Congenital hearing loss

AMH Korver, RJH Smith, G Van Camp… - Nature reviews Disease …, 2017 - nature.com
Congenital hearing loss (hearing loss that is present at birth) is one of the most prevalent
chronic conditions in children. In the majority of developed countries, neonatal hearing …

[HTML][HTML] Molecular motors in neurons: transport mechanisms and roles in brain function, development, and disease

N Hirokawa, S Niwa, Y Tanaka - Neuron, 2010 - cell.com
The kinesin, dynein, and myosin superfamily molecular motors have fundamental roles in
neuronal function, plasticity, morphogenesis, and survival by transporting cargos such as …

[HTML][HTML] Usher syndrome: Hearing loss, retinal degeneration and associated abnormalities

P Mathur, J Yang - Biochimica et Biophysica Acta (BBA)-Molecular Basis …, 2015 - Elsevier
Usher syndrome (USH), clinically and genetically heterogeneous, is the leading genetic
cause of combined hearing and vision loss. USH is classified into three types, based on the …

[HTML][HTML] Molecular and cytological profiling of biological aging of mouse cochlear inner and outer hair cells

H Liu, KP Giffen, L Chen, HJ Henderson, TA Cao… - Cell reports, 2022 - cell.com
Age-related hearing loss (ARHL) negatively impacts quality of life in the elderly population.
The prevalent cause of ARHL is loss of mechanosensitive cochlear hair cells (HCs). The …

The physiology of mechanoelectrical transduction channels in hearing

R Fettiplace, KX Kim - Physiological reviews, 2014 - journals.physiology.org
Much is known about the mechanotransducer (MT) channels mediating transduction in hair
cells of the vertrbrate inner ear. With the use of isolated preparations, it is experimentally …

Hair cell transduction, tuning, and synaptic transmission in the mammalian cochlea

R Fettiplace - Comprehensive Physiology, 2011 - Wiley Online Library
Sound pressure fluctuations striking the ear are conveyed to the cochlea, where they vibrate
the basilar membrane on which sit hair cells, the mechanoreceptors of the inner ear …

Mechanotransduction by hair cells: models, molecules, and mechanisms

PG Gillespie, U Müller - Cell, 2009 - cell.com
Mechanotransduction, the transformation of mechanical force into an electrical signal, allows
living organisms to hear, register movement and gravity, detect touch, and sense changes in …

Actin bundles dynamics and architecture

S Rajan, DS Kudryashov, E Reisler - Biomolecules, 2023 - mdpi.com
Cells use the actin cytoskeleton for many of their functions, including their division,
adhesion, mechanosensing, endo-and phagocytosis, migration, and invasion. Actin bundles …

Inner ear gene therapies take off: current promises and future challenges

S Delmaghani, A El-Amraoui - Journal of clinical medicine, 2020 - mdpi.com
Hearing impairment is the most frequent sensory deficit in humans of all age groups, from
children (1/500) to the elderly (more than 50% of the over-75 s). Over 50% of congenital …

The sensory and motor roles of auditory hair cells

R Fettiplace, CM Hackney - Nature reviews neuroscience, 2006 - nature.com
Cochlear hair cells respond with phenomenal speed and sensitivity to sound vibrations that
cause submicron deflections of their hair bundle. Outer hair cells are not only detectors, but …