Anne Marie Oudesluys-Murphy

Newborn Hearing Screening vs Later Hearing Screening and Developmental Outcomes in Children With Permanent Childhood Hearing Impairment

CONTEXTnNewborn hearing screening programs have been implemented in many countries because it was thought that the earlier permanent childhood hearing impairment is detected, the less developmentally disadvantaged children would become. To date, however, no strong evidence exists for universal introduction of newborn hearing screening.nnnOBJECTIVEnTo study the effect of newborn hearing screening vs distraction hearing screening, conducted at 9 months of age, on development, spoken communication, and quality of life.nnnDESIGN, SETTING, AND PARTICIPANTSnBetween 2002 and 2006, all 65 regions in The Netherlands replaced distraction hearing screening with newborn hearing screening. Consequently, the type of hearing screening offered was based on availability at the place and date of birth and was independent of developmental prognoses of individual children. All children born in The Netherlands between 2003 and 2005 were included. At the age of 3 to 5 years, all children with permanent childhood hearing impairment were identified. Evaluation ended December 2009.nnnMAIN OUTCOME MEASURESnPerformance (education and spoken and signed communication), development (general and language), and quality of life.nnnRESULTSnDuring the study period, 335,560 children were born in a newborn hearing screening region and 234,826 children in a distraction hearing screening region. At follow-up, 263 children in newborn hearing screening regions (0.78 per 1000 children) and 171 children in distraction hearing screening regions (0.73 per 1000 children) had been diagnosed with permanent childhood hearing impairment. Three hundred one children (69.4%) participated in analysis of general performance measures. There was no difference between groups in the primary mode of communication or type of education. Analysis of extensive developmental outcomes included 80 children born in newborn hearing screening regions and 70 in distraction hearing screening regions. Multivariate analysis of variance showed that overall, children in newborn hearing screening regions had higher developmental outcome scores compared with children in distraction hearing screening regions (Wilks λ = 0.79; F(12) = 2.705; P = .003). For social development, the mean between-group difference in quotient points was 8.8 (95% CI, 0.8 to 16.7) and for gross motor development, 9.1 (95% CI, 1.1 to 17.1). For quality of life, the mean between-group difference was 5.3 (95% CI, 1.7 to 8.9), also in favor of children in newborn hearing screening regions.nnnCONCLUSIONnCompared with distraction hearing screening, a newborn hearing screening program was associated with better developmental outcomes at age 3 to 5 years among children with permanent childhood hearing impairment.

Neonatal hearing screening

Severe congenital hearing impairment is an important handicap affecting 0.1% of live-born infants and 1%–2% of graduates of Neonatal Intensive Care Units. The prognosis for intellectual, emotional, language and speech development in the hearing-impaired child is improved when the diagnosis is made early and intervention is begun before the age of 6 months. The usual age at diagnosis of hearing impairment is at least 18–30 months (or even later in cases of less severe hearing impairment) where there are no screening programmes. When screening is carried out using distraction methods at the age of approximately 9 months some hearing-impaired infants are missed and those discovered are at least 15–18 months before intervention begins. Neonatal screening could give hearing-impaired children the best chances for optimal care and development. Universal neonatal hearing screening is necessary, because, when neonatal hearing screening is restricted to high risk groups 30%–50% of infants with hearing loss are not discovered. The methods available for neonatal hearing screening are discussed in this paper.ConclusionIn our view automated measurement of auditory brainstem responses is the most valuable method for universal neonatal hearing screening.

Evaluation of an automated auditory brainstem response infant hearing screening method in at risk neonates.

An automated auditory brainstem response (ABR) methodthe ALGO-1 Plus-has been developed for hearing screening in healthy neonates. The aim of this study was to test the validity of this automated ABR screening method in at-risk neonates in a neonatal intensive care unit. Two hundred and fifty at-risk neonates were selected for screening according to the criteria of the American Joint Committee on Infant Hearing. All 250 neonates were screened with the ALGO-1 Plus for bilateral hearing loss. When two consecutive screenings pointed to bilateral hearing loss (“refer”) further audiological investigations were performed and where necessary therapeutic measures were taken. All children who ”passed” the screening unilateral or bilateral enrolled in a nationwide behavioural screening programme at the age of 9 months as well as in a 6-monthly follow up programme documenting speech and language development. A total of 245 (98%) neonates passed the ALGO-1 screening, 230 (92%) at the first attempt and 15 (6%) at the second attempt. Five (2%) were referred with bilateral hearing loss. One of these died of congenital rubella shortly after screening and bilateral congenital hearing loss of >35 dB was confirmed in the other 4. None of the infants who passed the screening were discovered to have moderate to severe bilateral hearing loss (> 40 dB) with behavioural screening (n=183/233) or at follow up (n=233/233). In this study, all at-risk neonates with bilateral congenital hearing loss were detected with ALGO-1 Plus screening. No falsenegatives were discovered.

Awareness of congenital cytomegalovirus among doctors in the Netherlands

BACKGROUNDnBecause of limited treatment options for congenital cytomegalovirus (CMV) infection, preventive strategies are important. Knowledge and awareness are essential for the success of preventive strategies.nnnOBJECTIVESnTo investigate the knowledge of congenital CMV among doctors involved in mother and child care in the Netherlands.nnnSTUDY DESIGNnA questionnaire on CMV infection was sent to doctors by snowball sampling. Knowledge concerning epidemiology, transmission, symptoms and signs of CMV infection in adults and children, and treatment options were evaluated.nnnRESULTSnThe questionnaire was completed by 246 doctors involved in mother and child care. The respondents estimated a prevalence of congenital CMV varying between 0.1 and 500 per 1000 live-born infants. The mean knowledge scores regarding transmission and postnatal symptoms increased with a more advanced career stage (i.e. older age). Gender and parenthood did not contribute to knowledge, but the field of expertise did. Respondents in the field of pediatrics had the highest mean score on postnatal symptoms and long-term effects. Respondents working in the field of gynecology and obstetrics were unaware of the precise transmission route of CMV. More than one-third of the respondents assumed that treatment was readily available for congenital CMV infection.nnnCONCLUSIONSnThe knowledge of CMV infection among doctors in the Netherlands contained several gaps. Increasing knowledge and awareness is expected to enhance the prevention of transmission, to improve recognition, and to stimulate diagnostic investigations and follow-up programs.

Cytomegalovirus infection in the Netherlands: Seroprevalence, risk factors, and implications

BACKGROUNDnCytomegalovirus (CMV) infections occur worldwide and are usually asymptomatic in healthy individuals. In fetuses and immunocompromised persons, they can cause severe disease and disabilities.nnnOBJECTIVEnTo determine the CMV seroprevalence and risk factors for CMV infection in the Netherlands.nnnSTUDY DESIGNnIn a cross-sectional population-based study (PIENTER-2, 2006-2007), sera and questionnaire data were collected from 6386 individuals. Sera were tested for CMV-specific IgG antibodies using enzyme-linked immunosorbent assay (ELISA).nnnRESULTSnThe CMV seroprevalence in the general population (6 months-79 years) was 45.6%. Age and country of origin were the most prominent independent risk factors. The seroprevalence was significantly lower in native Dutch and Western individuals (41.5%) than in non-Western individuals (76.7%). Multivariable logistic regression analysis showed that age, lower educational level, first-generation migrancy, and among native Dutch/Western individuals, female gender and having contact with young children, were independently associated with CMV seropositivity. The geometric mean concentrations of antibodies increased with age and were higher in women than in men.nnnCONCLUSIONnCMV seroprevalence in the Netherlands is relatively low compared to other countries. This is in line with our finding of a higher seroprevalence among migrants compared to the native population. The higher seroprevalence in women and individuals who have contact with young children is especially important for women of reproductive age. Preventing CMV infection in these women, through counseling on hygiene or possible future vaccination, may lead to a decrease of congenital CMV infections.

Screening for refractive errors in children: the plusoptiX S08 and the Retinomax K-plus2 performed by a lay screener compared to cycloplegic retinoscopy.

PURPOSEnTo evaluate the performance of the autorefractor Retinomax K-plus2 and the photoscreener plusoptiX S08 in measuring refractive errors by comparing them with cycloplegic retinoscopy (CR) and to assess limitations associated with their use.nnnMETHODSnCross-sectional study to compare data from CR, performed by an orthoptist, to data from Retinomax K-plus2 and plusoptiX S08 performed by a lay screener. Sensitivity and specificity for the detection of significant refractive errors were determined according to American Academy of Pediatric Ophthalmology and Strabismus criteria.nnnRESULTSnTwo hundred children were included, with a mean age of 5.2 ± 2.6 years (3 months to 11 years). Compared to CR, the plusoptiX S08 showed a mean difference of -1.13 ± 1.25 D (95% limits of agreement [LOA], -3.59 to +1.32) for spherical equivalent (SE) and -0.23 ± 0.53 D (LOA, -1.28 to +0.81) for the cylinder. Mean difference for the Retinomax K-plus2 before cycloplegia was -0.08 ± 0.58 D (LOA, -1.23 to +1.06) for SE and 0.03 ± 0.38 D (LOA, -0.72 to +0.78) for the cylinder; after cycloplegia -2.11 ± 1.64 D (LOA, -5.33 to +1.10) for SE and -0.06 ± 0.47 D (LOA, -0.98 to +0.86) for the cylinder. Sensitivity for detecting hyperopia >3.5 D with the plusoptiX S08 was 33.3%, the Retinomax before cycloplegia 31.0% and after cycloplegia 84.6% and high for detecting myopia, astigmatism, and anisometropia.nnnCONCLUSIONSnRetinomax K-plus2 and plusoptiX S08 have high sensitivity for the detection of myopia, astigmatism, and anisometropia compared to cycloplegic retinoscopy; however, when used without cycloplegia, hyperopia is underestimated.

Costs of different strategies for neonatal hearing screening: a modelling approach.

OBJECTIVE To compare the cost effectiveness of various strategies for neonatal hearing screening by estimating the cost per hearing impaired child detected. DESIGN Cost analyses with a simulation model, including a multivariate sensitivity analysis. Comparisons of the cost per child detected were made for: screening method (automated auditory brainstem response or otoacoustic emissions); number of stages in the screening process (two or three); target disorder (bilateral hearing loss or both unilateral and bilateral loss); location (at home or at a child health clinic). SETTING The Netherlands TARGET POPULATION All newborn infants not admitted to neonatal intensive care units. MAIN OUTCOME MEASURE Costs per child detected with a hearing loss of 40 dB or more in the better ear. RESULTS Costs of a three stage screening process in child health clinics are €39.0 (95% confidence interval 20.0 to 57.0) per child detected with automated auditory brainstem response compared with €25.0 (14.4 to 35.6) per child detected with otoacoustic emissions. A three stage screening process not only reduces the referral rates, but is also likely to cost less than a two stage process because of the lower cost of diagnostic facilities. The extra cost (over and above a screening programme detecting bilateral losses) of detecting one child with unilateral hearing loss is €1500–4000. With the currently available information, no preference can be expressed for a screening location. CONCLUSIONS Three stage screening with otoacoustic emissions is recommended. Whether screening at home is more cost effective than screening at a child health clinic needs further study.

DECIBEL study: Congenital cytomegalovirus infection in young children with permanent bilateral hearing impairment in the Netherlands

BACKGROUNDnA significant number of asymptomatic newborns infected with congenital cytomegalovirus (CMV) will present with permanent childhood hearing impairment (PCHI) during early childhood.nnnOBJECTIVESnTo investigate the role of congenital CMV infection in causing PCHI in the Netherlands, and assess the efficacy of two different hearing screening strategies and the developmental outcome following each strategy.nnnSTUDY DESIGNnWe included 192 children with PCHI at the age of 3-5 years, who were offered hearing screening in their first year of life. Dried blood spots from 171 children were available for CMV detection using real-time PCR. The results of eight previously tested samples were also available. Clinical baseline characteristics were collected from medical records and the Child Development Inventory was used to investigate the developmental outcome.nnnRESULTSnThe rate of congenital CMV among the 179 children was 8% (14/179) and 23% (9/39) among children with profound PCHI. Two of eight CMV-positive children with PCHI at the age of 3-5 years had passed the newborn hearing screening (NHS) test. Developmental outcome measures showed a significantly greater delay in language comprehension in children with both PCHI and congenital CMV infection (the largest in symptomatic children) than in the children with PCHI without congenital CMV infection.nnnCONCLUSIONSnCongenital CMV infection is important in the etiology of PCHI. Universal NHS is not a guarantee of normal hearing and development in childhood for children with congenital CMV infection. This is a problem which might be solved by universal congenital CMV screening.

Problem behavior of individuals with down syndrome in a nationwide cohort assessed in late adolescence

OBJECTIVEnTo assess problem behavior in adolescents with Down syndrome and examine the association with sex and severity of intellectual disability.nnnSTUDY DESIGNnCross-sectional data of a Dutch nationwide cohort of Down syndrome children aged 16-19 years were collected using a written parental questionnaire. Problem behavior was measured using the Child Behavior Checklist and compared with normative data. The degree of intellectual disability was determined using the Dutch Social competence rating scale.nnnRESULTSnThe response rate was 62.8% (322/513), and the mean age 18.3 years (SD ± 0.8). The total score for problem behavior was higher in adolescents with Down syndrome than in adolescents without Down syndrome (26.8 vs 16.5; P < .001). Overall, 51% of adolescents with Down syndrome had problem scores in the clinical or borderline range on 1 or more Child Behavior Checklist subscales; this is more than twice as high as adolescents without Down syndrome. Adolescents with Down syndrome had more internalizing problems than their counterparts without Down syndrome (14% and 9%, respectively, in the clinical range); the percentages for externalizing problems were almost equal (7% and 9%, respectively, in the clinical range). The highest problem scores in adolescents with Down syndrome were observed on the social problems and thought problems subscales (large to very large standardized differences). Male sex and/or more severe mental disabilities were associated with more behavioral problems.nnnCONCLUSIONSnSerious problem behavior is more prevalent in adolescents with Down syndrome. This demonstrates the need for a focus on general behavior improvement and on the detection and treatment of specific psychopathology in individuals with Down syndrome.

Causes of permanent childhood hearing impairment

The causes of Permanent Childhood Hearing Impairment (PCHI) are often quoted as being hereditary in 50%, acquired in 25%, and unknown in 25% of cases. Interest in the causes of PCHI has grown recently due to increasing diagnostic possibilities. We investigated the evidence for the reported distribution of these causes.