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.

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.

How have services for diabetes, eye, hearing and foot health been integrated for adults? Protocol for a scoping review.

INTRODUCTION: The global population is ageing, and by 2050, there will be almost 2.1 billion people over the age of 60 years. This ageing population means conditions such as diabetes are on the increase, as well as other conditions associated with ageing (and/or diabetes), including those that cause vision impairment, hearing impairment or foot problems. The aim of this scoping review is to identify the extent of the literature describing integration of services for adults of two or more of diabetes, eye, hearing or foot services. METHODS AND ANALYSIS: The main database searches are of Medline and Embase, conducted by an information specialist, without language restrictions, for studies published from 1 January 2000 describing the integration of services for two or more of diabetes, eye, hearing and foot health in the private or public sector and at the primary or secondary level of care, primarily targeted to adults aged ≥40 years. A grey literature search will focus on websites of key organisations. Reference lists of all included articles will be reviewed to identify further studies. Screening and data extraction will be undertaken by two reviewers independently and any discrepancies will be resolved by discussion. We will use tables, maps and text to summarise the included studies and findings, including where studies were undertaken, which services tended to be integrated, in which sector and level of the health system, targeting which population groups and whether they were considered effective. ETHICS AND DISSEMINATION: As our review will be based on published data, ethical approval will not be sought. This review is part of a project in Aotearoa New Zealand that aims to improve access to services for adults with diabetes or eye, hearing or foot conditions. The findings will be published in a peer-reviewed journal and presented at relevant conferences.

Genetically modified pigs: Emerging animal models for hereditary hearing loss.

Hereditary hearing loss (HHL), a genetic disorder that impairs auditory function, significantly affects quality of life and incurs substantial economic losses for society. To investigate the underlying causes of HHL and evaluate therapeutic outcomes, appropriate animal models are necessary. Pigs have been extensively used as valuable large animal models in biomedical research. In this review, we highlight the advantages of pig models in terms of ear anatomy, inner ear morphology, and electrophysiological characteristics, as well as recent advancements in the development of distinct genetically modified porcine models of hearing loss. Additionally, we discuss the prospects, challenges, and recommendations regarding the use pig models in HHL research. Overall, this review provides insights and perspectives for future studies on HHL using porcine models.

Towards universal access: A review of global efforts in ear and hearing care.

Hearing loss affects 1.6 billion people worldwide and disproportionately affects those in low- and middle-income countries. Despite being largely preventable or treatable, ear and hearing conditions result in significant and lifelong morbidity such as delayed language development, reduced educational attainment, and diminished social well-being. There is a need to augment prevention, early identification, treatment, and rehabilitation for these conditions. Expanded access to hearing screening, growth of the hearing health workforce, and innovations in ear and hearing care delivery systems are among the changes that are needed. To that end, the World Health Organization has prioritized ear and hearing care as a component of Universal Health Coverage, and recent publications have advanced the priority for ear and hearing care. Efforts are underway at the national levels around the world, as evidenced by countries like Zambia and Nigeria that have integrated ear and hearing care within national health strategies. While significant strides have been made in improving access, a critical need remains for additional research, advocacy, and intervention to ensure that no one is left behind in the goal to achieve universal access to ear and hearing care.

Education Intervention on 'Hearing Impairment - Its Assessment and Management' among Medical Practitioners in Sikkim, India.

BACKGROUND: Health care providers must be aware of the choices in aural rehabilitation methods and assessment procedures available presently to those with hearing impairment in order to facilitate early identification and intervention and ensure quality of care to those hearing impaired individuals. The referrals from the medical fraternities for the audiological services are not uniform across India. Part of reason may be that awareness about the roles of and responsibilities of audiologist's is poor among medical practitioners. In some regions of India medical practitioners may be only grossly be aware of audiologists per se. They may not know enough to refer all individuals with hearing or balance disorders for the required audiological services. AIM: To evaluate changes in pre survey and post survey after education intervention specifically planned for medical practitioners in the state of Sikkim, India. Education intervention included awareness about hearing impairment and its assessment and management by audiologists. METHOD: A pre and post survey comparison research design with purposive convenient sampling technique was applied. Participants were medical practitioners working in geographical area of Sikkim at the time of the study. Inspection of demographic data of respondents showed that they were in their age range of 24 and 60 years and with work experience ranging 1-40 years. Participants were invited for an awareness talk which included, information about hearing loss, early identification and early intervention, tests and management of hearing loss in different age groups, need for hearing aids/cochlear implants, auditory training, and the role of an audiologist in the management of hearing loss and ways to prevent hearing loss. A pre and post awareness program responses were collected from all the participants using a customized questionnaire tool. RESULTS: Respondents exhibited, in the pre-survey questionnaire, lack of awareness in many aspects of audiology, including assessing hearing impairment, diagnosing hearing loss, and the role of an audiologist. There was an overall, statistically significant difference in the level of performance on the pre-awareness and post-awareness responses. CONCLUSION: Following an awareness campaign among the medical professionals in Sikkim, there was a considerable change in their level of awareness of hearing impairment, its assessment, and management by audiologists. The reach of the awareness campaign was significant as around 70% of subjects showed significant change in their knowledge and attitude towards hearing impairment, its management.

Outcomes and device use in children with bone-conduction hearing devices in South Africa.

BACKGROUND:  Bone-conduction hearing devices (BCHD) can provide hearing solutions in settings where middle ear pathology is rife. OBJECTIVES:  Describe functional hearing outcomes and device use of children fitted with BCHD. METHOD:  Retrospective review of 79 children fitted with BCHD between January 2017 and May 2022. Outcomes included device use and subjective reports measured with the Parents' Evaluation of Aural/Oral Performance of Children (PEACH) and the Teachers' Evaluation of Aural/Oral Performance of Children (TEACH). Analysis of variance established association between mean data logging and type and degree of hearing loss. Thematic analyses were done for qualitative outcomes. RESULTS:  Average usage was 7.0 h/day (5.4 SD; range 0.1-24). PEACH ratings indicated 93.3% of children wore their BCHD 'always' or 'often', with 80% displaying Typical auditory performance at 1-month follow-up. TEACH ratings indicated 84.2% of children wore their BCHD 'always' or 'often', with 78.9% showing typical auditory behaviour. Increased usage was noted for conductive, mixed, moderate and severe hearing losses. There was a mean delay of 17.2 months (23.4 SD; range 0-90) between age of diagnosis and fitting. Thematic analyses identified two main themes: advantages and barriers to BCDH use. CONCLUSION:  Average device use fell short of the internationally recommended 10 h/day. Higher BCHD use was associated with higher functional listening performance scores. Long waiting times for medical or surgical intervention for conductive hearing losses can delay BCHD fitting.Contribution: Limited information is available to examine outcomes in children fitted with BCHD.

Hearing Loss Detection and Early Intervention Strategies in Kenya.

Background: Thirty-four million children globally have disabling hearing loss, with the highest prevalence in low- and middle-income countries (LMICs). Early identification and management is crucial, yet barriers to screening and treatment of hearing loss are extensive in LMICs. Unaddressed hearing loss negatively impacts individuals and communities. The WHO's 2021 World Report on Hearing urges the development of Ear and Hearing Care (EHC) programs to improve access to all aspects of care, including screening, diagnostics, management, and developmental support. A joint Nairobi- and Seattle-based group convened in 2021 to discuss strategies for program development in Kenya, as presented in this paper. Findings: Developing a national EHC program must include the necessary support services for a child with a diagnosed hearing loss, while simultaneously promoting engagement of family, community, and healthcare workers. Existing government and healthcare system policies and priorities can be leveraged for EHC programming. Strategies for success include strengthening connections between policymakers at national, county, and municipal levels and local champions for the EHC agenda, with a concurrent focus on policy, early detection and intervention, habilitation, and family-centered care. Updates to health policy and funding to support the accessibility of services and equipment should focus on leveraging national healthcare coverage for hearing technologies and services, strengthening referral pathways, training to bolster the workforce, and metrics for monitoring and evaluation. Additional strategies to support forward progress include strategic engagement of partners and leveraging local partners for phased scale-up. Conclusions and Recommendations: Recommendations to strengthen EHC within the Kenyan health system include concurrent leverage of existing health policies and priorities, partner engagement, and strengthening referral pathways, monitoring and evaluation, and training. These strategies may be generalized to other countries too.

Prevalence and predictors of self-reported hearing aid use and benefit in Norway: the HUNT study.

BACKGROUND: Knowledge on hearing aid use and benefit is important to ensure appropriate and effective treatment. We aimed to assess prevalence and predictors of hearing aid use and benefit in Norway, as well as possible birth cohort changes. METHODS: We analyzed two large cross-sectional, population-based hearing surveys of 63,182 adults in 1996-1998 and 2017-2019 (the HUNT study). We used multivariable regression models to examine independent predictors of hearing aid use and benefit, including demography, hearing-related variables, known risk factors for hearing loss and birth cohort. RESULTS: The nationally weighted hearing aid use in the adult population increased from 4.2% in 1997 to 5.8% in 2018. The use among individuals with disabling hearing loss (≥ 35 dB HL) increased from 46.3% to 64.4%. Most users reported some (47%) or great (48%) help from their hearing aids. In addition to the level of hearing loss and birth cohort, factors associated with hearing aid use included lower age, tinnitus, childhood-onset hearing loss, higher education, marriage, having children, being exposed to occupational noise or impulse noise, recurrent ear infections, and head injury. In addition to the level of hearing loss, factors related to hearing aid benefit included younger age, female gender, and higher income. Being bothered by tinnitus reduced the benefit. CONCLUSION: Our study shows an increase in self-reported hearing aid usage over time in Norway, with lower adoption rates and perceived benefits observed among the elderly. The results suggest that having a spouse and children positively influences the adoption of hearing aids. These findings emphasize the necessity of customized strategies to address demographic disparities and the need for innovative enhancements in hearing rehabilitation programs.

[Genetic counseling for hearing loss today].

Genetic counseling for hearing loss today originated from decoding the genetic code of hereditary hearing loss, which serves as an effective strategy for preventing hearing loss and constitutes a crucial component of the diagnostic and therapeutic framework. This paper described the main principles and contents of genetic counseling for hearing loss, the key points of counseling across various genetic models and its application in tertiary prevention strategies targeting hearing impairment. The prospects of an AI-assisted genetic counseling decision system and the envisions of genetic counseling in preventing hereditary hearing loss were introduced. Genetic counseling for hearing loss today embodies the hallmark of a new era, which is inseparable from the advancements in science and technology, and will undoubtedly contribute to precise gene intervention!

[Study on gene therapy for DPOAE and ABR threshold changes in adult Otof-/- mice].

Objective:This study aims to analyze the threshold changes in distortion product otoacoustic emissions（DPOAE） and auditory brainstem response（ABR） in adult Otof-/- mice before and after gene therapy, evaluating its effectiveness and exploring methods for assessing hearing recovery post-treatment. Methods:At the age of 4 weeks, adult Otof-/- mice received an inner ear injection of a therapeutic agent containing intein-mediated recombination of the OTOF gene, delivered via dual AAV vectors through the round window membrane（RWM）. Immunofluorescence staining assessed the proportion of inner ear hair cells with restored otoferlin expression and the number of synapses.Statistical analysis was performed to compare the DPOAE and ABR thresholds before and after the treatment. Results:AAV-PHP. eB demonstrates high transduction efficiency in inner ear hair cells. The therapeutic regimen corrected hearing loss in adult Otof-/- mice without impacting auditory function in wild-type mice. The changes in DPOAE and ABR thresholds after gene therapy are significantly correlated at 16 kHz. Post-treatment,a slight increase in DPOAE was observeds,followed by a recovery trend at 2 months post-treatment. Conclusion:Gene therapy significantly restored hearing in adult Otof-/- mice, though the surgical delivery may cause transient hearing damage. Precise and gentle surgical techniques are essential to maximize gene therapy's efficacy.

Extracellular vesicles for developing targeted hearing loss therapy.

Substantial efforts have been made for local administration of small molecules or biologics in treating hearing loss diseases caused by either trauma, genetic mutations, or drug ototoxicity. Recently, extracellular vesicles (EVs) naturally secreted from cells have drawn increasing attention on attenuating hearing impairment from both preclinical studies and clinical studies. Highly emerging field utilizing diverse bioengineering technologies for developing EVs as the bioderived therapeutic materials, along with artificial intelligence (AI)-based targeting toolkits, shed the light on the unique properties of EVs specific to inner ear delivery. This review will illuminate such exciting research field from fundamentals of hearing protective functions of EVs to biotechnology advancement and potential clinical translation of functionalized EVs. Specifically, the advancements in assessing targeting ligands using AI algorithms are systematically discussed. The overall translational potential of EVs is reviewed in the context of auditory sensing system for developing next generation gene therapy.

Improving quality of life in the elderly: hearing loss treatment with cochlear implants.

BACKGROUND: Hearing loss impacts health-related quality of life and general well-being and was identified in a Lancet report as one of the largest potentially modifiable factors for the prevention of age-related dementia. There is a lack of robust data on how cochlear implant treatment in the elderly impacts quality of life. The primary objective was to measure the change in health utility following cochlear implantation in individuals aged ≥ 60 years. METHODS: This study uniquely prospectively recruited a large multinational sample of 100 older adults (mean age 71.7 (SD7.6) range 60-91 years) with severe to profound hearing loss. In a repeated-measures design, pre and post implant outcome measures were analysed using mixed-effect models. Health utility was assessed with the Health Utilities Index Mark III (HUI3). Subjects were divided into groups of 60-64, 65-74 and 75 + years. RESULTS: At 18 months post implant, the mean HUI3 score improved by 0.13 (95%CI: 0.07-0.18 p < 0.001). There was no statistically significant difference in the HUI3 between age groups (F[2,9228] = 0.53, p = 0.59). The De Jong Loneliness scale reduced by an average of 0.61 (95%CI: 0.25-0.97 p < 0.014) and the Lawton Instrumental Activities of Daily Living Scale improved on average (1.25, 95%CI: 0.85-1.65 p < 0.001). Hearing Handicap Inventory for the Elderly Screening reduced by an average of 8.7 (95%CI: 6.7-10.8, p < 0.001) from a significant to mild-moderate hearing handicap. Age was not a statistically significant factor for any of the other measures (p > 0.20). At baseline 90% of participants had no or mild depression and there was no change in mean depression scores after implant. Categories of Auditory perception scale showed that all subjects achieved a level of speech sound discrimination without lip reading post implantation (level 4) and at least 50% could use the telephone with a known speaker. CONCLUSIONS: Better hearing improved individuals' quality of life, ability to communicate verbally and their ability to function independently. They felt less lonely and less handicapped by their hearing loss. Benefits were independent of age group. Cochlear implants should be considered as a routine treatment option for those over 60 years with bilateral severe to profound hearing loss. TRIAL REGISTRATION: ClinicalTrials.gov ( http://www. CLINICALTRIALS: gov/ ), 7 March 2017, NCT03072862.

Clarin-2 gene supplementation durably preserves hearing in a model of progressive hearing loss.

Hearing loss is a major health concern affecting millions of people worldwide with currently limited treatment options. In clarin-2-deficient Clrn2-/- mice, used here as a model of progressive hearing loss, we report synaptic auditory abnormalities in addition to the previously demonstrated defects of hair bundle structure and mechanoelectrical transduction. We sought an in-depth evaluation of viral-mediated gene delivery as a therapy for these hearing-impaired mice. Supplementation with either the murine Clrn2 or human CLRN2 genes preserved normal hearing in treated Clrn2-/- mice. Conversely, mutated forms of CLRN2, identified in patients with post-lingual moderate to severe hearing loss, failed to prevent hearing loss. The ectopic expression of clarin-2 successfully prevented the loss of stereocilia, maintained normal mechanoelectrical transduction, preserved inner hair cell synaptic function, and ensured near-normal hearing thresholds over time. Maximal hearing preservation was observed when Clrn2 was delivered prior to the loss of transducing stereocilia. Our findings demonstrate that gene therapy is effective for the treatment of post-lingual hearing impairment and age-related deafness associated with CLRN2 patient mutations.

Hearing Loss, Hearing Aids, and Satisfaction With Health Care in the National Health Interview Survey.

OBJECTIVE: Hearing loss may negatively impact satisfaction with health care via patient-provider communication barriers and may be amenable to hearing care treatment. STUDY DESIGN: Cross-sectional. SETTING: National Health Interview Survey, a nationally representative survey of noninstitutionalized US residents, 2013 to 2018 pooled cycles. METHODS: Participants described satisfaction with health care in the past year, categorized as optimal (very satisfied) versus suboptimal (satisfied, dissatisfied, very dissatisfied) satisfaction. Self-report hearing without hearing aids (excellent, good, a little trouble, moderate trouble, a lot of trouble) and hearing aid use (yes, no) were collected. Weighted Poisson regression models adjusted for sociodemographic and health covariates were used to estimate prevalence rate ratios (PRRs) of satisfaction with care by hearing loss and hearing aid use. RESULTS: Among 137,216 participants (mean age 50.9 years, 56% female, 12% black), representing 77.2 million Americans in the weighted model, 19% reported trouble hearing. Those with good (PRR = 1.20, 95% confidence interval [CI]: 1.18-1.23), a little trouble (PRR = 1.27, 95% CI, 1.23-1.31), moderate trouble (PRR = 1.29, 95% CI, 1.24-1.35), and a lot of trouble hearing (PRR = 1.26, 95% CI, 1.18-1.33) had a higher prevalence rate of suboptimal satisfaction with care relative to those with excellent hearing. Among all participants with trouble hearing, hearing aid users had a 17% decrease in the prevalence rate of suboptimal satisfaction with care (PRR = 0.83, 95% CI, 0.78-0.88) compared to nonusers. CONCLUSION: Hearing loss decreases patient satisfaction with health care, which is tied to Medicare hospital reimbursement models. Hearing aid use may improve patient-provider communication and patient satisfaction, although prospective studies are warranted to truly establish their protective effect.

Critical role of TPRN rings in the stereocilia for hearing.

Inner ear hair cells detect sound vibration through the deflection of mechanosensory stereocilia. Cytoplasmic protein TPRN has been shown to localize at the taper region of the stereocilia, and mutations in TPRN cause hereditary hearing loss through an unknown mechanism. Here, using biochemistry and dual stimulated emission depletion microscopy imaging, we show that the TPRN, together with its binding proteins CLIC5 and PTPRQ, forms concentric rings in the taper region of stereocilia. The disruption of TPRN rings, triggered by the competitive inhibition of the interaction of TPRN and CLIC5 or exogenous TPRN overexpression, leads to stereocilia degeneration and severe hearing loss. Most importantly, restoration of the TPRN rings can rescue the damaged auditory function of Tprn knockout mice by exogenously expressing TPRN at an appropriate level in HCs via promoter recombinant adeno-associated virus (AAV). In summary, our results reveal highly structured TPRN rings near the taper region of stereocilia that are crucial for stereocilia function and hearing. Also, TPRN ring restoration in stereocilia by AAV-Tprn effectively repairs damaged hearing, which lays the foundation for the clinical application of AAV-mediated gene therapy in patients with TPRN mutation.

Stem Cell-Based Therapies for Auditory Hair Cell Regeneration in the Treatment of Hearing Loss.

The incidence and prevalence of hearing loss is increasing globally at an accelerated pace. Hair cells represent the sensory receptors of auditory and vestibular systems. Hair cell absence, loss or degeneration due to congenital diseases, trauma, toxicity, infection or advancing age, results in disabling hearing loss. Regenerative medicine approaches consisting in stem cell-based hair cell rescue or regeneration, gene therapy, as well as cell and tissue engineering are expected to dramatically improve the therapeutic arsenal available for addressing hearing loss. Current strategies that are using different stem cell types to rescue or to induce hair cell proliferation and regeneration are presented. Gene and cell therapy methods that modulates transdifferentiation of surrounding cell types into hair cells are presented, together with their specific advantages and limitations. Several modalities for improving therapeutic targeting to the inner ear such as nanoparticle-mediated cell and gene delivery are introduced. Further steps in building more relevant high-throughput models for testing novel drugs and advanced therapies are proposed as a modality to accelerate translation to clinical settings.

Hearing Loss and Sociodemographic Barriers to Health Care Access Using the All of Us Research Program.

OBJECTIVE: To explore how gender and low-income status independently influence general health care access in patients with hearing loss. STUDY DESIGN: Cross-sectional study. SETTING: National database. METHODS: Patients with a diagnosis of sensorineural hearing loss from the National Institutes of Health All of Us database were included. Data entered from May 2018 to November 2022 was analyzed. Patient demographics such as age, gender, educational level, and insurance status were assessed. Multivariate logistic regressions were performed for statistical evaluation. RESULTS: A subset of 8875 patients (48.3% male, mean age 69) were evaluated. After multivariate analysis, female participants were more likely than male participants to report difficulty affording prescribed medications (odds ratio [OR]: 1.7, p < .0005) and specialists (OR: 1.4, p < 0.005). Female patients were also more likely to delay care due to elder care responsibilities (OR: 2.6, p < .0005), employment obligations (OR: 1.7, p < .0005), and feelings of apprehension in seeing a provider (OR: 1.7, p < .0005). Finally, female participants reported feeling less likely to be involved in their own medical care compared to males (OR: 1.2, p < .005). Low-income (<$25,000) participants reported less likely to feel respected (OR: 3.2, p < .0005) and delivered understandable health information (OR: 2.3, p < .0005) by providers compared to participants of higher income. CONCLUSION: This work suggests that patients with hearing loss, female gender, and lower socioeconomic status independently introduce barriers to health care access and utilization. These factors should be considered in efforts to promote equity in the care of patients with hearing loss.

Genetics evaluation outcomes of patients with pediatric hearing loss: 2008-2022 retrospective study.

OBJECTIVE: This study aims to explore how genetics evaluation and testing for patients with pediatric onset hearing loss affects their diagnosis and management. METHODS: Retrospective chart review was completed for patients with pediatric hearing loss that were evaluated by a genetic counselor from 2008 to 2022 with data entry into a REDCap database. Descriptive statistical analysis was completed. RESULTS: Four hundred twenty-nine patients with pediatric onset hearing loss were evaluated by genetics. Majority of patients presented with bilateral (67 %) and sensorineural (83 %) hearing loss. Genetic testing was recommended for 76 % of patients with pediatric hearing loss evaluated by a genetic counselor with 70 % completing some or all recommended tests. Overall genetic testing diagnostic rate was 34 %, with 41 % of diagnoses syndromic. Yearly trends noted an increasing number of patients evaluated, tests ordered, and subsequently an increased number of diagnoses overtime. For diagnostic results, management recommendations were made for 45 % of patients (35/78) and for 92 % of family members (72/78). This compared to total management recommendations for all patients (82/429, 19 %) and family members (110/429, 26 %). CONCLUSION: This study identified a genetic testing diagnostic rate for pediatric hearing loss of 34 % over 14 years. This study notes the beneficial outcomes of patients with hearing loss and their families meeting with a genetic counselor and the importance of collaboration with hearing loss management colleagues. It highlights the value a genetic counselor consult can add to a patient's diagnostic journey, in addition to how genetic testing impacts management for patients and their families.

Hospitalizations, Emergency Department Visits, and Home Health Use Among Older Adults With Sensory Loss.

Objectives: This study examines healthcare resource use (hospitalizations, emergency department [ED] visits, and home health episodes) among adults 65 and older diagnosed with hearing, vision, or dual sensory loss (SL) seen in the primary care setting of an academic health system. Methods: Multivariable logistic regression models were used to examine the relationship between SL (identified using ICD-10 codes) and healthcare resource use for 45,000 primary care patients. Results: The sample included 5.5% (N = 2479) with hearing loss, 10.4% (N = 4697) with vision loss, and 1.0% with dual SL (N = 469). Hearing loss increased the likelihood of having an ED visit (OR = 1.22, CI: 1.07-1.39), and home health services (OR = 1.27, CI: 1.07-1.51) compared to older adults without any SL. Vision loss reduced the likelihood of having a hospitalization (OR = .81, CI: .73-.91). Discussion: Findings support research into the drivers of healthcare use among older adults with sensory loss.