A murine model for the del(GJB6-D13S1830) deletion recapitulating the phenotype of human DFNB1 hearing impairment: generation and functional and histopathological study.

Inherited hearing impairment is a remarkably heterogeneous monogenic condition, involving hundreds of genes, most of them with very small (< 1%) epidemiological contributions. The exception is GJB2, the gene encoding connexin-26 and underlying DFNB1, which is the most frequent type of autosomal recessive non-syndromic hearing impairment (ARNSHI) in most populations (up to 40% of ARNSHI cases). DFNB1 is caused by different types of pathogenic variants in GJB2, but also by large deletions that keep the gene intact but remove an upstream regulatory element that is essential for its expression. Such large deletions, found in most populations, behave as complete loss-of-function variants, usually associated with a profound hearing impairment. By using CRISPR-Cas9 genetic edition, we have generated a murine model (Dfnb1em274) that reproduces the most frequent of those deletions, del(GJB6-D13S1830). Dfnb1em274 homozygous mice are viable, bypassing the embryonic lethality of the Gjb2 knockout, and present a phenotype of profound hearing loss (> 90 dB SPL) that correlates with specific structural abnormalities in the cochlea. We show that Gjb2 expression is nearly abolished and its protein product, Cx26, is nearly absent all throughout the cochlea, unlike previous conditional knockouts in which Gjb2 ablation was not obtained in all cell types. The Dfnb1em274 model recapitulates the clinical presentation of patients harbouring the del(GJB6-D13S1830) variant and thus it is a valuable tool to study the pathological mechanisms of DFNB1 and to assay therapies for this most frequent type of human ARNSHI.

Disentangling the effects of hearing loss and age on amplitude modulation frequency selectivity.

The processing and perception of amplitude modulation (AM) in the auditory system reflect a frequency-selective process, often described as a modulation filterbank. Previous studies on perceptual AM masking reported similar results for older listeners with hearing impairment (HI listeners) and young listeners with normal hearing (NH listeners), suggesting no effects of age or hearing loss on AM frequency selectivity. However, recent evidence has shown that age, independently of hearing loss, adversely affects AM frequency selectivity. Hence, this study aimed to disentangle the effects of hearing loss and age. A simultaneous AM masking paradigm was employed, using a sinusoidal carrier at 2.8 kHz, narrowband noise modulation maskers, and target modulation frequencies of 4, 16, 64, and 128 Hz. The results obtained from young (n = 3, 24-30 years of age) and older (n = 10, 63-77 years of age) HI listeners were compared to previously obtained data from young and older NH listeners. Notably, the HI listeners generally exhibited lower (unmasked) AM detection thresholds and greater AM frequency selectivity than their NH counterparts in both age groups. Overall, the results suggest that age negatively affects AM frequency selectivity for both NH and HI listeners, whereas hearing loss improves AM detection and AM selectivity, likely due to the loss of peripheral compression.

Applications of Grounded Theory Methodology to Investigate Hearing Loss: A Methodological Qualitative Systematic Review With Developed Guidelines.

OBJECTIVES: Qualitative methodologies are commonly adopted in hearing loss research. Grounded theory methodology is increasingly used to establish novel theories explaining experiences related to hearing loss. Establishing and improving the quality of grounded theory studies has been emphasized as critical to ensuring theoretical trustworthiness. Thus, the primary aim of the present study was to systematically review hearing loss research studies that have applied grounded theory methodology and assess the methodological quality of those grounded theory applications. Secondarily aims were to (i) explore how grounded theory methodology has been applied to investigate hearing loss, and (ii) use the findings of the review to develop a set of guidelines to aid the future high-quality application of grounded theory methodology to hearing loss research. DESIGN: Original peer-reviewed studies applying grounded theory methodology and published in English were identified through systematic searches in 10 databases; Applied Social Sciences Index and Abstracts, British Nursing Index, Cumulative Index to Nursing and Allied Health Literature, EBSCO, Global Health, MEDLINE (OvidSP), PsycINFO, PubMed, Scopus, and Web of Science. The quality of studies was assessed according to 12 grounded theory principles using the Guideline for Reporting, Evaluating, and applying the core principles of Grounded Theory studies (GUREGT) tool. Data were analyzed using qualitative inductive thematic analysis. RESULTS: After the removal of duplicates, 155 articles were retrieved. Of those, 39 met the criteria for inclusion in the systematic review. An increase in the adoption of grounded theory methodology to investigate hearing loss was identified with the number of published studies tripling in the last 5 years. Critical appraisal using the GUREGT tool identified four studies as high-quality. Most included studies were of moderate study quality (n = 25), and 10 were classified as being of low study quality. Using inductive thematic analysis, the included studies investigated one of four areas relating to hearing loss: (a) Living with hearing loss, (b) Identity and hearing loss, (c) Coping strategies for hearing loss, and (d) Audiological counseling and rehabilitation. Analysis also identified four main grounded theory factors frequently overlooked in hearing loss research: the different schools of grounded theory, sampling strategy, sample size, and the depth of grounded theory application. CONCLUSIONS: Use of grounded theory methodology is increasing at a rapid rate in hearing loss research. Despite this, studies conducted in the field to date do not meet and apply the full spectrum of grounded theory principles, as outlined by the GUREGT tool. To improve methodological rigor in future studies using grounded theory, we propose a set of guidelines that address the most commonly overlooked methodological considerations in hearing loss studies to date. The guidelines are designed to aid researchers to achieve high methodological quality in any field, improve qualitative rigor, and promote theoretical credibility.

Remote or in-clinic? The effect of service delivery mode on hearing aid output: study protocol for a double-blinded, randomised trial in adults with mild to moderate sensorineural hearing loss.

BACKGROUND: Teleaudiology can potentially improve access to hearing healthcare services. Remote hearing aid fittings offer a new mode of service delivery that removes barriers of geography and access to an audiologist. Real-ear measurements (REMs) are the gold standard for hearing aid output verification but require in-clinic appointments. This study will investigate whether remote hearing aid fittings can provide clinically equivalent outcomes when compared to current, in-clinic, best practice guidelines. RESEARCH DESIGN: A repeated measure, double-blinded crossover design will be used. Participants will be randomly allocated to one of two groups to determine order of intervention, balanced for degree of hearing loss. STUDY SAMPLE: Sixty adults with mild to moderate hearing loss and at least 1 year of experience with hearing aids will be recruited. DATA COLLECTION AND ANALYSIS: Participants will complete two hearing aid fitting protocols, one using an in-clinic fitting process and the other using a remote (at-home) fitting process. In-clinic fittings will include REMs with adjustments to standard (NAL-NL2) prescription targets. The two fitting protocols will then be randomly assigned to participants in a crossover design, so participants and researchers will be blinded to the order of the two fitting protocols. Participants will then have a 4-week period with follow-up appointments for participant-directed gain adjustment. For each fitting protocol, participants will complete objective measurements of final hearing aid output with REMs, speech-in-noise testing, subjective measurements of hearing aid performance, and quality of life measurements. They will then begin an identical period of living with, adjusting, and objective assessment with the other fitting protocol. Data will be analysed as repeated measures with statistical control for potential confounding variables. RESULTS: Data will compare the four-frequency average real-ear aided response (4FREAR) for hearing aids programmed in-clinic and hearing aids programmed remotely, after participant-directed gain adjustments. Secondary measures will assess clinically significant differences in estimated speech intelligibility, hearing-related quality of life, hearing aid benefit, sound quality and preference, and speech-in-noise ability. CONCLUSIONS: This study will inform the development of best practice guidelines for remote hearing aid fittings. If no clinically significant differences are found between in-clinic and remote fit hearing aids, it has the potential to expand teleaudiology initiatives. TRIAL REGISTRATION: Australian New Zealand Clinical Trial Registry, ACTRN12623000028606p . Date of registration: 12 January 2023.

Availability of resources for paediatric hearing care in a South African province.

BACKGROUND:  Unavailability of healthcare resources can lead to poor patient outcomes. The latter is true for infants with hearing loss and require early hearing detection and intervention (EHDI). AIM:  To determine the availability and distribution of resources for EHDI in state hospitals in the Eastern Cape (EC) province, South Africa. SETTING:  Sixteen state hospitals (nine district, four regional and three tertiary hospitals). METHODS:  Descriptive cross-sectional survey completed between July 2022 and October 2022. RESULTS:  Thirteen hospitals had audiologists (n = 4) or speech therapists and audiologists (n = 9). Specific to equipment, 10 hospitals had a screening otoacoustic emissions or automated auditory brainstem response, 8 hospitals had diagnostic middle ear analysers and only 3 hospitals had diagnostic auditory brainstem response and/or auditory steady state response. Twelve hospitals did not have visual response audiometry (VRA) and 94% had no hearing aid verification systems. Budget allocations were uneven, with only 10 hospitals, i.e., 4 districts, all regional and 2 tertiary hospitals being allocated varying amounts. Subsequently, only 50% provided newborn hearing screening, 56% provided diagnostic evaluations and 14 hospitals fitted hearing aids. CONCLUSION:  Results revealed a limited and uneven distribution of resources, which negatively impacted the provision of EHDI. Even distribution of healthcare resources and further research aimed at strengthening hearing health services is recommended as these could potentially improve equitable access to EHDI and the overall quality of healthcare provided.Contribution: This study highlights the need for even distribution of resources and strengthening of health systems, especially in the dawn of the National Health Insurance.

A call for linguistic and culturally congruent family-centred early hearing detection and intervention programmes in South Africa.

Early Hearing Detection and Intervention (EHDI) programmes are recognised as the standard of care for newborns and infants presenting with hearing impairment, globally. However, widespread implementation of these programmes is far from being realised and faces numerous challenges within the South African context. The United Nations' sustainable development goal 3.8 and South Africa's national development plan seek to achieve equitable access to healthcare service, including EHDI. However, healthcare access is a complex concept which encompasses the dimensions: availability, affordability, acceptability and accommodation in healthcare. South Africa has made great progress towards universal implementation of EHDI programmes. Despite this progress, availability and affordability of these programmes are limited and their acceptability has received limited research focus in this context. Furthermore, accommodation of caregivers, as co-drivers of EHDI programmes and ensuring that EHDI programmes are linguistically and culturally congruent have also been overlooked within the South African context.Contribution: Increased robust efforts in improving access through availability and affordability of EHDI programmes are warranted in South Africa. However, improving access to these programmes through availability and affordability initiatives alone will not result in a pragmatic improvement in their accessibility. Acceptability of these programmes and accommodations such as involving caregivers and family members of children with hearing impairment as equal partners in EHDI programmes and being cognisant of their linguistic and cultural needs must be considered.

Help-seeking journey to accessing audiology services in a peri-urban community in South Africa.

BACKGROUND:  Hearing impairment is an invisible disability affecting one in five people globally. Its ability to affect participation in activities of daily living means that it requires prompt identification and intervention. OBJECTIVE:  This article aims to define the process of accessing audiologists from the onset of symptoms for adults with hearing impairment in a peri-urban community in South Africa. METHOD:  Twenty-three participants were recruited through purposive sampling from an audiology department of a public hospital. Semi-structured interviews were conducted using an interview guide, and data were mapped according to the participants' responses from the onset of ear and hearing symptoms to the point of audiologist consultation for analysis. RESULTS:  Seventeen (74%) participants had long journeys to accessing the audiologist after seeking help from multiple providers, with those with short journeys (26%) being referred mostly by public healthcare providers. Despite participants being from one peri-urban community, their journeys were influenced by socio-economics, health illiteracy and other structural factors. Finally, Ear-Nose-Throat specialists linked participants with audiology services. CONCLUSION:  Accessing audiology services is a complex process in some contexts. The disparities in the social environment, lifestyle factors and pluralistic healthcare models influence access to audiologists. Healthcare providers must take cognisance of the journeys of adults with hearing impairment in their clinical interventions. Universal health coverage, in the form of the planned National Health Insurance (NHI) for all South African citizens, will play an important role in addressing the societal inequalities in accessing healthcare. Factors leading to long journeys should be addressed to facilitate early intervention.Contribution: The study raises implications for the planned NHI in South Africa, suggesting that universal health coverage could play a vital role in addressing societal inequalities in accessing healthcare, including audiology services.

Virtual Staff Training to Support Communication for Older Adults With Untreated Hearing Loss in Care Settings.

PURPOSE: The current quasi-experimental study aimed to develop and evaluate a virtual staff training on age-related hearing loss at a care organization for older adults. METHOD: Training included the use of affordable headset amplifiers and a hands-on activity in which hearing loss was simulated. Staff were encouraged to offer amplifiers to assist in communication given the high prevalence of untreated hearing loss among older adults and the increased communication difficulty that results from mask-wearing. RESULTS: Quantitative results (N = 51) from the pre/post questionnaire suggest that staff members gained knowledge about hearing loss and communication through the training session. Qualitative data over the 6-month post training suggest that some older adults had not only improved speech understanding but also improved quality of interactions with staff. The main reasons for not using the amplifiers were that staff would forget they had access to the amplifiers or the older adult would refuse to use the device. CONCLUSION: This article highlights successes of the training as well as ideas for future trainings suggested by staff members. A key finding was the need to identify a core group of staff members who would be charged with facilitating use of personal amplification for older adults in the organization. In addition, providing multiple brief trainings over time was suggested to improve adoption of good communication practices among staff. [Journal of Gerontological Nursing, 50(4), 48-56.].

Plasma Levels of Interferon Gamma associated with Hearing Loss and Hearing Loss Sensation through the Handicap Questionnaire Inventory for the Elderly Screening Version.

OBJECTIVE: The aim of this study was to verify the association between the auditory handicap found in the Hearing Handicap Inventory for the Elderly-Screening Version (HHIE-S) questionnaire and hearing loss and the plasma levels of inflammatory biomarkers. MATERIALS AND METHODS: Cross-sectional study with 76 participants, 67 (88%) females and 9 (12%) males, with a mean age of 70 years. Tonal threshold audiometry and self-assessment with HHIE-S questionnaire were performed to measure the plasma levels of interleukin-2 (IL-2), IL-4, IL-6, and IL-10; tumor necrosis factor alpha; and interferon gamma (IFN-γ) flow cytometry method. For all data analyzed, the significance level adopted was P < 0.05 and 95% confidence interval. RESULTS: An inverse correlation was observed between the increase in plasma levels of IFN-γ and normal auditory handicap (P = 0.015; rs = -0.280). The severe handicap group showed an increase in the averages I (P = 0.005; rs = 0.350) and II (P = 0.016; rs = 0.368) in the right ear and the light/moderate handicap group increased the means I (P = 0.027; rs = 0.350) and II (P = 0.046; rs = 0.310) of the left ear. A statistically significant association was found between the speech recognition threshold (SRT) test results of the right ear and the severe handicap group (P = 0.002; rs = 0.271). CONCLUSIONS: There was an association between the increase in plasma levels of IFN-γ and normal auditory handicap. Additionally, statistically significant associations were observed between the mild/moderate and severe handicap groups with the increase in hearing means and an increase in SRT associated with the severe handicap group.

Demographic analysis of hearing impairment based on various parameters in patients with cochlear implant.

Objectives: To analyse the demographic and clinical variables in children having undergone cochlear implant surgery because of deafness. METHODS: The cross-sectional study was conducted from January to November 2022 at the Centre for Research in Experimental and Applied Medicine laboratory of the Department of Biochemistry and Molecular Biology, Army Medical College, Rawalpindi, Pakistan, in collaboration with the Ear, Nose and Throat Department of Combined Military Hospital, Rawalpindi, and comprised children of eith gender aged up to 10 years who had received cochlear implant. Data was collected through questionnaire-based detailed interviews. Syndromic Hearing Loss, Non-Syndromic Hearing Loss, and Acquired Hearing Loss were identified among the subjects. Data was analysed using SPSS 22. RESULTS: Of the 250 cases, 147(58.8%) were boys, 146(58.4%) were aged 0-5 years, 219(87.6%) had prelingual onset of disease, and 202(80.8%) had a non-progressive disease course. In 203(81.2%) cases, normal developmental milestones were seen. Parental consanguinity was observed in 219(87.6%) cases. However, 63(25.2%) patients had a first-degree relative who had a history of deafness. In 170(68%) cases, hearing loss was hereditary, whereas in 80(32%) it was acquired. Meningitis was the most commonly identified risk factor 55(68.75%). Acquired risk factors and family history had significant association with hearing loss (p<0.05). Speech perception significantly improved in all 219(100%) patients with prelingual hearing loss who underwent cochlear implantation. CONCLUSIONS: Majority of the cases were found to be male, had a prelingual disease onset and a non-progressive disease course. Family history was a significant factor, while meningitis was the most common acquired cause of hearing loss.

Talker change detection by listeners varying in age and hearing loss.

Despite a vast literature on how speech intelligibility is affected by hearing loss and advanced age, remarkably little is known about the perception of talker-related information in these populations. Here, we assessed the ability of listeners to detect whether a change in talker occurred while listening to and identifying sentence-length sequences of words. Participants were recruited in four groups that differed in their age (younger/older) and hearing status (normal/impaired). The task was conducted in quiet or in a background of same-sex two-talker speech babble. We found that age and hearing loss had detrimental effects on talker change detection, in addition to their expected effects on word recognition. We also found subtle differences in the effects of age and hearing loss for trials in which the talker changed vs trials in which the talker did not change. These findings suggest that part of the difficulty encountered by older listeners, and by listeners with hearing loss, when communicating in group situations, may be due to a reduced ability to identify and discriminate between the participants in the conversation.

Trends in prevalence of hearing loss in adults in the USA 1999-2018: a cross-sectional study.

BACKGROUND: A better understanding of how the prevalence of hearing loss and its associated factors change over time could help in developing an appropriate program to prevent the development of hearing loss. METHODS: Population-representative cross-sectional data from the United States National Health and Nutrition Examination Survey (NHANES) were used to estimate the trends in the prevalence of hearing loss among adults in the USA over the period 1999-2018. A total of 15,498 adult participants aged 20 years or older had complete audiometric examination data. Logistic regression was employed to evaluate the trend in hearing loss; weighted Rao-Scott χ2 tests and univariate logistic regression analyses were used to examine the association between hearing loss and relevant factors. RESULTS: The overall hearing loss prevalence in 1999-2018 was 19.1% 19.1 (95% CI, 18.0-20.2%). The prevalence of hearing loss decreased in cycles (P for trend < 0.001). For participants aged 20-69 years, the prevalence decreased from 15.6% (95% CI, 12.9-18.4%) in 1999-2000 to 14.9% (95% CI, 13.2- 16.6%) in 2015-2016; for participants aged > 70 years the prevalence decreased from 79.9% (95% CI, 76.1-83.8%) in 2005-2006 to 64.5% (95% CI, 58.8-70.2%) in 2017-2018. Participants with hearing loss were likely to be older, male, non-Hispanic white, and to have not completed high school. Mild hearing loss was more prevalent among those aged 20-79 years; in those aged over 80 years the prevalence of moderate hearing loss exceeded that of mild loss. Among all otologically normal participants, hearing thresholds increased with age across the entire frequency range. CONCLUSIONS: The prevalence of hearing loss in USA adults changed over the period 1999-2018. The trends observed provide valuable insight for making public health plans and allocating resources to hearing care. Further investigation is necessary to monitor hearing loss and its potential risk factors.

Ivacaftor attenuates gentamicin-induced ototoxicity through the CFTR-Nrf2-HO1/NQO1 pathway.

OBJECTIVES: Gentamicin is one of the most common ototoxic drugs that can lower patients' quality of life. Oxidative stress is a key factors inducing sensory hair cell death during gentamicin administration. So far, there are no effective drugs to prevent or treat gentamicin- induced hearing loss. A recent study found cystic fibrosis transmembrane conductance regulator (CFTR) as a new target to modulate cellular oxidative balance. The objective of this study was to estimate the effect of the CFTR activator ivacaftor on gentamicin-induced ototoxicity and determine its mechanism. METHODS: The hair cell count was analyzed by Myosin 7a staining. Apoptosis was analyzed by TUNEL Apoptosis Kit. Cellular reactive oxygen species (ROS) level was detected by DCFH-DA probes. The Nrf2 related proteins expression levels were analyzed by western blot. RESULTS: An in vitro cochlear explant model showed that gentamicin caused ROS accumulation in sensory hair cells and induced apoptosis, and this effect was alleviated by pretreatment with ivacaftor. Western blotting showed that ivacaftor administration markedly increased the protein expression of nuclear factor erythroid 2-related factor 2 (Nrf2), heme oxygenase-1 (HO1), and NAD(P)H:quinone oxidoreductase 1 (NQO1). The protective effect of ivacaftor was abolished by the Nrf2 inhibitor ML385. DISCUSSION: Our results indicate the protective role of the CFTR-Nrf2-HO1/NQO1 pathway in gentamicin-induced ototoxicity. Ivacaftor may be repositioned or repurposed towards aminoglycosides-induced hearing loss.

Australian families of deaf and hard of hearing children: Are they using sign?

BACKGROUND: Deaf and hard of hearing (DHH) children may experience communication delays, irrespective of early intervention and technology. Australian Sign Language (Auslan) is one approach in early intervention to address language delays. Current prevalence of Auslan use among Australian families with DHH children is unknown. AIMS: The first aim was to determine the proportion of families enrolled in an Australian statewide hearing loss databank who use Auslan with their DHH child. The second aim was to explore the relationships between indicators of child hearing loss (bilateral or unilateral hearing loss, degree of hearing loss, and device use: hearing aids and cochlear implants), family factors (maternal education, attendance at early intervention, family history of deafness, and socio-economic disadvantage) and the family's reported use of Auslan. METHODS: We analysed the enrolment data from 997 families who participated in an Australian statewide hearing loss databank between 2012 and 2021. We described the proportion of families who used Auslan with their DHH child at home. The association between indicators of child hearing loss and family factors, and the parental reports of communication approach were examined using correlation analyses. RESULTS: Eighty-seven of 997 parents (8.7%) reported using Auslan with their DHH child. Of these, 26 (2.6%) used Auslan as their primary language. The use of Auslan at home was associated with the following indicators of child hearing loss: bilateral hearing loss, profound compared to mild hearing loss, and cochlear implant and hearing aid use compared to no device use. The family factors associated with the use of Auslan were: referral or attendance at early intervention compared to those who did not attend, and a family history of deafness compared to those with none. No association was found between maternal education and socio-economic disadvantage and the use of Auslan. CONCLUSION: This Australian study found a low proportion (8.7%) of families with a DHH child who reported using Auslan. Seven child hearing loss and family factors were considered, and five were significantly associated with using Auslan at home. Children with a greater degree of hearing loss, attendance at early intervention and family history of deafness tended to use Auslan.

Increased risk of psychiatric disorder in patients with hearing loss: a nationwide population-based cohort study.

BACKGROUND: Hearing loss has been shown to be a risk factor for psychiatric disorders. In addition, long-term hearing loss is associated with increased hospitalization and mortality rates; however, the increased risk and duration of effect of hearing loss in combination with other chronic diseases on each psychiatric disorder are still not clearly defined. The purpose of this article is to clarify the risk of hearing loss for each disorder over time. METHODS: This was a retrospective cohort study, and a national health insurance research database in Taiwan was utilized. All (n = 1,949,101) Taiwanese residents who had a medical visit between 2000 and 2015 were included. Patients with hearing loss and a comparative retrospective cohort were analyzed. Every subject was tracked individually from their index date to identify the subjects who later received a diagnosis of a psychiatric disorder. The Kaplan‒Meier method was used to analyze the cumulative incidence of psychiatric disorders. Cox regression analysis was performed to identify the risk of psychiatric disorders. RESULTS: A total of 13,341 (15.42%) and 31,250 (9.03%) patients with and without hearing loss, respectively, were diagnosed with psychiatric disorders (P < 0.001). Multivariate analysis indicated that hearing loss significantly elevated the risk of psychiatric disorders (adjusted HR = 2.587, 95% CI 1.723-3.346, p < 0.001). CONCLUSION: Our findings indicate that patients with hearing loss are more likely to develop psychiatric disorders. Furthermore, the various psychiatric disorders are more likely to occur at different times. Our findings have important clinical implications, including a need for clinicians to implement early intervention for hearing loss and to pay close attention to patients' psychological status. Trial registration TSGHIRB No. E202216036.

Guía de práctica clínica sobre el manejo del schwannoma vestibular / Clinical practice guideline on the management of vestibular schwannoma

Introducción: El schwannoma vestibular (SV) es el tumor más frecuente del ángulo pontocerebeloso. La mayor accesibilidad a las pruebas radiológicas ha incrementado su diagnóstico. Teniendo en cuenta las características del tumor, la clínica y la edad del paciente se han propuesto tres estrategias terapéuticas, observación, cirugía o radioterapia. La elección de la más adecuada para cada paciente es un motivo de controversia frecuente. Material y métodos: El presente trabajo incluye una revisión exhaustiva sobre cuestiones relativas al SV que pueden servir de guía clínica en el manejo de pacientes con estas lesiones. La presentación se ha orientado en forma de preguntas que el clínico se hace habitualmente y las respuestas están redactadas y/o revisadas por un panel de expertos nacionales e internacionales consultados por la Comisión de Otología de la SEORL-CCC. Resultados: Se ha elaborado un listado con los 13 bloques temáticos más controvertidos sobre el manejo del SV en forma de 50 preguntas y se han buscado las respuestas a todas ellas mediante una revisión sistemática de la literatura (artículos publicados en PubMed y Cochrane Library entre 1992 y 2023 sobre cada bloque temático). Treinta y tres expertos, liderados por la Comisión de Otología de la SEORL-CCC, han analizado y discutido todas las respuestas. En el Anexo 1 pueden encontrarse 14 preguntas adicionales divididas en cuatro bloques temáticos. Conclusiones: Esta guía de práctica clínica sobre el manejo del SV ofrece respuestas consensuadas a las preguntas más habituales que se plantean sobre este tumor. La ausencia de suficientes estudios prospectivos hace que los niveles de evidencia sobre el tema sean en general medios o bajos. Este hecho incrementa el interés de este tipo de guías de práctica clínica elaboradas por expertos.(AU)

IntroductionVestibular schwannoma (VS) is the most common tumour of the cerebellopontine angle. The greater accessibility to radiological tests has increased its diagnosis. Taking into account the characteristics of the tumour, the symptoms and the age of the patient, three therapeutic strategies have been proposed: observation, surgery or radiotherapy. Choosing the most appropriate for each patient is a frequent source of controversy. Material and methods: This paper includes an exhaustive literature review of issues related to VS that can serve as a clinical guide in the management of patients with these lesions. The presentation has been oriented in the form of questions that the clinician usually asks himself and the answers have been written and/or reviewed by a panel of national and international experts consulted by the Otology Commission of the SEORL-CCC. Results: A list has been compiled containing the 13 most controversial thematic blocks on the management of VS in the form of 50 questions, and answers to all of them have been sought through a systematic literature review (articles published on PubMed and Cochrane Library between 1992 and 2023 related to each thematic area). Thirty-three experts, led by the Otology Committee of SEORL-CCC, have analyzed and discussed all the answers. In Annex 1, 14 additional questions divided into 4 thematic areas can be found. Conclusions: This clinical practice guideline on the management of VS offers agreed answers to the most common questions that are asked about this tumour. The absence of sufficient prospective studies means that the levels of evidence on the subject are generally medium or low. This fact increases the interest of this type of clinical practice guidelines prepared by experts.(AU)

Factors Associated with Lower Social Activity in German Older Adults at Increased Risk of Dementia: A Cross-Sectional Analysis.

Background: Studies demonstrate associations between low social activity in older adults and cognitive decline. Little has been investigated regarding which factors are associated with low social activity in older adults at increased risk of dementia. Objective: We investigate which sociodemographic, psychological, health-related, and environmental factors are associated with low social activity in older adults at increased risk of dementia. Additionally, we describe the stages of health behavior change, the types of social activities, and the duration of the current level of social activity. Methods: We used baseline data of 1,015 participants from the AgeWell.de trial. We conducted logistic and Poisson regression analyses to investigate factors associated with low social activity. We report descriptive statistics on the stages of change in the sample, the types of social activities most frequently pursued, and the duration of the current level of social activity. Results: Lower income, non-usage of public transport, depressive symptoms, cognitive, mobility, and hearing impairment were negatively associated with social activity. The majority of the sample was in the maintenance stage, followed by the precontemplation stage. The most common social activities were traveling and hobbies with others. Participants have maintained their current level of social activity for several years. Conclusions: We identified a lack of resources (income, transport), depressive symptoms and poorer health (cognitive, mobility and hearing impairment) as barriers to social activity. Interventions promoting social activity in older adults at risk of dementia may specifically target individuals with these risk factors. Low-threshold opportunities for social activity may be particularly beneficial.

Prediction of pure tone thresholds using the speech reception threshold and age in elderly individuals with hearing loss.

OBJECTIVE: Early detection and effective management of hearing loss constitute the key to improving the quality of life of individuals with hearing loss. However, in standardized pure tone audiometry, it is sometimes difficult for elderly patients to understand and follow all instructions. Audiologists also require time, expertise, and patience to ensure that an elderly can identify the faintest levels of stimuli during a hearing test. Therefore, this study aimed to devise and validate a formula to predict the pure tone threshold at each frequency across 0.5-4 kHz (PTTs) using speech reception threshold. METHODS: The 1226 audiograms of hearing-impaired individuals aged 60-90 years were reviewed. The random sample function randomly assigned 613 participants to the training and testing sets each. A linear model was created to predict the PTT value at each frequency based on variables significant at all frequencies across 0.5-4 kHz. The adjusted-R2 value was considered to indicate the performance of the predictive model. Pearson's correlation coefficient was used to describe the relationship between the actual and predicted PTT at 0.5, 1, 2, and 4 kHz among the testing set to measure the performance of the proposed model. RESULTS: The predictive model was devised using variables based on the speech recognition threshold (SRT) after adjusting with age in the training set. The overall prediction accuracy demonstrated a higher adjusted-R2 ranging from 0.74 to 0.89 at frequencies of 0.5, 1, and 2 kHz, whereas a low percentage of explained variance was observed at 4 kHz (adjusted-R2 = 0.41). This predictive model can serve as an adjunctive clinical tool for guiding determination of the PTTs. Moreover, the predicted PTTs can be applied in the hearing aid programming software to set appropriate hearing aid gain using standard prescriptive formulas.

(Why) Do Transparent Hearing Devices Impair Speech Perception in Collocated Noise?

Hearing aids and other hearing devices should provide the user with a benefit, for example, compensate for effects of a hearing loss or cancel undesired sounds. However, wearing hearing devices can also have negative effects on perception, previously demonstrated mostly for spatial hearing, sound quality and the perception of the own voice. When hearing devices are set to transparency, that is, provide no gain and resemble open-ear listening as well as possible, these side effects can be studied in isolation. In the present work, we conducted a series of experiments that are concerned with the effect of transparent hearing devices on speech perception in a collocated speech-in-noise task. In such a situation, listening through a hearing device is not expected to have any negative effect, since both speech and noise undergo identical processing, such that the signal-to-noise ratio at ear is not altered and spatial effects are irrelevant. However, we found a consistent hearing device disadvantage for speech intelligibility and similar trends for rated listening effort. Several hypotheses for the possible origin for this disadvantage were tested by including several different devices, gain settings and stimulus levels. While effects of self-noise and nonlinear distortions were ruled out, the exact reason for a hearing device disadvantage on speech perception is still unclear. However, a significant relation to auditory model predictions demonstrate that the speech intelligibility disadvantage is related to sound quality, and is most probably caused by insufficient equalization, artifacts of frequency-dependent signal processing and processing delays.