Hearing health survey of the population in Bangkok.

In this cross-sectional random survey among Thai adults living in Bangkok, we aimed to identify the prevalence of hearing problems and examine their relationship with individual factors. We administered a self-report questionnaire and performed pure-tone air conduction threshold audiometry. A total of 2463 participants (1728 female individuals) aged 15-96 years were included. The hearing loss prevalence was 53.02% and increased with age. The prevalence of a moderate or greater degree of hearing impairment was 2.8%. Participants aged 65 years and over had 8.56 and 6.79 times greater hearing loss and hearing impairment than younger participants, respectively. Male participants were twice as likely to have hearing loss and hearing impairment as female individuals. Participants with higher education levels showed less likelihood of having hearing loss and hearing impairment than those with no or a primary school education. Participants who ever worked under conditions with loud noise for > 8 h per day had 1.56 times greater hearing loss than those without such exposure. An inconsistent correlation was found between hearing loss, hearing impairment and noncommunicable diseases (diabetes, hypertension, and obesity). Although most participants had mild hearing loss, appropriate care and monitoring are necessary to prevent further loss in such individuals. The questionnaire-based survey found only people with hearing problems that affect daily communication.

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

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

Clinical Reasoning: A 24-Year-Old Man With Gait Impairment, Hearing Loss, and Recurrent Fever.

We present a case study of a 24-year-old man who reported mild balance and walking difficulties for 2 years. He had a history of recurrent fever, skin lesions, headache, and elbow pain, but most of these events resolved spontaneously. There was no significant family history. On examination, we observed frontal bossing, sensorineural hearing loss, and gait ataxia. This case underscores the significance of identifying clinical indicators in patients with neurologic symptoms, particularly recurrent fever, to establish a precise and thorough differential diagnosis.

Chronological change of left ventricular global longitudinal strain in patients with maternally inherited diabetes and deafness: A case series.

RATIONALE: Maternally inherited diabetes and deafness (MIDD) is a rare genetic disorder arising from mitochondrial DNA mutations, characterized by a combination of diabetes mellitus and sensorineural deafness. It is known that MIDD patients with cardiomyopathy have a poor prognosis, but there are no established guidelines for the diagnosis and follow-up of cardiomyopathy in MIDD patients. PATIENT CONCERNS: Patient 1 was a 48-year-old woman who visited the hospital with cardiomegaly and had been taking oral hypoglycemic agents for 8 years. Patient 2 was a 21-year-old man, the son of patient 1, who visited the hospital for genetic screening. Patient 2 was also diagnosed diabetes mellitus 2 years ago. DIAGNOSIS: Patient 1 was found to have restrictive cardiomyopathy on echocardiography and underwent endomyocardial biopsy and genetic testing to determine the etiology. The m.3243A>G mutation was confirmed and she was diagnosed with MIDD accompanied with diabetes and hearing loss. Additionally, patient 2 had m.3243 A>G mutation and was diagnosed with MIDD due to diabetes and hearing loss. INTERVENTIONS: Because MIDD does not have a specific treatment, patient 1 took ubidecarenone (coenzyme Q10), acetylcarnitine, and multivitamin along with the treatment for diabetes control and heart failure. Patient 2 was taking ubidecarenone (coenzyme Q10), acetylcarnitine, and multivitamin along with treatment for diabetes. OUTCOMES: She subsequently underwent routine transthoracic echocardiography, and a progressive decline in global longitudinal strain (GLS) was first observed, followed by a worsening of the patient's clinical situation. Patient 2 had concentric remodeling and decreased GLS. On periodic echocardiography, GLS decreased at a very slow rate, and the patient's clinical course was stable. LESSONS: The findings of this report contribute to the understanding of the clinical course of MIDD-associated cardiomyopathy and highlight the potential of GLS as a sensitive marker for disease progression.

Assessing Speech Audibility via Syllabic-Rate Neural Responses in Adults and Children With and Without Hearing Loss.

An objective method for assessing speech audibility is essential to evaluate hearing aid benefit in children who are unable to participate in hearing tests. With consonant-vowel syllables, brainstem-dominant responses elicited at the voice fundamental frequency have proven successful for assessing audibility. This study aimed to harness the neural activity elicited by the slow envelope of the same repetitive consonant-vowel syllables to assess audibility. In adults and children with normal hearing and children with hearing loss wearing hearing aids, neural activity elicited by the stimulus /su∫i/ or /sa∫i/ presented at 55-75 dB SPL was analyzed using the temporal response function approach. No-stimulus runs or very low stimulus level (15 dB SPL) were used to simulate inaudible conditions in adults and children with normal hearing. Both groups of children demonstrated higher response amplitudes relative to adults. Detectability (sensitivity; true positive rate) ranged between 80.1 and 100%, and did not vary by group or stimulus level but varied by stimulus, with /sa∫i/ achieving 100% detectability at 65 dB SPL. The average minimum time needed to detect a response ranged between 3.7 and 6.4 min across stimuli and listener groups, with the shortest times recorded for stimulus /sa∫i/ and in children with hearing loss. Specificity was >94.9%. Responses to the slow envelope of non-meaningful consonant-vowel syllables can be used to ascertain audible vs. inaudible speech with sufficient accuracy within clinically feasible test times. Such responses can increase the clinical usefulness of existing objective approaches to evaluate hearing aid benefit.

The impact of amplification on quality of life in women with Turner syndrome.

BACKGROUND: Individuals with Turner syndrome (TS, ORPHA 881) experience barriers in communication throughout life as they navigate both early conductive, and progressive sensorineural hearing loss amid other healthcare needs. Hearing loss is self-identified as one of the largest unmet healthcare needs. PURPOSE: The purpose of this study was to investigate the impact of treatment for hearing loss on communication confidence and quality of life measures for individuals with TS. RESEARCH DESIGN: We employed a prospective cross-sectional study design that included both online survey data and audiometric data for a subset of participants. STUDY SAMPLE: We recruited 179 adults with TS at the Turner Syndrome Society of the United States (TSSUS) Conference, and through a variety of regional TS organizations' social media platforms. Audiological data was collected onsite at the conference for a subset of 67 participants; 8 of which who were followed after receiving subsequent treatment with hearing aids. DATA COLLECTION AND ANALYSIS: The online survey design included demographic questions, the Communication Confidence Profile (CCP), and the RAND 36-Item Health Survey 1.0. Audiometric data included tympanometry, puretone air, and puretone bone conduction thresholds. Descriptive statistics, parametric, and non-parametric tests were used to analyze both survey and audiometric data. RESULTS: 74% of participants had a self-reported diagnosis of hearing loss, of which 61% were previously recommended amplification. Only 38% of participants reported using hearing aids. For those participants who wore hearing aids, Total CCP Score, 'Confidence in Ability to Hear Under Various Conditions', and 'Energy/Vitality' metrics were significantly greater than those with untreated hearing loss warranting a hearing aid. Collectively, Total CCP Score and 'Confidence in Ability to Hear Under Various Conditions' increased significantly when participants were fit with hearing aids. CONCLUSION: The results support previous data where hearing loss is a self-identified healthcare concern among women with Turner syndrome, yet many fail to receive appropriate hearing evaluation or treatment. Additionally, the use of hearing aids may improve communication confidence and quality of life in women with Turner syndrome. Furthermore, this study confirms the need for long-term audiological care and monitoring in women with Turner syndrome.

Does the Location of a Small Tympanic Membrane Perforation Affect the Degree of Hearing Loss in Adult Patients with Inactive Mucosal Chronic Suppurative Otitis Media?

BACKGROUND: Tympanic membrane perforation due to inactive mucosal chronic suppurative otitis media is a common problem in otolaryngology, with consequent conductive hearing loss. Still, there is controversy about the relationship between the location of the tympanic membrane perforation and the degree of hearing impairment. AIM OF THE STUDY: To assess the correlation between the location of a small tympanic membrane perforation and the degree of conductive hearing loss in adult patients with inactive mucosal chronic suppurative otitis media. PATIENTS AND METHODS: A prospective cross-sectional study enrolled 74 adult patients with small tympanic membrane perforations (perforation involves less than one quadrant of the tympanic membrane) and conductive hearing loss (airbone gap ≥ 20 dB HL) due to inactive mucosal chronic suppurative otitis media for at least 3 months. The locations of the tympanic membrane perforations were classified as anterosuperior, anteroinferior, posterosuperior, and poster inferior perforations. Audiometric analysis and a CT scan of the temporal bone were done for all patients. The means of the air and bone conduction pure tone hearing threshold averages at frequencies 500, 1000, 2000, and 4000 Hz were calculated, and consequently, the air-bone gaps were calculated and presented as means. The ANOVA test was used to compare the means of the air-bone gaps, and the Scheffe test was used to determine if there were statistically significant differences regarding the degree of conductive hearing loss in relation to different locations of the tympanic membrane perforation. RESULTS: The ages of the patients ranged from 20 to 43 years (mean = 31.9 ± 6.5 years), of whom 43 (58%) were females and 31 (42%) were males. The means of the air-bone gaps were 32.29 ± 5.41 dB HL, 31.34 ± 4.12 dB HL, 29.87 ± 3.48 dB HL, and 29.30 ± 4.60 dB HL in the posteroinferior, posterosuperior, anteroinferior, and anterosuperior perforations, respectively. Although the air-bone gap's mean was greater in the posteroinferior perforation, statistical analysis showed that it was insignificant (P-value=0.168). CONCLUSION: In adult patients with inactive chronic suppurative otitis media, the anteroinferior quadrant is the most common location of the tympanic membrane perforation, and there was an insignificant correlation between the location of a small tympanic membrane perforation and the degree of conductive hearing loss.

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.

Impact Of Sensorineural Hearing Loss On Subjective Tinnitus Quality In Patients With Bilateral Tinnitus.

INTRODUCTION: Tinnitus is a frequent condition that indicates the sensation of sound in the absence of a corresponding external stimulus and can significantly impair the quality of life. The main risk factor for developing tinnitus is hearing loss. The diagnosis of tinnitus is based on history, assessment of tinnitus severity, clinical examination, and audiological tests. The main purpose of this research was to examine the relationship between the presence and level of hearing loss and the characteristics of tinnitus in patients with bilateral subjective tinnitus. METHODS: Total number of 50 participants, 20 men, and 30 women were included in the research. Demographic data, data on hearing impairment obtained by tone audiometry, and data on difficulties caused by tinnitus obtained in two questionnaires - Tinnitus Handicap Inventory (THI) and Tinnitus Functional Index (TFI) were used. RESULTS: Age above 30 years is significantly associated with tinnitus with hearing loss. Hearing impairment is also significantly more often associated with an auditory TFI index >6.7, a total THI index >20, and an emotional THI index >3. Hearing loss was noted in 76% of patients. CONCLUSION: Tinnitus represents a significant burden for patients, therefore it is important to assess the impact of tinnitus on daily activities and quality of life.

The genetic basis and the diagnostic yield of genetic testing related to nonsyndromic hearing loss in Qatar.

Hearing loss is the most predominant sensory defect occurring in pediatrics, of which, 66% cases are attributed to genetic factors. The prevalence of hereditary hearing loss increases in consanguineous populations, and the prevalence of hearing loss in Qatar is 5.2%. We aimed to investigate the genetic basis of nonsyndromic hearing loss (NSHL) in Qatar and to evaluate the diagnostic yield of different genetic tests available. A retrospective chart review was conducted for 59 pediatric patients with NSHL referred to the Department of Adult and Pediatric Medical Genetics at Hamad Medical Corporation in Qatar, and who underwent at least one genetic test. Out of the 59 patients, 39 were solved cases due to 19 variants in 11 genes and two copy number variants that explained the NSHL phenotype. Of them 2 cases were initially uncertain and were reclassified using familial segregation. Around 36.8% of the single variants were in GJB2 gene and c.35delG was the most common recurrent variant seen in solved cases. We detected the c.283C > T variant in FGF3 that was seen in a Qatari patient and found to be associated with NSHL for the first time. The overall diagnostic yield was 30.7%, and the diagnostic yield was significantly associated with genetic testing using GJB2 sequencing and using the hearing loss (HL) gene panel. The diagnostic yield for targeted familial testing was 60% (n = 3 patients) and for gene panel was 50% (n = 5). Thus, we recommend using GJB2 gene sequencing as a first-tier genetic test and HL gene panel as a second-tier genetic test for NSHL. Our work provided new insights into the genetic pool of NSHL among Arabs and highlights its unique diversity, this is believed to help further in the diagnostic and management options for NSHL Arab patients.

Self-reporting of hearing loss and tinnitus in the diagnosis of ototoxicity by meglumine antimoniate in patients treated for American tegumentary Leishmaniasis.

INTRODUCTION: American Tegumentary Leishmaniasis (ATL) treatment is based on pentavalent antimonials (Sb5+), but these drugs have been associated to several adverse effects. Hearing loss and tinnitus during treatment with meglumine antimoniate (MA) have already been reported. This study aimed to describe the usefulness of self-reporting of hearing loss and tinnitus in diagnosing MA-induced ototoxicity. METHODS: A prospective longitudinal study was conducted with 102 patients with parasitological diagnosis of ATL, treated with different MA schemes. The presence of clinical auditory toxicity was defined as the emergence or worsening of self-reporting hearing loss and/or tinnitus during monitoring. Measures of sensitivity, specificity, and the positive and negative predictive value of the patient's self-reporting of hearing loss and tinnitus in relation to the result of the audiometric test (considered the gold standard) were calculated. RESULTS: The age of the evaluated patients ranged from 15 to 81 years, with a median of 41 years, and most were male (73.5%). Seventy-five patients (73.5%) had cutaneous leishmaniasis and 27 (26.5%) mucosal leishmaniasis. Eighty-six patients (84.3%) received intramuscular (IM) treatment and 16 (15.7%) were treated with intralesional MA. During treatment, 18 (17,6%) had tinnitus and 7 (6,9%) had complaint of hearing loss. 53 (52%) patients had cochlear toxicity confirmed by tone threshold audiometry and high frequency audiometry, from which 60% received a dose of 20 mg Sb5+/kg/day (p = 0.015) and 96.2% were treated with IM MA (p = 0.001). Tinnitus has greater specificity and positive predictive value than hearing loss, with a low number of false positives, but with a high false negative value. CONCLUSION: Although the large number of false negatives suggests that self-report of hearing loss or tinnitus cannot be considered a good screening test for referring the patient to an audiometry, the low number of false positives suggests the need to value the patient's complaint for referral. Otherwise, this study reinforces the importance of audiological monitoring during treatment with MA, especially in those patients with self-reporting of hearing loss or tinnitus when treated with 20 mg Sb5+/kg/day via IM.

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.

Changing Management of Intravestibular Schwannomas in the Era of Cochlear Implantation for Single-Sided Deafness.

OBJECTIVE: Intralabyrinthine schwannomas (ILSs) are a rare cause of deafness. Patients with ILS confined to the semicircular canals and the vestibule (intravestibular schwannomas) are potential candidates for cochlear implantation for hearing rehabilitation, a new option for patients with unilateral hearing loss since the 2019 FDA approval of cochlear implant (CI) for single-sided deafness. In this report, we describe an evolving management approach for ILSs causing hearing loss. PATIENTS: Adults (≥18 years) who underwent simultaneous ILS resection and CI between January 2019 and June 2023 (n = 3). INTERVENTION: Transmastoid labyrinthectomy with simultaneous cochlear implantation. MAIN OUTCOME MEASURES: Hearing performance with cochlear implantation measured as CNC Word Recognition scores and AzBio Sentence scores. RESULTS: Three patients with ILS confined to the semicircular canals and vestibule underwent simultaneous tumor resection via labyrinthectomy with CI placement. In all cases, complete tumor resection and full CI insertion were achieved. No patients experienced postoperative complications. Patients 1 and 2 underwent 6- and 9-month postactivation testing, respectively, with CNC scores 64% to 80% and AzBio 81% to 99% in the implanted ears. Patient 3 scored 0% on CNC and AzBio testing at 3 months and deferred her 6-month audiometry. CONCLUSIONS: Patients with ILS confined to the vestibule and semicircular canals can be considered for simultaneous tumor resection and CI placement.

Estimation and projection of the burden of hearing loss in China: findings from the Global Burden of Disease Study 2019.

OBJECTIVES: The comprehensive description of hearing loss in China and the shifting pattern remain unclear. We conducted the study to estimate the burden of hearing loss in China and project the trends from 2020 to 2034. STUDY DESIGN AND METHOD: Data on the disease burden of hearing loss were extracted from the Global Burden of Disease Study 2019. Estimated annual percentage changes (EAPCs) were calculated to quantify the trends of the age-standardized rates. Projections of hearing loss burden were made until 2034 using Bayesian age-period-cohort analysis. RESULTS: In China, prevalent cases of hearing loss increased from 224.4 million in 1990 to 426.5 million in 2019, representing an increase of 90.1 %. The age-standardized prevalence rate of hearing loss ranged from 22,592.8/100,000 in 1990-22,612.4/100,000 in 2019, with an EAPC of 0.003 %, representing a stable trend. Of the category of hearing loss, mild hearing loss accounted for the highest proportion, with 331.4 million people. More than 95 million people had moderate-to-complete hearing loss. Moreover, hearing loss was mostly attributable to age-related and other factors for adults and otitis media for children younger than 10 years. Based on the projection results, there will be 561 million people (40.1 % of the total population) have hearing loss by 2034. CONCLUSIONS: The prevalent cases of hearing loss in China substantially increased over the last 30 years. Over two in five Chinese people will have hearing loss by 2034, thus suggesting more solutions should be established to reduce the burden of hearing loss.

Can Mandarin-speaking prelingual deaf adults benefit from cochlear implant?

BACKGROUND: With advances in cochlear implant (CI) technology, prelingual deaf adults may experience improved speech perception and quality of life (QoL). It is still a challenge for Mandarin-speaking CI user with tone recognition due to CI technology focused on intonation language. OBJECTIVES: To evaluate the long-term post-CI auditory performance and social-emotional benefits in prelingual deaf Mandarin-speaking adults and the difference between them and post-lingual deaf adults. MATERIAL AND METHODS: Fifty-five adult implanted ears were included (forty-six postlingual deaf group; nine prelingual deaf group). Post-CI long-term outcomes were using vowels, consonants, disyllabic words, Mandarin monosyllable words, categories of audiology performance, speech intelligibility rating, subjective social-emotional questionnaires. RESULTS: Post-CI auditory performance and speech intelligibility of prelingual deafness adults was significantly inferior to that of those with postlingual deafness. However, both groups presented improved social-emotional benefits, with no significant difference between both groups. CONCLUSIONS: Adult CI recipients who deaf before the age of 4 can experience benefits in social-emotional life functioning, regardless of their limited auditory performance and speech intelligibility. Therefore, prelingual Mandarin-speaking deaf adults, especially those using oral communication, can be considered as relative indications for cochlear implantation. SIGNIFICANCE: To clarify and validate the benefits among Mandarin-speaking prelingual deaf adult recipients.

[Splicing mutations of GSDME cause late-onset non-syndromic hearing loss].

Objective:To dentify the genetic and audiological characteristics of families affected by late-onset hearing loss due to GSDMEgene mutations, aiming to explore clinical characteristics and pathogenic mechanisms for providing genetic counseling and intervention guidance. Methods:Six families with late-onset hearing loss from the Chinese Deafness Genome Project were included. Audiological tests, including pure-tone audiometry, acoustic immittance, speech recognition scores, auditory brainstem response, and distortion product otoacoustic emission, were applied to evaluate the hearing levels of patients. Combining with medical history and physical examination to analyze the phenotypic differences between the probands and their family members. Next-generation sequencing was used to identify pathogenic genes in probands, and validations were performed on their relatives by Sanger sequencing. Pathogenicity analysis was performed according to the American College of Medical Genetics and Genomics Guidelines. Meanwhile, the pathogenic mechanisms of GSDME-related hearing loss were explored combining with domestic and international research progress. Results:Among the six families with late-onset hearing loss, a total of 30 individuals performed hearing loss. The onset of hearing loss in these families ranged from 10 to 50 years（mean age: 27.88±9.74 years）. In the study, four splicing mutations of the GSDME were identified, including two novel variants: c. 991－7C>G and c. 1183+1G>T. Significantly, the c. 991－7C>G was a de novo variant. The others were previously reported variants: c. 991-1G>C and c. 991-15_991-13del, the latter was identified in three families. Genotype-phenotype correlation analysis revealed that probands with the c. 991－7C>G and c. 1183+1G>T performed a predominantly high-frequency hearing loss. The three families carrying the same mutation exhibited varying degrees of hearing loss, with an annual rate of hearing deterioration exceeding 0.94 dB HL/year. Furthermore, follow-up of interventions showed that four of six probands received intervention（66.67%）, but the results of intervention varied. Conclusion:The study analyzed six families with late-onset non-syndromic hearing loss linked to GSDME mutations, identifying four splicing variants. Notably, c. 991－7C>G is the first reported de novo variant of GSDME globally. Audiological analysis revealed that the age of onset generally exceeded 10 years,with variable effectiveness of interventions.

Analysis of characteristics of and risk factors for otological symptoms after COVID-19 infection.

The purpose of this study was to explore the characteristics of and risk factors for otological symptoms after contracting COVID-19. We invited 468 participants who had been infected with COVID-19 to participate in a survey. 310 (66.2%) were women and 158 (33.8%) were men. The mean age is 38.73 (12.21) years. The questionnaire included their basic information, symptoms and symptom duration after SARS-CoV-2 infection, number of vaccine doses received, and details regarding otological symptoms. In total, 106/468 (22.6%) participants experienced tinnitus, 66/468 (14.1%) hearing loss, 103/468 (22.0%) aural fullness, and 71/468 (15.2%) dizziness. Women were more prone to experience tinnitus (P = 0.022) and dizziness (P = 0.001) than men. The group with hearing loss were older (P = 0.025), and their initial COVID-19 symptoms lasted longer (P = 0.028) than those of patients without. Patients with aural fullness were more likely to experience fatigue than patients without (P = 0.002). Patients experiencing dizziness were more likely to experience pharyngalgia (P = 0.040) and fatigue (P = 0.005) than those without. The number of vaccine doses was positively associated with the resolution of otological symptoms (P = 0.035). Multiple logistic regression analysis revealed that sex was an independent risk factor for tinnitus (odds ratio [OR], 1.802; 95% confidence interval [CI], 1.099-2.953; P = 0.020), the duration of initial COVID-19 symptoms for hearing loss (OR, 1.055; 95% CI, 1.008-1.105; P = 0.023), and sex for dizziness (OR, 2.870; 95% CI, 1.489-5.535; P = 0.002). Sex, age, COVID-19-related fatigue, and the duration of initial COVID-19 symptoms may affect the occurrence of otological symptoms, and vaccines may aid their resolution.

Association between blood volatile organic aromatic compound concentrations and hearing loss in US adults.

OBJECTIVE: Benzene, ethylbenzene, meta/para-xylene, and ortho-xylene, collectively referred to as benzene, ethylbenzene, and xylene (BEX), constitute the main components of volatile organic aromatic compounds (VOACs) and can have adverse effects on human health. The relationship between exposure to BEX and hearing loss (HL) in the adult U.S. population was aimed to be assessed. METHODS: Cross-sectional data from the National Health and Nutrition Examination Survey (NHANES) for the years 2003-2004, 2011-2012, and 2015-2016 were analyzed. This dataset included complete demographic characteristics, pure-tone audiometry measurements, and volatile organic compound detection data from the NHANES database. A weighted multivariate logistic regression model was employed to investigate the associations between blood BEX concentrations HL, low-frequency hearing loss (SFHL), and high-frequency hearing loss (HFHL). RESULTS: 2174 participants were included, with weighted prevalence rates of HL, SFHL, and HFHL being 46.81%, 25.23%, and 45.86%, respectively. Exposure to benzene, ethylbenzene, meta/para-xylene, and ortho-xylene, and cumulative BEX concentrations increased the risk of hearing loss (odds ratios [ORs] were 1.36, 1.22, 1.42, 1.23, and 1.31, respectively; all P < 0.05). In the analysis with SFHL as the outcome, ethylbenzene, m-/p-xylene, o-xylene, benzene, and overall BEX increased the risk (OR 1.26, 1.21, 1.28, 1.20, and 1.25, respectively; all P < 0.05). For HFHL, exposure to ethylbenzene, m-/p-xylene, o-xylene, benzene, and overall BEX increased the risk (OR 1.36, 1.22, 1.42, 1.22, and 1.31, respectively; all P < 0.05). CONCLUSION: Our study indicated that a positive correlation between individual or cumulative exposure to benzene, ethylbenzene, meta/para-xylene, and ortho-xylene and the risk of HL, SFHL, and HFHL. Further research is imperative to acquire a more comprehensive understanding of the mechanisms by which organic compounds, notably BEX, in causing hearing loss and to validate these findings in longitudinal environmental studies.

Military and Nonmilitary TBI Associations with Hearing Loss and Self-Reported Hearing Difficulty among Active-Duty Service Members and Veterans.

OBJECTIVE: Identify associations between self-reported history of military and nonmilitary traumatic brain injury (TBI) on hearing loss and hearing difficulty from the Noise Outcomes in Servicemembers Epidemiology (NOISE) study. STUDY DESIGN: Cross-sectional. SETTING: Multi-institutional tertiary referral centers. PATIENTS: Four hundred seventy-three Active-Duty Service members (ADSM) and 502 veterans. EXPOSURE: Self-reported history of no TBI, military TBI only, nonmilitary TBI only, both military and nonmilitary TBI. MAIN OUTCOME MEASURES: Pure-tone hearing thresholds, Speech Recognition In Noise Test (SPRINT), Hearing Handicap Inventory for Adults (HHIA), and Speech, Spatial and Qualities of Hearing Scale (SSQ)-12. RESULTS: 25% (120/473) of ADSM and 41% (204/502) of veterans self-reported a TBI. Military TBI was associated with poorer hearing thresholds in all frequency ranges in veterans (adjusted mean difference, 1.8 dB; 95% confidence interval [CI], 0.5-3.0; 3.3, 0.8-5.8; 5.1; 1.7-8.5, respectively), and in the high frequency range in ADSM (mean difference, 3.2 dB; 95% CI, 0.1-6.3). Veterans with military TBI only and nonmilitary TBI only had lower odds of correctly identifying speech in noise than veterans with no TBI (odds ratio [OR], 0.78; 95% CI, 0.72-0.83; 0.90; 0.84-0.98). ADSM with a military TBI (OR, 5.7; 95% CI, 2.6-12.5) and veterans with any TBI history (OR, 2.5; 95% CI, 1.5-4.3; OR, 2.2; 95% CI, 1.3-3.8; OR, 4.5; 95% CI, 2.1-9.8) were more likely to report hearing difficulty on HHIA. SSQ-12 results corroborated HHIA findings. CONCLUSIONS: Military TBI was associated with poorer hearing thresholds in veterans and ADSM, and poorer SPRINT scores in veterans. Military TBI was associated with poorer self-perceived hearing ability in ADSM. All types of TBI were associated with poorer self-perceived hearing ability in veterans, although the strength of this association was greatest for military TBI.

Audiological Manifestations in Patients with Granulomatosis with Polyangiitis.

Background and Objectives: Granulomatosis with Polyangiitis (GPA) is a rare, autoimmune, multisystemic disease characterized by vasculitis and necrotizing granuloma that commonly affects the upper and lower respiratory tract and kidneys. Audiovestibular dysfunction in GPA diseases may have different clinical presentations. The aim of the present study was to evaluate hearing function in patients with GPA and to compare the results with a healthy control group. Materials and Methods: A total of 34 individuals participated in the study. The GPA group consisted of 14 participants, and the control group was composed of 20 healthy participants with no signs or symptoms of ear disease. The ages ranged from 18 to 65 years old, with a mean age of 43.8 years. The participants underwent a complete audiological evaluation using otoscopy, impedance audiometry, pure tone audiometry, speech audiometry-evaluation of speech thresholds, and speech recognition in quiet. Both ears were tested. All of the participants of the study were native Lithuanian speakers. Data were statistically analyzed using the Statistical Analysis System software SAS® Studio 3.8. A p value < 0.05 was regarded as statistically significant. Results: 92.85% of patients from the GPA group reported hearing-related symptoms: hearing loss, tinnitus, and fullness in the ears. The arithmetic means of all hearing thresholds at frequencies from 125 Hz to 8000 Hz were significantly higher in the GPA group. The results revealed statistically significant differences between the two groups in the Speech Detection Threshold, Speech Recognition Threshold, Speech Discomfort level, and Word Recognition Scores. Conclusions: The frequency of hearing loss, the average hearing thresholds, and speech thresholds were higher in GPA patients than in healthy individuals. The most common type of hearing loss was sensorineural. Audiological assessments should be considered during the routine evaluation of patients with GPA disease to prevent hearing-related disabilities.