Article

Recommendations for monitoring hearing in children using a risk factor registry

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  • Queensalnd Health
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Purpose The current Joint Committee on Infant Hearing guidelines recommend that infants with syndromes or craniofacial abnormalities (CFAs) who pass the universal newborn hearing screening (UNHS) undergo audiological assessment by 9 months of age. However, emerging research suggests that children with these risk factors are at increased risk of early hearing loss despite passing UNHS. To establish whether earlier diagnostic audiological assessment is warranted for all infants with a syndrome or CFA, regardless of screening outcome, this study compared audiological outcomes of those who passed UNHS and those who referred. Method A retrospective analysis was performed on infants with a syndrome or CFA born between July 1, 2012, and June 30, 2017 who participated in Queensland, Australia's state-wide UNHS program. Results Permanent childhood hearing loss (PCHL) yield was higher among infants who referred on newborn hearing screening (51.20%) than in those who passed. Nonetheless, 27.47% of infants who passed were subsequently diagnosed with hearing loss (4.45% PCHL, 23.02% transient conductive), but PCHL was generally milder in this cohort. After microtia/atresia, the most common PCHL etiologies were Trisomy 21, other syndromes, and cleft palate. Of the other syndromes, Pierre Robin sequence featured prominently among infants who passed the hearing screen and were subsequently diagnosed with PCHL, whereas there was a broader mix of other syndromes that caused PCHL in infants who referred on screening. Conclusion Children identified with a syndrome or CFA benefit from early diagnostic audiological assessment, regardless of their newborn hearing screening outcome.
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Background Congenital permanent childhood hearing impairment (PCHI) impairs communication skills and, possibly, mental health and employment prospects. Management within 1 year of birth can alleviate most of its adverse effects. Neonatal screening for this disorder is feasible but its benefit for all babies is disputed. We investigated whether neonatal screening of all babies born in hospital, in addition to the standard health visitor distraction test, would increase the rates of early referral, confirmation, and management. Methods Between 1993 and 1996, two teams of four part-time testers and equipment moved between two pairs of hospitals to achieve four periods with neonatal screening and four without neonatal screening, each of 4-6 months' duration. Babies did or did not undergo neonatal screening dependent on during which periods they were born. We used a transient evoked otoacoustic emissions test and, in babies who failed this test, an automated auditory brainstem response test on the same day. We referred babies with positive results for audiological assessment. Findings 53 781 babies were included in the trial, and 25 609 were born during periods with neonatal screening. Neonatal screening achieved 87% coverage of births, with a false-alarm rate of 1.5%, and an overall yield of 90 cases of bilateral PCHI of 40 dB or more relative to hearing threshold level per 100 000 target population, equivalent to 80% of the expected prevalence of the disorder in the population. 71 more babies with moderate or severe PCHI per 100 000 target population were referred before age 6 months during periods with neonatal screening than during periods without. Early confirmation and management of PCHI were significantly increased. The rate of false-negative results from neonatal screening was significantly lower than that for the distraction test (4 vs 27% p=0.041). Interpretation Neonatal screening is effective in identification of congenital PCHI early and may be particularly useful for babies with moderate and severe PCHI for whom early management may have the most benefit.
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Our goals were to (1) study the prevalence of hearing impairment in a large cohort of infants with preauricular skin tags or ear pits and compare it with that among all other newborns participating in our universal newborn hearing screening program during the same period and (2) evaluate the effectiveness of transient evoked otoacoustic emissions as a hearing-screening tool in this population. During the study period of 7.5 years, 68484 infants were screened for hearing impairment, of whom 637 (0.93%) had preauricular skin tags and/or ear pits. The population was divided into 3 groups: (1) a low-risk group for hearing impairment; (2) a high-risk group for hearing impairment; and (3) a very high-risk group for hearing impairment. The screening results and audiological follow-up for these infants were examined retrospectively. A significantly higher prevalence of permanent hearing impairment was found among infants with preauricular skin tags or ear pits (8 of 1000), compared with infants without tags or pits (1.5 of 1000). In the low-risk group, the prevalence was 3.4 of 1000, compared with 0.5 of 1000 in infants with and without preauricular tags or pits, respectively. In the high-risk group, the prevalence was 77 of 1000, compared with 20 of 1000 in infants with and without preauricular tags or pits, respectively. The odds ratio for hearing impairment associated with preauricular skin tags and/or ear pits after adjusting for level of risk group was 4.9. All infants diagnosed with permanent hearing impairment, with the exception of 1 with late-onset impairment, were detected by in-hospital transient-evoked otoacoustic emissions screening. Infants with preauricular skin tags or ear pits are at increased risk for permanent hearing impairment. Transient-evoked otoacoustic emissions were found to be an effective hearing-screening tool in this population.
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Infants with the diagnosis of persistent fetal circulation were evaluated for hearing loss. From Jan 1, 1982, to Jan 1, 1984, 28 infants with this diagnosis were retrospectively identified, and 18 were evaluated by formal audiologic testing. Additionally, 22 infants were prospectively followed by serial auditory evaluation from Jan 1, 1984, to Jan 1, 1986. Of the 40 infants evaluated, 21 were identified as having hearing impairment (52.5%), 14 of whom required hearing aids. For 82% of those retrospectively identified hearing-impaired infants who required hearing aids, parental concern was expressed for their lack of hearing acuity. This factor could have aided in the earlier recognition of these infants' impairment. Among those infants followed prospectively, formal audiologic testing, in some cases serially, was needed to diagnose a progressive hearing loss that was expressed at 6 to 8 months after discharge from the neonatal intensive care unit. Perinatal factors associated with the development and management of persistent fetal circulation were identified and compared in infants with confirmed hearing loss and those with normal hearing. Variables related to those infants with hearing loss were as follows: degree of alkalosis, duration of ventilation, and possibly use of furosemide. We concluded from these results that infants with persistent fetal circulation have an extremely high incidence of sensorineural hearing loss and suggest serial formal audiologic evaluations to aid in detection of hearing-impaired infants.
Article
The contribution of parental suspicion in the original identification of a 16 year cohort of 171 children with varying degrees of hearing impairment who were screened and identified in childhood was studied. Only a quarter of the children with permanent hearing loss were identified as a result of parental concern. The presence of parental suspicion preceding the audiological diagnosis was also measured. Of the children with severe or profound deafness, the parents only suspected the presence of hearing loss in 44%. Parental suspicion was even lower for those with a mild or moderate permanent hearing loss, and for those with an otherwise symptomless conductive hearing loss caused by otitis media with effusion. Some parents did identify hearing impairment in their children, and parental suspicion should never be professionally disregarded. Most parents, however, experienced initial difficulty in recognising their children's hearing loss, even when the children were comparatively old. The study confirms the need to continue to identify deafness early by both parental vigilance and sensitive hearing screening programmes.
Article
Background: Targeted surveillance using a risk factor registry is the recommended method to detect hearing loss following the newborn screening period. However, currently, there is limited evidence linking some of the risk factors listed on the Joint Committee on Infant Hearing registry to postnatal hearing loss. Purpose: The goal of this study was to investigate the risk factors that were most likely to predict the occurrence of postnatal hearing loss using formal analysis of a large cohort. Research design: A retrospective study of children referred to the targeted surveillance program in Queensland, Australia. Study sample: This study involved children who were born in Queensland, Australia, between September 2004 and December 2009, had received a bilateral "pass" result during newborn hearing screening, had one or more risk factors identified during screening that triggered a referral to the targeted surveillance program, and had completed their follow-up audiology appointment series according to Queensland's diagnostic audiology protocols. Data collection and analysis: Data for the cohort was extracted from the state-wide data management system Oz Systems eScreenerPlus (eSP). Data analysis included yield calculations for each risk factor, χ² analysis, and logistic regression analysis. Results: During the study period, 2107 children met the inclusion criteria and were included in this study. Of these, 56 children (2.7%) were identified with a postnatal hearing loss. Statistical analysis revealed that two risk factors, family history (odds ratio [OR]: 1.92; 95% CI: 1.04-3.56), and craniofacial anomalies (OR: 2.61; 95% CI: 1.19-5.70) predicted the occurrence of postnatal hearing loss in children. In contrast, the risk factor of low birth weight (LBW) (OR: 0.14; 95% CI: 0.05-0.39) did not. Conclusion: This study suggests that children with the risk factors of family history and craniofacial anomalies should have their hearing monitored throughout early childhood, whereas children with the risk factor of LBW should not. Two additional risk factors, syndrome and prolonged ventilation, indicated favorable results for monitoring; however, a full analysis was unable to be completed due to statistical limitations. There was insufficient evidence within this study to support monitoring of children with the remaining risk factors of severe asphyxia, congenital infection, bacterial meningitis, professional concern, and hyperbilirubinemia. Further research with large cohorts of children with and without risk factors needs to be completed to further understand the relationship between risk factors and postnatal hearing loss.
Article
A 10-year cohort born in the East London District of Redbridge from 1990 to 2000, was enrolled into a targeted neonatal hearing screen (TNHS). Selection was based on the risk factor criteria of the American Joint Committee on Infant Hearing. The screen was typical of most other selective neonatal screens that were being undertaken in the UK in the 1990s. The cohort was followed up until the children had entered primary school. From the cohort of 32,890 neonates, 1148 (3.5%) were considered at risk of deafness and of these, 1054 received a neonatal Auditory Brainstem Response (ABR) air conduction test. There was a yield of 17 (5.2/10,000) with a moderate or worse bilateral permanent childhood hearing impairment (PCHI). By primary school entry the prevalence of moderate or worse PCHI had risen to 15.2/10,000 with 12.7/10,000 having a congenital deafness. Although 40% had been identified by the neonatal screen it could have been optimized to give a yield of 6.7/10,000. The potential results were compared to the model for targeted screens constructed by the US Preventative Services Task Force (USPS TF). There were important differences between the actual and constructed models. These differences are further discussed in the paper. The East London screen was more efficient but even the optimized screen was less effective than the estimates provided by the hypothetical model, with only half the predicted number of cases being confirmed before the model's target age of 10 months. The USPS TF model has been used to measure the potential added value of Universal Neonatal Hearing Screens (UNHS). However, it does not reflect the selective screens that have been undertaken in the UK, and substantiating evidence will not be available from this source. The conclusion from the reported longitudinal cohort study is that the hypothetical model overestimates the worth of targeted screening and by implication underestimates the potential added value of universal screens. Without a universal newborn hearing screen early detection of congenital deafness is not achievable for the majority of deaf and hearing impaired children.
Article
: The aim of this study was to investigate whether there was evidence-based support for targeted surveillance programs using a risk-factor registry to detect postnatal hearing loss or whether other programs were available that may be more effective than targeted surveillance. Particularly, it addressed questions that arose along the targeted surveillance pathway, including: () the risk factors used to determine a referral, () referral frequencies, () the relationship between risk factors and a postnatal hearing loss, and () other systems that may be more efficient than the use of risk factors. : A systematic literature review was completed in peer-reviewed and non-peer-reviewed databases. : Publications were obtained using the databases MEDLINE, Cumulative Index of Nursing and Allied Health Literature, and Excerpta Medica on line, and by inspecting the reference lists of relevant articles and expert-committee papers. Quality assessment ratings and data extraction were completed by the research team. : In total, 40 articles met the inclusion criteria and were included in this review. The outcomes drawn from these studies were inconclusive as they were based on small sample sizes and largely from single sites only. With this in mind, from the information obtained: () gestational age, low-birth weight, toxoplasmosis, other infections, rubella, cytomegalovirus (CMV), herpes simplex virus infections, craniofacial anomalies, respirator support, and the administration of aminoglycosides were the more commonly reported risk factors in programs; () referral frequency for monitoring appointments was approximately 3%; and () CMV, extracorporeal membrane oxygenation, congenital diaphragmatic hernia, and persistent pulmonary hypertension of the newborn were associated with postnatal hearing loss, whereas, preauricular skin tags and ear pits, low-birth weight, and toxoplasmosis were not. The review also identified that second-phase universal screening programs, CMV screening, and genetic screening should be explored as potential additions or alternatives to targeted surveillance using risk factors. : Further large-scale, population-based research on available programs for the detection of postnatal hearing loss should be conducted to improve present practice and assist with the development of evidence-based guidelines for hearing monitoring.
Article
The importance of monitoring hearing throughout early childhood cannot be understated. However, there is a lack of evidence available regarding the most effective method of monitoring hearing following the newborn screen. The goal of this study was to describe a targeted surveillance program using a risk factor registry to identify children with a postnatal hearing loss. All children who were born in Queensland, Australia between September 2004 and December 2009, received a bilateral 'pass' on newborn hearing screening, and had at least one risk factor, were referred for targeted surveillance and were included in this study. The cohort was assessed throughout early childhood in accordance with Queensland's diagnostic assessment protocols. During the study period, 7320 (2.8% of 261,328) children were referred for targeted surveillance, of which 56 were identified with a postnatal hearing loss (0.77%). Of these, half (50.0%) were identified with a mild hearing loss, and 64.3% were identified with a sensorineural hearing loss. In regards to risk factors, syndrome, craniofacial anomalies, and severe asphyxia had the highest yield of positive cases of postnatal hearing loss for children referred for targeted surveillance, whereas, low birth weight, bacterial meningitis, and professional concern had a particularly low yield. Limitations of the targeted surveillance program were noted and include: (1) a lost contact rate of 32.4%; (2) delays in first surveillance assessment; (3) a large number of children who required on-going monitoring; and (4) extensive diagnostic assessments were completed on children with normal hearing. Examination of the lost contact rate revealed indigenous children were more likely to be documented as lost contact. In addition, children with one risk factor only were significantly more likely to not attend a surveillance appointment. Positive cases of postnatal hearing loss were detected through the targeted surveillance program. However, the limitations of the program question the usefulness of this service delivery model. For targeted surveillance to continue, time frames for assessment, assessments performed, and discharge criteria need to be revisited. The contribution of individual and combined risk factors in detecting postnatal hearing loss should also be examined in more detail.
Article
The goal of this study was to investigate the prevalence of delayed-onset hearing loss in preschool children who previously passed newborn hearing screening in Shanghai, China. Between October 2009 and September 2010, 21,427 preschool children were enrolled from five areas of Shanghai, who had passed newborn hearing screening. Children were screened for delayed-onset hearing loss with pediatric audiometers. Children with positive results in initial and re-screening tests were assessed audiologically and for risk indicators according to the Year 2007 Statement of the American Joint Committee on Infant Hearing (JCIH). During the study period, 445 children (2.08%) were referred for audiologic assessment; 16 (0.75/1000, 95% CI 0.38-1.12) had permanent delayed-onset hearing loss. Of these, five (0.23/1000) had bilateral moderate hearing loss; seven (0.33/1000) had mild bilateral hearing loss; and four (0.19/1000) had unilateral moderate or mild hearing loss. JCIH risk indicators were established for six children (6/16, 37.5%), including parental concern regarding speech and language developmental delay, neonatal intensive care unit with assisted ventilation or hyperbilirubinemia, recurrent otitis media with effusion, craniofacial malformation, and family history (n=1 for each). The remaining ten (62.5%) had no related risk factors. A significant proportion of preschool children have undiagnosed delayed-onset hearing loss. Hearing screening in preschool is recommended for an early detection.
Article
Newborn hearing screening has been nationally implemented, but longitudinal cohort follow-up is required to inform Children's Hearing Services of the requirements for postneonatal care pathways. A 10-year cohort of 35 668 births enrolled into a Universal Neonatal Hearing Screen was followed up until the children had completed the first year of primary school. There were 3.65/1000 children with a permanent hearing impairment of any degree embarking on their education. 1.51/1000 had a moderate or worse bilateral deafness but only 0.9/1000 with this degree of deafness had been identified by newborn screening. Postneonatal care pathways were required to identify those with congenital impairments missed by the screen (0.11/1000), those moving into the district (0.25/1000) and those with late onset deafness (0.25/1000). An additional postneonatal yield of 1.2/1000 had mild or unilateral impairments. When all degrees of impairment were considered 51% of the children with a permanent hearing impairment had required identification by postneonatal care pathways. Despite the success of the newborn hearing screen, the provision of postneonatal pathways remains essential for identifying deafness in early childhood.
Article
We present population-based, childhood prevalence rates of and neonatal risk factors for permanent hearing loss among extremely premature infants. By using an inception-cohort, longitudinal study design for 1974-2003, we studied permanent hearing loss among 1279 survivors with gestational age of < or =28 weeks and birth weight of <1250 g (mortality rate: 42.7%; lost to follow-up monitoring: 4.7%) Newborn hearing screening, performed by experienced pediatric audiologists, used click-evoked auditory brainstem response testing after 1975. Survivors underwent repeated behavioral audiologic testing and multidisciplinary follow-up monitoring. Permanent hearing loss was defined as mild/moderate (26-70 dB hearing level), severe/profound (71 to >90 dB hearing level), delayed-onset (diagnosed after previously normal hearing), or progressive (increase in loss of > or =15 dB hearing level). Permanent hearing loss rates were established at 3 years of age, with newborn, infant, and >5-year final hearing outcomes being recorded. Risk factors were compared for children with and without hearing loss, odds ratios were calculated, and prediction performance was determined through area under the curve analysis. Forty (3.1%) of 1279 survivors 3 years of age had permanent hearing loss and 24 (1.9%) had severe/profound loss, with no changes over time. Bilateral delayed-onset loss occurred for 4 children (10%) and progressive loss for 11 children (28%). One child had auditory neuropathy, and 29 (73%) had multiple disabilities. Prolonged oxygen use, gastrointestinal surgery, patent ductus arteriosus ligation, and low socioeconomic index yielded good prediction of permanent hearing loss; oxygen use was the most significant predictor of severe/profound loss. Permanent hearing loss remains an adverse outcome of extreme prematurity, complicated by significant delayed-onset and progressive loss. Prolonged supplemental oxygen use is a marker for predicting permanent hearing loss; this requires detailed analysis of the pathophysiologic features, to reduce the prevalence of permanent hearing loss.
Article
The Joint Committee on Infant Hearing 2007 Position Statement includes in utero toxoplasmosis infection as a risk indicator for delayed-onset or progressive sensorineural hearing loss. It is recommended that children with congenital toxoplasmosis infection undergo audiologic monitoring to identify congenital and delayed-onset sensorineural hearing loss. To determine the prevalence of sensorineural hearing loss and to develop evidence-based guidelines for audiologic monitoring of children born with congenital toxoplasmosis infection. Systematic search of Medline, EMBASE and Cochrane databases and manual search of references. Longitudinal studies reporting an inception cohort identified at birth, with serologic confirmation of toxoplasmosis infection, and long-term serial audiometric evaluation. Independent extraction of patient and audiometric data. Descriptive statistics. The five studies meeting our inclusion criteria report a prevalence of toxoplasmosis-associated hearing loss from 0% to 26%. Improved treatment regimens for toxoplasmosis may account for this range. Three treatment groups were identified and a subgroup analysis of the compiled data was performed. In children receiving limited or no treatment, the prevalence of toxoplasmosis-associated SNHL was found to be 28%. In children prescribed 12 months of antiparasitic treatment but in whom treatment was not confirmed to have started prior to 2.5 months of age and in whom compliance was not ensured, the prevalence of SNHL was 12%. In children treated with 12 months of antiparasitical therapy initiated prior to 2.5 months of age with serologically-confirmed compliance, the prevalence of SNHL was 0%. Only two longitudinal studies were identified and neither reported any cases of delayed-onset or progressive toxoplasmosis-associated SNHL. Children who have received a 12-month course of antiparasitical therapy initiated prior to 2.5 months with serologically-confirmed compliance should have repeat audiometric evaluation at 24-30 months of age. Children with congenital toxoplasmosis that had no treatment, partial treatment, delayed onset of treatment, or compliance issues should undergo annual audiologic monitoring until able to reliably self-report hearing loss.
Article
Neurophysiologic and behavioral assessments of auditory function were performed on 224 very low birth weight (less than or equal to 1500 gm) infants requiring intensive care in the nursery. The subjects were studied prospectively from 36 weeks to 4 years of age, as available for follow-up. To classify them according to their neonatal status, we applied a principal components analysis to a number of variables representative of the extent of illness and of patient care in early postnatal life. The subjects were then divided into neonatal status quartiles and evaluated for hearing outcome. All those with sensorineural hearing loss fell exclusively into the lowest neonatal status quartile. Sensorineural hearing loss was statistically associated (1) with greater amounts of furosemide administration for longer durations and in combination with aminoglycoside antibiotics and (2) with more episodes of low pH, hypoxemia, or both, higher total bilirubin levels, and substantially lower neonatal status scores. Birth weight, gestational age, highest creatinine level, Apgar score, and aminoglycosides alone were not systematically related to hearing capacity. Subjects in the lowest neonatal status quartile also had a considerably higher incidence of middle ear disorders, characterized by elevated thresholds and prolonged auditory brain stem-response latencies reflective of conductive hearing loss. We conclude that protracted illness and its associated treatment, independently of specific diagnostic categories, constitute important risk factors for permanent hearing loss and for transient hearing loss in early life.
Article
In order to assess the importance of pertinent perinatal risk factors in causing hearing loss (HI), a retrospective evaluation was made of the records of 146 affected children born in the city of Göteborg between 1970 and 1979. The incidence among six-year-olds born between 1970 and 1974 was 3.8 per 1000 newborns. If only HI of more severe degree was taken into account (above 40dB in the best ear), the remaining incidence was 1.4/1000. Sensorineural HI (SNHI) accounted for 87 per cent of the cases. A positive hereditary tendency for HI was found in 55 per cent. In 61 per cent the origin of the HI was presumably prenatal, either positive heredity alone or in the form of facio-auricular anomalies, syndromes and toxic influences (infection and alcohol) during early pregnancy. Postnatal infections (meningitis, parotitis and secretory otitis media) could be ascertained as causes in about 20 per cent of the cases. For 12 per cent no aetiology could be determined. Perinatal aetiology was probable or possible in about 10 per cent of the children. The frequency of SNHI was found to be increased among survivors of neonatal intensive care, VLBW, LBW and SFD infants. Caesarean section, ventouse and breech delivery were not associated with increased rates, nor were hyperbilirubinaemia, exchange transfusion or birth asphyxia. No cases could be traced to aminoglycoside treatment. Neonatal sepsis/meningitis may have been the cause in two of the 146 cases. Hypoxia as a consequence of apnoea and respiratory distress syndrome necessitating mechanical ventilation appeared to be the major risk-factor in the neonatal period. However, in comparison with genetic predisposition, potentially damaging perinatal factors appeared to be of minor importance.
Article
The pre-, peri-, and postnatal histories for two infants who suffered respiratory distress syndrome (RDS) are presented. Each infant was diagnosed with RDS within 24 hours after birth, placed on high-frequency jet ventilation, and passed auditory brainstem response (ABR) screening prior to hospital discharge. Both infants were enrolled in a neonatal follow-up program with no report of hearing loss during the first year of life. At 2.5 years of age, each infant was found to have severe-to-profound sensorineural hearing loss. Neither infant suffered kidney failure, rubella, cytomegalovirus, or genetic involvement. Audiologic monitoring after hospital discharge was not performed because each infant passed the ABR screen. Thus, the date of onset and/or progression of hearing loss are unknown. Until additional research findings are available, we recommend that the hearing of any infant diagnosed with RDS be monitored frequently until 3 years of age.
Ten children, who developed hearing disturbance after discharge from the neonatal intensive care unit (NICU), are reported. All were born at full term, between November 1988 and November 1992, weighed at least 2500 g and had normal auditory brainstem response (ABR) at discharge from the NICU. All ten underwent thorough evaluation to determine the cause of the subsequent hearing loss. All had severe cardiovascular and/or pulmonary disorders at birth, and persistent pulmonary hypertension of the newborn (PPHN) was diagnosed in eight. A total of 25 PPHN cases were treated at our facility during the 1988-1992 period, the rate of hearing disturbance in PPHN patients being 32%. The 10 infants in this study were mechanically ventilated for, on average, 30 days. High frequency oxygenation (HFO) was required in five cases, extra corporeal membrane oxygenation (ECMO) in six. During this period, only eight ECMO-treated infants survived. The rate of hearing loss in surviving cases was therefore very high, 75% (6 8). All 10 of our cases were treated with an aminoglycoside, furosemide and a muscle relaxant. We recommend follow-up at 6 and 12 months of age, as these children are at very high risk of developing hearing disturbance.
Article
Among 547 preterm infants of < or = 34 weeks gestation born between 1987 and 1991, 8 children (1.46%) developed severe progressive and bilateral sensorineural hearing loss. Perinatal risk factors of infants with hearing loss were compared with those of two control groups matched for gestation and birth weight and for perinatal complications. Our observations demonstrated an association of hearing loss with a higher incidence of perinatal complications. Ototoxicity appeared closely related to a prolonged administration and higher total dose of ototoxic drugs, particularly aminoglycosides and furosemide. Finally, we strongly recommend to prospectively and regularly perform audiologic assessment in sick preterm children as hearing loss is of delayed onset and in most cases bilateral and severe.
Article
To determine the prevalence and temporal changes of sensorineural hearing loss (SNHL) among children with clinically inapparent (asymptomatic) congenital cytomegalovirus (CMV) infection identified from a cohort of newborn infants screened for congenital CMV infection. The study population consisted of 307 children with documented asymptomatic congenital CMV infection, 76 uninfected siblings of children with asymptomatic congenital CMV infection, and 201 children whose neonatal screen for congenital CMV infection showed negative results. Audiologic evaluations were completed for all children to determine their hearing status. SNHL occurred only in children with congenital CMV infection. Of the children with asymptomatic congenital CMV infection, 22 (7.2%; 95% confidence interval, 4.5% to 10.6%) had SNHL. Among the children with hearing loss, further deterioration of hearing occurred in 50.0%, with the median age at first progression at 18 months (range, 2 to 70 months). Delayed-onset SNHL was observed in 18.2% of the children, with the median age of detection at 27 months (range, 25 to 62 months). Fluctuating SNHL was documented in 22.7% of the children with hearing loss. Asymptomatic congenital CMV infection is likely a leading cause of SNHL in young children. The continued deterioration of hearing and delayed onset of SNHL in these children emphasizes the need for continued monitoring of their hearing status.
Article
Survivors of extracorporeal membrane oxygenation (ECMO) therapy and high-frequency oscillatory (HFO) therapy during the newborn period were followed to evaluate their hearing. Eleven of the 66 ECMO survivors (16.7%) were diagnosed with significant hearing loss after being discharged from the neonatal intensive care unit (NICU). This rate of hearing loss is consistent with other reports of hearing loss in ECMO survivors. The majority of ECMO survivors with hearing loss developed a bilateral sloping hearing loss; the high frequencies were more impaired than the low frequencies. The hearing of nine ECMO survivors with hearing loss was assessed with auditory brainstem evoked responses (ABRs) in the newborn period prior to discharge from the NICU. Seven of nine ECMO survivors with hearing loss passed their newborn ABR screen. This result is consistent with the results of other researchers. Eight newborns receiving ECMO therapy had congenital diaphragmatic hernias (CDH). Three of these newborns (37.5%) were subsequently diagnosed as having a hearing loss. The combination of CDH and ECMO therapy may be a strong predictor of hearing loss. Six HFO survivors with hearing losses were also followed. Their losses were similar to the ECMO survivors with hearing loss, including the progressive nature of the loss (all five of these newborns with neonatal ABR screens passed them only to later be diagnosed with a hearing loss). A growing body of research indicates that newborns experiencing severe oxygen deprivation are at risk for progressive hearing loss.
Article
Survivors of extracorporeal membraneous oxygenation (ECMO) treatment are at risk for serious neurodevelopmental disorders or neurologic sequelae, but little has been reported about the prevalence of sensorineural hearing loss in these patients. Of 123 ECMO survivors born between 1987 and 1996, at least 30 survivors have confirmed sensorineural hearing loss. Progressive hearing loss has been confirmed in 10 subjects.
Article
Persistent pulmonary hypertension of the newborn (PPHN) and its conventional medical treatment are associated with sensorineural hearing loss, yet current recommendations for regular audiological evaluations of PPHN survivors are lacking. We report a case of delayed-onset, progressive sensorineural hearing loss in a 3-year-old patient with a history of PPHN and a normal auditory brainstem evoked response at 6 weeks of age. The relatively late detection of significant sensorineural hearing loss in this otherwise healthy 3-year-old illustrates the need for audiological evaluation at regular intervals in patients with a history of PPHN.
Article
Although previous studies have documented the feasibility and benefits of universal newborn hearing screening in selected hospitals, none have reviewed the effectiveness of regionally mandated participation of large numbers of hospitals with variable levels of motivation to succeed. The purpose of this study was to measure hospital participation and overall screening success in a statewide program for universal newborn hearing screening and to track improvements in program establishment and outpatient follow-up over time. Four Colorado hospitals began voluntarily performing hearing screening before hospital discharge on all newborns in 1992. By 1996, 26 Colorado hospitals were participating in universal newborn hearing screening. The publication of screening results from these early years served as a catalyst for legislation requiring increased hospital participation in establishing universal screening programs. Data systems were subsequently developed to improve statistical tracking and follow-up. Eight years' worth of cumulative study data as well as the results from calendar year 1999 (the year of greatest hospital participation) were reviewed for collective measures of successful screening and follow-up. Three hospitals did not initiate newborn hearing screening programs until after the study period ended in 1999. Of the 57 hospitals that were screening newborns in 1999, the chosen method of screening at 52 hospitals was automated auditory brainstem response testing; 3 hospitals used otoacoustic emission testing, and the remaining 2 hospitals used 2-stage screening. Hearing loss was defined as a threshold of 35 decibels or greater in 1 or both ears at the time of confirmatory testing. During the full 8-year study period, 1992 to 1999, 148 240 newborns were screened. A total of 291 infants who were born during the study period received a diagnosis of congenital hearing loss. In this cohort of 291 children, the cumulative frequency of bilateral hearing loss was 71% (range: 48%-94% by calendar year), the frequency of sensorineural hearing loss was 82% (range: 67%-88%), and the frequency of 1 or more risk factors was 47% (range: 37%-61%). During calendar year 1999, a total of 63 590 births were recorded at 60 birthing hospitals in Colorado. The families of 263 (0.4%) of these newborns refused newborn hearing screening. Of the remaining 63 327 newborns, 87% (55 324 infants) were screened for hearing acuity before hospital discharge, a far greater percentage than the 19% of all newborns screened during the first 5 years of voluntary hospital participation, and approaching the American Academy of Pediatrics's recommendation of 95% of newborns completing hospital-based testing in a successful screening program. As a result of this statewide hearing screening program, congenital hearing loss was diagnosed in 86 Colorado newborns during 1999, representing an occurrence rate of approximately 1 affected child in every 650 newborns. In this group of 86 infants, 59 had bilateral sensorineural hearing loss, 17 had unilateral sensorineural hearing loss, 4 had bilateral conductive hearing loss, and 6 had unilateral conductive hearing loss. Mild hearing loss was present in 6 infants, moderate hearing loss was present in 42 infants, severe hearing loss was present in 33 infants, and profound hearing loss was present in the remaining 5 infants. Only 32 of the 86 affected newborns in 1999 had 1 or more risk factors for hearing loss subsequently identified. After failing an initial hospital-based screening at 1 of the 57 participating hospitals in 1999, 2.3% of infants screened (1283 newborns) were referred for follow-up testing, easily exceeding the standard of <4% recommended by the American Academy of Pediatrics. Similarly, the false-positive rate of 2.2% during 1999 exceeded the recommended standard of <3%. Of the infants who failed their initial screening, 76% (978 infants) had documented follow-up testing to confirm or exclude congenital hearing loss, a percentage significantly improved from a follow-up rate of 48% during the first 5 years of screening, although not yet achieving the standard of 95% recommended by the American Academy of Pediatrics. Nine participating hospitals, however, were able to document appropriate follow-up for 95% or more of the infants who failed their initial screening tests. The median age of diagnosis of congenital hearing loss during 1999 was 2.1 months; 71% of affected infants were identified by 3 months of age (the recommended standard for age of diagnosis), and 92% of affected newborns were identified by 5 months of age. Measures of screening success were compared for large, mid-sized, and small hospitals. Increasing hospital size, as measured by the number of births per year, was associated with an increasing percentage of newborns who were successfully screened. It was notable that smaller hospital size was associated with increased referral rates for follow-up testing, whereas larger hospital size was associated with the highest recapture rate for follow-up testing. Universal screening for congenital hearing loss is demonstrated to be feasible in a large regional effort of legislatively mandated participation. The success of such an endeavor is dependent on educational efforts for community professionals, commitment on the part of program planners, and data systems that more accurately track and recall infants who fail initial hospital-based screening.
Article
Congenital cytomegalovirus (CMV) infection is a major cause of sensorineural hearing loss (SNHL) and neurologic impairment in children. Although the majority of children with symptomatic congenital CMV infection develop hearing loss, many symptomatic infants have normal hearing. The purpose of this study was to identify indicators present in the newborn period that have predictive value for the development of hearing loss in children with symptomatic congenital CMV infection. Of the 190 children who had symptomatic congenital CMV infection and were born between 1966 and 1997 and enrolled in a follow-up study, hearing outcome was known for 180 children. Follow-up data were analyzed using univariate and multivariate logistic regression analyses to determine the specific demographic, newborn clinical, and laboratory findings predictive of hearing loss. The amount of infectious CMV in urine was quantified in a subset of 21 children who were born between 1994 and 1998. The presence of intrauterine growth retardation, petechiae, hepatosplenomegaly, hepatitis, thrombocytopenia, and intracerebral calcifications was associated with the development of hearing loss on univariate analysis. The presence of microcephaly and other neurologic abnormalities was not predictive of hearing loss. Logistic regression analysis revealed that only petechiae and intrauterine growth retardation independently predicted hearing loss. None of the demographic and other newborn findings predicted progressive hearing loss. The children who developed hearing loss had higher urine CMV titers during infancy than those with normal hearing. In children with symptomatic congenital CMV infection, evidence of disseminated infection with or without the presence of neurologic involvement at birth was predictive of the development of hearing loss. However, it was not possible to identify factors that are independently predictive of the development of progressive hearing loss.
Article
To endeavor to explain why some graduates of extracorporeal membrane oxygenation (ECMO) therapy develop sensorineural hearing loss (SNHL) whereas others do not, to study the variability seen in the degree of SNHL, to attempt to explain why some graduates with SNHL experience progressive worsening whereas others do not, and to describe the time course of the onset of SNHL on the basis of identified risk factors. A retrospective chart review with proportional-hazards regression analysis to identify specific risk factors for SNHL from a list of patient and treatment variables. Children's Hospital Boston, a pediatric tertiary-care facility and ECMO center. Neonatal ECMO graduates born in 1986-1994 who survived to discharge and underwent audiologic evaluations (n = 111) and a random sample of ECMO graduates who survived to discharge and did not undergo audiologic evaluations (n = 30). Audiologic data, including the presence or absence of SNHL, the severity of SNHL at the most recent evaluation, the stability or progressive worsening of hearing (with the first evaluation compared with the most recent evaluation), and the occurrence of delayed-onset SNHL. Twenty-nine (26%) of 111 ECMO graduates who underwent audiologic testing had SNHL at the last evaluation. Of these 29 subjects with SNHL, 21 (72%) had progressive SNHL, of whom 14 (48%) had delayed-onset SNHL. The age of identification of SNHL ranged from 4 months to 8 years 11 months. Factors identified with proportional-hazards regression analyses as being associated significantly with the time to onset of SNHL were a primary diagnosis of congenital diaphragmatic hernia (hazard ratio: 2.60), length of ECMO therapy (hazard ratio: 7.18), and number of days children received aminoglycoside antibiotics (hazard ratio: 5.56). Kaplan-Meier "time-to-event" curves were constructed to illustrate the time course of onset of SNHL, as affected by each of the variables identified as significant risk factors. These findings illustrate the need for early, routine, audiologic evaluations throughout childhood for all ECMO graduates. Children at even greater risk for developing SNHL because of a history of congenital diaphragmatic hernia, prolonged ECMO therapy, and/or a lengthy course of aminoglycoside antibiotic therapy should be monitored even more closely throughout childhood, depending on the child's individual risk indicators, as suggested here. On the basis of these risk indicators, efforts can be made to minimize the risk of hearing loss while a child is being treated with ECMO. In addition, these risk indicators can assist with counseling families of ECMO graduates regarding the child's specific risk of developing SNHL and how it can be managed should it occur.
Article
The goal of this study was to determine the percentage of children who have a postnatal permanent childhood hearing impairment (PCHI) and the percentage thereof who have risk indicators for a postnatal hearing loss. Data were drawn retrospectively from the clinical charts of children who had bilateral PCHI (>40 dB hearing level, better ear, unaided) and had undergone universal newborn hearing screening (UNHS) between 1995 and 2000 in various Austrian hospitals. A hearing loss was recognized as postnatal when a child passed UNHS but was later found to have a hearing impairment. The presence of risk indicators, as suggested by the Year 2000 Statement of the American Joint Committee on Infant Hearing (JCIH), was assessed by reviewing the children's clinical charts. Of a total of 105 children with bilateral PCHI, 23 (22%) showed postnatal impairment. After correction of this number for underascertainment, postnatal impairment was estimated to account for 25% of all bilateral PCHI at age 9 years. Risk indicators were found in 17 children but did not fully correspond to those proposed by the JCIH. The risk factors found were a family history of hearing loss (3 children), meningitis (2), craniofacial malformation (2), persistent pulmonary hypertension (1), congenital cytomegaly infection (1), extracorporeal membrane oxygenation (1), recurrent otitis media with effusion (1), and, in addition to the JCIH list, ototoxic therapy (5), and birth before 33rd gestational week (2) (1 child had a combination of the last 2). Six children showed no risk indicators for the postnatal hearing loss. Our findings suggest that approximately 25% of bilateral childhood hearing loss is postnatal, which supports the leading role of UNHS in detecting PCHI. Provisions for also identifying postnatal cases nevertheless are justified. Because in some of these children no risk indicators are detectable and in others the hearing deterioration starts after age 3 years, audiologic monitoring of at-risk children up to this age may not be sufficient. Additional methods, such as hearing screening at nursery schools or schools, are recommended.
Article
To define hearing outcomes in children with congenital cytomegalovirus (CMV) infection born to mothers with non-primary CMV infection. A cohort of 300 children with congenital CMV infection identified by newborn virologic screening at the University of Alabama Hospital and a private community hospital in which the type of maternal infection could be classified constituted the study population. Maternal infections were categorized by analyzing serum samples. Children were followed prospectively and underwent serial audiologic evaluations. The frequency of hearing loss was not different between children born to mothers with non-primary infection (10%) and those with primary infection (11%). Significantly more children in the primary infection group had progressive and severe/profound hearing loss compared with children in the non-primary group. The frequency of bilateral, delayed onset, high-frequency, and fluctuating hearing loss was not different between the 2 groups. The mean age of diagnosis of hearing loss was 39 +/- 53 months for children born to mothers with non-primary infection and 13 +/- 21 months for the primary infection group (P = .16). Maternal preexisting seroimmunity to CMV does not provide complete protection against hearing loss in infants with congenital CMV infection.
Article
The aim of this study was to evaluate the audiological outcome of long-term follow-up of infants with asymptomatic congenital cytomegalovirus (CMV) infection as defined by the presence of CMV DNA in neonatal urine. 12599 pregnant women underwent screening for CMV IgG and IgM antibodies between 1996 and 2003. Eighteen infants with congenital CMV infection were identified. These infants underwent the newborn hearing screening test or auditory brainstem response test. Follow-up hearing assessments were performed with the auditory brainstem response and behavioral audiometry. The seropositive rate of CMV IgG antibody among the pregnant women was 75.3%, and the yearly seropositive rate decreased over the study period. One hundred and forty-six pregnant women were positive for IgM antibody, and 18 neonates (12.3%) had congenital CMV infection. Sensorineural hearing loss (SNHL) was detected in 4 (25%) of the 16 infants with asymptomatic infection and 1 (50%) of the 2 infants with symptomatic infection during the first 6 months of life. Two infants who passed the newborn hearing screening had a delayed-onset SNHL in follow-up examinations up to 4 years of age. Two had progressive hearing loss and 2 had improvement of hearing loss. Screening of pregnant women for CMV infection and repeated audiological examinations of infants are necessary because there are infants with delayed-onset SNHL or improved SNHL caused by asymptomatic congenital CMV infection.
Article
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.
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 enroll 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.