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 observe the clinical efficacy of Jiangshabanxia nano-paste on nausea and vomiting in end-stage patients and its effect on the quality-of-life (QOL) in cancer patients. Methods 120 end-stage patients with nausea and vomiting symptoms above grade III were randomly divided into observation group and control group. They were treated with Jiangshabanxia nano-paste and placebo paste respectively. The paste patch was changed every 24 hours and used continuously for 7 days. The nausea and vomiting symptom score, the quality-of-life measurement score and KPS score of cancer patients in the two groups were observed to evaluate the curative effect. Results After 7 days of treatment, the symptom scores of nausea and vomiting in the observation group decreased significantly, the KPS score of the observation group increased, and the effective rate was higher than that in the control group. The score of QOL measurement showed that after treatment, the score of main symptom areas and other symptom areas (except external dyspnea, diarrhea and economic difficulties) in the observation group decreased, and the score of overall health area increased. After treatment, the score of main symptom areas and other symptom areas (except external dyspnea, diarrhea and economic difficulties) in the observation group was lower than that in the control group, and the scores of overall health area in the observation group were higher than those in the control group. Conclusion Jiangshabanxia nano-paste has a good clinical efficacy nausea and vomiting in end-stage patients, it also can improve the quality of life end-stage cancer patients.

Objective To study the clinical characteristics of Japanese encephalitis (JE) in 34 adult patients and to improve the level of diagnosis of this disease.Methods The clinical manifestations,laboratory results and radiological features of 34 adult patients with JE in our hospital from July 2017 to September 2017 were summarized and the progonsis was observed.The modified Rankin Scale (mRS) was used to evaluate the progonsis.Results Eighteen patients were males and 16 patients were females with the average age of (45.39 ± 16.34) years in 34 patients who were diagnosed as JE.The major clinical features of JE patients included fever (34,100％) with the average temperature of (39.4 ± 1.1) ℃ on admission,headache (26,76％),seizures (7,21％),decreased consciousness (25,74％) on day 2.6 ± 1.4 after the onset,respiratory failure (9,26％) on day 3.8 ± 1.6 after the onset.The major features of laboratory results included white blood cells increase (15,44％),blood hematocrit decrease (25,74％),eosinophil absolute value decrease (29,85％),cerebrospinal fluid pressure increase (12,35％),cerebrospinal fluid protein increase (27,79％),cerebrospinal fluid white blood cells increase (30,88％).Brain MRI scan of abnormal signal was found abnormal in up to 54％patients (14/26),involving the thalamus,basal ganglia,mesencephalon,temporal lobe,hippocampus and occipital lobe,especially in the area of bilateral thalamus and mesencephalon.The follow-up showed three cases were dead;mRS score was 0 in twenty-one cases,1 or 2 in five cases,3 or 4 in three cases,5 in two cases five-six months after onset;the sequelaes were cognitive impairment in nine patients and movement disorder in five patients.Conclusions The clinical symptoms of JE in adults are severe.The main clinical manifestations of JE are hyperthermia,disturbance of consciousness,seizures and respiratory failure,with characteristic imaging findings on brain MRI.JE is a disease with high mortality and severe long-term sequelae.

Objective To evaluate the immune effects of bivalent inactivated rotavirus vaccine (IRV) and investigate the viability of development of bivalent IRV.Methods Firstly,bivalent IRV was prepared by mixing G1 IRV and G3 IRV with equal amount,G1 IRV and G3 IRV as monovalent control,PBS as negative control.Secondly,those vaccines were vaccinated to the mice by intramuscular injection.Then,to evaluate the immune effects of bivalent IRV,the levels of serum or fecal rotavirus specific IgG and IgA were assessed by ELISA,the levels of serum neutralized antibody were measured by microneutralization assay,the number of IFN-γ or IL-4 secreting cells were analyzed by ELISPOT assay.Results Compared to negative control group,bivalent IRV induced the higher levels of serum and fecal G1 and G3 rotavirus specific antibody.It was found that there were no significant differences for the levels of serum IgG and IgA,fecal IgG and IgA,serum neutralized antibody between induced by bivalent IRV and induced by G1 type monovalent vaccines ; but there were significantly increase for the levels of serum IgG (t =2.691,P＜0.05) and serum neutralized antibody (t =2.561,P＜0.05) between induced by bivalent IRV and induced by G3 monovalent vaccines,there were no significant differences for other antibodies between induced by bivalent IRV and induced by G3 monovalent vaccines.At the same time,compared to negative control group,bivalent IRV induced significantly increase in the number of IFN-γ or IL-4 secreting cells in spleen lymphocytes.It was found that there were no significant differences for the number of IFN-γ or IL-4 secreting cells stimulated by G1 rotavirus between bivalent IRV and G1 monovalent vaccines; but there were significantly increase for the number of IL-4 secreting cells (t =2.327,P＜0.05) stimulated by G3 rotavirus between bivalent IRV and G3 monovalent vaccines,there were no significant differences for the number of IFN-γ secreting cells stimulated by G3 rotavirus between bivalent IRV and G3 type monovalent vaccines.Conclusion The bivalent IRV can induce effective immune response,in which there were no inhibitory interference between the components of bivalent IRV,which provided the experimental basis for the development of bivalent IRV.

Objective To evaluate the clinical efficacy and safety of endovascular treatment for intracranial venous sinus thrombosis based on individual condition. Methods Twelve patients with intracranial venous sinus thrombosis were treated with endovascular management according to the severity and course of disease after they failed to respond to anticoagulant therapy. The clinical signs and symptoms,cerebrospinal fluid pressure and arteriovenous circulation time were observed and followed up (including MRV). Intravenous thrombolysis and mechanical thrombus maceration were carried out in all 12 patients,while intravenous thrombolysis, mechanical thrombus maceration in combination with intra-arterial thrombolysis were employed in 3. After the treatment, anticoagulant therapy was carried out for 6 months.The patients were followed up for 12 to 24 months. Results Of the twelve patients, clinical signs and symptoms included slight headache (2 cases), mild hemiplegia (1 case), ambiopia or blurred vision (3 cases). The cerebrospinal fluid pressure returned to under 26 cm H2O (1 cm H2O =0.098 kPa)following treatment from 28 to 38 cm H2O [ mean (32. 4 ±3.0) cm H2O] in preoperative measurement and the arteriovenous circulation time returned to below 10 s in all patients following treatment. Neither recurrence of thrombosis nor new symptoms of neurologic dysfunction was observed. No procedure-related intracranial or systemic hemorrhagic complications occurred both during and after the operation with the exception of a subcutaneous bleeding at the venopuncture site. Conclusion Endovascular treatment is effective and safe for patients with intracranial venous sinus thrombosis.

The synchronous fluorescence spectra of myoglobin were studies for the first time. The fluorescence peaks observed in the spectra were assigned. When the wavelength interval (Δλ) is 80 nm, the main peak at 335 nm is originated from the tryptophan residues in the myoglobin molecule. When Δλis 20 mn, the peak at 308 nm is mainly due to the tyrosine residues in the myoglobin molecule and in a small part due to the tryptophan residues.Two peaks at 322 and 596 nm were observed in the spectrum of myoglobin for Δλ = 40 nm. The peak at 322 nm is due to both tyrosine and tryptophan residues. The peak at 596 nm is attributed to the heme group in the myoglobin molecule .

Objective To study the association of single nucleotide polymorphism (SNP) of the protein tyrosine phosphatase-1B (PTP-1B) gene with type 2 diabetes mellitus and obesity in Chinese. Methods SNPs in the PTP-1B gene were detected by direct sequencing to PCR products, and the detected SNPs were genotyped in case-spouse samples with the technique of fluorescence real-time PCR. Results Totally 6 SNPs were found in PTP-1B gene. Three SNPs (I5/37 C→A,I6/82 A→G, I7/301 C→T) were in the intron regions and the other 3 (E8/45 C→T, E9/35 G→A, E10/372 G→A)in the exon regions. Among them, E9/35 G→A was a newly found mutation site. The A allele frequency of I5/37 C→A, T allele frequency of I7/301 C→T and G allele frequency of I6/82 A→G in type 2 diabetes were significantly higher than those in the normal spouse group (P