STRC Gene Hearing Loss: Comprehensive Guide

Context: Research for Egor’s son, diagnosed with moderate bilateral sensorineural hearing loss caused by compound heterozygous STRC gene mutations.

Key Findings

  • STRC-related hearing loss (DFNB16) is 2nd most common hereditary hearing loss
  • Affects ~2.3M people worldwide
  • Typically congenital, bilateral, mild-to-moderate (40-50 dB), generally STABLE over lifetime
  • Hair cells remain ALIVE and structurally intact (just lack stereocilin protein)
  • This creates broad window for future gene therapy (infancy through adulthood)

Treatment Horizon

  • Dual-AAV gene replacement restored hearing in mice
  • OTOF gene therapy restored hearing in dozens of deaf children (2022-2025 trials)
  • Regeneron has preclinical STRC program (AAV.104) in pipeline
  • Hearing aids: standard of care, highly effective for moderate loss

Files

  • English: ~/DeepResearch/2026-02-07-strc-hearing-loss-treatment.md
  • Russian: ~/DeepResearch/2026-02-07-strc-hearing-loss-treatment-RU.md

Connections