Hearing loss in beta-thalassaemia: An Italian multicentre case-control study.

BACKGROUND: Despite numerous studies, the true scenario of hearing loss in beta-thalassaemia remains rather nebulous. MATERIALS AND METHODS: Pure tone audiometry, chelation therapy, demographics and laboratory data of 376 patients (mean age 38.5 ± 16.6 years, 204 females, 66 non-transfusion-dependent) and 139 healthy controls (mean age 37.6 ± 17.7 years, 81 females) were collected. RESULTS: Patient and control groups did not differ for age (p = 0.59) or sex (p = 0.44). Hypoacusis rate was higher in patients (26.6% vs. 7.2%; p < 0.00001), correlated with male sex (32.6% in males vs. 21.8% in females; p = 0.01) and it was sensorineural in 79/100. Hypoacusis rate correlated with increasing age (p = 0.0006) but not with phenotype (13/66 non-transfusion-dependent vs. 87/310 transfusion-dependent patients; p = 0.16). Sensorineural-notch prevalence rate did not differ between patients (11.4%) and controls (12.2%); it correlated with age (p = 0.01) but not with patients' sex or phenotype. Among adult patients without chelation therapy, the sensorineural hypoacusis rate was non-significantly lower compared to chelation-treated patients while it was significantly higher compared to controls (p = 0.003). CONCLUSIONS: Sensorineural hypoacusis rate is high in beta-thalassaemia (about 21%) and it increases with age and in males while disease severity or chelation treatment seems to be less relevant. The meaning of sensorineural-notch in beta-thalassaemia appears questionable.

Dispersed DNA variants underlie hearing loss in South Florida's minority population.

BACKGROUND: We analyzed the genetic causes of sensorineural hearing loss in racial and ethnic minorities of South Florida by reviewing demographic, phenotypic, and genetic data on 136 patients presenting to the Hereditary Hearing Loss Clinic at the University of Miami. In our retrospective chart review, of these patients, half self-identified as Hispanic, and the self-identified racial distribution was 115 (86%) White, 15 (11%) Black, and 6 (4%) Asian. Our analysis helps to reduce the gap in understanding the prevalence, impact, and genetic factors related to hearing loss among diverse populations. RESULTS: The causative gene variant or variants were identified in 54 (40%) patients, with no significant difference in the molecular diagnostic rate between Hispanics and Non-Hispanics. However, the total solve rate based on race was 40%, 47%, and 17% in Whites, Blacks, and Asians, respectively. In Non-Hispanic Whites, 16 different variants were identified in 13 genes, with GJB2 (32%), MYO7A (11%), and SLC26A4 (11%) being the most frequently implicated genes. In White Hispanics, 34 variants were identified in 20 genes, with GJB2 (22%), MYO7A (7%), and STRC-CATSPER2 (7%) being the most common. In the Non-Hispanic Black cohort, the gene distribution was evenly dispersed, with 11 variants occurring in 7 genes, and no variant was identified in 3 Hispanic Black probands. For the Asian cohort, only one gene variant was found out of 6 patients. CONCLUSION: This study demonstrates that the diagnostic rate of genetic studies in hearing loss varies according to race in South Florida, with more heterogeneity in racial and ethnic minorities. Further studies to delineate deafness gene variants in underrepresented populations, such as African Americans/Blacks from Hispanic groups, are much needed to reduce racial and ethnic disparities in genetic diagnoses.

Association between Chronic Obstructive Pulmonary Disease and Hearing Loss with Impaired Speech Recognition: A Cross-Sectional Study.

INTRODUCTION: Studies have identified a greater risk of sensory neural hearing loss in individuals with chronic obstructive pulmonary disease (COPD) compared to healthy individuals, but it is unclear whether they are at increased risk of hearing loss with impaired speech recognition. The aim of this study was to assess whether COPD is associated with hearing loss that affects speech recognition. METHODS: This is a case-control study. We screened individuals from health facilities in the municipality of Jundiai. We enrolled a test group of individuals with COPD and an age-matched control group composed of individuals with asthma. The selected individuals attended an appointment with a chest physician, responded to questionnaires, and underwent tonal and speech audiometry. Adjusted binary logistic regression analysis evaluated whether COPD was associated with reduced speech recognition. RESULTS: We enrolled 36 individuals with COPD and 72 with asthma. Individuals with COPD were more likely to have a reduced speech recognition compared to asthmatic individuals (reduced recognition of three-syllable words: adjusted OR 3.72, 95 CI [1.38-10.02]) (reduced recognition of monosyllable words: adjusted OR 4.74, 95 CI [1.52-14.76]). CONCLUSION: We conclude that individuals with COPD from primary and secondary healthcare facilities have at least 38% greater risk of hearing loss with reduced speech recognition compared to an age-matched control group of individuals with asthma recruited from the same facilities. We recommend that longitudinal studies evaluate whether regular screening could contribute to the prevention or early treatment of hearing loss in individuals with moderate-severe COPD.

Patient characteristics of pediatric inpatients with sensorineural hearing loss: Insights from the 2016 KID database.

This study examines how patient demographics impact pediatric sensorineural hearing loss (SNHL) prevalence using the 2016 Kids' Inpatient Database (KID). By analyzing age, gender, race, income, insurance, and region, the study provides insights for early intervention and diagnosis. Multivariate regression analysis reveals associations between these factors and SNHL occurrence. OBJECTIVE: This study aims to assess the influence of patient demographics on pediatric SNHL prevalence, offering guidance for early detection and intervention strategies. STUDY DESIGN: Using 2016 KID data, this retrospective analysis investigates how patient factors like age, gender, race, income, insurance, and region relate to SNHL prevalence. Multivariate regression is employed to control for potential confounders. METHODS: Data from 6,266,285 patient discharges, including 9997 hearing loss cases, are analyzed. SNHL prevalence is calculated, and demographic variables are examined. Weighted odds ratios and multivariate regression are used to assess associations. RESULTS: The study finds an overall SNHL prevalence of 101.67 cases per 100,000 discharges in 2016. Non-Hispanic White patients show the highest prevalence. Black patients are more likely to be diagnosed, while Hispanic patients are less likely. Multivariate analysis highlights race, income, insurance, region, age, and sex as significant confounders. CONCLUSION: This study underscores the impact of patient demographics on pediatric SNHL prevalence. Factors like race, income, insurance, and region play a role. These findings aid in risk assessment, early identification, and tailored interventions. Further research can uncover socioeconomic disparities and underlying mechanisms.

Late-onset, progressive sensorineural hearing loss in the paediatric population: a systematic review.

PURPOSE: To review possible risk factors for permanent delayed-onset, progressive sensorineural hearing loss (SNHL) in the paediatric population to recommend follow-up protocols for early detection. METHODS: PRISMA-compliant systematic review was performed, including observational studies on the paediatric population up to 16 years old who have passed the newborn hearing screening programme (NHSP), investigating the development of late-onset, progressive SNHL. Electronic searches were performed through Medline, Embase, Cochrane, and Emcare. RESULTS: 37 studies were included. 21 showed an association between late-onset SNHL and congenital cytomegalovirus (cCMV) infection (age at hearing loss diagnosis 0.75 to 204 months, mean 45.6 ± 43.9), while 16 between late-onset SNHL and other congenital or perinatal factors, namely Neonatal Intensive Care Unit (NICU) stay, prematurity, neonatal respiratory failure, mechanical ventilation, extracorporeal membrane oxygenation (ECMO) support, hypocapnia, hypoxia, alkalosis, seizure activity, congenital diaphragmatic hernia (CDH), inner ear malformation, and gene mutations (age at hearing loss diagnosis 2.5 to 156 months, mean 38.7 ± 40.7). CONCLUSIONS: cCMV infection may cause late-onset SNHL, which can be missed on standard NHSP. There is, therefore, evidence to support universal screening programmes to enable detection in even asymptomatic neonates. Ongoing audiological follow-up for all children with cCMV is advisable, to enable timely treatment. In the paediatric population presenting conditions such as NICU stay > 5 days, prematurity ≤ 34 weeks gestation, severe neonatal respiratory failure, mechanical ventilation, ECMO support, and CDH surgery, an audiological follow-up from 3 months of age up to at least 3-4 years of age, and at least annually, should be recommended.

Prevalence of perilymphatic fistula in patients with sudden-onset sensorineural hearing loss as diagnosed by Cochlin-tomoprotein (CTP) biomarker detection: its association with age, hearing severity, and treatment outcomes.

PURPOSE: To determine the prevalence of perilymphatic fistula (PLF) in sudden-onset sensorineural hearing loss (SSNHL) patients by employing the Cochlin-tomoprotein (CTP) detection test, a specific diagnostic marker for perilymph. We also analyzed the clinical characteristics associated with hearing outcomes in this cohort. METHODS: A total of 74 eligible patients were prospectively enrolled. Following myringotomy, middle ear lavage (MEL) samples underwent the CTP test to identify perilymph leakage. Intratympanic dexamethasone (IT-DEX) therapy was administered, and hearing outcomes were assessed. Control groups comprised patients with chronic otitis media (n = 40) and non-inflammatory middle ears (n = 51) with concurrent MEL sample collection. RESULTS: CTP was positive in 16 (22%) patients. No control samples showed positive results. Multiple regression analysis indicated that age and pre-treatment hearing levels significantly contributed to the CTP value. We found a positive correlation between CTP values, age, and pre-treatment pure-tone averages. Notably, CTP values in SSNHL cases aged 60 and above were significantly higher than in those below 60 years. Patients with positive CTP had significantly worse recovery rates after IT-DEX treatment. CONCLUSION: This study is the first prospective investigation demonstrating a positive relationship between CTP values, age, and hearing severity in SSNHL, indicating that PLF might be the essential cause of SSNHL, particularly in the elderly. Our findings suggest that IT-DEX may be less effective for PLF-associated SSNHL. Future research could reveal that PLF repair surgery is a viable treatment strategy for SSNHL. This study was registered under the UMIN Clinical Trials Registry (UMIN000010837) on 30/May/2013.

Audiovestibular Involvement in Patients With Systemic Sclerosis.

INTRODUCTION: Audiovestibular dysfunction has been reported in many autoimmune connective tissue diseases, including systemic sclerosis (SSc). OBJECTIVE: To assess the prevalence and features of audiological and vestibular disturbances in SSc patients and evaluate their relationship with disease duration, clinical features, nailfold videocapillaroscopy pattern, and immunologic profiles. METHOD: A matched case-control study was conducted in a rheumatology clinic of a second-level hospital over 24 months. All patients underwent a detailed ear, nose, and throat examination, as well as audiometric and vestibular assessments, including pure tone audiometry, speech audiometry, immittance tests, and the Video Head Impulse Test. RESULTS: Thirty-five SSc patients and 24 healthy controls were included in the study. In the SSc group, subjective hearing loss was reported by 17.1% of patients, vertigo by 14.3%, tinnitus by 11.4%, and dizziness by 5.7%. Sensorineural hearing loss was identified in 42.9% of SSc patients, significantly higher than in the control group ( p = 0.013). There was no correlation between audiological manifestations and clinical symptoms, organ involvement, immunologic characteristics, and treatment. Vestibular dysfunction was detected in 60% of SSc patients, significantly higher than the control group ( p = 0.05). A significant correlation was found between abnormal Video Head Impulse Test and the presence of anti-RNA polymerase III and anti-Th/To antibodies ( p = 0.05 and p = 0.034, respectively). CONCLUSION: Our study revealed an increased prevalence of sensorineural hearing loss and vestibulopathy in SSc patients.

Demographic analysis of hearing impairment based on various parameters in patients with cochlear implant.

Objectives: To analyse the demographic and clinical variables in children having undergone cochlear implant surgery because of deafness. METHODS: The cross-sectional study was conducted from January to November 2022 at the Centre for Research in Experimental and Applied Medicine laboratory of the Department of Biochemistry and Molecular Biology, Army Medical College, Rawalpindi, Pakistan, in collaboration with the Ear, Nose and Throat Department of Combined Military Hospital, Rawalpindi, and comprised children of eith gender aged up to 10 years who had received cochlear implant. Data was collected through questionnaire-based detailed interviews. Syndromic Hearing Loss, Non-Syndromic Hearing Loss, and Acquired Hearing Loss were identified among the subjects. Data was analysed using SPSS 22. RESULTS: Of the 250 cases, 147(58.8%) were boys, 146(58.4%) were aged 0-5 years, 219(87.6%) had prelingual onset of disease, and 202(80.8%) had a non-progressive disease course. In 203(81.2%) cases, normal developmental milestones were seen. Parental consanguinity was observed in 219(87.6%) cases. However, 63(25.2%) patients had a first-degree relative who had a history of deafness. In 170(68%) cases, hearing loss was hereditary, whereas in 80(32%) it was acquired. Meningitis was the most commonly identified risk factor 55(68.75%). Acquired risk factors and family history had significant association with hearing loss (p<0.05). Speech perception significantly improved in all 219(100%) patients with prelingual hearing loss who underwent cochlear implantation. CONCLUSIONS: Majority of the cases were found to be male, had a prelingual disease onset and a non-progressive disease course. Family history was a significant factor, while meningitis was the most common acquired cause of hearing loss.

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.

[ENT manifestations of congenital cytomegalovirus infection]. / Les atteintes ORL de l'infection ­congénitale à cytomégalovirus.

Cytomegalovirus is the most common cause of congenital infection worldwide. 90 % of children infected in utero are born without symptoms, but 15 % of them will develop disorders within the first five years of life. The most common disorders affect the inner ear, resulting in sensorineural hearing loss and/or vestibular dysfunction (VD). VD is often unrecognized and confused with conditions -affecting the central nervous system. It can cause delays in psychomotor development and predispose to overall developmental delay. Early diagnosis and treatment are essential to prevent or limit these sequelae. Antiviral treatment during the pre- and neonatal periods should be considered.

Le cytomégalovirus est la cause la plus fréquente d'infection congénitale dans le monde. 90 % des enfants infectés in utero naissent sans symptôme, mais 15 % d'entre eux vont développer des atteintes au cours des cinq premières années de vie. Les plus fréquentes touchent l'oreille interne, engendrant une­surdité neurosensorielle et/ou une dysfonction vestibulaire (DV). La DV est souvent méconnue et confondue avec des atteintes du système nerveux central. Elle peut provoquer des retards du ­développement psychomoteur et prédisposer à un retard global du développement. Un diagnostic et une prise en charge précoces sont essentiels pour prévenir ou limiter ces séquelles. Un traitement antiviral en période pré et néonatale doit être considéré.

[Endothelial dysfunction as a pathogenetic factor of sensorineural hearing loss]. / Endotelial'naya disfunktsiya kak patogeneticheskii faktor sensonevral'noi tugoukhosti.

Chronic sensorineural hearing loss (SNHL) is a common disease that leads to disability of the population. Despite the many reports devoted to SNHL, the question of the pathogenesis of the disease is still open. Many researchers consider the development of SNHL as a manifestation of microangiopathy. The mechanism of development of microangiopathy in SNHL is multifactorial, but most researchers agree that endothelial dysfunction (ED) triggers a complex of pathological changes in the vessels of the inner ear. OBJECTIVE: Review of the results of scientific research in recent years on the problem of etiopathogenesis of sensorineural hearing loss from the perspective of endothelial dysfunction in the formation of auditory disorders.

[Assessment of the risk of hearing loss in children with cystic fibrosis]. / Otsenka riska patologii slukha pri mukovistsidoze u detei.

BACKGROUND: Cystic fibrosis (CF) is a severe hereditary disease with a multisystem lesion. Manifestations of CF include severe infectious purulent lesions of all parts of the respiratory tract, including purulent rhinosinusitis with nasal polyps. The involvement of the sinonasal region and the need for systemic use of ototoxic drugs (primarily aminoglycosides to treat resistant bacterial infection) potentially create a risk of both conductive and sensorineural hearing loss (SNHL). The available data on the epidemiology of hearing disorders in CF is contradictory. Currently, genetic determinants of the development of aminoglycoside SNHL have been identified. MATERIAL AND METHODS: For 136 CF patients (75 girls, 61 boys) aged 3 to 17 (9.4±3.9) years were performed audiological examination: tympanometry, transient-evoked otoacoustic emission and the pure tone threshold audiometry (standard frequency range) (n=126). History of systemic therapy with aminoglycosides was evaluated for each patient. Sequencing of c.35delG mutations in the GJB2 gene (nuclear DNA) and A1555G in the 12S rRNA gene (mitochondrial DNA) was performed in 215 patients with cystic fibrosis (the group partially overlaps with the audiological group), and as a control - 106 children with bronchial asthma and 103 healthy children, their age ranged from 3 to 17 (8.8±3.8) years. RESULTS: Audiological examination of CF children reveled a prevalence of conductive hearing loss comparable to the general population (2.4%). The frequency of SNHL was 1.6%, wich exceeds that of non-CF children. A genetic study revealed one case of heterozygous carriage of the c.35delG mutation in the GJB2 gene in a patient with bronchial asthma. In the group of patients with CF (n=215), mutations in the connexin 26 gene were not detected. No A1555G mutation was detected either in the group of patients with CF or in the control groups. CONCLUSIONS: Children with CF are at risk for the development of sensorineural, but not conductive hearing loss. Routine total screening for A1555G and c.35delG mutations probably seems not to be recommended.

Association between air pollution and sudden sensorineural hearing loss (SSHL): A systematic review and meta-analysis.

Recent studies have indicated that air pollution (AP) has harmful effects on hearing and ear diseases such as Sudden Sensorineural Hearing Loss (SSHL). The purpose of this study was to evaluate the impact of exposure to AP on SSHL incidence. Valid electronic databases were searched to retrieve studies published until December 1, 2022, using appropriate keywords. The result of the search was 1146 studies, and after screening according to the defined criteria, in total 8 studies were obtained. The risk of bias (ROB) in the studies and their quality were assessed. Finally, the meta-analysis with a significance level of 5% was performed. The findings revealed that the mean level of SO2, CO, NO2, and PM10 in the patient group was more than that of the control group, and p-values were 0.879, 0.144, 0.077, and 0.138, respectively. There was an indirect relation between air pollutants and SSHL, and PM2.5 showed a significant effect (p < 0.05). Given the limited research and the use of different statistical methods, more research is suggested to confirm this association and to determine the mechanisms by which AP exposure may cause SSHL.

Rising of LOXHD1 as a signature causative gene of down-sloping hearing loss in people in their teens and 20s.

BACKGROUND: Down-sloping sensorineural hearing loss (SNHL) in people in their teens and 20s hampers efficient learning and communication and in-depth social interactions. Nonetheless, its aetiology remains largely unclear, with the exception of some potential causative genes, none of which stands out especially in people in their teens and 20s. Here, we examined the role and genotype-phenotype correlation of lipoxygenase homology domain 1 (LOXHD1) in down-sloping SNHL through a cohort study. METHODS: Based on the Seoul National University Bundang Hospital (SNUBH) genetic deafness cohort, in which the patients show varying degrees of deafness and different onset ages (n=1055), we have established the 'SNUBH Teenager-Young Adult Down-sloping SNHL' cohort (10-35 years old) (n=47), all of whom underwent exome sequencing. Three-dimensional molecular modelling, minigene splicing assay and short tandem repeat marker genotyping were performed, and medical records were reviewed. RESULTS: LOXHD1 accounted for 33.3% of all genetically diagnosed cases of down-sloping SNHL (n=18) and 12.8% of cases in the whole down-sloping SNHL cohort (n=47) of young adults. We identified a potential common founder allele, as well as an interesting genotype-phenotype correlation. We also showed that transcript 6 is necessary and probably sufficient for normal hearing. CONCLUSIONS: LOXHD1 exceeds other genes in its contribution to down-sloping SNHL in young adults, rising as a signature causative gene, and shows a potential but interesting genotype-phenotype correlation.

The association of genetic polymorphisms in protocadherin 15 with sudden sensorineural hearing loss in a Chinese population.

BACKGROUND: Sudden sensorineural hearing loss (SSNHL) is a multifactorial disease, and its etiology is still unknown. SSNHL may be caused by environmental factors and genetic changes. PCDH15 is associated with susceptibility to hearing loss. The relationship between PCDH15 and SSNHL remains unknown. METHODS: In this study, the potential association between PCDH15 polymorphism and SSNHL in Chinese population was evaluated. Two single nucleotide polymorphisms PCDH15-rs7095441 and rs11004085 in 195 SSNHL patients and 182 healthy controls were determined by TaqMan technology. RESULTS: In Chinese population, the TT genotype and T allele of rs7095441 are associated with increased susceptibility to SSNHL. The relationships between rs7095441 and the degree of hearing loss were analyzed, and TT genotype increased the risk of hearing loss. Among SSNHL patients, patients with TT genotype of rs7095441 have an increased risk of vertigo. CONCLUSION: This study found that the TT genotype of SNP rs7095441 can increase the risk of SSNHL in Chinese population.

Prevalence and Clinical Characteristics of Mitochondrial DNA Mutations in Korean Patients With Sensorineural Hearing Loss.

BACKGROUND: Mutations in mitochondrial DNA (mtDNA) are associated with several genetic disorders, including sensorineural hearing loss. However, the prevalence of mtDNA mutations in a large cohort of Korean patients with hearing loss has not yet been investigated. Thus, this study aimed to investigate the frequency of mtDNA mutations in a cohort of with pre- or post-lingual hearing loss of varying severity. METHODS: A total of 711 Korean families involving 1,099 individuals were evaluated. Six mitochondrial variants associated with deafness (MTRNR1 m.1555A>G, MTTL1 m.3243A>G, MTCO1 m.7444G>A and m.7445A>G, and MTTS1 m.7471dupC and m.7511T>C) were screened using restriction fragment length polymorphism. The prevalence of the six variants was also analyzed in a large control dataset using whole-genome sequencing data from 4,534 Korean individuals with unknown hearing phenotype. RESULTS: Overall, 12 of the 711 (1.7%) patients with hearing loss had mtDNA variants, with 10 patients from independent families positive for the MTRNR1 m.1555A>G mutation and 2 patients positive for the MTCO1 m.7444G>A mutation. The clinical characteristics of patients with the mtDNA variants were characterized by post-lingual progressive hearing loss due to the m.1555A>G variant (9 of 472; 1.9%). In addition, 18/4,534 (0.4%) of the Korean population have mitochondrial variants associated with hearing loss, predominantly the m.1555A>G variant. CONCLUSION: A significant proportion of Korean patients with hearing loss is affected by the mtDNA variants, with the m.1555A>G variant being the most prevalent. These results clarify the genetic basis of hearing loss in the Korean population and emphasize the need for genetic testing for mtDNA variants.

Association of Psoriasis With Hearing Loss: A Systematic Review and Meta-Analysis.

BACKGROUND: The relation between psoriasis and hearing loss has been unclear. OBJECTIVE: To investigate the association of psoriasis with hearing loss. METHODS: We searched MEDLINE and Embase on 12th November 2022 for studies on the association between psoriasis and hearing loss. We conducted a random-effects model meta-analysis to calculate pooled mean difference (MD) in the pure tone thresholds, pooled odds ratio for sensorineural hearing loss, and pooled hazard ratio for sudden sensorineural hearing loss related to psoriasis. RESULTS: We included 12 case-control/cross-sectional and 3 cohort studies with 202,683 subjects. Psoriasis was associated with hearing loss at 500 Hz (pooled MD 2.21, 95% CI (CI) 0.13 to 4.29), 1000 Hz (pooled MD 2.97, 95% CI 1.01 to 4.93), 2000 Hz (pooled MD 5.13, 95% CI 2.45 to 7.82), 4000 Hz (pooled MD 9.3, 95% CI 5.1 to 13.51), and 6000 Hz (pooled MD 11.04, 95% CI 5.05 to 17.03). Patients with psoriasis had increased odds for sensorineural hearing loss (pooled odds ratio 3.85, 95% CI 1.07-13.9) and risk for sudden sensorineural hearing loss (pooled hazard ratio 1.45; 95% CI 1.22-1.71). CONCLUSION: Psoriasis is associated with hearing loss, especially at high frequencies.

Is intrauterine exposure to COVID-19 infection a risk factor for infant hearing loss?

PURPOSE: To determine whether exposure to intrauterine COVID-19 infection causes congenital or late-onset hearing loss in infants. MATERIAL AND METHOD: The hearing screening results of infants born in a tertiary hospital between March 2020 and April 2022 with and without a history of intrauterine exposure to COVID-19 infection (36 infants each) were retrospectively analyzed within one month after birth in all infants and additionally at six months after intrauterine COVID-19 infection exposure in the study group. The automated auditory brainstem response (AABR) test was used for the hearing evaluation. RESULTS: The polymerase chain reaction test was negative in study group exposed to intrauterine COVID-19 infection. The number of infants admitted to the intensive care unit (ICU), and the length of ICU stay were significantly higher in this group (p < 0.01). Six infants (16.6 %) in the study group failed the first AABR test bilaterally, but five of these infants passed the second AABR test. A bilateral severe sensorineural hearing loss was detected in one infant (2.77 %). All the infants in the study group underwent the AABR test again at six months, and all infants, except this infant, passed the test. In the control group, five infants (13.88 %) failed the first AABR test bilaterally, but they all passed the second test. CONCLUSIONS: Exposure to COVID-19 infection in the intrauterine period does not cause congenital or late-onset hearing loss (within six months) in infants; therefore, gestational COVID-19 infection is not a risk factor for infant hearing loss.

Characterization of otologic symptoms appearing after COVID-19 vaccination.

OBJECTIVES: Anecdotal reports of sudden sensorineural hearing loss (SSNHL) following COVID-19 vaccination have emerged in the otolaryngology community. Studies have demonstrated no association between COVID-19 vaccination and SSNHL. We aim to characterize the spectrum of otologic symptoms following COVID-19 vaccination. METHODS: A cross-sectional study of patients seen in the otology clinic at an academic center was performed. Patients completed a questionnaire on the development of new otologic symptoms within 4 weeks of COVID-19 vaccination. Diagnostic and audiometric data was collected retrospectively for patients reporting otologic symptoms. RESULTS: Between May and July 2021, 500 patients were screened. Median age was 56.6 years old, with 59.4 % female and 40.2 % male. 420 patients (84.0 %) were vaccinated, with 58.4 % receiving Pfizer, 29.1 % receiving Moderna, and 3.8 % receiving Johnson & Johnson. 61 patients (14.5 %) reported one or more otologic symptoms within 4 weeks of vaccination, including 21 (5.0 %) with hearing loss, 26 (6.2 %) with tinnitus, 33 (7.9 %) with dizziness, and 19 (4.5 %) with vertigo. Of the 16 patients (3.2 %) reporting tinnitus with no associated hearing loss, 8 were diagnosed with subjective tinnitus and 4 were diagnosed with temporomandibular joint syndrome. Of the 18 patients reporting hearing loss, 11 had exacerbations of underlying pathologies (e.g. Meniere's disease, presbycusis) and 7 were newly diagnosed with SSNHL (1.4 %). CONCLUSIONS: Patients reporting otologic symptoms following COVID-19 vaccination received various diagnoses of uncertain etiology. The incidence of SSNHL in these patients is comparable to the general otology patient population. Additional studies are required to determine the incidence of specific diagnoses following vaccination.

Epidemiology of idiopathic sudden sensorineural hearing loss in the era of big data.

OBJECTIVES: Idiopathic sudden sensorineural hearing loss (ISSNHL) is a medical emergency, and delayed treatment can have permanent sequelae. However, the etiology of ISSNHL is diverse and unclear; thus, it is idiopathic. To develop an insight into this condition, patients with ISSNHL must be clearly identified. We propose an operational definition for the unambiguous identification of ISSNHL patients. Patients are identified through suggested definitions, and prevalence and general information are investigated. METHODS: A retrospective study of patients with ISSNHL was performed using the Health Insurance and Review Assessment-National Patient Sample from 2009 to 2016. To present a new operational definition, a systematic review was conducted for studies on ISSNHL from January 2007 to June 2021. After constructing several operant definitions using the conditions that can specify patients with ISSNHL in big data, we compared each definition to propose an operational definition. RESULTS: The important conditions required to classify patients with ISSNHL using big data were the International Classification of Diseases (ICD)-10 code, number of pure tone audiometry (PTA) tests, and whether steroids were prescribed. Among them, those who had undergone PTA tests more than twice could be clearly identified as patients with ISSNHL. CONCLUSION: As the use of big data becomes smoother, research using national medical data is being conducted; however, the results of the studies may vary depending on how a patient with ISSNHL is classified. Clear identification of patients with ISSNHL will be beneficial for better management of this condition.