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Systematic Hearing Performance Evaluation Process for Adolescents with Cochlear Implantation at Early Ages
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Understanding and treating paediatric hearing impairment

Christian Wrobel1, Maria-Patapia Zafeiriou2, Tobias Moser3

  • 1Department of Otolaryngology and InnerEarLab, University Medical Center Göttingen, 37099 Göttingen, Germany; Multiscale Bioimaging Cluster of Excellence (MBExC), University of Göttingen, Germany.

Ebiomedicine
|January 10, 2021
PubMed

Insights

Sensorineural hearing impairment affects many newborns and adolescents. Innovative treatments like gene therapy and stem cell-derived hair cell regeneration show promise for improved hearing restoration.

Area of Science:

  • Otolaryngology
  • Genetics
  • Regenerative Medicine

Background:

  • Sensorineural hearing impairment is common in newborns and adolescents, with over 50% of congenital cases having a genetic origin.
  • Monogenic deafness presents potential targets for future gene therapy interventions.
  • Current clinical management includes hearing aids, cochlear implants, and speech therapy, but significant unmet needs for hearing restoration persist.

Purpose of the Study:

  • To review the pathophysiology of pediatric hearing impairment.
  • To provide an update on preclinical developments in innovative hearing restoration approaches.

Main Methods:

  • Review of current literature on sensorineural hearing impairment.
  • Analysis of advancements in genetic diagnostics, disease modeling, and regenerative medicine.

Main Results:

  • Progress in clinical phenotyping, genetic diagnostics, and counseling for hearing impairment.
  • Disease modeling in transgenic mice has elucidated genetic hearing impairment mechanisms.
  • Preclinical studies indicate upcoming gene therapy trials and next-generation cochlear implants.

Conclusions:

  • Gene therapy and advanced cochlear implants are anticipated within the next decade.
  • Stem cell-derived sensory hair cells and neurons are advancing disease modeling, drug screening, and regenerative strategies.
  • Despite progress, a major clinical need remains for enhanced hearing restoration methods.

Keywords:
CochleaCochlear implantGene therapyHearing aidOptogeneticsStem cell

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