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14. K. Cryns et al. A genotype-phenotype correlation for GJB2 (connexin 26 deafness//J. Med. Genet. -2004. - Vol. 41. - P. 147-154.
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20. Risk factors and prelevance of newborn hearing loss. Rev.paul.pediatr. vol. 31, № 3, Sao Paulo Sept., 2013.
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22. Zahnert T. The differential diagnosis of hearing loss. Dtsch Arztebl int., 2011, vol.108, N 25. P. 433-444.
Amonov Aminjon Shavkatovich, attending medical doctor "Department of congenital and acquired diseases of ENT organs" Republican specialized scientific-practical medical center of Pediatrics, the Republic of Uzbekistan
Vladislav Evgenevich Kuzovkov, Professor, head of the Department of diagnosis and rehabilitation of hearing impairments, Saint-Petersburg research Institute of ear, throat, nose and speech, the Russian Federation
E-mail: amonov.aminjon@mail.ru
Cochlear implantation in children with the inner ear congenital dysgenesia — mondini anomaly
Abstract: Cochlear implantation is a complex surgical intervention in children with inner ear anomalies. In patients with congenital sensorineural hearing loss the Mondini anomaly is most often diagnosed. During surgery it was found that using of the classical access and the introduction of the active electrode through the fenestra of the cochlea is possible in most patients with Mondini anomaly.
Keywords: inner ear anomaly, Mondini anomaly, cochlear implantation, children.
Purpose
To evaluate the possibility and probable advantages and disadvantages of introducing an active electrode of cochlear implant through the fenestra of the cochlea in case of Mondini anomaly.
Materials and methods
The present study included 8 patients with inner ear malformations (Mondini anomaly) at the age of one year up to 5 years. All children underwent surgery with the diagnosis of severe congenital bilateral sensorineural hearing loss or deafness (in the RSRPMCP in 2014-2015 years).
Results
The results of the study have shown that the classical access to cochlear implantation performance is possible in most patients with Mondini anomaly (75%) and the introduction of the active
electrode in this category of patients through the fenestra of the cochlea is almost always possible (87,5%).
Cochlear implantation (CI) is the only effective treatment for patients with severe sensorineural hearing loss and deafness [6; 7]. In most cases, the surgery is carried out using the standard method with the introduction of the active electrode through the fenestra of the vestibule or through cochleostomy. Often in case of SHL congenital anomalies of inner ear can be found. Patients with the inner ear malformations are the most difficult category of patients, both from the point ofview of the surgical intervention features, and from the point ofview of oral-aural rehabilitation [1; 2; 4]. The difficulty of the surgical intervention tactics choice in these patients is caused by inner ear anatomical structures large variability and therefore, the complexity in approaches to cochlear implantation standardiz-
Cochlear implantation in children with the inner ear congenital dysgenesia — mondini anomaly
ing [3; 5; 8]. In patients with congenital sensorineural hearing loss the Mondini anomaly is mostly diagnosed, which constitute an incomplete separation of the cochlea and is associated with modiolus hypoplasia [10; 11]. This type of cochlear anomaly occurs in case of embryonic development violation on the 7th week, and is often combined with expanded aqueductus vestibuli (Table).
Till now the question of surgical approach to the CI in patients with Mondini anomaly carrying is still open, in particular, the choice of place of electrode insertion is actively discussed. This is due to the fact that cochlear implantation in individuals with this pathology associated with a high risk of postoperative complications (intraoperative liquorrhea, the risk of facial nerve damage) and unsatisfactory results receiving [1; 9]. Thus, the increase in the number of patients Table 1. - Classification of cochlea abnormalities according to
Within the frameworks of preoperative examination all patients underwent multislice computed tomography (MSCT) of the temporal bones, magnetic resonance imaging (MRI) of the inner ear, registration of short — latency auditory evoked potentials, and otomicroscopy.
The methodology of the CI carrying out in all cases was the same until the opening of the tympanic cavity and included retro-auricular S-shaped incision, the cutaneous and periosteal flaps formation, the bed for the implant preparing, extended mastoidotomy, and posterior tympanotomy. Further surgical tactics depended on a preliminary assessment of MSCT of the temporal bones and MRI of the inner ear, as well as on the intraoperative findings that determined access to the tympanic cavity and the ganglionic canal:
— presence (absence) of the fenestra cochlea;
— degree of the sigmoid sinus presentation;
— degree of the facial nerve interposition;
— probability of the intraoperative liquorrhea development.
Classic access to the tympanic cavity has been performed in 6
(75%) patients, including 5 patients (62.5%) in which during the operation the fenestra cochlea has been well visualized, and therefore the active electrode has been introduced through its membrane. In 1 (12.5%) patients the fenestra cochlea has not been visualized in-traoperatively, and that fact forced surgeons to provide the access to the ganglionic canal through the cochleostomy.
Combined access (access through the mastoidotomy and approach via the external acoustic meatus) has been carried out in 2 patients (25%) with incomplete cochlea separation of the 2nd type.
Thus, intraoperative detection of the fenestra cochlea was impossible in only 1 of 8 patients with Mondini anomaly, and that
with Mondini anomaly, as well as the insufficient knowledge of the surgical stage of cochlear implantation characteristics in this group of patients, determine the relevance of the present study.
The aim of our study was to evaluate the possibility and probable advantages and disadvantages ofintroducing of the cochlear implant active electrode through the fenestra of the cochlea in patients with Mondini anomaly.
Materials and methods
The present study 8 patients with Mondini anomaly in age from one up to 5 years, which have been operated in the Department of congenital and acquired diseases of ENT organs of the Republican specialized scientific-practical center of pediatrics from 2104 till 2105 years.
the time of fetal development violations by L. Sennaroglu [9].
forced to provide the access to the ganglionic canal through the cochleostomy. In this case the cochleostomy has been installed at the promontorium lower edge under the stapedius muscle tendon and in front of the facial nerve canal.
During 6 (75%) surgical interventions during the fenestra cochlea membrane dissection or cochleostomy installation an intraoperative liquorrhea developed. In 2 (25%) cases the expressed liquorrhea (Gusher syndrome) has been observed, in 4 (50%) patients the filling liquorrhea has been observed. Tactics of the liquorrhea elimination depends on its severity and place of fenestra cochlea dissection (the fenestra cochlea membrane or cochleostomy).
For the liquorrhea relief a test electrode has been inserted, that provided temporary reliefofthe otoliquorrhea. After the test electrode removal the active has been instantly inserted into the fenestra cochlea. Meanwhile the adequate visualization ofthe fenestra cochlea has been provided using the aspirator. For the final liquorrhea stopping a scrupulous fenestra cochlea or cochleostomy tamponade using the temporal automuscle fragment with the size 5x8 mm has been carried out.
The electrode variant has been chosen after evaluation of the fenestra cochlea depth by test electrode insertion and its measurement. In all cases the medium electrode has been used length of the electrode framework is 24 mm).
The intraoperative implant verification, which consisted of implant telemetry, the reflex from the stapedius muscle tendon registration and the neural response telemetry, has been conducted. For control of the quality of the active electrode in the ganglionic canal insertion the postoperative radiography of the temporal bones has been provided. In all cases an adequate electrode insertion has been observed.
Cochlear malformation Description
Michel anomaly (3rd week) The complete absence of cochleovestibular structures, often the internal acoustic meatus is aplastic, most often the aqueductus vestibule is normal
Cochlear aplasia (end of the 3rd week) The cochlea is absent, vestibule and canalicular system are normal, expanded or hypoplastic, the internal acoustic meatus more often is expanded, the aqueductus vestibuli more often is normal
General cavity (4th week) Cochlea and vestibule — single space without internal architecture, normal or deformed canalicular system or its absence, the internal acoustic meatus is mostly expanded, than narrowed; most often — aqueductus vestibuli is normal.
Incomplete separation, 1st type (5th week) The cochlea is represented by a single cavity without internal architecture, the vestibule is expanded, the internal acoustic meatus most often is expanded; the canclicular system is absent, expanded or normal; the aqueductus vestibuli is normal.
Cochlear hypoplasia (6th week) A strict separation of the cochlear and vestibular structures, the cochlea in the form of a bubble of small size, the absence or hypoplasia of the vestibule and the canclicular system; the internal acoustic meatus is narrowed or normal, the aqueductus vestibuli is normal.
Incomplete separation, 2nd (Mondini anomaly) (7th week) The cochlea consist of 1.5 coils, the middle and apical coils are cystouosly expanded, the size of the cochlea is close to the norm, the vestibule is slightly expanded, the canclicular system is normal, the aqueductus vestibuli is expanded.
Conclusions
1. The classic access to cochlear implantation carrying is possible in most patients with Mondini anomaly (75%).
2. The active electrode insertion in this category of patients is almost always can be carried out through the fenestra cochlea (87,5%).
3. In all cases it was possible to eliminate the liquorrhea using the tamponade with the temporal muscle fragment.
4. None of the patients developed such complications as face mimic muscles paresis, bacterial meningitis.
5. In all patient with Mondini anomaly, participating this study, the medium electrode with the length of 24 mm can be fully inserted.
References:
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3. Kuzovkov V. E., Pashchina O. A., Diab M. H., Features of surgical technique in cochlear implantation in patients with malformations of the inner ear.//Materials of the XVIII Congress of ENT specialists of Russian Federation, St. Petersburg,2011 Vol. 2. P. 62-66.
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Arifdjanov Nodir Sobirovich, Surgeon in the 2nd Department of surgery, Republican Specialized Scientific-Practical Center of Pediatrics E-mail: mbshakur@mail.ru
Peculiarities of the therapy of cicatrix stenosis of esophagus in children in remote period after burn
Abstract: The article presents the results f checking and treatment of 12 children from 5 to 10 years old in remote period after burn. On the basis of the obtained data it was determined that application of biodegradable stents SX-ELLA made of polydioxanone can replace systematic balloon dilatations in the therapy of long cicatrix stenosis of esophagus after chemical burn in children.
Keywords: cicatrix stenosis of esophagus, surgical treatment, endo prosthesis, children.
nificant difficulties [1, 4]. Treatment of chemical burns of esophagus and its cicatrix stenosis is a complex problem.
Topicality. The number of chemical burns of esophagus and cicatrix stenosis in children does not tend to decrease; that is conditioned by significant increase of application in everyday life of household chemicals containing alkali and acids, which can cause severe burns of esophagus and in rare cases burns in stomach [1, 6].
Esophageal strictures appearing after burn are still one of the most widely spread causes of dysphagia after timorous and peptic stenosis of esophagus [2, 5]. In relation with the wide development of endoscopy recently there is significant improvement of the diagnostics of esophagus and stomach burns, their differentiation according to the degree of lesion providing prognosis of the further development of the process [3]. Diagnostics of cicatrix stenosis of esophagus with the help of esophagoscopy and x-ray also has no sig-
The most spread method in the therapy of cicatrix stenosis of esophagus in children is bouginage [1]. When it is applied good and satisfactory results can be achieved in 78% of the children [5]. After reconstructive-plastic operations on esophagus 35-40% of the operated patients had various postoperative complications [1]. According to foreign references, lethality of these operations varies from 9% to 15% [6]. Besides that, in childhood functional results such as swallowing and growth are especially important. In these cases therapeutic alternative, such as esophageal stenting is necessary.
Metallic stents used at the modern time (coated or partially coated) can stay in the lumen of esophagus from 3 weeks to 13 months (in adults). In 50% cases significant technical problems
