Thiourea-Cu(OTf)2/NIS-synergistically promoted stereoselective glycoside formation with 2-azidoselenoglycosides or thioglycosides as donors.

A novel promoter system for glycosylation is described. A catalytic amount of thiourea and Cu(OTf)2 together with a slight excess of N-iodosuccinimide synergistically promotes glycosylation at room temperature. The combination of reagents applies to some 2-azidoselenoglycoside and thioglycoside donors. A wide range of alcoholic acceptors underwent smooth conversion to O-(2-azido)glycosides with good stereoselectivities. In addition, the value of this method has been highlighted by its convenient operation and outstanding functional group compatibility.

Identifying Genes that Affect Differentiation of Human Neural Stem Cells and Myelination of Mature Oligodendrocytes.

Human neural stem cells (NSCs) are self-renewing, multipotent cells of the central nervous system (CNS). They are characterized by their ability to differentiate into a range of cells, including oligodendrocytes (OLs), neurons, and astrocytes, depending on exogenous stimuli. An efficient and easy directional differentiation method was developed for obtaining large quantities of high-quality of human OL progenitor cells (OPCs) and OLs from NSCs. RNA sequencing, immunofluorescence staining, flow cytometry, western blot, label-free proteomic sequencing, and qPCR were performed in OL lines differentiated from NSC lines. The changes in the positive rate of typical proteins were analyzed expressed by NSCs, neurons, astrocytes, OPCs, and OLs. We assessed Gene Ontology (GO) enrichment and Kyoto Encyclopedia of Genes and Genomes (KEGG) pathways of differentially expressed (DE) messenger RNAs (mRNAs) related to the differentiation of NSCs and the maturation of OLs. The percentage of NSCs differentiated into neurons, astrocytes, and OLs was 82.13%, 80.19%, and 90.15%, respectively. We found that nestin, PAX6, Musashi, and vimentin were highly expressed in NSCs; PDGFR-α, A2B5, NG2, OLIG2, SOX10, and NKX2-2 were highly expressed in OPCs; and CNP, GALC, PLP1, and MBP were highly expressed in OLs. RNA sequencing, western blot and qPCR revealed that ERBB4 and SORL1 gradually increased during NSC-OL differentiation. In conclusion, NSCs can differentiate into neurons, astrocytes, and OLs efficiently. PDGFR-α, APC, ID4, PLLP, and other markers were related to NSC differentiation and OL maturation. Moreover, we refined a screening method for ERBB4 and SORL1, which may underlie NSC differentiation and OL maturation. Potential unreported genes and proteins may regulate differentiation of human neural stem cells into oligodendrocyte lineage. Neural stem cells (NSCs) can differentiate into neurons, astrocytes, and oligodendrocyte (OLs) efficiently. By analyzing the DE mRNAs and proteins of NSCs and OLs lineage, we could identify reported markers and unreported markers of ERBB4 and SORL1 that may underlie regulate NSC differentiation and OL maturation.

Differentiation of geniculate ganglion venous malformation from schwannoma: dynamic T1-weighted imaging provides unique diagnostic value.

OBJECTIVE: To distinguish geniculate ganglion venous malformation (GGVM) from schwannoma (GGS) by using high-resolution CT (HRCT), routine MRI, and dynamic T1-weighted imaging (T1WI) characteristics. METHODS: Surgically confirmed GGVMs and GGSs between 2016 and 2021 were retrospectively included. Preoperative HRCT, routine MR, and dynamic T1WI were performed on all patients. Clinical data, imaging characteristics including lesion size, involvement of facial nerve (FN), signal intensity, enhancement pattern on dynamic T1WI, and bone destruction on HRCT were evaluated. Logistic regression model was developed to identify independent factors for GGVMs, and the diagnostic performance was accessed by receiving operative curve (ROC) analysis. Histological characteristics were explored for both GGVMs and GGSs. RESULTS: Twenty GGVMs and 23 GGSs with mean age of 31 were included. On dynamic T1WI, 18 GGVMs (18/20) showed "pattern A" enhancement (a progressive filling enhancement), while all 23 GGSs showed "pattern B" enhancement (a gradual whole-lesion enhancement) (p < 0.001). Thirteen GGVMs (13/20) showed the "honeycomb" sign whereas all GGS (23/23) showed extensive bone changes on HRCT (p < 0.001). Lesion size, involvement of FN segment, signal intensity on non-contrast T1WI and T2-weighted imaging (T2WI), and homogeneity on enhanced T1WI were obviously differed between two lesions (p < 0.001, p = 0.002, p < 0.001, p = 0.01, p = 0.02, respectively). Regression model showed the "honeycomb" sign and "pattern A" enhancement were independent risk factors. Histologically, GGVM was characterized by interwoven dilated and tortuous veins, while GGS was characterized by abundant spindle cells with dense arterioles or capillaries. CONCLUSIONS: The "honeycomb" sign on HRCT and "pattern A" enhancement on dynamic T1WI are the most promising imaging characteristics for differentiating GGVM from GGS. CLINICAL RELEVANCE STATEMENT: The characteristic sign and enhancement pattern on HRCT and dynamic T1-weighted imaging allow preoperative differentiation of geniculate ganglion venous malformation and schwannoma feasible, which will improve clinical management and benefit patient prognosis. KEY POINTS: • The "honeycomb" sign on HRCT is a reliable finding to differentiate GGVM from GGS. • GGVM typically shows "pattern A" enhancement (focal enhancement of the tumor on early dynamic T1WI, followed by progressive contrast filling of the tumor in the delayed phase), while "pattern B" enhancement (gradual heterogeneous or homogeneous enhancement of the whole lesion) is observed in GGS on dynamic T1WI.

3,5-Di(trifluoromethyl)phenyl(cyano)iodonium triflate as a novel and potential activator for p-tolyl thioglycoside donors.

3,5-Di(trifluoromethyl)phenyl(cyano)iodonium triflate is described as an accessible, stable, and powerful thiophile that can activate batches of p-tolyl thioglycoside donors at room temperature. Various alcoholic acceptors were efficiently glycosylated, providing the desired glycosides. The novel activation protocol features mild conditions as well as high compatibility with some classic strategies for the stereoselective construction of some biologically relevant glycosidic linkages, as exemplified by α-idosides, α-galactoamines, ß-mannosides, and ß-rhamnosides.

Simultaneous Bilateral Cochlear Implantation in Very Young Children Improves Adaptability and Social Skills: A Prospective Cohort Study.

OBJECTIVES: To investigate the value of using the Gesell Development Diagnosis Scale (GDDS) to predict developmental outcomes in very young children who undergo simultaneous bilateral cochlear implantation. DESIGN: In this prospective cohort study, a repeated-measures investigation was conducted in a tertiary referral hospital. A total of 62 children receiving simultaneous bilateral cochlear implantations were enrolled from April 2017 to August 2018. They were divided into 2 groups depending on the operative age: "Infants" group (6 to 12 months, N = 38) or "Children" group (12 to 36 months, N = 24). Data on the surgical outcomes, auditory development, speech production, and developmental indicators were collected until 2 years after the initial fitting. The primary outcome measure was the GDDS, a neuropsychological development examination. Secondary outcomes included the following: complication rate, aided pure-tone average, Infant-Toddler Meaningful Auditory Integration Scale, Categories of Auditory Performance-II, Meaningful Use of Speech Scale, Speech Intelligibility Rating, and the LittlEARS Auditory Questionnaire. RESULTS: The mean ages at implantation in infants and children groups were 9.2 ± 1.17 and 16.6 ± 3.60 months, respectively. Significant differences were found in the social skills ( p = 0.001) and adaptability ( p = 0.031) domains of GDDS. The younger the age of bilateral cochlear implants surgery, the higher developmental quotient of language, social skills, and adaptability the child could achieve after 2 years. The complication rates in the infants and children groups were 0% versus 2.1% ( p = 0.57). There was no surgical complication in the infants group. In the children group, 1 case with enlarged vestibular aqueduct and Mondini malformation had a receiver-implant misplacement on the right side (2%, 1/48). In the two groups, auditory performance and speech production had improved similarly. In the infants group, social skills developmental quotient at baseline had a significant positive relationship with Meaningful Use of Speech Scale after 2 years. CONCLUSIONS: Simultaneous bilateral cochlear implantation in younger children improves adaptability and social skills. GDDS is a sensitive tool of evaluating short-term effect of bilateral cochlear implants in neuropsychological development and constitutes a reliable predictor of speech production for the very younger pediatric cochlear implant users.

Study of 2,881 Nasal Bone Fractures using Multivariate Analysis.

Objective: This retrospective cohort study is aimed to provide a certain reference for the clinical prevention and treatment of nasal bone fracture, and further formulated a more perfect diagnosis and treatment plan. Methods: In detailed cases, 2881 patients with nasal bone fracture were recorded. Its general clinical data, cause of injury, fracture site, and fracture typing were collected through the database. All hospitalized patients admitted to the Ninth People's Hospital Affiliated to the School of Medicine of Shanghai Jiao Tong University with integrated medical records could be retrospectively included from June 2013 to July 2018 and comprehensively analyzed for their gender, age, fracture type and cause of injury. Results: The sex ratio of nasal bone fracture was 2.44:1. The most patients with nasal bone fracture were 19-29 years old (35.6%). The injury rate of traffic accidents was the highest, 33.8%, followed by violent strikes, 24.1%. Statistical analysis showed that the number of patients with nasal bone combined with maxillary frontal bone fracture and type II nasal bone fracture was significantly higher than other fracture types. Logistic multiple regression analysis showed that the relative risk of nasal bone fracture in men was lower (odds ratio, OR = 0.807, P < .05), and the risk of nasal bone fracture decreased with age (OR = 0.978, P < .001). Compared with car accident injury, the relative risk of simple nasal bone fracture comes from violence, exercise or collision [OR = 1.244, P < .05; OR = 1.410, P < .05; OR = 1.453, P < .05]). Conclusion: Given these findings, it's evident that nasal bone fractures exhibit distinct patterns based on individual characteristics, causes of trauma, and injury site, and relevant strategy research should be conducted.

Endoscopic transcanal facial nerve decompression in Bell's palsy: A pilot study.

PURPOSE: To explore the surgical effects of endoscopic facial nerve decompression in Bell's palsy. MATERIALS AND METHODS: This retrospective study included 15 patients with Bell's palsy. All had grade VI (House-Brackmann grading system) complete unilateral facial paralysis before surgery and a >95% reduction in amplitude on electroneurography testing compared to the unaffected side. Their MRI results indicated perineural edema in the geniculate ganglion area. Endoscopic decompression surgery was performed soon after they presented at our hospital. The time between onset of facial paralysis and surgery ranged from 25 to 93 days. All patients had no relevant surgical history or ear diseases. RESULTS: At 1-year follow-up, 13 of the 15 (87%) patients had recovered to normal or near-normal facial function (House-Brackmann grade I-II), and all patients had reached House-Brackmann grade III or lower facial function. No obvious air-bone gap or sensorineural hearing loss occurred after surgery, and there were no severe complications or synkinesis. CONCLUSIONS: Endoscopic transcanal facial nerve decompression provides a less traumatic and improved exposure of the geniculate ganglion, and may also help prevent permanent severe facial sequela. Results of intraoperative facial nerve stimulation may be related to the length of time required for recovery. The optimal time of surgery after onset of paralysis needs to be investigated further, to identify a post-drug surgical therapy which may be more acceptable for patients. Patients' response to conservative treatments should be assessed as soon as possible so as not to delay surgery.

Identifying the functions of two biomarkers in human oligodendrocyte progenitor cell development.

BACKGROUND: Human oligodendrocyte precursor cells (hOPCs) are an important source of myelinating cells for cell transplantation to treat demyelinating diseases. Myelin oligodendrocytes develop from migratory and proliferative hOPCs. It is well known that NG2 and A2B5 are important biological markers of hOPCs. However, the functional differences between the cell populations represented by these two biomarkers have not been well studied in depth. OBJECTIVE: To study the difference between NG2 and A2B5 cells in the development of human oligodendrocyte progenitor cells. METHODS: Using cell sorting technology, we obtained NG2+/-, A2B5+/- cells. Further research was then conducted via in vitro cell proliferation and migration assays, single-cell sequencing, mRNA sequencing, and cell transplantation into shiverer mice. RESULTS: The proportion of PDGFR-α + cells in the negative cell population was higher than that in the positive cell population. The migration ability of the NG2+/-, A2B5+/- cells was inversely proportional to their myelination ability. The migration, proliferation, and myelination capacities of the negative cell population were stronger than those of the positive cell population. The ability of cell migration and proliferation of the four groups of cells from high to low was: A2B5- > NG2- > NG2+ > A2B5+. The content of PDGFR-α+ cells and the ability of cell differentiation from high to low was: NG2- > A2B5- > A2B5+ > NG2+. CONCLUSION: In summary, NG2+ and A2B5+ cells have poor myelination ability due to low levels of PDGFR-α+ cells. Therefore, hOPCs with a higher content of PDGFR-α+ cells may have a better effect in the cell transplantation treatment of demyelinating diseases.

Diagnostic value of dynamic contrast-enhanced magnetic resonance imaging in Bell's palsy.

BACKGROUND: Bell's palsy (BP) is the most common form of acute facial nerve disorder and is characterized by rapid onset peripheral facial palsy of unknown etiology. PURPOSE: To explore the diagnostic value of dynamic contrast-enhanced (DCE) magnetic resonance imagine (MRI) in patients with BP particularly in involved segments. MATERIAL AND METHODS: A retrospective analysis was performed on the patients with BP who underwent routine MRI examinations and volumetric interpolated breath-hold examination (VIBE) sequence-based DCE-MRI before surgery in our department from January 2015 to July 2020. DCE-MRI data postprocessing was performed on Siemens Workstation Extended MR Work Space 2.6.3.5. Statistical analyses were performed using SPSS®v.19.0. The inter-observer reliability was evaluated with kappa identity test and McNemar's test. RESULTS: Twenty-three patients were included. On conventional contrast-enhanced MRI, the two observers were inconsistent in their diagnosis of lesion segments of facial nerve (Kappa 0.426, P = 0.009). Compared to the results of the surgery, the diagnostic consistency of both observers was general (Kappa 0.476, P < 0.001 and Kappa 0.430, P < 0.001, respectively). The diagnostic results of DCE-MRI for lesion segments of the facial nerve were consistent between the two observers (Kappa 0.929, P < 0.001). Compared to the results of the surgery, the diagnostic consistency of both observers was good (Kappa 0.753, P < 0.001 and Kappa 0.731, P < 0.001, respectively). CONCLUSION: Compared to conventional MRI, DCE-MRI has good stability and repeatability in the diagnosis of the lesion segments of the facial nerve as well as a good specificity and accuracy.

Quick stabilization of capillary for rapid determination of potassium ions in the blood of epilepsy patients by capillary electrophoresis without sample pretreatment.

Mutations in the potassium channel genes may be linked to the development of epilepsy and affect the blood potassium levels. Therefore, accurate determination of potassium in the blood will be critical to diagnose the cause of epilepsy. CE is a competent technique for the fast detection of multiple ions, but complicated matrices of a blood sample may cause significant variation of migration times and the peak shape. In this work, a procedure for rapid stabilization of the capillary inner surface through preflushing of a blood sample was employed. The process takes only 40 min for a capillary and then it can be used for more than 2 weeks. No pretreatment of the blood sample or other surface modification of the capillary is needed for the analysis. The RSDs of the migration time and peak area were reduced to 1.5 and 5.1% from 12.6 and 14.5%, respectively. The proposed method has been successfully applied to the determination of the potassium contents in the blood sample of patients with epilepsy at different stages. The recoveries of potassium ions in these blood samples are in a range from 86.5 to 104.5%.

Long non-coding RNA ZEB2-AS1 promotes the proliferation, metastasis and epithelial mesenchymal transition in triple-negative breast cancer by epigenetically activating ZEB2.

The triple-negative breast cancer is the most malignant type of breast cancer. Its pathogenesis and prognosis remain poor despite the significant advances in breast cancer diagnosis and therapy. Meanwhile, long noncoding RNAs (LncRNAs) play a pivotal role in the progression of malignant tumors. In this study, we found that LncRNA-ZEB2-AS1 was dramatically up-regulated in our breast cancer specimens and cells (MDA231), especially in metastatic tumor specimens and highly invasive cells, and high lncRNA-ZEB2-AS1 expression is associated with clinicopathologic features and short survival of breast cancer patients. LncRNA-ZEB2-AS1 promotes the proliferation and metastasis of MDA231 cells in SCID mice. Thus, it is regarded as an oncogene in triple-negative breast cancer. It is mainly endo-nuclear and situated near ZEB2, positively regulating ZEB2 expression and activating the epithelial mesenchymal transition via the PI3K/Akt/GSK3ß/Zeb2 signaling pathway. Meanwhile, EGF-induced F-actin polymerization in MDA231 cells can be suppressed by reducing lncRNA-ZEB2-AS1 expression. The migration and invasion of triple-negative breast cancer can be altered through cytoskeleton rearrangement. In summary, we demonstrated that lncRNA-ZEB2-AS1 is an important factor affecting the development of triple-negative breast cancer and thus a potential oncogene target.

Gene expression profiles between cystic and solid vestibular schwannoma indicate susceptible molecules and pathways in the cystic formation of vestibular schwannoma.

Previous research has shown that although NF2 gene mutation is the major cause of vestibular schwannoma (VS), it may not directly participate in cystic VS (CVS). To elucidate the underlying potential genetic mechanisms in the cystic formation of VS, we compared differences in gene expression between solid VS (SVS) and CVS via a bioinformatics analysis. The cDNA microarray method and miRNA sequencing were performed on 29 representative VSs (17 CVSs and 12 SVSs). A differential expression analysis was used to identify differentially expressed mRNAs (DEmRNAs) and miRNAs (DEmiRNAs). Then, miRNA-mRNA regulatory networks were constructed. Gene ontology (GO), a KEGG pathway enrichment analysis, and the protein-protein interaction (PPI) were used to analyze the co-differentially expressed DEmRNAs at the functional level. From the differential expression analyses, 1304 DEmRNAs, 55 DEmiRNAs, and hub genes including PTEN, FOXO1, FOXO3, VEGFA, and SIRT1 were identified. Histological evidence is presented to confirm the makeup of the hubs, which corresponded with the cDNA microarray. Our analysis revealed that the maps of apoptosis, cellular response to hypoxia, and the PI3K-Akt, AMPK, FOXO, and chemokine signaling pathways were significantly enriched. In addition, the TUNEL assay, immunoblotting analysis, and transmission electron microscope revealed increased degenerative changes in CVS. These findings could be the foundation for understanding the potential role of differential genes in the cystic formation of VS and be helpful in exploring the potential biomarkers for the differential diagnosis, prognosis, and development of drug targets for CVS.

Age-dependent copy number variations of TP53 tumour suppressor gene associated with altered phosphorylation status of p53 protein in sporadic schwannomas.

PURPOSE: Point mutations of TP53 tumour suppressor are very rare in schwannomas. We aim to characterize the frequency of exonic copy-number changes of the gene in the tumour and to examine the association between TP53 alterations, phosphorylation status of p53 protein and clinical phenotypes. METHODS: The alterations of TP53 were screened by a combination of Sanger sequencing and multiplex ligation-dependent probe amplification (MLPA) in a total of 44 vestibular schwannomas. The mutation index (MI) in a tumour was defined as the number of exons mutated/ the number of exons tested. Phosphorylation status of p53 protein was investigated by immunoblotting and immunofluorescence. RESULTS: MLPA analysis showed single and multi-exon deletion mutations of TP53 in 65.7% of the cases. Comparisons of clinical features between mutated and non-mutated patients established an association of TP53 mutations with progressive phenotypes, including an earlier formation and a larger tumour. In addition, there were significant correlations between MI and both patients' age and tumour size. The Ser 392 phosphorylation level of p53 varied among tumours, and correlation analysis revealed an age-dependent phosphorylation pattern. The majority of tumours with hyperphosphorylated p53 were from mutated and young patients, suggesting an association of Ser392 phosphorylation with the mutational status of TP53 involved in the acceleration of tumour growth in young individuals. Moreover, Ser 392 phosphorylation contributed to a nuclear accumulation of p53 in schwannona cultures with TP53 mutation. CONCLUSIONS: An interplay between the mutation status of TP53, phosphorylation patterns and tumour behaviors might be established in the disease.

Human Neural Stem Cells with GDNF Site-Specific Integration at AAVS1 by Using AAV Vectors Retained Their Stemness.

The neural stem cells (NSCs) have the ability to self-renew, and to migrate to pathologically altered regions of the central nervous system. Glial cell derived neurotrophic factor (GDNF) could protect dopamine neurons and rescue motor neurons in vivo, which has been proposed as a promising candidate for the treatments of degenerative neurological diseases. In order to combine the advantages of neurotrophic factors and stem cells in clinical therapy, we established the modified hNSCs that has site-specific integration of GDNF gene by using recombinant adeno-associated virus (rAAV) vectors. The hNSCs were co-infected by rAAV2-EGFP-GDNF and rAAV2-SVAV2 which provide integrase to specifically integrate GDNF gene into AAVS1 site. The GDNF-hNSCs maintained their original stem cell characteristics and the ability to differentiate into neurons in vitro. In the animal model, the GDNF-hNSCs were specifically transplanted into CA1 area of hippocampi and could migrate to the dentate gyrus region and differentiate into neuronal cells while maintaining GDNF expression. hNSCs with GDNF gene site-specific integration at AAVS1 by using AAV vectors retained their stemness and effectively expressed GDNF, which indicates the potential of employing transplanted hNPCs for treatment of brain injuries and degenerative neurological diseases.

Impact of cochlear implantation on the management strategy of patients with neurofibromatosis type 2.

PURPOSE: To investigate the outcomes of cochlear implantation in patients with neurofibromatosis type 2 (NF2), and to discuss the current management strategy for NF2 patients. METHODS: The medical records of NF2 patients who received cochlear implants (CI) at our center between 2012 and 2016 were retrospectively reviewed. Pre-operative hearing status, tumor status, treatment of tumors, and auditory outcomes post-implantation were evaluated. RESULTS: Twelve patients were included in the study. Five were implanted with the tumor in situ; two of them received radiotherapy pre-implantation, and three were implanted without any previous treatment. Four patients were implanted simultaneously with tumor removal. Three patients were implanted as second-stage after failed hearing preservation surgery. The mean pure tone audiometry with the implant was 44 dB (range 25-80 dB) and the mean sentence recognition score (SRS) in a quiet environment without lip reading was 63% (range 0-97%). A poorer objective auditory outcome was identified in one patient who showed no response to electrical promontory stimulation (EPS), but the sound perception was still helpful. In total, 11 of 12 (91%) patients were daily users, and the other patient used the implant as a "sleeper" device due to its interference with contralateral hearing provided by a hearing aid. CONCLUSION: CI is an effective option in auditory rehabilitation and should be considered primarily for NF2 patients with intact cochlear nerve. EPS might be a predictor for cochlear implant performance. Good contralateral hearing may present a barrier to daily use.

The therapy with ethosomes containing 5-fluorouracil for laryngotracheal stenosis in rabbit models.

The aim of this study is to evaluate the efficacy of ethosomes encapsulated with 5-fluorouracil (5-FU) in treatment of laryngotracheal stenosis in rabbit models. The 5-FU ethosome was prepared by the thin film hydration method, and the amorphous, size distribution and the encapsulation efficiency was investigated. The tracheal mucosa were scraped about 0.5 cm with a nylon brush to induce the scar in airway grow, then models were divided into three groups: 5-FU ethosome group, 5-FU group and saline group, drug were injected into scar of every group by paracentesis guided under endoscope, respectively. The stenosis states were observed under laryngo fiberscope immediate, 7, 14 and 21 days after administrated. Airway stenosis of 5-FU ethosome group has no significant difference when compared with 5-FU group at 7 days after administration, but 5-FU ethosome significantly reduced the airway stenosis after 21-day administration when compared with 5-FU group again and has no restenosis during the period under observation. The fact that ethosomes encapsulated with 5-FU were effective for laryngotracheal stenosis suggests that it has potential as a new method for ameliorating airway stenosis originating from granulation tissue.

[Long-term effect of oligodendrocyte precursor cell transplantation on a rat model of white matter injury in the preterm infant].

OBJECTIVE: To investigate the long-term effect of oligodendrocyte precursor cell (OPC) transplantation on a rat model of white matter injury (WMI) in the preterm infant. METHODS: A total of 80 Sprague-Dawley rats aged 3 days were randomly divided into sham-operation group, model control group, 5-day ventricular/white matter transplantation group, 9-day ventricular/white matter transplantation group, 14-day ventricular/white matter transplantation group (n=10 each). All groups except the sham-operation group were treated with right common carotid artery ligation and hypoxia for 80 minutes to establish a rat model of WMI in the preterm infant. OPCs were prepared from the human fetal brain tissue (10-12 gestational weeks). At 5, 9, and 14 days after modeling, 3×105 OPCs were injected into the right lateral ventricle or white matter in each transplantation group, and myelin sheath and neurological function were evaluated under an electron microscope at ages of 60 and 90 days. RESULTS: Electron microscopy showed that at an age of 60 days, each transplantation group had a slight improvement in myelin sheath injury compared with the model control group; at an age of 90 days, each transplantation group had significantly thickened myelin sheath and reduced structural damage compared with the model control group, and the 14-day transplantation groups had the most significant changes. There were no significant differences in the degree of myelin sheath injury between the ventricular and white matter transplantation groups at different time points. At an age of 60 or 90 days, the transplantation groups had a significantly higher modified neurological severity score (mNSS) than the sham-operation group and a significantly lower mNSS than the model control group (P<0.05). CONCLUSIONS: OPC transplantation may have a long-term effect in the treatment of WMI in the preterm infant, and delayed transplantation may enhance its therapeutic effect.

Clinical comparison of two subtypes of cystic vestibular schwannoma: surgical considerations and outcomes.

Cystic vestibular schwannoma (CVS) is classified as Type A and Type B based on the overall cyst location and cyst wall thickness in magnetic resonance imaging. A retrospective analysis was performed to compare surgical considerations and outcomes between Type A and Type B groups of CVS. We selected 188 patients diagnostic for CVS with surgical resection, and divided them into Type A and Type B groups. General information, preoperative symptoms, the result of neuroimaging, and audiological tests were recorded. Surgical approach, completeness of tumor resection, and intraoperative facial nerve (FN) integrity were taken down. After operation, the short-term and long-term FN functions, complications, and recurrence rate were evaluated. The total tumor removal rate in Type A group was higher than that in Type B group (86.1 vs 72.5 %, p = 0.021). Anatomical FN integrity was preserved in 173 patients (92.0 %), with no significant differences between Type A and Type B. FN function was better in Type A group at hospital discharge. Besides, a good FN function rate was inversely proportional to the tumor size. The long-term FN function and all of the complications had no significant differences between the two groups. Patients in the Type B group are prone to have a lower total tumor removal rate and transient FN dysfunction. The long-term FN function was similar in both groups. Tumor size is another important indication of FN function. All postoperative complications occurred in patients with a tumor larger than grade 3, regardless of the subtypes of CVS.

Hearing Restoration for Adults with Vestibular Schwannoma in the Only Hearing Ear: Ipsilateral or Contralateral Cochlear Implantation?

OBJECTIVE: To explore auditory outcomes following cochlear implantation (CI) in patients with vestibular schwannoma (VS) in the only hearing ear. METHODS: Three patients, all with a long history of hearing loss on one side and with newly diagnosed VS on the other side, underwent ipsilateral or contralateral CI. Their clinical data were collected retrospectively. Postoperative hearing outcomes were measured during follow-up and compared with the preoperative test results. A thorough search of the English-language literature was performed. RESULTS: Patients 1 and 2 underwent CI in the ipsilateral and contralateral ear, respectively, without tumor removal; patient 3 underwent CI after tumor resection. At the last follow-up, the result of pure-tone audiometry was 25, 45, and 25 dB, respectively. An open-set speech discrimination score was achieved in all 3 patients, with monosyllabic word recognition of 60, 30, and 75%, respectively. Besides the patients included in our study, 28 CI cases with VS in the only hearing ear have been reported up to now. CONCLUSIONS: In patients with VS in the only hearing ear, significant hearing deterioration with no obvious tumor growth is a good indication for ipsilateral CI. Long-term deafness in the tumor-free ear is not an absolute contraindication for CI.

A Novel Missense Mutation of NOG Interferes With the Dimerization of NOG and Causes Proximal Symphalangism Syndrome in a Chinese Family.

OBJECTIVES: NOG is an antagonist to bone morphogenetic proteins and plays an important role in proper bone and joint development. Dominant mutations in NOG may lead to a series of symphalangism spectrum disorders. In this study, we aimed to identify the genetic cause and the pathogenic mechanism of an autosomal dominant disorder with cosegregating proximal symphalangism and conductive hearing impairment in a Chinese family. METHODS: Mutation screening of NOG was performed in the affected family members by polymerase chain reaction (PCR) amplification and direct sequencing. Western blotting analysis of NOG was performed in the leukocyte samples of the family members. RESULTS: A novel p.W150C heterozygous mutation in NOG was identified cosegregating with the proximal symphalangism disorder in the family. Western blotting analysis showed that the p.W150C mutation interferes with the dimerization of the mutant NOG. CONCLUSIONS: Our results agreed with previously published results of in vitro studies and suggested that impaired dimerization of mutant NOG is an important pathogenic mechanism for the NOG-related symphalangism spectrum disorder.