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.

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.

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 Sudden Sensorineural Hearing Loss After COVID-19 Vaccination.

IMPORTANCE: Emerging reports of sudden sensorineural hearing loss (SSNHL) after COVID-19 vaccination within the otolaryngological community and the public have raised concern about a possible association between COVID-19 vaccination and the development of SSNHL. OBJECTIVE: To examine the potential association between COVID-19 vaccination and SSNHL. DESIGN, SETTING, AND PARTICIPANTS: This cross-sectional study and case series involved an up-to-date population-based analysis of 555 incident reports of probable SSNHL in the Centers for Disease Control and Prevention Vaccine Adverse Events Reporting System (VAERS) over the first 7 months of the US vaccination campaign (December 14, 2020, through July 16, 2021). In addition, data from a multi-institutional retrospective case series of 21 patients who developed SSNHL after COVID-19 vaccination were analyzed. The study included all adults experiencing SSNHL within 3 weeks of COVID-19 vaccination who submitted reports to VAERS and consecutive adult patients presenting to 2 tertiary care centers and 1 community practice in the US who were diagnosed with SSNHL within 3 weeks of COVID-19 vaccination. EXPOSURES: Receipt of a COVID-19 vaccine produced by any of the 3 vaccine manufacturers (Pfizer-BioNTech, Moderna, or Janssen/Johnson & Johnson) used in the US. MAIN OUTCOMES AND MEASURES: Incidence of reports of SSNHL after COVID-19 vaccination recorded in VAERS and clinical characteristics of adult patients presenting with SSNHL after COVID-19 vaccination. RESULTS: A total of 555 incident reports in VAERS (mean patient age, 54 years [range, 15-93 years]; 305 women [55.0%]; data on race and ethnicity not available in VAERS) met the definition of probable SSNHL (mean time to onset, 6 days [range, 0-21 days]) over the period investigated, representing an annualized incidence estimate of 0.6 to 28.0 cases of SSNHL per 100 000 people per year. The rate of incident reports of SSNHL was similar across all 3 vaccine manufacturers (0.16 cases per 100 000 doses for both Pfizer-BioNTech and Moderna vaccines, and 0.22 cases per 100 000 doses for Janssen/Johnson & Johnson vaccine). The case series included 21 patients (mean age, 61 years [range, 23-92 years]; 13 women [61.9%]) with SSNHL, with a mean time to onset of 6 days (range, 0-15 days). Patients were heterogeneous with respect to clinical and demographic characteristics. Preexisting autoimmune disease was present in 6 patients (28.6%). Of the 14 patients with posttreatment audiometric data, 8 (57.1%) experienced improvement after receiving treatment. One patient experienced SSNHL 14 days after receiving each dose of the Pfizer-BioNTech vaccine. CONCLUSIONS AND RELEVANCE: In this cross-sectional study, findings from an updated analysis of VAERS data and a case series of patients who experienced SSNHL after COVID-19 vaccination did not suggest an association between COVID-19 vaccination and an increased incidence of hearing loss compared with the expected incidence in the general population.

Chronic kidney disease is associated with increased risk of sudden sensorineural hearing loss and Ménière's disease: a nationwide cohort study.

Several studies have demonstrated the harmful effects of chronic kidney disease (CKD) on the audiovestibular system. Through a time-to-event analysis, we aimed to compare the association of CKD with sudden sensorineural hearing loss (SSNHL), and Ménière's disease against a control population without CKD. We used a total of 1,025,340 patients from the Korean National Health Insurance Service database from 2002 to 2013. The CKD group (n = 2572) included patients diagnosed with CKD more than three times between January 2003 and December 2005. The non-CKD group (n = 5144) consisted of two patients without CKD for every patient with CKD. Each patient was monitored until December 2013. We calculated the incidence, survival rate, and hazards ratio (HR) of SSNHL and Ménière's disease. In the CKD group, the incidence of SSNHL and Ménière's disease was 1.39 and 3.64 per 1000 person-years, respectively. Patients with CKD showed an adjusted HR of 2.15 and 1.45 for SSNHL and Ménière's disease, respectively. Middle-aged patients with CKD were associated with a higher incidence of developing SSNHL and Ménière's disease than those without CKD. Female patients with CKD had a higher risk of developing SSNHL; however, there was no significant difference in the risk of Ménière's disease in patients with CKD according to sex. Our findings suggest that CKD is associated with an increased incidence of SSNHL and Ménière's disease. Therefore, audiovestibular surveillance should be considered in patients with CKD.

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.

Prevalence of Sensorineural Hearing Loss in Pediatric Patients with Sickle Cell Disease: A Meta-analysis.

OBJECTIVES: To determine the prevalence of Sensorineural Hearing Loss (SNHL) attributable to Sickle Cell Disease (SCD) in the global pediatric population and to identify factors contributing to its severity. STUDY DESIGN: Meta-analysis. METHODS: We performed a comprehensive literature search for scientific articles in PubMed, Scopus, CINAHL, Web of Science, and the Cochrane Library that reported the incidence of hearing loss in populations under 18 years of age with excluding studies analyzing patients on iron chelation therapy, adults, or those without objective audiological analysis. RESULTS: We identified 138 initial studies with 17 selected for analysis after applying the exclusion criteria. A total of 1,282 SCD patients and 553 controls were included in the meta-analysis. There was a statistically significant increase in the prevalence of SNHL in children with SCD compared to the general population with a cumulative risk ratio of 3.33. CONCLUSION: This is the first systematic investigation of the relationship between SCD and SNHL in pediatric patients across the globe. The increased prevalence of SNHL in the pediatric SCD population warrants future research into the predictors of SNHL severity and merits routine audiometric monitoring of SCD patients to reduce the social and developmental morbidity of hearing loss at a young age. PROSPERO Registration #: CRD42019132601. Laryngoscope, 131:1147-1156, 2021.

Association between Sudden Sensorineural Hearing Loss and Preexisting Thyroid Diseases: A Nationwide Case-Control Study in Taiwan.

Background: Little evidence is available about the risk of sudden sensorineural hearing loss (SSNHL) in patients with thyroid diseases. We assessed whether a diagnosis of thyroid disease, particularly hyperthyroidism or hypothyroidism, is associated with SSNHL risk in an Asian population. Material and Methods: This case-control study was conducted with population-based data from Taiwan's National Health Insurance Research Database from January 2000 to December 2013. The case group comprised 3331 adult patients with newly diagnosed SSNHL, and four controls without SSNHL for each case matched by sex, age, monthly income, and urbanization level of residence. Underlying Thyroid diseases were retrospectively evaluated in the case and control groups. Multivariate logistic regression analyses were used to explore relations between thyroid diseases and SSNHL. Results: Of the 3331 cases, 5.7% had preexisting thyroid diseases, whereas only 4.0% of the 13,324 controls had the same condition. After adjustment for sex, age, monthly income, urbanization level of residence, history of hypertension, diabetes mellitus, chronic otitis media, and hyperlipidemia, associations were identified between a history of either hypothyroidism (adjusted odds ratio [AOR], 1.54; 95% CI, 1.02-2.32; p = 0.042) or hyperthyroidism (AOR, 1.41; 95% CI, 1.07-1.85; p = 0.015) and an elevated risk of SSNHL. In subgroup analysis, the correlation between hypothyroidism and increased SSNHL risk remained significant only for patients aged over 50 years (AOR, 1.61; 95% CI, 1.01-2.57; p = 0.045), and that between hyperthyroidism and SSNHL was significant only for female patients (AOR, 1.48; 95% CI, 1.09-2.01; p = 0.012). Treatment for hypothyroidism and hyperthyroidism did not alter the association in subgroup analyses. Conclusion: Preexisting hypothyroidism and hyperthyroidism appear associated with SSNHL susceptibility in Taiwan. Physicians should be wary of this elevated risk of SSNHL among patients with previously diagnosed thyroid dysfunction, especially women and patients aged more than 50 years.

Expanded carrier screening for preconception reproductive risk assessment: Prevalence of carrier status in a Mexican population.

OBJECTIVE: Genetic carrier screening has the potential to identify couples at risk of having a child affected with an autosomal recessive or X-linked disorder. However, the current prevalence of carrier status for these conditions in developing countries is not well defined. This study assesses the prevalence of carrier status of selected genetic conditions utilizing an expanded, pan-ethnic genetic carrier screening panel (ECS) in a large population of Mexican patients. METHODS: Retrospective chart review of all patients tested with a single ECS panel at an international infertility center from 2012 to 2018 were included, and the prevalence of positive carrier status in a Mexican population was evaluated. RESULTS: Eight hundred five individuals were analyzed with ECS testing for 283 genetic conditions. Three hundred fifty-two carriers (43.7%) were identified with 503 pathogenic variants in 145 different genes. Seventeen of the 391 participating couples (4.34%) were identified as being at-risk couples. The most prevalent alleles found were associated with alpha thalassemia, cystic fibrosis, GJB2 nonsyndromic hearing loss, biotinidase deficiency, and familial Mediterranean fever. CONCLUSION: Based on the prevalence and severity of Mendelian disorders, we recommend that couples who wish to conceive regardless of their ethnicity background explore carrier screening and genetic counseling prior to reproductive medical treatment.

Identifying Disadvantaged Groups for Cochlear Implantation: Demographics from a Large Cochlear Implant Program.

OBJECTIVE: To identify demographic predictors of patients undergoing cochlear implantation evaluation and surgery. METHODS: Consecutive adult patients between 2009 and 2018 who underwent cochlear implantation evaluation at a university cochlear implantation program were retrospectively identified to determine (1) cochlear implantation qualification rate and (2) pursuit of surgery rate with respect to age, gender, race, primary spoken language, marital status, insurance type, and distance to the cochlear implantation center. RESULTS: A total of 823 cochlear implantation evaluations were analyzed. Overall, 76.3% of patients qualified for cochlear implantation and 61.5% of these patients pursued surgery. Age was the only independent predictor for cochlear implantation qualification, such that, for each year younger, the odds of qualifying for cochlear implantation increased by 2.5% (OR 0.98; 95% CI: 0.96-0.99). Age, race, marital status, and insurance type were each independent predictors of the decision to pursue surgery. The odds of pursuing surgery increased by 2.8% for each year younger (OR 1.03; 95% CI: 1.01-1.05). Compared to White patients, non-Whites were half as likely to pursue surgery (OR 0.47; 95% CI: 0.25-0.88). Single (OR 0.49; 95% CI: 0.26-0.94) and widowed patients (OR 0.46; 95% CI: 0.23-0.95) were about half as likely to pursue surgery as compared to married patients. Patients with military insurance were 13 times more likely to pursue surgery as compared to patients with Medicare (OR 13.0; 95% CI: 1.67-101.4). CONCLUSION: Younger age is an independent predictor for a higher cochlear implantation qualification rate, suggesting the possibility for delayed candidacy referral. Rate of surgical pursuit in qualified cochlear implantation candidates is lower for racial minorities, single and widowed patients, and older patients.

Economic cost of congenital CMV in the UK.

OBJECTIVE: Congenital cytomegalovirus (cCMV) is the most common infectious cause of congenital disability. It can disrupt neurodevelopment, causing lifelong impairments including sensorineural hearing loss and developmental delay. This study aimed, for the first time, to estimate the annual economic burden of managing cCMV and its sequelae in the UK. DESIGN: The study collated available secondary data to develop a static cost model. SETTING: The model aimed to estimate costs of cCMV in the UK for the year 2016. PATIENTS: Individuals of all ages with cCMV. MAIN OUTCOME MEASURES: Direct (incurred by the public sector) and indirect (incurred personally or by society) costs associated with management of cCMV and its sequelae. RESULTS: The model estimated that the total cost of cCMV to the UK in 2016 was £732 million (lower and upper estimates were between £495 and £942 million). Approximately 40% of the costs were directly incurred by the public sector, with the remaining 60% being indirect costs, including lost productivity. Long-term impairments caused by the virus had a higher financial burden than the acute management of cCMV. CONCLUSIONS: The cost of cCMV is substantial, predominantly stemming from long-term impairments. Costs should be compared against investment in educational strategies and vaccine development programmes that aim to prevent virus transmission, as well as the value of introducing universal screening for cCMV to both increase detection of children who would benefit from treatment, and to build a more robust evidence base for future research.

Use of otoacoustic emissions to improve outcomes and reduce disparities in a community preschool hearing screening program.

INTRODUCTION: Hearing loss substantially impacts pediatric development, and early identification improves outcomes. While intervening before school-entry is critical to optimize learning, early-childhood hearing screening practices are highly variable. Conditioned play audiometry (CPA) is the gold standard for preschool hearing screening, but otoacoustic emission (OAE) testing provides objective data that may improve screening outcomes. OBJECTIVES: To compare outcomes of a community-based low-income preschool hearing program before and after implementation of OAE in a single-visit, two-tiered paradigm. We hypothesized that this intervention would reduce referral rates and improve follow-up while maintaining stable rates of diagnosed sensorineural hearing loss. METHODS: We performed a cohort study of 3257 children screened from July 2014-June 2016. Department of Public Health data were analyzed pre- and post-implementation of second-line OAE testing for children referred on CPA screening with targeted follow-up by DPH staff. Primary outcomes included referral rates, follow-up rates, and diagnosis of sensorineural hearing loss. RESULTS: Demographics, pure-tone pass rates, and incidence of newly-diagnosed permanent hearing loss were similar across years. After intervention, overall pass rates increased from 92% to 95% (P = 0.0014), while only 0.7% remained unable to be tested (P<0.0001). 5% of children were unable to be tested by CPA screening but passed OAE testing, obviating further evaluation. Referral rate decreased from 8% to 5% (P = 0.0014), and follow-up improved from 36% to 91% (P<0.0001). Identification of pathology in children with follow-up increased from 19% to over 50%. Further, disparities in pass rates and ability to test seen in Year 1 were eliminated in Year 2. CONCLUSION AND RELEVANCE: In a community setting, implementation of second-line OAE screening for CPA referrals reduced referral rates, increased identification of hearing loss, reduced outcome disparities, and improved follow-up rates. This study provides lessons in how to improve outcomes and reduce disparities in early-childhood hearing screening.

Audiologist-led screening of acoustic neuromas in patients with asymmetrical sensorineural hearing loss and/or unilateral tinnitus: our experience in 1126 patients.

OBJECTIVE: To determine whether patients within an otolaryngology department presenting with asymmetrical sensorineural hearing loss and/or unilateral tinnitus can be safely and cost-efficiently screened for acoustic neuroma by audiologists as a first or only point of contact. METHODS: A prospective case series and cost analysis were conducted at a tertiary referral centre. Between April 2013 and March 2017, 1126 adult patients presented to the audiology department with asymmetrical sensorineural hearing loss and/or unilateral tinnitus. All were screened for acoustic neuroma with magnetic resonance imaging, based on pre-determined criteria. The main outcome measure was the presence of acoustic neuroma or other pathology on magnetic resonance imaging. RESULTS: Twenty-five patients (2.22 per cent) were found to have an acoustic neuroma (size range: 3-20 mm) and were referred to the otolaryngologist for further assessment. The remaining patients were appropriately managed and discharged by the audiologists without ENT input. This resulted in an overall cost saving of £164 850. CONCLUSION: Patients with asymmetrical sensorineural hearing loss and/or unilateral tinnitus can be safely screened for acoustic neuroma and independently managed by audiologists as a first or only point of contact, resulting in considerable departmental cost savings.

Satisfaction with Life among Mothers of Pediatric Cochlear Implant Candidates: The Impact of Implant Operation and Sociodemographic Factors.

OBJECTIVES: To evaluate the satisfaction with life among mothers of pediatric cochlear implant candidates regarding implant surgery and sociodemographic factors. MATERIALS AND METHODS: Mothers of 160 pediatric patients with profound sensorineural hearing loss who underwent unilateral cochlear implant surgery were included. A questionnaire form with items on sociodemographic-familial characteristics and Satisfaction with Life Scale (SWLS) was employed via face-to-face interview method before and 12 months after the implant surgery. RESULTS: The SWLS scores significantly improved after the implant surgery [from 19.1 (7.0) to 28.9 (4.0), p<0.000]. Being unemployed vs. employed [17.9 (6.9) vs. 24.0 (5.3), p=0.000], having another child with hearing disability [13.5 (5.7) vs. 19.7 (6.9), p=0.001], younger (12-24 months) vs. older (>24 months) age of the child at the time of implant surgery [7.1 (0.4) vs. 19.7 (6.6), p=0.001], absence vs. presence of regular follow-up visits [13.0 (0.0) vs. 19.4 (7.1), p=0.002], and presence vs. absence of change in social life after the diagnosis of disease [17.3 (6.5) vs. 20.9 (7.1), p=0.001] were associated with significantly lower SWLS scores among mothers. SWLS scores were positively correlated with patient's age at the time of implant surgery (r=0.206, p=0.009), whereas negatively correlated with the number of household members (r=-0.406, p=0.000) and number of children (r=-0.310, p=0.000). CONCLUSION: In conclusion, our findings revealed the association of cochlear implantation with a significant increase in mother's life satisfaction, despite the unemployment, presence of another child with hearing disability, and crowded household. Our findings emphasize on the consideration of family systems with special attention to mother's emotional experiences and occupational competence in the intervention programs.

Preschool Children Hearing Impairment: Prevalence, Diagnosis and Management in a Developing Country.

OBJECTIVES: Preschool children hearing impairment is a common otologic diseases worldwide. The burdens of this preventable condition can be reduced in developing country. This study was carried out to determine the prevalence, diagnosis and management of hearing impairment among children less than 5 years seen in our center. METHOD: This was a prospective hospital based study of preschool children with complaints of hearing impairment in Ekiti state university teaching hospital, Nigeria. This study was conducted over a period of two years (February 2016 to January 2018). Data was obtained from consented patients by using pretested interviewers assisted questionnaire. Data obtained was collated and analyzed by using SPSS software version 18.0. D escriptive statistics was used to present and expressed the data as simple tables and charts. RESULTS: The prevalence of hearing impairment in this study was 5.9% with peak value of 37.6% at preschool age 3 years. There were 42.6% males and 57.4% females. Majority (51.5%) of the patients were urban dwellers. The aetiologic factors of hearing impairment include; 15.8% neonatal jaundice, 13.9% febrile illness, 12.9% otitis media and 11.9% birth asphyxia. Prelingual presentation was commonest in 57.4%. Common clinical features includes yet to speak, restlessness/stubborn and not responding to command in 60.4%, 48.5% and 46.5% respectively. Commonest type of hearing impairment was sensorineural hearing loss is 61.4%. Type A tympanometry (normal) was the commonest findings in 82.2%. CONCLUSION: Preschool children hearing impairment with late presentation is a common otologic diseases burden to parent and otorhinolaryngologist in our center. The common causes were preventable with resulting permanent auditory damage.

Azithromycin and Sensorineural Hearing Loss in Adults: A Retrospective Cohort Study.

OBJECTIVE: To examine whether short-term use of azithromycin increases the risk of sensorineural hearing loss (SNHL) in adults with uncomplicated infections. STUDY DESIGN: A retrospective cohort study using Medicaid claims data, 1999 to 2010. PATIENTS: Adults (18-64 years old) who had continuous enrollment in Medicaid for the 12 months (baseline) before the date of first dispensation (index date) of oral azithromycin or amoxicillin ±â€Šclavulanate for uncomplicated infections. MAIN OUTCOME DEFINITION: We operationalized sensorineural hearing loss (SNHL) by a charge for audiometry and followed by a diagnosis of SNHL within 30 days. ANALYSIS: We adjusted for the baseline covariates through propensity score matching. Adults were followed for up to 120 days after the index date. The hazard of SNHL in azithromycin-exposed adults was compared with those who had amoxicillin ±â€Šclavulanate using a Cox proportional hazard model. We performed several sensitivity analyses by varying the follow-up time, SNHL definition, adjusting for cumulative antibiotic use, and switching exposure status during the follow-up period. RESULTS: A total of 493,774 patients entered the study cohort. The unadjusted incidence rates of SNHL were 38 and 41 cases per 10,000 patient-years following exposure to azithromycin and amoxicillin ±â€Šclavulanate, respectively. The adjusted (matched) hazard ratio (HR) of SNHL for azithromycin versus amoxicillin ±â€Šclavulanate was 0.91 (95% confidence interval [CI], 0.77-1.07). The sensitivity analyses findings were consistent with the primary analysis. CONCLUSION: Azithromycin short-term use was not associated with an increased risk of SNHL in comparison to amoxicillin ±â€Šclavulanate.

Lassa fever-induced sensorineural hearing loss: A neglected public health and social burden.

Although an association between Lassa fever (LF) and sudden-onset sensorineural hearing loss (SNHL) was confirmed clinically in 1990, the prevalence of LF-induced SNHL in endemic countries is still underestimated. LF, a viral hemorrhagic fever disease caused by Lassa virus (LASV), is endemic in West Africa, causing an estimated 500,000 cases and 5,000 deaths per year. Sudden-onset SNHL, one complication of LF, occurs in approximately one-third of survivors and constitutes a neglected public health and social burden. In the endemic countries, where access to hearing aids is limited, SNHL results in a decline of the quality of life for those affected. In addition, hearing loss costs Nigeria approximately 43 million dollars per year. The epidemiology of LF-induced SNHL has not been characterized well. The complication of LF induced by SNHL is also an important consideration for vaccine development and treatments. However, research into the mechanism has been hindered by the lack of autopsy samples and relevant small animal models. Recently, the first animal model that mimics the symptoms of SNHL associated with LF was developed. Preliminary data from the new animal model as well as the clinical case studies support the mechanism of immune-mediated injury that causes SNHL in LF patients. This article summarizes clinical findings of hearing loss in LF patients highlighting the association between LASV infection and SNHL as well as the potential mechanism(s) for LF-induced SNHL. Further research is necessary to identify the mechanism and the epidemiology of LF-induced SNHL.

Valganciclovir Use Among Commercially and Medicaid-insured Infants With Congenital CMV Infection in the United States, 2009-2015.

PURPOSE: The aim of this study was to assess the clinical characteristics and trends in valganciclovir use among infants diagnosed with congenital cytomegalovirus (CMV) disease in the United States. METHODS: We analyzed data from medical claims dated 2009-2015 from the Truven Health MarketScan® Commercial Claims and Encounters and Medicaid databases. We identified infants with a live birth code in the first claim who were continuously enrolled for at least 45 days. Among infants diagnosed with congenital CMV disease, identified by an ICD-9-CM or ICD-10-CM code for congenital CMV infection or CMV disease within 45 days of birth, we assessed data from claims containing codes for any CMV-associated clinical condition within the same period, and data from claims for hearing loss and/or valganciclovir within the first 180 days of life. FINDINGS: In the commercial and Medicaid databases, we identified 257 (2.5/10,000) and 445 (3.3/10,000) infants, respectively, diagnosed with congenital CMV disease, among whom 135 (53%) and 282 (63%) had ≥1 CMV-associated condition, 30 (12%) and 32 (7%) had hearing loss, and 41 (16%) and 78 (18%) had a claim for valganciclovir. Among infants with congenital CMV disease who had a claim for valganciclovir, 37 (90%) among commercially insured infants and 68 (87%) among Medicaid-insured infants had ≥1 CMV-associated condition and/or hearing loss. From 2009 to 2015, the percentages with a claim for valganciclovir increased from 0% to 29% among commercially insured infants and from 4% to 37% among Medicaid-insured infants (P < 0.0001). IMPLICATIONS: During 2009-2015, there was a strong upward trend in valganciclovir claims among insured infants who were diagnosed with congenital CMV disease, the majority of whom had CMV-associated conditions and/or hearing loss.