CoP修饰Ti_(3)C_(2)T_(x)MXene纳米复合材料作为高效析氢反应电催化剂

高效、经济和环保是电化学水分解制氢电催化剂的关键要素。二维(2D)MXene材料因其独特的物理化学性质而受到广泛关注。虽然有许多不同种类的MXene材料,但只有少数具有本征析氢反应(HER)催化活性。然而,MXene材料具有很多优点,如较大的比表面积、高电导率和丰富的表面官能团,因此可以作为与其他物质复合的理想平台。本研究首先通过密度泛函理论(DFT)预测了CoP与Ti_(3)C_(2)T_(x)MXene(其中T_(x)=―F和―OH官能团)具有低的氢吸附自由能(ΔGH^(*))。接着,我们合成了CoP-Ti_(3)C_(2)T_(x)MXene纳米复合材料,并在0.5 mol∙L^(−1)H_(2)SO_(4)中测试了其电催化HER性能。该材料在电流密度为10 mA∙cm^(−2)时表现出了低的过电位(135 mV)和Tafel斜率为48 mV∙dec^(−1)。理论计算表明,CoP-Ti_(3)C_(2)T_(x)MXene纳米复合材料的优异电催化性能源于Ti_(3)C_(2)T_(x)的高金属导电性、良好的界面电荷转移、快速的氢吸附/解吸过程以及优化的电子结构。

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.

Diverse phenotype of Méenière’s disease associated with family history,thyroid disorder,migraine and associated disorders

Objective:To better understand the clinical phenotype of Méenière’s disease(MD),we examined family history,thyroid disorder,migraine,and associated disorders in complaints of people living with MD.Method:We designed the study as a retrospective and examined data gathered from 912 participants with MD.Their data were originally collected by the Finnish M′eni`ere Federation(FMF).The survey data included individual case histories for environmental factors,comorbidities,disease-specific complaints,impact-related questions,cognitive complaints,health-related quality of life(HRQoL),and sense of coherence(SOC).Results:We observed significant differences between those with and without sporadic occurrence,family history,thyroid disorder,and migraine-associated complaints.Family history explained 20%of variability in patient complaints.Patients with a family history of MD whose disease started at younger age experienced balance problems,more severe vertigo spells,more severe vestibular drop attacks(VDA),and less nausea,although they had good SOC.Thyroid disorder explained 14%of variability in patient complaints.MD patients with a thyroid disorder comorbidity suffered more often from constant dizziness,balance problems,greater impact of hearing problems,cognitive complaints,and poor HRQoL.Migraine explained 12%of variability in patients’complaints and was associated with poor SOC and cognitive balance problems.MD patients with both thyroid disorder and migraine used antidepressants more often than other groups.Logistic regression analysis showed comorbidities of ischemic brain disorder(among 7.1%),kidney insufficiency(among 1.2%),and diabetes(among 7.3%)had statistically significant but restricted association with balance and gait problems,VDA,and reduced HRQoL.Conclusions:Family history of MD and thyroid disorder or migraine comorbidities in MD influence the complaint pattern and partially explain complex symptom profiles,including symptoms of cognitive problems.Confounders play a minimal role in complaint profile and impact of MD whereas comorbidities influence the complaint structure and partly explain the complex symptom profile in MD.

Meibomian glands segmentation in infrared images with limited annotation

●AIM:To investigate a pioneering framework for the segmentation of meibomian glands(MGs),using limited annotations to reduce the workload on ophthalmologists and enhance the efficiency of clinical diagnosis.●METHODS:Totally 203 infrared meibomian images from 138 patients with dry eye disease,accompanied by corresponding annotations,were gathered for the study.A rectified scribble-supervised gland segmentation(RSSGS)model,incorporating temporal ensemble prediction,uncertainty estimation,and a transformation equivariance constraint,was introduced to address constraints imposed by limited supervision information inherent in scribble annotations.The viability and efficacy of the proposed model were assessed based on accuracy,intersection over union(IoU),and dice coefficient.●RESULTS:Using manual labels as the gold standard,RSSGS demonstrated outcomes with an accuracy of 93.54%,a dice coefficient of 78.02%,and an IoU of 64.18%.Notably,these performance metrics exceed the current weakly supervised state-of-the-art methods by 0.76%,2.06%,and 2.69%,respectively.Furthermore,despite achieving a substantial 80%reduction in annotation costs,it only lags behind fully annotated methods by 0.72%,1.51%,and 2.04%.●CONCLUSION:An innovative automatic segmentation model is developed for MGs in infrared eyelid images,using scribble annotation for training.This model maintains an exceptionally high level of segmentation accuracy while substantially reducing training costs.It holds substantial utility for calculating clinical parameters,thereby greatly enhancing the diagnostic efficiency of ophthalmologists in evaluating meibomian gland dysfunction.

基于深度学习的睑板腺腺体分割方法研究

目的:探讨应用深度学习技术解决睑板腺腺体自动分割问题的效果与价值。方法:采集并筛选出193幅红外睑板腺图像构建图像数据库,由3名临床医师对图像进行人工标记;引入UNet++网络与自动数据增广策略构建睑板腺腺体自动分割模型,采用精确率、敏感性、特异性、准确率和交并比分析该模型的可行性与有效性。结果:以人工标注结果为金标准,基于UNet++的睑板腺腺体自动分割模型取得94.31%的准确率,敏感性、特异性分别为82.15%和96.13%,腺体分割表现具有较好的稳定性,模型处理单张图像的平均用时仅为0.11s。结论:引入深度学习技术实现睑板腺腺体的自动分割,具有良好的准确性、稳定性和高效性,可服务于睑板腺功能障碍患者腺体形态参数的计算,辅助相关疾病的临床诊断和筛查,提高诊断效率。

Salivary proteins and microbiota as biomarkers for early childhood caries risk assessment

Early childhood caries （ECC） is a term used to describe dental caries in children aged 6 years or younger. Oral streptococci, such as Streptococcus mutans and Streptococcus sorbrinus, are considered to be the main etiological agents of tooth decay in children. Other bacteria, such as Prevotella spp. and Lactobacillus spp., and fungus, that is, Candida albicans, are related to the development and progression of ECC. Biomolecules in saliva, mainly proteins, affect the survival of oral microorganisms by multiple innate defensive mechanisms, thus modulating the oral microflora. Therefore, the protein composition of saliva can be a sensitive indicator for dental health. Resistance or susceptibility to caries may be significantly correlated with alterations in salivary protein components. Some oral microorganisms and saliva proteins may serve as useful biomarkers in predicting the risk and prognosis of caries. Current research has generated abundant information that contributes to a better understanding of the roles of microorganisms and salivary proteins in ECC occurrence and prevention. This review summarizes the microorganisms that cause caries and tooth-protective salivary proteins with their potential as functional biomarkers for ECC risk assessment. The identification of biomarkers for children at high risk of ECC is not only critical for early diagnosis but also important for preventing and treating the disease.

Clinically relevant human temporal bone measurements using novel high-resolution cone-beam CT

Objective: To test the feasibility of measuring fine temporal bone structures using a newly established cone-beam computed tomography(CBCT)system.Materials and methods: Six formalin-fixed human cadaver temporal bones were imaged using a high-resolution CBCT system that has 900 frames and copper t aluminum filtration. Fine temporal bone structures, including those of the facial nerve canal and vestibular structures, were identified and measured.Results: The fine structures of the middle ear, including the tympanic membrane, tendon of the tensor tympani, cochleariform process of the semicanal of the tensor tympani, pyramidal eminence, footplate of the stapes, full path of the facial nerve within the temporal bone, supralabyrinthine space, semicircular canals, pathway of the subarcuate canal, and full path of the vestibular aqueduct, were clearly demonstrated. The vestibular aqueduct has a midpoint width of 0.4 ± 0.0 mm and opercular width of 0.5 ± 0.1 mm(mean ± SD). The length of the internal acoustic meatus was 10.6 ± 1.2 mm(mean ± SD), and the diameter of the internal acoustic meatus was 3.7 ± 0.3 mm(mean ± SD).Conclusion: This novel high-resolution CBCT system has potentially broad applications in the diagnosis of inner ear disease and in monitoring associated pathological changes, surgical planning, navigation for the ear surgery, and temporal bone training.

Chinese consensus on management of tyrosine kinase inhibitor-associated side effects in gastrointestinal stromal tumors

Tyrosine kinase inhibitors(TKIs) have improved the overall survival of patients with gastrointestinal stromal tumors(GISTs), but their side effects can impact dose intensity and, consequently, the clinical benefit. To date, no guideline or consensus has been published on the TKI-associated adverse reactions. Therefore, the Chinese Society of Surgeons for Gastrointestinal Stromal Tumor of the Chinese Medical Doctor Association organized an expert panel discussion involving representatives from gastrointestinal surgery, medical oncology, cardiology, dermatology, nephrology, endocrinology, and ophthalmology to consider the systemic clinical symptoms, molecular and cellular mechanisms, and treatment recommendations of GISTs. Here, we present the resultant evidence-and experience-based consensus to guide the management of TKI-associated side events in clinical practice.

DNA N^6-methyladenine demethylase ALKBH1 enhances osteogenic differentiation of human MSCs

ALKBH1 was recently discovered as a demethylase for DNA N6-methyladenine （N6-mA）, a new epigenetic modification, and interacts with the core transcriptional pluripotency network of embryonic stem cells. However, the role of ALKBH1 and DNA N6-mA in regulating osteogenic differentiation is largely unknown. In this study, we demonstrated that the expression of ALKBH1 in human mesenchymal stem cells （MSCs） was upregulated during osteogenic induction. Knockdown of ALKBH1 increased the genomic DNA N6-mA levels and significantly reduced the expression of osteogenic-related genes, alkaline phosphatase activity, and mineralization. ALKBHl-depleted MSCs also exhibited a restricted capacity for bone formation in vivo. By contrast, the ectopic overexpression of ALKBH1 enhanced osteoblastic differentiation. Mechanically, we found that the depletion of ALKBH1 resulted in the accumulation of N6-mA on the promoter region of ATF4, which subsequently silenced ATF4 transcription. In addition, restoring the expression of ATP by adenovirus-mediated transduction successfully rescued osteogenic differentiation. Taken together, our results demonstrate that ALKBH1 is indispensable for the osteogenic differentiation of MSCs and indicate that DNA N6-mA modifications area new mechanism for the epigenetic regulation of stem cell differentiation.

“Wait and scan” management of patients with vestibular schwannoma and the relevance of non-contrast MRI in the follow-up

Vestibular schwannoma(VS) is a slow-growing benign neoplasm. There has been an evolution in the management of VS from active treatments(microsurgery and stereotactic radiotherapy) to conservative management(wait and scan). Regular MRI scanning is necessary to monitor tumor progression. Conservative management causes significantly less complications and offers a higher quality of life compared with active treatments. The mean growth rate of VS varies from 0.4 to 2.9 mm/year, and spontaneous shrinkage is observed in 3.8 percent of tumors during observation. If significant growth occurs, active treatment is considered. Significant growth is defined as an increase of at least 3 mm in the largest extrameatal diameter in any plane between the first and last available scans. The vestibulocochlear nerve is surrounded by cerebrospinal fluid, which provides natural contrast for MRI; thus, gadolinium may not be needed to detect VS. Specific sequences have high sensitivity, specificity, and accuracy for detection of progression. Hypointense signal in the ipsilateral inner ear fluid might be a useful sign to distinguish VS from meningioma. In this paper, we summarize the current status of research on conservative management and non-contrast MRI for the detection of VS.

Future Precipitation Extremes in China under Climate Change and Their Physical Quantification Based on a Regional Climate Model and CMIP5 Model Simulations

The atmospheric water holding capacity will increase with temperature according to Clausius-Clapeyron scaling and affects precipitation.The rates of change in future precipitation extremes are quantified with changes in surface air temperature.Precipitation extremes in China are determined for the 21st century in six simulations using a regional climate model,RegCM4,and 17 global climate models that participated in CMIP5.First,we assess the performance of the CMIP5 models and RCM runs in their simulation of extreme precipitation for the current period(RF:1982-2001).The CMIP5 models and RCM results can capture the spatial variations of precipitation extremes,as well as those based on observations:OBS and XPP.Precipitation extremes over four subregions in China are predicted to increase in the mid-future(MF:2039-58)and far-future(FF:2079-98)relative to those for the RF period based on both the CMIP5 ensemble mean and RCM ensemble mean.The secular trends in the extremes of the CMIP5 models are predicted to increase from 2008 to 2058,and the RCM results show higher interannual variability relative to that of the CMIP5 models.Then,we quantify the increasing rates of change in precipitation extremes in the MF and FF periods in the subregions of China with the changes in surface air temperature.Finally,based on the water vapor equation,changes in precipitation extremes in China for the MF and FF periods are found to correlate positively with changes in the atmospheric vertical wind multiplied by changes in surface specific humidity(significant at the p<0.1 level).

数字普惠金融、金融素养和家庭收入贫困

金融扶贫一直是学者和政策制定者关注的焦点,多数研究主要关注外部金融发展的减贫效应,较少关注家庭本身的金融素养对减贫的影响。基于此,本文利用北京大学数字普惠金融指数和中国家庭追踪调查(CFPS)数据匹配形成的新数据,研究了外部数字普惠金融和内部金融素养对家庭收入贫困的联合影响。研究发现,中国家庭的基本金融素养和高级金融素养均较为缺乏,且贫困收入家庭的金融素养显著低于非贫困收入家庭。在有效缓解内生性问题前提下,结果表明数字普惠金融和金融素养对家庭绝对和相对收入贫困均有显著负向影响,即两者均可减缓家庭收入贫困,数字普惠金融的减贫效应优于金融素养的减贫效应。金融素养主要通过缓解融资约束、平滑消费和增加风险偏好来缓解家庭收入贫困,而数字普惠金融通过金融素养缓解家庭收入贫困的效应不显著,主要是通过提升家庭风险管理水平来实现。进一步分析发现,数字普惠金融的减贫效果主要源于数字普惠金融的使用深度,其减贫效应主要存在于中西部地区家庭,高级金融素养的减贫效应主要存在于东部地区家庭。本研究为理解家庭收入贫困提供了新视角,对各国减贫政策的制定具有重要借鉴意义。

Inner ear barriers to nanomedicine-augmented drug delivery and imaging

There are several challenges to inner ear drug delivery and imaging due to the existence of tight biological barriers to the target structure and the dense bone surrounding it. Advances in imaging and nanomedicine may provide knowledge for overcoming the existing limitations to both the diagnosis and treatment of inner ear diseases. Novel techniques have improved the efficacy of drug delivery and targeting to the inner ear, as well as the quality and accuracy of imaging this structure. In this review, we will describe the pathways and biological barriers of the inner ear regarding drug delivery, the beneficial applications and limitations of the imaging techniques available for inner ear research, the behavior of engineered nanomaterials in inner ear applications, and future perspectives for nanomedicine-based inner ear imaging.

数字金融的研究现状、热点与前沿--基于Cite Space的可视化分析

分析数字金融的研究现状与热点前沿,有利于学者们更好地了解其发展趋势,为后续学者开展更深入的研究提供参考。基于此,本文以2013-2021年间WOS核心合集和CNKI期刊数据库为数据源,从作者和机构合作、文献共被引、关键词和突变词分析等方面,对数字金融研究进行可视化分析。研究发现:国内外的发文量均逐年递增,但存在一定差异,国内发文速度较快并在数量上赶超国外;与国外相比,我国已经初步形成以黄益平教授及其所在的北京大学数字金融研究中心为首的研究力量。国外热点主要聚焦于区块链的研究与应用、数字化转型和金融包容性三大主题,国内热点则集中在数字金融内涵、数字金融的减贫效应、数字金融支持实体经济以及数字金融的风险与监管研究四大方面,发展脉络从数字金融的机遇与风险讨论演进至发展过程中显现的“三大鸿沟”和监管等问题的研究。未来可从数字金融的减贫效应异质性、监管创新以及数字技术深入应用产生的新态势等方面进行更深入研究。

Autoinflammatory characteristics and short-term effects of delivering high-dose steroids to the surface of the intact endolymphatic sac and incus in refractory Ménière’s disease

Objective:To investigate immune-related genetic background in intractable Meniere’s disease(MD)and the immediate results of a novel therapy by delivering steroids to the surface of the intact endolymphatic sac(ES)and incus in a sustainable manner.Case report and methods:Candidate genes involved in immune regulation were sequenced using a nextgeneration sequencing method in a patient with intractable MD.Mutations were confirmed using the Sanger sequencing method.The ES was exposed,and gelatin sponge particles were immersed in highdose methylprednisolone solution and placed onto the surface of ES.“L”-shaped gelatin sponge strips were immersed in dexamethasone solution and served as a guiding device for the steroids by touching the incus and gelatin sponge particles on the surface of the ES.Gelatin sponge particles immersed in dexamethasone solution were placed around the gelatin sponge strips and sealed using fibrin glue.Results:Autoinflammation in the refractory MD case was indicated by genotype,including novel heterozygous mutations of PRF1,UNC13D,SLC29A3,ITCH,and JAK3,as well as phenotype.The vertigo was fully relieved immediately after operation.Tinnitus and aural fullness were resolved 3 weeks after operation,whereas hearing improved in 2 mon postoperation.No recurrence was noted during the 5-monfollow-up,and the final MRI supported the novel therapeutic hypothesis.Conclusion:Autoinflammation was involved in a refractory MD.This novel therapy,which involves the delivery of steroids to the surface of the intact ES and incus,is effective in relieving vertigo and tinnitus and improves hearing function of refractory MD.

Dexamethasone does not affect endolymphatic hydrops(EH)in patients with Meniere's disease within 24 h,and intratympanic administration of gadolinium plus dexamethasone simplifies high-quality imaging of EH using a novel protocol of 7 min

Background:Intratympanic administration of gadolinium chelate allows for a better visualization of endolymphatic hydrops(EH)using MRI than intravenous injection and was recently further improved to facilitate high-quality imaging of EH in 7 min.The aim of the present study was to simplify the intratympanic administration protocol by mixing gadolinium chelate with therapeutic dexamethasone and to evaluate the effects of this mixture on the visualization of EH in MRI.Materials and methods:In an in vitro study,the potential impact of gadolinium-diethylenetriamine pentaacetic acid(Gd-DTPA)on the stability of dexamethasone was evaluated by analyzing dynamic changes in dexamethasone with high-performance liquid chromatography(HPLC)after mixing with GdDTPA.Ten patients with definite Meniere's disease(MD)were recruited to study the potential interference of dexamethasone on MRI visualization of EH,and 49 patients with MD were recruited to evaluate the effect of intratympanic injection of Gd-DTPA mixed with dexamethasone on MRI of EH using a 3T MR machine and a novel heavily T2-weighted 3-dimensional fluid-attenuated inversion recovery reconstructed using a magnitude plus zero-filled interpolation(hT2FLAIR-MZFI)sequence.Results:The retention times and peak area of dexamethasone in HPLC were not modified by the addition of Gd-DTPA.EH grading in the cochlea and vestibule was not influenced in any ear by intratympanic injection of dexamethasone.Excellent inner ear images were obtained from all patients,and EHs with various grades were displayed.There were significant correlations between diagnosis and cochlear EH(p<0.01,Spearman's Rho),between diagnosis and vestibular EH(p<0.01,Spearman's Rho),and between cochlear and vestibular EH(p<0.01,Spearman's Rho).The distribution of Gd-DTPA plus dexamethasone negatively correlated with the grade of vestibular EH.Injury of the endolymph-perilymph barrier was detected in one cochlea and three vestibules of 59 inner ears with MD.Conclusions:Intratympanic administration of Gd-DTPA plus dexamethasone yielded high-quality MRI images of EH in patients with MD using a novel 7-min protocol and simplified the clinical application.Intratympanic administration of Gd-DTPA plus dexamethasone might be used to test its therapeutic effect in future work.

Expert consensus on early childhood caries management

Early childhood caries(ECC)is a significant chronic disease of childhood and a rising public health burden worldwide.ECC may cause a higher risk of new caries lesions in both primary and permanent dentition,affecting lifelong oral health.The occurrence of ECC has been closely related to the core microbiome change in the oral cavity,which may be influenced by diet habits,oral health management,fluoride use,and dental manipulations.So,it is essential to improve parental oral health and awareness of health care,to establish a dental home at the early stage of childhood,and make an individualized caries management plan.Dental interventions according to the minimally invasive concept should be carried out to treat dental caries.This expert consensus mainly discusses the etiology of ECC,caries-risk assessment of children,prevention and treatment plan of ECC,aiming to achieve lifelong oral health.

Molecular mechanisms of inhibiting glucosyltransferases for biofilm formation in Streptococcus mutans

Glucosyltransferases(Gtfs)play critical roles in the etiology and pathogenesis of Streptococcus mutans(S.mutans)-mediated dental caries including early childhood caries.Gtfs enhance the biofilm formation and promotes colonization of cariogenic bacteria by generating biofilm extracellular polysaccharides(EPSs),the key virulence property in the cariogenic process.Therefore,Gtfs have become an appealing target for effective therapeutic interventions that inhibit cariogenic biofilms.Importantly,targeting Gtfs selectively impairs the S.mutans virulence without affecting S.mutans existence or the existence of other species in the oral cavity.Over the past decade,numerous Gtfs inhibitory molecules have been identified,mainly including natural and synthetic compounds and their derivatives,antibodies,and metal ions.These therapeutic agents exert their inhibitory role in inhibiting the expression gtf genes and the activities and secretion of Gtfs enzymes with a wide range of sensitivity and effectiveness.Understanding molecular mechanisms of inhibiting Gtfs will contribute to instructing drug combination strategies,which is more effective for inhibiting Gtfs than one drug or class of drugs.This review highlights our current understanding of Gtfs activities and their potential utility,and discusses challenges and opportunities for future exploration of Gtfs as a therapeutic target.

Runxl protects against the pathological progression of osteoarthritis

Runt-related transcription factor-1(Runxl)is required for chondrocyte-to-osteoblast lineage commitment by enhancing both chondrogenesis and osteogenesis during vertebrate development.However,the potential role of Runxl in joint diseases is not well known.In the current study,we aimed to explore the role of Runxl in osteoarthritis induced by anterior cruciate ligament transaction(ACLT)surgery.We showed that chondrocyte-specific Runxl knockout(Runx1f/fCol2a1-Cre)aggravated cartilage destruction by accelerating the loss of proteoglycan and collagen II in early osteoarthritis.Moreover,we observed thinning and ossification of the growth plate,a decrease in chondrocyte proliferative capacity and the loss of bone matrix around the growth plate in late osteoarthritis.We overexpressed Runxl by adeno-associated virus(AAV)in articular cartilage and identified its protective effect by slowing the destruction of osteoarthritis in cartilage in early osteoarthritis and alleviating the pathological progression of growth plate cartilage in late osteoarthritis.ChIP-seq analysis identified new targets that interacted with Runxl in cartilage pathology,and we confirmed the direct interactions of these factors with Runxl by ChIP-qPCR.This study helps us to understand the function of Runxl in osteoarthritis and provides new clues for targeted osteoarthritis therapy.

A novel PIK3CD C896T mutation detected in bilateral sudden sensorineural hearing loss using next generation sequencing:An indication of primary immunodeficiency

Objective:To investigate immune-related genetic background in bilateral sudden sensorineural hearing loss (SSNHL). Case report and methods: The case is a 45-year-old man presenting with a 7-year history of bilateral profound SSNHL. Blood biochemical testing demonstrated increased levels of total cholesterol (5.88 mmol/L). Tests for hepatitis B showed a positive antibody against the hepatitis B core antigen. Complement C3 was below the normal value, and complement C4 and IgG were in the lower range of normal values. CT images showed a normal inner ear and vestibular aqueduct but round window membranous ossification on both sides. A total number of 232 immune-associated genes were sequenced using the next generation sequencing technique. Results: Mutations were detected in 5 genes, including the phosphoinositide 3-kinase catalytic subunit delta (PIK3CD), caspase recruitment domain-containing protein 9 (CARD9), complement factor H-related (CFHR2), immunoglobulin lambda-like polypeptide 1 Protein (IGLL1), and transmembrane channel-like gene family 8 (TMC8). In the PIK3CD gene, a C896T substitute in exon 7 was detected. This mutation causes primary immunodeficiency and is an autosomal dominant disease. Conclusion: The PIK3CD C896T mutation responsible for primary immunodeficiency may contribute to the onset of bilateral SSNHL with subsequent rapid progression.