Developmental anomalies of the ear - Treatment

Goals of treatment for ear malformations

Improving hearing function, eliminating cosmetic defects.

Non-drug treatment of ear developmental anomalies

In bilateral conductive hearing loss, normal speech development of the child is facilitated by wearing a hearing aid with a bone vibrator. Where there is an external auditory canal, a standard hearing aid can be used.

A child with microtia has the same chance of developing otitis media as a healthy child, since the mucous membrane from the nasopharynx continues into the auditory tube, middle ear, and mastoid process. There are known cases of mastoiditis in children with microtia and atresia of the external auditory canal (surgical treatment is necessary).

Surgical treatment of ear developmental anomalies

Treatment of patients with congenital malformations of the outer and middle ear is usually surgical, and in severe cases of hearing loss, hearing aids are used. In case of congenital malformations of the inner ear, hearing aids are used. Below are the methods of treating the most frequently observed anomalies of the outer and middle ear.

Developmental abnormalities of the auricle that result from excessive growth (macrotia) are manifested by an enlargement of the entire auricle or part of it. Macrotia usually does not entail functional disorders; it is eliminated surgically.

Auriculoplasty for grade I microtia. The peculiarity of the ingrown auricle is its location under the skin of the temporal region. During the operation, the upper part of the auricle should be released from under the skin and the skin defect should be closed. For this, operations are performed according to the method of F. Burian or G. Kruchinsky.

F. Burian's method involves cutting the skin over the ingrown part of the auricle. The resulting skull wound is covered with a displaced skin flap cut from the scalp and fixed with sutures. A free skin flap is transplanted to the back surface of the auricle.

Kruchinsky-Gruzdeva method. A tongue-shaped incision is made on the back 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 as 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 deformation is eliminated by wedge-shaped excision of the lateral pedicle.

Normally, the angle between the upper pole of the auricle and the lateral surface of the skull is 30 degrees, and the angle between the boat and the auricle is 40 degrees. In patients with protruding auricles, these angles increase to 90 and 120-160 degrees, respectively. Many methods have been proposed for correcting protruding auricles. The most common and convenient is the Converse-Tanser method.

An S-shaped incision is made in the skin along the back surface of the auricle, 1.5 cm from the free edge. The back surface of the auricular cartilage is exposed. The borders of the antihelix and the lateral pedicle are marked with needles through the front surface. The cartilage of the auricle is cut, then its antihelix is thinned and its pedicle is formed with continuous or interrupted sutures in the form of a "cornucopia".

Additionally, a 0.3 x 2 cm section of cartilage is cut out from the auricle cavity, and the edges of the incision are sutured. The auricle is fixed to the soft tissues of the mastoid process with two U-shaped sutures. Then, sutures are applied to the skin wound and the contours of the auricle are formed with gauze bandages.

Barsky's operation. An elliptical skin flap is excised from the back surface of the auricle. The cartilage is exposed, two parallel incisions are made, forming a cartilaginous strip, which is turned out towards the front surface of the auricle. Then sutures are applied, which, when tightened, form an antihelix. The skin of the back surface is sutured.

K. Sibileva's method. An elliptical skin flap is excised on the back surface of the auricle, the lower incision is made along the postauricular fold. The contours of the antihelix and its lateral crus are applied with paint and needles. Strips of cartilage are excised along the marked lines 1-2 mm wide over a length of 3-4 mm. Additionally, a row of notches is applied to the cartilage with parallel incisions. A continuous mattress suture is applied to the edges of the cartilage incisions, and another row of mattress sutures are applied, retreating from the first line by 3-4 mm.

Operation according to G. Kruchinsky. An S-shaped skin flap is excised on the back surface of the auricle, 1.5 cm away from the edge of the helix. Using paint and needles, the direction of the future antihelix is marked and the ear cartilage is dissected. Two more parallel incisions are made outside the first incision, and one additional one is made medially. The auricle is folded, forming the antihelix. Additionally, a strip of cartilage is excised along the edge of the auricle depression. The wound is sutured. The antihelix is reinforced with two or three through mattress sutures on gauze rolls. The threads are passed below the strips of cartilage without sewing it on.

Operation according to D. Andreeva. A spindle-shaped skin flap is excised on the back surface of the auricle. A crescent-shaped strip of cartilage 3 mm wide is marked with two parallel incisions. Two or three U-shaped sutures are applied to the free edges and pulled, forming the relief of the antihelix. The auricle is fixed to the periosteum of the mastoid process with the same threads.

Operation according to A. Gruzdeva. An S-shaped incision is made on the back surface of the auricle, 1.5 cm from the edge of the helix. The skin of the back surface is mobilized up to the edge of the helix and the postauricular fold. The boundaries of the antihelix and lateral crus of the antihelix are marked with needles. The edges of the incised cartilage are mobilized, thinned and sutured in the form of a tube (body of the antihelix) and a groove (crus 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 cavum choncha. 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 reinforced with gauze bandages fixed with mattress sutures.

Meatotympanoplasty

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 preserving 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 appropriateness and timing of meatotympanoplasty.

Selection of patients for hearing-improving surgery. The decisive factors in patient selection should be the results of CT of the temporal bones. N. A. Mileshina developed a 26-point system for assessing CT data of the temporal bone in children with atresia of the external auditory canal. The data are entered into the protocol separately for each ear.

For example, patients with microtia of any degree and conductive hearing loss of II-III degree, with a slightly reduced (or normal size) pneumatized tympanic cavity, mammillary cavity, differentiated and physiologically located malleus and incus in the absence of pathology of the labyrinth windows, inner ear and facial nerve canal, with a score of 18 or more, can undergo hearing-improving surgery - meatotympanoplasty.

In patients with microtia and conductive hearing loss of grades III-IV, accompanied by gross congenital pathology of the auditory ossicles, labyrinth windows, the third part of the facial nerve canal, with a score of 17 or less, the hearing-improving stage of the operation will not be effective. It is rational to perform only plastic surgery to reconstruct the auricle for these patients.

Patients with stenosis of the external auditory canal are recommended dynamic observation with CT of the temporal bones to exclude cholesteatoma of the external auditory canal and middle ear cavities. If signs of cholesteatoma are detected, the patient should undergo surgical treatment aimed at removing the cholesteatoma and correcting the 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 postauricular region, an incision is made in the skin and soft tissues along the posterior edge of the rudiment, the mastoid process area is exposed, the cortical and periantral cells of the mastoid process, the cave, the entrance to the cave are opened with a bur until the incus is widely exposed, and an external auditory canal with a diameter of 15 mm is formed.

A free flap is cut out of the temporal fascia and placed on the incus and the bottom of the formed auditory canal, 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 pedicle. The soft tissues and skin edges of the wound are sutured to the level of the ear lobe, the distal incision of the rudiment is fixed to the edge of the postauricular wound near the hair growth zone, the proximal edge of the flap is lowered and the auditory canal is in the form of a tube to completely close the bone walls of the auditory canal, which ensures good healing in the postoperative period. The formed auditory canal is tamponed with turundas with iodoform.

In cases of sufficient skin grafting, the postoperative period is smooth. Tampons are removed after the operation on the 7th day, then changed 2-3 times a week for 1-2 months, using ointments with glucocorticoids (hydrocortisone).

In the early postoperative period, with pronounced reactive processes, a course (6-8 procedures) of magnetolaser irradiation can be carried out. It is also recommended to apply dressings with heparin or traumeel ointments, taking traumeel C orally in an age-appropriate dose for 10 days. On average, hospitalization periods are 16-21 days with subsequent outpatient treatment for up to 2 months.

Meatotympanoplasty for isolated atresia of the external auditory canal according to Jarsdofer. The author uses direct access to the middle ear, which avoids the formation of a large mastoid cavity and problems 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, the periosteum is incised closer to the temporomandibular joint. If a rudimentary tympanic part of the temporal bone can be detected, the burr is started to work 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 process, which will subsequently become the anterior wall of the new auditory canal. Then, the atresia plate is gradually approached and thinned with diamond cutters. If the middle ear is not detected at a depth of 2 cm, the surgeon should change direction.

After removal of the atresia plate, the elements of the middle ear become clearly visible. The body of the incus and the head of the malleus are usually fused, the handle of the malleus is absent, the neck of the malleus is fused with the atresia zone. The long leg of the incus can be thinned, twisted and located vertically or medially in relation to the malleus. The stapes is also variable. The best situation is considered to be the detection of deformed auditory ossicles, but working as a single mechanism of sound transmission. In this case, the fascial flap is placed on the auditory ossicles without additional cartilage supports. When working with a bur, a small bone overhang should be left over the auditory ossicles, which allows the formation of a cavity (the auditory ossicles are in a central position).

Before the fascia placement stage, the anesthesiologist should reduce the oxygen pressure to 25% or switch to room air ventilation to avoid "inflation" of the fascia. If the neck of the malleus is fixed to the atresia zone, the bridge should be removed, but at the last moment, using a diamond burr and a low speed of the bur, to avoid injury to the inner ear.

In 15-20% of cases, prostheses are used, as in conventional types of ossiculoplasty. In cases of fixation of the stapes, it is recommended to stop the operation by forming the auditory canal and neomembrane, and to postpone ossiculoplasty for 6 months to avoid the creation of two unstable membranes (neomembrane and oval window membrane), as well as the likelihood of displacement of the prosthesis and injury to the inner ear.

The new ear canal should be covered with skin, otherwise scar tissue develops very quickly in the postoperative period. A split skin flap can be taken from the inner surface of the child's shoulder with a dermatome, the thinner part of the skin flap is placed on 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 the displacement of both the skin flap and the neotympanic flap and forms the ear canal canal.

The bony auditory canal can be formed only in one direction, and therefore its soft tissue part should be adapted to the new position. For this purpose, the auricle may be displaced upward or backward and upward by up to 4 cm. A C-shaped cutaneous incision is made along the border of the auricle. The tragus area is left intact, using it to close the anterior wall. After combining the bony and soft tissue parts of the auditory canal, the auricle is returned to its previous position and fixed with non-absorbable sutures. Absorbable sutures are applied at the border of the parts of the auditory canal. The retroauricular incision is sutured.

On average, hospitalization periods are also 16-21 days, followed by outpatient treatment for up to 2 months. A decrease in sound conduction thresholds by 20 dB is considered a good result.

Auriculoplasty by implantation methods

In cases where the lower jaw is smaller on the affected side (especially in Goldenhar syndrome), the ear should be reconstructed first, and then the lower jaw. Depending on the reconstruction technique, the costal cartilage taken for the auricle frame can also be used to reconstruct the lower jaw. If the lower jaw is not planned to be reconstructed, then the presence of asymmetry of the facial skeleton should be taken into account during auriculoplasty.

An important point in the management of such patients is the choice of the time of surgical intervention (in case of large deformations, where costal cartilage is required, auriculoplasty should be started at the age of the patient after 7-9 years). In case of mild deformations in infants, non-surgical correction can be performed by applying bandages.

Of the proposed methods of surgical correction of microtia, the most common is multi-stage auriculoplasty with costal cartilage. The disadvantage is the high probability of transplant resorption. Silicone and porous polyethylene are used as artificial materials.

There are several methods of reconstruction using endoprostheses. Auriculoplasty should be performed first for two reasons. The first reason is that any attempt at hearing reconstruction is accompanied by significant scarring, which significantly reduces the possibilities of using the skin of the parotid region (a larger intervention may be required for auriculoplasty and a not very good cosmetic result is possible). The second reason is that in the case of a unilateral lesion, the external rudiment and appendages are perceived as a severe congenital pathology, while hearing loss is regarded as something not worthy of attention, since the patient hears well due to the healthy ear and speech development does not suffer.

Since surgical correction of microtia is performed in several stages, the patient or his parents should be warned about the potential risks, including an unsatisfactory aesthetic result.

Patient selection. The patient must be of sufficient age, build, and height to allow for the harvesting of costal cartilages for the auricular framework. In a thin patient, the costochondral junction can be palpated and the amount of cartilage can be assessed. Insufficient costal cartilage may impede the success of the operation. Costal cartilage can be harvested from the affected side, but is preferably harvested from the opposite side. Severe local trauma or extensive burns to the temporal region preclude surgery due to widespread scarring and lack of hair. In the presence of chronic infections of the deformed or newly formed ear canal, surgery should be postponed.

Preoperative preparation consists of measuring the auricle of the abnormal and healthy ear. In lateral measurements, the vertical height, the distance from the outer corner of the eye to the crus of the helix, and the distance from the outer corner of the eye to the anterior fold of the lobe are determined. The axis of the auricle coincides with the axis of the nose. In frontal measurements, attention is paid to the height of the upper point of the auricle compared to the eyebrow, and the rudimentary lobe is compared to the lobe of the healthy ear.

A piece of X-ray film is applied to the healthy side, and the contours of the healthy ear are applied. The resulting sample is then used to create a frame for the auricle from costal cartilage. In case of bilateral microtia, the 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 have a high risk of developing cholesteatoma of the outer and middle ear. When cholesteatoma is detected, the middle ear should be operated on first. In these cases, the temporal fascia is used for subsequent auriculoplasty (the donor site is well hidden under the hair, and a large area of tissue can also be obtained for reconstruction on a long vascular pedicle, which allows removing scars and unsuitable tissue and covering the rib graft well). A split skin graft is applied on top of the rib cage and temporal fascia.

Ossiculoplasty is performed at the stage of retraction of the reconstructed auricle or after completion of all stages of auriculoplasty with behind-the-ear access. Another type of auditory function rehabilitation is implantation of a bone hearing aid.

Auriculoplasty for microtia. The most widely used method of surgical treatment of microtia is the Tanzer-Brent method - a multi-stage reconstruction of the auricle using several autogenous rib grafts.

The first stage involves transplanting the auricle framework formed from costal cartilages. To collect the costal cartilages, an incision is made in the skin and soft tissues along the edge of the costal arch and the cartilages of the 6th, 7th and 8th ribs on the side of the chest opposite the ear malformation are exposed. The body of the auricle and the antihelix are formed from the paired cartilages of the 6th and 7th ribs. The cartilage of the 8th rib is most convenient for forming the helix. The author prefers to form the helix crest in the most prominent way. The chest wound is sutured after making sure there is no pneumothorax.

A skin pocket for the rib graft is formed in the parotid region. In order not to disrupt tissue vascularization, it should be formed with the framework of the future auricle already prepared. The position and size of the auricle are determined using a template from an X-ray film from the healthy side in case of a unilateral anomaly or from the auricle of the patient's relatives in case of bilateral microtia. The cartilaginous framework of the auricle is inserted into the formed skin pocket. The rudiment of the auricle is left intact at this stage of the operation.

After 1.5-2 months, the second stage of auricular reconstruction can be performed - transferring the auricular lobe to a physiological position.

At the third stage, the auricle and the postauricular fold are formed, which are separated from the skull. The incision is made along the periphery of the curl, retreating several millimeters from the edge. The tissues in the postauricular area are pulled together with skin and fixing others, thereby somewhat reducing the wound surface; a hairline is created that is not significantly different from the healthy side. The wound surface is covered with a split skin graft taken from the thigh in the "panty zone". If the patient is indicated for meatotympanoplasty, it is performed at this stage of auriculoplasty.

The final stage of auriculoplasty includes the formation of the tragus and imitation of the external auditory canal. On the healthy side, a full-layer skin-cartilage flap is cut out from the auricle area using a J-shaped incision. From the auricle area on the affected side, some soft tissue is additionally removed to form a recess in the auricle. The tragus is formed in a physiological position.

The disadvantage of the method is the use of the child's rib cartilage for the curl, and there is a high probability of melting the cartilaginous framework in the postoperative period (according to various authors, up to 13% of cases). The large thickness and low elasticity of the formed auricle are also considered a disadvantage.

The Tanzer-Brent method was modified by S. Nagata. The skin incisions of the parotid region and the transfer of the ear lobe to a horizontal position proposed by him are performed already at the first stage of the reconstruction of the auricle. The tragus is included in the cartilaginous elements of the frame of the future auricle immediately. Here, cartilages of the VI-VIII ribs of the patient are also used, however, the probability of melting of the cartilaginous autotransplant compared to allotransplants is lower (up to 7-14%).

Such a complication as cartilage melting negates all attempts to restore the patient's auricle, leaving scars and tissue deformation in the area of intervention, therefore, to this day, there is a constant search for biologically inert materials that are capable of well and permanently preserving the shape given to them in the patient practically for life.

T. Romo's method involves the use of porous polyethylene as an auricular framework; the advantage of the method is considered to be the stability of the created forms and contours of the auricle, as well as the absence of melting of the cartilage. Separate standard fragments of the auricular framework have been developed.

At the first stage of reconstruction, a polyethylene frame of the auricle is implanted under the skin and superficial temporal fascia, at the second stage - the auricle is moved away from the skull and a postauricular fold is formed. Among possible complications, the authors note non-specific inflammatory reactions, loss of the temporoparietal fascial or free skin flaps and extraction of the polyethylene frame.

It is known that silicone implants retain their shape well and are biologically inert, which is why they are widely used in maxillofacial surgery. N. A. Mileshina and co-authors use a silicone frame in the reconstruction of the auricle. Implants made of soft, elastic, biologically inert, non-toxic silicone rubber 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 adjusting their shape and size during surgery. To avoid disruption of tissue blood supply, improve fixation and reduce the weight of the implant, it is perforated over the entire surface at a rate of 7-10 holes per 1 cm.

The stages of auriculoplasty with a silicone frame coincide with the stages of reconstruction proposed by S. Nagata.

The use of a ready-made silicone implant eliminates additional traumatic operations on the chest in cases of auricular reconstruction using a cartilaginous autograft, and also reduces the duration of the operation. A silicone auricular framework allows one to obtain an auricle that is close to normal in contours and elasticity, while the use of a cartilaginous allograft as an auricular framework has a low aesthetic result. However, when using silicone implants, one should remember about the possibility of their rejection.

The most common complications of auricular plastic surgery using costal cartilage are pneumothorax and lung collapse when isolating costal cartilages and using them as a framework for the future auricle. Other complications are associated with compression of transplanted tissues due to improper application of dressings in the postoperative period, infection of the wound through the previously formed external auditory canal or during surgery. Postoperative hematomas, facial nerve paralysis, NST, necrosis of transplanted flaps, and the development of keloid scars are also observed.

A W-shaped incision in the parotid skin to form a pocket for a silicone or cartilaginous implant prevents extrusion of the auricular framework. Separate formation of the anterior and posterior surfaces of the auricle is used to prevent disruption of the nutrition of the transplanted grafts.

Further management

To improve the nutrition of transplanted tissues, parenteral administration of drugs that improve microcirculation (rheopolyglucin, pentoxifylline, vinpocetine, ascorbic acid solution, nicotinic acid solution), as well as hyperbaric oxygenation, is recommended.

Special sterile medical wipes are used to cover donor surfaces. Hypertrophic scars may form in the auriculoplasty area, on the chest and on the donor areas of the buttocks. In this case, prolonged glucocorticoids are prescribed, which are injected into the base of the scar, as well as phonophoresis with enzymes (collagenase, hyaluronidase).

Postoperative stenosis of the external auditory canal may develop (40% of cases). In these cases, soft protectors are used in combination with ointments containing glucocorticoids. If there is 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 solutions at a dose of 32-64 U (10-12 injections) are recommended, depending on the patient's age.

Postoperative management of patients with atresia of the external auditory canal consists of prescribing courses of resorption therapy (electrophoresis with hyaluronidase on the area of postoperative stenosis and intramuscular administration of 32-64 U of hyaluronidase solution). A total of 2-3 courses of resorption therapy are recommended with an interval of 3-6 months.

Forecast

As a rule, the improvement of hearing function is 20 dB, which in case of bilateral anomaly requires hearing aids. In some cases, aesthetic correction does not satisfy the patient.

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