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Related Concept Videos

Hair Cells01:22

Hair Cells

46.0K
Hair cells are the sensory receptors of the auditory system—they transduce mechanical sound waves into electrical energy that the nervous system can understand. Hair cells are located in the organ of Corti within the cochlea of the inner ear, between the basilar and tectorial membranes. The actual sensory receptors are called inner hair cells. The outer hair cells serve other functions, such as sound amplification in the cochlea, and are not discussed in detail here.
46.0K
Hearing01:31

Hearing

58.1K
When we hear a sound, our nervous system is detecting sound waves—pressure waves of mechanical energy traveling through a medium. The frequency of the wave is perceived as pitch, while the amplitude is perceived as loudness.
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Related Experiment Video

Updated: Mar 8, 2026

Surgical Method for Virally Mediated Gene Delivery to the Mouse Inner Ear through the Round Window Membrane
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Rescue of Hearing by Gene Delivery to Inner-Ear Hair Cells Using Exosome-Associated AAV.

Bence György1, Cyrille Sage2, Artur A Indzhykulian2

  • 1Department of Neurobiology and Howard Hughes Medical Institute, Harvard Medical School, 220 Longwood Avenue, Boston, MA 02115, USA; Department of Neurology, Massachusetts General Hospital and NeuroDiscovery Center, Harvard Medical School, Building 149, Charlestown, Boston, MA 02129, USA.

Molecular Therapy : the Journal of the American Society of Gene Therapy
|January 14, 2017
PubMed
Summary
This summary is machine-generated.

Exosome-associated adeno-associated virus (exo-AAV) effectively delivers genes to all inner ear hair cells, unlike conventional AAV. This novel vector shows promise for treating hearing loss and advancing hair cell research.

Keywords:
LHFPL5TMHSadeno-associated virus vectorbalancecochleaexosomesgene therapyhair cellhearinginner ear

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Area of Science:

  • Otorhinolaryngology
  • Gene Therapy
  • Molecular Biology

Background:

  • Adeno-associated virus (AAV) is a proven vector for retinal gene therapy.
  • Gene therapy for hearing loss is limited by inefficient delivery to inner ear sensory hair cells.
  • Conventional AAV vectors fail to transduce outer hair cells in the mouse cochlea.

Purpose of the Study:

  • To develop a more efficient gene delivery vector for inner ear hair cells.
  • To evaluate the efficacy and safety of exosome-associated AAV (exo-AAV) for hair cell transduction.
  • To assess the therapeutic potential of exo-AAV in a mouse model of hereditary deafness.

Main Methods:

  • Development and characterization of exosome-associated AAV (exo-AAV) vectors.
  • In vitro transduction assays using mouse cochlear explants.
  • In vivo direct cochlear injection in mice.
  • Auditory and vestibular function tests to assess in vivo toxicity.
  • Evaluation of hearing rescue in Lhfpl5/Tmhs-/- mice.

Main Results:

  • Exo-AAV demonstrated superior transduction efficiency to all inner ear hair cells compared to conventional AAV1-GFP.
  • Exo-AAV showed no observable toxicity in vivo, with normal auditory and vestibular function.
  • Gene therapy using exo-AAV1 partially restored hearing in a mouse model of hereditary deafness.

Conclusions:

  • Exosome-associated AAV (exo-AAV) is a potent and safe gene delivery system for inner ear hair cells.
  • Exo-AAV represents a significant advancement for hair cell research and potential gene therapy for deafness.
  • This novel vector overcomes limitations of conventional AAV for inner ear gene delivery.