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Hearing impairment (HI) in children with a congenital or early onset is explained by genetic and environmental factors. Genetic factors are regarded as responsible for more than half of the cases of congenital HI and environmental factors are responsible for another 14-30%, but the etiology is still unknown in 20-40% [1]. Regarding the genetic causes, 30% are considered syndrome related, whereas 70% non-syndromic. Congenital infections and neonatal trauma may explain 20-30%. Without a well-run vaccination program, congenital rubella syndrome remains the most important cause of acquired infectious congenital HI, whereas in more developed areas, congenital cytomegalovirus (cCMV) has become the most common intrauterine infection. cCMV may cause 20% of congenital HI, but it is also responsible for late onset and progressive HI. Development, including advanced MRI, cellular biology and genetic analysis has within a few years provided diagnostic advancements in the etiology of HI.

Supplemental Digital Content is available in the text., Objectives: Early detection of neonatal hearing impairment moderates the negative effects on speech and language development. Universal neonatal hearing screening protocols vary in tests used, timing of testing and the number of stages of screening. This study estimated the cost-effectiveness of various protocols in the preparation of implementation of neonatal hearing screening in Albania. Design: A micro-simulation model was developed using input on demography, natural history of neonatal hearing impairment, screening characteristics and treatment. Parameter values were derived from a review of the literature and expert opinion. We simulated multiple protocols using otoacoustic emissions (OAE) and automated auditory brainstem response (aABR), varying the test type, timing and number of stages. Cost-effectiveness was analyzed over a life-time horizon. Results: The two best protocols for well infants were OAE followed by aABR (i.e., two-stage OAE-aABR) testing in the maternity ward and single-aABR testing. Incremental cost-effectiveness ratios were €4181 and €78,077 per quality-adjusted life-year gained, respectively. Single-aABR screening led to more cases being detected compared to a two-stage screening program. However, it also resulted in higher referral rates, which increased the total costs of diagnostics. Multi-staged screening decreased referral rates but may increase the number of missed cases due to false-negative test results and nonattendance. Conclusions: Only the 2-stage OAE-aABR (maternity ward) protocol was below the willingness-to-pay threshold of €10,413 for Albania, as suggested by the World Health Organization, and was found to be cost-effective. This study is among the few to assess neonatal hearing screening programs over a life-time horizon and the first to predict the cost-effectiveness of multiple screening scenarios.

Objective: To inventory provision and features of childhood hearing screening after the newborn period (CHS), primarily in Europe. Design: From each participating country or region, experts provided information through an extensive questionnaire: implementation year, age at screening, test method, pass criteria, screening location, screener profession, and quality indicators: coverage, referral, follow-up and detection rates, supplemented by literature sources. Study sample: Forty-two European countries or regions, plus Russia, Malawi, Rwanda, India, and China. Results: CHS was performed universally with pure-tone audiometry screening (PTS) in 17 countries or regions, whereas non-universal CHS was performed in eight with PTS or whisper tests. All participating countries with universal PTS had newborn hearing screening. Coverage rate was provided from three countries, detection rate from one, and referral and follow-up rate from two. In four countries, universal PTS was performed at two ages. Earliest universal PTS was performed in a (pre)school setting by nurses (n = 9, median age: 5 years, range: 3–7), in a healthcare setting by doctors and nurses (n = 7, median age: 4.5 years, range: 4–7), or in both (n = 1). Conclusions: Within universal CHS, PTS was mostly performed at 4–6 years by nurses. Insufficient collection of data and monitoring with quality indicators impedes evaluation of screening.

Universal newborn hearing screening (UNHS) contributes to the early diagnosis of hearing loss. However, not all permanent pediatric hearing impairments can be identified by UNHS.To investigate children who have successfully passed the UNHS, but have later-onset hearing loss at an early stage.UNHS of children, was reviewed retrospectively from databases at Karolinska University Hospital, Sweden. Gender, age, the reason for contact, the first contact and the most recent audiogram, the hearing diagnosis, the degree of hearing loss when they were enrolled in hearing rehabilitation, and the hearing aids they used were analysed.63 children who had passed the UNHS at birth and were diagnosed with a hearing impairment at a later stage were included in the study. The average age was 3.3 and 3.9 years old when the children were diagnosed and were finally enrolled in the hearing habilitation, respectively. The reasons for diagnostic evaluation of a suspected hearing loss at present study are preschool hearing tests at the Child Health Care Centres, parents suspect, and/or delayed speech and language development.Our findings suggest that a passed UNHS does not exclude a future delayed onset of hearing loss, particularly in children with risk factors.

Objectives: To assess the performance of newborn hearing screening (NHS) programmes, through selected quality measures and their relationship to protocol design. Design: NHS coverage, referral, follow-up and detection rates were aggregated. Referral rates were compared to age at screening step 1, number of steps, and test method: OAE or aABR. Study sample: A questionnaire on existing hearing screening was completed by experts from countries in Europe, plus Russia, Malawi, Rwanda, India and China. Results: Out of 47 countries or regions, NHS coverage rates were reported from 26, referral rates from 23, follow up from 12 and detection rates from 13. Median coverage rate for step 1 was 96%. Referral rate from step 1 was 6–22% where screening may be performed 24 h, and 4% for >72 h. Referral rates to diagnostic assessment averaged 2.1% after one to two steps using OAE only, 1.7% after two steps including aABR, and 0.8% after three to four steps including aABR. Median detection rate for bilateral permanent hearing impairment ≥40dB was 1 per 1000 infants. Conclusion: Referral rates were related to age, test method and number of screening steps. Quality measures were not available for many NHS programmes.

Objectives: The primary aim was to examine how event-related potentials (ERPs) and mismatch negativity (MMN) change and develop over time among children with hearing loss (HL) using hearing aids (HAs) or cochlear implants (CIs). Children with normal hearing (NH) were tested as a reference group. Methods: This three-year follow-up study included 13 children with sensorineural HL (SNHL); 7 children using bilateral HAs and 6 children using CIs; and 10 children with NH as a reference group. ERPs were recorded at baseline and after three years. At time for the original study the children were approximately 5-8 years old and at the follow-up study 8-11 years old. ERP recordings and data processing were identical in both sessions. A standard stimulus alternated with five different deviants (gap, intensity, pitch, location and duration), presented in a pseudorandom sequence, thus following the multi-feature paradigm, Optimum-1. MMN was calculated from the average ERP of each deviant minus the standard stimuli. Repeated measures ANOVA was used for the statistical analyses and the results were based on samples within a specific time interval; 80-224 ms. Results: There was a statistically significant difference in the obligatory responses between the NH and HA groups at baseline, but this difference disappeared after three years in our follow-up study. The children with HA also showed a significant difference in mean ERP at baseline compared to follow-up, and significant differences between the deviants at follow-up but not at baseline. This suggests an improvement over time among the children with HAs. On the other hand, the children with CIs did not differ from the NH children at baseline, but after three years their mean ERP was significantly lower compared to both the children with HA and NH, indicating a reduced development of the central auditory system in this age span among the children with CIs. Regarding MMN, there was an interaction between the duration deviant and time for the children with HA, also indicating a possible improvement over time among the HA children. Conclusions: This three-year follow-up study shows neurophysiological differences between children with HL and children with NH. The results suggest a delay in the central auditory processing among the HA children compared to children with NH, but a possible catch-up, over time, and this potential may be worth to be utilized. Regarding the CI children, similar improvement in this age span is missing, meaning there are differences between the subgroups of children with HL, i.e. the children with HAs vs. CIs. The results highlight the importance of distinguishing between subgroups of children with HL in further research. Funding Agencies|Swedish Research Council for Working Life and Social Sciences (Forskningsradet for Arbetsliv och Social Vetenskap)

Newborn hearing screening (NHS) varies regarding number and type of tests, location, age, professionals and funding. We compared the provision of existing screening programmes.A questionnaire containing nine domains: demography, administration, existing screening, coverage, tests, diagnosis, treatment, cost and adverse effects, was presented to hearing screening experts. Responses were verified. Clusters were identified based on number of screening steps and use of OAE or aABR, either for all infants or for well and high-risk infants (dual-protocol).Fifty-two experts completed the questionnaire sufficiently: 40 European countries, Russia, Malawi, Rwanda, India and China.It took considerable effort to find experts for all countries with sufficient time and knowledge. Data essential for evaluation are often not collected. Infants are first screened in maternity wards in most countries. Human development index and health expenditure were high among countries with dual protocols, three screening steps, including aABR, and low among countries without NHS and countries using OAE for all infants. Nationwide implementation of NHS took 6 years, on average.The extent and complexity of NHS programmes are primarily related to health expenditure and HDI. Data collection should be improved to facilitate comparison of NHS programmes across borders.

The purpose of this study was to determine the current prevalence of childhood hearing impairment (HI) in Sweden and to compare our data with previous studies from Sweden and other high-income countries.This was a cross-sectional study based on a register of children diagnosed with HI. Our results were compared with prevalence data from 18 studies from Sweden and other high-income countries, covering data collected from 1964 to the present.In December 2017, a total of 1911 out of 524,957 children 0-18 years of age and living in Stockholm County were enrolled in the regional programme for HI intervention.The overall prevalence of unilateral and bilateral HI20 dB was 3.6/1000. The overall prevalence of bilateral HI40 dB HL was 1.5/1000, split into age groups the prevalence was 0.4/1000 (1 year of age), 1/1000 (1-4 years), 1.5/1000 (5-9 years), 1.6/1000 (10-14 years), and 2.14/1000 (15-18 years). From 1 to 18 years of age, the prevalence increased by a factor of 3.5 for moderate to profound HI40 dB HL (0.7 to 2.4/1000).The prevalence of HI across childhood in Stockholm County today is not significantly different from previous reports from Sweden and other high-income countries.

The primary aim was to investigate whether computer-assisted reading intervention somehow can affect event-related potentials (ERP) and mismatch negativity (MMN) in hearing impaired (HI) children with hearing aids (HAs) and normal hearing (NH) children.The study included 15 HI children with sensorineural hearing loss (SNHL) using bilateral HAs and 14 NH children as a reference group; all children between the ages of 5 and 8. A multi-feature MMN-paradigm, Optimum-1, with a standard stimulus alternating with 5 different deviants was used. ERPs were recorded pre and post intervention, i.e. one month of repeatedly computer-assisted training (GraphoGame). MMN was calculated from the average ERP of each deviant minus standard. Data were based on samples within a specific time interval, 80-224 ms, and repeated measures ANOVA was used to analyze possible interactions.There was a significant difference between groups before training, though, the mean obligatory responses or MMN was not statistically significantly different before versus after training, neither among the NH nor the HI children. Further, the HI children did generally achieve lower levels in GraphoGame compared to the NH children. Altogether, our findings indicate differences between the groups and that training may affect the neurophysiological processing in the brain, gaining the HI children. Both MMN and positive mismatch response (pMMR) were seen among both the HA and NH children, irrespective to deviant type. Individually, changes of the MMN and pMMR after training seem unpredictable.There are statistically significant differences in both the obligatory responses in ERP and the MMNs between the NH and HI groups before the computer-assisted training. Though, these differences disappear after the intervention. This suggests possible training effects regarding the central auditory processing among the HI children.

The heterogeneity of tinnitus is likely accounting for the lack of efective treatment approaches. Headaches have been related to tinnitus, yet little is known on how headaches impact tinnitus. We use cross-sectional data from the Swedish Tinnitus Outreach Project to i) evaluate the association between headaches and tinnitus (n=1,984 cases and 1,661 controls) and ii) investigate the phenotypic characteristics of tinnitus subjects with tinnitus (n=660) or without (n=1,879) headaches. In a multivariable logistic regression model, headache was signifcantly associated with any tinnitus (odds ratio, OR=2.61) and more so with tinnitus as a big problem (as measured by the tinnitus functional index, TFI≥48; OR=5.63) or severe tinnitus (using the tinnitus handicap inventory, THI ≥58; OR=4.99). When focusing on subjects with tinnitus, the prevalence of headaches was 26% and reached 40% in subjects with severe tinnitus. A large number of socioeconomic, phenotypic and psychological characteristics difered between headache and non-headache subjects with any tinnitus. With increasing tinnitus severity, fewer diferences were found, the major ones being vertigo, neck pain and other pain syndromes, as well as stress and anxiety. Our study suggests that headaches could contribute to tinnitus distress and potentially its severity., GENDER-NET Co-Plus Fund GNP-182, Decibel Therapeutics, Inc., Svenska Lakaresallskapet SLS-779681, Tysta Skolan, Horselforskningsfonden 503, European Union's Horizon 2020 research and innovation programme under the Marie Skodowska-Curie grant 72204655, Swedish Medical Research Council (SMRC) K2014-99x-22478-01-3, Karolinska Institutet

Background: The association between the Joint Committee on Infant Hearing (JCIH) risk factors and etiology of hearing loss (HL) is not studied well in children.Objectives: To clarify the etiologic causes and evaluate the JCIH risk characteristics of children with HL.Methods: A retrospective study of 296 children with HL born between 2009.01 and 2013.12 in Stokholm. Demographic data, family and medical histories, audiologic results, imaging findings, and genetic results were ascertained and analyzed.Results: In 221 with bilateral hearing loss (BHL), family history and neonatal risk indicators were the most common risks (59 each), followed by syndrome related risks. In 75 with unilateral hearing loss (UHL), craniofacial anomaly was the most common risk, followed by family history. Etiology was established in 93 with BHL, in which syndromic HL accounted for 37.2%, chromosomal aberrations for 21.3%, and environmental causes for 19.1%. Etiology was established in 35 with UHL, in which ear malformation accounted for the most (74.3%), followed by environmental causes (14.3%).Conclusions and significance: Childhood HL can be attributed to a variety of causes with an etiology identifiable in 42.5% of BHL and 46.7% of UHL. BHL and UHL have different patterns of JCIH risk exposure and etiology.

This study examined the hearing aid use in older school-aged children with mild bilateral hearing loss. More specifically, it investigated children's and parents' estimation of use in comparison to datalogging as well as explored the situations children used their hearing aids.Sixteen children with mild bilateral hearing loss and their parents participated. Of those, 14 children used hearing aids. Children and parents completed a questionnaire on hours of hearing aid use and situations hearing aids were used. Datalogging of the hearing aids was recorded and compared to the outcome of the questionnaires.Datalogging indicated average hearing aid use time was 6.6 h. Children significantly overestimated their use of their hearing aids while approximately half the parents overestimated their child's use. Children used their hearing aids most often at school and in the car.Children with mild bilateral hearing loss overestimate the amount of time they are wearing their hearing aids. This may impact counselling and intervention on the use of hearing aids. Therefore, school-aged children should be included in the discussions around potentially increasing use of hearing aids.

Our aim was to explore whether a multi-feature paradigm (Optimum-1) for eliciting mismatch negativity (MMN) would objectively capture difficulties in perceiving small sound contrasts in children with hearing impairment (HI) listening through their hearing aids (HAs) and/or cochlear implants (CIs). Children aged 5-7 years with HAs, CIs and children with normal hearing (NH) were tested in a free-field setting using a multi-feature paradigm with deviations in pitch, intensity, gap, duration, and location. There were significant mismatch responses across all subjects that were positive (p-MMR) for the gap and pitch deviants (F(1,43) = 5.17, p = 0.028 and F(1,43) = 6.56, p = 0.014, respectively) and negative (MMN) for the duration deviant (F(1,43) = 4.74, p = 0.035). Only the intensity deviant showed a significant group interaction with MMN in the HA group and p-MMR in the CI group (F(2,43) = 3.40, p = 0.043). The p-MMR correlated negatively with age, with the strongest correlation in the NH subjects. In the CI group, the late discriminative negativity (LDN) was replaced by a late positivity with a significant group interaction for the location deviant. Children with severe HI can be assessed through their hearing device with a fast multi-feature paradigm. For further studies a multi-feature paradigm including more complex speech sounds may better capture variation in auditory processing in these children.

Objectives: The primary aim was to find out whether a computer-assisted reading intervention program with a phonic approach can affect event-related (ERPs) and mismatch negativity (MMN) in hearing impaired (HI) children using cochlear implants (CIs). Methods: This study involved a test group of 15 HI children with CIs and a control group of 14 normal hearing (NH) children. The children were 4 years and 10 months to 8 years and 1 month old. ERPs were recorded immediately before and after 4 weeks of training with a computer-assisted reading intervention, GraphoGame. A multi-feature paradigm, Optimum-1, was used, i.e. a standard stimulus alternated with five different deviants: gap intensity, pitch, location and duration. MMN was calculated from the mean amplitude ERP of each deviant minus the standard stimulus response in a specific time interval, 80 - 224 ms. Repeated measures ANOVA was used for the statistical analysis. Results: The results did not show any significant changes with the computerassisted training in the ERPs and MMNs among the HI children with CIs. The presence of both MMN and a positive mismatch response (pMMR), which might reflect an immaturity, complicates interpreting the results in this age group. Individually, there was a mix of MMNs and pMMRs among all participants, pre and post training, and the change of each deviant after intervention was not predictable. Conclusions: There are no significant changes in ERP or MMN after intervention, however lack of significances must be interpreted with caution. Besides the presence of both MMNs and pMMRs, only modest changes are to be expected on an individual basis and small samples hinder making statistical conclusions regarding the trainings effects. The study contributes to some more descriptive pieces of ERPs and MMNs among the HI children with CIs. The issues of MMN and pMMR are highlighted. Funding Agencies|Swedish Research Council for Working Life and Social Sciences (Forskningsradet for Arbetsliv och Social Vetenskap); Linnaeus Centre HEAD at Linkoping University; Cognition, Communication and Learning (CLL) at Lund University; Foundation of Hearing Research; Horselforskningsfonden; Horselskadades Riksforbund

The following are available online at http://www.mdpi.com/2077-0383/9/8/2412/ s1, We gratefully acknowledge the support and generosity of Nancy Pedersen, head of LifeGene., Many individuals with tinnitus report experiencing hyperacusis (enhanced sensitivity to sounds). However, estimates of the association between hyperacusis and tinnitus is lacking. Here, we investigate this relationship in a Swedish study. A total of 3645 participants (1984 with tinnitus and 1661 without tinnitus) were enrolled via LifeGene, a study from the general Swedish population, aged 18–90 years, and provided information on socio-demographic characteristics, as well as presence of hyperacusis and its severity. Tinnitus presence and severity were self-reported or assessed using the Tinnitus Handicap Inventory (THI). Phenotypes of tinnitus with (n = 1388) or without (n = 1044) hyperacusis were also compared. Of 1661 participants without tinnitus, 1098 (66.1%) were women and 563 were men (33.9%), and the mean (SD) age was 45.1 (12.9). Of 1984 participants with tinnitus, 1034 (52.1%) were women and 950 (47.9%) were men, and the mean (SD) age was 47.7 (14.0) years. Hyperacusis was associated with any tinnitus [Odds ratio (OR) 3.51, 95% confidence interval (CI) 2.99–4.13], self-reported severe tinnitus (OR 7.43, 95% CI 5.06–10.9), and THI ≥ 58 (OR 12.1, 95% CI 7.06–20.6). The association with THI ≥ 58 was greater with increasing severity of hyperacusis, the ORs being 8.15 (95% CI 4.68–14.2) for moderate and 77.4 (95% CI 35.0–171.3) for severe hyperacusis. No difference between sexes was observed in the association between hyperacusis and tinnitus. The occurrence of hyperacusis in severe tinnitus is as high as 80%, showing a very tight relationship. Discriminating the pathophysiological mechanisms between the two conditions in cases of severe tinnitus will be challenging, and optimized study designs are necessary to better understand the mechanisms behind the strong relationship between hyperacusis and tinnitus., GENDER-NET Co-Plus Fund GNP-182, Decibel Therapeutics, Inc., Svenska Lakaresallskapet SLS-779681, Tysta Skolan, Horselforskningsfonden 503, European Union (EU) 72204655 848261, NIHR Nottingham Biomedical Research Centre, Swedish Medical Research Council (SMRC) K2014-99X-22478-01-3, Karolinska Institutet, National Institute for Health Research (NIHR)

Objective Examine deaf and hard of hearing (DHH) children's phonological processing skills in relation to a reference group of children with normal hearing (NH) at two baselines pre intervention. Study the effects of computer-assisted phoneme–grapheme correspondence training in the children. Specifically analyze possible effects on DHH children's phonological processing skills. Methods The study included 48 children who participated in a computer-assisted intervention study, which focuses on phoneme–grapheme correspondence. Children were 5, 6, and 7 years of age. There were 32 DHH children using cochlear implants (CI) or hearing aids (HA), or both in combination, and 16 children with NH. The study had a quasi-experimental design with three test occasions separated in time by four weeks; baseline 1 and 2 pre intervention, and 3 post intervention. Children performed tasks measuring lexical access, phonological processing, and letter knowledge. All children were asked to practice ten minutes per day at home supported by their parents. Results NH children outperformed DHH children on the majority of tasks. All children improved their accuracy in phoneme–grapheme correspondence and output phonology as a function of the computer-assisted intervention. For the whole group of children, and specifically for children with CI, a lower initial phonological composite score was associated with a larger phonological change between baseline 2 and post intervention. Finally, 18 DHH children, whereof 11 children with CI, showed specific intervention effects on their phonological processing skills, and strong effect sizes for their improved accuracy of phoneme–grapheme correspondence. Conclusion For some DHH children phonological processing skills are boosted relatively more by phoneme–grapheme correspondence training. This reflects the reciprocal relationship between phonological change and exposure to and manipulations of letters. Neurofysiologiska aspekter på hörande

The aim of this review was to study the effectiveness of ventilation tube (VT) treatment in children with secretory otitis media (SOM), assessed by improved hearing, normalized language and quality of life (QoL), and recurrent acute otitis media (rAOM), assessed by number of episodes of AOM and QoL. Data Sources. Cochrane Library, PubMed, and Embase databases were searched for randomized and nonrandomized controlled trials and cohort studies in English, Scandinavian, German, and French languages between 1966 and April 2007. Additional literature was retrieved from reference lists in the articles.A total of 493 abstracts were evaluated independently by 2 members of the project group, 247 full-text versions were assessed for inclusion criteria and quality using structured evaluation forms, and 63 articles were included in the review.This review shows that there is strong scientific evidence (grade 1) that VT treatment of SOM improves hearing for at least 9 months and that QoL is improved for up to 9 months (grade 2 scientific evidence). There was insufficient evidence to support an effect of VT treatment for rAOM. There was also insufficient evidence to determine whether the design or material of the VT or the procedure used for insertion had any influence on the effect; however, there was some evidence (grade 3) that aspiration of secretion at insertion does not prolong VT treatment. Further research is needed to address these issues.

Auditory neuropathy (AN) was reported 30 years ago in 1979 when Davis and Hirsh presented the first case with normal or near normal hearing threshold but absent auditory brainstem responses. Many names have been given since then including paradoxical hearing loss, brainstem auditory processing syndrome, central auditory dysfunction, neural synchrony disorder or neural dyssynchrony. The term auditory neuropathy was first given by Sininger and colleagues in 1995. More and more AN articles have been published in recent years. The present short review and case report focus on the most important characteristics from a clinical point of view in order to let young physicians know AN, and consequently make correct diagnosis.

Congenital cytomegalovirus (CMV) infection is asymptomatic in 90% of infected newborns but approximately 10-20% of these infants are at risk of developing sequelae later, mostly hearing deficit. The aims of the study were to investigate the prevalence of congenital CMV infection in a Swedish population of newborns and investigate the relative risk of hearing deficit in newborns with congenital CMV infection. The dried blood spot (DBS) samples of 6060 newborns in southern Stockholm during 12 months (October 2003-June 2004; August 2004-October 2004) were analysed for CMV DNA by TaqMan based real-time PCR. Hearing deficit was assessed by otoacoustic emission (OAE) within a newborn screening programme. 12 infants out of 6060 or 0.2% (95% CI 0.1-0.3%) had congenital CMV infection. One boy among the 12 infected infants had unilateral hearing loss, indicating that the risk of hearing loss is greatly increased (about 20 times) in CMV infected infants. No child developed ocular complications such as chorioretinopathy during 3 y of follow-up. Congenital CMV has an impact on child health but can easily be overlooked due to lack of signs in the neonatal period. Surveillance for congenital CMV is important in addition to programmes for prevention and treatment.

This study explored nonword repetition (NWR) and nonword decoding in normal-hearing (NH) children and in children with bilateral cochlear implants (CI). Participants were 11 children, with CI, 5:0-7:11 years (M = 6.5 years), and 11 NH children, individually age-matched to the children with CI. This study fills an important gap in research, since it thoroughly describes detailed aspects of NWR and nonword decoding and their possible associations. All children were assessed after having practiced with a computer-assisted reading intervention with a phonics approach during four weeks. Results showed that NH children outperformed children with CI on the majority of aspects of NWR. The analysis of syllable number in NWR revealed that children with CI made more syllable omissions than did the NH children, and predominantly in prestressed positions. In addition, the consonant cluster analysis in NWR showed significantly more consonant omissions and substitutions in children with CI suggesting that reaching fine-grained levels of phonological processing was particularly difficult for these children. No significant difference was found for nonword-decoding accuracy between the groups, as measured by whole words correct and phonemes correct, but differences were observed regarding error patterns. In children with CI phoneme, deletions occurred significantly more often than in children with NH. The correlation analysis revealed that the ability to repeat consonant clusters in NWR had the strongest associations to nonword decoding in both groups. The absence of as frequent significant associations between NWR and nonword decoding in children with CI compared to children with NH suggest that these children partly use other decoding strategies to compensate for less precise phonological knowledge, for example, lexicalizations in nonword decoding, specifically, making a real word of a nonword.

The present study investigated cognitive abilities (i.e. Phonological Processing Skills (PhPS), lexical access, complex and visual Working Memory (WM), and letter knowledge) in Deaf and Hard of Hearing children (DHH) 5, 6 and 7 years of age using cochlear implants or hearing aids. Children with Normal Hearing (NH) served as a reference group. All children took part of a computer-assisted intervention with a phonics approach for 4 weeks aimed to support PhPS. The first aim of the study was to examine associations between cognitive abilities and Phonological Processing Skills (PhPS) pre intervention in DHH and NH children respectively. The second aim was to examine cognitive predictors of phonological gain post intervention. Finally, the influence of background variables on phonological gain was examined in NH and DHH respectively and in DHH children with weak PhPS particularly. Results showed comparable performance level in NH and DHH children on the majority of cognitive tasks, but weaker PhPS and lexical access in the DHH children. A significant association between PhPS and complex WM was only evident in DHH children. This finding suggests that DHH recruit more cognitive resources in phonological processing. A phonological representation task was the single predictor of phonological gain in DHH children. Children with initial weak performance on this task but had letter-naming skills, displayed relatively more phonological gain from the phonics training. Children with difficulties with the phonological representation task were older when diagnosed and had an older age at amplification. Further, these children displayed broader cognitive difficulties, suggesting that reduced access to auditory stimulation may have wide ranging effects on cognitive development.

The present study examines computer-assisted reading intervention with a phonics approach for deaf and hard of hearing (DHH) children in Sweden using cochlear implants or hearing aids, or a combination of both. The study included forty-eight children, 5, 6 and 7 years of age. Sixteen children with normal hearing (NH) served as a reference group. The first purpose of the study was to compare NH and DHH children’s reading ability at pre and post intervention. The second purpose was to investigate effects of the intervention. Cognitive and demographic factors were analyzed in relation to reading improvement. Results showed no statistically significant difference for reading ability at the group level, although NH children showed overall higher reading scores at both test points. Age comparisons revealed a statistically significant higher reading ability in the NH 7-year olds compared to the DHH 7-year olds. The intervention proved successful for word decoding accuracy, passage comprehension and as a reduction of nonword decoding errors in both NH and DHH children. Reading improvement was associated with complex working memory and phonological processing skills in NH children. Correspondent associations were observed with visual working memory and letter knowledge in the DHH children. Age was the only demographic factor that was significantly correlated with reading improvement. The results suggest that DHH children’s beginning reading may be influenced by visual strategies that might explain the reading delay in the older children.

Hearing and cognitive development in deaf and hearing-impaired children : effects of intervention

Each component of the human ear performs a specific function in hearing. The actual process of sound transduction takes place in the auditory portion of the inner ear, the fluid-filled cochlea. In the cochlea, the sensitivity and efficiency of sensory apparatus to convert mechanical energy into neural activity, largely depends on the fluidic and ionic environment. In the lateral wall of cochlea, the secretory epithelium stria vascularis plays an important role in the maintenance of fluidic and ionic homeostasis. A variety of gene mutations disturbs the cochlear homeostasis and subsequently leads to hearing impairment. The review covers several aspects of cochlear homeostasis, from cochlear fluid and the functional role of stria vascularis, cochlear K + recycling and its molecular substrates to genetic deafness with abnormal cochlear homeostasis.

Auditory stimulation provided by a cochlear implant (CI) to deaf or severely hearing-impaired children has an impact on their development in most areas (Geers et al., 2008; Lyxell et al., 2008, 200...