Factors associated with nonword repetition skills in 4-to 6-year-old deaf and hard-of-hearing and typically hearing children.

Children identified as deaf and hard of hearing (DHH) exhibit an increased risk of speech and language difficulties. Nonword repetition (NWR) is a potential tool for identifying language difficulties in children with limited experience with the target language. In this study, we explored associations between auditory, linguistic, demographic and cognitive factors and NWR performance in DHH children and their typically hearing (TH) peers. We also examined the effect of the group on NWR outcomes when different factors were statistically controlled for. Our study included 68 4- to 6-year-old children: 18 with bilateral hearing aids (BiHAs), 18 with bilateral cochlear implants (BiCIs) and 32 with TH. The participants completed the Finnish Nonword Repetition Test comprising 24 test items with varying syllable lengths. The assessment methods also included tests of phonological, lexical, language comprehension and sentence repetition skills, along with a measure of nonverbal intelligence. The results showed that none of the auditory, linguistic, demographic and cognitive factors examined in the present study were correlated with NWR performance in the BiHA group, while significant correlations between NWR skills and linguistic abilities were observed in the BiCI group. The DHH children showed extraordinary difficulties in NWR compared to their TH peers, and these group differences remained after controlling for linguistic and demographic variables. The NWR task may be a potential clinical tool for identifying DHH children at risk for poor language outcomes and in need of speech and language intervention.

Monthly variation in univentricular heart and transposition of the great arteries - 10-year national population-based cohort study.

OBJECTIVE: Monthly variation in the total prevalence of the univentricular heart (UVH) and dextro-transposition of the great arteries (d-TGA) at gestational age 7 + 0 weeks was assessed to determine potential environmental factors, such as viral or bacterial infections, underlying the variation. STUDY DESIGN: The nationwide retrospective ten-year population-based cohort consisted of 592 733 births and 2764 terminated pregnancies due to fetal anomaly. The pre- or postnatally diagnosed cases of UVH (n = 440) and simple d-TGA (n = 127) from five national registers included live births, stillbirths, and pregnancy terminations due to fetal anomaly. We evaluated the variation in the monthly total prevalence of UVH and d-TGA at gestational age 7 + 0 weeks. The monthly variation of UVH and d-TGA was also compared with monthly variation in reported viral and bacterial infections. RESULTS: In the UVH and d-TGA, we observed significant monthly variation in total prevalence. However, we observed no correlations in the studied viral or bacterial infections and the number of cases. CONCLUSIONS: Assessing monthly variation in total prevalence at early pregnancy, including pregnancy terminations and stillbirths, and using first-trimester timing provides the most accurate information on the variation. The reasons for monthly variation remain unclear, but we observed no associations with specific viral or bacterial infections.

Perinatal and perioperative factors associated with mortality and an increased need for hospital care in infants with transposition of the great arteries: A nationwide 11-year population-based cohort.

INTRODUCTION: Newborn infants with transposition of the great arteries (d-TGA) need immediate care for an optimal outcome. This study comprised a nationwide 11-year population-based cohort of d-TGA infants, and assessed whether the implementation of a nationwide systematic fetal screening program, or other perinatal, or perioperative factors, are associated with mortality or an increased need for hospital care. MATERIAL AND METHODS: The national cohort consisted of all live-born infants with simple d-TGA (TGA ± small ventricular septal defect, n = 127) born in Finland during 2004-2014. Data were collected from six national registries. Prenatal diagnosis and perinatal and perioperative factors associated with mortality and length of hospitalization were evaluated. RESULTS: Preoperative mortality was 7.9%, and the total mortality was 8.7%. The prenatal detection rate increased after introducing systematic fetal anomaly screening from 5.0% to 37.7% during the study period (P < .0001), but the total mortality rate remained unchanged. All prenatally diagnosed infants (n = 27) survived. Lower gestational age (odds ratio 0.68, P = .012) and higher maternal age at birth (odds ratio 1.16, P = .036) were associated with increased mortality in multivariable analysis. Older infant age at time of operation (P = .002), longer aortic clamp time (P < .001), and higher maternal body mass index (P = .027) were associated with longer initial hospital stay. An extended need for hospital care during the first year of life was multi-factorial. CONCLUSIONS: In our cohort, none of the prenatally diagnosed d-TGA infants died. As a result of the limited prenatal detection rates, however, the sample size was insufficient to reach statistical significance. The d-TGA infants born with lower gestational age and to older mothers had increased mortality.

Congenital diaphragmatic hernia with heart defect has a high risk for hypoplastic left heart syndrome and major extra-cardiac malformations: 10-year national cohort from Finland.

INTRODUCTION: Congenital diaphragmatic hernia (CDH) has a well-known risk of congenital heart defects with poor prognosis. This study was conducted to determine the national total prevalence and prenatal detection rates of CDH with heart defects and its association with major extra-cardiac malformations and to further evaluate the impact of the heart defect severity on survival. MATERIAL AND METHODS: A 10-year national cohort was derived from four national registries, including live births, stillbirths, and terminations of pregnancy for fetal anomalies. The study cohort was sorted according to cardiac defect severity. RESULTS: The total prevalence of CDH with heart defects was 0.6/10 000 births and live birth prevalence 0.3/10 000 live births. Of 145 cases with CDH, 37 (26%) had a concurrent heart defect. The overall prenatal detection rate of heart defects was 41%. The total prevalence (483/10 000) and live birth prevalence (500/10 000) of hypoplastic left heart syndrome were 124 and 250 times higher than in the general population in Finland, respectively. Additional major extra-cardiac malformations were found in 68% of cases. The survival rate for CDH with major heart defects was 11 and 38% with minor heart defects. CONCLUSIONS: The total prevalence of hypoplastic left heart syndrome was significantly higher in CDH patients than in the general population in Finland. Prenatal detection rate for heart defects in CDH patients was 41%. Major extra-cardiac malformations were more common than previously reported. The prognosis of CDH with major heart defects remained poor.