Causes and problems of hearing diseases. Risk of development and provoking factors. Complications of plastic correction of deformed ears

Malformations of the external ear canal(atresia or stenosis of the external auditory canal), auditory ossicles, labyrinth - more severe congenital pathology; accompanied by hearing loss.

Bilateral defects are the cause of the patient's disability.

Etiology. Congenital malformations of the hearing organ occur with a frequency of approximately 1:700-1:10,000-15,000 newborns, most often on the right side; in boys, on average, 2-2.5 times more often than in girls. In 15% of cases, the hereditary nature of the defects is noted, 85% are sporadic episodes.

Classification. Existing classifications of congenital malformations of the hearing organ are numerous and are based on clinical, etiological and pathogenetic characteristics. Below are the most common ones. There are four degrees of deformation of the outer and middle ear. Defects of the first degree include changes in the size of the auricle (elements of the auricle are recognizable). Defects of the II degree - deformations of the auricle varying degrees, in which part of the auricle is not differentiated. Defects of the third degree are considered to be the ears in the form of a small rudiment, displaced anteriorly and downward; IV degree defects include the absence of the auricle. With II degree defects, as a rule, microtia is accompanied by an anomaly in the development of the external auditory canal.

The following forms of defects are distinguished.
Local defects.

Hypogenesis of the hearing organ:
❖ mild;
❖ moderate;
❖ severe.

Dysgenesis of the hearing organ: mild;
❖ moderate; about a severe degree.

Mixed forms.

The R. Tanzer classification includes 5 degrees:
I - anotia;
II - complete hypoplasia (microtia):
❖ A - with atresia of the external auditory canal,
❖ B - without atresia of the external auditory canal;
III - hypoplasia of the middle part of the auricle;
IV - hypoplasia of the upper part of the auricle:
❖ A - folded ear,
❖ B - ingrown ear,
❖ C - complete hypoplasia of the upper third of the auricle;
V - protruding ears.

Classification by G.L. Balyasinskaya:
Type A - change in the shape, size and position of the auricle without disruption of auditory function:
❖ A 1 - congenital changes in the elements of the middle ear without significant defects in the outer ear.

Type B - combined changes in the auricle, external auditory canal without disruption of the structures of the middle ear:
❖ B 1 - combined change in the auricle, atresia of the external auditory canal, underdevelopment of the chain of auditory ossicles;
❖ B II - combined underdevelopment of the auricle, external auditory canal, tympanic cavity in the presence of an antrum.
Type B - absence of elements of the outer and middle ear:
❖ B 1 - absence of elements of the outer and middle ear, changes in the inner ear. Accordingly, each type in the classification is given recommendations on methods of surgical treatment.

IN plastic surgery Recently, the classification of N. Weerda and R. Siegert has been used and cited in literary sources.
I degree of dysplasia - all elements of the auricle are recognizable; surgical tactics: skin or cartilage do not require additional reconstruction.
❖ Macrotia.
❖ Prominent ears.
❖ Collapsed ear.
❖ Underdevelopment of part of the curl.
❖ Minor deformations: unrolled curl, flat cup (scapha), “satyr’s ear”, tragus deformation, additional fold (“Stahl’s ear”).
❖ Coloboma of the auricle.
❖ Deformations of the lobe (large and small lobes, coloboma, absence of the lobe).
❖ Deformation of the ear cup

II degree of dysplasia - only some elements of the auricle are recognizable; surgical tactics: partial reconstruction with additional use of skin and cartilage.
❖ Severe developmental deformities of the upper part of the auricle (collapsed ear) with tissue deficiency.
❖ Hypoplasia of the auricle with underdevelopment of the upper, middle or lower parts.

III degree - profound underdevelopment of the auricle, represented only by the lobe, or the complete absence of the outer ear, usually accompanied by atresia of the external auditory canal; surgical tactics: total reconstruction using large cartilage and skin flaps.

Classification of atresia of the auditory canal HLF. Schuknecht.
Type A - atresia in the cartilaginous part of the auditory canal; 1st degree hearing loss.
Type B - atresia in both the cartilaginous and bony parts of the auditory canal; hearing loss II-III degree.
Type C - all cases of complete atresia and hypoplasia of the tympanic cavity.
Type D - complete atresia of the auditory canal with weak pneumatization of the temporal bone, accompanied by abnormal location channel facial nerve and capsules of the labyrinth (the identified changes are contraindications for hearing-improving surgery).

Diagnostics. Diagnostics includes examination, testing of auditory function, medical genetic research and consultation maxillofacial surgeon.

According to most authors, the first thing that an otolaryngologist should evaluate when a child is born with an ear anomaly is hearing function. In children early age They use objective hearing research methods: determining thresholds for recording short-latency auditory evoked potentials, recording otoacoustic evoked emissions, and conducting acoustic impedance measurements. In patients over 4 years of age, hearing acuity is determined by the intelligibility of perception of spoken and whispered speech, as well as by pure-tone threshold audiometry. Even with a unilateral anomaly and an apparently healthy second ear, the absence of a hearing impairment must be proven. Microtia is usually accompanied by conductive hearing loss of grade III (60-70 dB). However, there may be lesser or greater degrees of conductive and sensorineural hearing loss.

For diagnosed bilateral conductive hearing loss, wearing hearing aid with bone vibrator promotes normal speech development. Where there is an external auditory canal, a standard hearing aid can be used. A child with microtia is as likely to develop otitis media as a child with healthy child, since the mucous membrane continues from the nasopharynx into auditory tube, middle ear and mastoid process. There are known cases of mastoiditis in children with microtia and atresia. Moreover, antibiotics are prescribed in all cases of acute otitis media, despite the lack of otoscopic findings.

Children with rudimentary ear canal should be evaluated for cholesteatoma. Although visualization is difficult, otorrhea, polyp, or pain may be the first signs of cholesteatoma of the external auditory canal. In all cases of detection of cholesteatoma of the external auditory canal, surgical treatment is indicated for the patient. Currently, in ordinary cases, to decide on the issue of surgical reconstruction of the external auditory canal and ossiculoplasty, we recommend focusing on the data of a hearing study and computed tomography of the temporal bone.

Detailed data from computed tomography of the temporal bone in assessing the structures of the outer, middle and inner ear in children with congenital atresias external auditory canal are necessary in order to determine the technical feasibility of forming the external auditory canal, the prospects for improving hearing, and the degree of risk upcoming operation. Below are some common anomalies. Congenital anomalies of the inner ear can only be confirmed by computed tomography of the temporal bones. The most famous of them are Mondini's anomaly, stenosis of the labyrinthine windows, stenosis of the internal auditory canal, anomaly of the semicircular canals, even their absence.

The main task of medical genetic counseling for any hereditary diseases- diagnosis of syndromes and establishment of empirical risk. A genetic consultant collects a family history, compiles a medical pedigree of the family of those consulting, and conducts an examination of the proband, siblings, parents and other relatives. Specific genetic studies should include dermatoglyphics, karyotyping, and determination of sex chromatin. The most common congenital malformations of the hearing organ occur in Königsmarck, Goldenhar, Treacher-Collins, Möbius, and Nager syndromes.

Treatment. Treatment of patients with congenital malformations of the outer and middle ear is usually surgical; in severe cases of hearing loss, hearing replacement is performed. For congenital defects of the inner ear, hearing aids are performed. Below are treatments for the most common abnormalities of the outer and middle ear.

Macrotia - anomalies in the development of the auricle, resulting from its excessive growth, are manifested by an increase in the entire auricle or its parts. Macrotia usually does not entail functional disorders, the method of treatment is surgical. Below are diagrams of the most commonly used methods for correcting macrotia. The peculiarity of an ingrown ear is its location under the skin of the temporal region. During the operation you should release top part auricle from under the skin and close the skin defect.

Kruchinsky-Gruzdeva method. A V-shaped incision is made on the posterior surface of the preserved part of the auricle so that the long axis of the flap is located along the postauricular fold. A section of cartilage is excised at the base and fixed in the form of a spacer between the restored part of the ear and the temporal region. The skin defect is restored with a previously cut flap and a free skin graft. The contours of the auricle are formed with gauze rolls. In case of pronounced antihelix (Stahl's ear), the deformity is eliminated by wedge-shaped excision of the lateral leg of the antihelix.

Normally, the angle between the upper pole of the auricle and the lateral surface of the skull is 30°, and the scaphoconchal angle is 90°. In patients with protruding ears, these angles increase to 90 and 120-160°, respectively. To correct protruding ears Many methods have been proposed. The most common and convenient method is Converse-Tanzer and A. Gruzdeva, in which an S-shaped skin incision is made along the back surface of the auricle, retreating 1.5 cm from the free edge. The posterior surface of the auricle cartilage is exposed. The boundaries of the antihelix and the lateral crus of the antihelix are drawn through the anterior surface with needles; the cartilage of the auricle is cut along the marks and thinned. The antihelix and its stem are formed using continuous or interrupted sutures in the form of a “cornucopia”.

Additionally, a section of cartilage measuring 0.3x2.0 cm is cut out from the recess of the auricle. The auricle is fixed to the soft tissues of the mastoid process with two U-shaped sutures. The wound behind the ear is sutured. Gauze bandages secure the contours of the auricle.

Operation according to A. Gruzdeva. An S-shaped skin incision is made on the posterior surface of the auricle, 1.5 cm from the edge of the helix. The skin of the posterior surface is mobilized to the edge of the helix and the postauricular fold. The boundaries of the antihelix and the lateral crus of the antihelix are marked with needles. The edges of the dissected cartilage are mobilized, thinned and sutured into a tube (body of the antihelix) and a groove (leg of the antihelix). Additionally, a wedge-shaped section of cartilage is excised from the lower crus of the helix. The antihelix is ​​fixed to the cartilage of the conchal fossa. Excess skin on the back surface of the auricle is excised in the form of a strip. A continuous suture is applied to the edges of the wound. The contours of the antihelix are strengthened with gauze bandages and fixed mattress sutures.

Atresia of the external auditory canal. The goal of rehabilitation of patients with severe ear malformations is to form a cosmetically acceptable and functional external auditory canal for transmitting sounds from the auricle to the cochlea while maintaining the function of the facial nerve and labyrinth. The first task that should be solved when developing a rehabilitation program for a patient with microtia is to determine the feasibility and timing of meatotympanoplasty.

The results of computed tomography of the temporal bones should be considered the decisive factors in patient selection. A 26-point assessment of computed tomography data of the temporal bone in children with atresia of the external auditory canal was developed. Data is entered into the protocol separately for each ear.

If the score is 18 or more, you can perform a hearing-improving operation - meatotympanoplasty. In patients with atresia of the external auditory canal and conductive hearing loss III-IV degrees, accompanied by gross congenital pathology of the auditory ossicles, labyrinthine windows, facial nerve canal, with a score of 17 or less, the hearing-improving stage of the operation will not be effective. If this patient has microtia, it is only rational to carry out plastic surgery for reconstruction of the auricle.

Patients with stenosis of the external auditory canal are indicated for dynamic observation with computed tomography of the temporal bones to exclude cholesteatoma of the external auditory canal and middle ear cavities. If signs of cholesteatoma are detected, the patient must undergo surgical treatment aimed at removing cholesteatoma and correcting stenosis of the external auditory canal.

Meatotympanoplasty in patients with microtia and atresia of the external auditory canal according to S.N. Lapchenko. After hydropreparation in the area behind the ear, an incision is made in the skin and soft tissue along the posterior edge of the rudiment. Typically, the planum mastoideum is exposed, the cortical and perianthral cells of the mastoid process, the cave, the entrance to the cave are opened with a bur, and the external auditory canal with a diameter of 15 mm is formed. A free flap is cut out from the temporal fascia and placed on the anvil and the bottom of the ear canal being formed, the rudiment of the auricle is transferred behind the auditory canal, the postauricular incision is extended downwards and a skin flap is cut out on the upper leg. The soft tissue and skin edges of the wound are sutured to the level of the lobe, the distal incision of the rudiment is fixed to the edge of the postauricular wound near the hair growth area, the proximal edge of the flap is lowered into the ear canal in the form of a tube for complete closure bone walls ear canal, which ensures good healing in postoperative period. In cases of sufficient skin grafting, the postoperative period proceeds smoothly: the tampons after surgery are removed on the 7th day, then changed 2-3 times a week for 1-2 months, using glucocorticoid ointments.

Meatotympanoplasty for isolated atresia of the external auditory canal according to R. Jahrsdoerfer. The author uses direct access to the middle ear, which avoids a large mastoid cavity and difficulties with its healing, but recommends it only to an experienced otosurgeon. The auricle is retracted anteriorly, a neotympanic flap is isolated from the temporal fascia, and a periosteal incision is made closer to the temporomandibular joint. If it is possible to detect a rudimentary tympanic bone, they begin to work with a bur in this place forward and upward (as a rule, the middle ear is located directly medially). A common wall is formed between the temporomandibular joint and the mastoid bone. This will become the anterior wall of the new ear canal. The formed direction will lead the surgeon to the atresia plate, and the pneumatized cells to the antrum. The atresia plate is thinned with diamond cutters.

If the middle ear is not detected at a depth of 2.0 cm, the surgeon should change direction. After removal of the atresia plate, the elements of the middle ear are clearly visible: the body of the incus and the head of the malleus are, as a rule, fused, the handle of the malleus is absent, the neck of the malleus is fused with the area of ​​atresia. The long process of the incus may be thinned, tortuous, and positioned vertically or medially to the malleus. The location of the stirrup is also variable. In 4% of cases the stapes was completely motionless; in 25% of cases the author discovered the passage of the facial nerve through the tympanic cavity. The second limb of the facial nerve was located above the round window niche, and there was a high probability of injury to the facial nerve while working with the bur. The notochord was discovered by R. Jahrsdoerfer in half of the cases (with its close location to the elements of the middle ear, the probability of injury is always high). The best situation is to detect auditory ossicles, albeit deformed, but working as a single sound transmission mechanism. In this case, the fascial flap is placed on the auditory ossicles without additional cartilage supports. At the same time, when working with a bur, you should leave a small bone canopy over the auditory ossicles, which allows you to form a cavity, and the auditory ossicles are in central position. Before applying the fascia, the anesthesiologist should reduce the oxygen pressure to 25% or switch to room air ventilation to avoid “inflating” the fascia. If the neck of the malleus is fixed to the area of ​​atresia, the bridge should be removed, but only in last moment, using a diamond cutter and low bur speed to avoid injury to the inner ear.

In 15-20% of cases, prostheses are used, as in usual types ossiculoplasty. In cases of stapes fixation, it is recommended to stop this part of the operation. The ear canal and neomembrane are formed, and ossiculoplasty is postponed for 6 months to avoid the creation of two unstable membranes (neomembrane and oval window membrane), the possibility of displacement of the prosthesis and injury to the inner ear.

The new ear canal must be covered with skin, otherwise scar tissue will develop very quickly in the postoperative period. The author takes a split skin flap with a dermatome from the inner surface of the child’s shoulder. It should be remembered that a thick skin flap will curl up and be difficult to work with; too thin will be easily vulnerable when suturing or wearing a hearing aid. The thinner part of the skin flap is applied to the neomembrane, the thicker part is fixed to the edges of the ear canal. The placement of the skin flap is the most difficult part of the operation; then a silicone protector is inserted into the ear canal up to the neomembrane, which prevents displacement of both the skin and neotympanic flap and forms the ear canal.

The bony auditory canal can be formed in only one direction, and therefore its soft tissue part should be adapted to the new position. To do this, it is permissible to shift the auricle upward or backward and up to 4.0 cm. A C-shaped skin incision is made along the border of the concha, the tragus area is left intact, it is used to close the anterior wall, which prevents gross scarring. After combining the bone and soft tissue parts of the ear canal, the auricle is returned to its previous position and fixed with non-absorbable sutures. Absorbable sutures are placed at the border of the parts of the ear canal, and the retroauricular incision is sutured.

The results of the operation depend on the number of initial points when assessing computed tomography data of the temporal bone. Early ossiculofixation was noted by the author in 5% of cases, stenosis of the auditory canal - in 50%. Late complications operations - the appearance of foci of neoosteogenesis and cholesteatoma of the auditory canal.

On average, hospitalization requires 16-21 days, the subsequent outpatient period of follow-up treatment takes up to 2 months. A decrease in sound conduction thresholds by 20 dB is considered a good result; it is achieved, according to various authors, in 30-45% of cases. IN postoperative management For patients with atresia of the external auditory canal, courses of resorption therapy can be included.

Microtia. The pendants must be removed before the reconstruction of the auricle begins in order to avoid disturbances in the vascularization of the transplanted tissue. The mandible may be smaller on the affected side, especially in Goldenhar syndrome. In these cases, it is necessary to initially reconstruct the ear, then lower jaw. Depending on the reconstruction technique, the costal cartilage taken for the auricle frame can also be used for the reconstruction of the lower jaw. If reconstruction of the lower jaw is not planned, auriculoplasty should take into account the asymmetry of the facial skeleton. Currently, ectoprosthetics in childhood possible, but due to the characteristics of fixation and hygiene, it is more common in adults and children over 10 years of age.

From the proposed methods surgical correction Microtia is the most common multi-stage auriculoplasty with costal cartilage. An important decision in the management of these patients is the timing of surgery. For large deformities where costal cartilage is needed, auriculoplasty should begin after 7-9 years. The disadvantage of the operation is high probability graft resorption.

Silicone and porous polyethylene are used from artificial materials. When reconstructing the auricle in patients with microtia and atresia of the external auditory canal, auriculoplasty should be done first, since any attempt at reconstructing hearing will be accompanied by severe scarring, which significantly reduces the possibility of using the skin of the parotid area, and may not be entirely good cosmetic result. Since surgical correction of microtia requires several stages of surgery, the patient should be fully warned of the potential risks, including unsatisfactory aesthetic results. Below are some basic principles for surgical correction of microtia.

The patient must be of sufficient age and height to be able to harvest rib cartilage for the ear frame. Rib cartilage can be taken from the affected side, but preferably from the opposite side. It should be remembered that severe local trauma or a significant burn of the temporal region prevents surgery due to widespread scarring and lack of hair. At chronic infections deformed or newly formed ear canal surgical intervention should be postponed. If the patient or his parents expect unrealistic results, the operation should not be performed.

The auricle of the abnormal and healthy ear is measured, the vertical height is determined, the distance from the outer corner of the eye to the helix, the distance from the outer corner of the eye to the anterior fold of the lobe, the height of the upper point of the auricle is determined in comparison with the eyebrow, and the lobe of the rudiment is compared with the lobe healthy ear. The contours of the healthy ear are drawn onto the x-ray film. The resulting pattern is subsequently used to create the frame of the auricle from costal cartilage. For bilateral microtia, a sample is created from the ear of one of the patient's relatives.

Auriculoplasty for cholesteatoma. Children with congenital stenosis of the external auditory canal are at high risk of developing cholesteatoma of the external and middle ear. If cholesteatoma is detected, middle ear surgery should be performed first. In these cases, subsequent auriculoplasty uses the temporal fascia (the donor site is well hidden under the hair, a large area of ​​tissue can be obtained for reconstruction on a long vascular pedicle, allowing the removal of scars and inappropriate tissue and good coverage of the rib implant). A split skin graft is placed on top of the rib frame and temporalis fascia.

Ossiculoplasty is performed either at the stage of formation of the auricle distant from the skull, or after completion of all stages of auriculoplasty. Another type of rehabilitation of auditory function is implantation of a bone hearing aid. Below are the most commonly used proprietary methods of auriculoplasty in patients with microtia. The most widely used method of surgical treatment of microtia is the Tanzer-Brent method - a multi-stage treatment in which the auricle is reconstructed using several autogenous rib implants.

A skin pocket for the rib implant is formed in the parotid area. It should be formed by already having a prepared auricle frame. The position and size of the auricle are determined using a pattern made from x-ray film. The cartilaginous frame of the auricle is inserted into the formed skin pocket. The authors leave the auricle rudiment intact at this stage of the operation. After 1.5-2 months, the next stage of auricle reconstruction can be carried out - transferring the earlobe to a physiological position. At the third stage, the Tanzer forms the auricle and the postauricular fold spaced from the skull. The author makes an incision along the periphery of the curl, retreating a few millimeters from the edge. The tissues in the area behind the ear are tightened with skin and fixing sutures, thereby somewhat reducing the wound surface and creating a hairline that does not differ significantly from the growth line on the healthy side. Wound surface covered with a split skin graft taken from the thigh in the “panty area”. If meatotympanoplasty is indicated for the patient, then it is performed at this stage of auriculoplasty.

The final stage of auriculoplasty includes the formation of a tragus and imitation of the external auditory canal: on the healthy side, a full-thickness skin-cartilaginous flap is cut out from the conchal area using a J-shaped incision. Part of the soft tissue is additionally removed from the conchal area on the affected side to form a conchal depression. The tragus is formed in a physiological position. The disadvantage of the method is the use of child's costal cartilages measuring 3.0x6.0x9.0 cm, with a high probability of melting of the cartilage frame in the postoperative period (up to 13% of cases); large thickness and low elasticity of the formed auricle.

A complication such as cartilage melting nullifies all attempts made to restore the patient’s auricle, leaving scars and tissue deformation in the area of ​​intervention. That is why to this day there is an ongoing search for bioinert materials that can well and constantly retain their given shape. The frame of the auricle is made of porous polyethylene. Separate standard fragments of the auricle frame have been developed. The advantage of this method of reconstruction of the auricle is the stability of the created shapes and contours of the auricle, and the absence of the likelihood of cartilage melting. At the first stage of reconstruction, a polyethylene frame of the auricle is implanted under the skin and superficial temporal fascia. At stage II - abduction of the auricle from the skull and formation of the postauricular fold. From possible complications nonspecific inflammatory reactions, loss of the temporoparietal fascial or skin graft, and extrusion of the Meyorog frame (1.5%) were noted.

It is known that silicone implants retain their shape well and are bioinert, and therefore they are widely used in maxillofacial surgery. A silicone frame is used in the reconstruction of the auricle. Implants are made of soft, elastic, biologically inert, non-toxic silicone rubber. They withstand any type of sterilization, retain elasticity, strength, do not dissolve in tissues and do not change shape. Implants can be processed with cutting instruments, which allows their shape and size to be adjusted during surgery. To avoid disruption of the blood supply to tissues, improve fixation and reduce the weight of the implant, it is perforated with through holes over the entire surface at the rate of 7-10 holes per 1.0 cm.

The stages of auriculoplasty with a silicone frame coincide with the stages of reconstruction. The use of a ready-made silicone implant eliminates additional traumatic operations on the chest in cases of reconstruction of the auricle using autologous cartilage, and reduces the duration of the operation. The silicone frame of the auricle allows you to obtain an auricle that is close to normal in contour and elasticity. When using silicone implants, you should be aware of the possibility of their rejection.

There is a certain quota of cases of postoperative stenosis of the external auditory canal, and it is 40%. The use of a wide ear canal, removal of all excess soft tissue and cartilage around the external auditory canal, and close contact of the skin flap with the bony surface and fascial flap prevent stenosis. The use of soft protectors in combination with glucocorticoid ointments may be useful in initial stages development of postoperative stenosis. In cases of a tendency to reduce the size of the external auditory canal, a course of endaural electrophoresis with hyaluronidase (8-10 procedures) and injections of hyaluronidase in a dose (10-12 injections) is recommended depending on the age of the patient.

In patients with Treacher-Collins and Goldenhar syndromes, in addition to microtia and atresia of the external auditory canal, there are developmental disorders of the facial skeleton due to underdevelopment of the mandibular branch and the temporomandibular joint. They are advised to consult an oral surgeon and an orthodontist to decide whether mandibular ramus retraction is necessary. Correction of congenital underdevelopment of the lower jaw in these children significantly improves their appearance. Thus, if microtia is detected as a symptom of congenital hereditary pathology of the facial area, in rehabilitation complex Patients with microtia should be included in consultation with oral and maxillofacial surgeons.