ABSTRACT
@#BACKGROUND. Over 5% of the world’s population or 466 million people have disabling hearing loss (432 million adults and 34 million children). Profound sensorineural hearing loss and deafness can be treated successfully with a cochlear implant. A cochlear implant (CI) stimulates the auditory nerve, bypassing the defective cochlea, and provides auditory information to the developing brain, thus improving oral language. Children, who receive an early diagnosis of deafness and proper speech rehabilitation, and undergo early cochlear implantation, are able to develop auditory and linguistic skills at par with their hearing peers. METHOD. 6 deaf patients who underwent unilateral Cochlear Implantation at the NCMCH from January 2017 to June 2018 were included in the study. Participants were divided into 3 groups on the basis of age at which participants underwent implantation. RESULTS. Pre-op average MAIS was 1,6/40, Category of Auditory Performance (CAP) - 0,77 and SIR - 0,7/5 in all implanted age groups. Score increased over time to attain average score MAIS -29.6/40, CAP- 3.3/7 SIR-2.7/5 in all implanted groups at one year after implantation. CONCLUSION. There is a need for more research on patient age and gender composition, and outcomes of cochlear implantation and hearing devices in this study population. A robust set of study measures would provide statistical significance. Parent-child relationships affect hearing and speech therapy results, so there’s a need to improve parents’ knowledge regarding these therapies.
ABSTRACT
@#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.
ABSTRACT
@#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.
ABSTRACT
@#BACKGROUND. Hearing loss is likely to be the most common congenital abnormality in newborns, with a reported prevalence of 1 to 2 per 1000 live births. It is vitally important to diagnose infant hearing loss or deafness at its early stages. Early detection and intervention is critical to prevent the adverse consequences of a delayed diagnosis on speech, language and cognitive development. Universal screening of hearing loss has been introduced in practice with distortion product of otoacoustic emission (DPOAE). The automated auditory brainstem response (AABR) screener is a dedicated hearing screening device which provides information not only about the outer and middle ear and cochlea but also about the auditory pathway up to the brainstem. METHODS. The study was descriptive and based on a retrospective analysis of the two year period databases (2014-2016) from the newborn hearing screening program. We have started the hearing screening from December 2012. Between 2014 to 2017 we have been screened total of 11218 newborns. We tested automated audiotory brainstem response of the newborns in their 1- 3 days of birth, using Maico MB11 Beraphone machine (German) according to the Joint Committee on Hearing Screening guidence. We assessed the test result of “pass” as “normal hearing, “refer” as to rescreen and tested again after 1 month. Infants referred again were gone to a specialist for further audio logic analysis (behavioral tests, auditory brainstem response, otoacoustic emissions and auditory steady state response). RESULTS. Our study was the first in Mongolia. Total of 10290 newborns passed bilaterally out of 11218 and 1323 referred in the first screening. For the rescreen test, 1088 out of 1134 infants resulted with “pass” and 46 infants with “refer”. For those infants, we did ABR test in their 3 and 6 months, and result of 9 children (18 ears) have bilateral profound hearing loss, 2 children (2 ears) have bilateral mixed profound hearing loss and 13 (26 ears) have bilateral profound conductive hearing loss. Coverage rate was 34,7%. First refer rate was 11,7%. Follow rate was 39,7%. We conducted the cochlear implantation surgery for 2 children out of 11. Currently, we are preparing other infants with bilateral profound hearing loss of 0-3 years old for the follow up CI surgery. CONCLUSION. In Mongolia, the data of infant hearing difficulties and prevalence of deafness is lacking. Although newborn hearing screening program has been approved in 2014 and brought opportunities to conduct universal neonatal hearing screening program. But at the moment, its only available at NCMCH which located in Ulaanbaatar city. 11 cases have a bilateral profound hearing loss out of 11218 newborns. Our study result shows similiar pattern (1.1 - 1.3 ear deafness in every 1000 birth. Lenarz et all.2008). Automated audiotory brainstem response was efficient in early identification of newborn hearing loss with high sensitivity and specification rates. Small population in the remote locations, high birth percentage, short period in the hospital after birth, lack of technology and human resources, and other factors result in higher level of referral rate newborn hearing screening and lower level of follow up and confirmation rate referals in Mongolia.
ABSTRACT
@#BACKGROUND. Otitis media (OM) is any inflammation of the middle ear. It is very common in children. The purpose of Survey is to analyzing result that insertion of tympanostopmy tube treatment for children who had otitis media. METHOD. The study covers total 482 tympanostomy surgery cases conducted, between 2013-2015, at ENT Surgery Department, National Center for Child and Maternal Health. Titanium tube had been placed for the patients with either adhesive otitis media and otitis media with effusion. In order to evaluate surgery results, follow up examinations were carried out in 2 weeks, 1, 3 and 6 months using otoscopy, audiometer, ABR and ASSR test. RESULTS. Most of the children (82.4%) in whom a ventilating tube have been placed in tympanic membrane had exudative otitis and the remaining (17.6%) children had adhesive otitis. In means of age group, children with 0-2 years, 3-4 years, 5-6 years, 7 and more were, 39.9% (n=173), 42.7% (n=206), 15.6% (n=75) and 5.8% (n=28) respectively. After the tympanostomy surgery tympanic membrane color gets better, turns pearl grey, fluctuation improves, membrane cavity pressure lowers to -50daPa and hearing status ranged 20.4-5.4dB which allows the patient to continue normal speech development. CONCLUSION. Among 482 patients whom involved surgical procedure of Insertion of tympanostomy tube treatment last 3 years in Department of Pediatric Otolaryngologists in Maternal and Child Health of Mongolia and otitis media with effusion and adhesive otitis media type were occurred 30% to 40% from above cases. After tube placement tympanic membrane movement improved and color of tympanic membrane is getting pearl-grey and middle ear pressure decreasing until to 50daPa and children hearing range changed 20.4 to 5.4 db. So we could prevent these children suffer speech disorder .