Severe hearing loss in Dlxl mutant mice

Hear Res. 2006 Apr;214(1-2):84-8. doi: 10.1016/j.heares.2006.02.008.


The Dlx homeobox gene family participates in regulating middle and inner ear development. A significant role for Dlxl, in particular,has been demonstrated in the development of the middle ear ossicles, but the functional consequences of Dlx.l gene mutation on hearing thresholds has not been assessed. The present study characterizes auditory brainstem responses to click and tonal stimuli in a non-lethal variant of a Dlxl gene knockout. We found that peripheral hearing thresholds for click and tonal stimuli were significantly elevated in homozygous Dlxl knockout (Dlxl-/ ) compared to both heterozygous (Dlxl+/ ) and wild type (Dlxl+/+) mice. Thus, abnormal mor-phogenesis of the incus and stapes that has been documented previously with histological measures is now known to result in a severe peripheral hearing deficit.

Publication types

  • Research Support, N.I.H., Extramural
  • Research Support, Non-U.S. Gov't

MeSH terms

  • Animals
  • Ear Ossicles / pathology*
  • Ear Ossicles / physiopathology
  • Evoked Potentials, Auditory, Brain Stem / physiology
  • Hearing Loss, Sensorineural / genetics*
  • Hearing Loss, Sensorineural / pathology*
  • Hearing Loss, Sensorineural / physiopathology
  • Homeodomain Proteins / genetics*
  • Mice
  • Mutation*
  • Severity of Illness Index
  • Transcription Factors


  • Distal-less homeobox proteins
  • Homeodomain Proteins
  • Transcription Factors