A novel mutation in COL1A1 causing osteogenesis imperfecta/hearing loss.

Objectives: To screen the COL1A1 and COL1A2 gene mutation sites in a family with type I osteogenesis imperfecta (OI)/hearing loss and analyze the characteristics and recovery of hearing loss in patients with osteogenesis imperfecta.

Methods: The basic clinical data of OI proband and her parents were collected, and the COL1A1 and COL1A2 genes were detected in peripheral blood by PCR amplification and generation Sanger sequencing. Literature of stapedial surgery in patients with osteogenesis imperfecta was collected.

Hierarchical Macroporous Agarose Materials with Polyethyleneimine-Assisted Multiple Boronate Affinity Binding Sites for the Separation of Neomycin.

Quantification of neomycin residues in food samples demands an efficient purification platform. Herein, hierarchical macroporous agarose monoliths with multiple boronate affinity sites were established for selective separation of neomycin. The silica core was synthesized by "one-step" Stöber procedures followed by modification with amino group and incorporation of polyethyleneimine.

Feasibility Study on the Fused Filaments of Injection-Molding-Grade Poly(Ethylene Terephthalate) for 3D Printing.

Unlike that of glycol-modified Poly(ethylene terephthalate) (PETG), the crystallinity of PET can be post-adjusted to enhance the mechanical properties of 3D-printed parts such as food-contact tableware and bio-implants. The aforementioned PET material could be 3D printed to produce the desired parts for performance evaluation before mass production by injection molding. In this study, using differential scanning calorimetry (DSC), we examined the pellets, extruded filament, and printed specimen to identify variations in melting and crystalline temperatures, as well as crystallinity.

Effect of treatment modality on the long-term survival of patients with early glottic squamous cancer: a retrospective cohort study based on the SEER database.

Background: The optimal treatment strategy for patients with early glottic (T1-2N0M0) squamous cancer remains unclear.

Methods: A retrospective population-based analysis was performed using the Surveillance, Epidemiology, and End Results (SEER) database. Propensity score matching (PSM) was used to balance treatment arms, and Cox regression analysis was used to determine prognostic factors for survival.

Primary Middle Ear Meningioma with Intact Tympanic Membrane: A Case Report and Literature Review.

Primary ectopic meningioma of the middle ear is relatively rare in clinical practice. It is often difficult to distinguish it from chronic otitis media or otitis media with effusion due to its similar and atypical clinical symptoms. We report a case of epithelial tympanic ectopic meningioma with the main complaints of otalgia, aural fullness, and hearing loss.

Treatment With Calcineurin Inhibitor FK506 Attenuates Noise-Induced Hearing Loss.

Attenuation of noise-induced hair cell loss and noise-induced hearing loss (NIHL) by treatment with FK506 (tacrolimus), a calcineurin (CaN/PP2B) inhibitor used clinically as an immunosuppressant, has been previously reported, but the downstream mechanisms of FK506-attenuated NIHL remain unknown. Here we showed that CaN immunolabeling in outer hair cells (OHCs) and nuclear factor of activated T-cells isoform c4 (NFATc4/NFAT3) in OHC nuclei are significantly increased after moderate noise exposure in adult CBA/J mice. Consequently, treatment with FK506 significantly reduces moderate-noise-induced loss of OHCs and NIHL.

Narrow-band red emitting oxonitridosilicate phosphor LaSrSiNO:Pr (x≈ 1.69).

The new oxonitridosilicates LnSrSiNO (Ln = La, Ce) were synthesized by high temperature solid-state reactions. The crystal structures were solved and refined from both single-crystal and powder X-ray diffraction data. These oxonitridosilicate compounds crystallize in the monoclinic space group P2/n (no.

Iron-chelated thermoresponsive polymer brushes on bismuth titanate nanosheets for metal affinity separation of phosphoproteins.

Separation of phosphoproteins plays an important role for identification of biomarkers in life science. In this work, bismuth titanate supported, iron-chelated thermoresponsive polymer brushes were prepared for selective separation of phosphoproteins. The iron-chelated thermoresponsive polymer brushes were synthesized by surface-initiated atom transfer radical polymerization of N-isopropylacrylamide and glycidyl methacrylate, followed by a ring opening reaction of epoxy group, and chelation of the obtained cis-diols with Fe ions.

Narrow-band blue emitting nitridomagnesosilicate phosphor SrMgSiN:Eu for phosphor-converted LEDs.

We report a novel narrow-band blue emitting phosphor Sr7.92Mg7Si9N22:0.08Eu2+.

Selective Phosphorylation of AMPA Receptor Contributes to the Network of Long-Term Potentiation in the Anterior Cingulate Cortex.

Phosphorylation of AMPA receptor GluA1 plays important roles in synaptic potentiation. Most previous studies have been performed in the hippocampus, while the roles of GluA1 phosphorylation in the cortex remain unknown. Here we investigated the involvement of the phosphorylation of GluA1 in the LTP in the anterior cingulate cortex (ACC) using mice with a GluA1 knock-in mutation at the PKA phosphorylation site serine 845 (s845A) or CaMKII/PKC phosphorylation site serine 831 (s831A).

MEKK4 Signaling Regulates Sensory Cell Development and Function in the Mouse Inner Ear.

Unlabelled: Mechanosensory hair cells (HCs) residing in the inner ear are critical for hearing and balance. Precise coordination of proliferation, sensory specification, and differentiation during development is essential to ensure the correct patterning of HCs in the cochlear and vestibular epithelium. Recent studies have revealed that FGF20 signaling is vital for proper HC differentiation.

Tumor necrosis factor-alpha-mutant mice exhibit high frequency hearing loss.

Exogenous tumor necrosis factor-alpha (TNF-α) plays a role in auditory hair cell death by altering the expression of apoptosis-related genes in response to noxious stimuli. Little is known, however, about the function of TNF-α in normal hair cell physiology. We, therefore, investigated the cochlear morphology and auditory function of TNF-α-deficient mice.

Mitochondrial peroxiredoxin 3 regulates sensory cell survival in the cochlea.

This study delineates the role of peroxiredoxin 3 (Prx3) in hair cell death induced by several etiologies of acquired hearing loss (noise trauma, aminoglycoside treatment, age). In vivo, Prx3 transiently increased in mouse cochlear hair cells after traumatic noise exposure, kanamycin treatment, or with progressing age before any cell loss occurred; when Prx3 declined, hair cell loss began. Maintenance of high Prx3 levels via treatment with the radical scavenger 2,3-dihydroxybenzoate prevented kanamycin-induced hair cell death.

Circulating MicroRNAs as potential risk biomarkers for hematoma enlargement after intracerebral hemorrhage.

Background And Purpose: MicroRNAs have recently been shown to regulate the downstream bioprocesses of intracerebral hemorrhage. The aim of this study was to investigate whether miRNAs can be used as biomarkers to predict secondary hematoma enlargement (HE) in patients with ICH.

Methods: Consecutively, 79 ICH patients admitted within 6 h of symptom onset and 30 healthy individuals were enrolled in this study.

Intra-tympanic delivery of short interfering RNA into the adult mouse cochlea.

Trans-tympanic injection into the middle ear has long been the standard for local delivery of compounds in experimental studies. Here we demonstrate the advantages of the novel method of intra-tympanic injection through the otic bone for the delivery of compounds or siRNA into the adult mouse cochlea. First, a fluorescently-conjugated scrambled siRNA probe was applied via intra-tympanic injection into the middle ear cavity and was detected in sensory hair cells and nerve fibers as early as 6 h after the injection.

Traumatic noise activates Rho-family GTPases through transient cellular energy depletion.

Small GTPases mediate transmembrane signaling and regulate the actin cytoskeleton in eukaryotic cells. Here, we characterize the auditory pathology of adult male CBA/J mice exposed to traumatic noise (2-20 kHz; 106 dB; 2 h). Loss of outer hair cells was evident 1 h after noise exposure in the basal region of the cochlea and spread apically with time, leading to permanent threshold shifts of 35, 60, and 65 dB at 8, 16, and 32 kHz.

[A pilot study on the diagnosis of laryngopharyngeal reflux diseases].

Objective: To study the value of the reflux symptom index (RSI) and reflux finding score (RFS) in laryngopharyngeal reflux disease (LPRD).

Methods: Twenty-five patients with RSI scores > 13 or RFS scores > 7 and sixteen patients with RSI scores > 13 and RFS scores > 7 were suspected as LPRD. Forty one patients with the RSI scores > 13 and/or RFS scores > 7 were suspected as LPRD, 15 patients with the RSI scores View Article and Find Full Text PDF

[Cochlear reimplantation].

Objective: To analyze retrospectively the outcome of 6 cases of cochlear reimplantation to summarize its experience.

Methods: The operation method of cochlear reimplantation was similar to routine cochlear implantation. A "H" incision for the muscle-periosteum was used to shorten the operation time.