Sound strategies for hearing restoration

GSG Géléoc, JR Holt - Science, 2014 - science.org
Background Sensorineural hearing loss is the most common sensory deficit in the world,
with nearly 300 million affected individuals. The problem is multifactorial and can arise from …

Emerging gene therapies for genetic hearing loss

H Ahmed, O Shubina-Oleinik, JR Holt - … of the Association for Research in …, 2017 - Springer
Gene therapy, or the treatment of human disease using genetic material, for inner ear
dysfunction is coming of age. Recent progress in developing gene therapy treatments for …

A synthetic AAV vector enables safe and efficient gene transfer to the mammalian inner ear

LD Landegger, B Pan, C Askew, SJ Wassmer… - Nature …, 2017 - nature.com
Efforts to develop gene therapies for hearing loss have been hampered by the lack of safe,
efficient, and clinically relevant delivery modalities,. Here we demonstrate the safety and …

Tmc gene therapy restores auditory function in deaf mice

C Askew, C Rochat, B Pan, Y Asai, H Ahmed… - Science translational …, 2015 - science.org
Genetic hearing loss accounts for up to 50% of prelingual deafness worldwide, yet there are
no biologic treatments currently available. To investigate gene therapy as a potential …

[HTML][HTML] Meniere's disease might be an autoimmune condition?

A Greco, A Gallo, M Fusconi, C Marinelli, GF Macri… - Autoimmunity …, 2012 - Elsevier
OBJECTIVES: To review our current knowledge of the pathogenesis of Meniere's disease,
including viral infection and immune system-mediated mechanisms, and to discuss the …

Cell death and proliferation in acute slices and organotypic cultures of mammalian CNS

L Lossi, S Alasia, C Salio, A Merighi - Progress in neurobiology, 2009 - Elsevier
Analysis of the interplay between cell proliferation and death has been greatly advantaged
by the development of CNS slice preparations. In slices, interactions between neurons and …

Adeno-associated virus-mediated gene delivery into the scala media of the normal and deafened adult mouse ear

LA Kilpatrick, Q Li, J Yang, JC Goddard, DM Fekete… - Gene therapy, 2011 - nature.com
Murine models are ideal for studying cochlear gene transfer, as many hearing loss-related
mutations have been discovered and mapped within the mouse genome. However, because …

[HTML][HTML] Recent advancements in the regeneration of auditory hair cells and hearing restoration

R Mittal, D Nguyen, AP Patel, LH Debs… - Frontiers in molecular …, 2017 - frontiersin.org
Neurosensory responses of hearing and balance are mediated by receptors in specialized
neuroepithelial sensory cells. Any disruption of the biochemical and molecular pathways …

[HTML][HTML] Advances and challenges in adeno-associated viral inner-ear gene therapy for sensorineural hearing loss

K Bankoti, C Generotti, T Hwa, L Wang… - … Therapy-Methods & …, 2021 - cell.com
There is growing attention and effort focused on treating the root cause of sensorineural
hearing loss rather than managing associated secondary characteristic features. With recent …

[HTML][HTML] Regenerating hair cells in vestibular sensory epithelia from humans

RR Taylor, A Filia, U Paredes, Y Asai, JR Holt, M Lovett… - Elife, 2018 - elifesciences.org
Human vestibular sensory epithelia in explant culture were incubated in gentamicin to
ablate hair cells. Subsequent transduction of supporting cells with ATOH1 using an Ad-2 …