Genetically modified pigs:Emerging animal models for hereditary hearing loss

Hereditary hearing loss(HHL),a genetic disorder that impairs auditory function,significantly affects quality of life and incurs substantial economic losses for society.To investigate the underlying causes of HHL and evaluate therapeutic outcomes,appropriate animal models are necessary.Pigs have been extensively used as valuable large animal models in biomedical research.In this review,we highlight the advantages of pig models in terms of ear anatomy,inner ear morphology,and electrophysiological characteristics,as well as recent advancements in the development of distinct genetically modified porcine models of hearing loss.Additionally,we discuss the prospects,challenges,and recommendations regarding the use pig models in HHL research.Overall,this review provides insights and perspectives for future studies on HHL using porcine models.

Modified intratympanic steroid therapy for sudden sensorineural hearing loss via tympanic tube and gelfoam as a salvage treatment

Background: Sudden sensorineural hearing loss(SSNHL) is a prevalent emergency in ear, nose, and throat practice. Previous studies have demonstrated that intratympanic steroid therapy(IST) can serve as a salvage treatment for SSNHL after the failure of systemic steroid therapy(SST).Objective: This study aimed to analyze the efficacy of modified IST involving the insertion of a tympanic tube and gelfoam as a salvage treatment for patients with SSNHL, and to explore its associated factors.Methods: Totally, 74 patients who were aged 22–81 years with SSNHL were enrolled and allocated to either the control group(n = 25) or the treatment group(n = 49) based on their treatment modalities. All patients received SST lasting for at least 7 days. Subsequently, patients in the treatment group, after SST failure, underwent IST twice a week for 2–6 weeks, while the control group did not. Efficacy was assessed by the improvement in pure tone average at the affected frequency at the beginning and end of IST.Results: Hearing improvement in all patients after IST in the treatment group was 9.71 ± 14.84 dB, with significant improvement at affected frequencies(250-8000 Hz) compared with the control group(P < 0.05). The findings indicated the duration from the onset of SSNHL to the beginning of IST as an independent factor for pure tone average improvement after treatment(P = 0.002), whereas age, duration of SST, and time of IST were not(P > 0.05).Conclusion: The modified IST was demonstrated to be a safe and effective method as a salvage treatment for SSNHL. This study explored the efficacy of a modified IST approach, incorporating the utilization of tympanic tubes and gelfoam as key components. The findings underscore the advantages of gelfoam as a strategic drug carrier placed in the round window niche. By minimizing drug loss, extending action time, and increasing perilymph concentration, gelfoam enhances the therapeutic impact of IST, contributing to improved hearing outcomes in patients with SSNHL.

Therapeutic efficacy of methylprednisolone sodium succinate via diverse administration routes for mid-to high-frequency sudden sensorineural hearing loss

BACKGROUND Sudden sensorineural hearing loss(SSNHL),characterized by a rapid and unexplained loss of hearing,particularly at moderate to high frequencies,presents a significant clinical challenge.The therapeutic use of methylprednisolone sodium succinate(MPSS)via different administration routes,in combination with conventional medications,remains a topic of interest.AIM To compare the therapeutic efficacy of MPSS administered via different routes in combination with conventional drugs for the treatment of mid-to high-frequency SSNHL.METHODS The medical records of 109 patients with mid-to high-frequency SSNHL were analyzed.The patients were divided into three groups based on the route of administration:Group A[intratympanic(IT)injection of MPSS combined with mecobalamin and Ginkgo biloba leaf extract injection],Group B(intravenous injection of MPSS combined with mecobalamin and Ginkgo biloba leaf extract injection),and Group C(single IT injection of MPSS).The intervention effects were compared and analyzed.RESULTS The posttreatment auditory thresholds in Group A(21.23±3.34)were significantly lower than those in Groups B(28.52±3.36)and C(30.23±4.21;P<0.05).Group A also exhibited a significantly greater speech recognition rate(92.23±5.34)than Groups B and C.The disappearance time of tinnitus,time to hearing recovery,and disappearance time of vertigo in Group A were significantly shorter than those in Groups B and C(P<0.05).The total effective rate in Group A(97.56%)was significantly greater than that in Groups B and C(77.14%and 78.79%,χ^(2)=7.898,P=0.019).Moreover,the incidence of adverse reactions in Groups A and C was significantly lower than that in Group B(4.88%,3.03%vs 2.57%,χ^(2)=11.443,P=0.003),and the recurrence rate in Group A was significantly lower than that in Groups B and C(2.44%vs 20.00%vs 21.21%,χ^(2)=7.120,P=0.028).CONCLUSION IT injection of MPSS combined with conventional treatment demonstrates superior efficacy and safety compared to systemic administration via intravenous infusion and a single IT injection of MPSS.This approach effectively improves patients'hearing and reduces the risk of disease recurrence.

Sensorineural Hearing Loss in Multidrug-Resistant Tuberculosis Patients in Kinshasa (Democratic Republic of Congo): Prospective Cohort Study of Therapeutic Regimen with Aminoglycoside versus Bedaquiline

Context: Multidrug-resistant tuberculosis (MDR-TB) remains a major public health problem in developing countries such as the Democratic Republic of Congo (DRC), which continues to face the emergence of MDR-TB cases. Because of the ototoxic effects of AGs, the World Health Organization (WHO) has recommended the introduction of the bedaquiline regimen. However, very few data are available regarding the susceptibility of bedaquiline to induce hearing loss, hence the present study set out to compare the AG-based regimen and the bedaquiline-based regimen in the occurrence of hearing loss in MDR-TB patients. Methods: This is a prospective multicenter cohort study that included 335 MDR-TB patients, performed in Kinshasa (DRC) during the period from January 2020 to January 2021. Sociodemographic, clinical, biological and audiometric data were analyzed using Stata 17. Repeated-measures analysis of variance was used to compare changes in the degree of hearing loss over time between the two groups of patients on AG and bedaquiline regimens. The double-difference method was estimated using regression with fixed-effects. A p value < 0.05 was considered the threshold for statistical significance. Results: The degree of hearing loss was similar between the two groups at the first month [AGs (28 dB) vs BDQ (30 dB);p = 0.298]. At six months, the mean degree of hearing loss was significantly greater in the aminoglycoside regimen group [AGs (60.5 dB) vs BDQ (44 dB);p < 0.001]. The double difference was significant, with a greater increase in hearing loss in the AGs group (diff-in-diff 18.3;p < 0.001). After adjustment for age and serum albumin, the group receiving the AG-based regimen had a 2-point greater worsening than those with bedaquiline at the sixth month (diff-in-diff 19.8;p Conclusion: Hearing loss is frequent with both treatment regimens, but more marked with the Aminoglycoside-based regimen. Thus, bedaquiline should also benefit for audiometric monitoring in future MDR-TB patients.

Universal newborn hearing screening program in Saudi Arabia: Current insight

Newborn hearing screening(NHS) programs are essential to identify hearing loss early in life and to improve outcomes in children. In Saudi Arabia, the national NHS program has been operational since 2016;however, few studies have evaluated its status, and none have covered all provinces across the country. This cross-sectional retrospective study provides an overview of the program's status across all provinces, focusing on screening coverage rates, referral/fail rates, and follow-up procedures. In 2021, 199,034 newborns were screened, with a coverage rate of 92.6% and an overall referral/fail rate of 1.87%. These performance measures provide a foundation for future progress and improvements. This study highlights the importance of ongoing efforts to enhance the program's effectiveness and sustainability.

Ionically Imprinting-Based Copper(Ⅱ)Label-Free Detection for Preventing Hearing Loss

Copper is a microelement with important physiological functions in the body.However,the excess copper ion(Cu^(2+))may cause severe health problems,such as hair cell apoptosis and the resultant hearing loss.Therefore,the assay of Cu^(2+)is important.We integrate ionic imprinting technology(IIT)and structurally colored hydrogel beads to prepare chitosan-based ionically imprinted hydrogel beads(IIHBs)as a low-cost and high-specificity platform for Cu^(2+)detection.The IIHBs have a macroporous microstructure,uniform size,vivid structural color,and magnetic responsiveness.When incubated in solution,IIHBs recognize Cu^(2+)and exhibit a reflective peak change,thereby achieving label-free detection.In addition,benefiting from the IIT,the IIHBs display good specificity and selectivity and have an imprinting factor of 19.14 at 100μmol·L^(-1).These features indicated that the developed IIHBs are promising candidates for Cu^(2+)detection,particularly for the prevention of hearing loss.

Controlled release of dexamethasone from fibrin sealant for intratympanic administration in inner ear therapy

The aim of the present work was to show the sustainability of fibrin sealant in releasing dexamethasone and adjust the protocol for clinical application of the novel method in the treatment of Meniere’s disease (MD) and sudden sensorineural hearing loss (SSHL).Gelation occurred shortly after mixing dexamethasone-containing fibrinogen with thrombin.Dexamethasone was constantly released for at least 16 d at a stable level after 7d in protocol 1 (low-dose),while it was robustly released within 4 d and slowed afterward until 10 d in protocol 2(high-dose).There were significant differences among the time points in Protocol 2 (p<0.01,ANOVA),and the exponential model with the formula y=15.299*e~(-0.483*t) fits the association.The estimated concentration of dexamethasone released on 7 d in protocol 2 was slightly lower than that observed in protocol 1.The fibrin sealant is capable of constantly releasing dexamethasone with adjustable dynamics.Targeted and minimally invasive administration of the material can be achieved in the clinic by sequential injections of the fluids using a soft-tipped catheter.

Effect of foot reflexology on an infant with sensorineural hearing loss:A case report

BACKGROUND Our study contributes to the further understanding of the mechanism of foot reflexology.Foot reflexology has been reported to affect hearing recovery,but no physiological evidence has been provided.This lack of evidence hampers the acceptance of the technique in clinical practice.CASE SUMMARY A girl was taken to North Sichuan Medical University Affiliated Hospital for a hearing screen by her parents.Her parents reported that her hearing level was the same as when she was born.The girl was diagnosed with sensorineural hearing loss(SNHL)by a doctor in the otolaryngology department.After we introduced the foot reflexology project,the parents agreed to participate in the experiment.After 6 months of foot reflexology treatment,the hearing threshold of the girl recovered to a normal level,below 30 dB.CONCLUSION Foot reflexology should be encouraged in clinical practice and for families of infants with SNHL.

Navigating Silence: A Comprehensive Review of Parental Roles in the Rehabilitation of Children with Hearing Loss

This narrative literature review delves into the multifaceted realm of parental involvement in the rehabilitation of children with permanent hearing loss. While existing research has made strides in elucidating parental roles in this context, critical gaps persist, necessitating a comprehensive exploration to inform future endeavors. Our review synthesizes a wide array of studies, identifying these gaps and emphasizing the significance of addressing them. Themes emerging from the literature include the varying degrees of parental engagement, the impact of cultural and socio-economic factors, and the challenges faced by families navigating rehabilitation processes. The synthesis of this literature not only highlights the current state of knowledge but also provides a roadmap for future research efforts. By addressing these gaps, we aim to contribute to a more nuanced understanding of parental involvement in the rehabilitation of children with permanent hearing loss, ultimately fostering improved support systems and holistic care for affected families.

Usher syndrome:Genetic diagnosis and current therapeutic approaches

Usher Syndrome(USH)is the most common deaf-blind syndrome,affecting approximately 1 in 6000 people in the deaf population.This genetic condition is characterized by a combination of hearing loss(HL),retinitis pigmentosa,and,in some cases,vestibular areflexia.Among the subtypes of USH,USH type 1 is considered the most severe form,presenting profound bilateral congenital deafness,vestibular areflexia,and early onset RP.USH type 2 is the most common form,exhibiting congenital moderate to severe HL for low frequencies and severe to profound HL for high frequencies.Conversely,type 3 is the rarest,initially manifesting mild symptoms during childhood that become more prominent in the first decades of life.The dual impact of USH on both visual and auditory senses significantly impairs patients'quality of life,restricting their daily activities and interactions with society.To date,9 genes have been confirmed so far for USH:MYO7A,USH1C,CDH23,PCDH15,USH1G,USH2A,ADGRV1,WHRN and CLRN1.These genes are inherited in an autosomal recessive manner and encode proteins expressed in the inner ear and retina,leading to functional loss.Although non-genetic methods can assist in patient triage and disease extension evaluation,genetic and molecular tests play a pivotal role in providing genetic counseling,enabling appropriate gene therapy,and facilitating timely cochlear implantation(CI).The CRISPR/Cas9 system and viral-based gene replacement therapy have recently emerged as highly promising techniques for treating USH.Regarding drug therapy,PTC-124 and Nb54 have been identified as promising drug interventions for genetic HL in USH.Simultaneously,CI has proven to be critical in the restoration of hearing.This review aims to summarize the genetic and molecular diagnosis of USH and highlight the importance of early diagnosis in Cuzzuol BR et al.Diagnosis and current treatments of USH WJO https://www.wjgnet.com 2 January 19,2024 Volume 11 Issue 1 guiding appropriate treatment strategies and improving patient prognosis.

Evaluation of Digits-in-Noise Test and Hearing Handicap Inventory for Adults Screening in Patients with Occupational Noise-Induced Hearing Loss

Objective:To explore the clinical evaluation role of the Digits-in-Noise(DIN)test and Hearing Handicap Inventory for Adults Screening(HHIA-S)for patients with occupational noise-induced hearing loss and to observe and analyze their application values.Methods:Fifty patients with suspected occupational noise-induced hearing loss were randomly selected from the Department of Otolaryngology at the hospital as the research target.The collection period for the research cases spanned from January 2022 to November 2023,and all patients had a history of noise exposure.The DIN test and HHIA-S were used for hearing examinations,with clinical,comprehensive diagnosis serving as the gold standard to study their diagnostic performance.Results:The compliance rate of the DIN test was 88.00%,the HHIA-S’s compliance rate was 80.00%,and the combined compliance rate was 94.00%.The compliance rate of the DIN test and the combined compliance rates of the patients were statistically significant compared to the clinical gold standard data(P<0.05),while there was no difference between the compliance rate of the HHIA-S and the gold standard(P>0.05).The data shows that the sensitivity of the combined diagnosis is significantly higher than the sensitivity data of the DIN test and HHIA-S examination alone(P<0.05).Its specificity is 100.00%,and the accuracy data of the joint diagnosis in the degree were higher than those of the DIN test alone(P>0.05)and the HHIA-S alone(P<0.05).Conclusion:For patients with occupational noise-induced hearing loss,the joint evaluation of the DIN test and HHIA-S can significantly improve their diagnostic value with high sensitivity and accuracy.

Prevalence of Noise-Induced Hearing Loss Related to the Mills in the Markets of the Municipality of Parakou in 2021

Introduction: Noise is the second leading cause of hearing loss in adults after presbycusis. The objective of this work was to study hearing loss induced by the noise of mills in the markets of Parakou. Methods: This was a descriptive and analytical cross-sectional study, conducted from February 3 to June 3, 2021 in the markets of Parakou. It concerned millers and sellers located within a 5 meter radius around the mills and among whom pure-tone audiometry was performed to detect a hearing loss. Subjects with no particular medical health history, under 55 years of age and having been working in these markets since more than 12 months, were included. Results: In this study, 103 subjects were selected, including 43 millers and 61 sellers. Their average age was 29 ± 13 years. The sex ratio was 0.49. The average length of service in the profession was 8 years with the extremes of 3 months and 47 years. They were exposed to noise on average 10 hours per day and 6 days a week. The average duration of weekly noise exposure was 23 h 28 min ± 13 h 32 min with the extremes of 5 h 00 min and 52 h 30 min. The average level of noise exposure was 90 dB with the extremes of 72 and 110 dB. 24 subjects reported symptoms related to noise such as headache, tinnitus, and hearing loss, with respective proportions of 22.33%, 20.39% and 06.80%. The prevalence of noise-related hearing loss was 26.21% (n = 27/103). Subjects with a notch at 4000 Hz and normal Average Hearing Loss (AHL) (20.39%) had a seven-time greater risk of developing noise-induced hearing loss (OR = 6.58;95% CI [2.54 - 18.8], p Conclusion: Hearing loss related to the noise of mills affected both millers and sellers near the mills in markets, hence the importance of regulating mills.

Effects of Drilling in Mastoid Cavity over Hearing in the Contralateral Ear

In advanced otological surgeries, powered instruments form an indispensable part. The risk of deterioration to hearing in the operated ear is a commonly discussed issue, however, there remains a possibility of affecting the hearing in the contralateral ear due to transcranial vibration. So in this study we aimed to assess the possibility of the non-operated ear being affected by the noise generated during ear surgeries and whether it is temporary or permanent in nature. Methodology: This study included 63 patients diagnosed with unilateral disease who underwent mastoid surgery. Preoperatively all the patients were subjected to Pure tone audiometry (PTA), Transient evoked otoacoustic emission (TEOAE) and Distortion product otoacoustic emission (DPOAE). Patients were operated using both cutting and diamond burrs of ranging from sizes 1 - 6 mm. Total drilling time was recorded. Results: Post-operative hearing evaluation was done at 1 week, 4 weeks and 12 weeks. The sound emitted by various burrs was recorded by Sound Level Meter. Out of the total 58 patients that followed up, 46 showed change in at least one of the hearing parameters. Patients showing changes had a higher drilling time as compared to those with no changes. Of these, the changes associated with the total drilling time and with cutting burr time were found to be significant. The hearing changes seen on PTA, TEOAE and DPOAE were transient in nature with only one patient having a persistent decreased high frequency threshold at the end of 12 weeks. It was also found that cutting burrs produce more sound as compared to diamond burrs and a larger size burr of a type produces more sound than a smaller one of its type. Conclusion: The drilling of mastoid bone during ear surgeries can transiently impair the hearing in the contralateral ear which is of great significance in patients with only one hearing ear.

Induced pluripotent stem cells:Landscape for studying and treating hereditary hearing loss

Hearing loss(HL) is one of the most widespread sensory disorders,affecting approximately 1 in 500 newborns.Heritable diseases of the inner ear are the leading causes of prelingual HL.Treating of hereditary HL and understanding its underlying mechanisms remain difficult challenges to otolaryngologists.As stem cells are capable of self-renewal and differentiation,they are ideally suited both for disease modeling and regenerative medicine.Recently,description of induced pluripotent stem cells(iPSCs) has allowed the field of disease modeling and personalized therapy to become far more accessible and physiologically relevant,as iPSCs can be generated from patients of any genetic background.This review briefly describes the advantages of iPSCs technology and discusses potential applications of this powerful biological tool in studying and treating hereditary HL.

NON-SYNDROMIC HEARING LOSS AND HIGH- THROUGHPUT STRATEGIES TO DECIPHER ITS GENETIC HETEROGENEITY

Hearing loss (HL) is the most common sensory disorder, affecting all age groups, ethnicities, and gen-ders. According to World Health Organization (WHO) estimates in 2005, 278 million people worldwide have moderate to profound HL in both ears. Results of the 2002 National Health Interview Survey indicate that nearly 31 million of all non-institutionalized adults (aged 18 and over) in the United States have trouble hearing. Epidemiological studies have estimated that approximately 50%of profound HL can be attributed to genetic causes. With over 60 genes implicated in nonsyndromic hearing loss, it is also an extremely het-erogeneous trait. Recent progress in identifying genes responsible for hearing loss enables otolaryngologists and other clinicians to apply molecular diagnosis by genetic testing. The advent of the $1000 genome has the potential to revolutionize the identification of genes and their mutations underlying genetic disorders. This is especially true for extremely heterogeneous Mendelian conditions such as deafness, where the muta-tion, and indeed the gene, may be private. The recent technological advances in target-enrichment methods and next generation sequencing offer a unique opportunity to break through the barriers of limitations im-posed by gene arrays. These approaches now allow for the complete analysis of all known deafness-causing genes and will result in a new wave of discoveries of the remaining genes for Mendelian disorders. This re-view focuses on describing genotype-phenotype correlations of the most frequent genes including GJB2, which is responsible for more than half of cases, followed by other common genes and on discussing the im-pact of genomic advances for comprehensive genetic testing and gene discovery in hereditary hearing loss.

A comparison of effects of systemic and intratympanic steroid therapies for sudden sensorineural hearing loss:A meta-analysis

Objective:To evaluate the efficacy and safety of intratympanic and systemic steroid therapies in the initial treatment of Sudden Sensorineural Hearing Loss (SSNHL) patients. Methods:A comprehensive search of PubMed, Wanfang database and CNKI (China National Knowledge Infrastructure) was performed covering the period from January 1990 to July 2014. A meta-analysis was conducted after filtering by the criteria of Cochrane Collaboration. Three hundred fifty six subjects in nine studies allocated to the group of intratympanic steroid therapies and 343 controls receiving systemic steroid therapies met the criteria for meta-analysis. The data were extracted and analyzed using the RevMan 5.3 meta-analysis software. Results: The total effectiveness rate in SSNHL patients receiving intratympanic steroid therapies did not differ statistically from patients receiving systemic therapies (RR ? 1.08, 95%CI ? 0.99e1.99, P ? 0.10), although the rate of full hearing recovery in this group differed significantly from patients receiving systemic therapies (RR ? 1.29, 95%CI ? 1.00e1.66, P ? 0.05). Conclusion: Local steroid therapy appears to generate higher rate of complete hearing recovery than systemic steroid treatment as an initial treatment for SSNHL, which may be especially useful for patients in whom systemic steroids are contraindicated.

Impact of next-generation sequencing on molecular diagnosis of inherited non-syndromic hearing loss

Hearing loss is one of the most common birth defects,with inherited genetic defects play an important role,contributing to about 60%of deafness occurring in infants.However,hearing impairment is genetically heterogeneous,with both common and rare forms occurring due to mutations in estimated 500 genes.Due to the large number and presumably low mutation frequencies of those genes,it would be highly expensive and time-consuming to address this issue by conventional gene-by-gene Sanger sequencing.Next-generation sequencing is a revolutionary technology that allows the simultaneous screening of mutations in a large number of genes.It is cost effective compared to classical strategies of linkage analysis and direct sequencing when the number or size of genes is large,and thus has become a highly efficient strategy for identifying novel causative genes and mutations involved in heritable disease.In this review, we describe major NGS methodologies currently used for genetic disorders and highlight applications of these technologies in studies of molecular diagnosis and the discovery of genes implicated in non-syndromic hearing loss.

The genetic load for hereditary hearing impairment in Chinese population and its clinical implication

Objective To understand the genetic load in the Chinese population for improvement in diagnosis, prevention and rehabilitation of deafness. Methods DNA samples, immortalized cell lines as well as detailed clinical and audiometric data were collected through a national genetic resources collecting network. Two conventional genetic approaches were used in the studies. Linkage analysis in X chromosome and autosomes with microsatellite markers were performed in large families for gene mapping and positional cloning of novel genes. Candidate gene approach was used for screening the mtDNA 12SrRNA, GJB2 and SLC26A4 mutations in population -based samples. Results A total of 2,572 Chinese hearing loss families or sporadic cases were characterized in the reported studies, including seven X-linked, one Y-linked, 28 large and multiplex autosomal dominant hearing loss families, 607 simplex autosomal recessive hereditary hearing loss families, 100 mitochondrial inheritance families, 147 GJB2 induced hearing loss cases, 230 cases with enlarged vestibular aqueduct (EVA) syndrome, 169 sporadic cases with auditory neuropathy, and 1,283 sporadic sensorineural hearing loss cases. Through linkage analysis or sequence analysis, two X-linked families were found transmitting two novel mutations in the POU3F4 gene, while another X -linked family was mapped onto a novel locus, nominated as AUNX1 (auditory neuropathy, X-linked locus 1). The only Y-linked family was mapped onto the DFNY1 locus(Y-linked locus 1, DFNY1). Eight of the 28 autosomal dominant families were linked to various autosomal loci. In population genetics studies, 2,567 familial cases and sporadic patients were subjected to mutation screening for three common hearing loss genes: mtDNA 12S rRNA 1555G, GJB2 and SLC26A4. The auditory neuropathy cases in our samples were screened for OTOF gene mutations. Conclusions These data show that the Chinese population has a genetic load on hereditary hearing loss. Establishing personalized surveillance and prevention models for hearing loss based on genetic research will provide the opportunity to decrease the prevalence of deafness in the Chinese population.

Functional and patient-reported outcomes of bone-anchored hearing aids (BAHA): A prospective case series study

Objective:The purpose of this study was to evaluate the functional and patient-reported outcomes,and their correlation,after percutaneous bone-anchored hearing aid(BAHA)implantation.Methods:A prospective study was conducted between January 2018 and December 2020 in a tertiary care center.All adult patients who were implanted with a percutaneous BAHA device during this evaluation period were included in the study.Complete auditory function and patients reported outcome measures(PROMs)were assessed in the preoperative period and 6 months after the implant activation.The PROMs included a generic form(Medical Outcome Study 36 Short Form Healthy Survey(MOS SF-36)),and three disease-specific forms(Hearing Handicap Inventory(HHI),Satisfaction with Amplification in Daily Life Scale(SADLS),and Tinnitus Handicap Inventory(THI)).Results:Twenty-two patients with an average age of 53 years were included in the study.The overall functional gain with the BAHA in sound-field pure tone average(PTA)was 29 dB,with no statistically significant differences according to surgical indication(F(3,18)=2.319,p=0.110).The greater the preoperative air-bone gap,the greater the functional gain obtained(r=0.505,p<0.05).In the PROMs,we found a significant improvement in HHI scores(p<0.005)and a significant increase in overall SADLS scores(p<0.05)with the use of percutaneous BAHA devices.We did not verify any statistically significant correlation between functional and PROMs results.Conclusions:The BAHA is a safe and effective alternative hearing rehabilitation option in selected patients.The PROMs results prove patient's overall satisfaction.

Age-related hearing loss accelerates the decline in fast speech comprehension and the decompensation of cortical network connections

Patients with age-related hearing loss face hearing difficulties in daily life.The causes of age-related hearing loss are complex and include changes in peripheral hearing,central processing,and cognitive-related abilities.Furthermore,the factors by which aging relates to hearing loss via changes in audito ry processing ability are still unclear.In this cross-sectional study,we evaluated 27 older adults(over 60 years old) with age-related hearing loss,21 older adults(over 60years old) with normal hearing,and 30 younger subjects(18-30 years old) with normal hearing.We used the outcome of the uppe r-threshold test,including the time-compressed thres h old and the speech recognition threshold in noisy conditions,as a behavioral indicator of auditory processing ability.We also used electroencephalogra p hy to identify presbycusis-related abnormalities in the brain while the participants were in a spontaneous resting state.The timecompressed threshold and speech recognition threshold data indicated significant diffe rences among the groups.In patients with age-related hearing loss,information masking(babble noise) had a greater effect than energy masking(speech-shaped noise) on processing difficulties.In terms of resting-state electroencephalography signals,we observed enhanced fro ntal lobe(Brodmann’s area,BA11) activation in the older adults with normal hearing compared with the younger participants with normal hearing,and greater activation in the parietal(BA7) and occipital(BA19) lobes in the individuals with age-related hearing loss compared with the younger adults.Our functional connection analysis suggested that compared with younger people,the older adults with normal hearing exhibited enhanced connections among networks,including the default mode network,sensorimotor network,cingulo-opercular network,occipital network,and frontoparietal network.These results suggest that both normal aging and the development of age-related hearing loss have a negative effect on advanced audito ry processing capabilities and that hearing loss accele rates the decline in speech comprehension,especially in speech competition situations.Older adults with normal hearing may have increased compensatory attentional resource recruitment represented by the to p-down active listening mechanism,while those with age-related hearing loss exhibit decompensation of network connections involving multisensory integration.