Audiologic and vestibular assessment in children and adolescents with transfusion dependent beta thalassemia major: The era of deferasirox film coated tablet.

BACKGROUND: Hearing impairment has frequently been described in ß-thalassemia patients with a significant impact on the patients' quality of life. Most studies provided evidence of deferoxamine (DFO) dose-related ototoxicity, however, the data is scarce regarding deferasirox (DFX) as a sole iron chelator. AIM: We aimed to assess the prevalence and risk factors of sensorineural hearing loss (SNHL) and vestibular dysfunction in regularly transfused ß-thalassemia patients who had been treated with DFX film coated tablets. METHODS: We conducted a case control study on 57 transfusion dependent ß-thalassemia patients with a mean age of 15.3 years who received DFX FCT as monotherapy for at least one consecutive year, and 57 healthy age and sex-matching controls. Comprehensive audiological evaluations using pure tone audiometry (PTA) and transient evoked otoacoustic emission (TEOAE) as well as vestibular evaluation using Video-nystagmography (VNG) were done. RESULTS: SNHL was identified in 12 patients (21.1 %) using PTA and a statistically significant difference was detected between controls and patients at 6 KHz and 12 KHz frequencies. A higher incidence of SNHL was detected using TEOAE, 22 patients (43.1 %) failed to pass TEOAE, with a statistically significant decrease in the signal at frequencies 1, 4 KHz bilaterally and at frequencies 1.5, 2 KHz in the right ear compared to controls. Canal paresis was detected in 21 (36.8 %) of thalassemic children using bithermal caloric test with significantly more unilateral weakness than control children (P = 0.008). We found no significant correlation between audio-vestibular dysfunction and age, sex, serum ferritin, frequency of blood transfusion and dose of DFX FCT in thalassemic children. CONCLUSION: We conclude that the incidence of SNHL and vestibular dysfunction was high among transfusion dependent ß-thalassemia patients. Therefore, we recommend performing pre-treatment baseline audio-vestibular assessment and yearly audio-vestibular monitoring to early detect high risk patients and initiate timely management to prevent permanent damage.

Vestibular function assessment in Idiopathic sudden sensorineural hearing loss: a prospective study.

PURPOSE: Idiopathic sudden sensorineural hearing loss (ISSHL) can cause acute damage not only to the auditory function, but also to the vestibular function in addition to damage to the hearing function. The aim of this study was to perform vestibular assessment using caloric test and video head impulse test in patients with idiopathic sudden sensorineural hearing loss. In addition, to evaluate the relationship of dizziness with vestibular tests and post-treatment responses of vestibular tests. METHODS: This is an observational, longitudinal and prospective study, including patients diagnosed with idiopathic sudden sensorineural hearing. Patients were divided into two groups according to the presence of vestibular complaints at presentation: Group 1: Patients with vestibular complaints, Group 2: Patients without vestibular complaints. All subjects underwent pure tone audiometry (PTA) testing, cold caloric test and video head impulse test (vHIT) during their admission and on the 10th day, 3rd month, and 1st year of their follow-up outpatient clinic controls. A unilateral weakness (UW) in the caloric test response was quantified according to the Jongkees formula. RESULTS: A positive and significant relationship was found between the degree of hearing loss according to the ASHA criteria pre-treatment and the level of improvement created according to Siegel criteria at the 10th day, 3rd month, 1st year after treatment (respectively p = 0.001, p = 0.001, p < 0.001). When both short-term and long-term results were evaluated after treatment, a positive improvement in the degree of hearing loss was observed. A statistically significant difference was observed between Groups 1 and 2 when the pre-treatment, at 10 days, 3 months and 1 year after treatment the caloric test UW value was compared (respectively p = 0.020, p = 0.004, p = 0.004, p = 0.004). A statistically significant difference was observed between Groups 1 and 2 when the pre-treatment, at 10 days, 3 months and 1 year after treatment vHIT lateral canal VOR value was compared (respectively p = 0.000, p = 0.001, p = 0.000, p = 0.004). When both short-term and long-term results were evaluated after treatment, a positive improvement was observed in both caloric test results and lateral vHIT VOR values. Pre-treatment, post-treatment 10th day, 3rd month, 1st year vHIT anterior and posterior canal VOR values were found to be VOR˃0.8 in all patients. No difference was observed in anterior and posterior canal VOR values. CONCLUSION: Vertigo in patients with ISSHL "as objectively confirmed through caloric testing and vHIT" can be considered a sign of severe cochlear damage. Our study demonstrated a significantly increased risk of vestibular affect in patients with ISSHL, especially in the presence of vertigo. Thus, we conclude that the focus in ISSHL should not only be on the cochlea but also on the vestibular system.

Risk Factors Associated with Delays in Hearing Loss Identification in Pediatric Patients.

OBJECTIVE: To identify sociodemographic factors associated with pediatric late-identified hearing loss (LIHL) and classify novel subgroups within the LIHL population. STUDY DESIGN: Retrospective cohort. SETTING: Tertiary children's hospital. METHODS: Our cohort included children with permanent hearing loss (HL) between 2012 and 2020 (n = 1087). Patients with early-identified HL were compared to patients with LIHL (>6 months of age at diagnosis), and 3 subgroups: (1) late-identified congenital HL: failed NHS but had a diagnostic audiogram >6 months old; (2) late-onset HL: passed NHS and identified with HL after 6 months old; (3) late-identified, unknown-onset: unknown NHS results, identified after 6 months old. Geospatial analysis was performed using ArcGIS Pro. RESULTS: Compared with early-identified children, children with LIHL were more likely to have more comorbidities (odds ratio [OR] = 1.12, [1.01, 1.23]), be an under-represented minority (URM) (OR = 1.92, [1.27, 2.93]) and have a higher social vulnerability index (SVI) (adjusted odds ratio [AOR] = 2.1, [1.14, 3.87]). However, subgroups in the LIHL cohort had variable associations. Children with late-identified unknown onset hearing loss were uniquely associated with a primarily non-English speaking household (AOR = 1.84, [1.04, 3.25]), whereas children with late-onset hearing loss were less likely to have public insurance (AOR = 0.47, [0.27, 0.81]. There were no significant associations for children with late-identified congenital hearing loss. Neighborhood disadvantage, as measured by SVI, had an increased association with late-identified unknown onset HL (AOR = 4.08, [2.01, 8.28]) and a decreased association with late-onset HL (AOR = 0.40, [0.22, 0.72]). CONCLUSION: Sociodemographic factors serve as proxies for health care access, and these factors vary across LIHL pathways. Understanding the risk factors associated with each LIHL subgroup may help address disparities in pediatric HL identification.

Assessment of prognostic biomarkers in sudden sensorineural hearing loss: A systematic review and meta-analysis.

Sudden sensorineural hearing loss (SSNHL) is defined as hearing loss of more than 30 dB in less than 72 h. SSNHL is a frequent complaint and an emergency in otolaryngology. Various biomarkers have been used to determine the prognosis of SSNHL. This systematic review and meta-analysis aims to evaluate the relationship between the different biomarkers and the prognosis of SSNHL. We searched English-language literature up to October 2022 in four databases, including PubMed, Google Scholar, Cochrane, and Science Direct. This search was conducted in accordance with the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) statement. This study was reported in the International Prospective Register of Systematic Reviews (PROSPERO) database (ID = CRD42022369538). All studies examining the role of neutrophil to lymphocyte ratio (NLR) concluded that higher NLR is associated with a worse prognosis. The results of studies regarding the relationship between platelet to lymphocyte ratio (PLR) and tumor necrosis factor (TNF) are controversial. Other factors shown to be associated with SSNHL include Glycated hemoglobin (HbA1C), blood glucose, iron levels, serum endocan, salusin-beta, and bone turnover biomarkers. This meta-analysis showed that PLR, NLR, and neutrophils were significantly different between recovered and non-recovered patients. PLR, NLR, and neutrophil count are reliable tools to assess the prognosis of patients with SSNHL.

Association between individual, household, and area-level socioeconomic status indicators and sensorineural hearing loss in adults in southwest Iran: a population-based study.

Introduction: Hearing loss is the fourth most common chronic disease, but studies on the relationship between hearing loss and socioeconomic factors are limited. We aimed to examine the association between hearing loss and socioeconomic factors among 35-70 year adults in southwest Iran. Materials and methods: This population-based cross-sectional study was conducted in the baseline of Hoveyzeh cohort study in adults aged 35-70 in southwest Iran between 2017 and 2021. Information on socioeconomic factors, demographic characteristics, comorbidities, family history of hearing loss, and noise exposure was collected. We assessed the relationship between three levels of socioeconomic factors (individual, household, and area level) with sensorineural hearing loss (SNHL). Multiple logistic regression was used to adjust the potential confounders. Results: Among a total of 1,365 assessed participants, 485 patients were diagnosed as having hearing loss, and the other 880 individuals were diagnosed without hearing loss, which is considered the case and the control group, respectively. At the individual level of socioeconomic, the odds of having hearing loss in the participants with high school education and diploma, [OR = 0.51 (95%CI:0.28-0.92)], and the individuals with university education [OR = 0.44 (95%CI:0.22-0.87)] were significantly lower than the illiterate participants. At the household socioeconomic level, the odds of having hearing loss were lower for those with poor [OR = 0.63 (95%CI:0.41-0.97)] and moderate [OR = 0.62 (95%CI:0.41-0.94)] wealth status vs. those with the poorest wealth status. In the area level socioeconomic, although the odds of hearing loss in the residents of affluent areas were slightly lower than the residents of deprived areas, there was no significant difference among the groups. Conclusion: The individuals with hearing loss may have insufficient education and income.

Cochlear nerve dysplasia in unilateral severe to profound congenital sensorineural hearing loss - Prevalence in Australian children and the impact of socioeconomic disadvantage on its management.

INTRODUCTION: Congenital unilateral sensorineural hearing loss (cuSNHL) carries potentially significant social, educational, and developmental consequences. Early diagnosis enables investigation, and consideration of options for management and early intervention, helping to mitigate the effects of hearing loss. Cochlear nerve dysplasia (CND) is a prominent cause of cuSNHL and may affect candidacy for cochlear implantation. Socioeconomic disadvantage may impact on a patient's family's capacity to participate in necessary intervention and follow-up. METHODS: Infants with severe-profound cuSNHL referred to a large Australian quaternary pediatric center between October 2004 and December 2020 were retrospectively included. Audiometric and clinical data, and the presence of hearing loss risk factors were obtained from a prospectively collated database. In Australia MRI scans are provided free-of-charge to citizens and residents. MRI scans were reviewed to determine the status of the nerves within the internal acoustic meatus (IAM grade) along with attendance rates. Travel distance to the hospital was also calculated. Reasons for non-attendance at MRI were obtained from patient medical records and correspondence. Socioeconomic, educational, and occupational indices, and travel distances were obtained using patient residential postcodes with reference to Australian Bureau of Statistics data. RESULTS: A total of 98 patients were reviewed, 64.3% (n = 63) of whom underwent MRI. The median age at diagnosis was 40 days (IQR 27). The prevalence of CND was 75% (n = 47). Importantly, there was no significant difference in the degree of hearing loss between IAM grades (F(4,57) = 1.029, p = 0.405). Socioeconomic indices were significantly lower in patients not attending MRI investigations compared with patients who did attend. Travel distance was not significantly different between the two groups. CONCLUSION: CND is a prominent cause of cuSNHL in Australian infants. MRI at a young age allows parent education regarding management options and timely intervention where indicated. Socioeconomic disadvantage significantly impacts on participation in further routine assessment of cuSNHL, potentially limiting management options for these children long term.

Assessment of seasonal pattern of idiopathic sudden sensorineural hearing loss: a retrospective cross-sectional study.

BACKGROUND: A seasonal trend of patients with idiopathic sudden sensorineural hearing loss may direct research into possible aetiology. METHODS: This study reviewed data from the medical records of patients who presented from 2004 to 2019 and who were diagnosed with new-onset idiopathic sudden sensorineural hearing loss. Seasonal pattern was assessed using chi-square and Rayleigh tests, and further confirmed by Monte Carlo simulation. RESULTS: The study included 740 patients with a mean age of 48.3 years and a median age of 49 years. There was no statistical evidence for a difference in the distribution of sensorineural hearing loss cases for the four seasons of each year or with the cumulative data. New-onset idiopathic sudden sensorineural hearing loss cases averaged around 11 per month; there was no statistical evidence for a seasonal difference, as determined either by the Rayleigh test or with Monte Carlo simulation. CONCLUSION: There was no evidence to support the claim that idiopathic sudden sensorineural hearing loss incidence displays a seasonal pattern. More research is necessary to explore potential external factors such as climate or infection.

Development of an audiological assessment and diagnostic model for high occupational noise exposure.

PURPOSE: To explore the diagnostic auditory indicators of high noise exposure and combine them into a diagnostic model of high noise exposure and possible development of hidden hearing loss (HHL). METHODS: We recruited 101 young adult subjects and divided them according to noise exposure history into high-risk and low-risk groups. All subjects completed demographic characteristic collection (including age, noise exposure, self-reported hearing status, and headset use) and related hearing examination. RESULTS: The 8 kHz (P = 0.039) and 10 kHz (P = 0.005) distortion product otoacoustic emission amplitudes (DPOAE) (DPs) in the high-risk group were lower than those in the low-risk group. The amplitudes of the summating potential (SP) (P = 0.017) and action potential (AP) (P = 0.012) of the electrocochleography (ECochG) in the high-risk group were smaller than those in the low-risk group. The auditory brainstem response (ABR) wave III amplitude in the high-risk group was higher than that in the low-risk group. When SNR = - 7.5 dB (P = 0.030) and - 5 dB (P = 0.000), the high-risk group had a lower speech discrimination score than that of the low-risk group. The 10 kHz DPOAE DP, ABR wave III amplitude and speech discrimination score under noise with SNR = - 5 dB were combined to construct a combination diagnostic indicator. The area under the ROC curve was 0.804 (95% CI 0.713-0.876), the sensitivity was 80.39%, and the specificity was 68.00%. CONCLUSIONS: We expect that high noise exposure can be detected early with this combined diagnostic indicator to prevent HHL or sensorineural hearing loss (SNHL). TRIAL REGISTRATION NUMBER/DATE OF REGISTRATION: ChiCTR2200057989, 2022/3/25.

Targeted screening for congenital cytomegalovirus: A micro-costing analysis.

AIM: We aimed to determine the cost and potential cost-savings of delivering a targeted congenital cytomegalovirus (cCMV) screening programme through a universal newborn hearing screening (UNHS) programme to detect cCMV-related hearing loss in infants from Victoria, Australia. METHODS: We completed a micro-costing analysis from a health-care perspective using data from a targeted cCMV screening programme piloted between June 2019 and March 2020. The programme involved collection of saliva samples to test for cCMV in infants who: received a 'refer' result on their second newborn hearing screen; were aged 21 days or less; and born at one of four maternity hospitals in Victoria, Australia. All costs to complete targeted cCMV screening were recorded in Australian 2020 dollars. Potential costs and benefits of adding targeted cCMV screening to the pre-existing UNHS programme were compared to when no screening was available up to 18 years to determine the likely cost or cost savings. RESULTS: The cost of adding targeted cCMV screening to Victoria's UNHS is $202 per infant screened. The total cost per positive case identified is $21 456. The overall cost of adding targeted salivary cCMV screening at the point of a second 'refer' result on the UNHS programme in Victoria's four largest hospitals is estimated to be $28 966 for the first year. CONCLUSION: Targeted screening for cCMV provides families the opportunity to detect and, if appropriate, treat cCMV in the first month of life in line with current recommendations. It falls within the range between cost neutral and cost saving.

Assessment of prestin level changes as an inner-ear biomarker in patients with idiopathic sudden sensorineural hearing loss.

OBJECTIVE: To assess the correlation of serum prestin level and audiological findings in adults with idiopathic sudden sensorineural hearing loss. METHODS: Audiometry and serum prestin measurements were performed at study entry (T0), at day 14 (end of treatment, T1) and at day 30 (T2). RESULTS: A total of 25 idiopathic sudden sensorineural hearing loss patients and 25 healthy adults were included. The geometric mean prestin level in the case and control groups at T0 was 227.7 pg/ml and 130.5 pg/ml, respectively. The geometric mean prestin level in the case group demonstrated a downward trend at T1 and T2 (214.0 pg/ml and 180.1 pg/ml, respectively; p < 0.001). Of 17 patients with high baseline prestin levels (over 150 pg/ml), prestin levels tended to decrease in 11 patients, and 5 of them (45.5 per cent) showed good recovery. CONCLUSION: The prestin concentrations increased in two-thirds of patients with idiopathic sudden sensorineural hearing loss. Future work is recommended to determine the location of injury.

Assessment of cognitive function in young children with type 1 diabetes mellitus using electrophysiological tests.

BACKGROUND/OBJECTIVES: Diabetes mellitus is a chronic disease that affects many body systems, including the nervous and auditory systems. It is noted that there is a scarcity of research on the effect of diabetes on cognitive functions in particular and auditory functions in general in children with type 1 diabetes. Therefore, this study was designed to assess cognitive and auditory functions in children with type 1 diabetes mellitus and to correlate the reflection of diabetes control on cognitive functions. METHODS: This study is a case-control study that included 100 children divided into two groups, the patient group, which includes 50 children with type 1 diabetes, and the control group, which consists of 50 healthy children. Subjects in the current study were submitted to pure tone audiometry, speech recognition threshold test, immittancemetry study, and measurement of cortical auditory evoked and P300 potentials (CAEPs and P300). These audiometric measures were statistically analyzed and correlated with the clinical characteristics of the study group. RESULTS: The latency of P300 and CAEPs was significantly increased while the amplitude of P300 and CAEPs was significantly decreased in the patient group compared to the control group (p < 0.001). P300 and CAEPs latency has a positive correlation with HbA1c levels (r = 0.460). In addition, there was significant differences between the two groups regarding the hearing threshold at 8000 Hz, and 28% of patients had bilateral sensorineural hearing loss (SNHL) at 8 kHz. CONCLUSION: The prolonged P300 and CAEPs latency and decreased amplitude in patients indicate a cognitive decline in individuals with type 1 diabetes compared to healthy individuals. HbA1c levels may increase the risk of cognitive impairment in children. In addition, the risk of bilateral SNHL increased at 8 kHz in children with type 1 diabetes mellitus.

Assessment of hearing loss in patients with rosacea.

OBJECTIVES/AIMS: Rosacea is not only a skin condition but a systemic inflammatory disease that includes chronic inflammation, vascular alterations, and autoimmunity in pathogenesis. We aimed to evaluate the presence of a sensorineural hearing loss in the patients with rosacea in comparison with the healthy control group and, also to compare the audiometric results according to the severity of disease among the patient group. METHODS: Fifty-three patients with erythematelangiectatic or papulopustular type of rosacea and 105 age- and sex-matched healthy controls were included. Each participant had audiometric measurements after a complete ear-nose-throat examination by the same otorhinolaryngologist. RESULTS: The results of air and bone conduction thresholds showed statistically significant differences in particularly high frequencies between the groups in both the right and left ear (for all p < 0.05), but there was no correlation between audiometric measurements and the severity or the type of rosacea (p > 0.05). CONCLUSIONS: Regardless of disease severity or type, rosacea patients are likely to have sensorineural hearing loss, and it is important to refer these patients in the early period.

The genetic etiology of hearing loss in Japan revealed by the social health insurance-based genetic testing of 10K patients.

Etiological studies have shown genetic disorders to be a major cause of sensorineural hearing loss, but there are a limited number of comprehensive etiological reports based on genetic analysis. In the present study, the same platform using a diagnostic DNA panel carrying 63 deafness genes and the same filtering algorithm were applied to 10,047 samples obtained from social health insurance-based genetic testing of hearing loss. The most remarkable result obtained in this comprehensive study was that the data first clarified the genetic epidemiology from congenital/early-onset deafness to late-onset hearing loss. The overall diagnostic rate was 38.8%, with the rate differing for each age group; 48.6% for the congenital/early-onset group (~5y.o.), 33.5% for the juvenile/young adult-onset group, and 18.0% for the 40+ y.o. group. Interestingly, each group showed a different kind of causative gene. With regard to the mutational spectra, there are certain recurrent variants that may be due to founder effects or hot spots. A series of haplotype studies have shown many recurrent variants are due to founder effects, which is compatible with human migration. It should be noted that, regardless of differences in the mutational spectrum, the clinical characteristics caused by particular genes can be considered universal. This comprehensive review clarified the detailed clinical characteristics (onset age, severity, progressiveness, etc.) of hearing loss caused by each gene, and will provide useful information for future clinical application, including genetic counseling and selection of appropriate interventions.

Vestibular mapping assessment in idiopathic sudden sensorineural hearing loss.

OBJECTIVE: The aim of this study was to investigate patterns of semicircular canal (SCC) and otolith organ dysfunction by vestibular mapping, and to determine the clinical implications of treatment outcomes in idiopathic sudden sensorineural hearing loss (ISSNHL). METHODS: We retrospectively reviewed 135 consecutive patients diagnosed with ISSNHL from January 2016 to December 2020. Patients underwent video-head impulse tests (vHIT) for each SCC, cervical vestibular-evoked myogenic potential test for the saccules, ocular vestibular-evoked myogenic potential test for the utricles, and hearing tests. Hearing outcomes were evaluated according to the American Academy of Otolaryngology-Head and Neck Surgery criteria and factors associated with prognosis were assessed. We also conducted vestibular mapping assessments and hierarchical cluster analysis. RESULTS: Overall, utricular impairment (76, 56.3%) was the most frequent diagnosis in the 135 ISSNHL patients, followed by saccular impairment (59, 43.7%) and posterior SCC impairment (30, 22.2%). The mean number of affected end organs was 1.37 ± 1.24, with higher numbers in the complete recovery group than in the partial/no recovery groups. In a multivariate analysis, higher initial hearing level and abnormal vHIT results in the posterior SCC were associated with poor prognosis in ISSNHL. In hierarchical cluster analysis, horizontal SCC and anterior SCC showed the highest similarity but were in different clusters than posterior SCC, and the saccule and utricle were in separate clusters from the three SCCs. CONCLUSIONS: The vestibular end organ showed various patterns of dysfunction in patients with ISSNHL. Of the five vestibular end organs, only abnormal posterior SCC was associated with poor prognosis for hearing recovery.

Objective Assessment System for Hearing Prediction Based on Stimulus-Frequency Otoacoustic Emissions.

Stimulus-frequency otoacoustic emissions (SFOAEs) can be useful tools for assessing cochlear function noninvasively. However, there is a lack of reports describing their utility in predicting hearing capabilities. Data for model training were collected from 245 and 839 ears with normal hearing and sensorineural hearing loss, respectively. Based on SFOAEs, this study developed an objective assessment system consisting of three mutually independent modules, with the routine test module and the fast test module used for threshold prediction and the hearing screening module for identifying hearing loss. Results evaluated via cross-validation show that the routine test module and the fast test module predict hearing thresholds with similar performance from 0.5 to 8 kHz, with mean absolute errors of 7.06-11.61 dB for the routine module and of 7.40-12.60 dB for the fast module. However, the fast module involves less test time than is needed in the routine module. The hearing screening module identifies hearing status with a large area under the receiver operating characteristic curve (0.912-0.985), high accuracy (88.4-95.9%), and low false negative rate (2.9-7.0%) at 0.5-8 kHz. The three modules are further validated on unknown data, and the results are similar to those obtained through cross-validation, indicating these modules can be well generalized to new data. Both the routine module and fast module are potential tools for predicting hearing thresholds. However, their prediction performance in ears with hearing loss requires further improvement to facilitate their clinical utility. The hearing screening module shows promise as a clinical tool for identifying hearing loss.

Congenital Cytomegalovirus Infection: Epidemiology, Timely Diagnosis, and Management.

Congenital cytomegalovirus (cCMV) infection is common because of the ubiquitous nature of the virus and the lack of an effective prevention strategy during pregnancy. Most infants with cCMV are asymptomatic, although a notable subset can have sequelae including, most commonly, sensorineural hearing loss and neurodevelopmental disability, which may not be present at birth. Timely screening for cytomegalovirus in the first weeks after birth is critical to appropriately diagnose congenital infection, evaluate affected infants, and determine the treatment course. Antiviral therapy with valganciclovir can optimize end hearing and neurodevelopmental outcomes in symptomatic infants. This review discusses the epidemiology and clinical manifestations of cCMV, targeted and universal screening approaches, and treatment and monitoring of infants with cCMV.

[Assessment of German-Language Information on Sudden Sensorineural Hearing Loss in the Internet]. / Beurteilung von im Internet verfügbaren deutschsprachigen Patienteninformationen zum Hörsturz.

OBJECTIVES: As a result of digitalization, the internet embodies the essential information medium. Especially, patients with sudden sensorineural hearing loss (SSNHL) require profound education due to unclear scientific evidence. Thus, our study investigated a German-language internet search about SSNHL. DESIGN: The first 30 Google-search results with the term "Hörsturz" (SSNHL in German) were categorized, readability-statistic with different readability-scores (FRES: 0=complex, 100=easy; FKL; SMOG; GFI) calculated, and misinformation documented. A structured content-analysis was performed with the DISCERN-questionnaire (1=low, 5=high quality). Certification of the Health-On-The-Net-Foundation (HON) assessed the abidance of recommended standards. RESULTS: 18 websites (60.0%) accounted for digital media, 7 (23.3%) manufacturers of medical devices, 2 (6.7%) government institutions, and respectively 1 (3.3%) healthcare provider, support-group, and scientific article. Mean word count was 1307.0±840.2, last update 17.1±32.5 months ago, and FRES 36.1±13.9, with the most difficult text by the scientific article (13.7). Mean of DISCERN was 2.2±0.7 with worst rating of manufacturers of medical devices (1.6±0.5). 2 websites (6.7%) were HON-certified, and 14 (46.7%) contained misinformation. CONCLUSION: Internet-based patient-information should be assessed cautiously due to poor readability, potential conflict of interests, low quality, or wrong information. Hence, healthcare providers and professional associations are urged to provide high-quality patient-information in the internet.

[Assessment of the population and subpopulation composition of lymphocytes and their relationship in patients with sensorineural hearing loss]. / Otsenka populyatsionnogo i subpopulyatsionnogo sostava limfotsitov i ikh vzaimootnosheniya u patsientov s neirosensornoi tugoukhost'yu.

Study of the role of the phenotypic composition of lymphocytes, shifts in which can cause the development of various pathological processes, including sensorineural hearing loss, is an important area of modern research. Purpose of the study. To study the population and subpopulation composition of lymphocytes and their intercellular cooperation in persons with occupational sensorineural hearing loss. MATERIAL AND METHODS: Phenotyping of blood lymphocytes (CD3+, CD4+, CD8+, CD9+, CD16+, CD20+, CD21+, CD23+, CD25+, CD95+) was performed by indirect immunofluorescence. RESULTS: Individuals with sensorineural hearing loss were found to have hyperactivation in both T- and B-cell links immunity. CONCLUSIONS: The revealed changes indicate a violation of immunoregulation through factors of intercellular cooperation, and the established relationships between populations and subpopulations of lymphocytes indicate increased activity components of the immune system, their important role in the implementation of the immune response in persons with sensorineural hearing loss.

Exome Sequencing for Isolated Congenital Hearing Loss: A Cost-Effectiveness Analysis.

OBJECTIVES/HYPOTHESIS: To assess the relative cost-effectiveness of exome sequencing for isolated congenital deafness compared with standard care. STUDY DESIGN: Incremental cost-effectiveness and cost-benefit analyses were undertaken from the perspective of the Australian healthcare system using an 18-year time horizon. METHODS: A decision tree was used to model the costs and outcomes associated with exome sequencing and standard care for infants presenting with isolated congenital deafness. RESULTS: Exome sequencing resulted in an incremental cost of AU$1,000 per child and an additional 30 diagnoses per 100 children tested. The incremental cost-effectiveness ratio was AU$3,333 per additional diagnosis. The mean societal willingness to pay for exome sequencing was estimated at AU$4,600 per child tested relative to standard care, resulting in a positive net benefit of AU$3,600. Deterministic and probabilistic sensitivity analyses confirmed the cost-effectiveness of exome sequencing. CONCLUSIONS: Our findings demonstrate the cost-effectiveness of exome sequencing in congenital hearing loss, through increased diagnostic rate and consequent improved process of care by reducing or ceasing diagnostic investigation or facilitating targeted further investigation. We recommend equitable funding for exome sequencing in infants presenting with isolated congenital hearing loss. LEVEL OF EVIDENCE: N/A. Laryngoscope, 131:E2371-E2377, 2021.