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Related Experiment Video

Updated: Dec 13, 2025

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Inner Ear Gene Therapies Take Off: Current Promises and Future Challenges.

Sedigheh Delmaghani1, Aziz El-Amraoui1

  • 1Progressive Sensory Disorders, Pathophysiology and Therapy Unit, Institut Pasteur, Institut de l'Audition, INSERM-UMRS1120, Sorbonne Université, 63 rue de Charenton, 75012 Paris, France.

Journal of Clinical Medicine
|July 26, 2020
PubMed
Summary

Inherited hearing loss affects millions, with current treatments only managing symptoms. New gene therapies show promise for restoring auditory and vestibular functions, offering hope for future cures.

Keywords:
AAVCRISPR/Cas9RNAiantisense oligonucleotidecochleagene therapygenome editinglipid nanoparticle-mediated deliverysensorineural hearing losssensory disorders

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Area of Science:

  • Otolaryngology
  • Genetics
  • Regenerative Medicine

Background:

  • Hearing impairment is a prevalent sensory deficit across all age groups, with over 50% of congenital deafness being hereditary.
  • Major causes include genetic predisposition, aging, acoustic trauma, ototoxic drugs, and noise exposure.
  • Current management relies on hearing aids and cochlear implants, which do not restore native inner ear function.

Purpose of the Study:

  • To review advancements in understanding inherited deafness and its underlying mechanisms.
  • To highlight the development of novel therapeutic strategies for sensorineural hearing loss.
  • To discuss the successful restoration of auditory and vestibular functions in preclinical models.

Main Methods:

  • Review of studies on inherited deafness and animal models.
  • Analysis of molecular, cellular, and physiological mechanisms of hearing loss.
  • Evaluation of gene transfer and gene editing approaches for inner ear repair.

Main Results:

  • Deciphering molecular mechanisms of inherited deafness through genetic studies.
  • Development of gene-based therapies showing promise in animal models.
  • Successful restoration of auditory and vestibular functions in specific inner ear conditions.

Conclusions:

  • Gene therapy and gene editing offer potential curative treatments for sensorineural deafness.
  • Preclinical successes pave the way for future clinical applications in restoring hearing.
  • Further research is crucial for translating these findings into effective human therapies.