A major gene affecting age-related hearing loss in C57BL/6J mice

Hear Res. 1997 Dec;114(1-2):83-92. doi: 10.1016/s0378-5955(97)00155-x.


A major gene responsible for age-related hearing loss (AHL) in C57BL/6J mice was mapped by analyses of a (C57BL/6J x CAST/Ei) x C57BL/6J backcross. AHL, as measured by elevated auditory-evoked brainstem response (ABR) thresholds, segregated among backcross mice as expected for a recessive, primarily single-gene trait. Both qualitative and quantitative linkage analyses gave the same genetic map position for the AHL gene (Ahl on chromosome 10, near D10Mit5. Marker assisted selection was then used to produce congenic lines of C57BL/6J that contain different CAST-derived segments of chromosome 10. ABR test results and cochlear histopathology of aged progenitors of these congenic lines are presented. Ahl is the first gene causing late-onset, non-syndromic hearing loss that has been reported in the mouse.

Publication types

  • Research Support, U.S. Gov't, P.H.S.

MeSH terms

  • Aging / pathology*
  • Animals
  • Auditory Threshold / physiology
  • Chromosome Mapping*
  • Chromosomes, Human, Pair 10
  • Cochlea / pathology*
  • Evoked Potentials, Auditory, Brain Stem / genetics*
  • Genes, Recessive / genetics
  • Genotype
  • Humans
  • Mice
  • Mice, Inbred C57BL
  • Presbycusis / genetics*
  • Presbycusis / pathology
  • Spiral Ganglion / pathology