Hearing Impairment In Infants Research Articles

Hearing Screening of Newborns and Infants by Otoacoustic Emission

Introduction: This study was undertaken to assess the hearing of newborns and infants using otoacoustic emission as a screening tool. Screening of hearing impairment in infants is done before the third month of life, and intervention, if any, required by the age of 6 months is of prime importance. The aim of this study was to screen 215 newborns for hearing.Materials and Methods: About 215 neonates and infants were included after a thorough ENT examination and history taking. Two stage DPOAE was done on the newborns with 2, 3, 4, and 6 kHz frequencies in both ears. Newborns who failed the first OAE in any ear were tested for a second OAE in both ears within 15 days to 3 months.Result: Out of 215 cases, 10.2% (n = 22) babies had referred results for 1st DPOAE in which low birth weight, prematurity, respiratory distress syndrome, post-natal sepsis, and maternal anemia and pregnancy-induced hypertension were among the identified infantile and maternal risk factors respectively. For these infants, repeated DPOAE screening was done. On repeat DPOAE testing, all babies passed the DPOAE 2nd test.Conclusion: This study was an attempt to show the importance of developing a hearing screen with DPOAE when repeated appropriately. The hearing of all infants should be screened at no later than one month of age; those that do not pass screening should have a comprehensive audiological evaluation at no later than three months of age, and infants with confirmed hearing loss should receive appropriate intervention by six months of age.

Risk Factors Associated With Hearing Impairment in Infants and Children: A Systematic Review.

The purpose of the present systematic review was to synthesize evidence on associated risk factors of hearing loss (HL) in children. Evidence-based research articles on HL published between January 2013 and December 2022 using PubMed, Cochrane, and Scopus databases were searched. The study included children between zero and three years of age who have permanent bilateral/unilateral HL (BHL/UHL) by employing case-control studies, randomized controlled trials, nonrandomized studies, prospective or retrospective cohort studies, and studies with or without comparison groups. The Newcastle-Ottawa Scale (NOS) and the Joanna Briggs Institute (JBI) critical appraisal checklist for longitudinal and cross-sectional studies were used to rate the quality of the chosen studies. The studies that would be considered were reviewed by two independent authors, and a third author was contacted if there was a dispute. A preliminary literature search uncovered 505 articles from the electronic search and 41 studies by hand searching. Duplicate records were eliminated, leaving 432 records. The abstract and title were read, and 340 studies were eliminated. There were 92 articles in total that qualified for full-text screening. Among these, 75 articles were disregarded since they lacked information or failed to assess the risk factors for HL. The qualitative synthesis, therefore, included 17 articles. Most often, cross-sectional study designs were used in the studies that were reviewed, which were then followed by longitudinal studies. Three of the studies that were reviewed used a prospective cohort design. The quality of all the included studies was rated to be good. The current review revealed that the primary statistically significant risk factors for HL included ventilator support; craniofacial anomalies; low birth weight (LBW); forceps delivery; loop diuretics; meningitis; asphyxia; intensive care; consanguinity; sepsis; Apgar scores between 0 and 4 at one minute; toxoplasmosis, other agents, rubella, cytomegalovirus, and herpes(TORCH) infections; and hyperbilirubinemia.

Analysis of Serum Inflammatory Markers in Infants Under 6 Months of Age with Non-Syndromic Moderate and Severe Hearing Loss Associated with GJB2 Gene Mutations.

BACKGROUND The GJB2 gene is reported to be the main hereditary factor responsible for non-syndromic hearing impairment in infants. Several kinds of hearing loss have been linked to elevated inflammatory markers. This study aimed to evaluate serum levels of IL-2, IL-4, IL-6, IL-10, IL-17, alpha-TNF, and γ-IFN and the severity of hearing loss. MATERIAL AND METHODS Ninety newborns were divided into 3 groups: severe hearing impairment (31 infants), moderate hearing impairment (30 infants), and normal hearing (29 infants). Hearing screening was performed using otoacoustic emissions test. Mutations of the GJB2 gene were detected with Sanger sequencing. The patients had DNFB1 mutation. Seven blood inflammatory markers were tested using Cytometric Bead Array. We performed the t test to examine differences in expression of 7 inflammatory markers between sexes in the groups. The correlation between indicators within groups was studied using the Pearson correlation test. Correlation of different indicators among groups was studied using the Spearman correlation test. RESULTS When compared among the 3 groups (severe, moderate hearing impairment, and normal hearing group), we found that IL-10 had a positive correlation with the severity of GJB2-associated hearing loss, which was statistically significant (P<0.05). CONCLUSIONS This research aimed to assess the relationship of 7 serum inflammatory markers with GJB2-associated hearing loss in infants. Inflammatory marker IL-10 had a positive correlation with the severity of GJB2-associated infant hearing loss, and it might have the potential to become a future therapeutic target.

Risk factors for hearing impairment in infants and toddlers in the Gaza governorates: a case-control study

BackgroundThe risk factors for congenital or early-onset hearing loss vary across countries. This study aimed to explore the risk factors for hearing impairment in children younger than 3 years in Gaza governorates to inform preventive strategies. MethodsThis case-control study started in February, 2017, and ended in July, 2018. Ethical approval was obtained from the Helsinki Committee in Palestine, a national committee established in 1986 that reviews all proposals for heath research, and whose approval is a requirement before any health research in Palestine. Data were collected through face-to-face household interviews. Cases were defined as children diagnosed with moderate, severe, or profound sensorineural hearing impairment within the first 3 years of life (n=169), whereas controls were children who had been screened and found to have unimpaired hearing (n=169). Cases and controls were randomly selected through a systematic sampling approach from the screening lists of the Atfaluna Society for Deaf Children, a registered Palestinian non-governmental organisation that has been working in the field of deaf education and allied services since 1992 and that regularly leads hearing screening campaign for neonates and children younger than 5 years in primary health centres over all Gaza governorates. SPSS version 21 was used for data entry and logistic regression analysis with the chi-square test, odds ratio, and t test. FindingsMaternal unemployment was more prevalent in cases than in controls (p=0·025). Having a family history and consanguinity were more prevalent in cases than in controls (p=0·001 for both). Taking folic acid during the preconception period (p=0·007) and iron supplements during pregnancy (p=0·002) were associated with a lower prevalence of hearing loss. The proportion of mothers of cases who were registered as having received antenatal care during the first trimester was lower for cases than for controls (odds ratio 0·341 [95% CI 0·181–0·640]; p=0·002). Prematurity (p=0·006), low birthweight (p<0·0001), and admission to a neonatal intensive care unit (p=0·002) were strongly associated with hearing impairments. The study also showed that children with recurrent otitis media were at a greater risk of hearing impairment (p<0·0001), as were children who had been exposed to sporadic loud noises, such as nearby bombing, or continuous loud noise at home, such as from nearby factories. (p=0·01). InterpretationThe study concluded that hearing impairment among children younger than 3 years is associated with a wide array of socioeconomic, health, and environmental vulnerabilities. A health promotion programme should be established to address consanguinity, prematurity prevention, and timely and appropriate preconception and antenatal care, alongside universal screening for all children, particularly those at risk as identified in this study. FundingNone.

Diagnostic ability of otoacoustic emission and automated auditory brainstem response in hearing screening of high-risk newborn

Background: Early identification of hearing impairment in infants and appropriate intervention can prevent severe psychosocial, educational, and linguistic repercussions. Infants who are not identified before 6 months of age have delays in speech and language development.Methods: This was a prospective cohort study OAE and AABR were administered to high-risk neonates discharged from NICU and the diagnostic ability to pick up hearing loss was assessed by trained audiologist. Statistical analysis was interpreted by McNemar paired Chi-square test (χ2) test. Factors which correlated with deafness were interpreted by Pearson chi-square (χ2) test.Results: Among 144 babies screened, 26 failed OAE while 6 failed AABR. Referral rate was 18.1% with OAE and 4.2% with AABR. All 6 babies failed with 40db and 90 db screening. Mean duration of NICU stay had a positive correlation with AABR positivity. Babies with higher duration of NICU stay had greater probability of hearing loss. Age and gender had no significant correlation with hearing loss. Sensitivity of OAE as 16.7 % and the specificity was 81.9 %. Positive likelihood ratio with OAE was.923. Negative likelihood ratio with OAE was 1.02. One neonate out of 6 with jaundice had profound hearing loss. Conclusions: It was concluded that in high-risk neonates the diagnostic value of DPOAE for identification of hearing loss, when used alone, was limited since OAE has higher referral rate and lower specificity compared to AABR. Babies with higher duration of NICU stay had greater probability of hearing loss.

Incidence of hearing impairment in at risk babies

Babies treated in neonatal intensive care are prone for hearing problems and with the decrease in infant mortality, babies who survive many perinatal risk factors are increasing. Deafness in 1st three years of life may impair the full development &amp; maturation of auditory system &amp; it is well known that deafness in infancy &amp; childhood interferes with normal development of speech &amp; language. To prevent this &amp; to initiate rehabilitative procedure as early in life as possible a screening method to detect auditory disabilities in newborns is of great importance. Based on this background the present study determine to evaluate to know the incidence of hearing impairment in infants at risk.This is a prospective observational study conducted in JJM Medical College, Davanagere, Karnataka. A total 940 patients attended to JJM Medical College and Hospital and diagnosed with hearing impairment according to American Joint Committee statement on infant hearing screening (JCIH) criteria. All the patients under 2 years with history of high risk factors – pre–term, low birth weight, birth asphyxia, neonatal seizures, and hyperbilirubinemia were selected for the study. Those who failed in this test underwent repeated OAE after 6 weeks, followed by brain stem evoked response audiometry (BERA) if the second OAE was negative. Out of 940 high risk cases, 350 had profound hearing loss, 83 had severe hearing loss, 125 had moderate hearing impairment, 36 had mild hearing impairment &amp;346 had normal hearing sensitivity. Out of 48 patients with normal hearing sensitivity, 53 patients were preterm, 166 had hyperbilirubinemia, 23 had neonatal convulsions, 68 birth asphyxia, 89 were of low birth weight. Out of 147 cases 31 patients had mild/moderate hearing impairment.Neonatal jaundice carries the highest risk of hearing impairment followed by birth asphyxia, neonatal convulsions and low birth weight.BERA is the tool which can confirm the normal sensitivity of hearing whenever required &amp; is very useful in early detection of hearing loss and planning rehabilitative procedures.

Hearing Impairment in Infants with Asphyxia

Hearing Impairment in Infants with Asphyxia

Hearing Impairment in Infants with Asphyxia.

In recent decades, despite significant advances in medicine and perinatal care, asphyxia remains a serious condition, leading to significant mortality and morbidity. The incidence of severe perinatal asphyxia varies from 1 to 3 per 1000 live births, in developed countries 5-10 per 1000 or more in developing countries, and is the third most common cause of neonatal death (23%) after preterm birth (28%) and severe infection (26%). Neonatal asphyxia is often accompanied by multiple organ failure, mainly involving the kidneys, brain, and heart. Asphyxia results in significant tissue hypoperfusion and reduced oxygen supply, which can cause neurological damage and damage to cochlear hair cells. Under our supervision were 35 (n=30) newborns who were in the neonatal intensive care unit of the Perinatal Center of the Khorezm region in the period from 2018 to 2019. Of these, 22 (I (main) group n=22), who were in the neonatal pathology department, had signs of perinatal CNS damage; The comparison group included 13 newborns (n=13), with Apgar scores >7 in the first minute. There is a clear correlation between the degree of hearing impairment in infants and the degree of pathology of the CNS. However, the degree of impairment of the auditory analyzer increased in parallel with the degree of asphyxia and severity (P≤0,05).

Role of Otoacoustic Emission Test in Early Diagnosis of Hearing Impairment in Infants.

Hearing impairment is most common congenital dissorder and undiagnosed hearing loss can cause disorder in speech, language with delay in social and emotional developments. Early detection of Hearing Impairment in Infants and too access the relationship between selected risk factor and hearing loss. Neonates age upto 15days randomly selected from pediatric and obstetrics and gynaecology department, born during this period were screened by OAE before their discharge from the hospital and after stabilizing high risk neonates. Informed consent of neonates parent/guardian was obtained before babies were subjected to OAE. A total of 400 neonates were screened for hearing loss by otoacoustic emission. Out of that 18 (4.5%) neonates failed the test in initial screening and were asked for follow up screening. Two neonates were lost to follow up. Out of 16 neonates, 2 (12.5%) neonate failed the test on follow up screening by otoacoustic emission and was referred for BERA. Neonates were classified as low and high risk basis of risk factors present. Out of 76 neonates having high risk of hearing loss, 16 failed the test. Similarly out of 324 neonates with low risk of hearing loss, only one neonate failed the test. Chi square analysis has revealed highly significant results (p < 0.001). Late identification of hearing loss presents a significant public health concern. However, without screening, children with hearing loss are usually not identified until 2years of age, which results in significant delays in voice communication, language communication, social, cognitive, and emotional development. In contrast, early recognition, and intervention prior to 3months of historic period has a significant positive impact on development.

"Association of High Risk Factors and Hearing Impairment in Infants-A Hospital Based Study".

The aim of the study was to find the association of various risk factors with permanent hearing impairment in infants. Acase-control study was designed on 420 infants with permanent hearing impairment and normal hearing. The case control ratio was 1:1. Alternate sampling method was used for selecting the control group. Review of medical records and parent interview was done to collect the information of risk factors. Family history(adj. OR 7.5; 95% CI 3, 14; P = 0.000), Consanguinity (adj. OR: 4; 95% CI 2,4; P = 0.000), intra uterine infection (adj. OR 18, 95% CI: 2.3-126.5, P = 0.000), post natal infection (adj. OR 3, 95% CI: 1.3-5, P = 0.004), low Apgar score (adj.OR: 4.6, 95% CI: 1.3-15), craniofacial anomaly (OR-4.6, 95% CI: 1.4-9.5, P = 0.005) and low birth weight (adj. OR: 2.3, 95% CI: 1.2-3.8) were significantly associated with hearing impairment. Among the risk factors, intra uterine infection was having highest significant association with permanent hearing impairment. This is followed by family history, low Apgar score, craniofacial anomaly, consanguinity, post natal infection and low birth weight.

Neonatal hearing screening

Neonatal hearing screening has been developped in a large number of countries. The rational to build such nationwide programs is robust. The prevalence of hearing impairment of various etiologies is high (1/1,000), diagnosis of hearing impairment in infants is uneasy and is made most of the time after the age of 18 months when treatment is less efficient and, last, appropriate test to screen for hearing impairment are available: Otoacoustic Emission and Auditory Evoked Potential. In France the screening is organised at the regional level. The organization of such a program is complexe. Midwifes and nurses should be trained to informed the parents and to perform the test. If the test is abnormal the infant will be oriented to a specialzed department of pediatrics for appropriate diagnosis and treatment.

Permanent childhood hearing impairment in infants admitted to the neonatal intensive care unit: nested case-control study.

The prevalence of permanent childhood hearing impairment (PCHI) in infants admitted to a neonatal intensive care unit (NICU) is higher than that in the general population. Our study objective was to identify risk factors associated with PCHI in infants who required admission to the NICU for more than 48 h. We performed a case-control study, including infants of all gestational ages who were admitted to NICU for more than 48 h and who underwent newborn hearing screening between 2005 and 2019. Infants admitted to NICU and diagnosed with PCHI by formal audiology were classified as "cases". The "controls" were infants who were admitted to NICU and did not have PCHI. Cases and controls (1:4) were matched based on their birth gestation, birthing place, and treating NICU. The prevalence of PCHI in infants admitted to NICU was 6.3% as compared with our general population prevalence of 0.25%. There were 77 cases and 269 controls during the study period. The median age at diagnosis of PCHI in these infants was 132 days (interquartile range 75.5-518.5). Using regression analysis, "any ventilation episodes", presence of seizures, and major congenital anomalies were significantly associated with PCHI in infants of all gestational ages. There were higher prevalence of PCHI in preterms (<32 weeks) who received furosemide and lower prevalence with antenatal use of magnesium sulphate.Conclusions: In our study, the prevalence of hearing loss was high in infants admitted to NICU. Gestation-specific risk factors identified in this case-control study would help in counselling of parents. What is Known: •In the UK, 1-2/1000 infants are born with hearing loss and infants admitted to the neonatal unit for 48 h or more have increased prevalence of hearing loss(1 in 100 live births). •Identification of risk factors in infants admitted to neonatal unit would help with risk stratification and further management. What is New: •In our study, infants admitted to the neonatal unit had higher prevalence of hearing loss (6.3 in 100 live births). •In infants across all gestational age "any ventilation episodes", presence of seizures, and severe congenital anomalies were associated with a statistically significant increase in prevalence of hearing loss. Higher prevalence of hearing loss was noted in preterm infants (<32 weeks) who received furosemide treatment and lower prevalence was noted with antenatal use of magnesium sulphate.

Hearing Impairment in Infants with Hypoxic Ischemic Encephalopathy Treated with Hypothermia.

Therapeutic hypothermia acts as the standard of care for infants with moderate to severe hypoxic ischemic encephalopathy (HIE). A proportion of neonates who undergo hypothermia due to HIE have shown to develop various degrees of hearing impairment. Analyzing and identifying infants at high risk of developing hearing difficulties is fundamental for early intervention of such auditory complications. The aim was to assess clinical factors in the development of hearing impairment following therapeutic hypothermia in HIE infants. A retrospective analysis was performed on infants hospitalized in our neonatology department in Poznan University of Medical Sciences, Poland. All infants experienced moderate to severe HIE, and were treated with therapeutic hypothermia. Risk factors for hearing impairment were identified in all infants included in the study. Clinical data during hospital stay and follow-up hearing status were analyzed. A total of 87 HIE infants were included in the study. Seventy-six infants (40 male and 36 female) had otoacoustic emission (OAE) examination following birth, of which 14 (18.4%) demonstrated abnormal (positive) results. Infants with abnormal OAE results had significantly lower blood pH (6.86 ± 0.16, p = 0.001) and base excess (BE) (-22.46 ± 2.59, p = 0.006). Of the 49 infants who returned for follow-up assessment, 4 (8.2%) were diagnosed with sensorineural bilateral hearing impairment (1 infant, mild [<40 dB], 2 moderate [41-70 dB], and 1 profound [>90 dB]). The biochemical analysis following birth revealed significantly lower umbilical BE levels (-23.90 ± 4.99, p = 0.041) and higher lactate levels (160.67 ± 4.93, p = 0.019) in the infants with eventual sensorineural hearing deficit. Infants with moderate or severe HIE are at risk of delayed onset hearing loss. Diligent efforts to monitor auditory status are required, even if early screening results for hearing are insignificant. Exploring biochemical parameters, such as lactate, BE, and blood pH, can prove beneficial in identifying HIE infants at risk of developing a hearing impairment.

Knowledge and attitude of nurses towards infant hearing impairment in North India

As research indicates that nurses are one of the core medical professionals who play a key role in the ICU as they can identify high risk babies who need audiological intervention, provided they should have adequate knowledge available in relation to different facts about pediatric hearing loss. To provide appropriate information to the nurses, audiologists should have basic layout regarding the status of the nurses in respect to the knowledge and attitude-related pediatric hearing loss and significant audiological management. The present research is being conducted to assess the knowledge and attitude of issues in pediatric hearing loss among nurses across various range of hospital set up in northern India. A questionnaire-based study (online survey) of 22 questions was designed and adopted from similar studies to assess the knowledge and attitude among nurses regarding hearing impairment among pediatric population. A total of 115 nurses from primary nursing care, corporate and government hospitals participated in the present study. The outcome of the present study revealed poor knowledge and attitude towards hearing impairment of infants among nurses across north India. Henceforth, there is an urgent need to educate these professionals as prevention is better than cure. Keywords: Newborn hearing screening, Nurses, India.

English

BACKGROUND Hearing is one of the most important senses which constitutes the basis for acquiring language, communication, cognitive and psychosocial development. Hearing impairment in infants should be recognized as soon as possible after birth for early interventions. Hearing status of a newborn in this study is being assessed by two subsequent tests with Oto-Acoustic Emissions (OAE) followed by Brain stem Evoked Response Audiometry (BERA). We wanted to study the efficacy and role of two stage Otoacoustic Emission (OAE) test for screening high risk new-borns to detect hearing impairment. METHODS A prospective observational study was conducted from July 2014 to July 2017 at Govt. Medical College, Thrissur, Kerala. OAE screening was done in two stages for 500 high risk infants admitted in newborn intensive care unit (NBICU) during the study period. First OAE test was done on the day of admission and 2nd test was done after one month. All the infants underwent Brainstem Evoked Response Audiometry (BERA) test, a month after the second OAE test. Data was analysed for efficacy of the tests. RESULTS First OAE was passed by 290 babies i.e. 58 % whereas 210 babies (42 %) showed a result of ‘refer’ in both the ears. Second OAE tests were done after 1 month which showed a result of ‘pass’ by 460 babies (92 %) and a ’refer’ by 40 babies (8 %). All babies which underwent BERA 1 month after second OAE were included in the study; out of those babies, 10 babies showed impaired hearing and they were referred for further evaluation and intervention. In our study, OAE was 100 % sensitive in the first and second tests. Specificity of OAE was 59.1 % and 93.87 % in the first and second tests respectively. The study showed 2 % permanent congenital hearing impairment (PCHI). CONCLUSIONS OAE is an effective tool providing a quick, harmless and less expensive method for screening of hearing loss in infants, irrespective of comorbidities. No single test can detect all defects in the auditory pathway. As a primary option, a two-stage evaluation with OAE can easily detect infants who need further evaluation and early intervention. A two-stage screening with OAE will give a highly sensitive and reasonably specific test which can be easily implemented in all levels of the healthcare system. KEYWORDS High Risk Neonates, Hearing Impairment, OAE, BERA

Newborn hearing screening coverage and detection rates of hearing impairment across China from 2008-2016

BackgroundNewborn hearing screening (NHS) can reduce the economic and social burden of hearing impairment. To track the progress of the goal set by the National Program of Action for Child Development (NPACD) and to estimate the detection rates of hearing impairment, the present study examined NHS coverage in 2008–2010 and 2016 and the detection of hearing impairment across China in 2016.MethodsLicensed medical institutions across China were surveyed in 2012 and 2018 by the National Center for Birth Defects Monitoring of China to collect data for the 2008–2010 period and for 2016 on live births, initial screening rates (total and referral), secondary screening rates (total and referral), and rates of hearing impairment diagnosis among infants who were referred in the secondary screening. To calculate universal newborn hearing screening (UNHS) coverage, the number of newborns who received NHS within 4 weeks after birth was divided by the number of live births. The detection rate of hearing impairment was calculated by combining referral rates on primary and secondary screening with the rate of diagnosis.ResultsNational UNHS coverage increased from 29.9% in 2008 to 86.5% in 2016, with different regions showing different increases. During this period, the number of provinces with UNHS coverage over 90.0% increased from 2 to 17, with UNHS coverage in 2016 being substantially higher in eastern provinces (93.1%) than in western provinces (79.4%). In 2016, the detection rate of hearing impairment across the country was 0.23% (95% CI 0.15–0.25%), and it varied from 0.17% in western provinces to 0.22% in central provinces and 0.28% in eastern provinces. The lowest rate was 0.02% in Heilongjiang Province and the highest rate was 0.63% in Hainan Province.ConclusionsNational UNHS coverage increased substantially from 2008 to 2016, although provinces and regions still showed differences. The detection rate of infant hearing impairment in China is comparable to that in other countries. A national individual-level information system is urgently needed in China to facilitate the integration of screening, diagnosis and treatment of infant hearing impairment, which may also lead to a more accurate estimate of the detection rate.

Hearing assessment and follow-up study of aeonatal deafness gene screening homozygous mutation infants

Objective:To analyze the hearing assessment characteristics and follow-up of some deafness gene screening homozygous infants in Zhuhai. Method:The clinical data of 28 newborns with homozygous mutations transferred to Zhuhai Maternal and Child Health Hospital from Feb. 1, 2015 to Oct. 25, 2018 in hospitals of Zhuhai City were retrospectively analyzed. All the children were screened for hearing. The hearing characteristics and long-term follow-up results of homozygous mutations at different gene sites were analyzed by auditory diagnosis and behavioral follow-up from 1 to 3 years. Result:Fourteen cases of GJB2 c.109G>A with a homozygous mutation, 11 cases passed the hearing screening, the audiological diagnosis was normal, and the behavior test and follow-up were normal from 1 to 3 years. Hearing screening was not passed in 3 newborns, mild to moderate abnormalities of single or bilateral ears were diagnosed by audiology, 1 000 Hz without positive, and middle ear lesions were diagnosed. Eight cases of GJB2 c.235del C homozygous mutation were followed up by behavioral audiometry and follow-up from 1 to 3 years after cure. Among them, 5 cases were diagnosed as severe hearing impairment of bilateral ears and 3 cases as mild and moderate hearing impairment. One case of GJB3 547G>A homozygous mutation was followed up for 1-3 years, and all of them failed to pass the follow-up of behavioral audiometry and follow-up. Four cases of SLC26A4 IVS7-2A>G, 1 case of SLC26A4 1229C>T homozygous mutation, all of them failed to pass the neonatal hearing screening. All the patients were diagnosed as severe hearing impairment of binaural hearing, and the follow-up of 1-3 years' follow-up did not pass the follow-up tests. Conclusion:GJB2 C.235del C, SLC26A4 IVS7-2A>G locus homozygous mutation infant hearing impairment was mainly severe hearing impairment in bilateral ears, and there was no change in 1-3 years follow-up. GJB2 C.109G A homozygous mutant infants had normal hearing, and it was suggested that they should be followed up closely. It is very important to give correct and reasonable genetic counseling to parents with GJB2 C.109G A homozygous mutation without unnecessary panic.

Hearing impairment in premature newborns-Analysis based on the national hearing screening database in Poland.

ObjectivesThe incidence of sensorineural hearing loss is between 1 and 3 per 1000 in healthy neonates and 2–4 per 100 in high-risk infants. The national universal neonatal hearing screening carried out in Poland since 2002 enables selection of infants with suspicion and/or risk factors of hearing loss. In this study, we assessed the incidence and risk factors of hearing impairment in infants ≤33 weeks’ gestational age (wga).MethodsWe analyzed the database of the Polish Universal Newborns Hearing Screening Program from 2010 to 2013. The study group involved 11438 infants born before 33 wga, the control group—1487730 infants. Screening was performed by means of transient evoked otoacoustic emissions. The risk factors of hearing loss were recorded. Infants who failed the screening test and/or had risk factors were referred for further audiological evaluation.ResultsHearing deficit was diagnosed in 11% of infants ≤25 wga, 5% at 26–27 wga, 3.46% at 28 wga and 2–3% at 29–32 wga. In the control group the incidence of hearing deficit was 0.2% (2.87% with risk factors). The most important risk factors were craniofacial malformations, very low birth weight, low Apgar score and mechanical ventilation. Hearing screening was positive in 22.42% newborns ≤28 wga and 10% at 29–32 wga and in the control group.ConclusionsHearing impairment is a severe consequence of prematurity. Its prevalence is inversely related to the maturity of the baby. Premature infants have many concomitant risk factors which influence the occurrence of hearing deficit.

About prediction of hearing impairment in infants with extremely low birth weight

В статье представлены результаты изучения интра- и постнатальных факторов, детерминирующих нарушение слуха в периоде новорожденности и к 3-х месячному возрасту у детей, рожденных преждевременно с перинатальной патологией. Установлена их диагностическая и прогностическая информативность для разработки эффективного прогностического алгоритма в системе катамнестического наблюдения. Доказана высокая прогностическая информативность данных первичного аудиологического обследования у недоношенных, родившихся с экстремально низкой массой тела

Prevalence of hearing impairment in children at risk

Hearing impairment is prevalent in the general population; early intervention facilitates proper development.AimTo establish the prevalence of hearing impairment in infants at risk, born between June 2006 and July 2008, and to correlate the variables with hearing loss.Type of studydescriptive and cross-sectional.Materials and Methods188 newborns were evaluated using evoked otoacoustic emissions and distortion product and auditory behavior. Tests were repeated if the results were altered. If altered results persisted, the child was referred for impedance testing and, when necessary, for medical evaluation. Infants with normal conduction were referred for brainstem auditory evoked potential testing.ResultsOf 188 children two (1.1%) were excluded, and 174 (92.6%) had results within normal limits. Hearing impairment was found in 12 children (6.3%); hearing loss was retrocochlear in three infants (25%). Unilateral hearing loss was present in two infants (16.7%); bilateral hearing loss was present in 10 infants (83.3%).ConclusionThe high prevalence of hearing impairment in this population underlines the importance of early audiological testing.