A Scoping Review of Otologic Manifestations of Hematologic Malignancies.

OBJECTIVE: To examine the otologic and neurotologic symptoms, physical examination findings, and imaging features secondary to hematologic malignancies. METHODS: Following Preferred Reporting Items for Systematic Reviews and Meta-Analyses guidelines, databases, including PubMed, Scopus, and CINAHL, were searched for articles including patients with otologic manifestations of leukemia, lymphoma and multiple myeloma. Data collected included patient and study demographics, specific hematologic malignancy, timing and classification of otologic symptoms, physical examination findings, imaging features and methods of diagnosis. Pooled descriptive analysis was performed. RESULTS: Two hundred seventy-two articles, of which 255 (93.8%) were case reports and 17 (6.2%) were case series, reporting on 553 patients were identified. Otologic manifestations were reported on 307 patients with leukemia, 204 patients with lymphoma and 42 patients with multiple myeloma. Hearing loss and unilateral facial palsy were the most common presenting symptoms for 111 reported subjects with leukemia (n = 46, 41.4%; n = 43, 38.7%) and 90 with lymphoma (n = 38, 42.2%; n = 39, 43.3%). Hearing loss and otalgia were the most common presenting symptoms for 21 subjects with multiple myeloma (n = 10, 47.6%; n = 6, 28.6%). Hearing loss and unilateral facial palsy were the most common otologic symptoms indicative of relapse in subjects with leukemia (n = 14, 43.8%) and lymphoma (n = 5, 50%). CONCLUSION: Hearing loss, facial palsy, and otalgia might be the first indication of a new diagnosis or relapse of leukemia, lymphoma, or multiple myeloma. Clinicians should have a heightened level of suspicion of malignant etiologies of otologic symptoms in patients with current or medical histories of these malignancies.

Prevalence of hearing loss, tinnitus, vertigo and sudden deafness among patients with polymyositis and dermatomyositis.

Little is known about a possible association of autoimmune inner ear disease among patients diagnosed with polymyositis (PM)/dermatomyositis (DM). This study aimed to explore differences in the prevalence of inner ear symptoms among patients with and without PM/DM using a nationwide population-based dataset. Data for this study were retrieved from the Taiwan National Health Insurance Research Database. The study sample included 1622 patients diagnosed with PM/DM and 8109 propensity-score matched comparison patients without PM/DM. We performed multivariate logistic regressions to calculate odds ratios (ORs) and 95% confidence interval (CI) for tinnitus, hearing loss, sudden deafness, and vertigo among patients with PM/DM versus comparison patients. Chi-square tests showed statistically significant differences between patients with PM/DM and comparison patients in the prevalence of tinnitus (16.1% vs. 12.7%, p < 0.001), non-conductive hearing loss (9.2% vs. 6.8%, p < 0.001), and vertigo (14.4% vs. 11.1%, p < 0.001). The adjusted ORs for tinnitus, non-conductive hearing loss, and vertigo, respectively, were 1.332 (95% CI = 1.147-1.547), 1.399 (95% CI = 1.154-1.696), and 1.374 (95% CI = 1.173-1.611) for patients with PM/DM when compared to comparison patients. Our study finds that patients with PM/DM have higher prevalence rates of tinnitus, non-conductive hearing loss, and vertigo than comparison patients.

Speech, Language, Hearing, and Otopathology Results From the International Smith-Magenis Syndrome Patient Registry.

PURPOSE: Smith-Magenis syndrome (SMS), a rare, genetically linked complex developmental disorder caused by a deletion or mutation within chromosome 17p11.2, is associated with delays in speech-language development, otopathology, and hearing loss, yet previous studies lack comprehensive descriptions of hearing and communication profiles. Here, analyses of patient registry data expand what is known about speech, language, hearing, and otopathology in SMS. METHOD: International speech-language and hearing registry survey data for 82 individuals with SMS were analyzed using descriptive and inferential statistics. Hearing loss, history of otitis media and pressure equalization (PE) tubes, communication mode, expressive/receptive language, and vocal quality were analyzed for all subjects and subjects grouped by age. Statistical methods included descriptive statistics and Pearson's chi-square tests of independence to test for differences between age groups for each variable of interest. Association analyses included Pearson's correlations. RESULTS: Hearing and otological analyses revealed that 35% of subjects had hearing loss, 66% had a history of otitis media, and 62% had received PE tubes. Speech-language analyses revealed that 60% of subjects communicated using speech, 79% began speaking words at/after 24 months of age, 92% combined words at/after 36 months, and 41% used sign language before speech. There was a significant association between the age that first words were spoken and the age that PE tubes were first placed. Communication strengths noted in more than 40% of subjects included social interest, humor, and memory for people, past events, and/or facts. CONCLUSIONS: Significant delays and impairment in speech-language were common, but the majority of those with SMS communicated using speech by age 6 years. Age was a significant factor for some aspects of hearing loss and communication. Neither hearing loss nor otitis media exacerbated language impairment. These results confirm and extend previous findings about the nature of speech, language, hearing, and otopathology in those with SMS.

Hearing Loss and Dementia: Where to From Here?

Victorian era psychologists were the first to comment on associations between sensory and cognitive function. More recently, hearing loss has been shown as a marker of risk for dementia. However, it is not known whether this association represents a causal impact of hearing loss, nor whether treating hearing loss may help prevent dementia. Most studies on relationships between hearing loss and cognitive outcomes are observational, are at risk of confounding, and cannot reach conclusions about causation. A recent high quality randomized controlled trial, relatively uncommon in audiology, reported no impact of a comprehensive hearing intervention in mitigating cognitive decline in older adults. Although secondary analysis revealed potential benefits in a sub-sample of adults, this finding may be spurious. Encouraging policymakers, patients, and other health care practitioners to address hearing loss in terms of dementia prevention may be inappropriate on the grounds of both relevance at individual level and lack of clear evidence of benefit. In addition, advocating need to address hearing loss in terms of mitigating dementia risk may reduce the importance of addressing hearing loss in its own right. Linking hearing loss to dementia risk may also exacerbate the stigma of hearing loss, inadvertently discouraging people from seeking help for hearing. We suggest that treating hearing loss may have important benefits in preventing or delaying diagnosis of dementia via improving orientation and functioning in daily life, without changing the underlying pathology. Rather than linking hearing loss to dementia risk, we suggest a positive message focusing on the known benefits of addressing hearing loss in terms of improved communication, quality of life, and healthy aging.

[A case of sudden hearing loss combined with familial hyperlipidemia].

Hyperlipidemia is characterized by elevated levels of blood lipids. The clinical manifestations are mainly atherosclerosis caused by the deposition of lipids in the vascular endothelium. The link between abnormal lipid metabolism and sudden hearing loss remains unclear. This article presents a case study of sudden hearing loss accompanied by familial hyperlipidemia. Pure tone audiometry indicated intermediate frequency hearing loss in one ear. Laboratory tests showed abnormal lipid metabolism, and genetic examination identified a heterozygous mutation in theAPOA5 gene. Diagnosis: Sudden hearing loss; hypercholesterolemia. The patient responded well to pharmacological treatment. This paper aims to analyze and discuss thepotential connection between abnormal lipid metabolism and sudden hearing loss.

Complete Restoration of Hearing Loss and Cochlear Synaptopathy via Minimally Invasive, Single-Dose, and Controllable Middle Ear Delivery of Brain-Derived Neurotrophic Factor-Poly(dl-lactic acid-co-glycolic acid)-Loaded Hydrogel.

Noise-induced hearing loss (NIHL) often accompanies cochlear synaptopathy, which can be potentially reversed to restore hearing. However, there has been little success in achieving complete recovery of sensorineural deafness using nearly noninvasive middle ear drug delivery before. Here, we present a study demonstrating the efficacy of a middle ear delivery system employing brain-derived neurotrophic factor (BDNF)-poly-(dl-lactic acid-co-glycolic acid) (PLGA)-loaded hydrogel in reversing synaptopathy and restoring hearing function in a mouse model with NIHL. The mouse model achieved using the single noise exposure (NE, 115 dBL, 4 h) exhibited an average 20 dBL elevation of hearing thresholds with intact cochlear hair cells but a loss of ribbon synapses as the primary cause of hearing impairment. We developed a BDNF-PLGA-loaded thermosensitive hydrogel, which was administered via a single controllable injection into the tympanic cavity of noise-exposed mice, allowing its presence in the middle ear for a duration of 2 weeks. This intervention resulted in complete restoration of NIHL at frequencies of click, 4, 8, 16, and 32 kHz. Moreover, the cochlear ribbon synapses exhibited significant recovery, whereas other cochlear components (hair cells and auditory nerves) remained unchanged. Additionally, the cochlea of NE treated mice revealed activation of tropomyosin receptor kinase B (TRKB) signaling upon exposure to BDNF. These findings demonstrate a controllable and minimally invasive therapeutic approach that utilizes a BDNF-PLGA-loaded hydrogel to restore NIHL by specifically repairing cochlear synaptopathy. This tailored middle ear delivery system holds great promise for achieving ideal clinical outcomes in the treatment of NIHL and cochlear synaptopathy.

Contribution of Tinnitus and Hearing Loss to Depression: NHANES Population Study.

OBJECTIVES: Hearing loss affects the emotional well-being of adults and is sometimes associated with clinical depression. Chronic tinnitus is highly comorbid with hearing loss and separately linked with depression. In this article, the authors investigated the combined effects of hearing loss and tinnitus on depression in the presence of other moderating influences such as demographic, lifestyle, and health factors. DESIGN: The authors used the National Health and Nutrition Examination Survey data (2011-2012 and 2015-2016) to determine the effects of hearing loss and tinnitus on depression in a population of US adults (20 to 69 years). The dataset included the Patient Health Questionnaire-9 for depression screening, hearing testing using pure-tone audiometry, and information related to multiple demographic, lifestyle, and health factors (n = 5845). RESULTS: The statistical analysis showed moderate to high associations between depression and hearing loss, tinnitus, and demographic, lifestyle, and health factors, separately. Results of logistic regression analysis revealed that depression was significantly influenced by hearing loss (adjusted odds ratios [OR] = 3.0), the functional impact of tinnitus (adjusted OR = 2.4), and their interaction, both in the absence or presence of the moderating influences. The effect of bothersome tinnitus on depression was amplified in the presence of hearing loss (adjusted OR = 2.4 in the absence of hearing loss to adjusted OR = 14.9 in the presence of hearing loss). Conversely, the effect of hearing loss on depression decreased when bothersome tinnitus was present (adjusted OR = 3.0 when no tinnitus problem was present to adjusted OR = 0.7 in the presence of bothersome tinnitus). CONCLUSIONS: Together, hearing loss and bothersome tinnitus had a significant effect on self-reported depression symptoms, but their relative effect when comorbid differed. Tinnitus remained more salient than hearing loss and the latter's contribution to depression was reduced in the presence of tinnitus, but the presence of hearing loss significantly increased the effects of tinnitus on depression, even when the effects of the relevant demographic, lifestyle, or health factors were controlled. Treatment strategies that target depression should screen for hearing loss and bothersome tinnitus and provide management options for the conditions.

Sensorineural hearing loss among children at risk: A 16-year audiological records review in a tertiary referral center.

OBJECTIVES: Hearing loss (HL) constitutes an increasing worldwide health problem. Neonatal hearing screening improved early detection and management to alleviate HL detriments on the person and society. Still, HL in childhood, beyond infancy, is under-investigated, especially in developing countries. This study aimed to explore the prevalence of HL in childhood amongst Jordanian children with HL risk factors and investigate the associated risk factors. METHODS: Retrospective cross-sectional review of audiological records in a tertiary public and teaching hospital. The data of 1307 children aged 0-15 years who underwent audiological assessment from 2000 to 2016 were included. A review of diagnostic audiological and medical records was conducted to investigate the prevalence of sensorineural HL in high-risk (HR) children and the most contributing risk factors. RESULTS: Descriptive statistical analysis showed that the prevalence of sensorineural HL was 29.2% in the study sample. The HL was bilateral in 95% and mild to moderate HL in 73%. The mean age at the diagnosis was around 4.5 years. The most common risk factors were parental concern about their child's hearing, ototoxic drug use, and developmental and speech delay. The Chi-squared test showed that parental concern and ototoxic drug use were associated with an increased probability of having HL. CONCLUSION: The prevalence of HL amongst at-risk children in Jordan is relatively high, and the diagnosis is delayed. The results highlight the importance of implementing a hearing screening program in at-risk children. This needs to start from birth and include a serial follow-up to detect cases of delayed-onset HL.

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

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

Clinical and molecular characteristics of two Italian kindreds with hypoparathyroidism, deafness and renal dysplasia (HDR) syndrome.

PURPOSE: Hypoparathyroidism, deafness, and renal dysplasia (HDR) syndrome, also known as Barakat syndrome, is a rare autosomal dominant disease characterized by the triad of hypoparathyroidism, deafness, and renal abnormalities. The disorder is caused by the haploinsufficiency of the zinc finger transcription factor GATA3 and exhibits a great clinical variability with an age-dependent penetrance of each feature. We report two unrelated kindreds whose probands were referred to our outpatient clinic for further evaluation of hypoparathyroidism. METHODS: The proband of family 1, a 17-year-old boy, was referred for severe hypocalcemia (5.9 mg/dL) incidentally detected at routine blood tests. Abdomen ultrasound showed bilateral renal cysts. The audiometric evaluation revealed the presence of bilateral moderate hearing loss although the patient could communicate without any problem. Conversely, the proband of family 2, a 19-year-old man, had severe symptomatic hypocalcemia complicated by epileptic seizure at the age of 14 years; his past medical history was remarkable for right nephrectomy at the age of 4 months due to multicystic renal disease and bilateral hearing loss diagnosed at the age of 18 years. RESULTS: Based on clinical, biochemical, and radiologic data, HDR syndrome was suspected and genetic analysis of the GATA3 gene revealed the presence of two pathogenetic variants in exon 3, c.404dupC and c.431dupG, in the proband of family 1 and 2, respectively. CONCLUSION: HDR syndrome is a rare cause of hypoparathyroidism and must be excluded in all patients with apparently idiopathic hypoparathyroidism. A correct diagnosis is of great importance for early detection of other HDR-related features and genetic counseling.

Congenital Cytomegalovirus-Associated Sensorineural Hearing Loss in Children: Identification Following Universal Newborn Hearing Screening, Effect of Antiviral Treatment, and Long-Term Hearing Outcomes.

OBJECTIVES: Congenital cytomegalovirus (cCMV) is the most common cause of nongenetic sensorineural hearing loss (SNHL) in children. We examined the longitudinal hearing outcomes of children with cCMV in relation to their newborn hearing screening findings, and their use of antiviral therapy. DESIGN: The study was based on a retrospective chart review using a database of pediatric patients (N = 445) seen at the University of Minnesota Lions clinic. Chart review identified infants with cCMV, and records were reviewed for information about universal newborn hearing screen (UNHS) results, the clinical course of SNHL, and the use of antiviral therapy. RESULTS: A total of 44 children were identified with cCMV. In this group, 33 (75%) had SNHL of varying degree and age at onset. Notably, 17 (39%) children passed UNHS bilaterally. Of those children, 6 (35%) ultimately acquired bilateral or unilateral SNHL, detected at a mean age of 20 months (median age, 12 months). Five out of 10 children (50%) that did not pass UNHS in one ear acquired late-onset hearing loss in the contralateral ear, identified at a mean age of 24 months (median age, 4 months). Eleven (25%) children passed UNHS bilaterally and continued to demonstrate normal hearing in both ears at their most recent follow-up visit at a mean age of 19 months (SD, 18 months). Of the 33 children with cCMV and SNHL, 18 (55%) received antiviral medication (ganciclovir and/or valganciclovir). While, on average, both treated and untreated ears experienced a progression of hearing loss over time, the group that received antiviral treatment experienced less overall hearing change compared with the untreated group (baseline-adjusted expected mean difference, -10.5 dB; 95% confidence interval, -28.1 to 7.2 dB). CONCLUSIONS: Among children with cCMV included in this study who passed UNHS in both ears, 35% demonstrated delayed-onset SNHL. Notably, of those children who referred unilaterally, 50% later demonstrated SNHL in the contralateral ear. These findings have implications for audiological monitoring, and potentially antiviral therapy, of children with cCMV. As implementation of universal cCMV screening moves forward, a key aspect of follow-up will be appropriate long-term audiologic monitoring.

Identification of eight genomic protective alleles for mitochondrial diabetes by Kinship-graph convolutional network.

AIMS: Nearly 85% of maternally inherited diabetes and deafness (MIDD) are caused by the m.3243A>G mutation in the mitochondrial DNA. However, the clinical phenotypes of MIDD may also be influenced by the nuclear genome, this study aimed to investigate nuclear genome variants that influence clinical phenotypes associated with m.3243A>G mutation in MIDD based on whole-genome sequencing of the patients belonging to pedigrees. MATERIALS AND METHODS: We analyzed a whole-genome sequencing (WGS) dataset from blood samples of 38 MIDD patients with the m.3243A > G mutation belonging to 10 pedigrees, by developing a Kinship-graph convolutional network approach, called Ki-GCN, integrated with the conventional genome-wide association study (GWAS) methods. RESULTS: We identified eight protective alleles in the nuclear genome that have protective effects against the onset of MIDD, related deafness, and also type 2 diabetes. Based on these eight protective alleles, we constructed an effective logistic regression model to predict the early or late onset of MIDD patients. CONCLUSIONS: There are protective alleles in the nuclear genome that are associated with the onset-age of MIDD patients and might also provide protective effects on the deafness derived from MIDD patients.

Depression as a mediator of the association between vision and/or hearing loss and recent substance use: NHANES 2013-2018.

BACKGROUND: Previous research has demonstrated a significant association between vision and/or hearing loss and lifetime substance use. OBJECTIVE: The objective of this analysis was to assess whether depression mediates the association between vision and/or hearing loss and recent substance use (RSU). METHODS: Data from 9408 NHANES 2013-2018 participants were used for a survey-weighted analysis to assess whether the indirect effect (IE) of disability status (neither, vision loss only, hearing loss only, both) on the outcome RSU (past 30-day use of marijuana, cocaine, methamphetamine, or heroin) was mediated by recent (past 2 weeks) depression (Patient Health Questionnaire- 9 items score; none = 0-4, mild or greater = 5+), adjusting for confounders. RESULTS: The estimated prevalence of vision and/or hearing loss, mild or greater depression, and RSU were 6.7 %, 24.1 %, and 16.8 %. RSU was significantly positively associated with disability status before (p = .018) but not after adjusting for depression (p = .160), and the indirect effects were statistically significant (p < .001). CONCLUSIONS: The data are consistent with the hypothesis that recent depression mediates the association between vision and/or hearing loss and RSU. Initiatives may be needed that incorporate a focus on the prevention, management, or care for depression to intervene on the pathway between hearing and/or vision loss and RSU.

An Italian case series' description of thiamine responsive megaloblastic anemia syndrome: importance of early diagnosis and treatment.

BACKGROUND: Individuals with thiamine-responsive megaloblastic anemia (TRMA) mainly manifest macrocytic anemia, sensorineural deafness, ocular complications, and nonautoimmune diabetes. Macrocytic anemia and diabetes may be responsive to high-dosage thiamine treatment, in contrast to sensorineural deafness. Little is known about the efficacy of thiamine treatment on ocular manifestations. CASES PRESENTATION: Our objective is to report data from four Italian TRMA patients: in Cases 1, 2 and 3, the diagnosis of TRMA was made at 9, 14 and 27 months. In 3 out of 4 subjects, thiamine therapy allowed both normalization of hyperglycemia, with consequent insulin suspension, and macrocytic anemia. In all Cases, thiamine therapy did not resolve the clinical manifestation of deafness. In Cases 2 and 3, follow-up showed no blindness, unlike Case 4, in which treatment was started for megaloblastic anemia at age 7 but was increased to high doses only at age 25, when the genetic diagnosis of TRMA was performed. CONCLUSIONS: Early institution of high-dose thiamine supplementation seems to prevent the development of retinal changes and optic atrophy in TRMA patients. The spectrum of clinical manifestations is broad, and it is important to describe known Cases to gain a better understanding of this rare disease.

Associations Between Age-Related Hearing Loss, Cognitive Impairment, and Multiple Chronic Conditions in a Group Care Setting.

PURPOSE: The purpose of this study was to explore the relationships between hearing loss, cognitive status, and a range of health outcomes over a period of 2 years in a sample of older adults who are enrolled in Program of All-Inclusive Care for the Elderly, which is a Medicare/Medicaid beneficiary program for individuals who are nursing home eligible but living in the community at time of enrollment. METHOD: The sample (N = 144) includes a diverse (47% White/non-Hispanic, 35% Black/African American, and 16% Latin/Hispanic) group of adults ranging from 55 to 93 years old. We used medical chart data to measure respondents' cognitive and health status, including chronic conditions and hospital use. Hearing status was measured once at the beginning of the 2-year review period. We used logistic regression and negative binomial hurdle models for analyses. We used latent class analysis (LCA) to explore the extent to which respondents cluster into a set of "health profiles" characterized by their hearing, cognitive status, and health conditions. RESULTS: We found that hearing loss is weakly associated with heart disease and diabetes and associated with cerebrovascular disease and falls; cognitive impairment is also associated with cerebrovascular disease and the number of falls. LCA indicates that respondents cluster into a variety of health profiles with a consistent pairing of hearing loss and depression. CONCLUSIONS: The results are largely consistent with associations reported in epidemiological studies that include age-related hearing loss. Of particular interest in this study is the LCA that suggested that all of the profiles associated with a high likelihood of hearing loss included a high risk of depression. The co-occurrence of these two factors highlights the need to identify and treat hearing loss in older adults, especially as part of the treatment plan for individuals with depressive symptoms.

COX inhibitor use during definitive radiotherapy is associated with worse hearing preservation in patients with vestibular schwannoma.

PURPOSE: Patients with vestibular schwannoma undergoing definitive radiotherapy commonly experience hearing loss due to tumor and treatment effects; however, there is limited data evaluating concurrent medication use and other clinicopathologic factors associated with hearing preservation during and after radiotherapy. We performed a retrospective cohort study reviewing consecutive patients from 2004 to 2019 treated with radiotherapy for vestibular schwannoma at our institution. METHODS: Ninety four patients with concurrent medications, baseline audiograms, and post-radiotherapy audiograms available were evaluable. We performed chi-squared analyses of the frequency of various clinicopathologic factors and t-tests evaluating the degree of hearing loss based on audiograms. RESULTS: At a median follow-up of 35.7 months (mean: 46.5 months), the baseline pure-tone average (PTA) of the ipsilateral ear worsened from 38.4 to 59.5 dB following completion of radiotherapy (difference: 21.1, 95% CI 17.8-24.4 dB, p < 0.001). 36 patients (38.3%) reported regular use of cyclooxygenase (COX) inhibitors (including acetaminophen and NSAIDs) during radiotherapy. The mean increase in PTA was significantly higher for patients taking COX inhibitors (25.8 dB vs 18.1 dB, p = 0.024) in the ipsilateral ear but not for the contralateral side. COX inhibitor use remained independently associated with worse PTA in the multivariate analysis. CONCLUSION: COX inhibitor use during definitive radiotherapy is associated with worse hearing loss in the affected ear but not for the contralateral side. This suggests the ototoxic effects of COX inhibitors may influence the effects of radiotherapy. These results could have clinical implications and warrant further investigation.

A bidirectional Mendelian randomisation study to evaluate the relationship between body constitution and hearing loss.

Hearing loss and hearing disorders represent possible mediating pathways in the associations between noise exposures and non-auditory health outcomes. In this context, we assessed whether the noise-obesity associations should consider hearing functions as possible mediators and applied Mendelian randomisation (MR) to investigate causal relationships between body constitution and hearing impairments. We obtained genetic associations from publicly available summary statistics from genome-wide association studies in European ancestry adult populations (N= from 210,088 to 360,564) for (i) body constitution: body mass index (BMI), waist circumference (WC) and body fat percentage (BFP), and (ii) hearing loss: sensorineural hearing loss, noise-induced hearing loss, and age-related hearing impairment (ARHI). We employed colocalisation analysis to investigate the genetic associations for BMI and ARHI liability within an FTO locus. We conducted bi-directional MR for the 'forward' (from body constitution to hearing) and 'reverse' directions. We applied the random effects inverse variance-weighted method as the main MR method, with additional sensitivity analyses. Colocalisation analysis suggested that BMI and ARHI shared a causal variant at the FTO gene. We did not find robust evidence for causal associations from body constitution to hearing loss and suggested that some associations may be driven by FTO variants. In the reverse analyses, ARHI was negatively associated with BMI [effect size - 0.22 (95% CI - 0.44 to - 0.01)] and BFP [effect size - 0.23 (95% CI - 0.45 to 0.00)], supporting the notion that ARHI may diminish body constitution. Finally, our data suggest that there is no strong evidence that hearing explains the association between noise exposure and body constitution.

OCT imaging of endolymphatic hydrops in mice: association with hearing loss.

BACKGROUND: The etiology of Ménière's disease (MD) is still not completely clear, but it is believed to be associated with endolymphatic hydrops (EH), which is characterized by auditory functional disorders. Vasopressin injection in C57BL/6J mice can induce EH and serve as a model for MD. Optical Coherence Tomography (OCT) has shown its advantages as a non-invasive imaging method for observing EH.AimInvestigating the relationship between hearing loss and EH to assist clinical hearing assessments and indicate the severity of hydrops. METHODS: C57BL/6J mice received 50 µg/100g/day vasopressin injections to induce EH. Auditory function was assessed using auditory brainstem response (ABR) and distortion product otoacoustic emissions (DPOAE). OCT was used to visualize the cochlea. RESULT: OCT observed accumulation of fluid within the scala media in the cochlear apex. ABR showed significant hearing loss after 4 weeks. DPOAE revealed low-frequency hearing loss at 2 weeks and widespread damage across frequencies at 4 weeks. CONCLUSION: The development of hearing loss in mouse models of MD is consistent with EH manifestations.SignificanceThis study demonstrates the possibility of indirectly evaluating the extent of EH through auditory assessment and emphasizes the significant value of OCT for imaging cochlear structures.

A Case Report of Riboflavin Treatment and Cochlear Implants in a 4-Year-Old Girl with Progressive Hearing Loss and Delayed Speech Development: Brown-Vialetto-Van Laere Syndrome.

BACKGROUND Brown-Vialetto-Van Laere (BVVL) syndrome is a rare autosomal recessive disorder caused by mutations in intestinal riboflavin transporter genes, resulting in a motor neuron disorder of childhood, which can be associated with sensorineural deafness. This report describes a 4-year-old Polish girl with progressive hearing loss and delayed speech development diagnosed with Brown-Vialetto-Van Laere syndrome who was treated with riboflavin (vitamin B2) and cochlear implants. CASE REPORT The case report concerns a girl from Poland who, at the age of 2 years 10 months, developed progressive atypical neurological symptoms of unknown etiology: ataxia of the upper and lower limbs, gait abnormalities, generalized muscle weakness, visual and hearing problems, and regression of speech development. A karyotype study (whole-exome sequencing) revealed alterations within SLC52A2, leading to the diagnosis of Brown-Vialetto-Van Laere syndrome and initiation of high-dose riboflavin treatment. As a 4-year-old child, she presented to the Institute of Physiology and Pathology of Hearing - World Hearing Center in Poland with progressive hearing loss and speech regression. Hearing tests revealed bilateral profound sensorineural hearing loss with auditory neuropathy. Surgical treatment was applied in the form of bilateral cochlear implantation. CONCLUSIONS This report shows the importance of genetic testing in infants who present with atypical symptoms or signs. In this case, the diagnosis of Brown-Vialetto-Van Laere syndrome resulted in timely correction of the genetic riboflavin (vitamin B2) deficiency and improved hearing following the use of cochlear implants.

Universal neonatal hearing screening before and during the COVID-19 pandemic.

OBJECTIVE: To verify the frequency of risk factors for hearing loss in newborns and their possible associations with universal neonatal hearing screening results before and during the COVID-19 pandemic. METHODS: Historical cohort study with data analysis of newborns attended in a reference hearing health service of the Unified Health System (SUS) between January 2017 and December 2021. RESULTS: Those born in 2020 and 2021 were 91% less likely to fail the screening than those born in 2017, 2018, and 2019; therefore, they had a lower percentage of referrals for a retest. There was a decrease in congenital syphilis (1.00%), decrease in HIV (0.95%), and an increase in toxoplasmosis (0.58%) and increase in rubella cases in 2021 in relation to 2017. Syphilis had lower frequency rates during the COVID-19 pandemic (2020-2021). CONCLUSION: Newborns born in the pandemic year compared to those born pre-pandemic showed a reduction in the presence of two risk indicators for hearing loss and, consequently, a lower chance of failing the UNHS and a lower percentage of referral for retest.