Abnormal Hearing Research Articles

The chiisanoside derivatives present in the leaves of Acanthopanax sessiliflorus activate autophagy through the LRP6/GSK3β axis and thereafter inhibit oxidative stress, thereby counteracting cisplatin-induced ototoxicity

IntroductionCisplatin is extensively employed in the treatment of multiple solid malignant tumors. Nevertheless, side effects such as cisplatin-induced ototoxicity (CIO) pose obstacles to tumor therapy.The important natural product chiisanoside from Acanthopanax sessiliflorus has abundant activity against CIO.MethodsIn this study, 26 chiisanoside derivatives were screened, and compound 19 demonstrated significant protective activity against CIO damage. A cisplatin—induced HEI—OC1 cell injury model and a mouse ototoxicity model were established. The regulatory effects were revealed through transcriptome sequencing, and the protein expression levels were analyzed by molecular docking, ELISA, Western blotting, and immunofluorescence.ResultsIt was found that compound 19 inhibited cell apoptosis, alleviated abnormal hearing and spiral ganglion damage. Transcriptome sequencing revealed its regulatory effects. Compound 19 treatment increased autophagy levels, thereby alleviating mitochondrial dysfunction and reducing the accumulation of reactive oxygen species (ROS).In-depth studies have found that the autophagy inhibitor 3-methyladenine (3-MA) weakens the regulatory effect of compound 19 on autophagy and inhibits the clearance of damaged cells, resulting in oxidative stress damage, apoptosis and necrosis. By knocking down LRP6, it was found that the protective effect of compound 19 was eliminated, the autophagy level was significantly reduced, oxidative stress and ROS production were induced, and apoptosis after cisplatin exposure was promoted. Finally, the inhibitor LiCl was used to suppress the expression of GSK3β. It was found that inhibiting GSK3β could protect cells from cisplatin-induced damage by activating autophagy.DiscussionThese findings suggest that compound 19 is capable of preventing ototoxicity by activating autophagy via the LRP6/GSK3β axis and consequently inhibiting oxidative stress, offering a new approach for treating CIO and sensorineural hearing loss.

Comorbidities Associated with Vitiligo: Results from the EpiChron Cohort.

Background: Vitiligo is a pigmentation disorder that impacts approximately 0.5% to 2% of the global population. Growing interest surrounds the comorbidities associated with vitiligo. This study aimed to describe the socio-demographic characteristics of the patients with vitiligo in Aragón (Spain) and to investigate their associated comorbidities. Methods: A retrospective observational study was conducted using clinical data from individuals in the EpiChron Cohort (reference population of 1.3 million) who were diagnosed with vitiligo between 1 January and 31 December 2019. The prevalence of chronic comorbidities was calculated using logistic regression models, obtaining the odds ratio (OR) of each comorbidity (dependent variable) according to the presence or absence of vitiligo (independent variable). We used a cut-off point for a statistical significance of p-value < 0.05. Results: In total, 218 patients diagnosed with vitiligo were analyzed. The mean age was 44.0 years, and 56.42% were female. The largest proportion of patients (34.86%) were aged between 18 and 44 years. Among all vitiligo patients included, 71.5% presented multimorbidity, with an average of 3.21 diagnosed comorbidities. The conditions most frequently associated with vitiligo included thyroid disorders (OR: 3.01, p < 0.001), ocular and hearing abnormalities (OR: 1.54, p < 0.020), inflammatory skin disorders (OR: 2.21, p < 0.001), connective tissue diseases (OR: 1.84, p < 0.007), lower respiratory tract diseases (OR: 1.78, p < 0.014), urinary tract infections (OR: 1.69, p < 0.032), and cardiac arrhythmias (OR 1.84, p < 0.034). Conclusions: This research highlights the importance of understanding the broader health implications of vitiligo and provides a foundation for further exploration into the complex interplay between this dermatologic condition and a diverse range of comorbidities.

Audio-vestibular dysfunction in rheumatoid arthritis associated secondary Sjogren syndrome, and its correlation to serum level of anticardiolipin and muscarinic type 3 receptors antibodies: a cross-sectional observational study

BackgroundIn secondary Sjogren syndrome (sSS), the presence and extent of hearing and vestibular abnormalities are controversial. Hence, to ascertain if individuals with Rheumatoid arthritis (RA) associated secondary Sjogren syndrome (sSS) had subclinical audio-vestibular impairment as well as to investigate whether serum anticardiolipin and muscarinic receptors type 3 antibodies had a role in its pathogenesis, this cross-sectional study was conducted on 50 RA patients with secondary Sjogren syndrome manifestations and 50 RA patients without such manifestations were included, along with 50 apparently healthy volunteers who were aged, and sex-matched as controls. Full history taking, Otologic examination, Immunologic evaluation, and Audio-vestibular assessment were done for all subjects.ResultsTwenty-five percent of our patients experienced subjective audio-vestibular symptoms. In the audiogram, 30 (60%) of the 50 RA patients with sSS and 5 (10%) of RA patients without sSS had abnormal hearing loss compared to only 4 (8%) of the controls (p ≤ 0.001) with a predominance of high-frequency sensorineural hearing loss. Also, 20% of our patients experienced a higher frequency of head-shaking nystagmus than they did (p ≤ 0.001). 30% had a statistically significant increase in the frequency of abnormal caloric test results as compared to either controls (0%) or RA patients without sSS (0%); p ≤ 0.001 Finally, all audio-vestibular symptoms and tests exhibit a strong positive correlation with muscarinic receptors type 3 and anticardiolipin antibodies.ConclusionThe use of muscarinic receptors type 3 and anticardiolipin antibodies are beneficial for early diagnosis of audio-vestibular dysfunction in RA-associated secondary Sjogren syndrome patients. Therefore, this study is important since it justifies rheumatologists’ screening for inner ear damage in their RA patients especially if associated with secondary Sjogren.

Pre-op Deaf Post-op Dumb: A Case Report of a Tectal Plate Glioma and a Brief Literature Review

Any disorder of the brain stem that involves the auditory pathways and/or centers may produce hearing abnormalities. Auditory pathways are tonotopically organized, and they cross the midline at several points both below and at the level of the inferior colliculus but the central nucleus of the inferior colliculus and higher centers have a clear contralateral dominance. Ipsilateral auditory abnormalities are caused by lesions below or within the cochlear nuclei; bilateral auditory abnormalities are caused by lesions rostral to cochlear nuclei or they may be silent. A patient with a tectal plate glioma can present with bilateral hearing impairment due to the involvement of both inferior colliculi. Our case is a 28-year-old male who presented with progressive bilateral hearing impairment and a generalized headache despite normal pure-tone audiometry. Computed tomography (CT) of the brain revealed a tectal plate glioma (TPG) extending into the right thalamus, and an obstructive hydrocephalus. Following a ventriculoperitoneal shunt for cerebrospinal fluid (CSF) diversion, navigation-guided tumour excision was performed via a supracerebellar infratentorial approach. Postoperatively he developed unexpected aphasia probably due to ischemic changes in the cerebellar vermis and a focus of the adjacent right cerebellar hemisphere. This case highlights the rarity of bilateral auditory impairment in a TPG, surgical challenges posed by the tumour’s location, and potentially rare post-op complication of cerebellar mutism syndrome.

The Importance of Hearing Screening and Central Auditory Processing in School-Aged Children.

The aim of the study was to evaluate the prevalence of undiagnosed hearing impairment or central auditory processing disorders in children from I and VIII grades of primary schools in Warsaw. The participants in the study were 15,659 pupils from classes I and VIII attending primary schools in Warsaw. As part of the study, the hearing threshold for air conduction at frequencies of 0.5-8 kHz was determined, and a test assessing central auditory processing was performed: FPT (Frequency Pattern Test), DDT (Dichotic Digit Test), the Auditory Behaviour Scale (SAB), and an interview questionnaire developed for the program. Abnormal test results were found in 1946 children, of which abnormal hearing screening test results were found in 678 children tested, while reduced central auditory processing results were found in 1268 children. The program implemented draws attention to the fact that tests of central auditory processing are included in the testing protocol. As has been shown, peripheral hearing testing alone is not sufficient to exclude abnormalities concerning the sense of hearing.

Age-Related Hearing Loss: A Cross-Sectional Study of Healthy Older Australians

Introduction: Hearing loss is common in ageing populations, but thorough investigation of factors associated with objective hearing loss in otherwise healthy, community-dwelling older individuals is rare. We examined prevalence of age-related hearing loss (ARHL) in healthy, community-dwelling older adults, and determined whether sociodemographic, lifestyle, or health factors associate with hearing thresholds. Audiometry assessment was investigated with self-reports of hearing loss and hearing handicap. Methods: Australian participants (n = 1,260) of median age 73 years (IQR 71–76) joined ASPirin in Reducing Events in the Elderly (ASPREE)-Hearing, a sub-study of the ASPREE trial with exclusions including cognitive impairment, cardiovascular disease, independence-limiting physical disability, and uncontrolled hypertension. ASPREE collected demographics, anthropometrics, lifestyle, and health data. Audiometry measured better ear pure-tone average (PTA) across four frequencies (0.5–4 kHz) to establish hearing thresholds, categorised as normal or mild, moderate, and severe hearing loss. Questionnaires collected perceived hearing problems and noise exposure. Results: ARHL prevalence by audiometry was 49.7%, affecting men (59%) more than women (41%). A majority (54.5%) self-reported some hearing problems which mostly aligned with objective assessments; 45.6% self-reported a “little trouble” with hearing, while 35% had objective mild hearing loss; 8.3% reported having a “lot of trouble” hearing, while 13% had moderate hearing loss; and 0.6% reported being “deaf,” while 2% demonstrated severe hearing loss. There was a significant association (p < 0.001) between self-reported hearing handicap and audiometric measures of hearing loss. In multivariate analysis of health, demographics, and lifestyle risk factors, only age, gender (men), and education years (<12) remained associated (p < 0.05) with hearing loss. Hearing thresholds were not associated with smoking, living situation, alcohol use, hypertension, diabetes, or chronic kidney disease. Conclusion: ARHL robustly assessed by audiometry is common among healthy older Australians with men more likely to have abnormal hearing thresholds than women. Hearing loss was associated with fewer years of formal education, but not with a range of chronic conditions or alcohol use. Self-reported hearing loss correlates well with higher PTA hearing threshold levels in this healthy cohort where prevalence was lower than previously reported for the age group 70+ years. Hearing health education remains an important public health tool for this age. Targeting hearing in older patient health checks could be beneficial to mitigate the cognitive, social, and mental health consequences of ARHL, even if patients do not report a problem or handicap.

Case series; NUS1 deletions cause a progressive myoclonic epilepsy with ataxia

PurposeMutations in NUS1 cause a neurological congenital glycosylation disorder which encompasses a spectrum from developmental encephalopathy to musculoskeletal, hearing, and visual abnormalities. Pathogenic variants include both point mutations and genomic deletions. We report an adult phenotype of progressive myoclonus epilepsy (PME) and a review of cases with a complete or partial deletion of NUS1. MethodsOur patient, currently age 30, presented with an intellectual disability and developed progressive ataxia with myoclonic tremor, alongside generalised absence and tonic-clonic seizures. At age 28 he was diagnosed with a heterozygous 5.0 Mb deletion of 6q22.1q22.31 involving the NUS1 gene. We are unable to state whether this is a de novo mutation; his mother tested negative for the gene, but his father passed away before any genetic analysis could be performed. Along with the 22 patients reported in published literature, we identified 21 other genetically similar NUS1 deletion variants with sufficient clinical data through ClinVar. ResultsThe identification of NUS1 gene deletion disorder does not lead to a change in treatment but predicts a progressive clinical trajectory. Recognition of this helps differentiate neurological progression from the impact of anti-seizure medicine. ConclusionCopy number variants are an often-overlooked cause of PME. We also describe features of psychosis and spasticity and suggest that these may also be due to the NUS1 deletion, expanding the literature that exists on the phenotype of this very rare genetic disorder.

Hearing impairment following surgically repaired congenital heart disease in children: a prospective study

ObjectivesTo determine the prevalence of sensorineural hearing loss (SNHL) in children who underwent congenital cardiac surgery (CCS) by using a pre- and postoperative hearing test, a conventional audiometry, an extended...

An Analysis of Hearing Outcomes in Children with Hutchinson-Gilford Progeria Syndrome

Aims/Background Few hearing loss studies have been conducted in patients with progeria, and only the possibility of low-frequency conductive hearing loss has been mentioned. The primary objective of this study is to perform a comprehensive analysis of the clinical audiological characteristics of children with Hutchinson-Gilford progeria syndrome (HGPS), and the secondary objective is to analyse the causes of their hearing loss and what can be done to enable them to hear as well as possible. Methods Ten children with HGPS underwent impedance audiometry (tympanogram), otoacoustic emissions, and pure-tone audiometry tests. Otoscopic examination and imaging were also performed in the patients with abnormal hearing. Results Five patients had normal hearing, while the other five had varying degrees of hearing impairment manifesting as mild to moderate low-frequency or full-frequency conductive hearing loss. Otoscopic examination of those patients with abnormal hearing showed a narrowing of the external auditory canal, a thinning of the tympanic membrane, and a distorted cone of light. Further, computed tomography of one patient showed a poorly defined temporal bone morphology with scant pneumatization of the mastoid process. Conclusion Patients with HGPS differ from normally aging individuals with a predominant conductive pattern of hearing loss as opposed to sensorineural deafness, with more lower-frequency hearing impairment due to poor pneumatization of the mastoid process and the possible formation of osteophytes in the temporal bone.

MGA-related syndrome: A proposed novel disorder

MGA (OMIM: 616061) encodes a dual-specificity transcription factor that regulates the expression of Max-network and T-box familytarget genes, important in embryogenesis. Previous studies have linked MGA to various phenotypes, including neurodevelopmental disorders, congenital heart disease, and early-onset Parkinson's disease. Here, we describe the clinical phenotype of individuals with de novo, heterozygous predicted loss-of-function variants in MGA, suggesting a unique disorder involving both neurodevelopmental and congenital anomalies. In addition to developmental delays, certain congenital anomalies were present in all individuals in this cohort including cardiac anomalies, male genital malformations, and craniofacial dysmorphisms. Additional findings seen in multiple individuals in this cohort include hypotonia, abnormal brain imaging, hearing loss, sleep dysfunction, urinary issues, skeletal abnormalities, and feeding difficulties. These findings provide support for MGA as a gene intolerant to protein truncation with a broad phenotypic spectrum.

Quantitative analysis of the characteristics and influencing factors of hearing loss among workers exposed to noise in Tianjin

Objective: To analyze the characteristics of high-frequency average hearing loss in both ears of noise exposed workers in Tianjin in 2020, and quantitatively analyze the influencing factors of high-frequency hearing loss in both ears of workers. Methods: In March 2023, Collect and organize basic information about noise-hazardous enterprises and personal information of workers exposed to noise. Data from the Tianjin Occupational Disease and Health Hazard Factors Information Monitoring System from January 2020 to December 2020, and analyze the impact of basic information of employees, enterprise size, regional distribution, industry category, and economic type on the high-frequency average hearing loss of workers during work. Apply logistic regression to quantitatively analyze the influencing factors of abnormal high-frequency average hearing threshold of noise exposed workers. Results: The size, economic type, industry category, and regional distribution of enterprises, as well as the gender, age, length of service of workers, have an impact on the abnormal high-frequency average hearing threshold of noise exposed workers (χ(2)=733.56、3 497、27、1352.84、1197.62、2570.59、22.30、506.60, P<0.001) . Quantitative analysis using a logistic regression model showed that in the basic information of workers, noise exposed workers were male (OR=2.500, P<0.001) and aged 30-39, 40-49, and 50-59 years (OR=1.33, P<0.001; OR=1.68, P<0.001; OR=1.52, P< 0.001) , with a length of service of 4 to<10 years and≥10 years (OR=1.08, P<0.001; OR=1.615, P<0.001) being the influencing factors for high-frequency hearing loss in both ears of noise exposed workers; In terms of enterprise characteristics, medium-sized, small and micro enterprises (OR=1.12, P<0.001; OR=1.75, P<0.001; OR=2.09, P<0.001) , enterprises located in the fourth district around the city (OR=1.268, P<0.001) , and enterprises with economic types of collective economy, other economy, private economy, Hong Kong, Macao and Taiwan investment, shareholding system, and other industry economies (OR are all >1, P<0.001) are all factors affecting high-frequency hearing loss in noise exposed personnel. Conclusion: Noise is a common occupational hazard factor in Tianjin's enterprises, especially for workers in micro enterprises who face a high risk of hearing abnormalities. Therefore, enterprises need to strengthen the management and intervention of noise operations to prevent the occurrence of hearing loss in workers.

Hearing abnormalities in patients treated with teprotumumab.

Hearing abnormalities in patients treated with teprotumumab.

Audiological Characteristics of Vestibular Schwannoma Patients With Normal Pure-Tone Audiometry.

To investigate the audiological characteristics of vestibular schwannoma (VS) patients with normal pure-tone audiometry (PTA) results. A retrospective study. Forty-two VS patients with normal PTA results from October 2016 to October 2022 were included. Normal PTA was defined when the hearing threshold is ≤25 dB hearing loss (HL) in each test frequency and the PTA is ≤25 dB HL. Results of multiple audiological tests such as the auditory brainstem response (ABR), distortion product otoacoustic emission (DPOAE), multiple auditory steady-state responses threshold (ASSR), and speech discrimination score were retrospectively reviewed. Demographic data of these patients were also been collected. According to our results, the ABR and average ASSR threshold of the affected side were statistically significantly higher in VS patients with normal PTA. ABR waveforms on the affected side also showed more abnormalities. The DPOAE pass rates of the affected side were lower than the unaffected side while the amplitude and signal-to-noise ratio rate was also lower. In addition, we used magnetic resonance imaging 3-dimensional reconstruction images to measure the volume of tumors in these patients. We also found that higher ABR threshold means lager tumor size in patients with normal PTA. VS patients with normal PTA result cannot be assumed to have no impairment of hearing function. ABR, DPOAE, and ASSR results showed the characteristic changes in the affect ear. ABR threshold has the highest sensitivity for hearing abnormalities and is strong relative with tumor size in patients with normal PTA.

Effects of Maxillary Expansion on the Ear - A Review

Rapid maxillary expansion is a commonly used treatment modality for the correction of maxillary transverse deficiency in cleft and non-cleft patients. Additional beneficial effects of rapid maxillry expansion includes improvement in nasal stenosis, abnormal breathing pattern and conductive hearing loss. The improvements in the hearing have been linked to the improved function of the two soft palatal muscles, the tensor veli palatini and the levator veli palatini muscles. This study aims to explore the different studies which have evaluated the effects of rapid maxillary expansion on hearing in cleft and non cleft patients.

Three-Dimensional-Printed GelMA-KerMA Composite Patches as an Innovative Platform for Potential Tissue Engineering of Tympanic Membrane Perforations.

Tympanic membrane (TM) perforations, primarily induced by middle ear infections, the introduction of foreign objects into the ear, and acoustic trauma, lead to hearing abnormalities and ear infections. We describe the design and fabrication of a novel composite patch containing photocrosslinkable gelatin methacryloyl (GelMA) and keratin methacryloyl (KerMA) hydrogels. GelMA-KerMA patches containing conical microneedles in their design were developed using the digital light processing (DLP) 3D printing approach. Following this, the patches were biofunctionalized by applying a coaxial coating with PVA nanoparticles loaded with gentamicin (GEN) and fibroblast growth factor (FGF-2) with the Electrohydrodynamic Atomization (EHDA) method. The developed nanoparticle-coated 3D-printed patches were evaluated in terms of their chemical, morphological, mechanical, swelling, and degradation behavior. In addition, the GEN and FGF-2 release profiles, antimicrobial properties, and biocompatibility of the patches were examined in vitro. The morphological assessment verified the successful fabrication and nanoparticle coating of the 3D-printed GelMA-KerMA patches. The outcomes of antibacterial tests demonstrated that GEN@PVA/GelMA-KerMA patches exhibited substantial antibacterial efficacy against Staphylococcus aureus, Pseudomonas aeruginosa, and Escherichia coli. Furthermore, cell culture studies revealed that GelMA-KerMA patches were biocompatible with human adipose-derived mesenchymal stem cells (hADMSC) and supported cell attachment and proliferation without any cytotoxicity. These findings indicated that biofunctional 3D-printed GelMA-KerMA patches have the potential to be a promising therapeutic approach for addressing TM perforations.

Rapid hearing threshold assessment with modified auditory brainstem response protocols in dogs.

Auditory brainstem response (ABR) is the gold standard for hearing testing in dogs. ABR is commonly used in puppies to diagnose congenital sensorineural deafness. Long test times limit the use for a more comprehensive hearing screening in veterinary practice. This study aimed to establish a super-fast hearing screening protocol in dogs. Hearing thresholds were routinely measured with a mobile device designed for newborn hearing screening in 90 dogs. We introduced modifications of the ABR protocol, e. g., a binaural test mode, higher stimulus rates, a broadband chirp stimulus, and an algorithm for automatic peak V detection in a stepwise fashion. Hearing thresholds were then measured with fast protocols utilizing either 30 Hz click or 90 Hz broadband chirp stimuli with 80, 60, 40, 30, 20, 10, 0 and -10 dBnHL stimulation intensities. Interrater reliability, agreement between click and chirp hearing thresholds and correlations with clinical characteristics of the dogs were assessed. Using all innovations, the test time for hearing threshold assessment in both ears was reduced to 1.11 min (mean). The chirp stimulus accentuated both, peak V and the subsequent trough, which are essential features for judgement of the hearing threshold, but preceding peaks were less conspicuous. Interrater reliability and agreement between click and chirp hearing threshold was excellent. Dogs >10 years of age and dogs with abnormal hearing score or otitis score had significantly higher hearing thresholds than younger dogs (p ≤ 0.001) or dogs without abnormalities (p < 0.001). The results demonstrate that modifications in ABR protocols speed-up test times significantly while the quality of the recordings for hearing threshold assessment is maintained. Modified ABR protocols enable super-fast hearing threshold assessment in veterinary practice.

Application of speech induced ABR in rehabilitation intervention for hearing impaired children

Objective:Exploring the electrophysiological changes of auditory rehabilitation in young children with hearing impairment, providing more methods for early assessment and intervention. Methods:Twenty children aged 2-4 were enrolled, with moderate hearing loss and no other abnormalities in the ears. Divide them into group 1 with normal hearing, group 2 with abnormal hearing, group 3 with abnormal hearing receiving hearing aid intervention for one year, and group 4 with abnormal hearing receiving hearing aid and language training rehabilitation for one year. The SmartEP auditory evoked potential instrument was used to detect speech induced ABR and conduct screening for 'Standards and Evaluating Hearing and Language Abilities of Children with Hearing Impairment in 80 enrolled children after rehabilitation training, and the latency、amplitude of speech induced ABR waveform and evaluation scale scores for each group after rehabilitation intervention were compared. Results:Compared with the normal group, the latency of each wave in the other three groups was prolonged. The differences in each wave between Group 2 and Group 3 were statistically significant, while the differences in D and F waves between Group 3 and Group 4 were statistically significant. Compared with the normal group, the maximum amplitude at F0 decreased in the other three groups, and the differences in maximum amplitude between Group 2 and Group 3, Group 2 and Group 4, and Group 3 and Group 4 were statistically significant. Compared with the normal group, the scores of the auditory language assessment scale in the hearing intervention group and the hearing aid plus language training group were significantly higher than those in the abnormal group in terms of recognition rate. The recognition rates of hearing impaired children with language training foundation are similar to those of the normal group of children. Conclusion:Auditory rehabilitation can alter the electrophysiological aspects of hearing and serve as a basis for early assessment and intervention in young children.

Long-term auditory monitoring in children with Alport syndrome based on different degrees of renal injury

Objective:To investigate long-term auditory changes and characteristics of Alport syndrome（AS） patients with different degrees of renal injury. Methods:Retrospectively analyzing clinical data of patients diagnosed AS from January 2007 to September 2022, including renal pathology, genetic detection and hearing examination. A long-term follow-up focusing on hearing and renal function was conducted. Results:This study included 70 AS patients, of which 33（25 males, 8 females, aged 3.4-27.8 years） were followed up, resulting in a loss rate of 52.9%.The follow-up period ranged from 1.1to 15.8 years, with 16 patients followed-up for over 10 years. During the follow-up, 10 patients presenting with hearing abnormalities at the time of diagnosis of AS had progressive hearing loss, and 3 patients with new hearing abnormalities were followed up, which appeared at 5-6 years of disease course. All of which were sensorineural deafness. While only 3 patients with hearing abnormalities among 13 patients received hearing aid intervention. Of these patients,7 developed end-stage renal disease（ESRD）, predominantly males （6/7）. The rate of long-term hearing loss was significantly different between ESRD group and non-ESRD group（P=0.013）. There was no correlation between the progression of renal disease and long-term hearing level（P>0.05）. kidney biopsies from 28 patients revealed varying degrees of podocyte lesion and uneven thickness of basement membrane. The severity of podocyte lesion was correlated with the rate of long-term hearing loss（P=0.048）, and there was no correlation with the severity of hearing loss（P>0.05）. Among 11 cases, theCOL4A5mutationwas most common （8 out of 11）, but there was no significant correlation between the mutation type and hearing phenotype（P>0.05）. Conclusion:AS patients exhibit progressive hearing loss with significant heterogeneity over the long-term.. THearing loss is more likely to occur 5-6 years into the disease course. Hearing abnormalities are closely related to renal disease status, kidney tissue pathology, and gene mutations, emphasizing the need for vigilant long-term hearing follow-up and early intervention.

Ab. No. 167 Association between Balance, Cognition and Quality of Life in Middle Aged Postmenopausal Women: A Cross Sectional Study

Introduction: The hormonal changes occurring in women during and after menopause transition have a relevant impact on physical performance and health-related quality of life. The use of challenging balance conditions to detect sub clinical risk of balance impairment has been reported in aging population. Postmenopausal women may experience a decline in cognitive skills in their daily lives. A decrease in functional performance and cognitive function may lead to poor quality of life in postmenopausal women. There are studies reporting that low physical function can predict low cognitive function during menopause. The aim of the study is to find out association between balance, cognition and quality of life of middle-aged postmenopausal women. Methods: Middle aged 102 postmenopausal females aged 40-60years were included for study. A cross sectional study was conducted among 102 postmenopausal women who had natural history of menopause were included. Any women who had hysterectomy, hormonal replacement therapy, any severe musculoskeletal impairment, abnormal hearing and vision were excluded. Data was collected using the menopause specific quality of life questionnaire, timed up and go test for dynamic balance and Montreal cognitive assessment used for level of cognition. Result: Total 102 postmenopausal females aged 49.66±3.46 years. Mean scores for TUG (9.27 + 1.25), MOCA (21.46 + 2.90) and MENQOL (40.37 + 8.34) were seen. Correlation was found between TUG and MOCA were (p=.009) (r=.928), between TUG and MENQOL were (p=.056) (r=.574) and MENQOL with MOCA were(p=-.031) (r=.756) statistically significant. Conclusion: There is positive relationship between balance, cognition and quality of life is seen in middle aged postmenopausal females. Implications: Assessing and intervening early in the functional performance is essential to maintain and preserve cognitive function in aging by tailored cognitive motor training with holistic approach to maintain quality of life.

Clinical spectrum of congenital Zika virus infection in Brazil: Update and issues for research development.

This review aimed to provide an update on the morphological and/or functional abnormalities related to congenital Zika virus (ZIKV) infection, based on primary data from studies conducted in Brazil since 2015. During the epidemic years (2015-2016), case series and pediatric cohort studies described several birth defects, including severe and/or disproportionate microcephaly, cranial bone overlap, skull collapse, congenital contractures (arthrogryposis and/or clubfoot), and visual and hearing abnormalities, as part of the spectrum of Congenital Zika Syndrome (CZS). Brain imaging abnormalities, mainly cortical atrophy, ventriculomegaly, and calcifications, serve as structural markers of CZS severity. Most case series and cohorts of microcephaly have reported the co-occurrence of epilepsy, dysphagia, orthopedic deformities, motor function impairment, cerebral palsy, and urological impairment. A previous large meta-analysis conducted in Brazil revealed that a confirmed ZIKV infection during pregnancy was associated with a 4% risk of microcephaly. Additionally, one-third of children showed at least one abnormality, predominantly identified in isolation. Studies examining antenatally ZIKV-exposed children without detectable abnormalities at birth reported conflicting neurodevelopmental results. Therefore, long-term follow-up studies involving pediatric cohorts with appropriate control groups are needed to address this knowledge gap. We recognize the crucial role of a national network of scientists collaborating with international research institutions in understanding the lifelong consequences of congenital ZIKV infection. Additionally, we highlight the need to provide sustainable resources for research and development to reduce the risk of future Zika outbreaks.