrounding areas, can occur in the form of: 1) complete absence of the inner ear; 2) diffuse developmental anomaly of the membranous labyrinth; 3) limited anomaly of development of the membranous labyrinth (organ of Corti and auditory cells). The most striking example of the complete absence of the labyrinth and auditory nerve is the only case described by Michel (1863).

Diffuse anomalies Membranous labyrinths are found more often among the deaf and mute and can arise as independent developmental anomalies due to intrauterine infection. They can be expressed in the form of underdevelopment of the partitions between the volutes and scalae, the absence of Reisner's membrane, expansion of the endolymphatic canal with an increase in fluid, or, conversely, expansion of the perilymphatic space with an increase in perilymphatic fluid due to the collapse of Reisner's membrane, which leads to a narrowing of the endolymphatic canal; the organ of Corti may be in some places rudimentary, and in others completely absent; there are no cells of the spiral ganglion or they are underdeveloped. Often there may be no fibers of the auditory nerve trunk or their atrophy.

Stria vascularis may be completely absent, sometimes only in places, but it also happens the other way around: a strong increase in it up to half the lumen of the canal. The vestibular part usually remains normal in case of anomalies of the cochlear apparatus, but occasionally there is absence or underdevelopment of one or another part of it (otolithic membrane, maculae, cupulae, etc.).
Developmental anomalies, associated with intrauterine infection, arise either due to fetal meningitis or placental infection with syphilis.

Finally, there are anomalies development, relating only to the organ of Corti, its epithelium on the basilar plate and partly the peripheral nerve endings. All changes in the organ of Corti can be expressed differently: in some places they are completely absent, in others they can be underdeveloped or metaplastic.

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Deformation of the auricle is a violation of the shape or integrity of the outer part of the ear, which occurred as a result of a congenital disorder of the development of the organ or injury.

Considering the degree and complexity of the deformation of the auricle, the existing defect may be purely cosmetic or may cause impaired hearing function, up to its complete loss. In medicine, both unilateral and bilateral deformities are diagnosed. An existing defect of a congenital or acquired type can be eliminated with the help of plastic surgery, practicing otoplasty. In order to determine the degree of hearing impairment, immediately before surgery, the patient must undergo an examination by an otolaryngologist.

Deformation of the auricle is diagnosed immediately after the birth of the child; for this, a simple examination of the newborn by a doctor is sufficient. The main cause of deformation congenital type is a violation of fetal development during uterine development, when the outer rim of the auricle and the underlying cartilage are not formed correctly. If the structure of these areas is incorrect, the location of the auricle occurs at a large angle relative to the skull. When such cosmetic defect, like protruding ears, the symmetry of the ears is disturbed, and they can be quite large in size. Although deformation of the auricle of this type does not affect negative influence on hearing function, it is the cause of mental and emotional state child, which is due to constant ridicule from peers.

An important point in eliminating existing defects in childhood is the child’s readiness for surgical intervention. The best period preschool age is considered for surgery, but if the deformity does not affect mental condition, then intervention can be postponed until adolescence when the child himself understands the importance and necessity of the operation.

Plastic surgery in the process of intervention solves the main problems: correcting the shape and creating symmetry between the ears and relative to other parts of the face.

Classification of ear deformities

The basic principle of classifying ear deformities lies in the nature of their origin, hence it is customary to divide defects into the following types:

• congenital defects;
• acquired defects.

If a person is diagnosed with a deformity of the auricle, the causes of which lie in developmental disorders during the first three months of bearing a child, this is a congenital defect. During the formation of the outer parts of the ear, deformations may have the following characteristics:

• excessive development of cartilage tissue, which disrupts the proportionality of the auricle and makes it big size(macrotia);
• the position of the ears relative to the temporal bone is not parallel, but at an angle from 31 to 90 degrees (protruding ears);
• the upper part of the cartilage of the auricle is bent inward and directed towards the bottom;
• violation anatomical shape earlobes (double lobes, overdeveloped, fused), as well as complete absence of earlobes;
• change in the shape of the ear helix (rudimentary formations on the helix, absence of the helix and tubercle of the auricle, expanded ear helix with the upper part of the cartilage positioned outward);
• partial development of the ears (small ear sizes, flat or ingrown ears) - microtia.

If almost each of the defects - for the most part - relates to cosmetic ones, then microtia of the auricles can be associated with occlusion of the auditory canal, which leads to impaired hearing function or to its complete absence. In some clinical cases, microtia is a consequence of underdevelopment of the facial bones on the affected side. Bilateral microtia is a serious birth defect, which affects hearing function, facial symmetry, development of the lower jaw bones, speech is also impaired, and the patient receives a disability group.

Another important criterion for classifying ear deformities is the degree of complexity; in medicine there are three of them:

1. the auricle is underdeveloped, but there are areas of the organ that are developed at a sufficient level;
2. the auricle has a roll-shaped shape;
3. complete absence of the auricle.

In addition to congenital defects, acquired deformities of the ears occur. The main reason for their formation is:

• injuries to the ear area (for example, injury, violation of equipment and safety rules at the enterprise);
• diseases characterized by inflammation processes occurring inside the ear;
• surgical interventions performed poorly;
• burns;
• exposure to chemicals.

Most often, experts diagnose an acquired ear deformity called a keloid scar. The nature of its appearance is varied. The defect may be the cause of transferred inflammatory diseases or injuries received, manifests itself in the form of skin growths in the area of ​​the lobe or the entire ear. The formation of a keloid scar can occur even after a regular earlobe piercing.

Also, ear deformations are diagnosed according to the following types:

• partial - defect of the lower, upper or middle part of the auricle;
• subtotal - the cartilage of the ear fossa is present or completely absent;
• full - absence of an ear.

Features of surgical correction of ear deformities

In medicine, plastic surgery deals with the elimination of congenital and acquired defects of the outer ear, using the otoplasty technique. This method of surgical treatment is quite complex operation, which is directly related to the structure of the outer ear.

Most often, doctors diagnose a congenital defect such as protruding ears, while the angle of the auricle relative to the bones of the skull may be insignificant, or it may be clearly noticeable. Sometimes protruding ears can only be on one side; along with this defect, doctors often diagnose a lack of symmetry between the ears on both sides.

It is best to plan otoplasty in preschool age, when the child is under 7 years old. This period is due to the fact that the child’s psychological state does not suffer, he is not an object of ridicule from peers, but further development auricles will continue anatomically correct position. A person’s auricle grows until the age of nine, but if the child does not understand the need to perform surgical intervention, then otoplasty can be postponed until adolescence. The opportunity to undergo plastic surgery on the ears remains at any age.

Patients in more mature years feel the need for otoplasty when they want to rejuvenate their appearance and make their facial proportions aesthetically attractive.

Given the complexity of surgical intervention, the patient should be aware of a number of contraindications, in the presence of which the surgeon will not be able to correct the defect.

Contraindications include:

• pathological incoagulability of blood or disruption of this function caused by taking medications;
• pathologies of internal organs;
• the presence of infectious processes;
• tumors of a malignant or benign nature;
• ascending inflammatory processes ear;
• high blood pressure;
• diabetes;
• high probability of keloid scar formation.

Depending on the degree of deformation of the auricle, otoplasty surgery can last from half an hour to two hours.

The main characteristic of otoplasty is that it is performed in several stages; most often, two operations are sufficient, but sometimes more may be required. The interval between interventions ranges from two to four months, which is due to the complex structure of the outer ear and the small amount of soft tissue present in the area behind the ear. To create any part of the outer ear, doctors use a graft from the patient's own tissue. The most suitable is the cartilage of the rib area.

Immediately before the operation, the surgeon is obliged to inform the patient about all manipulations, as well as about the final result that will be achieved at the end of each stage of the operation. An important point is to explain how the rehabilitation period will proceed and how long it will last.

There are two otoplasty techniques practiced in plastic surgery:

1. aesthetic surgery - performed to change the size and shape of the ears, solves the problems of protruding ears and broken symmetry between the ears, helps correct most cosmetic congenital and acquired defects of the ears;
2. reconstructive surgery - helps to get rid of severe deformations of the auricles, solves the problem of missing sections of the ear by recreating them from a graft.

Regardless of what type of operation is needed in a particular clinical case, during otoplasty, surgeons work with both soft tissues auricle, and with areas of cartilage. Before the operation, the doctor must assess the condition of the soft tissues of the auricle, determine the complexity and type of deformation, and based on these indicators determine the type of otoplasty to be performed.

In the case where the patient is diagnosed with severe deformation and absolute restoration of the ear is necessary, the operation involves the introduction general anesthesia. Simple deformities can be corrected under local anesthesia.

Complications of plastic correction of deformed ears

The main problems that a patient faces after otoplasty are the accumulation of blood clots in the area where plastic surgery was performed. In the absence of proper antiseptic care, the patient may become infected with a postoperative wound. At individual characteristics a rough keloid scar may form on the skin.