Diagnosis Of Hearing Loss Research Articles

Hearing loss in patients with Morquio A syndrome: A scoping review.

Hearing impairment is a prevalent clinical feature in Morquio syndrome (mucopolysaccharidosis IVA or MPS IVA) patients, often presenting in diverse forms: conductive, sensorineural, or a combination known as mixed hearing loss. The mixed form entails a blend of both conductive and sensorineural elements, typically exhibiting a progressive trajectory. This scoping review aimed to comprehensively analyze available evidence pertaining to the pathophysiology, classification, epidemiology, and clinical management of hearing loss in individuals with MPS IVA. Targeted literature was searched using MEDLINE, Literatura Latino-Americana e do Caribe em Ciências da Saúde, Web of Science, the Cochrane Library, Trip Medical Database, Embase, ScienceDirect, and Google Scholar, with a second search cycle to identify gray literature. A systematic search strategy using Medical Subject Headings keywords was implemented: "Hearing Disorders" OR "Hearing Loss" AND "Mucopolysaccharidosis IV" or "Hearing Disorders" OR "Hearing Loss" AND "Mucopolysaccharidosis IV." The identified bibliography was uploaded to COVIDENCE platform for information management. Articles were screened by 3 independent reviewers following the eligibility criteria. Twenty-seven articles met the inclusion criteria, spanning information from 568 patients across 16 different countries. None of the studies had complete epidemiological information. Only 2 studies provided sufficient data to address the pathophysiology, while 3 addressed management and treatment. Hearing loss was reported in 210 of 568 patients. A total of 19.2% of patients reported recurrent ear infections. None of the studies reported vertigo, tinnitus, or dizziness in the patients. Pure-tone audiometry was the primary test used to diagnose and monitor auditory impairment in patients with Morquio syndrome. Five hundred sixty-eight patients with MPS IVA were identified, of whom 210 (37%) developed hearing loss, the most common of which was moderate. Despite the lack of information on the diagnosis and management of hearing loss in Morquio syndrome, this study found that approximately one-third of participants exhibited some form of auditory impairment, with the majority of these cases being sensorineural in nature.

COMMUNITY PREVALENCE OF HEARING LOSS AMONG OLDER ADULTA IN MA, RI, CT, AND NH: COMPARING MEASURES

Abstract Background Hearing impairment and hearing loss may negatively impact social engagement and social relationships. Understanding community rates of hearing loss would identify disparities and may inform service delivery and potentially improve quality of life for adults with hearing loss. The aim of this study is to describe community prevalence of hearing loss and to compare two measurement approaches: self-report and clinically derived from Medicare claims. Methods Data were from the Healthy Aging Data Report (healthyagingdatareports.org) for the New England states (MA, NH, CT, and RI) with indicators calculated from the American Community Surveys (MA: 2012-2016; RI:2016-2018; CT: 2014-2018 and NH: 2012-2016), and the Medicare Master Beneficiary Summary Files (MA: 2014-2015; RI: 2016-2017; CT: 2016-2017 and NH: 2014-2015). Results rates derived from CMS clinical diagnosis were higher (MA: 16.1%; RI: 16.4%; CT: 15.7%, and NH: 14.4%) compared to the self-reported ACS rates (MA: 14.2%; RI: 13.9%: CT: 12.4% and NH: 15.0%). The highest community prevalence rate of self-reported hearing loss (36.36%) was in Monroe MA. For clinically diagnosed hearing loss, Hanover NH (home to Dartmouth College and health system) reported the highest rate (26.12%). Having excellent access to health care is associated with higher community rates of clinically diagnosed hearing loss. Results showed that the community prevalence of hearing impairment was variable by community. The potential impact of hearing loss on social, emotional, and physical well-being indicates a need for interventions to diagnose and treat it.

Guidelines for Diagnosis of Noise-Induced Hearing Loss and Their Specificity.

A recent paper by Moore, Lowe and Cox has proposed guidelines for diagnosing noise-induced hearing loss (NIHL). It is referred to here as the MLC guidelines. Our aim was to assess the specificity of those guidelines (i.e., freedom from false-positive outcomes) and compare with pre-existing guidelines. We applied the MLC guidelines and pre-existing guidelines to three data sets composed of adults who do not have a history of material noise exposure and therefore cannot have NIHL. National Health Service (NHS) ENT clinic. Five hundred thirty-six patients with hearing difficulty and/or tinnitus who denied material noise exposure. Two large archival population studies of hearing were also assessed, which included 3250 participants without material noise exposure. False-positive outcome from guidelines. The MLC guidelines demonstrated moderate or high false-positive rates overall, the magnitude depending on the noise exposure scenario and whether clinical or population samples were considered. For the procedure applicable to steady broadband noise exposure, the false-positive rate averaged 56% in the population samples, compared to 31% for previous guidelines. For exposure to intense impulse sounds, the MLC guidelines take a different approach and the false-positive rate was about 70% in the population samples and even higher in the clinic sample. For exposure to intense tones, the MLC guidelines take yet another approach and the false-positive rate reached 80%. The MLC guidelines demonstrate poorer specificity than previous guidelines. Medical experts should be aware of their poor specificity and consequential likelihood of false-positive diagnoses of NIHL.

Pituitary Gland Macroadenoma with Associated Hearing Loss

Pituitary macroadenomas are benign neoplasms that can cause a range of symptoms due to a mass effect on surrounding structures. This case report describes a 51-year-old male presenting with progressive bilateral hearing impairment and significant deterioration in speech discrimination over several years. Examination revealed normal tympanic membranes and no neurological deficits, while pure tone audiometry indicated mild to moderate sensorineural hearing loss. Magnetic resonance imaging identified a 6 × 5.5 × 7 cm pituitary macroadenoma extending into the suprasellar region and invading the sphenoid and right cavernous sinuses. The patient underwent a two-stage surgical intervention followed by gamma knife surgery. Postoperative follow-up showed mild deterioration in hearing with unmeasurable speech discrimination scores. This case underscores the importance of considering pituitary macroadenomas in the differential diagnosis of unexplained hearing loss and highlights the role of comprehensive audiological and imaging evaluations in achieving accurate diagnosis and optimal management.

Exploring the Role of Early Diagnosis of Hearing Loss in Children and Its Impact on Educational Outcomes, Language Development, and Social Integration in Special Education Settings

The study explores the impact of early diagnosis of hearing loss on children's educational outcomes, language development, and social integration in special education settings. The research involved 150 children with early hearing loss, comparing the differences between early diagnosis (0-2 years) and late diagnosis (more than 2 years after birth). A quantitative research design was employed, using standardized test scores, teacher assessments, speech development tests, and social interaction surveys for data collection. The findings show that early diagnosis positively influences academic performance, language development, and peer interactions. Children diagnosed early demonstrated better academic outcomes (p < 0.05), more advanced language skills (p < 0.05), and improved social interactions (p < 0.05) compared to those diagnosed later. A strong positive correlation was found between early diagnosis and the effectiveness of support services (r = 0.65, p < 0.05). These results emphasize the importance of early intervention and robust support systems for children with hearing loss, highlighting the need for expanded newborn hearing screening programs and improvements in educational practices. Further research is recommended on long-term outcomes, the role of assistive technologies, and the influence of family involvement on improving outcomes following early diagnosis.

A hypothetical intervention on the use of hearing aids for the risk of dementia in people with hearing loss in UK Biobank.

Observational studies have reported that hearing aid (HA) use is associated with a reduced risk of dementia diagnosis, suggesting a possible protective effect. However, extant observational studies do not explicitly model causal effects, while randomised controlled trials on the effect of HA on dementia exhibit short follow-up. Here we used self-report, hearing tests, and healthcare records in UK Biobank to design a hypothetical intervention for the effect of HA use on the risk of dementia diagnosis in people with incident hearing loss (HL). HA users exhibited a higher risk of dementia diagnosis than non-users (RR=1.43, 95%CI=1.08-1.88). Associations between HA use and dementia diagnosis were robust across sensitivity analyses (RRs: 1.34-1.59) but adjustment for primary healthcare utilisation (0.77, 0.44-1.33) or primary and secondary care utilisation (0.68, 0.39-1.18) substantially decreased the observed effect. The decrease in effect estimates upon adjustment for primary (1.30, 0.95-1.78) and primary and secondary healthcare utilisation (1.30, 0.94-1.78) was smaller when participants with relatively early diagnoses of HL were included in the sample compared to when they were not. While the findings are not conclusive, they suggest residual confounding by healthcare utilisation and dating of HL diagnosis in participants without primary care data in UK Biobank.

Syndromic Association of Depigmentation With Congenital Hearing Loss: A Review of Three Children With Auditory Pigmentary Disorders.

Background: Congenital depigmentation may be associated with congenital sensorineural hearing loss leading to non-development of verbal speech. Objective: To illustrate the clinical features and work-up of 3 children diagnosed with auditory pigmentary disorders (APDs). Methodology: Case series with a review of the literature. Results: The APDs presented here in the 3 children include Waardenburg syndrome type 1, Clouston syndrome, and Waardenburg syndrome type 4 (Waardenburg-Shah syndrome). The characteristic clinical features, audiologic tests, imaging, and the necessary genetic tests carried out subsequently were noted and evaluated. All the children were male and were aged 2 years, 1 year, and 14 months, respectively. All of them had hearing loss and non-development of verbal speech and had some form of oculocutaneous depigmentation. The challenges in the diagnosis, the work-up, and the close differentials were discussed, and the relevant literature was reviewed. Conclusions: The APDs connect congenital depigmentation with prelingual hearing loss through various syndromic disorders. These disorders are not commonly encountered in routine clinical practice; therefore, their proper knowledge is essential for early diagnosis of congenital hearing loss and timely initiation of auditory and speech rehabilitation. This case series deals with a detailed illustration of a few syndromes of the APDs and highlights their clinical presentation and genetic background.

Association of Hearing Loss Onset and Etiology with Psychosocial Outcomes Among US Older Adults.

Understand the differential association of onset and etiology of hearing loss with psychosocial outcomes among older adults. This is a cross-sectional cohort study based on 2017-2020 National Health and Nutrition Examination Survey. Adults aged ≥70 years who completed audiometric testing and questionnaires on onset and etiologies of hearing loss were included (n = 612). Outcomes included report of social avoidance and major depressive disorder (MDD, PHQ-9). Multivariable regression was utilized to examine differential associations of onset and etiology of hearing loss on psychosocial outcomes while adjusting for relevant factors. In this nationally representative sample of US older adults with hearing loss, 20.8% [95%CI: 14.5-29.0%] reported social avoidance due to hearing loss and 7.2% [95%CI: 4.3-11.8] reported symptoms suggestive of MDD. Multivariable regression demonstrated self-reported onset of hearing loss between ages 6-19 years (OR:2.49 [95%CI: 1.52-4.10]) and 20-59 years (OR:1.95 [95%CI: 1.07-3.55]) was associated with higher avoidance of social interaction than reported onset at ≥70 years. Those with onset between 20-59 years (OR:4.28 [95%CI: 1.17-15.6]) and 60-69 years (OR:5.68 [95%CI: 1.85-17.5]) were more likely to report symptoms consistent with MDD than those with onset at ≥70. Multivariable regression did not demonstrate increased risk of avoiding social interactions or MDD due to hearing loss from noise exposure, ear infection, or ear disease versus aging alone. There was an association between earlier hearing loss onset with social avoidance and MDD. This suggests a need for research to investigate the impact of earlier diagnosis and intervention for hearing loss on psychosocial outcomes. 2b Laryngoscope, 134:5073-5079, 2024.

Disparities among Different Countries in Access for Diagnosis and Treatment of Pediatric Sensorineural Hearing Loss.

Timely diagnosis and management of pediatric sensorineural hearing loss (SNHL) is essential for best outcomes. This study evaluated the differences of health access among cochlear implant (CI) centers of countries with different health care systems. Retrospective. Multicenter, tertiary pediatric CI referral center in four different countries: United States (USA), France (FR), Australia (AUS), and Brazil (BR). Pediatric (age 0-21 years (0-16 years AUS)) patients receiving CI between January 2017 and December 2019. CI. Primary outcome measures included age at milestones in CI process and age at implantation. There were 1,673 patients who underwent CI surgery at four multinational designated institutions were identified (n = 143 AUS; 153 BRA; 1,158 FR; and 219 USA). Median age at time of hearing loss diagnosis ( p < 0.0001), median age at hearing aid (HA) fitting ( p < 0.0001), and CI evaluation ( p < 0.0001) differed between countries. However, age at CI surgery was not significantly different between countries. Time from diagnosis to HA fitting was impacted by country ( p < 0.0001) and language ( p = 0.01) but not by private compared with public insurance. There were 512 children with available data regarding onset of hearing loss as prelingual n = 358 (70%) and postlingual n = 154 (30%). In patients with prelingual severe to profound hearing loss, country and race/ethnic origin affected age at diagnosis ( p < 0.0001, p = 0.001), HA fitting ( p < 0.0001, p < 0.001), candidacy evaluation ( p = 0.0001, p = 0.02), and CI surgery ( p = 0.0027, p = 0.001). Age at pediatric CI was comparable across the CI centers compared in the four countries. This is encouraging given the effect of neuroplasticity on pediatric CI outcomes, despite different health care expenditures and delivery systems in the four countries. Improvement in care access for certain populations may decrease disparities in pediatric CI access worldwide.

Effectiveness of auditory measures in the diagnosis of cochlear synaptopathy and noise-induced hidden hearing loss: a case–control study

BackgroundCochlear synaptopathy is a disorder where auditory perceptual impairments, such as speech perception in noise and tinnitus, may not be accurately reflected by audiometric thresholds, even if the audiogram appears normal. It is proposed that the connections between hair cells and the auditory nerve are more susceptible to sound and aging damage than the cochlea’s hair cells. Cochlear synaptopathy can be present in ears with normal audiograms and undamaged hair cells, leading to hidden hearing loss. This study aims to construct electrophysiological and behavioral auditory parameters associated with persons exposed to loudness and having normal audiograms and auditory complaints to identify hidden hearing loss.ResultsA case–control study was done with forty participants with a documented history of exposure to loud sounds and various auditory complaints, compared to a control group of forty persons who had all been confirmed to have normal audiograms. The chosen behavioral core tests comprised the speech intelligibility in noise test (SPIN), gaps detection in noise (GIN test), and pitch pattern sequence test (PPS). The electrophysiological measures utilized in the study were the auditory brainstem response test (ABR) and electrocochleography (ECochG). The SPIN, PPS, and GIN test results demonstrated statistically significant disparities between the control and case groups. The amplitude ratio of wave I to wave V in ABR and the ratio of EcochG AP to SP demonstrated a statistically significant variance between the two groups. The SPIN test exhibited the highest AUC, signifying its superior diagnostic capability in identifying hidden hearing loss.ConclusionThe present study has shown that the SPIN, as a behavioral test, and the EcochG AP amplitude measure, as an electrophysiological test, provide the greatest auditory diagnostic capability for identifying cochlear synaptopathy. Wave I amplitude in the auditory brainstem response (ABR) and the SP/AP ratio in electrocochleography (EcochG) are promising non-behavioral measures of cochlear synaptopathy or hidden hearing loss.

Analysis of Adherence to AAO-HNSF Clinical Practice Guidelines for Sudden Hearing Loss.

To assess adherence to the 2019 American Academy of Otolaryngology-Head and Neck Surgery (AAO-HNSF) guidelines for the diagnosis and management of sudden hearing loss (SHL) and sudden sensorineural hearing loss (SSNHL). Retrospective cohort. TriNetX, a de-identified healthcare database. Evaluation focused on the percentage of patients undergoing proper guideline-adherent workup and treatment for SHL. Key measures include audiogram testing within 2 weeks for distinguishing SSNHL from CHL. For SSNHL patients, MRI or auditory brainstem response (ABR) testing should be obtained, and steroids and/or hyperbaric oxygen may be offered within 2 weeks. There were 24,203 SHL patients, 59.1% (n = 14,309) of whom underwent recommended audiogram testing, with 35.8% (n = 8,674) completing it within 2 weeks. Overall, 3,107 were diagnosed with unilateral SSNHL, 104 with unilateral conductive hearing loss (CHL), 121 with mixed hearing loss, and 10,977 were lost to follow-up. Among 3,107 SSNHL cases, just 25.5% (n = 791) obtained MRI/ABR within 1 month, and vestibular schwannoma was diagnosed in 3.5% (n = 28). Additionally, steroids were prescribed to 54.5% (n = 1,692), and <0.3% (n ≤ 10) received hyperbaric oxygen. In accordance with strong recommendations against or insufficient evidence to support the following diagnostics and treatments, 2.0% (n = 63) underwent a CT scan, less than 0.3% (n ≤ 10) were prescribed vasodilators or thrombolytics, and 3.5% (n = 108) were on antivirals. There is a significant opportunity for improvement in evaluating patients with SHL, specifically SSNHL. Proper adherence to guidelines may improve screening, detection, and management of neurotologic pathologies, including vestibular schwannoma.

Research on the prognosis and efficacy criteria of sudden sensorineural hearing loss

Sudden sensorineural hearing loss is a common and frequently-occurring disease. Looking at the efficacy evaluation of the diagnosis and treatment of sudden sensorineural hearing loss at home and abroad, each country has its own characteristics in the indicators and criteria of the treatment efficacy evaluation, and the common indicators of efficacy evaluation include: average pure-tone hearing threshold, the absolute value of hearing improvement of impaired frequency, the percentage of average pure-tone hearing threshold increase, speech discrimination score, speech recognition threshold, etc. The evaluation criteria mostly take the efficacy grade or the improvement level of average hearing threshold as the criterion of effective treatment, and the lack of unified standards is not conducive to the homogenization of global research related to sudden sensorineural hearing loss. In order to provide reference for the clinical evaluation of sudden sensorineural hearing loss in China, this study systematically reviewed the clinical practice guidelines and the evaluation indexes and criteria of clinical efficacy in high quality clinical multicenter studies of the disease in many countries.

Speech-in-noise hearing impairment is associated with increased risk of Parkinson’s: A UK Biobank Analysis.

BackgroundHearing impairment is implicated as a risk factor for Parkinson’s disease (Parkinson’s) incidence, with evidence suggesting that clinically diagnosed hearing loss increases Parkinson’s risk 1.5-1.6 fold over 2-5 years follow up. However, the evidence is not unanimous with additional studies observing that self-reported hearing capabilities do not significantly influence Parkinson’s incidence. Thus, additional cohort analyses that draw on alternative auditory measures are required to further corroborate the link between Parkinson’s and hearing impairment. ObjectivesTo determine whether hearing impairment, estimated using a speech-in-noise test (the Digit Triplet Test, DTT), is a risk factor for Parkinson’s incidence. MethodsThis was a pre-registered prospective cohort study using data from the UK Biobank. Data pertaining to 159,395 individuals, who underwent DTT testing and were free from Parkinson’s at the point of assessment, were analysed. A Cox Proportional Hazard model, controlling for age, sex and educational attainment was conducted. ResultsDuring a median follow up of 14.24 years, 810 cases of probable Parkinson’s were observed. The risk of incident Parkinson’s increased with baseline hearing impairment [hazard ratio: 1.57 (95%CI: 1.018, 2.435; P = 0.041)], indicating 57% increase in risk for every 10 dB increase in speech-reception threshold (SRT). However, when hearing impairment was categorised in accordance with UK Biobank SRT norms neither ‘Insufficient’ nor ‘Poor’ hearing significantly influenced Parkinson’s risk compared to ‘Normal’ hearing. ConclusionsThe congruence of these findings with prior research further supports the existence of a relationship between hearing impairment and Parkinson’s incidence.

Intersecting Pathways: Understanding Dementia, Hearing Loss, and Health Disparities

Abstract Background Dementia is a major public health concern, affecting care dependency and disability in older ages. In Europe, around 14.1 million people live with dementia, a number that is expected to increase to 15.9 million by 2040. Hearing loss (HL), followed by education, has been proposed as the most important modifiable risk factor for dementia. This study aims to investigate how sex, educational attainment and HL intersect to influence dementia-free life expectancy (DemFLE) in older adults. Methods Using population register data covering all Finnish residents aged 50 years or older from 2010 to 2019, we apply discrete-time multistate Markov models. Transition probabilities between states, such as HL and dementia, are estimated with multinomial logit models stratified by sex and education. Multistate life tables are used to compute sex- and education-specific DemFLE. Results We identified 316,361 individuals with a diagnosis of dementia and 317,115 individuals with a diagnosis of HL, representing 1,449,873 and 2,738,970 person-years respectively. 14% of individuals with HL were eventually diagnosed with dementia, compared to only 8% of individuals without HL. We hypothesise that DemFLE will vary significantly by sex and education, with additional modulation by the presence of HL. Specifically, we expect that the presence of HL will further reduce DemFLE, and that the magnitude of this reduction will be relatively greater among those with lower educational attainment. Conclusions This study contributes to the emerging literature on the impact of hearing loss on dementia. While both dementia and hearing loss are irreversible health conditions, hearing loss can be managed by hearing aids. If we find evidence that hearing loss is associated with different levels of risk of dementia by educational attainment, it would suggest avenues for effective interventions and public health policies to ensure more equal ageing processes in the population. Key messages • Hearing loss may intersect with education and sex in the risk of dementia, providing evidence for the importance of addressing hearing health inequalities in dementia prevention efforts. • Understanding the interactions between hearing loss, education and sex can inform policies to promote healthy ageing and reduce the burden of dementia on individuals and health systems.

From Simulations to Inference: Using Machine Learning to Tune Patient-Specific Finite-Element Models of the Middle Ear Towards Objective Diagnosis.

Computational models, particularly finite-element (FE) models, are essential for interpreting experimental data and predicting system behavior, especially when direct measurements are limited. A major challenge in tuning these models is the large number of parameters involved. Traditional methods, such as one-by-one sensitivity analyses, are time-consuming, subjective, and often return only a single set of parameter values, focusing on reproducing averaged data rather than capturing the full variability of experimental measurements. In this study, we applied simulation-based inference (SBI) using neural posterior estimation (NPE) to tune an FE model of the human middle ear. The training dataset consisted of 10,000 FE simulations of stapes velocity, ear-canal (EC) input impedance, and absorbance, paired with seven FE parameter values randomly sampled within plausible ranges. The neural network learned the association between parameters and simulation outcomes, returning the probability distribution of parameter values that can reproduce experimental data. Our approach successfully identified parameter sets that reproduced three experimental datasets simultaneously. By accounting for experimental noise and variability during training, the method provided a probability distribution of parameters, representing all valid combinations that could fit the data, rather than tuning to averaged values. The network demonstrated robustness to noise and exhibited an efficient learning curve due to the large training dataset. SBI offers an objective alternative to laborious sensitivity analyses, providing probability distributions for each parameter and uncovering interactions between them. This method can be applied to any biological FE model, and we demonstrated its effectiveness using a middle-ear model. Importantly, it holds promise for objective differential diagnosis of conductive hearing loss by providing insight into the mechanical properties of the middle ear.

Inclusive Soundscapes: How Race, Socioeconomic Status and Maternal Age Influence the Pediatric Cochlear Implant Journey.

This study aimed to assess how race, social vulnerability, and maternal age influence pediatric cochlear implant access and usage. Retrospective cohort. Tertiary Pediatric University Hospital. This study included individuals aged 0 to 18 who received a cochlear implant at our center between the years 2000 and 2022. Social vulnerability data from 2020 was obtained from the Centers for Disease Control and Prevention. Of the 302 patients included in our study, 43% were black and 50% were white. Patients from the highest to lowest social vulnerability quintiles comprised 31%, 25%, 18%, 10%, and 14% of our sample, respectively. Race was associated with social vulnerability index (SVI) (P < .001), with a mean score of 0.70 (±0.26) and 0.49 (±0.27) for black and white patients, respectively. Later age at hearing loss (HL) diagnosis and cochlear implantation (CI) were associated with more and most vulnerable SVI (P < .05). Delayed diagnosis was also associated with black and other racial groups (P = .041), and adolescent maternal age (P = .03). Greater SVI was associated with less daily cochlear implant usage (P = .004). The most vulnerable patients were more likely to be lost to follow-up (P = .03) despite no difference based on maternal age (P = .59) and insurance status (P = .47). This study underscores the significance of mitigating disparities in timely diagnosis of HL, consistent CI usage, and appropriate follow-up care. This is a first step toward the formulation of novel strategies aimed at overcoming barriers and developing appropriate intervention programs.

The outcomes of an audiological management programme for neonates with hyperbilirubinaemia.

Hyperbilirubinaemia is a contributing condition to the prevalence of neonatal hearing loss. Because of its pathophysiology, the use of Otoacoustic Emissions (OAE) and Automated Auditory Brainstem Response (AABR) testing is essential in diagnosing hearing loss. Two-tier screening models are typically used in developing world contexts; however, a combined approach to testing (using both tests) should be used for early detection. Blood serum levels should also be monitored to determine how they impact audiological test results. To determine the outcomes of using a combined testing approach of both OAE and Auditory Brainstem Response (ABR) for both screening and diagnostic testing of neonates with hyperbilirubinaemia and studying the relationship between the test results and the serum bilirubin levels. A cross-sectional, comparative design was utilised. Forty neonates were tested (80 ears). Neonates underwent hearing screening and diagnostic testing (ABR and/or AABR and DPOAE tests). The study was conducted at a hospital in South Africa. One-third (32.5%) of the neonates had comorbidities. Screening results indicated that the AABR test could identify more cases of abnormalities than DPOAEs (p= 0.001). Participants with a serum level greater than 10 mg/dL presented with abnormal diagnostic ABR test results while passing the DPOAE test (p 0.001). Combined use of ABR and DPOAE testing yielded a greater identification of auditory pathology than using either test alone. Serum bilirubin levels can be used as an indicator for combination testing.Contribution:Combined use of ABR and DPOAE testing leads to greater identification of auditory pathology.

Newborn Hearing Screening Success… Patient Care Failure.

To assess the efficacy of a newborn hearing screening program in an urban hospital 24 years after a longitudinal study identified failings in its program and recommended solutions. A hospital-specific, Pennsylvania database was queried to determine rates of in-hospital testing, referral, and diagnosis for Temple University Hospital (TUH), and to compare these with reporting sites statewide. At TUH, interviews with newborn hearing screeners and audiologists were conducted to identify barriers to care. In the year 2022, 2,006/2,069 newborns (97%) were successfully screened prior to hospital discharge at TUH. (63 babies were never tested). There were 73 who did not pass automated auditory brainstem response testing (ABR) in at least one ear. All of these 73 infants returned for repeat automated ABR testing and 50/73 passed in both ears. 23/2006 (1%) were referred to diagnostic testing. 13/23 were successfully scheduled. 11/23 passed in both ears. 2/13 were found to have hearing losses. 10 infants (43%) were lost to follow-up and were never diagnosed. Examining lost-to-follow-up rates statewide, we identified a relationship between successful follow-up and local median family income. Legislation on the state level and improvements in audiometric technology have led to highly successful in-hospital screening with relatively low false-positive rates. Despite this, inadequate outpatient follow-up testing and inequities in the American health care system continue to delay audiometric diagnosis and habilitation of congenital hearing loss. Lack of transportation, childcare for siblings, and newborn insurance status impede access to diagnostic testing. Level 3 evidence-retrospective review comparing cases and controls. Laryngoscope, 2024.

Barriers to auditory brainstem response testing under anesthesia

ObjectiveAuditory brainstem response (ABR) testing is the gold standard for diagnosis of hearing loss in children who cannot complete behavioral audiometry. Testing under general anesthesia is often recommended when natural sleep ABR and/or behavioral audiometry are unsuccessful. This study aims to determine which demographic and patient factors serve as barriers to receiving this diagnostic testing. MethodsA retrospective chart review from an internal database of patients who underwent ABR testing under anesthesia from 2017 to 2023 was completed. Patient demographics, clinical diagnoses, dates of initial recommendation, and dates of testing were recorded. ResultsA total of 395 patients met inclusion criteria, with a median time from initial evaluation to successful ABR under anesthesia of 5.1 months (range 0.1–209 months). This time was significantly higher in patients with public insurance compared to private insurance and in patients with the following medical complexities: cardiac disease, developmental delay, neurologic disease, eye disease, and genetic syndromes not associated with hearing loss. The interval was significantly shorter in patients with abnormal ear anatomy. ConclusionPatient factors, such as insurance type and certain medical diagnoses, may lead to delayed ABR testing under anesthesia and thus delayed diagnosis and management of hearing loss. This has implications for the timely care and treatment of children with hearing loss.

Predictors of Delays in the Management of Idiopathic Sudden Sensorineural Hearing Loss.

To study predictors of delayed presentation, diagnosis, and treatment of idiopathic sudden sensorineural hearing loss (ISSNHL). Retrospective medical chart review and patient telephone survey. Tertiary medical center. All patients who initially presented or referred with unilateral ISSNHL between 2016 and 2020 were included. Associations between epidemiological, demographic, and socioeconomic profiles and delays in presentation, diagnosis and treatment were studied. A total of 518 patients were suitable for study inclusion. The total delay in the emergency department (ED) setup was a median (interquartile range, IQR) of 1 (0-1) day, 7 (6-12) days in a community otolaryngologist setup, and 15 (8-25) days in a general practitioner setup. Patients who presented to the ED first also had fewer diagnostic and treatment delays. Those who presented to a community otolaryngologist first had significantly longer presentation delay (5 [4-8] days p < 0.001) and significantly longer treatment delays (1 [1-3] days p < 0.001). Patients who presented to a general practitioner first had significantly longer presentation delays compared with ED presentation, and the longest diagnostic and treatment delays (3 [2-5], 8 [4-12] days, and 4 [2-7] days, p = < 0.01, p = <0.01, and p < 0.001, respectively). There was no association between socioeconomic status or demography and presentation, diagnostic, or treatment delays. Total delay in ISSNHL management is affected by the venue of the first medical encounter. General practitioners' level of awareness of the need for empiric steroidal treatment of ISSNHL without delay should be raised. 3 Laryngoscope, 135:889-893, 2025.