Novel GPR156 variants confirm its role in moderate sensorineural hearing loss.

Hereditary hearing loss (HL) is a genetically heterogeneous disorder affecting people worldwide. The implementation of advanced sequencing technologies has significantly contributed to the identification of novel genes involved in HL. In this study, probands of two Turkish families with non-syndromic moderate HL were subjected to exome sequencing.

Mesoporous SiC-Based Photocatalytic Membranes and Coatings for Water Treatment.

Photocatalytically active silicon carbide (SiC)-based mesoporous layers (pore sizes between 5 and 30 nm) were synthesized from preceramic polymers (polymer-derived ceramic route) on the surface and inside the pores of conventional macroporous α-alumina supports. The hybrid membrane system obtained, coupling the separation and photocatalytical properties of SiC thin films, was characterized by different static and dynamic techniques, including gas and liquid permeation measurements. The photocatalytic activity was evaluated by considering the degradation efficiency of a model organic pollutant (methylene blue, MB) under UV light irradiation in both diffusion and permeation modes using SiC-coated macroporous supports.

Hybrid system coupling ozonation and nanofiltration with functionalized catalytic ceramic membrane for ibuprofen removal.

The investigations on the removal of ibuprofen (IBU) in a hybrid system coupling ozonation and nanofiltration with functionalized catalytic ceramic membrane are presented. The gaseous ozone into feed water in concentration of 11 g Nm was supplied. Positive influence of catalytic ozonation on ibuprofen decomposition was observed.

Real-Time Capturing of Microscale Events Controlling the Sintering of Lead-Free Piezoelectric Potassium-Sodium Niobate.

Sintering is a very important process in materials science and technological applications. Despite breakthroughs in achieving optimized piezoelectric properties, fundamentals of K Na NbO (KNN) sintering are not yet fully understood, facing densification versus grain growth competition. At present, microscale events during KNN sintering under reducing atmospheres are real-time monitored using a High Temperature-Environmental Scanning Electron Microscope.

Ceramic Membranes Photocatalytically Functionalized on the Permeate Side and Their Application to Water Treatment.

This work deals with direct coupling of membrane separation and photocatalytic degradation by using photocatalytic ceramic membranes. An unusual configuration is considered here, with the irradiation applied on the permeate side of the membrane in order to mineralize small organic molecules not retained by the membrane. Different types of such membranes are presented.

Surface Properties of Alkoxysilane Layers Grafted in Supercritical Carbon Dioxide.

Silicon oxide surface properties can be easily modified by grafting alkoxysilane molecules. Here, we studied the structure and the morphology of ultrathin layers prepared by the grafting of alkoxysilanes having different head groups (thiol, amine, and iodo) in supercritical carbon dioxide (CO) on model plane silicon oxide surfaces. Several characterization techniques (X-ray reflectivity, water contact angle, X-ray photoelectron spectroscopy, and atomic force microscopy (AFM)) were used to determine the physicochemical properties of the layers prepared at different temperatures.

Voxel-Based Statistical Analysis of 3D Immunostained Tissue Imaging.

Recently developed techniques to visualize immunostained tissues in 3D and in large samples have expanded the scope of microscopic investigations at the level of the whole brain. Here, we propose to adapt voxel-based statistical analysis to 3D high-resolution images of the immunostained rodent brain. The proposed approach was first validated with a simulation dataset with known cluster locations.

Gradenigo Syndrome Induced by Suppurative Otitis Media.

Gradenigo syndrome was defined as a clinical triad of otitis media, severe orbito-facial pain, and ipsilateral sixth cranial nerve palsy. It may occur as a complication of either acute or chronic otitis media. Herein, we will report a case of Gradenigo syndrome induced by suppurative chronic otitis media that was promptly diagnosed and aggressively treated.

Sol-Gel Synthesis and Fine Characterization of Hierarchically Porous and Multifunctional Silica-Based Membranes.

Rational design of thin films for improving their functional properties can result in complex architectures. This issue will be here illustrated in the case of sol-gel derived hierarchically porous and multifunctional silica-based membranes. Thin silica layers with isolated and randomly distributed macropores, isolated and ordered mesopores and an interconnected microporosity in the silica walls are thus obtained by combining different synthesis strategies.

A honey bee can threat ear: Sudden sensorineural hearing loss.

Sudden sensorineural hearing loss is an otologic emergency. Many etiological factors can lead to this pathology. Honey bee (Apis mellifera) sting may lead to local and systemic reactions due to sensitization of the patient.

The Relationship Between Hearing Thresholds and Hyperandrogenism in Polycystic Ovary Syndrome.

BACKGROUND The purpose of our study was to investigate the association between polycystic ovary syndrome (PCOS) and hearing thresholds. MATERIAL AND METHODS Forty women diagnosed with PCOS (mean age, 24.33±6.

The Reductive Dehydration of Cellulose by Solid/Gas Reaction with TiCl at Low Temperature: A Cheap, Simple, and Green Process for Preparing Anatase Nanoplates and TiO /C Composites.

Metal oxides and metal oxide/carbon composites are entering the development of new technologies and should therefore to be prepared by sustainable chemistry processes. Therefore, a new aspect of the reactivity of cellulose is presented through its solid/gas reaction with vapour of titanium(IV) chloride in anhydrous conditions at low temperature (80 °C). This reaction leads to two transformations both for cellulose and titanium(IV) chloride.

Evidence of Trivalent Am Substitution into UO.

UO is considered to be the most stable phase for uranium oxide. Its structural properties must be accurately understood to foresee and manage aspects such as its leaching behavior when spent nuclear fuel is stored in an oxidative environment. Moreover, as fuel irradiation causes the formation of fission products and activation products such as plutonium and minor actinides, it is probable that UO will be mixed with other chemical elements under real conditions of oxidation.

Variations in Multiple Syndromic Deafness Genes Mimic Non-syndromic Hearing Loss.

The genetics of both syndromic (SHL) and non-syndromic hearing loss (NSHL) is characterized by a high degree of genetic heterogeneity. We analyzed whole exome sequencing data of 102 unrelated probands with apparently NSHL without a causative variant in known NSHL genes. We detected five causative variants in different SHL genes (SOX10, MITF, PTPN11, CHD7, and KMT2D) in five (4.

ADAM30 Downregulates APP-Linked Defects Through Cathepsin D Activation in Alzheimer's Disease.

Although several ADAMs (A disintegrin-like and metalloproteases) have been shown to contribute to the amyloid precursor protein (APP) metabolism, the full spectrum of metalloproteases involved in this metabolism remains to be established. Transcriptomic analyses centred on metalloprotease genes unraveled a 50% decrease in ADAM30 expression that inversely correlates with amyloid load in Alzheimer's disease brains. Accordingly, in vitro down- or up-regulation of ADAM30 expression triggered an increase/decrease in Aβ peptides levels whereas expression of a biologically inactive ADAM30 (ADAM30(mut)) did not affect Aβ secretion.

In situ characterization of uranium and americium oxide solid solution formation for CRMP process: first combination of in situ XRD and XANES measurements.

Transmutation of americium in heterogeneous mode through the use of U1-xAmxO2±δ ceramic pellets, also known as Americium Bearing Blankets (AmBB), has become a major research axis. Nevertheless, in order to consider future large-scale deployment, the processes involved in AmBB fabrication have to minimize fine particle dissemination, due to the presence of americium, which considerably increases the risk of contamination. New synthesis routes avoiding the use of pulverulent precursors are thus currently under development, such as the Calcined Resin Microsphere Pelletization (CRMP) process.

Effect of gas adsorption on acoustic wave propagation in MFI zeolite membrane materials: experiment and molecular simulation.

The present study reports on the development of a characterization method of porous membrane materials which consists of considering their acoustic properties upon gas adsorption. Using acoustic microscopy experiments and atomistic molecular simulations for helium adsorbed in a silicalite-1 zeolite membrane layer, we showed that acoustic wave propagation could be used, in principle, for controlling the membranes operando. Molecular simulations, which were found to fit experimental data, showed that the compressional modulus of the composite system consisting of silicalite-1 with adsorbed He increases linearly with the He adsorbed amount while its shear modulus remains constant in a large range of applied pressures.

Increased expression of BIN1 mediates Alzheimer genetic risk by modulating tau pathology.

Genome-wide association studies (GWAS) have identified a region upstream the BIN1 gene as the most important genetic susceptibility locus in Alzheimer's disease (AD) after APOE. We report that BIN1 transcript levels were increased in AD brains and identified a novel 3 bp insertion allele ∼28 kb upstream of BIN1, which increased (i) transcriptional activity in vitro, (ii) BIN1 expression levels in human brain and (iii) AD risk in three independent case-control cohorts (Meta-analysed Odds ratio of 1.20 (1.

Sonochemical deposition of platinum nanoparticles on polymer beads and their transfer on the pore surface of a silica matrix.

This study reported the sonochemical deposition of platinum on the surface of polystyrene beads (PSBs) and the transfer of obtained Pt nanoparticles into a porous silica matrix using the PSB as a sacrificial template. Platinum nanoparticle deposition was ensured by the sonochemical reduction of Pt(IV) at room temperature in latex solutions containing polystyrene beads in the presence of formic acid under Ar or under Ar/CO atmosphere without any additives. After ultrasonic treatments for few hours, well dispersed Pt nanoparticles within the range of 3-5 nm deposited on PSB were obtained in both studied conditions.

Surface modification of titania powder P25 with phosphate and phosphonic acids--effect on thermal stability and photocatalytic activity.

Phosphorus is frequently reported as a doping element for TiO(2) as photocatalyst; however, the previously reported methods used to prepare P-doped TiO(2) do not allow control over the location of the phosphorus either in the bulk or at the surface or both. In this study, we report on the surface modification of Evonik P25 with phosphonic (H(3)PO(3)) and octylphosphonic acid [C(8)H(17)-PO(OH)(2)], done to limit the introduction of phosphorus only to the photocatalyst surface. The effect of this element on the thermal behavior and photocatalytic properties is reported through characterization using elemental analyses, solid state (31)P NMR, X-ray powder diffraction, N(2) porosimetry, dilatometry, etc.

Diffusion control of porous membrane by modifying the nanopore properties.

We have investigated the diffusion of various solvents on nanoporous membranes with various pore size and surface energy. We have modified the size of pore channel and surface energy of porous membrane through grafting different sized alkyl chain on inorganic membranes. Typically, disc type zirconia membranes with pore size of 3 nm and silica ones with 1 nm pore were purchased from Inocermic Co.