Anomalies of ear development. Pathologies of ear development

Anomalies of ear development include congenital changes in the size, shape or position of various elements of the outer, middle and inner ear. The variability of malformations of the auricle is very high. An enlargement of the auricle or its individual elements is referred to as macrotia, a decrease or complete absence of the auricle is referred to as microtia and anotia, respectively. Additional formations in the parotid region are possible - ear pendants or parotid fistulas. The position of the auricle, in which the angle between the auricle and the side surface of the head is 90°, is considered abnormal and is referred to as protruding ears.

Malformations of the external auditory canal (atresia or stenosis of the external auditory canal), auditory ossicles, labyrinth - a 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 second degree are deformations of the auricle of 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 impaired hearing 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 the 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 recent plastic surgery, the classification of N. Weerda and R. Siegert is 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”).
❖ Colobomas 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; hearing loss 1st degree.
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 an abnormal location of the facial nerve canal and labyrinthine capsule (the identified changes are contraindications for hearing-improving surgery).

Diagnostics. Diagnostics includes examination, examination of auditory function, medical genetic research and consultation with an oral and 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 young children, objective hearing research methods are used: determining thresholds by 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.

In cases of diagnosed bilateral conductive hearing loss, wearing a hearing aid with a 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 healthy child because the mucous membrane continues from the nasopharynx into the 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 a 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 when assessing the structures of the outer, middle and inner ear in children with congenital atresia of the 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 of the 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 is the diagnosis of syndromes and the 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 treatment method 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, the upper part of the auricle should be freed from under the skin and the skin defect should be closed.

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. Many methods have been proposed to correct protruding ears. 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 of III-IV degrees, accompanied by gross congenital pathology of the auditory ossicles, windows of the labyrinth, 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 rational to perform only plastic surgery to reconstruct 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 to completely cover the bone walls of the ear canal, which ensures good healing in the 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 a 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 at the last moment, using a diamond cutter and a low bur speed to avoid injury to the inner ear.

In 15-20% of cases, prostheses are used, as with conventional types of 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 of the operation are 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. Postoperative management of patients with atresia of the external auditory canal can include courses of resorption therapy.

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 first necessary to reconstruct the ear, then the 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 is possible, but due to the peculiarities of fixation and hygiene, it is more common in adults and children over 10 years of age.

Of the proposed methods of surgical correction of microtia, the most common is multi-stage auriculoplasty with rib 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 the high probability of 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 a not entirely good cosmetic result is possible. 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. For chronic infections of the deformed or newly formed ear canal, surgery 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 rudiment of the auricle 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. The wound surface is covered with a split skin graft taken from the thigh in the “pant 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 recess. 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. Possible complications include nonspecific inflammatory reactions, loss of the temporoparietal fascial or skin graft, and extrusion of the Meerog frame (1.5%).

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 the initial stages of the 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 a congenital hereditary pathology of the facial area, consultations with maxillofacial surgeons should be included in the rehabilitation complex of patients with microtia.

According to medical statistics, from 7 to 20 percent of people in the world have anomalies and malformations of the ear, which are less commonly called ear deformities when it comes to the auricle. Doctors note the predominance of men among patients with such disorders. Anomalies and malformations of the ear can be congenital, arising as a result of intrauterine pathologies, and acquired as a result of injury, slowing or accelerating the growth of this organ. Disturbances in the anatomical structure and physiological development of the middle and inner ear lead to deterioration or complete loss of hearing. In the field of surgical treatment of anomalies and malformations of the ear, the largest number of operations are named after doctors whose method has not undergone any new improvements throughout the history of the treatment of this type of pathology. Anomalies and malformations of the ear according to their location are discussed below.

Pinna or outer ear

The anatomical structure of the auricle is so individual that it can be compared to fingerprints - no two are alike. The normal physiological structure of the auricle is considered to be when its length approximately coincides with the size of the nose and its position does not exceed 30 degrees in relation to the skull. Ears are considered protruding when this angle is or exceeds 90 degrees. The anomaly manifests itself in the form of macrotia of the auricle or its parts in case of accelerated growth - for example, the earlobes or one ear, as well as its upper part, may increase. Less common is poliotia, which manifests itself in the presence of ear appendages to a completely normal auricle. Microtia is underdevelopment of the shell, up to its absence. The “sharp ear” of Darwin, who was the first to classify it as an element of atavism, is also considered an anomaly. Another manifestation of it is observed in the ear of a faun or the ear of a satyr, which is the same thing. Cat's ear is the most pronounced deformation of the auricle, when the upper tubercle is strongly developed and at the same time bent forward and downward. Coloboma or splitting of the auricle or earlobe also refers to anomalies and malformations of development and growth. In all cases, the functionality of the hearing organ is not impaired, and the surgical intervention is more of an aesthetic and cosmetic nature, as, in fact, with trauma and amputation of the auricle.

Back in the first half of the last century, studying the development of the embryo, doctors came to the conclusion that earlier than the middle and outer ear, the inner ear develops, its parts are formed - the cochlea and the labyrinth (vestibular apparatus). It was found that congenital deafness is explained by underdevelopment or deformation of these parts - aplasia of the labyrinth. Atresia or fusion of the ear canal is a congenital anomaly and is often observed together with other defects of the ear, and is also accompanied by microtia of the auricle, disorders in the eardrum, and auditory ossicles. Defects of the membranous labyrinth are called diffuse anomalies and are associated with intrauterine infections as well as fetal meningitis. For the same reason, a congenital preauricular fistula appears - a channel of several millimeters that goes inside the ear from the tragus. In many cases, surgery using modern medical technology can help improve hearing in cases of abnormalities in the middle and inner ear. Cochlear prosthetics and implantation are very effective.

– a group of congenital pathologies that 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 anamnesis, objective examination, assessment of sound perception, audiometry, impedance measurement or ABR test, computed tomography. Treatment is surgical.

General information

Developmental anomalies of the auricle are a relatively rare group of 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

Defects of the external ear are the result of disorders of intrauterine development of the fetus. Hereditary defects are relatively rare and are part of the genetically determined syndromes: Nager, Treacher-Collins, Konigsmark, Goldenhar. A significant part of the anomalies in the formation of the 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 examinations and prolonged exposure to high temperatures (hyperthermia). Radiation therapy for cancer and radioactive iodine are less common etiological factors.
• Mother's bad habits. Relatively often, disturbances in the intrauterine development of a child are provoked by chronic alcohol intoxication, narcotic substances, and the use of cigarettes and other tobacco products. Among drugs, cocaine plays the most significant role.
• Medications. A side effect of some groups of 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 disorders and the functioning of the mother's endocrine glands during pregnancy. The list includes the following pathologies: decompensated diabetes mellitus, phenylketonuria, thyroid lesions, 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 branchial arch. Under normal conditions, the precursor tissues of the outer ear are formed by the end of the 7th week of intrauterine development. At the 28th obstetric week, the appearance of the external ear corresponds to that of a newborn baby. 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 the delivery room. 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 the 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 commonly, the upper part of the shell is completely absent. 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 out, and the angle between the bones of the skull and the cartilage is more than 30 degrees, due to which the latter “protrudes” somewhat outward.

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 the skin located behind it. 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, the middle part of the helix 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 of the 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. The 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 of varying 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. In case of isolated lesions of the outer ear or their combination with pathologies of the auditory ossicles, the audiogram shows a deterioration in sound conduction while maintaining bone conductivity. 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 and the presence of concomitant 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 surgical techniques are used in pediatric otolaryngology:

• Otoplasty. Restoring the natural shape of the auricle is performed in two main ways - using synthetic implants or an autograft taken from the cartilage of the VI, VII or VIII 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

The health prognosis and cosmetic result depend on the severity of the defect and the timeliness of 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.

In progress evolution of the human ear evolved into a complex organ consisting of many components necessary to conduct sound. This chapter discusses the embryology of the outer and inner ear and the most common congenital anomalies.

Home function of the external and middle ear is to conduct a sound wave to the inner ear. Anomalies and malformations can lead to both cosmetic and functional disorders.

A) Embryology of the outer and middle ear. The embryonic development of the outer and middle ear is an extremely complex process. Developmental abnormalities usually result from genetic mutations or exposure to teratogenic environmental factors. Knowledge of embryology makes it easier to study the malformations described in this chapter.

All parts of the hearing organ develop simultaneously with each other and with all other organs of the head and neck. The inner ear is the first to form, and starting from the third week of gestation, it begins to develop separately from the outer and middle ear, which appear around the fourth week after conception. The tubotympanic pouch is formed from the endoderm of the first gill pouch.

Then during embryonic development There is a gradual expansion of the tympanic cavity, which seems to absorb the auditory ossicles and the structures surrounding them. In the eighth month of gestation, the auditory ossicles finally take their place in the tympanic cavity.

Schematic representation of the auricle, which shows the concept of the development of the auricle from the tubercles of His.
First tubercle, tragus; second tubercle, pedicle of the helix; third tubercle, ascending part of the helix;
fourth tubercle, horizontal part of the helix, partly antihelix and scaphoid fossa; fifth tubercle
the descending part of the helix, partly the antihelix and the scaphoid fossa; sixth tubercle, antitragus and part of the helix.

Auditory ossicles develop from the mesenchyme of the neural crest of the first (Meckel's cartilage) and second (Reichert's cartilage) branchial arches. From the first branchial arch the head of the malleus, a short process and the body of the incus are formed. The long process of the incus, the manubrium of the malleus, and the structures of the stapes are formed from the second branchial arch. The vestibular surface of the footplate of the stapes base and the annular ligament of the stapes develop from the mesoderm of the auditory capsule.

From ectoderm of the first gill cleft, located between the first and second gill arches, the external auditory canal develops. Invagination of the epithelium occurs, in which a canal is formed at approximately 28 weeks of gestation, after which the formation of the tympanic membrane becomes possible. The lateral part of the tympanic membrane develops from the ectoderm of the first branchial cleft, the medial part from the endoderm of the first branchial sac, and the central part from the neural crest mesenchyme.

Formation of the auricle begins from the fifth week of intrauterine life. Three tubercles are formed from the first and second gill tubercles. Then, from these six tubercles, the six characteristic elements of the auricle, which are present in adults, will develop: the first tubercle is the tragus; the second tubercle is the helix; the third tubercle is the ascending part of the helix; the fourth tubercle is the horizontal part of the helix, partly the antihelix and the scaphoid fossa; the fifth tubercle is the descending part of the helix, partially the antihelix and the scaphoid fossa; the sixth tubercle is the antitragus and part of the helix.

b) Common abnormalities of the external ear. As mentioned above, the outer and middle ears develop separately from the inner ear, because have different embryological origins. Disruption of normal embryogenesis, caused by genetic or environmental factors, can lead to the development of various anomalies of the outer and middle ear. Some of them are described below.

V) Ear abnormalities: protruding ears and lop ears. The literature describes disorders of ear development of varying degrees of severity. The most common symptoms are anotia, microtia (hypoplasia of the pinna), and protruding ears. Functional and aesthetic disturbances caused by these anomalies can cause significant emotional suffering in the patient.

Speakers ears forward (protruding ears) are quite common. The formation of a “hanging” ear is also possible: if during embryological development the antihelix does not unfold, the helix continues to curl lower and lower. There are also other minor malformations of the auricle. The most common symptoms are an increased angle between the auricle and the scalp (normally it is 15-30°), underdevelopment of the antihelix, excess cartilage tissue of the auricle, and deformation of the lobe (most often its protrusion anteriorly).

Otoplasty is called surgical restoration, reconstruction or reshaping of the ear. Already in childhood, the development of the auricle stops, so it is safe to perform otoplasty at this age. Many surgical techniques have been described. The Mustarde technique involves creating antihelix folds by placing several horizontal mattress sutures along the navicular fossa.

Furnas describes the application of a conchomastoid suture, with the help of which the scaphoid fossa is reduced and the auricle is shifted posteriorly. More radical methods involving excision of cartilage have been described by Pitanguy and Farrior. Typical complications of otoplasty are: insufficient correction, chondritis, hematoma, “telephone ear” deformity (excessive flexion of the middle third of the antihelix with insufficient flexion of its upper and lower portions). Otoplasty is discussed in more detail - please use the search form on the main page of the site.

Oblique (a), lateral (b) and posterior (c) views of the protruding ear.
A large navicular fossa and underdevelopment of the antihelix lead to displacement of the auricle to the lateral side and its pseudoptosis.
(d) Folding of the auricle, excessive curl of the helix.
(a, c) Patient before and (b, d) after otoplasty.

G) Preauricular fistulas and cysts. Preauricular cysts, fistulas and sinuses are quite common in pediatric patients. They are thought to develop due to abnormalities of the first branchial arch and first gill pouch. They are located anterior to the auricle, usually at the ascending part of the helix. Preauricular sinuses are usually closely associated with the helix cartilage, and incomplete removal often leads to recurrence. Typically, an elliptical incision of the skin around the fistula is made, after which tissue detachment occurs at the root of the helix. The elliptical incision can be extended upward (suprauricular approach), thereby improving visibility and simplifying removal.

Also common cutaneous preauricular appendages. Sometimes they contain cartilage inside. Most often they are localized anterior to the auricle, at the level of the supratragal notch. Presumably, their cause is excessive tissue growth in the embryonic period. If desired by the patient or parents, they can be removed.

d) Abnormalities of the first gill cleft. As mentioned above, the external auditory canal and the lateral part of the tympanic membrane are formed from the first gill cleft. Developmental disorders lead to the formation of cysts, sinuses and fistulas. Type I anomalies include duplications of the external auditory canal; they represent a fistula tract, which is very often adjacent to the parotid salivary gland. Type II anomalies include superficial sinuses and cysts on the anterior surface of the neck, below the angle of the mandible.

They are usually discovered earlier than type I anomalies. Both types of anomalies can become infected repeatedly. If there is discharge from the ear that persists despite conservative therapy, a malformation of the external ear should be suspected (especially if there are any deformities or abscesses in the neck). If a decision is made about surgical treatment, the formation must be completely removed, because they recur extremely often. Often the cyst or sinus is closely connected with the fibers of the facial nerve; in all cases, dissection should be carried out extremely carefully; Sometimes a partial parotidectomy is required.

(a) Pre- and (b) intraoperative appearance of a type I branchial cleft cyst.

e) . Occurs with an average frequency of 1:10000-1:20000. Approximately one third of patients have some degree of bilateral atresia. Atresia of the auditory canal with middle ear anomalies can be either isolated or coexist with other anomalies, such as microtia. As mentioned earlier, the external auditory meatus develops from the first branchial slit. If the process of canalization during embryonic development stops for some reason, stenosis or atresia of the auditory canal develops. Atresia of the bony part of the auditory canal is always secondary; it is formed in the presence of malformations of the temporal bone.

Diagnosis and treatment of atresia begin with a thorough assessment of the function of the auditory analyzer, after which the issue of hearing aids is decided. In preparation for surgery, as well as to diagnose congenital cholesteatoma, a CT scan of the temporal bones is performed. The anatomical structure of the middle ear and the course of the facial nerve canal are assessed. With unilateral atresia, the severity of conductive hearing loss is usually maximum, but if there is normal hearing in the second ear, treatment may be delayed.

At stenosis of the external auditory canal Hearing aids may be used. If complete, bone conduction hearing aids help. Early hearing aid is mandatory in case of bilateral atresia. Bone conduction hearing aids can be used after the first few months of life.

Surgical treatment performed at the earliest age of 6-7 years, usually after microtia surgery has already been performed, so that reconstruction can be performed outside of scar tissue. The goal of the operation is to create a functional sound pathway through which the sound wave can reach the cochlea; However, achieving this goal is quite difficult. Not all children are eligible for surgical treatment. Jahrsdoerfer proposed a 10-point scale that assesses the likelihood of a good functional outcome after canaloplasty.

Scale evaluates the following factors: presence of the stapes, volume of the middle ear cavity, course of the facial nerve, presence of the malleus-incus complex, pneumatization of the mastoid process, preservation of the incus-stapedius joint, condition of the round window, lumen of the oval window, condition of the auricle. The presence of each parameter adds one point to the scale (the presence of a stirrup adds two points). It is believed that children with a score of 8 or higher are most likely to have a favorable outcome from the operation.

Atresia of the external auditory canal is often combined with microtia.
A formation that externally resembles the navicular fossa and the external auditory canal,
ends in a blind pocket.

and) Microtia. The degree of microtia is assessed by the severity of the deformation of the auricle. Anotia is the complete absence of the ear. Meurman proposed a classification of microtia based on the severity of the deformation: in grade I, the auricle is reduced and deformed, but the main identifying contours are preserved; in grade II, the anterior vertical portion of the cartilage and skin is preserved, but the anterior portion of the shell is absent; Grade III corresponds to a practically absent auricle, when only the lobe, often atypically located, as well as remnants of cartilage and skin are preserved. Grade III is also sometimes called “peanut ear.”

Operational intervention for microtia and concomitant atresia of the auditory canal requires the interaction of a facial plastic surgeon and an otosurgeon. Most surgeons agree that pinna reconstruction for microtia can be performed as early as age six, as by this time the contralateral pinna is already about 85% of its final size and can be used as a reference.

Moreover, to this age the patient has a sufficient amount of cartilage tissue for transplantation and psychological preparation for the operation is easier. With unilateral myicrotia, sometimes they wait a little longer, because cartilage tissue becomes denser and more suitable for giving it the desired shape. Reconstruction of the external ear using autograft of costal cartilage has been described.

Besides cartilage autografts Some implants, such as porous high-density polyethylene (Medpor; Porex Surgical, Newnan, GA), may be used. These implants are placed under the temporoparietal facial flap and covered with a skin graft. The choice of material for reconstruction is previously discussed with the patient and family members.

Surgery is not the only reconstruction option; in many cases, ear replacement is acceptable. If you have ear canal atresia, improved hearing can be achieved using bone conduction hearing aids.

h) Key points:
From the first branchial arch the head of the malleus, a short process and the body of the incus are formed. The long process of the incus, the manubrium of the malleus, and the structures of the stapes are formed from the second branchial arch. The vestibular surface of the footplate of the stapes base and the annular ligament of the stapes develop from the mesoderm of the auditory capsule.
Type I branchial cleft cysts are a duplication of the external auditory canal and extend lateral to the facial nerve. Type II cysts extend downwards, towards the angle of the mandible; they can be located either lateral or medial from the facial nerve.

There are developmental anomalies of the outer, middle and inner ear. Congenital anomalies of the hearing organ are observed in approximately 1 in 7000-15000 newborns, most often on the right side. Boys suffer on average 2-2.5 times more often than girls. Most cases of ear malformations are sporadic, about 15% are hereditary.

Exposure during pregnancy:

• Infections (influenza, measles, rubella, herpes, CMV, chickenpox).
• Mother's age is over 40 years.
• Toxic effects of drugs (aminoglycosides, cytostatics, barbiturates), smoking, alcohol.
• Ionizing radiation.

Degrees of defects:

• 1st degree - anotia.
• 2nd degree - complete hypoplasia (microtia).
• Grade 3 - hypoplasia of the middle part of the auricle.
• Grade 4 - hypoplasia of the upper part of the auricle.
• 5th degree - protruding ears.

Congenital absence of the auricle (Q16.0) - complete absence of the auricle.

Clinical picture

In place of the missing ear there is a small depression (pit). The external auditory canal is absent. There is underdevelopment of the bones of the facial skeleton and hearing loss. The face is not symmetrical, the hair growth boundaries are low.

Diagnosis of congenital absence of the auricle

• consultation with an otorhinolaryngologist;
• otoscopy;
• CT, MRI of the brain.

Differential diagnosis: other congenital malformations, acquired diseases of the outer, middle and inner ear.

Treatment of congenital absence of the auricle

Treatment is prescribed only after confirmation of the diagnosis by a medical specialist. Surgical treatment and hearing aids are carried out.