Background: According to the World Health Organization (WHO), approximately 2,000 types of congenital anomalies affecting the human body have been identified, among which craniofacial anomalies rank between second and fourth in prevalence. Hearing impairment occurs in approximately 1.62 per 1,000 live births, while congenital malformations of the external and middle ear occur in about 1.6 per 10,000 live births. The WHO epidemiological report (2022) indicates that severe combined congenital malformations of the external and middle ear, including microtia and aural atresia, occur in approximately 2.35–7 per 10,000 live births. The prevalence of microtia is relatively higher in certain regions of Asia and South America (1 per 1,500–3,000 live births), whereas lower rates have been reported in Europe and Africa. Aim: To evaluate the feasibility and outcomes of surgical reconstruction of the auricle and the external auditory canal–tympanic cavity (meatotympanoplasty) in patients with combined congenital malformations of the external and middle ear. Materials and Methods: Audiological evaluation revealed an air conduction hearing loss of 75–80 dB. Computed tomography grading showed scores of 5–6, corresponding to grade III congenital malformation of the external and middle ear, and classified as type III according to the Nagata–Weerda classification.In cases of unilateral combined congenital external and middle ear malformations (auricular deformity with associated aural atresia), auricular reconstruction and external auditory canal formation were performed in two surgical stages with a 6-month interval. At postoperative day 21, removal of the external auditory canal stent revealed no evidence of canal stenosis, and epithelialization of the skin graft had begun. Result: Postoperative audiological assessments conducted at 30, 45, and 60 days demonstrated a statistically and clinically significant improvement in hearing thresholds, which correlated directly with the healing status of the newly reconstructed tympanic membrane. Conclusion The findings of this study suggest that in patients with combined congenital malformations of the external and middle ear, simultaneous reconstruction of the auricle and the external auditory canal–tympanic cavity in a single surgical stage may be feasible. This approach has the potential to reduce overall treatment duration, surgical burden, and psychological stress for patients. However, further studies involving larger patient cohorts and long-term follow-up are required to evaluate hearing stability and the incidence of postoperative complications, including restenosis and secondary tympanic membrane changes.

Background: Allergic and atrophic forms of chronic rhinitis are common among the population, characterized by a chronic progressive course with a tendency toward increasing prevalence. Worldwide, allergic rhinitis affects approximately 10–40% of adults and 2–25% of children. According to a study conducted in Mongolia, the prevalence of allergic rhinitis among adults in Ulaanbaatar was reported to be 23.6%, with an increasing trend anticipated in the future. In allergic and atrophic rhinitis, structural alterations occur in the nasal mucosa, including disruption of the normal architecture of the pseudostratified epithelium and ciliated cells, accompanied by changes in goblet cell number and dysfunction of their activity, resulting in a complex pathological process. In otorhinolaryngology practice in Mongolia, there is a relative lack of studies investigating the ultrastructural changes of the nasal mucosa in allergic and atrophic rhinitis. Aim: To compare ultrastructural and histological changes of the nasal mucosa in allergic and atrophic rhinitis. Materials and Methods: Ethical approval was obtained from the Research Ethics Review Committee of the Mongolian National University of Medical Sciences at the meeting held on April 24, 2020 (Protocol No. 2020/З-04). Appropriate ethical permission was also obtained from the EMJJ ENT Hospital. Nasal mucosal tissue samples were collected from surgically resected specimens of patients aged 20–45 years diagnosed with allergic and atrophic rhinitis. The mucosal specimens were processed at the Institute of Biomedical Sciences of the Mongolian National University of Medical Sciences. Samples were fixed and dehydrated according to the protocol, coated with 100 Å gold, and examined using a scanning electron microscope (FEI Quanta FEG 250, USA). In addition, paraffin blocks were prepared, sectioned at 4 μm thickness, stained with Hematoxylin and Eosin, and analyzed histologically. Results: In allergic rhinitis, the pseudostratified epithelium was relatively preserved; however, disorganization of ciliated cells, partial loss of cilia, and goblet cell hyperplasia were observed. In contrast, atrophic rhinitis showed epithelial thinning, near-complete loss of ciliated structures, glandular atrophy, and submucosal fibrosis. Conclusion Allergic rhinitis is associated with relatively mild and predominantly functional mucosal alterations, whereas atrophic rhinitis demonstrates severe structural damage with irreversible impairment of mucosal protective function.

Background: Hearing loss (HL) is one of the most common sensory disorders, affecting over 5-8% of the world's population. Approximately half of HL cases are attributed to genetic factors. In hereditary deafness, about 75-80% is inherited through autosomal recessive inheritance, and common pathogenic genes include GJB2 and SLC26A4. Pathogenic variants in the SLC26A4gene are the leading cause of hereditary hearing loss in humans, second only to the GJB2 gene. Variants in the SLC26A4gene cause hearing loss, which can be non-syndromic autosomal recessive deafness (DFNB4, OMIM #600791) associated with enlarged vestibular aqueduct (EVA) or Pendred syndrome (Pendred, OMIM #605646). DFNB4 is characterized by sensorineural hearing loss combined with EVA or less common cochlear malformation defect. Pendred syndrome is characterized by bilateral sensorineural hearing loss with EVA and an iodine defect that can lead to thyroid goiter. Currently, it is known that EVA is associated with variants in the SLC26A4 gene and is a penetrant feature of SLC26A4-related HL. Predominant mutations in these genes differ significantly across populations. For instance, predominant SLC26A4 mutations differ among populations, including p.T416P and c.1001G>A in Caucasians, p.H723R in Japanese and Koreans, and c.919-2A>G in Han Taiwanese and Han Chinese. On the other hand, there has been no study of hearing loss related to SLC26A4 gene variants among Mongolians, which is the basis of our research. Aim: We aimed to identify the characteristics of the SLC26A4 gene variants in Mongolian people with Enlarged vestibular aqueduct and Mondini malformation. Materials and Methods: In 2022-2024, We included 13 people with hearing loss and enlarged vestibular aqueduct, incomplete cochlea (1.5 turns of the cochlea with cystic apex- incomplete partition type II- Mondini malformation) were examined by CT scan of the temporal bone in our study. WES (Whole exome sequencing) analysis was performed in the Genetics genetic-laboratory of the National Taiwan University Hospital. Results: Genetic analysis revealed 26 confirmed pathogenic variants of bi-allelic SLC26A4 gene of 8 different types in 13 cases, and c.919-2A>G variant was dominant with 46% (12/26) in allele frequency, and c.2027T>A (p.L676Q) variant 19% (5/26), c.1318A>T(p.K440X) variant 11% (3/26), c.1229C>T (p.T410M) variant 8% (2/26) ) , c.716T>A (p.V239D), c.281C>T (p.T94I), c.1546dupC, and c.1975G>C (p.V659L) variants were each 4% (1/26)- revealed. Two male children, 11 years old (SLC26A4: c.919-2A>G) and 7 years old (SLC26A4: c.919-2A>G:, SLC26A4: c.2027T>A (p.L676Q))had history of born normal hearing and progressive hearing loss. Conclusions 1. 26 variants of bi-allelic SLC26A4 gene mutation were detected in Mongolian people with EVA and Mondini malformation, and c.919-2A>G was the most dominant allele variant, and rare variants such as c.1546dupC, c.716T>A (p.V239D) were detected. 2. Our study shows that whole-exome sequencing (WES) can identify gene mutations that are not detected by polymerase chain reaction (PCR) or NGS analysis.

Background: A rare angioproliferative condition of the larynx, Kaposhi sarcoma typically affects the skin. Immunosuppressive treatment following organ transplantation and human immunodeficiency virus infection are the causes. Every type of Kaposi sarcoma has human herpesvirus-8. Laryngeal kaposi sarcoma is uncommon in immunocompromised patients; since its initial identification in 1965, 18 cases have been documented globally. A CO2 laser-assisted laryngeal microsurgery is performed through the mouth cavity to remove tumor when kaposi sarcoma of the larynx obstructs the airway. Case report: A 77-year-old woman complained of hoarseness, dry mouth, odynophagia, and dysphagia three months prior when she arrived at the Mongolian-Japan Hospital. Two years ago, she acquired hard, sensitive lumps that were palpable on her right arm, left ankle, and right thigh. At that time, she was diagnosed with Kaposiform hemangiodermatitis and treated at the National Center for Dermatology. HIV test results were negative. Immunohistochemistry: CD31 +/-, CD34 /+/. Using flexible nasopharyngeal endoscopy to get the diagnosis: There was a mass that was about 1.5–2–5 cm in diameter, bluish in color, smooth and movable, and spongy and vascular on the larynx, on the nasopharynx, behind the palatine tonsills, and supraglottic. Surgery: Through the use of Kleinsasser laryngoscopy and a 0-degree endoscope, pathological tissues were extracted under general anesthesia using a laryngeal microsurgical instrument and a laparoscopic bipolar coagulator. The tissues were then sent for histological evaluation, which revealed Kaposi sarcoma, sarcoma grade 1. Results of treatment Pain decreased and quality of life increased following surgery. Upon nasopharyngeal endoscopy, the vocal cord mobility was normal and the surgical incision was clean. Conclusion: Kaposi sarcoma is an extremely uncommon illness. A lower quality of life and further issues can be avoided with an early diagnosis and suitable therapy. It also needs to be continuously monitored because it is a potentially repeatable disease.

Background: Due to the geographical location, population, and climatic characteristics of Mongolia, ear, nose, and throat diseases have not decreased to a certain extent, and non-communicable diseases, which are the main causes of morbidity and mortality, are increasing year by year in many countries. Urbanization, population density, environmental and air pollution, unhealthy lifestyles, and harsh working conditions have a significant impact on human health and increase the risk of developing non-communicable diseases. In our country, the study of ear, nose, and throat diseases among emergency workers who work continuously in all weather conditions throughout the four seasons of the year is relatively rare, which was the reason for conducting this study. Aim: Emergency services in some aimags and soums in the Central and Khangai regions have been working to identify and diagnose ear, nose, and throat diseases among employees. Materials and Methods: Using an organization-based prospective study method, clinical examinations were conducted from September 25 to October 18, 2024, among employees of the Central and Khangai regions of Orkhon, Selenge (6 soums), Tuv (4 soums), Khuvsgul (5 soums), Arkhangai (2 soums), Darkhan (1 soum), Bulgan (3 soums), 7 aimags, and 21 soums. The diagnosis of ear, nose, and throat diseases was made using ICD codes, and the data was analyzed using EXCEL software. Results: Among the employees and workers of the emergency services of the aimag, soum who participated in the survey (n=984), 139 (14%) were female and 845 (86%) were male, with an average age of 31.7 years and an average number of years of service of 10.3. The frequency of diseases classified by ICD code as a percentage of occurrence was as follows: J34.2: 211 (21%), J35.1: 96 (9.75%), J31.0: 58 (5.89%), H90: 15 (1.5%), H61.2: 14 (1.4%), H72.0: 10 (1.01%), H65.4: 6 (0.6%), J34.89: 5 (0.5%), K21.0: 5 (0.5%), H61.81: 3 (0.3%), D11.0: 2 (0.2%), 119 (12%) with comorbidities, and 426 (43.3%) diagnosed as healthy without ENT diseases. Conclusion 43.3% of the employees of the National Health Insurance Agency of some aimags and soums of the Central and Khangai regions were diagnosed as healthy or without ear, nose and throat diseases, and deviated nasal septum (J34.2), enlarged tonsils (J35.0), chronic rhinitis (J31.0), and conductive and sensorineural hearing loss (H90) were commonly found.

BACKGROUND. Sensorineural hearing impairment (SNHI) is the most common inherited sensory defect, affecting about 3 per 1000 children. More than 50% of these patients have a genetic cause (i.e. hereditary hearing impairment; HHI). Mutations in certain genes were noted to be extraordinarily popular in the deaf patients across different populations, making molecular screening feasible for these common deafness genes. One of the most important characteristics that we have learned concerning hereditary hearing loss is that common deafness genes and their mutations are usually different according to the ethnic background. As demonstrated in our previous studies performed in Taiwanese patients, the mutation spectrums of common deafness genes, such as the GJB2 gene and the SLC26A4 gene, are different from those in the Caucasian or even other Asian populations. These findings further underscore the indispensability of the collection of local data in terms of genetic counseling. In the collaborative project, we have successfully established a cohort of >100 hearingimpaired families, and clarified the genetic epidemiology of deafness in the Mongolian population. We identified several special deafness mutations such as GJB2 c.23+1G>A, c.559_604dup, and SLC26A4 c.919-2A>G, and our results revealed that Mongolian patients demonstrate a unique genetic profile in deafness as compared to other East Asian populations (paper in preparation). Meanwhile, by organizing a seminar at National Taiwan University Hospital in March 2017, we have transferred crucial concepts and techniques regarding how to perform genetic testing for deafness to the Mongolian colleagues. In the future, we plan to strengthen the mutual collaboration by expanding the clinical cohort and upgrading the genetic examination platform using the NGS techniques.

BACKGROUND. The most complications of inflammation of the middle ear temporal bone and skull brain are otitis media, suppurative otitis media, meningitis and skull brain abscess. Aim:To diagnose otitis media, to find the complication of suppurative skull brain and to study the treatment issues as well as to decrease the complication. METHOD. We treated that the patients who was treated the otitis media and complicated with suppurative skull brain between 2014-2015, also the treatment was studied by us in department of otolaryngology surgery of Shastin Central Hospital. RESULTS. In our study, we found the following results: It was a high prevalence with chronic otitis media with effusion in 60 (28.8%) people of younger age group with 25-35 years old. It was a female preponderance with 66% by prevalence. Chronic otitis media with effusion 120 (57.6%), chronic otitis media with mastoiditis 79 (37.9%), total number of patients chronic otitis media with and without mastoiditis 199 (95.5%). It shows disease are easy to throne to the complicated stage of its disease. There was 1 case (0.48%) with intracranial complication. We manage the 8 (4%) patients by antibiotic therapy, 200 (96%) patients by combined with medical and surgical therapy with average 9-12 days of hospital bed days. During 2014-2015, there was only 1 case with intracranial complication from chronic otitis media, shows our early diagnostic level of medical service are quite well. CONCLUSION. Therefore we conclude that prevention, early diagnosis are the main way to decrease the disease. The most important issue is to manage acute otitis media as early as possible before converting it chronic form.

BACKGROUND. In our country, the allergic rhinitis spreads through 23,5% of adults and asthma spreads through 20.9% of children. The allergic rhinitis causes rhinosinusitis, rhino polypus, inflammation of respiratory tract, middle ear infection, breath with mouth, insomnia or sleepless and losing of life quality. Therefore it affects badly on children’s focusing and grades. Aim: To detect the specific immunoglobulin E for 3-12 years old children with allergic rhinitis. Goals: 1. To detect the specific immunoglobulin E for 3-12 years old children with allergic rhinitis , to take a skin test from 7-12 years old children 2. to detect the specific immunoglobulin E for 20 children of control group METHODS. We did an otolaryngologic examination and took an anamnesis of “Allergic rhinitis and its impact on asthma” that from the international organization. We detected the specific immunoglobulin E with aeroallergens and 40 types of food allergens for 3-6 years old 20 children with allergic rhinitis and 20 healthy control group children in their peripheral blood by using Agnito BIO Lc, the allergic panel from Taiwan. Did skin test with positive and negative control for 7-12 years old 20 children by 40 types of diagnosing devices that from Allergopharma /from German/, Lofarma /from Italy/ firms. We produced the study results and did statistic information on SPSS 23 program. RESULTS. We did the allergic test in peripheral blood of 3-6 years old children following the standard of allergen-specific IgE and produced the results. 3 of them /19 children/were sensitive for epidermis of dog, cat and horse /15.7%/. All of them were sensitive for ground plants /100%/, 12 of them/63,1%/ were very sensitive/32+/ for mugwort, and 16 /84,2%/of them were sensitive for herbs, 14 /73,6%/ of them were sensitive for other allergens such as resin, candida. And 6 /31,5%/ of them were sensitive for mites. 8 of them were sensitive for food allergens additionally /42,1%/.12 of 17 children who had a skin test were sensitive for mugwort /70,5%/, 9 of them were sensitive for trees/aspen, hus, pinaster/ /52,9%/. There was any sensitiveness for house dust and container dust mite for 7-12 years old 17 children. It shows that there might be immunity for this age. CONCLUSION. The boys with allergic rhinitis were more than girls about 61,1%. It shows that the allergic rhinitis occurs in boys more than girls.3-6 children were sensitive /97,2%/ for ground plantsand sIgE level 7.26±5.18. 7-12 years old 20children were sensitive for herbs and mugwort more /94,1%/.

BACKGROUND. Sensorineural hearing impairment (SNHI) is the most common inherited sensory defect, affecting about 3 per 1000 children. More than 50% of these patients have a genetic cause (i.e. hereditary hearing impairment; HHI). Mutations in certain genes were noted to be extraordinarily popular in the deaf patients across different populations, making molecular screening feasible for these common deafness genes. One of the most important characteristics that we have learned concerning hereditary hearing loss is that common deafness genes and their mutations are usually different according to the ethnic background. As demonstrated in our previous studies performed in Taiwanese patients, the mutation spectrums of common deafness genes, such as the GJB2 gene and the SLC26A4 gene, are different from those in the Caucasian or even other Asian populations. These findings further underscore the indispensability of the collection of local data in terms of genetic counseling. In the collaborative project, we have successfully established a cohort of >100 hearing-impaired families, and clarified the genetic epidemiology of deafness in the Mongolian population. We identified several special deafness mutations such as GJB2 c.23+1G>A, c.559_604dup, and SLC26A4 c.919-2A>G, and our results revealed that Mongolian patients demonstrate a unique genetic profile in deafness as compared to other East Asian populations (paper in preparation). Meanwhile, by organizing a seminar at National Taiwan University Hospital in March 2017, we have transferred crucial concepts and techniques regarding how to perform genetic testing for deafness to the Mongolian colleagues. In the future, we plan to strengthen the mutual collaboration by expanding the clinical cohort and upgrading the genetic examination platform using the NGS techniques.

There are a lot of influencing factors of facial nerve palsy; experts believe that is most likely caused by a Virus (54%) and Bacterial infections. Noninfectious causes of facial nerve palsy induce tumors (28%) and less commonly influences head trauma (18%). The retrospective analysis of WHO, in 2012. There are some cases of postoperative complication in middle ear surgery is facial nerve palsy and the total recovery outcome of function was not good. From 2013 to 2016 in EMJJ hospital, Mongolia, we enrolled 16 cases with facial nerve damaged in intratympanic canal but we could not recruit some patients with facial palsy over 6 months. Each subject was tested with pure tone test, ABR, Tympanometry. These were performed for the detection of hearing loss after Temporal bone injury. Then we also investigated location of facial nerve damages of patients by MRI and CT before reconstructive surgery. After that surgery, all patients were given corticosteroid treatment (20mg/day) and physical therapy performed such as acupuncture for a week. Study results revealed that 6 cases after 18 days, 2 cases after 30 days, 1 patient after 45 days of reconstructive surgery regained good symmetry. Therefore, we considered that, postoperative treatments like physical therapy with B12, steroid had good benefits for operation result and to shorten the recovery time. There was a patient who had damaged facial nerve in the tympanic segment during Mastoidectomy. In that case, we performed cable nerve grafting using the r.auricularismagnium but we could not recover facial nerve function. Traumatic facial nerve paralysis is the second most common type. We discussed that performing reconstruction surgery within first 3 months after intratemporal facial nerve injury is extremely desirable and more effective. In our opinion, nerve recovery might be not successfully cause of injured myelin sheet of facial nerve during middle ear surgery.