Publications associées à Ototoxicité

Variants in USP48 encoding ubiquitin hydrolase are associated with autosomal dominant non-syndromic hereditary hearing loss

Non-Syndromic Hereditary Hearing Loss (NSHHL) is a genetically heterogeneous sensory disorder with about 120 genes already associated. Through exome sequencing (ES) and data aggregation, we identified a family with six affected individuals and one unrelate ...

Oxford University Press2021

Gamma Knife radiosurgery for intravestibular and intracochlear schwannomas

Constantin Tuleasca

BackgroundSchwannomas of the VIIIth cranial nerve are benign tumours, with vast majority occurring in vestibular division. Rarely, they can also arise from distal branches of cochlear, superior or inferior vestibular. We review our experience with Gamma Kn ...

2019

Delivery of CRISPR/Cas9 using AAV-PHP.B in the inner ear leads to allele-specific inactivation of the mutated Tmc1 allele and protects auditory function in Beethoven mice

Bernard Schneider, Paola Andrea Solanes Vega, Sofia Carmen Suzanne Spataro

Hearing Loss (HL) is the most prevalent sensorineural disorder, affecting 360 million people worldwide. As genetic causes lead to 50% of pre-lingual deafness, gene therapy is considered as a potential therapeutic strategy. Mutations in the TMC1 gene are li ...

MARY ANN LIEBERT, INC2019

Dicationic Ruthenium(II)-Arene-Curcumin Complexes Containing Methylated 1,3,5-Triaza-7-phosphaadamantane: Synthesis, Structure, and Cytotoxicity

Paul Joseph Dyson, Rosario Scopelliti, Tina Riedel

Dicationic ruthenium(II)-p-cymene complexes containing curcumin ligands (curcH = curcumin, bdcurcH = bisdemethoxycurcumin) and ionic N-methyl-1,3,5-triaza-7-phosphaadamantane [PTA-Me]X (X = BF4 or I) have been synthesized and fully characterized for the fi ...

Wiley-Blackwell2017

AAV based gene therapy for rare diseases : targeting astrocytes for functional recovery in Amyotrophic Lateral Sclerosis

Cylia Cloé Rochat

Of the seven thousand diseases that are described as rare, 80% of them have an identified genetic cause. With this in the mind, the development of technologies, such as gene therapy, to address the genetic factors involved in these pathologies, might be a ...

EPFL2017

Characterization of pulse amplitude and pulse rate modulation for a human vestibular implant during acute electrical stimulation

Silvestro Micera, Thuy Anh Khoa Nguyen, Maurizio Ranieri

Objective. The vestibular system provides essential information about balance and spatial orientation via the brain to other sensory and motor systems. Bilateral vestibular loss significantly reduces quality of life, but vestibular implants (VIs) have demo ...

Iop Publishing Ltd2016

Novel Chemosensors for Biologically Important Analytes

Ziya Köstereli

In this work, fluorescent-based chemosensors for the detection of important analytes in aqueous solution are described. The sensors are based on different detection concepts, including analyte-induced aggregation/de-aggregation, pattern-based sensing and m ...

EPFL2015

Precise toxigenic ablation of intermediate cells abolishes the "battery" of the cochlear duct

Friedrich Beermann

The extracellular potential of excitable and nonexcitable cells with respect to ground is ∼0 mV. One of the known exceptions in mammals is the cochlear duct, where the potential is ∼80-100 mV, called the endocochlear potential (EP). The EP serves as the "b ...

Society for Neuroscience2013

Two-photon microscopy of the mouse cochlea in situ for cellular diagnosis

Demetri Psaltis, Ye Pu, Ye Pu, Chia-Lung Hsieh, Xin Yang

Sensorineural hearing loss is the most common type of hearing loss worldwide, yet the underlying cause is typically unknown because the inner ear cannot be biopsied today without destroying hearing, and intracochlear cells have not been imaged with resolut ...

SPIE Society of Photo-optical Instrumentation Engineers2013