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 improve the understanding of idiopathic hypereosinophilic syndrome (HES) and to be aware of its potential of transforming to acute myeloid leukemia (AML). Methods: The clinical data of one patient diagnosed with HES progressed to AML in the 923rd Hospital of the People's Liberation Army Joint Service Support Force were analyzed, and relevant literatures were reviewed. Results: The patient was diagnosed with idiopathic HES that progressed to AML with FIP1-like-1-platelet-derived growth factor receptor α (FIP1L1-PDGFRA) after 2 years. The patients achieved complete remission after the treatment of chemotherapy combined with tyrosine kinase inhibitor and then received hematopoietic stem cell transplantation. The patient had more than 1 year disease-free survival until the deadline. Conclusion Idiopathic HES has the potential to transform to AML with FIP1L1-PDGFRA fusion gene positive, therefore regular follow-up should be emphasized.

A variety of views and approaches were found in the appraisal and judgments of the people's courts in various provinces and cities,on medical disputes involving absence of a hand or foot of newborns,which result in different or event contradictory court decisions.This study made an analysis of the characteristics,nature,and classification of such cases as well as countermeasures of medical institutions,with recommendations on rational and appropriate handling of such cases,for the purposes of legitimate rights maintenance of both doctors and patients,and promotion of mutual understanding between the two parities,and establishment of a harmonious doctor-patient relationship.

Consuming an adequate amount of iodine during pregnancy is critical for fetal neurologic development.Even a mild deficiency can impair cognitive ability.The World Health Organization recently has increased their recommended iodine intake during pregnancy from 200 to 250 μg/d and suggested that a median urinary iodine concentration of 150 ～ 249 μg/L indicates adequate iodine intake in pregnant women.Whether mild-to-moderate maternal iodine deficiency produces more subtle changes in cognitive function in offspring is unclear;no controlled intervention studies have measured long-term clinical outcomes.This review summarizes the iodine nutrition of pregnant women,and the effects of iodine deficiency in pregnancy on neurodevelopment in the child.To prevent fetal damage,iodine should be given before or early in pregnancy.

Objective To observe the effects of different levels manganese (Mn) on spatial learning and memory in neonate rats.Methods Neonate rats were distributed to control (normal saline) and MnCl210,20,30mg/kg groups randomly.Each groups included 10 litters in a cage with a dam.Neonate rats were intraperitoneal injection exposed to MnCl2 over PND 1-21.All groups were evaluated behavioral performance using open field and Morris water maze.Blood and hippocampus Mn levels were determined using ICP-MS.Results 1) For each group,blood Mn were (35.58 ± 13.77) μg/L,(80.00 ± 12.98) μg/L,(238.51 ± 31.43) μg/L,(348.47 ±34.07) μg/L and hippocampus Mn were (576.82 ± 79.78) μg/g,(798.33 ± 40.60) μg/g,(1017.23 ± 117.23)μg/g,(1278.76 ± 281.48) μg/g respectively.Blood and hippocampus Mn concentrations in Mn-exposed groups were significant increased compared to control (P ＜ 0.01),and there was a positive correlation in blood Mn and hippocampus Mn(OR =0.91,95％ CI=0.81-0.96,P＜ 0.01).2) Therewere no significant differences on travelled distance in open field among all groups,which meant that Mn exposure had no effect on their locomotion.3) In the hidden platform trials of the Morris water maze test,only on 3rd day,Mn-expose groups spent more time to find the platform compared to the control(P ＜ 0.01).The average escape latency were(21.77 ± 7.10)s,(33.78 ± 9.95)s,(37.17 ± 13.68) s,(41.92 ± 16.74) s respectively.Though the latency were increased with the Mn exposure levels increasing among the Mn-expose groups,no statistically significant differences were observed.There were no statistically effects on latency to find the platform of all groups in other training days.The result in probe trails showed that there were no statistically effects on swimming velocity,the number of crossing over the former platform and the time spent in the targeted quadrant.Conclusion Mn exposure exerts effects on the learning,but no doseeffect relationship.There are no effects on memory of neonate rats of Mn exposure.