A5009389388- Hearing, Cochlea, Tinnitus, Genetics
- Neural dynamics and brain function
- Hearing Loss and Rehabilitation
- Neuroscience and Neuropharmacology Research
- Biochemical Analysis and Sensing Techniques
- Muscle Physiology and Disorders
- Animal Vocal Communication and Behavior
- Neuroscience and Neural Engineering
- Linguistic Studies and Language Acquisition
- RNA and protein synthesis mechanisms
- RNA regulation and disease
- Italian Fascism and Post-war Society
- Cell Adhesion Molecules Research
- Vestibular and auditory disorders
- Marine animal studies overview
- Ear Surgery and Otitis Media
- Connexins and lens biology
- Neuroscience and Music Perception
- Acoustic Wave Phenomena Research
Institut Pasteur
2014-2025
Inserm
2014-2025
Université Paris Cité
2023-2025
Centre National de la Recherche Scientifique
2025
Sorbonne Université
2014-2017
Université Sorbonne Nouvelle
2017
Université Grenoble Alpes
2016
Laboratoire de Linguistique et Didactique des Langues Etrangères et Maternelles
2016
Significance Hearing and balance impairments are major concerns a serious burden for public health, but still lack an effective curative therapy. We assessed inner ear functions in mouse model of Usher syndrome type 1, developmental disorder characterized by profound congenital deafness deficit, after local gene Viral transfer the wild-type cDNA to mutant mice shortly birth resulted partial restoration hearing long-lasting, almost complete, removal defect. The present results provide basis...
Defects of CIB2, calcium- and integrin-binding protein 2, have been reported to cause isolated deafness, DFNB48 Usher syndrome type-IJ, characterized by congenital profound balance defects blindness. We report here two new nonsense mutations (pGln12* pTyr110*) in CIB2 patients displaying nonsyndromic hearing loss, with no evidence vestibular or retinal dysfunction. Also, the generated CIB2-/- mice display an early onset deafness normal functions. In these mice, mechanoelectrical transduction...
Abstract Protocadherin‐15 (Pcdh15) is a component of the tip‐links, extracellular filaments that gate hair cell mechano‐electrical transduction channels in inner ear. There are three Pcdh15 splice isoforms (CD1, CD2 and CD3), which only differ by their cytoplasmic domains; they thought to function redundantly during hair‐bundle development, but whether any these composes tip‐link mature cells remains unknown. By immunolabelling both morphological electrophysiological analyses post‐natal...
The precise architecture of hair bundles, the arrays mechanosensitive microvilli-like stereocilia crowning auditory cells, is essential to hearing. Myosin IIIa, defective in late-onset deafness form DFNB30, has been proposed transport espin-1 tips stereocilia, thereby promoting their elongation. We show that Myo3a−/−Myo3b−/− mice lacking myosin IIIa and IIIb are profoundly deaf, whereas Myo3a-cKO Myo3b−/− losing postnatally have normal cochlear bundles display robust mechanoelectrical...
Significance Patients lacking either otogelin or otogelin-like have congenital mild-to-moderate hearing impairment similar to that in patients stereocilin. Stereocilin is associated with 2 types of links specific the mechanosensitive antenna (stereocilia bundle) cochlear outer hair cells: The horizontal top connectors joining stereocilia one another and attachment crowns coupling tallest tectorial membrane, an acellular structure overlying sensory epithelium cochlea. By studying mutant mice...
Significance Presbycusis, or age-related hearing loss, is a major public health issue and the principal potentially modifiable risk factor for dementia. It caused by environmental factors largely uncharacterized genetic factors. We compared DNA sequences across genomic coding regions between familial sporadic cases of severe presbycusis controls with normal hearing. The frequency ultrarare predicted pathogenic variants in genes known to cause dominant early-onset forms deafness was...
Abstract Auditory neuropathies affect the spiral ganglion neurons of auditory nerve or their synapses with sensory hair cells, distorting sound information transmitted from ear to brain. Deciphering underlying pathophysiological mechanisms remains challenging owing diversity neuron subtypes and associated central circuits. An neuropathy mechanism is unraveled by investigating origin hyperexcitability in a mouse model for hereditary congenital deafness. Otogl encodes large Otogelin‐like...
Many genetic forms of congenital deafness affect the sound reception antenna cochlear sensory cells, hair bundle. The resulting deprivation jeopardizes auditory cortex (AC) maturation. Early prosthetic intervention should revive this process. Nevertheless, view assumes that no intrinsic AC deficits coexist with ones, a possibility as yet unexplored. We show here many GABAergic interneurons, from their generation in medial ganglionic eminence up to settlement AC, express two cadherin-related...
According to a novel hypothesis (Arnal et al. (2015), Current Biology 25:2051–2056), auditory roughness, or temporal envelope modulations between 30 and 150 Hz, are present in both natural artificial human alarm signals, which boosts the detection of these alarms various tasks. These results also shed new light on unpleasantness dissonant sounds humans, builds upon high level roughness such sounds. However, it is not clear whether this applies other species, as rodents. In particular,...
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Mice are increasingly used as models of human-acquired neurological or neurodevelopmental conditions, such autism, schizophrenia, and Alzheimer's disease. All these conditions involve central auditory processing disorders, which have been little investigated despite their potential for providing interesting insights into the mechanisms behind disorders. Alterations steady-state response to 40 Hz click trains associated with an imbalance between neuronal excitation inhibition, a mechanism...