Development and Optimization of Universal Bone Conduction Hearing Spectacles

Background: Non-implantable bone anchored hearing devices (BCHDs) are utilized for patients with conductive or mixed hearing loss who are unsuitable for conventional hearing aids or have unresolved middle ear issues. These devices can be surgically implanted or attached using adhesive plates, dental sticks, elastic headbands, or bone conduction spectacles. Optimal fitting of bone conduction spectacles requires appropriate frame selection and contact pressure in the temporal and mastoid areas. The ANSI S3.6 and DIN EN ISO 389-3 standards recommend a contact area of approximately 1.75 cm2 and a maximum force of 5.4 N for effective sound transmission and comfort. Methods: This study aimed to evaluate the technical fit and mechanical stability of universal bone conduction hearing spectacles compared to established systems. A Sen-Pressure 02 thin-film sensor connected to an Arduino Uno R3 board measured contact force in the temporal and mastoid areas. Several BCHDs were tested, including the Bruckhoff la belle BC D50/70, Radioear B71 headset, Radioear B71 elastic headband, Cochlear Baha SoundArc M, and Cochlear Baha elastic headband, on a PVC artificial head, with data analyzed using ANOVA and LSD post hoc tests. Results: The la belle BC D50/70 spectacles showed comparable contact force to established BCHDs, ensuring adequate sound transmission and comfort. Significant differences were observed between the systems, with the Radioear B71 headset exhibiting the highest forces. The la belle BC D50/70 had similar forces to the Radioear B71 elastic headband. Conclusion: The la belle BC D50/70 universal bone conduction hearing spectacles are a technically equivalent alternative to established BCHDs, maintaining pressure below 5.4 N. Future research should explore the impact of different contact forces on performance and comfort, and the integration of force control in modified spectacles. This study indicates that the la belle BC D50/70 is a viable alternative that meets audiological practice requirements.

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.

Social Effects of Hearing Loss on Patients in Jos, North Central Nigeria

Hearing loss has caused serious social effects among people living with it. Those who relate to patients with hearing loss (PHL) also share some part of the negative effects. Some specifics are communication disability, which impacts speech and language development, academic performance, and social or work life. In previous years, the problem was predicted to be aggravated as the expected life span of the population increased. The study was conducted among PHL and their family members in JUTH and Kazahyet Audiology Service in Jos. 150 PHL were purposively sampled. The study was guided by a Cross-sectional Survey Research Design, and the specific objectives were to (1) identify the social effects of hearing loss on the PHL, (2) determine the implication of social effects on PHL, and (3) determine the extent at which these social effects affect PHL. The outcome of the study shows that the social effects faced by PHL are poor relationships, no secrets, dependence on sign language, frustration, depression, and dependence on lip reading. Some stop schooling, and others feel laughed at and therefore choose the path of isolation. The implication is that hearing loss is expensive to manage. PHL always feel they are not contributing their quarter in life as usual;they feel stigmatized and are not easy to relate with. The study recommends that the government should come up with policies that will check man-made behaviors that exert negative social effects on PHL in our society, subsidize the cost of hearing aids and cochlear implants to be affordable for PHL, formulate a policy on mandatory newborn hearing screening before the infant is discharged from the hospital to help in the early identification of hearing loss. Finally, the early creation of awareness of the dangers or consequences of hearing loss will go a long way in preventing our society from involvement in high-risk behaviors that will cause hearing loss.

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.

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.

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.

Acquired sensorineural hearing loss,oxidative stress,and microRNAs

Hearing loss is the third leading cause of human disability.Age-related hearing loss,one type of acquired sensorineural hearing loss,is largely responsible for this escalating global health burden.Noise-induced,ototoxic,and idiopathic sudden sensorineural are other less common types of acquired hearing loss.The etiology of these conditions is complex and multi-fa ctorial involving an interplay of genetic and environmental factors.Oxidative stress has recently been proposed as a likely linking cause in most types of acquired sensorineural hearing loss.Short non-coding RNA sequences known as microRNAs(miRNAs)have increasingly been shown to play a role in cellular hypoxia and oxidative stress responses including promoting an apoptotic response.Sensory hair cell death is a central histopathological finding in sensorineural hearing loss.As these cells do not regenerate in humans,it underlies the irreversibility of human age-related hearing loss.Ovid EMBASE,Ovid MEDLINE,Web of Science Core Collection,and ClinicalTrials.gov databases over the period August 1,2018 to July 31,2023 were searched with"hearing loss,""hypoxamiRs,""hypoxia,""microRNAs,""ischemia,"and"oxidative stress"text words for English language primary study publications or registered clinical trials.Registe red clinical trials known to the senior author we re also assessed.A total of 222studies were thus identified.After excluding duplicates,editorials,retra ctions,secondary research studies,and non-English language articles,39 primary studies and clinical trials underwent full-text screening.This resulted in 11 animal,in vitro,and/or human subject journal articles and 8 registered clinical trial database entries which form the basis of this narrative review.MiRNAs miR-34a and miR-29b levels increase with age in mice.These miRNAs were demonstrated in human neuroblastoma and murine cochlear cell lines to target Sirtuin 1/peroxisome proliferato r-activated receptor gamma coactivator-1-alpha(SIRT1/P GC-1α),SIRT1p53,and SIRT1/hypoxia-inducible factor 1-alpha signaling pathways resulting in increased apoptosis.Furthermore,hypoxia and oxidative stress had a similar adve rse apoptotic effect,which was inhibited by resve ratrol and a myocardial inhibitorassociated transcript,a miR-29b competing endogenous mRNA.Gentamicin reduced miR-182-5p levels and increased cochlear oxidative stress and cell death in mice-an effect that was corrected by inner ear stem cell-derived exosomes.There is ongoing work seeking to determine if these findings can be effectively translated to humans.

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.

Tranylcypromine upregulates Sestrin 2 expression to ameliorate NLRP3-related noise-induced hearing loss

Noise-induced hearing loss is the primary non-genetic factor contributing to auditory dysfunction.However,there are currently no effective pharmacological interventions for patients with noise-induced hearing loss.Here,we present evidence suggesting that the lysine-specific demethylase 1 inhibitor–tranylcypromine is an otoprotective agent that could be used to treat noise-induced hearing loss,and elucidate its underlying regulatory mechanisms.We established a mouse model of permanent threshold shift hearing loss by exposing the mice to white broadband noise at a sound pressure level of 120 d B for 4 hours.We found that tranylcypromine treatment led to the upregulation of Sestrin2(SESN2)and activation of the autophagy markers light chain 3B and lysosome-associated membrane glycoprotein 1 in the cochleae of mice treated with tranylcypromine.The noise exposure group treated with tranylcypromine showed significantly lower average auditory brainstem response hearing thresholds at click,4,8,and 16 k Hz frequencies compared with the noise exposure group treated with saline.These findings indicate that tranylcypromine treatment resulted in increased SESN2,light chain 3B,and lysosome-associated membrane glycoprotein 1 expression after noise exposure,leading to a reduction in levels of 4-hydroxynonenal and cleaved caspase-3,thereby reducing noise-induced hair cell loss.Additionally,immunoblot analysis demonstrated that treatment with tranylcypromine upregulated SESN2 expression via the autophagy pathway.Tranylcypromine treatment also reduced the production of NOD-like receptor family pyrin domaincontaining 3(NLRP3)production.In conclusion,our results showed that tranylcypromine treatment ameliorated cochlear inflammation by promoting the expression of SESN2,which induced autophagy,thereby restricting NLRP3-related inflammasome signaling,alleviating cochlear hair cell loss,and protecting hearing function.These findings suggest that inhibiting lysine-specific demethylase 1 is a potential therapeutic strategy for preventing hair cell loss and noise-induced hearing loss.

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.

Additional comments on foot reflexology treatment for sensorineural hearing loss in infant

Currently,treatment options for infant sensorineural hearing loss(SNHL)are limited.This article describes a novel case of SNHL in an infant successfully treated with foot reflexology,along with observed brain activity changes before and after treatment,as indicated by functional magnetic resonance imaging.Hence,this commentary discusses the case and our viewpoints regarding foot reflexology for treating SNHL.

Clinical characteristics and analysis of vestibular-evoked myogenic potentials in patients with sudden sensorineural hearing loss in different ages

BACKGROUND Numerous studies have found that patients experiencing sudden sensorineural hearing loss(SSHL),with or without accompanying vertigo,often show impaired vestibular function.However,there is a dearth of studies analyzing vestibularevoked myogenic potentials(VEMPs)in SSHL patients across various age groups.AIM To investigate vestibular condition in SSHL patients across various age demographics.METHODS Clinical data of 84 SSHL patients were investigated retrospectively.Audiometry,cervical vestibular evoked myogenic potentials(c-VEMPs),and ocular vestibular evoked myogenic potentials(o-VEMPs)were conducted on these patients.Parameters assessed included the latencies of P1 and N1 waves,as well as the amplitudes of P1–N1 waves.Moreover,the study evaluated the influence of factors such as sex,affected side,configuration of hearing loss,and presence of accompanying vertigo.RESULTS Among the 84 SSHL patients,no significant differences were observed among the three groups in terms of gender,affected side,and the presence or absence of vertigo.Group II(aged 41–60 years)had the highest number of SSHL cases.The rates of absent o-VEMPs in the affected ears were 20.83%,31.58%,and 22.72%for the three age groups,respectively,with no statistically significant difference among them.The rates of absent c-VEMPs in the affected ears were 8.3%,34.21%,and 18.18%for the three age groups,respectively,with significant differences.In the unaffected ears,there were differences observed in the extraction rates of o-VEMPs in the unaffected ears among the age groups.In the three age groups,no significant differences were noted in the three age groups in the latencies of P1 and N1 waves or in the amplitude of N1–P1 waves for c-VEMPs and o-VEMPs,either on the affected side or on the unaffected side,across the three age groups.CONCLUSION The extraction rate of VEMPs is more valuable than parameters.Regardless of the presence of vertigo,vestibular organs are involved in SSHL.Notably,SSHL patients aged 41–60 appear more susceptible to damage to the inferior vestibular nerve and saccule.

Brain networks in newborns and infants with and without sensorineural hearing loss:A functional near-infrared spectroscopy study

BACKGROUND Understanding the impact of early sensory deficits on brain development is essential for understanding developmental processes and developing potential interventions.While previous studies have looked into the impact of prenatal experiences on language development,there is a lack of research on how these experiences affect early language and brain function development in individuals with sensorineural hearing loss(SNHL).AIM To investigate SNHL effects on early brain development and connectivity in 4-month-olds vs healthy newborns and controls.METHODS The research involved analyzing the functional brain networks of 65 infants,categorized into three groups:28 healthy newborns,224-month-old participants with SNHL,and 15 age-matched healthy participants.The resting-state functional connectivity was measured and compared between the groups using functional near-infrared spectroscopy and graph theory to assess the brain network properties.RESULTS Significant differences were found in resting-state functional connectivity between participants with SNHL and age-matched controls,indicating a developmental lag in brain connectivity for those with SNHL.Surprisingly,SNHL participants showed better connectivity development compared to healthy newborns,with connectivity strengths of 0.13±0.04 for SNHL,0.16±0.08 for controls,and 0.098±0.04 for newborns.Graph theory analysis revealed enhanced global brain network properties for the SNHL group,suggesting higher communication efficiency at 4 months.No significant differences were noted in network properties between 4-month-old SNHL participants and neonates.A unique pattern of central hubs was observed in the SNHL group,with 2 hubs in the left hemisphere compared to 6 in controls.CONCLUSION 4-month-old infants with SNHL have a distinct brain network pattern with efficient long-distance information transmission but less effective local communication compared to age-matched controls.

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.

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.

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.

Autosomal dominant non-syndromic hearing loss caused by a novel mutation in MYO7A:A case report and review of the literature

BACKGROUND Variants in the MYO7A gene commonly result in Usher syndrome,and in rare cases lead to autosomal dominant non-syndromic deafness(DFNA11).Currently,only nine variants have been reported to be responsible for DFNA11 and their clinical phenotypes are not identical.Here we present a novel variant causing DFNA11 identified in a three-generation Chinese family.CASE SUMMARY The proband was a 53-year-old Han male who presented with post-lingual bilateral symmetrical moderate sensorineural hearing loss.We learned from the patient’s medical history collection that multiple family members also had similar hearing loss,generally occurring around the age of 40.Subsequent investigation by high-throughput sequencing identified a novel MYO7A variant.To provide evidence supporting that this variant is responsible for the hearing loss in the studied family,we performed Sanger sequencing on 11 family members and found that the variant co-segregated with the deafness phenotype.In addition,the clinical manifestation of the 11 affected family members was found to be lateonset bilateral slowly progressive hearing loss,inherited in this family in an autosomal dominant manner.None of the affected family members had visual impairment or vestibular symptoms;therefore,we believe that this novel MYO7A variant is responsible for the rare DFNA11 in this family.CONCLUSION We report a novel variant leading to DFNA11 which further enriches the collection of MYO7A variants,and our review of the nine previous variants that have been identified to cause DFNA11 provides a reference for clinical genetic counseling.

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.

Hearing Assessment among Sudanese Rheumatoid Arthritis Patients versus Non-Rheumatoid Arthritis Individuals

Background: Rheumatoid arthritis (RA) disease is one of the most common chronic autoimmune diseases that affect many body systems including the auditory system. Objectives: To assess hearing thresholds and to ascertain types of hearing loss among Sudanese rheumatoid arthritis patients attending rheumatology clinic in Omdurman military hospital and matching them with non-rheumatoid arthritis subjects. Methodology: This descriptive and analytic (comparative) hospital based cross sectional study conducted from October 2020 to April 2021 which include 66 RA patients with age range (21 - 60 years) matched with 41 non-rheumatoid arthritis group sharing same characteristics (nation, gender and age). Pure tone audiometry, tympanometry and acoustic reflexes were done for all RA patients and matched groups. Statistical analysis of the data was carried out using the association and correlation tests for associations and t-test for independent samples. Results: Thirty-six (54.5%) of RA patients had hearing impairment versus 9 (22%) non-RA in PTA test. Twenty-four RA cases (36.3%) showed asymmetrical hearing threshold and graph in PTA between right and left ears. Thirty-four (51.5%) right and 36 (54.5%) left ears were normal degree followed by 23 (34.8%) in the right and 24 (36.4%) in the left ears were mild degree hearing loss. Among hearing impaired RA patients;20 right ears (62.5%) and 19 left ears (65.5%) had sensorineural hearing loss (SNHL), conductive hearing loss 11 (34.4%) right ears and 9 (31%) left ears. Mixed HL was in 1 right ear (3.1%) and 1 left ear (3.5%). The most common degree of SNHL type was mild in (75%) and (78.9%) in right and left ears respectively, moderate and moderate severe were (20%) in the right and (15.7%) in the left ears. Fifty-eight right ears (87.9%) and 56 left ears (84.8%) had type A tympanogram while 7 (10.6%) right ears and 9 left ears (13.6%) were type As tympanogram, one right and one left ears had type Ad tympanogram. Acoustic reflex was impaired in 17 right ears and 17 left ears (25.8% for both). There were significant statistical differences in most of the specific frequency means between the study group and non-rheumatoid group in the right and left ears p-value 0.05). Also there was no significant statistical association between anti-rheumatic drugs used and hearing threshold of RA patients (p-value > 0.05). Conclusion: Hearing impairment especially sensorineural type is common in Sudanese rheumatoid arthritis patients. Conductive hearing loss is less common and most likely due to ossicles diarthrodial joint stiffness. No influence of the disease activity or used anti-rheumatic drugs on hearing threshold of the RA patients was detected in this study.