Objective:To explore the clinical characteristics, audiological outcomes, and factors influencing the efficacy of pharmacological treatment in patients with sudden hearing loss associated with large vestibular aqueduct syndrome（LVAS）. Methods:A retrospective analysis was conducted on the clinical data of 77 bilateral LVAS patients（117 ears） hospitalized for sudden hearing loss from January 1, 2009, to December 31, 2023. The inclusion criteria required that patients to be diagnosed according to the Valvassori standard and had received standardized pharmacological treatment. Clinical features, audiological outcomes, and treatment efficacy were analyzed. Statistical methods were employed to identify factors associated with treatment outcomes. Results:The age of the enrolled patients ranged from 4 to 37 years. The age of onset for the initial hearing fluctuation varied between 0 and 24 years, with a mean age of 5.8 years. The male-to-female ratio was approximately balanced（37 males and 40 females）. The proportion of unilateral to bilateral sudden hearing loss was 1.0︰1.2, with unilateral right ear hearing loss being more frequently occurring（64.9%）. Triggering Factors: Triggers included no identifiable factors in 48.1% of cases, a history of head trauma（24.7%）, upper respiratory tract infections（11.7%）, onset following physical fatigue（11.7%）, and less frequently, noise exposure, alcohol consumption, or emotional stress（each 1.3%）. Clinical Symptoms: Hearing loss was the sole symptom in 35.1% of cases. Concurrent symptoms included vertigo in 44.2% and tinnitus in 46.8%. Patients with a disease duration of ≤14 days demonstrated a treatment efficacy rate of 75.0%. Among those who responded to treatment, 93.0% had profound or greater hearing loss prior to therapy, with an average improvement in hearing thresholds of 32 dB HL. In pretreatment, 68.9% of patients exhibited low-frequency air-bone gaps, increasing to 76.1% post-treatment. Additionally, 17.6% of treated ears demonstrated a ≥15 dB HL improvement in low-frequency bone conduction thresholds. In the non-responsive group, 7.3% of ears still showed some improvement in bone conduction thresholds. Statistically significant differences（P<0.05） were observed between the treatment-effective and non-effective groups concerning the age of initial hearing fluctuation, disease duration, and severity of hearing loss at onset. Conclusion:The efficacy of pharmacological treatment for sudden hearing loss in LVAS patients is influenced by the age at onset, duration of the disease, and severity of hearing impairment. Early diagnosis and timely intervention significantly enhance treatment efficacy, particularly in patients with a disease duration of ≤14 days and an initial sudden hearing loss. Patients with severe hearing loss, especially those with profound or greater impairment, exhibit greater sensitivity to treatment. Pharmacological interventions positively impact both air conduction and bone conduction thresholds, with the observed improvement in bone conduction thresholds warranting further investigation.
Humans ; Male ; Retrospective Studies ; Hearing Loss, Sudden/etiology* ; Female ; Vestibular Aqueduct/pathology* ; Adult ; Child ; Adolescent ; Child, Preschool ; Young Adult ; Treatment Outcome

Hyperlipidemia is characterized by elevated levels of blood lipids. The clinical manifestations are mainly atherosclerosis caused by the deposition of lipids in the vascular endothelium. The link between abnormal lipid metabolism and sudden hearing loss remains unclear. This article presents a case study of sudden hearing loss accompanied by familial hyperlipidemia. Pure tone audiometry indicated intermediate frequency hearing loss in one ear. Laboratory tests showed abnormal lipid metabolism, and genetic examination identified a heterozygous mutation in theAPOA5 gene. Diagnosis: Sudden hearing loss; hypercholesterolemia. The patient responded well to pharmacological treatment. This paper aims to analyze and discuss thepotential connection between abnormal lipid metabolism and sudden hearing loss.
Humans ; Audiometry, Pure-Tone ; Deafness/complications* ; Hearing Loss, Sensorineural/diagnosis* ; Hearing Loss, Sudden/diagnosis* ; Hyperlipidemias/complications* ; Lipids

Objective To explore the difference in efficacy of metoprolol versus ivabradine in the treatment of postural orthostatic tachycardia syndrome(POTS)in the elderly after COVID-19 infection.Methods A total of 110 patients diagnosed with POTS at our department from Decem-ber 1,2022 to January 31,2023 were included.According to their drug regimen,they were divided into metoprolol group(62 patients)and ivabradine group(48 patients).On the 28th day of out-patient follow-up,the resting heart rate,heart rate of 10 min of standing,symptom disappearance rate,hospitalization rate,and mortality rate were compared between the two groups.Results On the 28th day of treatment,the resting heart rate and postural heart rate for 10 min were decreased in both groups when compared with the levels at initial diagnosis(P＜0.01).And there were no significant differences in the two types of heart rate between the two groups on the 28th day(71.0±7.0 vs 72.1±7.0,P=0.401;76.5±7.2 vs 77.4±7.6,P=0.573).No obvious differences were observed between the two groups in symptom disappearance rate,hospitalization rate,or mortality rate(88.7％vs 89.6％,3.2％vs2.1％,0％vs 0％,P＞0.05).Conclusion Metoprolol and ivabradine can effectively treat POTS in the elderly patients after COVID-19 infection.

Objective To investigate the long-term effect of frailty on heart failure with reduced ejection fraction(HFrEF)in elderly patients.Methods A retrospective analysis was conducted on 245 HFrEF patients aged ≥75 years admitted to our hospital from October 2017 to October 2020 due to acute exacerbation of chronic heart failure(HF).Based on their clinical frailty scale(CFS)score,they were divided into frailty group(1-4,135 cases)and non-frailty group(5-9,110 cases).Their general clinical data,clinical medication,and prognosis were compared between the two groups,and the influencing factors for frailty and death were analyzed.Results Faster heart rate,higher NT-proBNP level,and larger proportions of male,diabetes,coronary heart disease,≥5 chronic diseases,LVEF ≤35％,anemia and increased troponin I level,while lower BMI,eGFR and score of activity of daily living scale were observed in the frailty group than the non-frailty group(P＜0.05,P＜0.01).The frail group had significantly lower utilization rates of angiotensin converting enzyme inhibitors(ACEI),angiotensin receptor blockers(ARB),angiotensin receptor enkephalin inhibitors(ARNI)β receptor blockers,and sodium-glucose cotransporter 2 inhibitors than the non-frailty group(P＜0.01).Additionally,the frailty group exhibited a higher incidence of emergency room visits/readmissions within 3 months and 2-year mortality than the non-frailty group(P＜0.05,P＜0.01).Binary logistic regression analysis revealed that ≥5 chronic diseases,LVEF ≤ 35％,BMI,and GFR were independent risk factors for frailty(OR=0.167,95％CI:0.064-0.453,P=0.000;OR=0.306,95％CI:0.160-0.586,P=0.000;OR=0.868,95％CI:0.786-0.958,P=0.005;OR=0.966,95％CI:0.943-0.991,P=0.007),while ≥5 chronic disea-ses and frailty were independent risk factors for death in HF patients(P＜0.05).Conclusion The incidence of frailty is high in elderly HF patients with HFrEF.They have poor compliance to guideline directed drug therapy(GDMT).Frailty is an independent risk factor for long-term mor-tality in the patients.

Objective To assess the safety of femoral artery puncture procedures guided by X-ray and ultrasound among elderly patients.Methods A total of 480 patients undergoing transcatheter interventional treatment for cardiovascular and cerebrovascular diseases through the femoral ar-tery in our hospital between January 2016 and December 2022 were enrolled in the study.Of them,326 patients receiving femoral artery puncture guided by X-ray fluoroscopy were assigned into X-ray group,while the other 154 patients guided by vascular Doppler ultrasound were into ultrasound group.With propensity score matching(PSM)in a ratio of 1∶1,finally 270 patients were included.Their general clinical data,success rate of puncture,puncture site,and incidence of vascular complications were compared between the two groups.Multivariate logistic regression analysis was used to identify the risk factors for vascular complications.Results Before PSM,there were no statistical differences in the mean distance from the skin fold to the bifurcation of the common femoral artery(2.5±1.0 cm vs 2.4±0.8 cm)or the distance from the fold to the in-guinal ligament(6.4±1.4 cm vs 6.3±1.7 cm)between the X-ray group and the ultrasound group(P＞0.05).After PSM,the X-ray group exhibited an obviously higher incidence of puncture points below the common femoral artery than the ultrasound group(14.8％vs 6.7％,P＜0.05),but no significant differences were observed in the one-time success rate of puncture or the occur-rence of vascular complications between the two groups(P＞0.05).Multivariate logistic regres-sion analysis indicated that the presences of non-common femoral artery and femoral artery calci-fication at the puncture site was independent risk factors for vascular complications(OR=8.379,95％CI:3.561-19.717;OR=3.922,95％CI:1.664-9.242).Conclusion There is no statistical disparity in safety between X-ray-versus ultrasound-guided femoral artery puncture procedures.Cli-nicians should choose appropriate puncture procedure or combine them together based on individual con-dition of patients.

Objective To investigate the efficacy of utilizing Kaneka dual lumen microcatheter in combination with anchored balloon in treatment of bifurcation lesions in elderly patients with ra-dial artery 6 F catheter.Methods A retrospective analysis was performed on 168 patients(≥60 years)receiving bifurcation treatment with a 6 F catheter via the radial artery at the Heart Center of our hospital from January 2020 to January 2023.According to application of dual lumen micro-catheters and anchoring balloon technology or not,they were assigned into an anchoring group(81 cases old)and a control group(87 cases).A comparison was made between the two groups in terms of features of coronary artery disease,operation procedure,and MACE.Multivariate logistic regression analysis was employed.Results Lower incidence of dissection,shorter operation time,less X-ray exposure dose,and decreased contrast agent dosage were observed in the anchoring group when compared with the control group(P＜0.05,P＜0.01).There was no statistical differ-ence in the MACE incidence between the two groups(4.9％vs 8.0％,P＞0.05).The side branch dissection and final TIMI grade＜3 of side branch flow were independent risk factors for postop-erative MACE in elderly patients with bifurcation lesions after surgery(P＜0.05,P＜0.01).Conclusion Kaneka dual lumen microcatheter combined with anchored balloon technology via radial artery 6 F catheter has the advantages in effectively shorting operation time,minimizing X-ray exposure,reducing contrast agent usage,and diminishing the incidence of side branch dis-section in treatment of bifurcation lesions in elderly patients undergoing surgical treatment.

Objective:To analyze the genetic counseling characteristics and interpretation of pathogenic variants for mitochondrial hearing loss.Methods:We analyzed a total of 513 unrelated families from Chinese Deafness Genome Project (CDGP), in which previous gene testing had found no pathogenic mutations with hearing loss (HL) related to the nuclear genes. We used targeted testing and complete mtDNA sequencing for the families′ available members.Results:Among the first individuals of the families to be tested, 20 cases (16 probands and 4 normal hearing consultants) were discovered with variants in mtDNA, including m.1095T>C, m.1310C>T, m.1494C>T and m.1555A>G in MT-RNR1, m.7445A>G, m.7505T>C, m.7510T>C and m.7511T>C in MT-TS1, and m.3243A>G in MT-TL1. We identified MT-RNR1 and MT-TS1 variants occurred as homoplasmic changes, while MT-TL1 variants occurred as heteroplasmic changes. Most mitochondrial hearing loss were characterized by slope or flat moderate to profound sensorineural HL. HL associated with the mtDNA pathogenic variant was variable onset age, severity and audiometric configuration. The penetrance for HL in individuals with the m.1095T>C variant might be low. Progression in the severity of HL was caused by the m.7445A>G pathogenic variant and the patients with m.7505T>C had cookie bite HL. MT-TL1 m.3243A>G was a common spot for pathogenic variants associated with nonsyndromic HL as well as syndromic HL with diabetes mellitus. Conclusions:Individuals with the mtDNA variants and their maternal relatives have a higher risk of HL and phenotypic heterogeneity in the age of onset, progression, and level of HL. The medical history should include review of any audiological testing of the probands and their family members on a maternal mtDNA background. The process of genetic evaluation needs rule out nuclear gene mutations and analyzes the genetic load of mitochondrial pathogenic variants in the probands or consultants. In these cases, we rely on clinical and genetic evaluation molecular analysis to appropriately counsel families for whom an exact cause of HL and to help them make informed medical decisions.

Postzygotic mutations are acquired in normal tissues throughout an individual's lifetime and hold clues for identifying mutagenic factors.Here,we investigated postzygotic mutation spectra of healthy individuals using optimized ultra-deep exome sequencing of the time-series samples from the same volunteer as well as the samples from different individuals.In blood,sperm,and muscle cells,we resolved three common types of mutational signatures.Signatures A and B represent clock-like mutational processes,and the polymorphisms of epigenetic regulation genes influence the pro-portion of signature B in mutation profiles.Notably,signature C,characterized by C＞T transitions at GpCpN sites,tends to be a feature of diverse normal tissues.Mutations of this type are likely to occur early during embryonic development,supported by their relatively high allelic frequencies,presence in multiple tissues,and decrease in occurrence with age.Almost none of the public datasets for tumors feature this signature,except for 19.6％of samples of clear cell renal cell carcinoma with increased activation of the hypoxia-inducible factor 1(HIF-1)signaling pathway.Moreover,the accumulation of signature C in the mutation profile was accelerated in a human embryonic stem cell line with drug-induced activation of HIF-1α.Thus,embryonic hypoxia may explain this novel signature across multiple normal tissues.Our study suggests that hypoxic condition in an early stage of embryonic development is a crucial factor inducing C＞T transitions at GpCpN sites;and indi-viduals'genetic background may also influence their postzygotic mutation profiles.

Objective:To analyze the sleep quality and sleep structure of patients with obstructive sleep apnea hypopnea syndrome (OSAHS) complicated with patent foramen ovale (PFO), and to study the effect of PFO on the sleep structure of OSAHS.Methods:Fifty-six patients with OSAHS complicated with PFO, 64 patients with simple OSAHS and 62 controls were collected from December 2018 to March 2020 in Centre of Sleep Disorders, the Second Affiliated Hospital of Zhengzhou University. Pittsburgh Sleep Quality Index and polysomnography were used to compare the sleep quality and sleep structure of the three groups.Results:Compared with the control group [6/62(9.68%)], OSAHS complicated with PFO group [54/56(96.43%)] and simple OSAHS group [53/64(82.81%)] had higher incidence of poor sleep quality (χ2=112.08, P<0.0l). Furthermore, compared with the control group, the OSAHS complicated with PFO group and simple OSAHS group showed reduced sleep efficiency [PSQI total score was 0.5 (0, 1), 2 (1, 3) and 2 (1, 2) respectively, H=74.549, P<0.01] and reduced proportions of rapid eye movement (REM; 20.45%±3.49%, 12.19%±5.95% and 15.11%±7.21%,respectively, F=21.17, P<0.01) and slow wave sleep (N3; 21.24%±4.12%, 14.15%±6.08%, 17.68%±6.35%, respectively, F=29.51, P<0.01); the N1 (4.47%±2.40%, 9.50%±5.34%, 9.55%±4.61%, respectively, F=30.07, P<0.05) and N2 sleep (53.88%±4.35%, 64.09%±7.49%, 58.14%±6.67% , respectively, F=46.21, P<0.05) were prolonged; the inocturnal lowest oxyhemoglobin saturation (SpO 2) level was lower, mean SpO 2 reduction at night was higher [3.00% (0, 4.00%),6.00% (5.00%, 8.75%) and 4.00% (4.00%, 5.00%), respectively, H=72.24, P<0.05], and periodic leg movement index [16.30(4.80, 32.82), 33.30(9.26, 54.80) and 23.10(8.38, 31.83),respectively, H=17.86, P<0.05], arousal index [11.60(7.73, 17.55), 23.90(14.03, 30.45) and 15.6(11.23, 20.78), respectively, H=22.80, P<0.05] and sleep apnea and hypopnea index (AHI; 1.60±1.38, 23.90±7.27 and 16.24±4.22,respectively, F=136.97, P<0.05) increased. Compared with the simple OSAHS group, the incidence of poor sleep quality was higher, the proportions of slow wave sleep (N3, F=29.51, P=0.047) and REM ( F=21.17, P=0.012) were decreased, N2 sleep ( F=46.21, P=0.000) was prolonged, mean SpO 2 reduction at night ( Z=54.28, P=0.000), wake after sleep onset [116.00(89.88, 143.00) min vs 135.00(118.50, 168.38) min, Z=25.71, P=0.023], arousal times [14.00(8.25, 8.00) vs 17.50(9.00,23.00),respectively, Z=19.68, P=0.041], microarousal ( Z=23.57, P=0.044), and AHI ( F=136.97, P=0.000) were increased in the OSAHS complicated with PFO group. Conclusions:OSAHS complicated with PFO patients had poor sleep quality and high incidence of sleep disorders. They had sleep disorder at night, which was characterized by the decrease of REM sleep and slow wave sleep, the prolongation of N2, the decrease of nocturnal SpO 2 and the increase of awakening times, and the increase of arousal times and AHI. PFO can aggravate the sleep disorder of OSAHS.

BACKGROUND:Ischemia/reperfusion (IR)injury during the pancreas transplantation can cause numerous postoperative complications, among which,secondary pancreatitis can cause small intestinal mucosal injury and result in severe Consepuence.OBJECTIVE:To observe the protective effect of ischemic preconditioning (IPC) on small intestinal mucosal barrier after pancreas transplantation in rats.DESIGN:Randomized controlled animal trial.SETTING:Department of Gastrointestinal Surgery,Xijing Hospital,Fourth Military Medical University of Chinese PLA.MATERIALS:This trial was done in the Laboratory of Gastrointestinal Surgery,Xijing Hospital,Fourth Military Medical University of Chinese PLA between September 2001 and April 2004.Eighty-three male SD rats were involved in this trial.METHODS: Forty-seven rats were randomly chosen to prepare diabetic rat models by penile-intravenous injection of 65 mg/kg streptozotocin.Thirty-six successful model rats were randomized into 3 groups,with 12 in each group:IR group,donor IPC(DIPC)group and recipient with two hindlims IPC(RIPC)group.Twelve of the remaining 36 normal rats served as control group,and the other 24 rats were used as donors.Laparotomy was conducted only in control group,and pancreas transplantation was conducted in the other 3 groups In DIPC group,the splenic vessels of donors were blocked for 5 minutes and reperfused for 5 minutes twice before obtaining pancreas from donor;In the RIPC group, blood flow of two hindlimbs of recipients was blocked for 5 minutes and reperfused for 5 minutes before reperfusing the pancreas of donor,and this procedure was repeated 3 times.IR group was untouched.MAIN OUTCOME MEASURES:① On the 5th day after operation,6 rats were randomly chosen from each group to detect small intestinal permeability[expressed with plasm fluorescent-isothiocyanate-dextran(FITC-dextran)concentration]and absorption function(expressed with plasm xylose concentration).② On the 5th day after operation.blood was taken from the left 6 rats in each group to detect serum tumor necrosis factor-α(TNF-α) and nitric oxide(NO)level as well as superoxide dismutase(SOD)and amylase activity.Ileal mucosal tissue was taken to detect wet weight of small intestinal mucosa,the height and width of microvilli,malonaldehyde(MDA)level and myeloperoxidase(MPO)activity.At the same time,mesenteric lymph node,liver and splenic tissue were taken to perform bacterial culture.Bacterial translocation was observed.RESULTS:After supplement,72 rats were involved in the result analysis.①Plasm FITC-dextran concentration of IR group were higher than that in control group,DIPC group and RIPC group,respectively(P＜0.01).②Plasm xylose concentration in the IR group was lower than that in the control group,DIPC group and RIPC group,respectively(P＜0.01).③Bacterial translocation rate in the IR group was higher than that in the control group,DIPC group and RIPC group,respectively(P＜0.01).④Small intestinal mucosal injury degree in the IR group was lower than that in the other 3 groups(P＜0.01).⑤Small intestinal MPO activity and MDA level in IR group were significantly higher than those in the other 3 groups(P＜0.01). Serum SOD activity and NO level were lower but amylase activity and TNF-α 1evel were higher in the IR group as compared with the other 3 groups(P＜0.01).CONCLUSION:IPC of two hindlimbs in both donor and recipient can protect small intestinal mucosal barrier and reduce bacterial translocation rate after pancreas transplantation in rats.