Hearing loss is the most prevalent disabling disease. Cochlear implantation（CI） serves as the primary intervention for severe to profound hearing loss. This consensus systematically explores the value of genetic diagnosis in the pre-operative assessment and efficacy prognosis for CI. Drawing upon domestic and international research and clinical experience, it proposes an evidence-based medicine three-tiered prognostic classification system（Favorable, Marginal, Poor）. The consensus focuses on common hereditary non-syndromic hearing loss（such as that caused by mutations in genes like GJB2, SLC26A4, OTOF, LOXHD1） and syndromic hereditary hearing loss（such as Jervell & Lange-Nielsen syndrome and Waardenburg syndrome）, which are closely associated with congenital hearing loss, analyzing the impact of their pathological mechanisms on CI outcomes. The consensus provides recommendations based on multiple round of expert discussion and voting. It emphasizes that genetic diagnosis can optimize patient selection, predict prognosis, guide post-operative rehabilitation, offer stratified management strategies for patients with different genotypes, and advance the application of precision medicine in the field of CI.
Humans ; Cochlear Implantation ; Prognosis ; Hearing Loss/surgery* ; Consensus ; Connexin 26 ; Mutation ; Sulfate Transporters ; Connexins/genetics*

Objective To establish a method for determining hydrogen sulfide(H2S)in blood and apply it to practical cases.Methods A delute solution was achieved by adding 0.8 mL saturated borax solution into 0.2 mL blood sample was diluted with.1 mL acetonitrile solution containing 0.1％formic acid was then taken in a test tube,followed by adding 0.1 mL dilute solution and 0.1 mL thiozine aqueous solution(1％).After thorough mixing,the mixture was left to stand for 30 minutes.Subsequently,the sample was subjected to liquid chromatography-tandem mass spectrometry(LC-MS/MS)analysis after centrifugation and membrane filtration.Results The results showed that H2S exhibited good linearity within the concentration range of 10～2 000 ng/mL,with the R2 value of 0.998 5.The detection limit was 5 ng/mL,and the quantification limit was 10 ng/mL.In three cases of H2S poisoning,sulfur ions were detected in the blood of the deceased individuals,with concentrations ranging from 0.17 to 0.56 μg/mL.Conclusion For the first time,this study established a LC-MS/MS method for determining H2S in blood,which can meet the detection needs of H2S poisoning cases.

Objective To compare the short-term clinical effects of unilateral dual-channel endoscopic discectomy(UBED)and percutaneous endoscopic intervertebral discectomy(PEID)in the treatment of L5S,Lumbar disc herniation,LDH.Methods From January 2019 to January 2021,a total of 57 cases of L5S,LDH treated by UBED or PEID were analyzed retrospectively,including 30 cases in UBED group and 27 cases in PEID group.The operation time,intraoperative fluoroscopy times,postoperative hospitalization days and surgical complications were compared between the two groups.The visual analogue scale(VAS)and oswestry disability index(ODI)were used to compare the postoperative quality of life of the two groups,and the modified MacNab criteria was used to evaluate the clinical efficacy in the last follow-up.Results The operation time of UBED group and PEID group was(75.30±8.44)minutes and(68.37±4.63)minutes,respectively,and the difference between the two groups was statistically significant(P＜0.05).VAS and ODI of 1 week,3 months,1 year and 1.5 years after surgery in 2 groups were significantly decreased compared with those before surgery,with statistical significance(P＜0.05).The VAS score of low back pain in UBED group was higher than that in PEID group[(3.87±1.14)points vs(2.70±0.67)points]at 1 week after surgery(P＜0.05),and there was no significant difference in VAS and ODI at the other time points(P＞0.05).There was no statistical difference in the results of modified MacNab criteria in the last follow-up(P＞0.05).There were 2 cases of dural tear in PEID group,1 case of dural tear in UBED group and 1 case of temporary nerve root injury in PEID group after operation,all of which were cured after symptomatic treatment.Conclusion Compared with PEID,UBED has a longer operation time,more trauma and more obvious low back pain in the short term after operation.The short-term curative effect of the two operations on L5S1 LDH is similar,the incidence of complications is low,and the times of fluoroscopy are few.Both operations are safe and effective.

Objective:To investigate the epidemiology of severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection in patients with maintenance hemodialysis (MHD) in Jiangsu province during SARS-CoV-2 pandemic in China from December 7, 2022 to January 27, 2023, and to analyze the influencing factors of all-cause death.Methods:It was a multi-center cross-sectional investigation. Structured questionnaire was used to collect patient information by medical staff of each hemodialysis center (room) as investigators. Part of the demography data and laboratory examination data came from the Jiangsu Province Hemodialysis Data Information System. MHD patients from hemodialysis centers (rooms) at all levels of medical institutions and independent hemodialysis institutions in Jiangsu province during the outbreak of SARS-CoV-2 infection were included, and the clinical characteristics and all-cause mortality of confirmed and suspected cases of SARS-CoV-2 infection were analyzed.Results:Questionnaire surveys and data analysis on 57 278 patients in 407 hemodialysis centers (rooms) were completed, accounting for 90.41% of the total number of MHD patients (63 357 cases) in Jiangsu province during the same period. There were 24 038 cases (41.97%) of SARS-CoV-2 infection and 14 805 cases (25.85%) of suspected infection, which were widely distributed in all dialysis centers in Jiangsu province. After clinical classification of 38 843 confirmed and suspected SARS-CoV-2 infection cases, 3 662 cases were severe and critical cases, accounting for 9.43% of the infected and suspected cases. Among the patients who had completed the questionnaires, there were 1 812 all-cause deaths, with an all-cause mortality rate of 3.16%. Multivariate logistic regression analysis showed that elderly (taking ≤50 years as a reference, 51-59 years: OR=1.583, 95% CI 1.279-1.933, P=0.001; 60-69 years: OR=3.972, 95% CI 3.271-4.858, P<0.001; 70-79 years: OR=7.236, 95% CI 5.917-8.698, P<0.001; ≥80 years: OR=11.738, 95% CI 9.459-14.663, P<0.001), male ( OR=1.371, 95% CI 1.229-1.529, P<0.001), and co-infection with hepatitis B virus (HBV) (positive serum HBV surface antigen, OR=0.629, 95% CI 0.484-0.817, P<0.001) were independent influencing factors for all cause mortality. Receiver-operating characteristic curve analysis showed that the area under the curve for male, age and current HBV infection prediction of all-cause death was 0.529 ( P<0.001), 0.724 ( P<0.001) and 0.514 ( P=0.042), respectively, and the cut-off value for age prediction of all-cause death was 65.5 years old. Compared with patients without HBV infection, MHD patients with HBV infection significantly reduced the proportion of severe and critically ill patients, all-cause hospitalizations and all cause deaths when infected with SARS-CoV-2 (4.99% vs. 6.41%, χ2=6.136, P=0.013; 8.90% vs. 11.44%, χ2=11.662, P<0.001; 2.01% vs. 3.37%, χ2=10.713, P=0.001, respectively). Conclusion:The MHD patients in Jiangsu province are susceptible to SARS-CoV-2. Elderly age and male gender are independent risk factors for death in MHD patients during the epidemic, while the HBV infection may be a protective factor for death of MHD patients infected with SARS-CoV-2.

Objective: To compare the functional changes of neurotransmitters in the dopamine ( DA) system of rats lesioned by 6-hydroxydopamine in unilateral medial forebrain bundle ( MFB) and unilateral striatum after 4 weeks of modeling． Methods : Adult male Sprague-Dawley rats (n = 62) were divided randomly into four groups : single-site model ( one site striatal lesion model，n = 18) ，four-site model ( four sites striatal lesion model，n = 18) ，MFB model (MFB lesion model，n = 18) and a sham operated control group (n = 8) ．Immunohistochemical tyrosine hydroxylase (TH) staining was used to calculate the loss rate of positive DA neurons．The functional changes of dopamine transporters(DAT) and D2 receptors in rat models of four groups were detected by radioligand-receptor binding assay in vitro，behavioral test and small-animal PET. Results : Immunohistochemical staining results of TH at 1-lesion，4-lesion and MFB model showed that the TH-positive cell loss rates of the lesion side in three models were 19. 1% ，84. 7% and 97. 1% ，respectively，indicating that the model was successfully constructed. The results of DAT / D2 receptor functions in the three models showed that，compared with the sham operated control group，the DAT binding ratio of radioactivity on the lesion side of 1-lesion，4-lesion and MFB model significantly decreased (P＜0. 05，P＜0. 05 and P＜0. 01) ．The DAT binding ratio of radioactivity on the lesion side was compared among 1，4 lesion and MFB model．It was found that the decrease degree of MFB model was significantly higher than that of the previous two models (P＜0. 01) ，and the decrease degree of 4-lesion model was significantly higher than that of 1-lesion model (P＜0. 05) ．Compared with the sham operated control group，the D2 receptor binding ratio of radioactivity on the lesion side of 1-lesion and 4-lesion model significantly decreased (P＜0. 05) ， and there was no significant difference in the degree of decrease between the two models，but the D2 receptor binding ratio of radioactivity on the lesion side of MFB model significantly increased (P＜0. 05) ．There was no difference in the distribution of DAT / D2 receptor on the lesion side and the normal side of the sham operated control group．Methamphetamine caused ipsilateral rotations to the lesion side in all models． There were no significant differences in methamphetamine-induced rotation among 1-lesion，4-lesion and MFB models．Bromocriptine caused ipsilateral rotations to the lesion side in 1-lesion and 4-lesion models but contralateral rotations in MFB model. There were no significant differences in bromocriptine-induced rotation between 1-lesion and 4-lesion models．The results of stepping test showed the motion initiation time of the lesion side was significantly longer than that of the normal side，the stepping length of the lesion side was significantly shorter than that of the normal side，and the adjusting steps of the lesion side was significantly less than that of the normal side (P＜0. 001) ，but there was no significant difference of the lesion side in the initiation time ，stepping length ，and adjusting steps among the three groups of models． Conclusion The striatal lesion and MFB lesion models showed different pathophysiological processes in terms of DA neurotransmitter functions and behavior．The MFB lesion model can mimic primary Parkinson's disease，while the striatal lesion model is similar to some Parkinson syndromes.

Objective To explore the features of frontotemporal lobes'resting-state functional connectivity(rsFC)in preschool children with autism spectrum disorders(ASD)based on functional near-infrared spectroscopy(fNIRS)and to explore the possible neurological markers for early identification of ASD.Methods Sixty-three preschool ASD children and 72 typical development(TD)children were enrolled.Selected bilateral dorsolateral prefrontal cortex(DLPFC),bilateral premotor cortex(PMC),and bilateral temporal lobe(TL)cortex as the regions of interest(ROI).Changes of Oxyhemoglobin in the 6 ROIs in resting-state were measured by using functional near-infrared spectroscopy(fNIRS).Compared the frontotemporal rsFC strength and calculate the laterality index(LI)between two groups.Results Compared with the TD group,rsFC strength was significantly lower in the ASD group(P<0.05),and the differences existed mainly within the left ROIs(0.21±0.11 vs.0.32±0.18),right ROIs(0.16±0.16 vs.0.30±0.14),bilateral DLPFCs(0.20±0.14 vs.0.39±0.17;0.15±0.13 vs.0.36±0.13),bilateral TLs(0.15±0.14 vs.0.28±0.17;0.14±0.15 vs.0.31±0.17),and between the 10 groups of ROIs-ROIs(including right DLPFC-left DLPFC,right DLPFC-right PMC,right DLPFC-left PMC,right DLPFC-right TL,right DLPFC-left TL,left DLPFC-right PMC,left DLPFC-left PMC,left DLPFC-right TL,left DLPFC-left TL,right TL-left TL).There were a significant differences in the rsFC's laterality index of DLPFC and whole-brain between the two groups(t=2.002,P=0.047;t=3.003,P=0.003),and the ASD group showed left-lateralized connectivity.Conclusion Frontotemporal lobe's resting-state functional connectivity is abnormal in preschool children with ASD,characterized by low short-range functional connectivity of bilateral DLPFCs and TLs,low long-range functional connectivity associated with DLPFCs,and left-lateralized connectivity.

It is difficult to treat severe burn combined injury caused by high voltage. This paper reports the successful treatment of a patient with large cranial area third degree burn, skull exposure, local skull carbonization combined with cerebrospinal fluid leakage caused by ten thousand volts high voltage current. Vacuum sealing drainage was given to protect the wounds from infection after multiple limited debridement in the early stage. The external of the inactivated skull was cleaned in the later stage. Finally, the bilateral anterolateral thigh flap combined with multiple burr holes was used to repair the wound. There were no severe postoperative complications, and the therapeutic effect was satisfactory.

It is difficult to treat severe burn combined injury caused by high voltage. This paper reports the successful treatment of a patient with third degree burn of extra large cranial area, skull exposure, local skull carbonization combined with cerebrospinal fluid leakage caused by ten thousand volts high voltage current. Vacuum sealing drainage was given to protect the wounds from infection after multiple limited debridement in the early stage. The external plate of the inactivated skull was cleaned in the later stage. Finally, the bilateral anterolateral thigh flap combined with multiple burr holes was used to repair the wound. There were no severe postoperative complications, and the therapeutic effect was satisfactory.

It is difficult to treat severe burn combined injury caused by high voltage. This paper reports the successful treatment of a patient with large cranial area third degree burn, skull exposure, local skull carbonization combined with cerebrospinal fluid leakage caused by ten thousand volts high voltage current. Vacuum sealing drainage was given to protect the wounds from infection after multiple limited debridement in the early stage. The external of the inactivated skull was cleaned in the later stage. Finally, the bilateral anterolateral thigh flap combined with multiple burr holes was used to repair the wound. There were no severe postoperative complications, and the therapeutic effect was satisfactory.

It is difficult to treat severe burn combined injury caused by high voltage. This paper reports the successful treatment of a patient with third degree burn of extra large cranial area, skull exposure, local skull carbonization combined with cerebrospinal fluid leakage caused by ten thousand volts high voltage current. Vacuum sealing drainage was given to protect the wounds from infection after multiple limited debridement in the early stage. The external plate of the inactivated skull was cleaned in the later stage. Finally, the bilateral anterolateral thigh flap combined with multiple burr holes was used to repair the wound. There were no severe postoperative complications, and the therapeutic effect was satisfactory.