Risk factors for sensorineural hearing loss in survivors with severe congenital diaphragmatic hernia

Recent improvements in perinatal management have improved the prognosis in patients with severe congenital diaphragmatic hernia (CDH). However, in surviving patients with severe CDH, hearing loss has sometimes been reported to occur during the follow-up period. Although some of the risk factors for developing sensorineural hearing loss (SNHL) have been reported in CDH, no definitive risk factors have yet been reported. We, therefore, investigated the risk factors regarding postnatal management in patients with severe CDH. In 16 surviving patients with severe CDH, which had all been detected antenatally, and whose lung-to-thoracic ratio was less than 0.2, four patients demonstrated late onset SNHL, which occurred between 1.5 and 5 years of age. The risk factors for SNHL regarding the postnatal treatment for CDH were analyzed between the four patients with SNHL and the remaining 12 patients without SNHL, regarding such factors as the use of ototoxic drugs, neuromuscular blocking agents, high-frequency oscillation (HFO), and inhaled nitric oxide, the duration of hypocapnia, hypoxia, severe acidosis, severe alkalosis, and mechanical ventilation. In addition, the types of neuromuscular blocking agents were also analyzed, including the administration of pancuronium bromide (PB) and vecuronium bromide (VB). The patients with SNHL were found to have a significantly higher risk than the patients without SNHL regarding the duration of loop diuretics usage and the duration of usage of both mechanical ventilation and HFO. Furthermore, all four patients with SNHL used PB. In contrast, none of the five patients using VB developed SNHL The duration and cumulative dose of PB used in the patients with severe CDH showed a significant correlation to the occurrence of SNHL. Although this study was retrospective, based on our data, the prolonged use of PB, in addition to the duration of treatment by loop diuretics, mechanical ventilation, and HFO usage, might, thus, be suggested to be a possible risk factor for late onset SNHL in patients with severe CDH.     

Ligurian experience on neonatal hearing screening: clinical and epidemiological aspects

AIM: Early identification and rehabilitation of newborns with congenital hearing impairment (HI) by Universal Neonatal Hearing Screening (UNHS). METHODS: The neonatal population was divided into two groups: babies with No Audiological Risk (NAR), and babies With Audiological Risk (WAR). NAR neonates underwent OAE testing, and in case of a doubtful (Refer) result, ABR testing was carried out. All WAR newborns underwent ABR testing within the third month of life. RESULTS: Between February 1, 2002 and December 31, 2004, UNHS was carried out on 32 502 newborns at the 13 regional birth centres, representing 98.7% of the whole regional neonatal population. The prevalence of HI in the population we tested was estimated at about 1 per thousand, while Bilateral Hearing Impairment (BHI) was estimated at 0.65 per thousand. A 3.7% prevalence of HI and a 2.8% prevalence of BHI was observed among the WAR population. Median age at the end of the diagnostic procedures was 6.7 months in the WAR population and 6.9 months in the NAR population. CONCLUSIONS: Our project is based on two levels of testing, which resulted in a 0.28% false-positive rate with 100% sensitivity and 99% specificity. Our screening is the first Italian experience that has been extended to a whole region and the results prove that regional neonatal hearing screening is feasible.     

A practical screening model for hearing loss in Iranian school-aged children

Background  Hearing loss is a common and considerable disability that harms educational performance of school children in developing countries like Iran. Lack of a simple and practical screening protocol often deters routine and systematic hearing screening at school entry. Methods  This study was to establish a practical screening model for hearing loss in school-aged children based on a community-based, retrospective casecontrol study in Ilam, the capital of Ilam province in Iran. Results from the audiologic and non-audiologic examination of 785 children in primary schools were selected and examined. The non-audiologic evaluation consisted of medical history, general physical examination, while the audiologic assessment consisted of otoscopy, audiometry and tympanometry. Results  Univariate analysis of non-audiologic variables showed an association between hearing loss and male gender (P<0.05) and the grade of study (P<0.05). The frequency of impaired hearing in the first grade of primary schools was significantly higher than the children in the second grade (P<0.05). In audiologic factors related to impaired hearing, otitis media with effusion (OME) was diagnosed significantly (P<0.05). Conclusion  Routine screening based on the identification of OME will facilitate the detection of a major amount of hearing impaired school-aged children.     

Prevalence and independent risk factors for hearing loss in NICU infants

AIM: To determine the prevalence and independent relationship between hearing loss and risk factors in a representative neonatal intensive care unit (NICU) population. METHODS: Automated auditory brainstem response (AABR) hearing screening has been introduced since 1998 in the Dutch NICUs. After a second AABR failure, diagnostic ABR was used to establish diagnosis of hearing loss. Newborns who died before the age of 3 months were excluded. In the present study only the NICU infants who were born with a gestational age <30 weeks and/or a birth weight <1000 g between October 1, 1998 and January 1, 2002 were included. Risk factors included in the study were familial hearing loss, in utero infections, craniofacial anomalies, birth weight <1500 g, hyperbilirubinemia, ototoxic medications, cerebral complications, severe birth asphyxia, assisted ventilation > or =5 days and syndromes. RESULTS: A nationwide cohort of 2186 newborns were included. Mean gestational age was 28.5 weeks (SD 1.6) and mean birth weight was 1039 g (SD 256). Prevalence of uni- or bilateral hearing loss was 3.2% (71/2186; 95% CI 2.6-4.1). Multivariate analysis revealed that the only independent risk factors for hearing loss were severe birth asphyxia (OR 1.7; 95% CI 1.0-2.7) and assisted ventilation > or =5 days (OR 3.6; 95% CI 2.1-6.0). CONCLUSION: The prevalence of hearing loss in a representative NICU population was 3.2%. Independent risk factors for hearing loss were severe birth asphyxia and assisted ventilation > or =5 days.     

Implementation and results of bedside hearing screening with automated auditory brainstem response in the neonatal intensive care unit

Aim: To evaluate implementation and results of neonatal hearing screening with automated auditory brainstem response (AABR) by bedside nurses in a single‐centre neonatal intensive care unit (NICU). Methods: Retrospective review of charts of 2074 newborns admitted over a 4‐year period. Results: One thousand eight hundred and 24 newborns (88%) were screened. A ‘pass’ result was obtained in 1761 patients (96.5%). From 63 infants with ‘refer’, 40 were tested with auditory brainstem response: in 28 hearing loss was confirmed. Three hundred and nine neonates were screened before postmenstrual age (PMA) of 34 weeks: 78% successfully passed the first test. Sixty‐seven infants with ‘refer’ at the first test before PMA of 34 weeks were re‐evaluated: 48 had normal hearing tests, 24 of whom still younger than 34 weeks. For 12 of 19 infants with ‘refer’ before 34 weeks, follow‐up was available: in 7 hearing loss was confirmed. Conclusion: Neonatal hearing screening with AABR can be easily performed by the bedside nurse in the NICU even in premature babies before 34 weeks PMA. A ‘pass’ result can be obtained in almost 80% of them; a ‘refer’ result at that age, however, must be interpreted cautiously, as false ‘refer’ occurred in 5/12 of these infants.     

Congenital cytomegalovirus infection - a common cause of hearing loss of unknown aetiology

Aim: The aim of this study was to investigate the role of congenital cytomegalovirus (CMV) infection as a cause of various types of sensorineural hearing loss (SNHL) in a group of nonsyndromic children with otherwise unknown aetiology of hearing loss. Furthermore, the occurrence of combined congenital CMV infection and connexin 26 (Cx26) mutations was investigated. Methods: The dried blood spot (DBS) cards of 45 children with various degrees of hearing deficits and 46 children with severe/profound hearing loss were tested for CMV DNA with polymerase chain reaction (PCR) technique. The DBS cards of the 46 children with severe/profound hearing loss were also analysed for Cx26 mutations. Results: Of the 45 children with various degrees of hearing loss, nine were positive for CMV DNA (20%). The nine children represented severe/profound, mild and unilateral hearing loss. From the 46 children with severe/profound hearing loss, nine of 46 (20%) were positive for CMV DNA. In addition, three of the CMV DNA‐positive children were carriers of mutations of Cx26. Conclusion: Congenital CMV infection is a high risk factor in hearing impairment among children.     

先天性症状性巨细胞病毒感染脑脊液病毒载量与听力损伤相关性

目的 探讨症状性先天性巨细胞病毒(cytomegalovirus,CMV)感染新生儿脑脊液(cerebrospinal fluid,CSF) CMV DNA载量与感音性听力损伤(sensorineural hearing loss,SNHL)的相关性.方法 36例先天性症状性CMV感染患儿,PCR法检测CSF CMV DNA载量,并于出生1个月内、生后6个月及1年左右行脑干听觉诱发电位检测.结果 (1)36例患儿,其中CSF CMV DNA阳性15例,阳性率41.2％,SNHL17例,SNHL发生率47.2％.(2) CSF CMV DNA阳性组SNHL发生率60.0％(9/15);阴性组SNHL发生率38.1％ (8/21),两组发生率比较差异无统计学意义(P =0.194).(3)CSFCMVDNA阳性组中,SNHL组与听力正常组CSF CMV DNA载量为3.35 ±0.68和3.17±0.56,两组载量比较,差异无统计学意义(P=0.36).结论 先天性症状性CMV感染患儿CSF CMV DNA是否阳性及其载量不是预测SNHL的指标.     

NICU‐only versus universal screening for newborn hearing loss: Population audit

Aim: Targeted newborn hearing screening for infants in neonatal intensive care units (NICUs) may be considered when resources preclude universal newborn hearing screening (UNHS). However, process outcomes have not been compared between stand‐alone NICU hearing screening programs and NICU screening within a full UNHS program. Methods: Comparison of two consecutive hearing screening programs delivered under similar conditions in the four NICUs in Victoria, Australia. All NICU infants were eligible for pre‐discharge automated auditory brainstem response (AABR) hearing screening. Capture, referral and diagnostic data were collected for all NICU infants during the NICU‐only (April 2003–February 2005) and subsequent UNHS (April 2005–June 2006) programs. Results: 4704 eligible infants were admitted during the 23‐month NICU‐only period, and 3160 during the 15‐month UNHS period. Double AABR using ALGO 3i equipment was planned for both programs but, due to clinician concern about this high‐risk clinical population, the NICU‐only protocol was amended to single AABR using AccuScreen equipment. Capture rates were 71.1% (NICU‐only) vs. 95.4% (UNHS) (P < 0.001), successful follow‐up rates were 85.8% vs. 96% (P= 0.004), and mean corrected age at the first audiology appointment was 51.5 vs. 40.2 days (P= 0.05). Conclusions: NICU screening offered within a larger UNHS program outperformed the stand‐alone NICU hearing screening program on all measured parameters. Greater resourcing might address shortcomings of the stand‐alone program but would also reduce its potential savings. The high loss to follow‐up also argues against the often‐advocated approach of referring all NICU infants for diagnostic audiologic testing, bypassing hearing screening altogether.     

基层地区新生儿医院内两步听力筛查模式探讨

目的 探讨基层地区医院内两步听力筛查模式的有效性.方法 选择2008年11月至2010年4月出生于安阳市妇幼保健院、并接受瞬态诱发性耳声发射筛查的新生儿,医院内第一次初筛单耳或双耳未通过的新生儿于出院前进行第二次初筛或生后42天进行复筛.记录听力筛查时间及双耳筛查结果,按完成听力筛查的时间将新生儿分组,分析通过率与筛查时间的关系、医院内一次初筛和两次初筛后总的通过率,以及医院内第二次初筛与42天复筛两组人群的通过率.结果 (1)完成听力第一次初筛的新生儿共10 060例,总体通过率49.1％ (4944/10 060),通过率随出生后筛查时间的延迟而提高.(2)未通过第一次初筛的新生儿中30.0％ (1535/5116)出院前接受第二次初筛,通过率78.4％(1204/1535),两次初筛后总体通过率61.1％ (6148/10 060),与一次初筛通过率(49.1％)差异有统计学意义(x2=291.3,P＜0.001).(3)未通过医院内听力初筛的婴儿中39.1％ (1531/3912)于42天接受复筛,通过率92.7％(1420/1531),与医院内第二次初筛通过率(78.4％)差异有统计学意义(x2=127.3,P＜0.001).结论 新生儿听力筛查时间是影响通过率的关键因素,随着出生后筛查时间的延迟,初筛通过率逐步提高;医院内两步筛查可提高初筛通过率,但医院内第二次初筛通过率低于42天复筛通过率.     

Progressive high frequency hearing loss: An additional feature in the syndrome of congenital adrenal hypoplasia and gonadotrophin deficiency

In an earlier report, we found that X-linked congenital adrenal hypoplasia may be associated with gonadotrophin deficiency. This combination has since been confirmed by many others. At the last examination, our patients were 22.4, 19.9 and 17.5 years old. They were doing well on replacement therapy with hydrocortisone, fluorohydrocortisone, and long-acting testosterone, but in all of them, a progressive hearing loss had appeared, starting at high freqencies at about 14 years of age. The loss progressed with age to lower frequencies, and the oldest patient had some remaining hearing capacity at 125–500 Hz only with a perceptive hearing loss of –95 dB at frequencies above 500 Hz. It is concluded that patients with this syndrome should be examined for hearing loss. X-linked adrenal hypoplasia may also be associated with glycerol kinase deficiency and myopathy. A molecular XP-deletion has suggested a locus for hypogonadotrophic hypogonadism distal to the glycerol kinase and adrenal hypoplasia loci. The observations in our patients suggest that the locus for at least this type of X-linked deafness may be in the same area.     

Profound deafness and the acquisition of spoken language in children

Profound congenital sensorineural hearing loss (SNHL) is not so infrequent, affecting 1 to 2 of every 1000 newborns in western countries. Nevertheless, universal hearing screening programs have not been widely applied, although such programs are already established for metabolic diseases. The acquisition of spoken language is a time-dependent process, and some form linguistic input should be present before the first 6 mo of life for a child to become linguistically competent. Therefore, profoundly deaf children should be detected early, and referred timely for the process of auditory rehabilitation to be initiated. Hearing assessment methods should reflect the behavioural audiogram in an accurate manner. Additional disabilities also need to be taken into account. Profound congenital SNHL is managed by a multidisciplinary team. Affected infants should be bilaterally fitted with hearing aids, no later than 3 mo after birth. They should be monitored until the first year of age. If they are not progressing linguistically, cochlear implantation can be considered after thorough preoperative assessment. Prelingually deaf children develop significant speech perception and production abilities, and speech intelligibility over time, following cochlear implantation. Age at intervention and oral communication, are the most important determinants of outcomes. Realistic parental expectations are also essential. Cochlear implant programs deserve the strong support of community members, professional bodies, and political authorities in order to be successful, and maximize the future earnings of pediatric cochlear implantation for human societies.     

新生儿重症监护病房新生儿耳聋基因致病变异携带者筛查的横断面调查

背景：我国耳聋发病率高与耳聋基因致病变异的携带率高有关,目前缺乏对NICU新生儿耳聋基因致病变异携带者的筛查数据。 目的：调查NICU新生儿中耳聋基因GJB2 和SLC26A4致病变异的携带率。 设计：横断面研究。 方法：纳入2016年1月至2021年12月在复旦大学附属儿科医院NICU住院、入院日龄≤28 d,且出院前完成高通量测序的新生儿,排除生后耳聋相关基因诊断阳性者。从病历系统中截取患儿的性别、胎龄、出生体重;从测序数据库中提取GJB2 基因和SLC26A4基因的检测结果、患儿人类表型标准用语信息。携带率（%）=杂合致病或可能致病（P/LP）变异例数/总研究对象人数。检索PubMed、Embase和万方数据库,纳入既往报道中国NICU人群、新生儿人群和孕妇人群中GJB2 基因和/或SLC26A4基因P/LP变异携带情况的文献,并行复习。 主要结局指标：GJB2 基因和SLC26A4基因的P/LP变异携带率。 结果：纳入14 924例新生儿,男8 587例（57.5%）,女6 337例,胎龄（35.6±3.7）周,出生体重（2 711.7±887.1）g。携带GJB2 基因P/LP变异的患儿2 009例（13.462%）,共检出18种杂合P/LP变异,其中c.109G>A最常见（10.902%）,其次为c.235del（1.749%）、c.299_300del（0.409%）、c.176_191del（0.154%）、c.508_511dup（0.074%）和c.257C>G（0.034%）。携带SLC26A4基因P/LP变异的患儿305例（2.044%）,共检出31种杂合P/LP变异,携带率最高的6种依次为c.919-2A>G（1.139%）、c.2168A>G（0.181%）、c.1226G>A（0.100%）、c.1229C>T（0.094%）、c.1174A>T（0.080%）和c.1003T>C（0.047%）。 结论：建议将GJB2 基因上的c.109G>A、c.508_511dup和c.257C>G以及SLC26A4基因的c.1003T>C位点纳入NICU新生儿耳聋基因致病变异携带者筛查。     

Communication,academic and social outcomes of childhood cancer survivors with hearing loss: A systematic review

Many children treated for cancer are at risk of hearing loss. However, little is known about how hearing loss impacts their communication, academic and social outcomes. To examine the impact, this systematic review aimed to synthesise and appraise quantitative and qualitative studies reporting on (i) participants with hearing loss treated with platinum-based chemotherapy or cranial radiotherapy during childhood; and (ii) speech, language, academic performance, or social participation findings. Systematic database searches yielded 23 relevant articles that were analysed using narrative synthesis. Difficulties were reported for some but not all communication, academic and social aspects; however, a quality assessment using Grading of Recommendations Assessment, Development and Evaluation (GRADE) revealed low to very low certainty in the findings. Future research should aim to increase the quality of the research evidence and explore how multidisciplinary services can provide evidence-based support for childhood cancer survivors with competing hearing, communication, and social difficulties.     

Post-natal hearing loss in universal neonatal hearing screening communities: current limitations and future directions

Abstract:   Universal hearing screening has dramatically improved outcomes for babies born with detectable hearing abnormalities; yet there are some infants who develop significant hearing problems after passing a neonatal screen. There is much conjecture as to the number and the characteristics of infants with post-natal hearing losses; yet evidence suggests that many children may be affected, and that a large proportion have no discoverable cause. Currently, screening programmes use lists of risk factors to enrol babies into surveillance programmes. This practice is problematic because audiological follow-ups are expensive and under-utilised, and parental disclosure is often inaccurate. The large databases from universal neonatal programmes could inform the development of effective, evidence-based practice and policy for the detection and intervention of children who develop post-natal hearing losses.     

Feasibility of neonatal hearing screening program with two-stage transient otoacoustic emissions in Turkey

BACKGROUND: The objective of this study was to investigate the incidence of hearing loss in neonates and evaluate the feasibility of a two-stage Transient Evoked Otoacoustic Emission (TEOAE) screening test. Maternal concerns about hearing screening were also studied. METHODS: Neonatal intensive care patients and well babies were screened using a two-stage TEOAE test, which was followed by an Auditory Brainstem Response (ABR) test for those babies who failed the first test twice. RESULTS: In total, 711 neonates were screened. At the end of the two TEOAE tests, the cumulative pass rate was 99.3% and false-positive rate was 0.3%. Five neonates (0.7%) were referred for the ABR test. Sensorineural hearing loss was found in three of them (0.4%). Of these three neonates, one was from the well baby nursery and two were from the NICU population. Families generally welcomed the screening program, with no refusals. Positive test results have not caused important maternal concerns. CONCLUSIONS: Congenital hearing impairment is a prevalent disease in Turkey. The two-stage TEOAE program is suitable for the neonatal hearing screening program. In general, hearing screening tests do not cause notable maternal concerns.