Hearing Impairment Research Articles

Group Play Therapy for Children with Hearing Loss

Objectives: Children with hearing loss often undergo considerable pain and distress due to their hearing impairment, leading to challenges in engaging constructively with others. Using games, mainly through play therapy, can significantly enhance interaction and adjustment in children, especially those with hearing impairments. This research seeks to evaluate the impact of play therapy on the adaptation and self-confidence of children dealing with hearing challenges. Methods: This randomized controlled trial was conducted at a welfare center affiliated with the Hamadan University of Medical Sciences in western Iran. Sixty-two children with hearing loss were selected from the welfare center. They were then assigned to the play therapy or control group using block random sampling. The pre- and post-intervention assessments included completing demographic information, an adaptability questionnaire for elementary school children, and a self-esteem inventory. Results: The implementation of play therapy led to a significant increase in adaptability immediately after the intervention and one month later (P<0.001). Moreover, a notable difference was observed between the two groups at the end of the study (P<0.001). Throughout the research, play therapy consistently elevated the self-esteem of the children. After the study, a noticeable difference was observed between the two groups (P<0.001). Discussion: lay therapy effectively increased adaptability and improved self-esteem in children with hearing loss. So, caregivers can use play therapy as therapeutic care to increase social adaptation and self-confidence in these children.

Artificial Intelligence in Sign Language Recognition: A Comprehensive Bibliometric and Visual Analysis

Sign language recognition (SLR) plays a crucial role in bridging the communication gap between individuals with hearing impairments and the auditory communities. This study explores the use of artificial intelligence (AI) in SLR through a comprehensive bibliometric analysis of 2,720 articles published from 1988 to 2024. Utilizing tools like VOSviewer and CiteSpace, the research uncovers the landscape of publication outputs, influential articles, leading authors, as well as the intellectual framework of current topics and emerging trends. The findings indicate that since the inception of SLR research in 1988, there has been a rapid expansion in the field, particularly from 2004 onwards. China and India lead in research productivity. Keyword and co-citation analyses highlight that Hidden Markov Model, Kinect, and Deep Learning have been focal points at various stages of SLR development, while transfer learning, Bidirectional Long Short-Term Memory, attention mechanisms, and Transformer models represent recent emerging trends. This research offers valuable insights for scholars and practitioners interested in AI-based SLR.

Oxidative and endoplasmic reticulum stress in diabetes-related hearing loss: Protective effects of thioredoxin

Diabetes mellitus (DM) induces complex physiological changes in the inner ear environment. This study investigates the roles of oxidative stress (OS) and endoplasmic reticulum stress (ERS) in diabetic hearing loss and explores the potential of thioredoxin (Trx) in regulating OS, ERS, and apoptosis-related factors to mitigate the progression of hearing impairment. We conducted auditory and serological assessments in 63 patients with type 2 diabetes and 30 healthy controls. Type 2 diabetes models were induced in wild-type and Trx transgenic (Tg) mice, with auditory brainstem response (ABR) used to evaluate hearing changes. Cochlear tissues were isolated to analyse markers of apoptosis, OS, and ERS. Both patients with diabetes and mouse models exhibited hearing loss, alongside increased serum levels of Trx1, TXNIP, and AOPP, indicating oxidative damage. H&E and succinate dehydrogenase (SDH) staining revealed varying degrees of hair cell loss from the base to the apex of the cochlea in diabetic mice, with decreased expression of the hair cell protein prestin gene. Notably, Tg mice showed significant delay in hearing loss progression. In vitro, advanced glycation end-products (AGEs) induced OS and ERS in cochlear-like HEI-OC1 cells, while Trx overexpression enhanced Nrf2 activity, alleviating AGE-induced cellular stress. In conclusion, Trx exhibits protective effects against diabetes-related hearing loss, potentially by enhancing Nrf2/HO-1/SOD2 function to reduce OS and ERS.

Congenital Cytomegalovirus Infection and Hearing Loss: It's Time to Screen.

Congenital CMV infection is the leading nongenetic cause of sensorineural hearing loss worldwide, yet most parents have never heard of it. The majority of infected newborns have no clinical signs of infection, although a substantial proportion may have hearing loss at birth or develop it later in life. As antiviral treatment with ganciclovir or valganciclovir initiated in the first month of age improves audiologic outcomes, there is an urgent need for timely identification of infected neonates. A targeted approach that tests neonates who refer on the newborn hearing screen has been implemented in many states and hospital programs, but it fails to identify about 40% of children who experience CMV-related hearing loss. A universal screening approach is optimal given the prevalence of congenital CMV infection, its associated sequelae, the availability of a simple saliva screening tool, the available antiviral treatment, and the directed therapies for hearing impairment.

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.

Assessing Current Knowledge of Hearing Impairment With Diabetes by Surveying Providers With CBDCE Certification.

The purpose of this study was to assess current knowledge, attitudes, and practices regarding hearing impairment and screening referrals in patients with diabetes among providers who have a Certified Diabetes Care and Education Specialist (CBDCE) Certification. A cross-sectional survey created through Qualtrics was emailed to health care providers in the United States with CDCES certification. Providers responded regarding knowledge and importance of hearing impairment compared to other diabetes complications, when they would refer a patient to an audiologist, the percentage of patients referred, and awareness of over-the-counter hearing aids. Descriptive statistics were calculated for all questionnaire items. One thousand four hundred and ninety-five CDCES providers completed the survey. Participants selected the most common conditions associated with diabetes as kidney dysfunction (96.7%), retinopathy (96.5%), obesity (95.6%), and foot infection (94.5%); 44.5% chose hearing impairment. Over 60% of providers were not familiar with how to refer patients to an audiologist and acknowledged being unfamiliar with recommended screening frequency as the most common barrier. Most providers had referred fewer than 20% of patients to an audiologist. Over half of providers were not aware of over-the-counter hearing aids. Among a national sample of health care providers with comprehensive knowledge in diabetes care, many providers do not associate hearing impairment with diabetes and rate other microvascular complications of higher importance.

Risk factors for cognitive decline in older Chinese adults: the impact of social activity on the relationship between hearing, functional mobility, and cognition

ObjectiveHearing and functional mobility impairments are recognized as risk factors for cognitive decline in older adults, yet the causal relationship underlying these associations is not well-understood. This study aims to explore whether engagement in social activities mediates the link between hearing or functional mobility impairment and cognitive decline.MethodsThis cross-sectional study was carried out in two cities in Jiangsu Province, Eastern China. Participants self-reported hearing impairment and social activity engagement, whereas functional mobility impairment was assessed using the Timed Up and Go Test (TUGT). Cognitive function was evaluated through the Modified Mini-Mental State Examination (MMSE). Logistic regression analysis explored factors influencing cognitive function, and mediation analysis was conducted to examine the relationship between hearing or functional mobility impairment and cognitive decline.ResultsThe study included 10,217 adults aged 60 and above. Among them, 19.35% reported hearing impairment, while 40.86% failed the Timed Up and Go Test (TUGT). The Modified Mini-Mental State Examination (MMSE) indicated a 30.40% prevalence of cognitive decline. Logistic regression analysis identified significant associations of cognitive function with factors such as gender, age, education level, residency, living arrangement, hyperlipidemia, cerebrovascular disease, alcohol consumption, smoking, Activities of Daily Living (ADLs), Instrumental Activities of Daily Living (IADLs), social activity, hearing, and functional mobility (p < 0.01). Mediation analysis, after adjusting for confounders, showed that social activity engagement partially mediated the impact of functional mobility impairment on cognitive decline (indirect effect: −0.0947, 95% Bootstrapped CI: −0.1228, −0.0695; proportion of total effect: 11.635%, p < 0.01). However, no mediation effect was observed in the relationship between self-reported hearing impairment and cognitive decline.ConclusionThis study revealed that social activity engagement plays a mediating role in the relationship between functional mobility and cognitive function, but it does not significantly influence the relationship between self-reported hearing impairment and cognitive decline. These findings suggest that social activity engagement could be a crucial factor in preventing cognitive deterioration among older adults with functional mobility impairments.

Effectiveness of virtual reality glasses with integrated sign language in reducing dental anxiety during pulpotomy in children with hearing impairment: a randomized controlled trial.

Children with hearing impairment (HI) face communication challenges during dental procedures due to hearing loss. Studies suggest that distraction techniques, like virtual reality (VR), can effectively divert their focus from stressful stimuli, resulting in a more comfortable dental experience. The present study aims to assess the effectiveness of distracting children with moderate to severe (HI) with virtual reality glasses that show cartoons in sign language during pulpotomy treatment compared to conventional management techniques. Forty children aged five to seven participated in a randomized controlled parallel two-arm clinical trial-the type of behavioral management employed determined which two groups children were randomly placed into. Group I (Study group) used virtual reality glasses as a diversion, while Group II (Control group) used the conventional behavior management approach. Local anesthesia was administrated, and a pulpotomy procedure was performed on the selected tooth, followed by stainless steel crown restoration (SSC). There were three methods used to assess dental anxiety before and after the procedure: the physiological method, which used heart rate (HR); the objective measure, which used the Venham Clinical Anxiety Scale (VCAS); and the subjective measure, which used the modified Facial Affective Scale (FAS). An independent t-test was employed for HR analysis of the difference between the groups as a continuous variable. The Pearson Chi-square test assessed differences between groups for categorical variables, such as (VCAS) (FAS). No significant differences were found in mean (HR) or (VCAS) between the two groups throughout the procedures: during local anesthesia (p = 0.659, 0.282), pulpotomy (p = 0.482, 0.451), and stainless steel crown preparation (p = 0.090, 0.284). Anxiety levels by (FAS) remained statistically comparable between the two groups before and after the procedures (p = 0.507, 0.749), respectively. The use of VR glasses revealed no significant advantages in managing children with HI during the dental visit compared to the conventional method of child behavior management. The trial was prospectively enrolled on 11/11/2023 under the identification number NCT06153823 on ClinicalTrials.gov.

Evaluating parental knowledge and attitudes toward childhood hearing loss: a cross-sectional study in Rawalpindi, Pakistan.

Childhood hearing loss poses a significant public health challenge in Pakistan, with prevalence rates over double the global average. Parental knowledge and attitudes play a crucial role in timely detection and intervention. However, limited research explores this issue within the Pakistani context. This study aimed to examine parental knowledge and attitudes regarding childhood hearing loss in Rawalpindi, Pakistan, and identify key demographic factors influencing parental perspectives. This cross-sectional survey was conducted in Rawalpindi, Pakistan from March 1 to August 31, 2023. A total of 150 parents (79 fathers and 71 mothers) of children aged 0-12 years were recruited through convenience sampling at public locations such as schools, malls, and hospitals. Data were collected through face-to-face interviews using the validated "Parental Knowledge and Attitudes towards Childhood Hearing Loss Questionnaire," administered by a graduate student to assess parental knowledge and attitudes about childhood hearing loss. Descriptive statistics determined the percentage of correct responses and knowledge/attitudes scores. Chi-square tests explored associations between demographic factors and parental perspectives. Overall, 67.3% of parents demonstrated good knowledge of childhood hearing loss. Notably, parents scored high on recognizing congenital causes (79.3%) but displayed low awareness of measles as a risk factor (29.3%). Significant gender disparities emerged in parental knowledge, with mothers exhibiting superior knowledge compared to fathers regarding newborn screening (63.4% vs. 46.8%), treatment availability (81.7% vs. 64.6%), and educational inclusion for children with hearing impairment (57.7% vs. 38.0%). Despite these knowledge gaps, parents generally held positive attitudes towards childhood hearing loss, with an average score of 94.7%. Gender and educational level significantly influence knowledge and attitudes. Although parents in Rawalpindi generally have a good understanding and positive attitudes towards childhood hearing loss, gaps in knowledge about preventable causes and gender disparities need attention. Providing tailored counselling for higher-risk, less educated groups and expanding policies for paediatric audiology services nationwide can help address these issues.

EARLY DETECTION AND LONGITUDINAL OUTCOMES OF HEARING IMPAIRMENT IN NICU GRADUATES AT SDM COLLEGE OF MEDICAL SCIENCES AND HOSPITAL

Objective: Hearing impairment is a prevalent congenital anomaly, particularly in neonates in NICUs, due to various risk factors. Early detection through screening programs is crucial for developmental outcomes. Methods: A prospective observational study was conducted at SDM College of Medical Sciences and Hospital, involving 425 neonates. Otoacoustic Emissions (OAE) testing was used for early detection and longitudinal outcome assessment. Results: Out of 425 neonates, 95.3% passed the initial OAE screening. However, 4.7% failed, necessitating further evaluation. Notable risk factors included neonatal jaundice and perinatal asphyxia, particularly impacting neonates with lower birth weights. Conclusion: The study underscores the importance of early and universal hearing screening in NICU settings, emphasizing the need for tailored protocols to address specific neonatal risks.

Hearing impairment and vestibular function in patients with a pathogenic splice variant in the LHX3 gene.

LHX3 is a gene encoding a LIM-homeodomain transcription factor important for the fetal development of several organs, such as the pituitary gland, spinal motor neurons and the inner ear. Pathogenic and likely pathogenic variants in the LHX3 gene are infrequent and result in a rare syndrome known as combined pituitary hormone deficiency-3, CPHD3. We have studied hearing and vestibular functions in a group of eight individuals, aged 8-36years, all of whom were homozygous for a specific variant in the LHX3 gene at chromosome 9q34. We reexamined the results of consecutive hearing tests from newborn until April 2024. Our data showed that all the tested patients had progressive sensorineural hearing deficiency ranging from moderately severe to complete loss. We have performed vestibular testing in six patients and, for the first time, demonstrated that a mutation in the LHX3 gene not only affects hearing, but is also associated with vestibular impairment. The human pathogenic variant c.455-2A > G in the LHX3 gene on chromosome 9q34, which present as a founder mutation in the population in northern Sweden, is responsible for phenotypes associated with progressive hearing loss and balance impairment. These findings prove that the LHX3 gene is crucial for the function of both the cochlear and vestibular organs.

Low Bone Mineral Density Is Associated with High-Frequency Hearing Impairment in Women Over 50: An Observational Study in Korea.

Osteoporosis and hearing impairment are known to be associated, but specific data regarding gender, bone mineral density (BMD) measurement sites, and hearing frequency ranges remain unclear. This study aimed to clarify the relationship between hearing loss and BMD in adults over the age of 50. Additionally, the study sought to determine the frequency ranges of pure tone audiometry (PTA) related to osteoporosis, identify BMD measurement sites, and investigate gender differences. A total of 1,523 adults (651 men and 872 women) over the age of 50, who participated in a medical health check-up at a university hospital, were included. PTA was conducted to assess hearing, and BMD was measured using dual-energy X-ray absorptiometry at the lumbar vertebrae (LV) and femur. In women over the age of 50, a significant association was observed between hearing impairment and osteoporosis (P<0.01), but no such association was found in men. Lumbar BMD (L1-4) in women was significantly associated with hearing loss at 4,000 and 8,000 Hz (both P<0.05), whereas femoral neck and total femur BMD showed no significant relationship. In multiple logistic regression analysis, the odds ratio (OR) between osteoporosis and hearing threshold at 4,000 Hz (OR, 2.078; 95% confidence interval [CI], 1.092-3.954) and 8,000 Hz (OR, 2.648; 95% CI, 1.543-4.544) remained statistically significant in women after adjusting for age and other risk factors. In women over the age of 50, low BMD at the LV is significantly associated with hearing impairment, particularly at the high frequencies of 4,000 and 8,000 Hz.

Behind Bars: Exploring Health and Geriatric Conditions Among Incarcerated Older People in Mexican prisons.

Introduction The aging process of the incarcerated population is a growing concern, yet there are few data on older adults in this demographic group. Hence, this study seeks to examine the health status of older adults who are incarcerated in Mexican prisons and its association with the duration of their imprisonment. Methods This is a secondary analysis of the 2021 Mexico National Prisons Survey. We analyzed 50-year-old and older prisoners, and performed a descriptive analysis of the sample's age, sex, sociodemographic variables, and chronic conditions. Multivariate analysis stratified by age was performed to assess the effect of the time spent in prison on older prisoners' health. Results The mean age was 56.95 (± 6.4 SD) and the mean duration of imprisonment was 8.93 years (± 6.94 SD). Regarding health conditions, 17.80% had diabetes, 29.62% had hypertension, 10.33% had suicidal ideation, 40.87% were visually impaired, 17.01% had hearing impairment, and 17.64% had mobility impairment. Multivariate analysis revealed that among categories of imprisonment duration, longer time imprisoned was associated with an increased risk of diabetes and hypertension for all groups but was not associated with mobility impairment or suicidal ideation except in the younger group. Conclusion Longer periods of incarceration appear to be associated with a greater occurrence of diabetes and hypertension in older prisoners. Sensory impairments and suicidal ideation are mainly identified in younger prisoners, while mobility impairments do not appear to be influenced by the time spent in prison. Further research needs to be done in prisons, where the addition of physical performance tests and cognitive tests could help further study geriatric conditions in older prisoners.

A Comparative Evaluation of the Genetic Variant Spectrum in the USH2A Gene in Russian Patients with Isolated and Syndromic Forms of Retinitis Pigmentosa

Pathogenic variants in the USH2A gene are the primary cause of both non-syndromic autosomal recessive inherited retinitis pigmentosa (RP) and the syndromic form, characterized by retinal degeneration and sensorineural hearing loss. This study presents a comparative assessment of the genetic variant spectrum in the USH2A gene among Russian patients in two clinical groups. A retrospective analysis was conducted on massive parallel panel sequencing data from 2415 blood samples of unrelated patients suspected of having hereditary retinal diseases. The copy number of USH2A exons was determined using the quantitative MLPA method with the MRC-Holland SALSA MLPA kit. Biallelic pathogenic and likely pathogenic variants in the USH2A gene were identified in 69 patients (8.7%). In the group of patients with isolated hereditary RP (55 patients), the most frequent pathogenic variants were p.(Glu4445_Ser4449delinsAspLeu) (20.9%), p.(Trp3955*) (15.5%), and p.(Cys934Trp) (5.5%). In patients with the syndromic form (14 patients), the most frequent variants were p.(Trp3955*) (35.7%) and c.8682-9A&gt;G (17.9%). It was found that patients with isolated vision impairment rarely had two “null” variants (17.8%), whereas this was common among patients with both hearing and vision impairment (71.4%) (p ≤ 0.05), explaining the severity of the disease and the earlier onset of clinical symptoms in the syndromic form of RP. Ten previously undescribed loss-of-function variants were identified. The estimated prevalence of USH2A-associated retinal dystrophy in Russia was 1.9 per 100,000 individuals. The obtained data on the differences in the spectra of genetic variants in the USH2A gene in the two studied groups highlight the importance of establishing genotype–phenotype correlations and predicting disease severity, aiming at potential early cochlear implantation and selection of target therapy.

PTSD Is Associated with Self-Perceived Hearing Handicap: An Evaluation of Comorbidities in Veterans Without Measured Hearing Loss.

Cases of self-reported hearing difficulty despite no traditionally measured hearing loss (pure tone audiometric thresholds ≤ 25 dB HL from 250 Hz through 8000 Hz) have risen with the return of Veterans from recent conflicts in Operation Enduring Freedom, Operation Iraqi Freedom, and Operation New Dawn. Auditory outcomes improved despite low compliance among those receiving treatment. Medical chart data appeared more comprehensive for Veterans with, rather than without, auditory complaints. One possibility is that self-reported hearing problems are associated with a subset of these comorbidities, the treatment of which improved auditory outcomes. This study examined the relationships between Veterans' self-reported auditory problems and other diagnosed medical conditions. A retrospective chart review was used. Participants were 286 Veterans, aged 21 to 52 years with pure-tone audiometric thresholds ≤ 25 dB HL at all measured frequencies in both ears. Veterans were dichotomized into a group with either self-reported hearing complaints (n = 143) or an age-matched control group with no auditory complaints (n = 143). A query of the Computerized Patient Record System was performed with the date range restricted to 2009 to 2018. Metrics of self-perceived hearing handicap, auditory processing disorder testing, and hearing aid use were collected. All diagnoses and related symptoms were recorded. A best subsets regression with principled model selection was performed to investigate the role of these comorbidities on self-perceived hearing loss. The self-report group had 16 comorbidities that were classified as prevalent, having occurred in ≥33.3% of the group, compared with the age-matched control group, which had 2 comorbidities. The number of diagnosed medical conditions was associated with self-perceived hearing impairment. Specifically, posttraumatic stress disorder (PTSD) and related symptom clusters constituted the largest group of comorbidities that were significantly associated with self-reported hearing problems. The significant association between PTSD and self-perceived hearing impairment warrants investigations on whether treatment of PTSD would reduce perceived hearing handicap severity. Further, PTSD assessments could be useful for audiologists to identify potential candidates for auditory complaints without measured hearing loss.

Audiometric Outcomes in Chronic Otitis Media with Mastoid Involvement: A Five-Year Clinical Overview

Background/Objectives. Otomastoiditis, an inflammatory condition affecting the middle ear and mastoid cells, poses significant risks for hearing impairment. This study aimed to analyze the clinical presentations, anatomical variations, and audiometric outcomes associated with acute and chronic otomastoiditis over a five-year period at the ENT Clinic of the Clinical County Emergency Hospital of Craiova. Methods. A retrospective clinical–statistical analysis was conducted on 145 patients aged 2 to 78 years, who were treated for otomastoiditis. The study involved a comprehensive review of clinical and audiometric data, with a focus on the type of hearing loss (conductive or mixed), audiometric thresholds, and the relationship between the anatomical form of the disease and the severity of hearing loss. Results. The majority of cases (93.83%) were chronic otomastoiditis, with 66.89% of patients presenting with mixed hearing loss and 33.10% with conductive hearing loss. Audiometric assessments revealed significant air conduction deficits, particularly at low and mid-range frequencies, with losses averaging 50–55 dB in cases of conductive hearing loss. Chronic cases demonstrated notable bone conduction impairments, indicating progressive cochlear damage. Statistical analysis identified a moderate correlation between the anatomical form of the disease and the severity of hearing loss, particularly in patients with cholesteatomatous-suppurative forms. Conclusions. This study underlines the critical need for the early and precise diagnosis of otomastoiditis, supported by audiometric evaluations. Our findings emphasize the substantial risk of progressive cochlear damage in chronic cases, underscoring the necessity for timely intervention to mitigate long-term hearing loss. These results offer valuable insights for clinicians, potentially guiding improved therapeutic approaches and contributing to enhanced patient outcomes in managing chronic otomastoiditis.

A Novel RNS Hardware Architecture of FRM Filter Banks for Digital Hearing Aids

This paper presents a novel hardware architecture for Frequency Response Masking (FRM) Filter Bank based on Residue Number System (RNS) arithmetic for Digital Hearing Aids (DHA). FRM is a computationally efficient approach for filter bank design that separates different frequencies based on the Audiograms of a patient. However, filter banks are critical power-hungry elements that require high computational resources. Therefore, the proposed architecture aims to provide an efficient implementation of FRM filter banks, which can improve the value and affordability of DHAs. The proposed architecture employs FIR filters in direct form, linear phase, and poly phase using RNS arithmetic. This approach enables the performance of arithmetic operations in parallel and independently. The concepts are further extended for the efficient implementation of FRM filter bank. The results of the proposed architecture show that direct implementation reduces the delay when the number of taps increases in the FIR filter. However, to reduce computational resources and optimize the area requirement, a Folded RNS architecture is also proposed. The Folded RNS architecture offers hardware savings of 23–27% for a 40-tap FIR filter and 29–31% for a 60-tap FIR filter. Additionally, it improves speed by 13–15% for a 40-tap FIR filter and 16–19% for a 60-tap folded RNS FIR filter. The implementation of FRM filter bank using the folded FIR filter shows the same trend in hardware saving and speed improvement as in the case of FIR filters. These results demonstrate that the proposed hardware architecture has the potential to improve the efficiency and affordability of DHAs while enhancing the listening comfort, which can enhance the quality of life for patients with hearing impairments.

Effect of Dielectric Properties of Cochlea on Electrode Insertion Guidance Based on Impedance Variation

The cochlear neuromodulator provides substantial auditory perception to those with impaired hearing. The accurate insertion of electrodes into the cochlea is an important factor, as misplaced may lead to further damage. The impedance measurement may be used as a marker of the electrode insertion guidance. It is feasible to investigate the impact of the dielectric properties of the cochlea tissue layers on the electrode insertion guidance using sophisticated bio-computational methods that are impractical or impossible to perform in cochlear implant (CI) patients. Although previous modeling approaches of the cochlea argued that the capacitive impact of the tissue layer can be neglected using the quasi-static (QS) approximation method, it is widely accepted that tissue acts as a frequency filter. Thus, the QS method may not always be appropriate due to short-duration pulses. This study aimed to investigate the impact of the frequency-dependent dielectric properties of the cochlea tissue layers on the impedance variation by following a systematic approach. The volume conductor model of the cochlea layers was developed, the dielectric properties of each tissue layer were attained, and the cochlea neuromodulator settings were applied to obtain the results based on both QS and transient solution (TS) methods. The results based on the QS and TS methods were compared to define to what extent these parameters affect the outcome. It was suggested that the capacitive impact of the cochlea layers should be considered after a certain frequency level.

Identification of a novel ST3GAL5 variant in a Chinese boy with GM3 synthase deficiency and literature review of variants in the ST3GAL5 gene.

GM3 synthase deficiency (GM3SD) is an autosomal recessive disorder resulting from mutations in the ST3GAL5 gene. It is characterized by intellectual disability, microcephaly, psychomotor and developmental delay, hearing and visual impairments, and changes in skin pigmentation. This study aims to broaden the genetic mutation spectrum of GM3SD through the report of a de novo mutation and a comprehensive summary of GM3SD phenotype to aid in genetic counseling and prenatal diagnosis. Compound heterozygous variants in ST3GAL5 (NM_003896.4: c.1000delC, p.Arg334Glufs*15 and c.207-1G > T, p.Cys70Glufs*81) were identified via trio-whole exome sequencing (trio-WES) and confirmed pathogenic through functional experiments. Notably, c.207-1G > T was a newly discovered variant. Additionally, previously reported GM3SD mutations were classified into R288X and non-R288X, revealing that R288X mutations were more likely to manifest developmental, emotional abnormalities, and severe feeding difficulties. This study reveals a novel mutation in ST3GAL5 and provides a comprehensive overview of GM3SD phenotype, aiding in the diagnosis and genetic counseling of GM3SD in clinical practice.

Characteristics of Hearing Impairment in Patients with Suspected Congenital Rubella Syndrome at the ENT Clinic of Prof. Dr. I.G.N.G Ngoerah Hospital Denpasar, Indonesia

Asymptomatic rubella virus infection can cause delays in the diagnosis of rubella in pregnant women, leading to the development of congenital rubella syndrome (CRS). The clinical manifestations of congenital rubella syndrome are called the rubella triad, which includes heart problems, eye problems, and hearing disorders. Sensorineural hearing loss is the most common type of hearing loss in children with rubella infection. This study aimed to determine the characteristics of hearing loss in CRS patients. This study used a retrospective descriptive research design, utilizing secondary data from the medical records of patients with CRS who underwent hearing examinations at the Ear, Nose, Throat (ENT) Polyclinic of Prof. dr. I.G.N.G. Ngoerah Hospital Denpasar, Indonesia. The study found that the most common age group among suspected CRS patients was 6 - 12 months (51.42%), the majority of these patients were male (62.86%), the most frequent clinical signs and symptoms in suspected CRS patients were congenital heart disease (CHD) as a major criterion (60%), and microcephaly as a minor criterion (77.14%). Based on the CRS case criteria, none of the suspected CRS patients were diagnosed with confirmed CRS, with most cases being classified as clinical CRS (65.71%). The hearing loss in suspected CRS patients was entirely sensorineural type with the majority of cases being bilaterally and to a very severe degree (53.57%)