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Highlights: [•] The proliferation of GBM cell lines displayed different sensitivities towards oligomycin or 2DG. [•] Differential sensitivities of GBM cell lines towards oligomycin were correlated with the activation of AMPKα. [•] Combining oligomycin and 2DG has drastic synergistic effects on suppressing cell growth and motility. [Copyright &y& Elsevier]

It is well established that permanent childhood hearing impairment (PCHI) has a detrimental impact on speech and language development. The past two decades have seen the gradual introduction of universal newborn hearing screening (UNHS) programmes coupled with early intervention programmes. We review studies that have capitalised on the advent of newborn hearing screening to assess the impact of early identifi cation of PCHI on language outcomes in deaf children. The research supports the conclusion that, in children with PCHI, newborn hearing screening and early identifi cation lead to beneficial effects on language development, with the most consistent evidence provided for links between early identifi cation of PCHI and positive language outcomes. Future research needs to encompass a wider range of outcomes and to assess the impact of UNHS in adolescents and young adults. [ABSTRACT FROM AUTHOR]

ABSTRACT. Objective. Posthemorrhagic ventricular dilation (PHVD) is a complication of intraventricular hemorrhage in preterm infants and is associated with a high risk of long-term disability. Furosemide and acetazolamide are used widely in the treatment of PHVD in the hope of avoiding the need for placement of a ventriculoperitoneal shunt, but these drugs have not been evaluated in a controlled trial. This article reports a multicenter, randomized, controlled trial designed to test the hypothesis that these drugs would reduce the rate of shunt placement (or death) and increase survival to 1 year of age without disability. Methods. Between 1992 and 1996, 177 infants who were less than 3 months past term and had ventricular width >4 mm above the 97th centile following intraventricular hemorrhage were assigned randomly to either standard therapy or standard therapy plus drug therapy with acetazolamide (100 mg/kg/d) plus furosemide (1 mg/kg/d). Infants who were enrolled in the trial had a median gestational age of 28.6 weeks and were enrolled at a mean postnatal age of 3.6 weeks. Forty-four percent were reported to have a cerebral parenchymal lesion on ultrasound scan at randomization. The primary outcome measure of death or shunt placement (known in all but 1 infant) occurred in 56 of 88 infants who were allocated to drug plus standard therapy compared with 46 of 88 who were allocated to standard therapy. The risk ratio was 1.23 (95% confidence interval: 0.95-1.59). Neurodevelopmental outcome information at a corrected age of 1 year (known in all but 3 of 149 surviving infants) included disability or neuromotor impairment in 54 of 67 infants (81%) who were allocated to drug plus standard therapy and 52 of 69 infants (66%) who were allocated to standard therapy. Seventy-two of 85 infants (85%) who were allocated to drug therapy either died or were disabled or impaired at 1 year compared with 62 of 89 infants (70%) who were treated with standard therapy (ris... [ABSTRACT FROM AUTHOR]

Background Survivors of childhood brain tumors or other acquired brain injury (ABI) are at risk of poor health-related quality of life (HRQoL); its valid and reliable assessment is essential to evaluate the effect of their illness on their lives. The aim of this review was to critically appraise psychometric properties of patient-reported outcome measures (PROMs) of HRQoL for these children, to be able to make informed decisions about the most suitable PROM for use in clinical practice. Methods We searched MEDLINE, EMBASE, and PsycINFO for studies evaluating measurement properties of HRQoL PROMs in children treated for brain tumors or other ABI. Methodological quality of relevant studies was evaluated using the consensus-based standards for the selection of health status measurement instruments checklist. Results Eight papers reported measurement properties of 4 questionnaires: Health Utilities Index (HUI), PedsQL Core and Brain Tumor Modules, and Child and Family Follow-up Survey (CFFS). Only the CFFS had evidence of content and structural validity. It also demonstrated good internal consistency, whereas both PedsQL modules had conflicting evidence regarding this. Conflicting evidence regarding test-retest reliability was reported for the HUI and PedsQL Core Module only. Evidence of measurement error/precision was favorable for HUI and CFFS and absent for both PedsQL modules. All 4 PROMs had some evidence of construct validity/hypothesis testing but no evidence of responsiveness to change. Conclusions Valid and reliable assessment is essential to evaluate impact of ABI on young lives. However, measurement properties of PROMs evaluating HRQoL appropriate for this population require further evaluation, specifically construct validity, internal consistency, and responsiveness to change. [ABSTRACT FROM AUTHOR]

We examined phonological recoding during silent sentence reading in teenagers with a history of dyslexia and their typically developing peers. Two experiments are reported in which participants' eye movements were recorded as they read sentences containing correctly spelled words (e.g., church), pseudohomophones (e.g., cherch), and spelling controls (e.g., charch). In Experiment 1 we examined foveal processing of the target word/nonword stimuli, and in Experiment 2 we examined parafoveal pre-processing. There were four participant groups–older teenagers with a history of dyslexia, older typically developing teenagers who were matched for age, younger typically developing teenagers who were matched for reading level, and younger teenagers with a history of dyslexia. All four participant groups showed a pseudohomophone advantage, both from foveal processing and parafoveal pre-processing, indicating that teenagers with a history of dyslexia engage in phonological recoding for lexical identification during silent sentence reading in a comparable manner to their typically developing peers. [ABSTRACT FROM AUTHOR]

Abstract: There has been considerable variability within the literature concerning the extent to which deaf/hard of hearing individuals are able to process phonological codes during reading. Two experiments are reported in which participants’ eye movements were recorded as they read sentences containing correctly spelled words (e.g., church), pseudohomophones (e.g., cherch), and spelling controls (e.g., charch). We examined both foveal processing and parafoveal pre‐processing of phonology for three participant groups—teenagers with permanent childhood hearing loss (PCHL), chronological age‐matched controls, and reading age‐matched controls. The teenagers with PCHL showed a pseudohomophone advantage from both directly fixated words and parafoveal preview, similar to their hearing peers. These data provide strong evidence for phonological recoding during silent reading in teenagers with PCHL. [ABSTRACT FROM AUTHOR]

Background: Sleep-disordered breathing (SDB) is often co-morbid with conductive hearing loss in early childhood due to a shared aetiology of adenotonsillar hypertrophy. Hearing loss is independently associated with impairment of executive function and behavioural difficulties. We hypothesised that these impairments in children with SDB may be mediated through hearing loss.Methods: Fifty-eight children including 37 snorers awaiting adenotonsillectomy and 21 healthy non-snoring controls, aged 3-5 years, were assessed with pure tone audiometry, Strengths and Difficulties (SDQ), Behaviour Rating of Executive Function (BRIEF-P), and Childhood Middle Ear Disease and Hearing questionnaires. Polysomnography in snoring children generated an obstructive apnoea/hypopnea index (OAHI). Two regression models examined the effect of SDB and the mediating impact of hearing loss on BRIEF and SDQ.Results: Snoring children had significantly poorer hearing, greater past exposure to hearing loss, and higher total SDQ and BRIEF-P scores than non-snoring controls. The first regression model, including all children, demonstrated that the impact of snoring on BRIEF_P, but not SDQ, was entirely mediated by a history of hearing loss exposure but not same-day audiometry. The second model examined snoring children only, categorising the group into 12 with obstructive sleep apnoea (OSA) (OAHI ≥ 5) and 25 without OSA. OSA had a direct effect on SDQ scores, but this was not mediated by a history of hearing loss.Conclusion: In early childhood, conductive hearing loss mediates the relationship between SDB, irrespective of severity, and parent report of executive function but not behaviour. Treatment of hearing loss in pre-school SDB might improve executive function. [ABSTRACT FROM AUTHOR]

Background: Children with bilateral permanent hearing impairment often have impaired language and speech abilities. However, the effects of universal newborn screening for permanent bilateral childhood hearing impairment and the effects of confirmation of hearing impairment by nine months of age on subsequent verbal abilities are uncertain. Methods: We studied 120 children with bilateral permanent hearing impairment identified from a large birth cohort in southern England, at a mean of 7.9 years of age. Of the 120 children, 61 were born during periods with universal newborn screening and 57 had hearing impairment that was confirmed by nine months of age. The primary outcomes were language as compared with nonverbal ability and speech expressed as z scores (the number of standard deviations by which the score differed from the mean score among 63 age-matched children with normal hearing), adjusted for the severity of the hearing impairment and for maternal education. Results: Confirmation of hearing impairment by nine months of age was associated with higher adjusted mean z scores for language as compared with nonverbal ability (adjusted mean difference for receptive language, 0.82; 95 percent confidence interval, 0.31 to 1.33; and adjusted mean difference for expressive language, 0.70; 95 percent confidence interval, 0.13 to 1.26). Birth during periods with universal newborn screening was also associated with higher adjusted z scores for receptive language as compared with nonverbal ability (adjusted mean difference, 0.60; 95 percent confidence interval, 0.07 to 1.13), although the z scores for expressive language as compared with nonverbal ability were not significantly higher. Speech scores did not differ significantly between those who were exposed to newborn screening or early confirmation and those who were not. Conclusions: Early detection of childhood hearing impairment was associated with higher scores for language but not for speech in midchildhood. N Engl J Med 2006;354:2131-41. [ABSTRACT FROM AUTHOR]

We aimed to investigate the incidence of false alarms that occurred with the pass/fail criteria used in a published series of neonatal hearing screening programmes, as a function of age. We analysed the database of 19137 normally hearing babies (38274 ears) tested in the Wessex Universal Neonatal Hearing Screening Project. Otoacoustic emissions were recorded prior to discharge from maternity units, using IL088 equipment. We assessed the pass/fail rate using the Wessex criteria and 10 other pass/fail criteria published in the literature. Using Pearson's correlation coefficient, a statistically significant correlation between signal-to-noise ratio at each of the frequency bands 1, 2, 3, 4 and 5 kHz and babies' age in hours at the 0.01 level was identified. The correlation was also significant (0.01 level) between age and frequency reproducibility in each of the bands at 1, 2, 3, 4 and 5 kHz as well as the whole reproducibility. The number of false alarms reduced significantly after the first 24 h of life with all the criteria examined. We conclude that in the first hours after birth due to insufficient maturation of the otoacoustic emission, there is a high rate of false alarms. This increase in the false alarm rate, whilst dependent on the criteria used, occurs with all criteria. This leads to the consideration of whether the establishment of age-dependent pass/fail criteria could reduce the false alarm rate and the subsequent strain on diagnostic centres. [ABSTRACT FROM AUTHOR]

The amplitude values of transient-evoked otoacoustic emissions, recorded from a large sample of neonates, were used to examine the asymmetry between ears tested and the differences due to the sex of the subject. Whilst the sex difference, with females having larger responses than males, has been a consistent finding in previous reports, the right/left ear difference, with the right ear giving a larger response than the left, has produced variable results that differed between laboratories. In this study, the sex difference was confirmed with females giving a 1.2 dB greater response than males. It was not affected by the age of the neonate. A significant effect of test order was found. The measured right/left difference was enhanced when the right ear was tested first but was diminished when the left ear was tested first. If the left ear is tested first then the measured right/left difference would be about 0.5 dB whereas, if the right ear is tested first, the measured right/left difference would be about 1.5 dB. When male/female comparisons were made for right and left ears separately and for the same ear tested first, the sex differences were the same for all four conditions. The sex and right/left differences have been confirmed as statistically significant effects and the order effect could explain the discrepancies and variability of the right/left differences reported in the literature. [Copyright &y& Elsevier]

The methodological factors involved in screening neonates for hearing loss, using transient-evoked otoacoustic emissions (TEOAEs) and automated auditory brainstem responses, have been evaluated from a large sample of neonates. The risk factors, commonly used to select babies for a targeted screen, have very little correlation with failing TEOAE testing. The parameters used to determine passing or failing the TEOAE test and the false alarm rate change markedly with age in the first few days of life as, of course, did the percentage of babies who failed the test. The stimulus level used was the default setting for the Otodynamics equipment but the stimulus level measured in the ear canal decreased over the first 140 h of life. It is thought that this reflects the impedance changes in outer and middle ears and possible changes in middle ear dynamics. The methodological variables investigated here can illuminate some of the differences in previous reports of neonatal screening, in particular the reported hit and false alarm rates. [Copyright &y& Elsevier]

Objective: To determine the underlying etiologies in a contemporary cohort of infants with infantile spasms and to examine response to treatment. Methods: Identification of the underlying etiology and response to treatment in 377 infants enrolled in a clinical trial of the treatment of infantile spasms between 2007 and 2014 using a systematic review of history, examination, and investigations. They were classified using the pediatric adaptation of International Classification of Diseases, Tenth Revision (ICD‐10). Results: A total of 219 of 377 (58%) had a proven etiology, of whom 128 (58%) responded, 58 of 108 (54%) were allocated hormonal treatment, and 70 of 111 (63%) had combination therapy. Fourteen of 17 (82%, 95% confidence interval [CI] 59% to 94%) infants with stroke and infarct responded (compared to 114 of 202 for the rest of the proven etiology group (56%, 95% CI 48% to 62%, chi‐square 4.3, P = .037): the better response remains when treatment allocation and lead time are taken into account (odds ratio 5.1, 95% CI 1.1 to 23.6, P = .037). Twenty of 37 (54%, 95% CI 38% to 70%) infants with Down syndrome had cessation of spasms compared to 108 of 182 (59%, 95% CI 52% to 66%, chi‐square 0.35, P = .55) for the rest of the proven etiology group. The lack of a significant difference remains after taking treatment modality and lead‐time into account (odds ratio 0.8, 95% CI 0.4 to 1.7, P = .62). In Down syndrome infants, treatment modality did not appear to affect response: 11 of 20 (55%) allocated hormonal therapy responded, compared to 9 of 17 (53%) allocated combination therapy. Significance: This classification allows easy comparison with other classifications and with our earlier reports. Stroke and infarct have a better outcome than other etiologies, whereas Down syndrome might not respond to the addition of vigabatrin to hormonal treatment. [ABSTRACT FROM AUTHOR]

Background: Infantile spasms constitutes a severe infantile epilepsy syndrome that is difficult to treat and has a high morbidity. Hormonal therapies or vigabatrin are the most commonly used treatments. We aimed to assess whether combining the treatments would be more effective than hormonal therapy alone.Methods: In this multicentre, open-label randomised trial, 102 hospitals (Australia [three], Germany [11], New Zealand [two], Switzerland [three], and the UK [83]) enrolled infants who had a clinical diagnosis of infantile spasms and a hypsarrhythmic (or similar) EEG no more than 7 days before enrolment. Participants were randomly assigned (1:1) by a secure website to receive hormonal therapy with vigabatrin or hormonal therapy alone. If parents consented, there was an additional randomisation (1:1) of type of hormonal therapy used (prednisolone or tetracosactide depot). Block randomisation was stratified for hormonal treatment and risk of developmental impairment. Parents and clinicians were not masked to therapy, but investigators assessing electro-clinical outcome were masked to treatment allocation. Minimum doses were prednisolone 10 mg four times a day or intramuscular tetracosactide depot 0·5 mg (40 IU) on alternate days with or without vigabatrin 100 mg/kg per day. The primary outcome was cessation of spasms, which was defined as no witnessed spasms on and between day 14 and day 42 from trial entry, as recorded by parents and carers in a seizure diary. Analysis was by intention to treat. The trial is registered with The International Standard Randomised Controlled Trial Number (ISRCTN), number 54363174, and the European Union Drug Regulating Authorities Clinical Trials (EUDRACT), number 2006-000788-27.Findings: Between March 7, 2007, and May 22, 2014, 766 infants were screened and, of those, 377 were randomly assigned to hormonal therapy with vigabatrin (186) or hormonal therapy alone (191). All 377 infants were assessed for the primary outcome. Between days 14 and 42 inclusive no spasms were witnessed in 133 (72%) of 186 patients on hormonal therapy with vigabatrin compared with 108 (57%) of 191 patients on hormonal therapy alone (difference 15·0%, 95% CI 5·1-24·9, p=0·002). Serious adverse reactions necessitating hospitalisation occurred in 33 infants (16 on hormonal therapy alone and 17 on hormonal therapy with vigabatrin). The most common serious adverse reaction was infection occurring in five infants on hormonal therapy alone and four on hormonal therapy with vigabatrin. There were no deaths attributable to treatment.Interpretation: Hormonal therapy with vigabatrin is significantly more effective at stopping infantile spasms than hormonal therapy alone. The 4 week period of spasm cessation required to achieve a primary clinical response to treatment suggests that the effect seen might be sustained, but this needs to be confirmed at the 18 month follow-up.Funding: The Castang Foundation, Bath Unit for Research in Paediatrics, National Institute of Health Research, the Royal United Hospitals Bath NHS Foundation Trust, the BRONNER-BENDUNG Stifung/Gernsbach, and University Children's Hospital Zurich. [ABSTRACT FROM AUTHOR]

Objective: To determine whether the benefits of universal newborn hearing screening (UNHS) seen at age 8 years persist through the second decade.Design: Prospective cohort study of a population sample of children with permanent childhood hearing impairment (PCHI) followed up for 17 years since birth in periods with (or without) UNHS.Setting: Birth cohort of 100 000 in southern England.Participants: 114 teenagers aged 13-19 years, 76 with PCHI and 38 with normal hearing. All had previously their reading assessed aged 6-10 years.Interventions: Birth in periods with and without UNHS; confirmation of PCHI before and after age 9 months.Main Outcome Measure: Reading comprehension ability. Regression modelling took account of severity of hearing loss, non-verbal ability, maternal education and main language.Results: Confirmation of PCHI by age 9 months was associated with significantly higher mean z-scores for reading comprehension (adjusted mean difference 1.17, 95% CI 0.36 to 1.97) although birth during periods with UNHS was not (adjusted mean difference 0.15, 95% CI -0.75 to 1.06). The gap between the reading comprehension z-scores of teenagers with early compared with late confirmed PCHI had widened at an adjusted mean rate of 0.06 per year (95% CI -0.02 to 0.13) during the 9.2-year mean interval since the previous assessment.Conclusions: The benefit to reading comprehension of confirmation of PCHI by age 9 months increases during the teenage years. This strengthens the case for UNHS programmes that lead to early confirmation of permanent hearing loss.Trial Registration Number: ISRCTN03307358. [ABSTRACT FROM AUTHOR]

Background We identified child-related determinants of health-related quality of life (HRQoL) in children aged 8–14 years who were treated for 2 common types of pediatric brain tumors. Methods Questionnaire measures of HRQoL and psychometric assessments were completed by 110 children on 3 occasions over 24 months. Of these 110, 72 were within 3 years of diagnosis of a cerebellar tumor (37 standard-risk medulloblastoma, 35 low-grade cerebellar astrocytoma), and 38 were in a nontumor group. HRQoL, executive function, health status, and behavioral difficulties were also assessed by parents and teachers as appropriate. Regression modeling was used to relate HRQoL z scores to age, sex, socioeconomic status, and 5 domains of functioning: Cognition, Emotion, Social, Motor and Sensory, and Behavior. Results HRQoL z scores were significantly lower after astrocytoma than those in the nontumor group and significantly lower again in the medulloblastoma group, both by self-report and by parent-report. In regression modeling, significant child-related predictors of poorer HRQoL z scores by self-report were poorer cognitive and emotional function (both z scores) and greater age (years) at enrollment (B = 0.038, 0.098, 0.136, respectively). By parent-report, poorer cognitive, emotional and motor or sensory function (z score) were predictive of lower subsequent HRQoL of the child (B = 0.043, 0.112, 0.019, respectively), while age at enrollment was not. Conclusions Early screening of cognitive and emotional function in this age group, which are potentially amenable to change, could identify those at risk of poor HRQoL and provide a rational basis for interventions to improve HRQoL. [ABSTRACT FROM PUBLISHER]

Aim We aimed to investigate the relationship between movement disorders, changes on brain magnetic resonance imaging ( MRI), and vigabatrin therapy in children with infantile spasms. Method Retrospective review and brain MRI analysis of children enrolled in the International Collaborative Infantile Spasms Study ( ICISS) who developed a movement disorder on vigabatrin therapy. Comparisons were made with controls within ICISS who had no movement disorder. Results Ten of 124 infants had a movement disorder and in eight it had developed on vigabatrin therapy. Two had a movement disorder that resolved on dose-reduction of vigabatrin, one had improvement on withdrawing vigabatrin, two had resolution without any dose change, and in three it persisted despite vigabatrin withdrawal. The typical brain MRI changes associated with vigabatrin therapy were noted in two infants. Ten control infants were identified. Typical MRI changes noted with vigabatrin were noted in three controls. Interpretation It is possible that in two out of eight cases, vigabatrin was associated with the development of a movement disorder. In six out of eight cases a causal relationship was less plausible. The majority of infants treated with vigabatrin did not develop a movement disorder. MRI changes associated with vigabatrin do not appear to be specifically related to the movement disorder. [ABSTRACT FROM AUTHOR]

Summary [ABSTRACT FROM AUTHOR]

To determine if the benefit of early confirmation of permanent childhood hearing impairment (PCHI) on children's receptive language development is associated with fewer behavioural problems. Follow-up of a total population cohort of 120 children with PCHI of moderate or greater severity (≥40 decibels relative to hearing threshold level) (67 males, 53 females; mean age 7y 11mo, range 5y 5mo-11y 8mo) and 63 hearing children (37 males, 26 females; mean age 8y 1mo, range 6y 4mo-9y 10mo). The main outcome measures were the Strengths and Difficulties Questionnaire (SDQ) completed by teachers and parents and the Vineland Adaptive Behaviour Scales (VABS) which are completed on the basis of a parental interview. Children with PCHI had lower standard scores than hearing children on the Daily Living Skills ( p=0.001) and the Socialisation ( p=0.001) scales of the VABS. They had significantly higher Total Behaviour Problem scores on the parent-rated ( p=0.002) and teacher-rated SDQ ( p=0.03). Children for whom PCHI was confirmed by 9 months did not have significantly fewer problems on the behavioural measures than those confirmed after that age ( p=0.635 and p=0.196). Early confirmation has a beneficial effect on receptive language development but no significant impact in reducing behavioural problems in children with PCHI. [ABSTRACT FROM AUTHOR]

To examine the underlying etiology of infantile spasms from the United Kingdom Infantile Spasms Study (UKISS), using the pediatric adaptation of ICD 10. Infants were enrolled in a randomized controlled trial or a parallel epidemiologic study. Etiological information included history, examination, and investigations. The infants were classified as proven etiology, if a neurologic disease was identified; as no identified etiology, if no neurologic disease was identified; and as not fully investigated, if a major piece of information was missing. Proven etiology was subclassified using the pediatric adaptation of ICD 10. The results were then examined to identify further methods of classification. Of 207 infants, 127 (61%) had proven etiology, 68 (33%) had no identified etiology, and 12 (6%) were not fully investigated. Etiologies were prenatal in 63, perinatal in 38, postnatal in 8, and 18 other. The most common etiologies were: hypoxic-ischemic encephalopathy (HIE) 21 (10%), chromosomal 16 (8%), malformations 16 (8%), stroke 16 (8%), tuberous sclerosis complex (TSC) 15 (7%), and periventricular leukomalacia or hemorrhage 11 (5%). The remaining 32 etiologies were all individually uncommon. Response to treatment is given for individual etiologies. Our method of classification allows the reporting of results by individual diseases, disease groups, or categories and is structured and clear. It avoids the use of poorly defined terms such as symptomatic and cryptogenic. It can adapt to new neurologic diseases, such as gene defects, and can be used for comparison of different groups of infants, thereby aiding meta-analysis. [ABSTRACT FROM AUTHOR]

OBJECTIVE. The objective of this study was to describe the health status and health-related quality of life preference-based outcomes of children with diagnosed bilateral permanent childhood hearing impairment and a comparison group of English-speaking children with normal hearing. METHODS. We studied 120 children who were aged 7 to 9 years and had bilateral permanent childhood hearing impairment of moderate or greater severity, identified from a cohort of 156 733 children who were born in 8 districts of southern England, and 63 English-speaking children with normal hearing and the same place of birth and age at assessment. Principal caregivers were interviewed by using the Health Utilities Index Mark III questionnaire for proxy-assessed usual health status assessment. Levels of function within each of the 8 attributes of the Health Utilities Index Mark III (cognition, vision, hearing, speech, ambulation, dexterity, emotion, and pain) were recorded. RESULTS. Bilateral permanent childhood hearing impairment is associated with significantly increased proportions of suboptimal levels of function and significantly lower single-attribute utility scores in 6 of the 8 attributes of the Health Utilities Index Mark III: vision, hearing, speech, ambulation, dexterity, and cognition. Compared with the children with normal hearing, the mean multiattribute utility score for the children with hearing impairment was significantly lower for both the whole group and the moderate, severe, and profound severity subgroups. The differences in the distributions of the multiattribute utility scores between the children with hearing impairment as a group and the children with normal hearing and between each of the severity subgroups and the children with normal hearing all were statistically significant. CONCLUSIONS. This study provides rigorous evidence of an association between bilateral permanent childhood hearing impairment and diminished health status and health-related quality of life preference-based outcomes during midchildhood. [ABSTRACT FROM AUTHOR]

Background: Children with bilateral permanent hearing impairment often have impaired language and speech abilities. However, the effects of universal newborn screening for permanent bilateral childhood hearing impairment and the effects of confirmation of hearing impairment by nine months of age on subsequent verbal abilities are uncertain.Methods: We studied 120 children with bilateral permanent hearing impairment identified from a large birth cohort in southern England, at a mean of 7.9 years of age. Of the 120 children, 61 were born during periods with universal newborn screening and 57 had hearing impairment that was confirmed by nine months of age. The primary outcomes were language as compared with nonverbal ability and speech expressed as z scores (the number of standard deviations by which the score differed from the mean score among 63 age-matched children with normal hearing), adjusted for the severity of the hearing impairment and for maternal education.Results: Confirmation of hearing impairment by nine months of age was associated with higher adjusted mean z scores for language as compared with nonverbal ability (adjusted mean difference for receptive language, 0.82; 95 percent confidence interval, 0.31 to 1.33; and adjusted mean difference for expressive language, 0.70; 95 percent confidence interval, 0.13 to 1.26). Birth during periods with universal newborn screening was also associated with higher adjusted z scores for receptive language as compared with nonverbal ability (adjusted mean difference, 0.60; 95 percent confidence interval, 0.07 to 1.13), although the z scores for expressive language as compared with nonverbal ability were not significantly higher. Speech scores did not differ significantly between those who were exposed to newborn screening or early confirmation and those who were not.Conclusions: Early detection of childhood hearing impairment was associated with higher scores for language but not for speech in midchildhood. [ABSTRACT FROM AUTHOR]

Summary: Background: Infantile spasms is a severe infantile seizure disorder that is difficult to treat and has a high morbidity. Absence of spasms on days 13 and 14 after randomisation is more common in infants allocated hormone treatments than in those allocated vigabatrin. We sought to assess whether early control of spasms is associated with improved developmental or epilepsy outcomes. Methods: Infants enrolled in the United Kingdom Infantile Spasms Study (UKISS) were randomly assigned hormone treatment (n=55) or vigabatrin (n=52) and were followed up until clinical assessment at 12–14 months of age. We assessed neurodevelopment with the Vineland adaptive behaviour scales (VABS) at 14 months of age on an intention to treat basis. Findings: Of 107 infants enrolled, five died and 101 survivors reached both follow-up assessments. Absence of spasms at final clinical assessment (hormone 41/55 [75%] vs vigabatrin 39/51 [76%]) was similar in each treatment group (difference 1·9%, 95% CI −18·3% to 14·4%; χ2=0·05; p=0·82). Mean VABS score did not differ significantly (hormone 78·6 [SD 16·8] vs vigabatrin 77·5 [SD 12·7]; difference 1·0, 95% CI −4·9 to 7·0; t99 =0·35, p=0·73). In infants with no identified underlying aetiology, the mean VABS score was higher in those allocated hormone treatment than in those allocated vigabatrin (88·2 [17·3] vs 78·9 [14·3]; difference 9·3, 95% CI 1·2 to 17·3; t95 =2·28, p=0·025). Interpretation: Hormone treatment controls spasms better than does vigabatrin initially, but not at 12–14 months of age. Better initial control of spasms by hormone treatment in those with no identified underlying aetiology may lead to improved developmental outcome. [Copyright &y& Elsevier]