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Kaisa Bjuresäter,1 Mattias Bergström Andrén,2,3 Elina Mäki-Torkko,4,5 Agneta Anderzén-Carlsson,6 Per-Inge Carlsson2,5 1Institution of Health Sciences, Faculty of Health, Science and Technology, Karlstad University, Karlstad, Sweden; 2Department of Audiology, Central Hospital, Region Värmland, Karlstad, Sweden; 3School of Health Sciences, Faculty of Medicine and Health, Örebro University, Örebro, Sweden; 4Audiological Research Center, Faculty of Medicine and Health, Örebro University, Örebro, Sweden; 5School of Medical Sciences, Faculty of Medicine and Health, Örebro University, Örebro, Sweden; 6University Health Care Research Center, Faculty of Medicine and Health, Örebro University, Örebro, SwedenCorrespondence: Kaisa Bjuresäter, Email kaisa.bjuresater@kau.seBackground: Hearing loss increases with age and due to increased life expectancy there is an increase in the number of individuals living with hearing loss. Older people with hearing loss residing in nursing homes are often dependent on healthcare professionals to help them with their hearing aids.Objective: The aim of the study was to translate and test the psychometric properties of a Swedish version of a Norwegian questionnaire and to assess healthcare professionals’ self-reported knowledge, experience, skills, competence, and information needs pertaining to residents’ hearing loss and hearing aids in the Swedish context.Materials and Methods: A Norwegian questionnaire was translated and adapted, and thereafter distributed to healthcare professionals in nine nursing homes in mid Sweden between 2020 and 2021, and 313 questionnaires were returned.Results: An exploratory factor analysis demonstrated adequate factorial structure in six factors, satisfying construct validity and internal consistency for the Swedish version. A confirmatory factor analysis showed a satisfactory model fit. Healthcare professionals reported having the skills required for handling hearing aids, but reported lower scores for having received information about hearing aids, taking initiatives to refer residents to hearing healthcare units, and checking for earwax. Registered nurses generally reported lower perceived knowledge and practical skills concerning hearing aids. Seventy-seven percent of the total group reported a need for information regarding hearing aid maintenance.Conclusion: Healthcare professionals reported that the majority of nursing home residents need help with their hearing aids, but only a minority of these professionals had received education on hearing loss and training in hearing aid maintenance. Enrolled nurses and care assistants demonstrated higher levels of competence in handling hearing aids compared to registered nurses. In order to ensure safe and effective care, as well as facilitate communication among older adults with hearing loss, healthcare professionals need appropriate education and training.Keywords: hearing loss, nursing homes, health care professionals, competence, instrument translation, psychometric testing

Unlabelled: Linguistic and motor abilities among low-birthweight 8-y-old children in the northern Finland Birth Cohort for 1985-1986 (n = 9322) were studied using parental and teacher evaluations. The parents of 8370 (90%) and teachers of 8525 (92%) children returned a mailed questionnaire concerning the children's speech, language, learning and motor abilities. Low-birthweight (LBW, < 2500 g) children (n = 279) appeared to have experienced more difficulties than normal-birthweight (NBW, > or = 2500 g) children (n = 8091). The parents evaluated the LBW boys to be the poorest in linguistic and motor skills compared with the other boys or any of the groups of girls. They are therefore presumably at risk of having problems at school, which was confirmed by the teachers' reports. There was also a clear relationship between speech/linguistic and motor disabilities.Conclusion: Multivariate logistic regression analyses showed that the lower birthweight and some sociodemographic factors, for example the mother's age being between 20 and 24 y, having more than four children in the family, a reconstructed family, as well as hearing impairment and male gender were the most important determinants of poor speech and language abilities at 8 y of age, with and without adjustment for neonatal risk factors (asphyxia, convulsions, respiratory distress syndrome, bronchopulmonary dysplasia, patent ductus arteriosus). Smallness for gestational age was also a risk factor for poor speech and language skills. Preterm birth was associated with poor skills only after adjustment for the neonatal risk factor. The parental and teacher evaluations were concordant concerning the LBW children's outcome. [ABSTRACT FROM AUTHOR]

Background: Studies of language in children with mild-to-moderate hearing impairment (HI) indicate that they often have problems in phonological short-term memory (PSTM) and that they have linguistic weaknesses both in vocabulary and morphosyntax similar to children with specific language impairment (SLI). However, children with HI may be more likely than children with SLI to acquire typical language skills as they get older. It has been suggested that the more persisting problems in children with SLI are due to a combination of factors: perceptual, cognitive and/or linguistic. Aims: The main aim of this study was to explore language skills in children with HI in comparison with children with SLI, and how children with both HI and language impairment differ from those with non-impaired spoken language skills. Methods & Procedures: PSTM, output phonology, lexical ability, receptive grammar and verb morphology were assessed in a group of children with mild-to-moderate HI (n = 11) and a group of children with SLI (n = 12) aged 5 years 6 months to 9 years 0 months. Outcomes & Results: The HI group tended to score higher than the SLI group on the language measures, although few of the differences were significant. The children with HI had their most obvious weaknesses in PSTM, vocabulary, receptive grammar and inflection of novel verbs. The subgroup of children with HI (five out of 10) who also showed evidence of grammatical output problems was significantly younger than the remaining children with HI. Correlation analysis showed that the language variables were not associated with age, whereas hearing level was associated with PSTM. Conclusions: Children with HI are at risk for at least a delay in lexical ability, receptive grammar and grammatical production. The problems seen in the HI group might be explained by their low-level perceptual deficit and weak PSTM. For the SLI group the impairment is more severe. From a clinical perspective an important conclusion is that the language development in children with even mild-to-moderate HI deserves attention and support. [ABSTRACT FROM AUTHOR]

Objective: The aims of the present study were to evaluate the role of the gap junction protein beta-2 gene (GJB2), encoding connexin 26 (Cx26), in children with moderate to profound prelingual nonsyndromic sensorineural hearing impairment (HI) and to investigate the carrier frequencies of the GJB2 gene mutations in a control population in Northern Finland.Methods: Mutation analysis was performed by direct sequencing and carrier detection by conformation sensitive gel electrophoresis further confirmed by direct sequencing.Results: Cx26 mutations were found in 15 of 71 (21.1%) (67 families) children with HI. Homozygosity for the mutation 35delG was shown to be the cause of HI in 13 of 15 (86.7%) children. Homozygosity for the M34T genotype was found in one child, and compound heterozygosity for the M34T/V37I genotype was found in another. Five families of those with suspected familial HI (29.4%) and six families out of those with sporadic HI (12.0%) had a homozygous or compound heterozygous mutation. The carrier frequency for the mutation 35delG was 1 of 78 (4 of 313) and that for the M34T was 1 of 26 (12 of 313).Conclusion: 35delG/35delG genotype was found to be a significant cause of moderate to profound prelingual nonsyndromic sensorineural HI in Northern Finland. M34T/M34T genotype was seen in only one child, but the carrier frequency of the M34T allele was about three times higher than that of the 35delG mutation. [ABSTRACT FROM AUTHOR]

Objectives: Over 90% of 4--5-year-old children in Sweden spend most of their awake time at preschool. The rooms are acoustically designed for educational purposes and not adapted to the specific preschool requirements. Also, sounds reaching a child's eardrum are not comparable to an adult's, as higher frequencies are affected differently by the torso, head, pinna and ear canal. Studies on children's perception and behavior support a sensitivity for high frequencies and studies of otoacoustic emissions indicative of hearing damage have further highlighted the risk. The intermittent sound characteristics in preschool may also contribute to an increased risk for hearing disorders, recently observed in a cohort study among preschool personnel. There is a great need to develop acoustic criteria that support preschool activities and child-specific hearing and health. To investigate the risk of noise-induced hearing problems for preschool children, our research team carried out two preparatory studies among preschool children. One among 12 children from two departments at a preschool in 2015, and one among 33 preschool children from six preschools in 2017. Material and methods: Noise levels were measured using stationary measurements and personal dosimeters. Hearing function was measured by Distortion Product Otoacoustic Emissions (DPOAE), which detects early changes in the hearing function after exposure to loud sounds. Results: The 2015 study showed a reduced function at 6 kHz (right ear) with a mean reduction of 2.59 dB p<0.05, after a day at preschool. Preliminary data from the 2017 follow-up, show a reduction over the week with similar effects sizes as the previous study but with the clearest and significant effects for 3 and 4 kHz (right ear). Pilot studies have also shown that a child dummy head receives up to 10 dB higher amplitudes around 6 kHz compared to an adult dummy head in a preschool sound environment. Given the child-specific amplification, certain higher frequency sounds may be perceived as painful and distressing by children. Initial support for these reactions was given in a qualitative study of 36, 4--6 years old preschool children. Uncontrollable sounds and distressing sounds i.e. angry, loud and scraping sounds were experienced both physically and emotionally painful. The importance of a child's perspective for acoustic interventions at preschools was further shown in a 2016 study where the reduction of noise levels was significantly associated with a 30% (OR=0.69) reduction of children's perception of scraping and screeching sounds. The study also associated scraping and screeching sounds with a 60% reduction of negative emotional reactions and an 80% reduction of stress-related symptoms. Conclusions: Taken together, current room-acoustic standards do not fully capture the acoustic needs of the preschool environment, nor that children experience the sound environment differently than adults. The current occupational safety regulations of the sound levels may not provide enough safety for children. There is, therefore, a great need to continue to study how we may prevent the risk of noise-induced stress and discomfort and hearing disorders among pre-school children. The pilot studies have formed a base for a current study that will relate detailed sound measurements from 33 preschools to measurements of around 330 children's perception (validated questionnaire), language (validated test) and impact on hearing (repeated measures of otoacoustic emissions). The aim is to identify child relevant acoustic criteria that may contribute to preventing noise-induced discomfort, stress and hearing disorders among pre-school children and adults. The posterpresentation will describe results from previous pilot studies and the plan for the larger study. [ABSTRACT FROM AUTHOR]

This study investigated the impact of bilateral bone conduction (BC) stimulation and sensorineural hearing loss on spatial release from masking, binaural intelligibility level difference, and lateralization. The study involved two groups of adults with mild to moderate sensorineural hearing loss: one group of 21 participants with symmetric hearing loss and another group of nine participants with asymmetric hearing loss. All tests were conducted through BC and air conduction (AC) headsets with non-individualized virtual positions of the sound sources and linear amplification based on individual hearing thresholds. The findings revealed a bilateral benefit for both groups of hearing-impaired individuals, with symmetric hearing loss yielding better results than asymmetric hearing loss. AC stimulation provided approximately twice the benefit in terms of dB compared to BC stimulation. A large part of this benefit originated from a favorable signal-to-noise ratio due to noise reduction from the head shadow. However, binaural processing was present in both hearing-impaired groups with bilateral BC stimulation. The ability to lateralize sounds based on interaural time delays was significantly impaired in participants with both types of hearing loss when stimulation was by BC. Despite these challenges, the study underscores the benefits of bilateral fitting of BC hearing aids, even in individuals with mild to moderate sensorineural hearing loss, whether symmetric or asymmetric., Competing Interests: Declarations. Competing interests: The authors declare no competing interests., (© 2024. The Author(s).)

Objective: To introduce the urban outdoor version of the Situated Phoneme (SiP) test and investigate its test-retest reliability., Design: Phonemic discrimination scores in matched-spectrum real-world (MSRW) maskers from an urban outdoor environment were measured using a three-alternative forced choice test paradigm at different phoneme-to-noise ratios (PNR). Each measurement was repeated twice. Test-retest scores for the full 84-trial SiP-test, as well as for four types of contrasting phonemes, were analysed and compared to critical difference scores based on binomial confidence intervals., Study Sample: Seventy-two adult native speakers of Swedish (26-83 years) with symmetric hearing threshold levels ranging from normal hearing to severe sensorineural hearing loss., Results: Test-retest scores did not differ significantly for the whole test, or for the subtests analysed. A lower amount of test-retest score difference than expected exceeded the bounds of the corresponding critical difference intervals., Conclusions: The urban outdoor SiP-test has high test-retest reliability. This information can help audiologists to interpret test scores attained with the urban outdoor SiP-test.

Background: The objective of this study is to estimate the prevalence of self-reported hearing difficulties, vision difficulties and combined vision and hearing difficulties in a Swedish adult population that varies according to migration status, sex, age, disability measured by ADL and IADL and educational attainment level., Methods: The study utilised data from the Survey of Health, Ageing and Retirement in Europe, which consisted of 2257 individuals aged 60 and above that were interviewed in Sweden in the 2019/2020, SHARE wave 8. To determine the prevalence of sensory difficulties (hearing, vision and dual-sensory difficulties) among various subgroups of the population, a multinomial logistic regression was used. The results of these analyses are presented in terms of predicted probabilities., Results: The study findings indicate that foreign-born older adults experience a lower prevalence of visual difficulties (6.2% [3.3-11.5] in comparison to their Swedish-born counterparts. Moreover, older adults with higher levels of education tend to report a lower prevalence of sensory difficulties overall. Furthermore, sex differences are apparent, with males reporting a higher prevalence of hearing difficulties (18.9% [15.5-22.8] vs. 12.8% [10.7-15.3]) and females reporting a higher prevalence of vision difficulties (12.7% [10.7-15.1] vs. 8.5%[6.8-10.5])., Conclusion: The findings highlight disparities in the prevalence and type of perceived sensory difficulties experienced by older adults, by factors such as age, sex, education and migration status. It is important to consider these demographic factors in healthcare planning and interventions aimed at mitigating sensory difficulties in the older population., (© 2024. The Author(s).)

Objective - To study the correlation between P1 latency and the results of clinical language tests (Reynell III and TROG-2), the latter were used as they are recommended for follow-up assessments of children with cochlear implants (Cis) by the Swedish National Quality Register for children with hearing impairment. Design - A clinical cohort study. Study sample - Cross-sectional and consecutive sampling of 49 children with CIs coming for clinical follow-up assessment from March 2017 - December 2019. Results - For all children tested, there was a significant negative correlation (Spearman's rho= -0.403, p  = 0.011) between hearing age and P1 latency. A significant correlation between P1 latency and the Reynell III result (Spearman's rho =  -0.810, p  = 0.015) was found. In the TROG-2 group, there was no significant correlation between their P1 latency and their language test results (Spearman's rho -0.239, p  = 0.196). Conclusion - This method seems to be feasible and easily accepted. The study was conducted in a heterogeneous group of children that we meet daily in our clinic. The results indicated that P1 latency has a negative correlation with language development among our youngest patients fitted with CIs and might be a clinical tool to assess the maturation of central auditory pathways.

Objective: To assess transient-evoked otoacoustic emissions (TEOAE) data from 15 years of a newborn hearing screening program and evaluate how well various criteria separate ears with and without hearing loss., Design: Retrospective review of TEOAE data using logistic regression, receiver operating characteristic curves, and cumulative percentage graphs.Study sample: Children with hearing loss who passed TEOAE screening as a newborn were compared to children who failed TEOAE screening and normal hearing children who either passed or failed. Exclusions were applied for acquired hearing loss or auditory neuropathy., Results: Ears with hearing loss that passed screening had significantly lower TEOAE response levels compared to ears with normal hearing. Noise levels, test times, and number of sweeps were also lower. Most of these ears had mild hearing loss. Logistic regression results showed that high-frequency TEOAE response level is the best predictor of hearing loss. A multivariate "logit" score calculated from the regression was the best indicator for separating ears with hearing loss from ears with normal hearing., Conclusions: TEOAE response levels or an algorithm which incorporates logit scores should be considered as a minimum passing criterion to increase the sensitivity of the TEOAE screening.

Purpose: The aim of the current study was to explore the construct validity and internal consistency reliability of the International Classification of Functioning, Disability and Health (ICF)-based original English version of the Hearing and Functioning in Everyday Life Questionnaire (HFEQ) and to revise the HFEQ based on the results., Method: This study used a cross-sectional survey design. The data were collected using an online survey. Adults with self-reported hearing disability ( n = 513) from the United States were included. The ICF components of body functions, activity and participation, and environmental factors were tested as the underlying structure of the HFEQ using confirmatory factor analysis and then adjusted by triangulation with previous content validation., Results: The results of the current study confirmed the ICF components of body functions, activity and participation, and environmental factors as underlying constructs of the HFEQ. However, after triangulation with previous content validation, fine adjustments were made. The revised version of the HFEQ includes two removed items and a fine-tuned factor structure., Conclusion: The results confirm that the structure of the HFEQ aligns with the ICF, and the overall results indicate that HFEQ has acceptable construct validity and internal consistency.

Background: The objective of this study is to explore the bidirectional, longitudinal associations between self-reported sensory functions (hearing/vision) and cognitive functioning among older adults in Sweden and Denmark during the period 2004-2017., Methods: The study is based on data from The Survey of Health, Ageing and Retirement in Europe and consists of 3164 persons aged 60 to 93 years. Within-person associations between sensory and cognitive functions were estimated using random intercept cross-lagged panel models., Results: The results indicated that cognitive and sensory functions were associated within their respective domains over time. The results on the bidirectional associations between sensory functions and cognition over time showed weak and statistically non-significant estimates., Conclusion: Our study showed no clear evidence for cross-lagged effects between sensory functions and cognitive functioning. Important to note, however, is that using longitudinal data to estimate change within persons is a demanding statistical test and various factors may have contributed to the absence of conclusive evidence in our study. We discuss several of these factors., Competing Interests: Declaration of competing interest The authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper., (Copyright © 2024 The Author(s). Published by Elsevier B.V. All rights reserved.)

Purpose: There is an increasing concern regarding hazardous recreational noise exposure among adolescents and young adults. Daily exposure to loud sound levels over a long period of time can increase the risk of noise-induced hearing loss. The full extent of the impact of recreational noise on hearing is not yet fully understood. The purpose of this review was to synthesize research that investigated hearing function in relation to recreational noise exposure in adolescents and young adults., Method: A systematic literature search of five databases covering the years 2000-2023 was performed. The articles included investigated audiological measurements of hearing function in relation to recreational noise exposure., Results: Four hundred sixty records were identified, of which 20 met the inclusion criteria and were included in the results. This review showed that although some recreational noise activities can be potentially harmful, there is an unclear relationship between exposure and outcome. Some findings indicated hearing threshold shifts or reduced otoacoustic emission amplitudes after recreational noise exposure, but most changes were short term and in the extended high-frequency range., Conclusions: There seemed to be inconsistencies regarding the utilization of methods of measuring exposure and outcome between studies. This might be one reason for the differing results in studies on the reported impact on hearing function from recreational noise exposure. To draw more certain conclusions about long-term effects, there is a need for longitudinal research that utilizes sound level measurements to assess low and high degrees of recreational noise exposure in relation to hearing function., Supplemental Material: https://doi.org/10.23641/asha.25114193.

Objectives: Self-assessment instruments are commonly used in audiological rehabilitation. However, several studies highlight the lack of multidimensionality in existing outcome measures, with the consequence that they only partially capture aspects of functioning in everyday life for people living with hearing loss. This study aimed to develop and investigate the content validity of a self-assessment instrument based on the validated Brief International Classification of Functioning, Disability, and Health Core Set for Hearing Loss., Design: The design was a two-part instrument development study. The first part focused on the item-generation process of the instrument, named the Hearing and Functioning in Everyday Life Questionnaire (HFEQ) during an experts' workshop. The second part focused on international content validation of the instrument using group interviews. Strategic sampling was used and 30 adults with hearing loss from India, South Africa, and the United States participated in the group interviews., Results: The expert's workshop resulted in the first version of the HFEQ containing 30 items. The results from group interviews show that the content of the HFEQ was considered to be valid concerning its relevance, comprehensiveness, and comprehensibility. A majority (73%) of the HFEQ items were perceived by the participants as relevant and easy to comprehend. For the remaining 27% of the items, the content was perceived to be relevant in all countries, but some terms and expressions were reported to require rewording or clearer examples. These modifications will be made in the next step of the development process., Conclusion: Content validation of the HFEQ demonstrates promising results, with participants perceiving the content as relevant and comprehensible. Further psychometric validation is required to investigate other psychometric properties, such as construct validity and reliability. The HFEQ has the potential to become a valuable new instrument for assessing everyday functioning in people with hearing loss in audiological rehabilitation and in research., (Copyright © 2023 Wolters Kluwer Health, Inc. All rights reserved.)

Objectives: Improvements in educational attainment, cognitive and sensory functions, and a decline in the prevalence of disabilities have been observed in older adults in Sweden and Denmark. In the present study, it was investigated whether better cognition, higher educational attainment, and improved sensory function among older adults aged 60 and older in these countries have contributed to decreasing rates of old-age disabilities., Methods: The analyses were based on repeated cross-sectional data from the Survey of Health, Ageing, and Retirement in Europe for the 2004-2017 period. Descriptive data were used to benchmark the declining prevalence of disabilities, improving cognitive and sensory functions, and increased educational level. The association between time and disabilities was analyzed with logistic regression models, and the contribution of the improved cognitive function, education, and sensory function to the declining prevalence of old-age disabilities was estimated using the Karlson-Holm-Breen method for mediation analysis., Results: The analysis suggests that the declining prevalence of old-age disabilities in Sweden and Denmark between 2004 and 2017 can largely be attributed to improved cognitive function and vision and to a lesser extent by education and hearing ability., Discussion: These findings raise important questions about the causal mechanisms producing the associations between cognition, education, and sensory functions and disability in older age. Future studies should explore the causal nature of the associations between these mediators and old-age disabilities. In addition, they should explore whether these findings differ across regional and cultural contexts and over different time periods., (© The Author(s) 2022. Published by Oxford University Press on behalf of The Gerontological Society of America.)

Deaf signers and hearing non-signers have previously been shown to recruit partially different brain regions during simple arithmetic. In light of the triple code model, the differences were interpreted as relating to stronger recruitment of the verbal system of numerical processing, that is, left angular and inferior frontal gyrus, in hearing non-signers, and of the quantity system of numerical processing, that is, right horizontal intraparietal sulcus, for deaf signers. The main aim of the present study was to better understand similarities and differences in the neural correlates supporting arithmetic in deaf compared to hearing individuals. Twenty-nine adult deaf signers and 29 hearing non-signers were enrolled in an functional magnetic resonance imaging study of simple and difficult subtraction and multiplication. Brain imaging data were analyzed using whole-brain analysis, region of interest analysis, and functional connectivity analysis. Although the groups were matched on age, gender, and nonverbal intelligence, the deaf group performed generally poorer than the hearing group in arithmetic. Nevertheless, we found generally similar networks to be involved for both groups, the only exception being the involvement of the left inferior frontal gyrus. This region was activated significantly stronger for the hearing compared to the deaf group but showed stronger functional connectivity with the left superior temporal gyrus in the deaf, compared to the hearing, group. These results lend no support to increased recruitment of the quantity system in deaf signers. Perhaps the reason for performance differences is to be found in other brain regions not included in the original triple code model., (© 2022 The Authors. Journal of Neuroscience Research published by Wiley Periodicals LLC.)

Objective: To study the impact of cardiovascular diseases (CVDs) on hearing deterioration among ageing adults in a longitudinal setting. Furthermore, to describe the pure tone threshold changes at the 0.125-8 kHz frequency range over 13 years., Design: A population-based follow-up study., Study Sample: A random sample of 850 adults, of whom 559 participated in the follow-up study. Otological examination, a structured interview, and pure tone audiometry were conducted. Multivariate regression models were used to estimate the effect of CVD (participants had at least one cardiovascular condition) on hearing deterioration of the better ear hearing level (BEHL), defined as a change in the pure-tone average (PTA) of the frequencies 0.5, 1, 2, and 4 kHz and separately at the lower (0.125, 0.25, and 0.5 kHz) and higher (4, 6, and 8 kHz) frequencies., Results: In the multivariable-adjusted analysis, the BEHL change at 13 years was 0.7 dB greater among participants with CVD ( p  = 0.3). The mean BEHL change during the 13-year follow-up was 12.0 dB (95% CI 11.4-12.6) among all participants., Conclusions: No significant association between CVD and hearing threshold changes was found.

Background: An effective newborn hearing screening programme has low referral rate and low loss to follow-up (LTFU) rate after referral from initial screening. This systematic review identified studies evaluating the effect of protocol and programme factors on these two outcomes, including the screening method used and the infant group., Methods: Five databases were searched (latest: April 2021). Included studies reported original data from newborn hearing screening and described the target outcomes against a protocol or programme level factor. Studies were excluded if results were only available for one risk condition, for each ear, or for < 100 infants, or if methodological bias was observed. Included studies were evaluated for quality across three domains: sample, screening and outcome, using modified criteria from the Ottawa-Newcastle and QUADAS-2 scales. Findings from the included studies were synthesised in tables, figures and text., Results: Fifty-eight studies reported on referral rate, 8 on LTFU rate, and 35 on both. Only 15 studies defined LTFU. Substantial diversity in referral and LTFU rate was observed across studies. Twelve of fourteen studies that evaluated screening method showed lower referral rates with aABR compared to TEOAE for well babies (WB). Rescreening before hospital discharge and screening after 3 days of age reduced referral rates. Studies investigating LTFU reported lower rates for programmes that had audiologist involvement, did not require fees for step 2, were embedded in a larger regional or national programme, and scheduled follow-up in a location accessible to the families. In programmes with low overall LTFU, higher LTFU was observed for infants from the NICU compared to WB., Conclusion: Although poor reporting and exclusion of non-English articles may limit the generalisability from this review, key influential factors for referral and LTFU rates were identified. Including aABR in WB screening can effectively reduce referral rates, but it is not the only solution. The reported referral and LTFU rates vary largely across studies, implying the contribution of several parameters identified in this review and the context in which the programme is performed. Extra attention should be paid to infants with higher risk for hearing impairment to ensure their return to follow-up., (© 2022. The Author(s).)

Studies have limitedly considered children with early-identified unilateral hearing impairment (UHI), and clinical practices regarding screening, diagnostics and habilitation in this group are rarely documented. In this study, routines for newborns with UHI from screening to diagnostics and habilitation were explored in Norway, Sweden and Finland. An online survey was sent to hospitals responsible for the hearing diagnostics of children requesting information about their practices regarding congenital UHI. Responses covered 95% of the children born in the three included countries. The results revealed large variations in ways of organising healthcare and in clinical decisions regarding hearing screening, diagnostics and habilitation of children with congenital UHI. Finally, implications for policy making and research are also discussed.

Objective: The aim of this study was to investigate the prevalence and incidence of hearing impairment (HI) in a longitudinal setting among adults., Design: An unscreened, population-based epidemiological 13-year follow-up study. Study sample: 850 randomly sampled 54 to 66-year-old baseline participants, of whom 559 participated in the follow-up study at the age of 68 to 79 years. A questionnaire-based interview, an otological examination and pure-tone audiometry were performed., Results: The overall prevalence of HI was 70.3%, defined by better ear hearing level (BEHL)  ≥ 20 dB in the 0.5-4 kHz frequency range. The prevalence was higher among men (78.6%) than among women (63.7%). The overall incidence rate for HI was 45.8 per 1000 person years and the 13-year cumulative incidence was 60.9%. The incidence was higher among men and older participants., Conclusion: HI is highly prevalent and incident among older adults in Northern Finland.

Objective: Hearing loss (HL) affects the everyday functioning of millions of people worldwide. The Brief International Classification of Functioning Disability and Health (ICF) core sets for HL was developed to meet the complex health care needs of adults with HL. Because the brief core set for HL has not yet been validated internationally, this study aimed to investigate its validity from an international perspective., Design: A cross-sectional validation study based on data from structured interviews with adults with HL., Study Sample: Participants ( n  = 571) from India, South Africa, Sweden and the US were included., Results: A six-factor solution explained 71% of the variance, focussing on issues related to communication, the social environment, participation in society, health care services, support, relationships and emotions ( α  = 0.915). Three ICF categories demonstrated low reliability - temperament and personality functions, seeing functions and school education ., Conclusion: The Brief ICF core set for HL is valid for adults with HL internationally. However, to further increase its international validity, we recommend adding the categories d920 recreation and leisure and replacing d850 school education with the more inclusive block, d810-d839 education.

Objective: The aim of this study was to develop and evaluate a Swedish version of the Hearing In Noise Test for Children (HINT-C)., Design: In the first part, the Swedish HINT lists for adults was evaluated by children at three signal to noise ratios (SNRs), -4, -1 and +2 dB. Lists including sentences not reaching 50% recognition at +2 dB SNR were excluded and the rest constituted the HINT-C. In the second part, HINT-C was evaluated in children and adults using an adaptive procedure to determine the SNR for 50% correctly repeated sentences. Study Sample In the first part, 112 children aged 6-11 years participated while another 28 children and 9 adults participated in the second part., Results: Eight out of 24 tested adult HINT lists did not reach the inclusion criteria. The remaining 16 lists formed the Swedish HINT-C which was evaluated in children 6-11 years old. A regression analysis showed that the predicted SNR threshold (dB) was 0.495-0.365*age (years + months/12) and the children reached the mean adult score at an age of 10.5 years., Conclusions: A Swedish version of HINT-C was developed and evaluated in children six years and older., (Copyright © 2020 The Authors. Published by Elsevier B.V. All rights reserved.)

Objectives: Abandonment of vision, hearing or mobility aids suggests common barriers and facilitators to ongoing device use. However, the possible interactive effects of combined hearing and vision disabilities on device use by those living with deafblindness are unclear. Here we summarise existing knowledge on variables influencing assistive technology use from the perspective of persons living with deafblindness. We used the WHO's International Classification of Functioning, Disability and Health (ICF) framework to contextualise the findings, asking 'What is currently known about variables influencing the (non-)use of assistive devices recommended for persons with deafblindness?', Design: A scoping review followed the Preferred Reporting Items for Systematic Reviews and Meta-Analyses extension for Scoping Reviews Checklist., Data Sources: PubMed; ProQuest: ERIC; ProQuest Dissertation; ProQuest: Sociological Thesaurus; Web of Science; Scientific Electronic Library Online; Bielefeld Academic Search Engine; Pascal & Francis; APA PsycINFO and Ebsco for CINAHL were searched through 9 November 2020., Eligibility Criteria: We included peer-reviewed studies that reported on assistive technology, device abandonment/utilisation and provided data from persons living with deafblindness., Data Extraction and Synthesis: Four team members independently scored 83 studies for eligibility., Results: Ten articles were chosen for data extraction. The emerging variables replicated established categories of barriers and facilitators: personal, device-related, environmental and intervention variables. The use of the ICF highlighted how an intermediate variable (eg, device acceptability) was necessary in order for a variable to become a barrier or a facilitator to device use., Conclusions: The variables influencing device use by persons with deafblindness followed the same categories described for single impairments. Usability was challenged in devices that rely on the 'other' sense. Haptic and tactile aids are rarely studied. The limited available information and the dire need for assistive technologies for people with deafblindness emphasises the urgency of research and technology development for this marginalised population., Competing Interests: Competing interests: None declared., (© Author(s) (or their employer(s)) 2021. Re-use permitted under CC BY-NC. No commercial re-use. See rights and permissions. Published by BMJ.)

Importance: Surgery remains the gold standard in cholesteatoma treatment. However, the rate of recurrence is significant and the development of new nonsurgical treatment alternatives is warranted. One of the possible molecular pathways to target is the Janus kinase/signal transducer and activator of transcription (JAK/STAT) pathway., Objective: To investigate the JAK/STAT pathway in the middle ear mucosa in patients with acquired cholesteatoma compared with middle ear mucosa from healthy controls., Design: Case-control study., Setting: Linköping University Hospital, Sweden, and Karolinska Institutet, Stockholm, Sweden. Sampling period: February 2011 to December 2016., Participants: Middle ear mucosa from 26 patients with acquired cholesteatoma undergoing tympanoplasty and mastoidectomy, and 27 healthy controls undergoing translabyrinthine surgery for vestibular schwannoma or cochlear implantation was investigated., Main Outcomes/measures: The expression of Interleukin-7 receptor alpha, JAK1, JAK2, JAK3, STAT5A, STAT5B, and suppressor of cytokine signaling-1 (SOCS1) were quantified using quantitative polymerase chain reaction. In addition, expression level of cyclin D2, transforming growth factor beta 1, thymic stromal lymphopoietin, CD3, and CD19 was evaluated., Results: In cholesteatoma-adjacent mucosa, SOCS1 was significantly upregulated (p= 0.0003) compared with healthy controls, whereas STAT5B was significantly downregulated (p = 0.0006). The expression of JAK1, JAK2, JAK3, and STAT5A did not differ significantly between groups., Conclusions and Relevance: To the best of our knowledge, this is the first article reporting dysregulation of the JAK/STAT pathway in cholesteatoma-adjacent mucosa. The main finding is that important players of the aforementioned pathway are significantly altered, namely SOCS1 is upregulated and STAT5B is downregulated compared with healthy controls.

The middle ear is a small and hard to reach compartment, limiting the amount of tissue that can be extracted and the possibilities for studying the molecular mechanisms behind diseases like cholesteatoma. In this paper 14 reference gene candidates were evaluated in the middle ear mucosa of cholesteatoma patients and two different control tissues. ACTB and GAPDH were shown to be the optimal genes for the normalisation of target gene expression when investigating middle ear mucosa in multiplex qPCR analysis. Validation of reference genes using c-MYC expression confirmed the suitability of ACTB and GAPDH as reference genes and showed an upregulation of c-MYC in middle ear mucosa during cholesteatoma. The occurrence of participants of the innate immunity, TLR2 and TLR4, were analysed in order to compare healthy middle ear mucosa to cholesteatoma. Analysis of TLR2 and TLR4 showed variable results depending on control tissue used, highlighting the importance of selecting relevant control tissue when investigating causes for disease. It is our belief that a consensus regarding reference genes and control tissue will contribute to the comparability and reproducibility of studies within the field., Competing Interests: The authors have declared that no competing interests exist.

Introduction  Hearing impairment is a common disease worldwide, with a comprehensive impact, and cochlear implantation (CI) is an intervention for profound hearing impairment. Objective  To study the outcome one and three years after unilateral CI on hearing, health-related quality of life and level of depression and anxiety, and the correlation between the outcomes. Second, to study whether age, gender, etiology, operated side, residual hearing or cognitive performance can predict the outcome. Methods  A prospective longitudinal study including adults with profound postlingual hearing impairment, with respect to hearing (speech recognition), health-related quality of life (Health Utilities Index 3) and level of depression and anxiety (Hospital Anxiety and Depression scale), pre-CI, and one and three years post-CI. The total sample was composed of 40 participants (40% of men), with a mean age of 71 years. Results  Speech recognition and the overall health-related quality of life improved one year post-CI ( p  = 0.000), without correlation (ρ= 0.27), and with no difference three years post-CI. The hearing attribute (in the health-related quality of life instrument) improved one and three years post-CI ( p  = 0.000). The level of anxiety did not change one and three years post-CI. The level of depression improved one year post-CI ( p  = 0.036), and deteriorated three years post-CI ( p  = 0.031). Age, etiology, operated side, residual hearing and cognitive performance did not predict the outcome, but the female gender did significantly improve speech recognition compared with men ( p  = 0.009). Conclusion  The CI significantly improved speech recognition, health-related quality of life and level of depression one year post-CI without mutual correlation, and women performed significantly better than men. There were no further improvements three years post-CI, apart from the hearing attribute., Competing Interests: Conflict of Interests The authors have no conflict of interests to declare.

We have previously shown that deaf signers recruit partially different brain regions during simple arithmetic compared to a group of hearing non-signers, despite similar performance. Specifically, hearing individuals show more widespread activation in brain areas that have been related to the verbal system of numerical processing, i.e., the left angular and inferior frontal gyrus, whereas deaf individuals engaged brain areas that have been related to the quantity system of numerical processing, i.e., the right horizontal intraparietal sulcus. This indicates that compared to hearing non-signers, deaf signers can successfully make use of processes located in partially different brain areas during simple arithmetic. In this study, which is a conceptual replication and extension of the above-presented study, the main aim is to understand similarities and differences in neural correlates supporting arithmetic in deaf compared to hearing individuals. The primary objective is to investigate the role of the right horizontal intraparietal gyrus, the left inferior frontal gyrus, the hippocampus, and the left angular gyrus during simple and difficult arithmetic and how these regions are connected to each other. A second objective is to explore what other brain regions support arithmetic in deaf signers. Up to 34 adult deaf signers and the same amount of hearing non-signers will be enrolled in an functional magnetic resonance imaging study that will include simple and difficult subtraction and multiplication. Brain imaging data will be analyzed using whole-brain analysis, region of interest analysis and connectivity analysis. This is the first study to investigate neural underpinnings of arithmetic of different difficulties in deaf individuals., (© 2019 Wiley Periodicals, Inc.)

Purpose: The aim of the study was to investigate the change in health-related quality of life (HRQoL) after canal wall up cholesteatoma surgery, using the Glasgow Benefit Inventory (GBI)., Methods: Data from a consecutive group of 47 adults scheduled for primary cholesteatoma surgery using canal wall up (CWU) with obliteration, from January 2005 to December 2009, were analysed. Information was extracted from a medical database, and complementary data from patient files and audiograms were collected and recorded retrospectively. The GBI questionnaire was used for the assessment of HRQoL after surgery., Results: There was no finding of residual or recurrent cholesteatomas in the study group. Hearing was improved at 1 and 3 years postoperatively. No patient suffered a total hearing loss. The overall GBI scores showed an improved HRQoL after surgery. Twenty-nine (85%) patients benefitted from surgery, 1 (3%) had no change, and 4 (12%) expressed deterioration., Conclusions: Cholesteatoma surgery using CWU with obliteration gives an improved HRQoL for the majority of patients. The GBI questionnaire provides complementary information to hearing and healing results after cholesteatoma surgery.

Background: Decision-making on treatment and (re)habilitation needs to be based on clinical expertise and scientific evidence. Research evidence for the impact of permanent unilateral hearing impairment (UHI) on children's development has been mixed and, in some of the reports, based on fairly small, heterogeneous samples. Additionally, treatment provided has been highly variable, ranging from no action taken or watchful waiting up to single-sided cochlear implantation. Published information about the effects of treatment has also been heterogeneous. Moreover, earlier reviews and meta-analyses published on the impact of UHI on children's development have generally focused on select areas of development., Objectives: This systematic review aimed to summarize the impact of children's congenital or early onset unilateral hearing impairment on listening and auditory skills, communication, speech and language development, cognitive development, educational achievements, psycho-social development, and quality of life., Methods: Literature searches were performed to identify reports published from inception to February 16th, 2018 with the main electronic bibliographic databases in medicine, psychology, education, and speech and hearing sciences as the data sources. PubMed, CINALH, ERIC, LLBA, PsychINFO, and ISI Web of Science were searched for unilateral hearing impairment with its synonyms and consequences of congenital or early onset unilateral hearing impairment. Eligible were articles written in English, German, or Swedish on permanent unilateral hearing impairments that are congenital or with onset before three years of age. Hearing impairment had to be of at least a moderate degree with PTA ≥40 dB averaged over frequencies 0.5 to 2 or 0.5-4 kHz, hearing in the contralateral ear had to have PTA 0.5-2 kHz or PTA 0.5-4 kHz ≤ 20 dB, and consequences of unilateral hearing impairment needed to be reported in an unanimously defined population in at least one of the areas the review focused on. Four researchers independently screened 1618 abstracts and 566 full-text articles for evaluation of study eligibility. Eligible full-text articles were then reviewed to summarize the results and assess the quality of evidence. Additionally, data from 13 eligible case and multi-case studies, each having less than 10 participants, were extracted to summarize their results. Quality assessment of evidence was made adapting the Grades of Recommendations, Assessment, Development, and Evaluation (GRADE) process, and reporting of the results adheres to the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) standards., Results: Three articles with the quality of evidence graded as very-low to low, fulfilled the eligibility criteria set. Due to the heterogeneity of the articles, only a descriptive summary could be generated from the results. Unilateral hearing impairment was reported to have a negative impact on preverbal vocalization of infants and on sound localization and speech perception both in quiet and in noise., Conclusions: No high-quality studies of consequences of early-onset UHI in children were found. Inconsistency in assessing and reporting outcomes, the relatively small number of participants, low directness of evidence, and the potential risk of confounding factors in the reviewed studies prevented any definite conclusions. Further well-designed prospective research using larger samples is warranted on this topic., (Copyright © 2019 Elsevier B.V. All rights reserved.)

Objective: To evaluate the canal wall up (CWU) technique combined with mastoid obliteration used in cholesteatoma surgery from the aspects of safety and function., Study Design: Retrospective chart review. Information was extracted from a medical database and complementary data from patient files and audiograms were collected and recorded retrospectively., Setting: A tertiary-stage hospital and a secondary-stage hospital. Surgeons of various levels of experience., Method: Data from a consecutive group of 230 primary operations for cholesteatoma surgery using CWU with obliteration employing the combined approach tympanoplasty (CAT) technique, from January 1994 to December 2009 were analyzed., Results: In 90% of the ears, the anatomy was normalized with an intact ear drum. The frequency of residual cholesteatoma was 1% and the frequency of recurrent cholesteatoma 8%. Hearing was improved one year postoperatively and remained improved three years postoperatively. No patient suffered a total hearing loss., Conclusions: CWU procedure comprising CAT with obliteration of the mastoid is a safe surgical method with a low frequency of residual and recurrent disease and a good hearing preservation.

Wolfram syndrome (WS) is caused by recessive mutations in the Wolfram syndrome 1 (WFS1) gene. Sensorineural hearing impairment (HI) is a frequent feature in WS and, furthermore, certain mutations in WFS1 cause nonsyndromic dominantly inherited low-frequency sensorineural HI. These two phenotypes are clinically distinct indicating that WFS1 is a reasonable candidate for genetic studies in patients with other phenotypes of HI. Here we have investigated, whether the variation in WFS1 has a pathogenic role in age-related hearing impairment (ARHI). WFS1 gene was investigated in a population sample of 518 Finnish adults born in 1938-1949 and representing variable hearing phenotypes. Identified variants were evaluated with respect to pathogenic potential. A rare mutation predicted to be pathogenic was found in a family with many members with impaired hearing. Twenty members were recruited to a segregation study and a detailed clinical examination. Heterozygous p.Tyr528His variant segregated completely with late-onset HI in which hearing deteriorated first at high frequencies and progressed to mid and low frequencies later in life. We report the first mutation in the WFS1 gene causing late-onset HI with audiogram configurations typical for ARHI. Monogenic forms of ARHI are rare and our results add WFS1 to the short list of such genes., (Copyright © 2017 Elsevier B.V. All rights reserved.)