Bi-allelic variants in the mitochondrial RNase P subunit PRORP cause mitochondrial tRNA processing defects and pleiotropic multisystem presentations.

Human mitochondrial RNase P (mt-RNase P) is responsible for 5' end processing of mitochondrial precursor tRNAs, a vital step in mitochondrial RNA maturation, and is comprised of three protein subunits: TRMT10C, SDR5C1 (HSD10), and PRORP. Pathogenic variants in TRMT10C and SDR5C1 are associated with distinct recessive or x-linked infantile onset disorders, resulting from defects in mitochondrial RNA processing. We report four unrelated families with multisystem disease associated with bi-allelic variants in PRORP, the metallonuclease subunit of mt-RNase P. Affected individuals presented with variable phenotypes comprising sensorineural hearing loss, primary ovarian insufficiency, developmental delay, and brain white matter changes. Fibroblasts from affected individuals in two families demonstrated decreased steady state levels of PRORP, an accumulation of unprocessed mitochondrial transcripts, and decreased steady state levels of mitochondrial-encoded proteins, which were rescued by introduction of the wild-type PRORP cDNA. In mt-tRNA processing assays performed with recombinant mt-RNase P proteins, the disease-associated variants resulted in diminished mitochondrial tRNA processing. Identification of disease-causing variants in PRORP indicates that pathogenic variants in all three subunits of mt-RNase P can cause mitochondrial dysfunction, each with distinct pleiotropic clinical presentations.

Hearing Loss and Dementia: Where to From Here?

Victorian era psychologists were the first to comment on associations between sensory and cognitive function. More recently, hearing loss has been shown as a marker of risk for dementia. However, it is not known whether this association represents a causal impact of hearing loss, nor whether treating hearing loss may help prevent dementia. Most studies on relationships between hearing loss and cognitive outcomes are observational, are at risk of confounding, and cannot reach conclusions about causation. A recent high quality randomized controlled trial, relatively uncommon in audiology, reported no impact of a comprehensive hearing intervention in mitigating cognitive decline in older adults. Although secondary analysis revealed potential benefits in a sub-sample of adults, this finding may be spurious. Encouraging policymakers, patients, and other health care practitioners to address hearing loss in terms of dementia prevention may be inappropriate on the grounds of both relevance at individual level and lack of clear evidence of benefit. In addition, advocating need to address hearing loss in terms of mitigating dementia risk may reduce the importance of addressing hearing loss in its own right. Linking hearing loss to dementia risk may also exacerbate the stigma of hearing loss, inadvertently discouraging people from seeking help for hearing. We suggest that treating hearing loss may have important benefits in preventing or delaying diagnosis of dementia via improving orientation and functioning in daily life, without changing the underlying pathology. Rather than linking hearing loss to dementia risk, we suggest a positive message focusing on the known benefits of addressing hearing loss in terms of improved communication, quality of life, and healthy aging.

Acoustic reflexes: should we be paying more attention?

OBJECTIVE: The clinical audiology test battery often involves playing physically simple sounds with questionable ecological value to the listener. In this technical report, we revisit how valid this approach is using an automated, involuntary auditory response; the acoustic reflex threshold (ART). DESIGN: The ART was estimated four times in each individual in a quasi-random ordering of task conditions. The baseline condition (referred to as Neutral) measured the ART following a standard clinical practice. Three experimental conditions were then used in which a secondary task was performed whilst the reflex was measured: auditory attention, auditory distraction and visual distraction tasks. STUDY SAMPLE: Thirty-eight participants (27 males) with a mean age of 23 years were tested. All participants were audiometrically healthy. RESULTS: The ART was elevated when a visual task was performed at the same time as the measurements were taken. Performing an auditory task did not affect the ART. CONCLUSIONS: These data indicate that simple audiometric measures widely used in the clinic, can be affected by central, non-auditory processes even in healthy, normal-hearing volunteers. The role of cognition and attention on auditory responses will become ever more important in the coming years.

Reporting of auditory symptoms over time: (in)consistencies, expectations and the nocebo effect.

Objective: Consistent symptom reporting for conditions like tinnitus that do not have an associated sign is critical for evaluating severity and intervention effectiveness, and for interpreting research findings. There is little research examining reporting of tinnitus and hearing difficulty over time. We address this here by comparing reported hearing difficulty and tinnitus at two time-points.Design: A cross-sectional study comparing symptom reporting in March 2019 and August/September 2021 using data from two online surveys of the same cohort. Although each survey was designed to address a different question, both asked about symptoms of tinnitus and hearing difficulties and enabled this exploratory analysis.Study sample: 6881 members of the UK general public aged 18+ years.Results: Inconsistent reporting was evident - many participants who reported experiencing tinnitus and/or hearing difficulties in 2019, said in 2021 that they had never had such symptoms before. Additionally, reports of new tinnitus/hearing difficulties in 2021 were unexpectedly high, equating to 18-month incidence rates of 13.6% and 11.7%, respectively.Conclusions: Psychosocial factors, expectations and context impact symptom reporting. This should be considered when treating patients and interpreting research findings. Using real-time data collection methods could thus provide a better understanding of experiences of tinnitus and hearing.

What factors are associated with infant hearing aid use? A parent survey using the Theoretical Domains Framework.

OBJECTIVE: Hearing aid use is lowest in 0-3-year-olds with hearing loss, placing spoken language development at risk. Existing interventions lack effectiveness and are typically not based on a theoretically driven, comprehensive understanding of the factors influencing infant hearing aid use. The present study is the first to address this gap in understanding. DESIGN AND STUDY SAMPLE: A 55-item online survey based on the Theoretical Domains Framework (TDF) was completed by 56 parents of 0-3-year-old hearing aid users. RESULTS: Participants reported a wide range of barriers across TDF domains, which were associated with parent-reported hearing aid use and more pronounced in parents of lower hearing aid users. The most strongly reported domains across participants were "emotion" (e.g. feelings of worry when using hearing aids), "beliefs about capabilities" (e.g. belief in ability to use hearing aids consistently), and "environmental context and resources" (e.g. child removing hearing aids). CONCLUSIONS: Parents report a wider range of barriers to infant hearing aid use than existing investigations suggest and current interventions address. Interventions would benefit from: (i) targeting a wider range of TDF domains in their design; and (ii) implementing the present TDF survey to identify and target family-specific barriers to infant hearing aid use.

Development of a novel high-throughput screen for the identification of new inhibitors of protein S-acylation.

Protein S-acylation is a reversible post-translational modification that modulates the localization and function of many cellular proteins. S-acylation is mediated by a family of zinc finger DHHC (Asp-His-His-Cys) domain-containing (zDHHC) proteins encoded by 23 distinct ZDHHC genes in the human genome. These enzymes catalyze S-acylation in a two-step process involving "autoacylation" of the cysteine residue in the catalytic DHHC motif followed by transfer of the acyl chain to a substrate cysteine. S-acylation is essential for many fundamental physiological processes, and there is growing interest in zDHHC enzymes as novel drug targets for a range of disorders. However, there is currently a lack of chemical modulators of S-acylation either for use as tool compounds or for potential development for therapeutic purposes. Here, we developed and implemented a novel FRET-based high-throughput assay for the discovery of compounds that interfere with autoacylation of zDHHC2, an enzyme that is implicated in neuronal S-acylation pathways. Our screen of >350,000 compounds identified two related tetrazole-containing compounds (TTZ-1 and TTZ-2) that inhibited both zDHHC2 autoacylation and substrate S-acylation in cell-free systems. These compounds were also active in human embryonic kidney 293T cells, where they inhibited the S-acylation of two substrates (SNAP25 and PSD95 [postsynaptic density protein 95]) mediated by different zDHHC enzymes, with some apparent isoform selectivity. Furthermore, we confirmed activity of the hit compounds through resynthesis, which provided sufficient quantities of material for further investigations. The assays developed provide novel strategies to screen for zDHHC inhibitors, and the identified compounds add to the chemical toolbox for interrogating cellular activities of zDHHC enzymes in S-acylation.

The NLRP3 inflammasome as a target for sensorineural hearing loss.

Sensorineural hearing loss is the most common type of hearing loss in adults and occurs due to damage of the inner ear caused by a range of factors including ageing, excessive noise, toxins, and cancer. Auto-inflammatory disease is also a cause of hearing loss and there is evidence that inflammation could contribute to hearing loss in other conditions. Within the inner ear there are resident macrophage cells that respond to insults and whose activation correlates with damage. The NLRP3 inflammasome is a multi-molecular pro-inflammatory protein complex that forms in activated macrophages and may contribute to hearing loss. The aim of this article is to discuss the evidence for the NLRP3 inflammasome and associated cytokines as potential therapeutic targets for sensorineural hearing loss in conditions ranging from auto-inflammatory disease to tumour-induced hearing loss in vestibular schwannoma.

Aided Cortical Auditory Evoked Potentials in Infants With Frequency-Specific Synthetic Speech Stimuli: Sensitivity, Repeatability, and Feasibility.

OBJECTIVES: The cortical auditory evoked potential (CAEP) test is a candidate for supplementing clinical practice for infant hearing aid users and others who are not developmentally ready for behavioral testing. Sensitivity of the test for given sensation levels (SLs) has been reported to some degree, but further data are needed from large numbers of infants within the target age range, including repeat data where CAEPs were not detected initially. This study aims to assess sensitivity, repeatability, acceptability, and feasibility of CAEPs as a clinical measure of aided audibility in infants. DESIGN: One hundred and three infant hearing aid users were recruited from 53 pediatric audiology centers across the UK. Infants underwent aided CAEP testing at age 3 to 7 months to a mid-frequency (MF) and (mid-)high-frequency (HF) synthetic speech stimulus. CAEP testing was repeated within 7 days. When developmentally ready (aged 7-21 months), the infants underwent aided behavioral hearing testing using the same stimuli, to estimate the decibel (dB) SL (i.e., level above threshold) of those stimuli when presented at the CAEP test sessions. Percentage of CAEP detections for different dB SLs are reported using an objective detection method (Hotellings T 2 ). Acceptability was assessed using caregiver interviews and a questionnaire, and feasibility by recording test duration and completion rate. RESULTS: The overall sensitivity for a single CAEP test when the stimuli were ≥0 dB SL (i.e., audible) was 70% for the MF stimulus and 54% for the HF stimulus. After repeat testing, this increased to 84% and 72%, respectively. For SL >10 dB, the respective MF and HF test sensitivities were 80% and 60% for a single test, increasing to 94% and 79% for the two tests combined. Clinical feasibility was demonstrated by an excellent >99% completion rate, and acceptable median test duration of 24 minutes, including preparation time. Caregivers reported overall positive experiences of the test. CONCLUSIONS: By addressing the clinical need to provide data in the target age group at different SLs, we have demonstrated that aided CAEP testing can supplement existing clinical practice when infants with hearing loss are not developmentally ready for traditional behavioral assessment. Repeat testing is valuable to increase test sensitivity. For clinical application, it is important to be aware of CAEP response variability in this age group.

Cultural competence in NHS hearing aid clinics: a mixed-methods case study of services for Deaf British sign language users in the UK.

BACKGROUND: This study identified and explored how National Health Service (NHS) hearing aid clinics address cultural competence concerning Deaf British Sign Language (BSL) users. This was approached by (i) investigating how organisational processes meet the needs of Deaf signers from a hospital and hearing aid clinic perspective, (ii) analysing policies and guidelines to investigate if they equip practitioners to meet the needs of Deaf signers and (iii) exploring with practitioners who work in hearing aid clinics about their experiences of working with Deaf signers. METHODS: This study utilised a mixed-methods multiple case study design, incorporating documentary analysis and semi-structured interviews. Interview analysis was conducted using Reflexive Thematic Analysis (RTA). The research encompassed two hearing aid clinics in separate hospitals, producing 19 documents and eight interviews (four at each site) with audiologists ensuring a representative mix of professional experience levels. RESULTS: Four themes emerged from the integrated analysis: (1) Understanding Deaf signers; (2) Communicating with Deaf signers; (3) Barriers and Facilitators and (4) Service improvement. A noticeable gap in understanding BSL as both a language and a cultural system was apparent across various policies, strategies, training programmes and staff expertise. Over-reliance on interpreters provided a false sense of accessibility and most participants felt tentative to engage directly with Deaf signers. Positive practices observed at Sites A and B encompassed accurate identification of patients as Deaf signers, improved interpreter availability, communication methods, enhanced training and the encouragement of professional self-awareness. CONCLUSION: This is the first study that explores cultural competence of hearing aid clinics and its staff concerning Deaf signers in the UK. The results show both clinics require development to become an effective provider for culturally Deaf signers. Examples of how to design culturally competent practices have been provided to assist hearing aid clinics. The findings may be applicable to other underrepresented groups who are not typical users of conventional, acoustic hearing aids provided by the NHS.

Do we need audiogram-based prescriptions? A systematic review.

OBJECTIVE: Hearing aids are typically programmed using the individual's audiometric thresholds and verified using real-ear measures. Developments in technology have resulted in a new category of direct-to-consumer devices, which are not necessarily programmed using the individual's audiometric thresholds. This review aimed to identify whether programming hearing aids using the individual's validated audiogram-based prescription, and verified using real-ear measures, results in better outcomes for adults with hearing loss. DESIGN: The review was registered in PROSPERO and reported in accordance with PRISMA guidelines. STUDY SAMPLE: After screening more than 1370 records, seven experimental studies met the eligibility criteria. RESULTS: Studies were categorised under three methods of fitting: (i) comparative fitting evaluating different settings based on the beliefs of the audiologist; (ii) client choice of preset responses; and (iii) self-fit by adjustment. The findings suggest that using a prescription based on the individual's audiogram improves outcomes relative to the comparative and client choice fitting approaches. Self-adjustment during daily use may produce equivalent (or better) outcomes than an audiogram-based prescription. The quality of evidence for the outcomes ranged from low to very low. CONCLUSIONS: This review has highlighted the dearth of high-quality studies on which to make evidence-based decisions on hearing aid fitting methods.

Longitudinal assessment of listening skills in UK infants with hearing aids using the LittlEARS® auditory questionnaire.

OBJECTIVE: To investigate listening skills in infant hearing aid users using the LittlEARS® Auditory Questionnaire (LEAQ). DESIGN: Caregivers completed the LEAQ, and hearing aid data logging was recorded, at infant age 3-7 months and 7-21 months. STUDY SAMPLE: Seventy infant hearing aid users with permanent bilateral hearing loss, no developmental comorbidities, aged 3-7 months at first visit. RESULTS: Infants with mild and moderate losses tended to have scores within the normative range at the early time point (88%), but 29% were below the normative range when older. Thirty percent of infants with severe hearing loss were outside the normative range at the early time point and 60% outside the normative range when older. Infants with profound loss were almost always (95%) outside the normative range. At the later time point, and for infants with severe-to-profound loss, low LEAQ scores were associated with fewer daily hours hearing aid use. Scores were poorer than previous reports in the literature for infant hearing aid users. CONCLUSIONS: This study provides further knowledge on infant listening performance and hearing aid use over time that can be used to guide management of individual cases and to develop and audit service quality improvements.

Establishing the reliability and the validity of the Arabic translated versions of the Effort Assessment Scale and the Fatigue Assessment Scale.

OBJECTIVE: The aim was to establish the reliability and the validity of Arabic translated versions of the Fatigue Assessment Scale (FAS) and the Effort Assessment Scale (EAS). DESIGN: The FAS and the EAS were translated from the original English following a recommended six-step approach for translating hearing-related questionnaires for different languages. The reliability of the scales was investigated using Cronbach's alpha, item-total correlation, and inter-item correlation. Construct validity was investigated using factor analysis and the hypothesis testing method. STUDY SAMPLE: The translated scales were completed by 146 participants from Jordan and Saudi Arabia (age range 19-86 years old, 39% male). Participants' hearing level ranged from normal to profound. RESULTS: Item 3 in the translated FAS was removed to improve the scale's construct validity. The translated version of the EAS was found to be as reliable and valid as the original EAS. CONCLUSIONS: The availability of standardised versions of the FAS and the EAS provides a quick and easy method for improving hearing rehabilitation in Arabic-speaking countries where audiology services can often be costly and not necessarily accessible to all individuals.

Web- and app-based tools for remote hearing assessment: a scoping review.

OBJECTIVE: Remote hearing screening and assessment may improve access to, and uptake of, hearing care. This review, the most comprehensive to date, aimed to (i) identify and assess functionality of remote hearing assessment tools on smartphones and online platforms, (ii) determine if assessed tools were also evaluated in peer-reviewed publications and (iii) report accuracy of existing validation data. DESIGN: Protocol was registered in INPLASY and reported according to PRISMA-Extension for Scoping Reviews. STUDY SAMPLE: In total, 187 remote hearing assessment tools (using tones, speech, self-report or a combination) and 101 validation studies met the inclusion criteria. Quality, functionality, bias and applicability of each app were assessed by at least two authors. RESULTS: Assessed tools showed considerable variability in functionality. Twenty-two (12%) tools were peer-reviewed and 14 had acceptable functionality. The validation results and their quality varied greatly, largely depending on the category of the tool. CONCLUSION: The accuracy and reliability of most tools are unknown. Tone-producing tools provide approximate hearing thresholds but have calibration and background noise issues. Speech and self-report tools are less affected by these issues but mostly do not provide an estimated pure tone audiogram. Predicting audiograms using filtered language-independent materials could be a universal solution.

Inter-rater reliability in classification of canonical babbling status based on canonical babbling ratio in infants with isolated cleft palate randomised to Timing of Primary Surgery for Cleft Palate (TOPS).

Canonical babbling (CB) is commonly defined as present when at least 15% of all syllables produced are canonical, in other words a canonical babbling ratio (CBR) ≥0.15. However, there is limited knowledge about inter-rater reliability in classification of CB status based on CBR and inter-rater differences in assessment of CBR. We investigated inter-rater reliability of experienced Speech Language Therapists (SLTs) on: classification of CB status based on CBR ≥ 0.15, CBRs and the total number of syllables per infant used to calculate CBR.Each infant (n = 484) was video-recorded at a clinical site in play interaction with their parent as part of the randomised controlled trial Timing of Primary Surgery for Cleft Palate. Each recording was subsequently assessed by three independent SLTs, from a pool of 29 SLTs. They assessed the recordings in real time.The three assessing SLTs agreed in classification of CB status in 423 (87.4%) infants, with higher complete agreement for canonical (91%; 326/358) than non-canonical (77%; 97/126). The average difference in CBR and total number of syllables identified between the SLT assessments of each infant was 0.12 and 95, respectively.This study provided new evidence that one trained SLT can reliably classify CB status (CBR ≥ 0.15) in real time when there is clear distinction between the observed CBR and the boundary (0.15); however, when the observed CBR approaches the boundary multiple SLT assessments are beneficial. Thus, we recommend to include assessment of inter-rater reliability, if the purpose is to compare CBR and total syllable count across infants or studies.Trial registration number here: www.clinicaltrials.gov, identifier NCT00993551.

GWAS Identifies 44 Independent Associated Genomic Loci for Self-Reported Adult Hearing Difficulty in UK Biobank.

Age-related hearing impairment (ARHI) is the most common sensory impairment in the aging population; a third of individuals are affected by disabling hearing loss by the age of 65. It causes social isolation and depression and has recently been identified as a risk factor for dementia. The genetic risk factors and underlying pathology of ARHI are largely unknown, meaning that targets for new therapies remain elusive, yet heritability estimates range between 35% and 55%. We performed genome-wide association studies (GWASs) for two self-reported hearing phenotypes, using more than 250,000 UK Biobank (UKBB) volunteers aged between 40 and 69 years. Forty-four independent genome-wide significant loci (p < 5E-08) were identified, considerably increasing the number of established trait loci. Thirty-four loci are novel associations with hearing loss of any form, and only one of the ten known hearing loci has a previously reported association with an ARHI-related trait. Gene sets from these loci are enriched in auditory processes such as synaptic activities, nervous system processes, inner ear morphology, and cognition, while genetic correlation analysis revealed strong positive correlations with multiple personality and psychological traits for the first time. Immunohistochemistry for protein localization in adult mouse cochlea implicate metabolic, sensory, and neuronal functions for NID2, CLRN2, and ARHGEF28. These results provide insight into the genetic landscape underlying ARHI, opening up novel therapeutic targets for further investigation. In a wider context, our study also highlights the viability of using self-report phenotypes for genetic discovery in very large samples when deep phenotyping is unavailable.

Evaluation of the I-PLAN Intervention to Promote Hearing Aid Use in New Adult Users: a Randomized Controlled Trial.

OBJECTIVE: Provision of information is already part of standard care and may not be sufficient to promote hearing aid use. The I-PLAN is a behavior change intervention that is designed to promote hearing aid use in adults. It consists of a prompt, an action plan and provision of information. The objective was to test the effectiveness of the I-PLAN prompt and plan components in promoting hearing aid use and benefit. Hypotheses were: there would be greater hearing aid use, benefit, self-regulation, and hearing aid use habit among participants who received the prompt or plan component, compared with no prompt or no plan component, and the effect would be the greatest in participants who received both prompt and plan; and self-regulation and habit would mediate the effect of prompt and/or plan components on hearing aid use and benefit. DESIGN: A 2 x 2 factorial randomized controlled trial design. Two hundred forty new adult patients (60 in each group) were randomized to: information (info) only; info + prompt; info + plan; or info + prompt + plan. All participants received treatment as usual in addition to I-PLAN components, which were provided in a sealed envelope at the end of the hearing aid fitting consultation. Participants in the prompt group were instructed to use their hearing aid box as a physical prompt to remind them to use the device. Participants in the plan group were instructed to write an action plan to encourage them to turn their intentions into action. Participants, audiologists, and researchers were blinded to group allocation. The primary outcome was self-reported proportion of time hearing aids were used in situations where they had listening difficulties. Secondary outcomes were hearing aid use derived from data logging, self-reported hearing aid benefit, self-reported self-regulation, and habit. Outcomes were measured at 6-week post-fitting. RESULTS: Contrary to predictions, participants who received the prompt component reported using their hearing aid less than participants without the prompt (p = 0.03; d = 0.24). The mean proportion of time hearing aid were used was 73.4% of the time in the prompt group compared with 79.9% of the time in the no prompt group. Participants who received the plan component reported using their hearing aids more frequently than those who did not receive the plan (Meanplan = 81.0% vs Meannoplan = 71.8% of the time; p = 0.01; d = 0.34). Receiving both prompt and plan components did not change self-reported proportion of time hearing aids were used but data-logging use was significantly reduced. The prompt reduced self-regulation of hearing aid use compared with the no prompt (p = 0.04; d = 0.28), while the plan promoted stronger hearing aid use habits than the no plan group (p = 0.02; d = 0.30). CONCLUSIONS: Audiologists should consider using action plans to promote hearing aid use. Despite the decrease in hearing aid use when using the hearing aid box as a physical prompt, hearing aid use was still high (≈70% of the time). The hearing aid box may have slightly reduced hearing aid use by undermining self-regulation. Participants may have delegated responsibility for hearing aid use to the prompt. Subsequent studies should evaluate different prompts and test the long-term benefit of the plan on hearing aid use via habit formation.

Exploring the lived experiences of British Sign Language (BSL) users who access NHS adult hearing aid clinics: an interpretative phenomenological analysis.

OBJECTIVE: To explore the lived experiences of culturally Deaf British Sign Language (BSL) users who access adult hearing aid services. DESIGN: Semi-structured qualitative interviews were conducted in BSL by the Deaf researcher and analysed using an Interpretative Phenomenological Analysis (IPA) approach. STUDY SAMPLE: Eight Deaf BSL expert informants who were experienced users of NHS adult hearing aid clinics. RESULTS: Participants expressed dissatisfaction about audiology staff's lack of Deaf awareness and did not feel valued as Deaf signers. Participants' motivations for hearing aid use primarily concerned audibility rather than speech. Mismatch of perspectives on 'hearing' between audiologists and Deaf patients are discussed in the context of culturally sensitive services. Inadequate or uncertain linguistic access during appointments is considered in light of patient agency. CONCLUSION: This is the first study to explore culturally Deaf signers' specific experiences of adult hearing aid services in the UK and their experiences of hearing aids. There are numerous reasons why Deaf signers wear hearing aids, but access to spoken language is not a priority. Limited Deaf awareness and cultural competence in adult hearing aid services can result in patient frustration and disempowerment. Suggestions for improvement in the Deaf signing patient experience are offered.

Deaf signers and hearing aids: motivations, access, competency and service effectiveness.

OBJECTIVES: This study concerns culturally Deaf signers in the UK who use hearing aids and (i) explores motivations for hearing aid use (ii) identifies barriers and facilitators to accessing NHS hearing aid services, (iii) examines cultural competency of hearing aid clinics and (iv) identifies factors influencing effective adult hearing aid service provision. DESIGN: Online survey in British Sign Language and English that was informed by Deaf service users. STUDY SAMPLE: 75 Deaf adult BSL users who wear hearing aids and use NHS hearing aid clinics. RESULTS: No specific reason emerged as outstandingly important for hearing aid use; however, assisting with lipreading (57%) and listening to music (52%) were rated as very/extremely important. Access issues reported were contacting clinics, poor communication with staff and lack of Deaf awareness. To be an effective and culturally competent hearing aid clinic for Deaf signers, a good understanding of Deaf culture and language was most rated as important (87%). CONCLUSION: The study is the first that explores hearing aid use and experiences of accessing hearing aid clinics from Deaf signers' perspectives. Enhancements to clinical practice are required to consider culturally Deaf people's motivations for hearing aid use and make services more BSL-friendly.

Associations between pre-stimulus alpha power, hearing level and performance in a digits-in-noise task.

OBJECTIVE: Baseline electroencephalography (EEG) alpha power, i.e. that measured prior to stimulus presentation, is a potential objective predictor of task performance. Here we assessed the predictive power of EEG alpha on performance accuracy in a digits-in-noise recognition task, factoring in hearing thresholds and age. DESIGN: EEG alpha power, recorded while participants listened to target digits presented in a noise background, was analysed during two different baseline periods: i) a pre-stimulus baseline (pre-STIM) free from any acoustic stimulus, and ii) a pre-target baseline (pre-TARG) recorded in background noise only. STUDY SAMPLE: Eighty-five participants with either normal hearing or aided hearing impairment (age range: 55-85 years old, 42 male). RESULTS: Hierarchical multiple regression analyses indicated that i) lower hearing thresholds and, to a lesser extent, higher pre-STIM alpha power were associated with improved performance accuracy ii) alpha power in pre-STIM and pre-TARG were highly correlated across individuals but pre-TARG alpha power was not a significant predictor of performance accuracy. CONCLUSION: Investigations of baseline EEG alpha power as a predictor of speech-in-noise performance accuracy should control for associations between hearing thresholds and measures of EEG baseline periods.

Is COVID-19 associated with self-reported audio-vestibular symptoms?

OBJECTIVE: To determine if a positive test for COVID-19 is associated with self-reported audio-vestibular symptoms. DESIGN: Self-reported changes in hearing, tinnitus, hyperacusis, and dizziness/rotatory vertigo were assessed in hospitalised and non-hospitalised COVID-19 patients during and after the acute phase of the disease and compared to non-COVID controls. STUDY SAMPLE: There were 150 severe cases of COVID-19 requiring hospital admission and 150 mild cases that were managed at home. Controls were 267 adults, 32 of whom had been hospitalised for a non-COVID-19 condition, and a further 85 who worked in hospital settings. RESULTS: Deterioration in hearing and/or tinnitus was reported in 8% of the COVID-19 cases (tinnitus had resolved in 2% after the acute phase), with no significant difference between severe and mild cases. Deterioration in hearing or tinnitus was not significantly different from controls. However, rotatory vertigo was reported by 5% in the COVID-19 groups and 1.1% in the controls, and this difference was statistically significant. CONCLUSIONS: There is no evidence that COVID-19 results in deterioration in hearing or tinnitus during the acute phase or after recovery in mild or severe cases. However, rotatory vertigo, which could be vestibular in origin, may be a clinical manifestation of COVID-19.