Anna M.H. Korver

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

CONTEXT Newborn 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. OBJECTIVE To 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. DESIGN, SETTING, AND PARTICIPANTS Between 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. MAIN OUTCOME MEASURES Performance (education and spoken and signed communication), development (general and language), and quality of life. RESULTS During 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. CONCLUSION Compared 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.

Awareness of congenital cytomegalovirus among doctors in the Netherlands

BACKGROUND Because of limited treatment options for congenital cytomegalovirus (CMV) infection, preventive strategies are important. Knowledge and awareness are essential for the success of preventive strategies. OBJECTIVES To investigate the knowledge of congenital CMV among doctors involved in mother and child care in the Netherlands. STUDY DESIGN A 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. RESULTS The 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. CONCLUSIONS The 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.

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

BACKGROUND A significant number of asymptomatic newborns infected with congenital cytomegalovirus (CMV) will present with permanent childhood hearing impairment (PCHI) during early childhood. OBJECTIVES To 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. STUDY DESIGN We 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. RESULTS The 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. CONCLUSIONS Congenital 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.

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.

Congenital cytomegalovirus infection in the Netherlands: Birth prevalence and risk factors

Congenital cytomegalovirus (CMV) infection is the most common congenital viral infection worldwide. The sequela encountered most frequently is hearing impairment, affecting approximately one out of five infants congenitally infected. Data on the birth prevalence and risk factors of congenital CMV infection in the Netherlands are scarce. The aim of this study was to determine the birth prevalence of congenital CMV in the Netherlands. A sample of 6,500 dried blood spots (DBS) from infants born in the Netherlands was tested anonymously for CMV DNA. The sample was stratified by the number of live births in different regions of the Netherlands of the year 2007. Additionally, on a regional level, risk factors for congenital CMV were analyzed. The birth prevalence of congenital CMV in the Netherlands was 0.54% (35/6,433, 95%CI 0.36–0.72). Congenital CMV infection was significantly higher in regions with more than 15% young children (0–5 years) compared with regions with a lower proportion of young children (OR 5.9, 95%CI 1.4–25.2). Congenital CMV infection was significantly higher in regions with more than 30% immigrants compared with regions with a lower proportion of immigrants (OR 2.2, 95%CI 1.1–4.6). This association was strongest for regions with more than 30% non‐Western immigrants (OR 3.3, 95%CI 1.5–7.5). Based on the knowledge of the natural history of congenital CMV infection, approximately 1,000 children are born with congenital CMV infection in the Netherlands annually, of whom eventually approximately 180 children (0.1% of all newborns) will be affected by long term sequelae, with hearing loss being the symptom encountered most frequently. J. Med. Virol. 83:1777–1782, 2011.

Rapid Genotyping of Cytomegalovirus in Dried Blood Spots by Multiplex Real-Time PCR Assays Targeting the Envelope Glycoprotein gB and gH Genes

ABSTRACT Genotyping of cytomegalovirus (CMV) is useful to examine potential differences in the pathogenicity of strains and to demonstrate coinfection with multiple strains involved in CMV disease in adults and congenitally infected newborns. Studies on genotyping of CMV in dried blood spots (DBS) are rare and have been hampered by the small amount of dried blood available. In this study, two multiplex real-time PCR assays for rapid gB and gH genotyping of CMV in DBS were developed. Validation of the assays with 39 CMV-positive plasma samples of transplant recipients and 21 urine specimens of congenitally infected newborns was successful in genotyping 100% of the samples, with gB1 and gB3 being the most prevalent genotypes. Multiple gB and gH genotypes were detected in 36% and 33% of the plasma samples, respectively. One urine sample from a newborn with symptomatic congenital CMV was positive for gB1 and gB2. DBS of congenitally infected newborns (n = 41) were tested using 9 μl of dried blood, and genotypes were detected in 81% (gB) and 73% (gH) of the samples, with gB3 being the most prevalent genotype. No clear association of specific genotypes with clinical outcome was observed. In conclusion, the CMV gB and gH PCR assays were found to be rapid, sensitive for detecting mixed infections, and suitable for direct usage on DBS. These assays are efficient tools for genotyping of CMV in DBS of congenitally infected newborns.

Early identification: Language skills and social functioning in deaf and hard of hearing preschool children.

OBJECTIVE Permanent childhood hearing impairment often results in speech and language problems that are already apparent in early childhood. Past studies show a clear link between language skills and the childs social-emotional functioning. The aim of this study was to examine the level of language and communication skills after the introduction of early identification services and their relation with social functioning and behavioral problems in deaf and hard of hearing children. STUDY DESIGN Nationwide cross-sectional observation of a cohort of 85 early identified deaf and hard of hearing preschool children (aged 30-66 months). METHODS Parents reported on their childs communicative abilities (MacArthur-Bates Communicative Development Inventory III), social functioning and appearance of behavioral problems (Strengths and Difficulties Questionnaire). Receptive and expressive language skills were measured using the Reynell Developmental Language Scale and the Schlichting Expressive Language Test, derived from the childs medical records. RESULTS Language and communicative abilities of early identified deaf and hard of hearing children are not on a par with hearing peers. Compared to normative scores from hearing children, parents of deaf and hard of hearing children reported lower social functioning and more behavioral problems. Higher communicative abilities were related to better social functioning and less behavioral problems. No relation was found between the degree of hearing loss, age at amplification, uni- or bilateral amplification, mode of communication and social functioning and behavioral problems. CONCLUSION These results suggest that improving the communicative abilities of deaf and hard of hearing children could improve their social-emotional functioning.

Cytomegalovirus DNA detection in dried blood spots and perilymphatic fluids from pediatric and adult cochlear implant recipients with prelingual deafness

BACKGROUND Congenital cytomegalovirus (CMV) infection is the leading cause of non-genetic congenital hearing loss. The contribution of congenital CMV to prelingual deafness and the pathophysiology is largely unknown. OBJECTIVE (1) To analyze the prevalence of congenital CMV among cochlear implant (CI) recipients with prelingual deafness. (2) To genotype CMV present in dried blood spots (DBS) and in the inner ear years after birth. STUDY DESIGN Children and adults with prelingual deafness who received a CI in 2010-2011 were included prospectively. Perilymphatic fluids were collected during CI surgery and, in the pediatric cases, DBS were retrieved for CMV DNA detection. Furthermore, a cohort of children with prelingual deafness who received a CI between 2003 and 2008 were included retrospectively. CMV detection in DBS and perilymph was followed by gB and gH genotyping. RESULTS Seventysix pediatric CI recipients were included. Seventy DBS were tested for CMV DNA, resulting in a prevalence of congenital CMV of 14% (10/70). Perilymphatic fluid was available from 29 pediatric CI recipients. One perilymph fluid, of a 21-month old girl with congenital CMV, asymptomatic at birth, was CMV DNA positive. The CMV strain in the perilymph was genotypically identical to the strain present in her DBS (gB1/gH2). Perilymph samples from 21 adult CI recipients were CMV DNA negative. CONCLUSIONS Our study stresses the important contribution of congenital CMV among pediatric CI recipients. Furthermore, our genotyping data support the hypothesis that CMV-related hearing loss is associated with ongoing viral replication in the inner ear up to years after birth.

Can You Hear What I Think? Theory of Mind in Young Children With Moderate Hearing Loss

Objectives: The first aim of this study was to examine various aspects of Theory of Mind (ToM) development in young children with moderate hearing loss (MHL) compared with hearing peers. The second aim was to examine the relation between language abilities and ToM in both groups. The third aim was to compare the sequence of ToM development between children with MHL and hearing peers. Design: Forty-four children between 3 and 5 years old with MHL (35 to 70 dB HL) who preferred to use spoken language were identified from a nationwide study on hearing loss in young children. These children were compared with 101 hearing peers. Children were observed during several tasks to measure intention understanding, the acknowledgement of the other’s desires, and belief understanding. Parents completed two scales of the child development inventory to assess expressive language and language comprehension in all participants. Objective language test scores were available from the medical files of children with MHL. Results: Children with MHL showed comparable levels of intention understanding but lower levels of both desire and belief understanding than hearing peers. Parents reported lower language abilities in children with MHL compared with hearing peers. Yet, the language levels of children with MHL were within the average range compared with test normative samples. A stronger relation between language and ToM was found in the hearing children than in children with MHL. The expected developmental sequence of ToM skills was divergent in approximately one-fourth of children with MHL, when compared with hearing children. Conclusion: Children with MHL have more difficulty in their ToM reasoning than hearing peers, despite the fact that their language abilities lie within the average range compared with test normative samples.

Early detection of hearing impairment reflected in better reading ability in teenage years

Commentary on : Pimperton H, Blythe H, Kreppner J, et al. The impact of universal newborn hearing screening on long-term literacy outcomes: a prospective cohort study. Arch Dis Child 2016;101:9–15[OpenUrl][1][Abstract/FREE Full Text][2]. Permanent congenital hearing impairment (PCHI) >40 dB HL in the better ear deprives children of language input necessary for speech and language development. When detected early by universal newborn hearing screening (UNHS) programmes, quickly followed by interventions to improve hearing, then speech and language, have better chances of developing normally.1 Reading ability reflects language development, and is essential for academic and employment prospects. Children who cannot hear find learning to read difficult without being able to recognise the sounds associated with written words. A small, but representative sample of the original British cohort published earlier by the same authors2 was enrolled … [1]: {openurl}?query=rft.jtitle%253DArch%2BDis%2BChild%26rft_id%253Dinfo%253Adoi%252F10.1136%252Farchdischild-2014-307516%26rft_id%253Dinfo%253Apmid%252F25425604%26rft.genre%253Darticle%26rft_val_fmt%253Dinfo%253Aofi%252Ffmt%253Akev%253Amtx%253Ajournal%26ctx_ver%253DZ39.88-2004%26url_ver%253DZ39.88-2004%26url_ctx_fmt%253Dinfo%253Aofi%252Ffmt%253Akev%253Amtx%253Actx [2]: /lookup/ijlink?linkType=ABST&journalCode=archdischild&resid=101/1/9&atom=%2Febmed%2F21%2F4%2F155.atom