Dysgenesis of melanocytes and cochlear dysfunction in mutant microphthalmia (mi) mice

Hear Res. 1994 Oct;80(1):10-20. doi: 10.1016/0378-5955(94)90003-5.


In order to evaluate the cytological homology of intermediate cells and melanocytes, and to investigate the function of melanocytes in the inner ear, hearing acuity and cochlear pathology were studied in three strains of mice, namely, wild type mice (+/+), albino mice without melanin (c2J/c2J), and microphthalmia mice with no melanocytes (mibw/mibw). Our histochemical data indicated that intermediate cells showed cytological characteristics almost identical to those of melanocytes and that disorders of melanin and/or melanocytes were reflected in the stria vascularis of each mouse. While c2J/c2J presented the same normal hearing acuity and normal structure of the stria vascularis as +/+, the hearing acuity of mibw/mibw mutants was severely impaired. Their stria vascularis was abnormally thin, lacking intermediate cells. According to these results, lack of melanin has little influence on hearing acuity; however, the absence of intermediate cells or melanocytes causes severe hearing loss, presumably due to a strial dysfunction.

Publication types

  • Comparative Study

MeSH terms

  • Animals
  • Auditory Threshold / physiology
  • Calcium-Transporting ATPases / metabolism
  • Cochlea / pathology*
  • Cochlea / physiopathology
  • Cochlea / ultrastructure
  • Dihydroxyphenylalanine / toxicity
  • Evoked Potentials, Auditory, Brain Stem / physiology
  • Hearing Loss, Sensorineural / etiology
  • Immunohistochemistry
  • Melanins / deficiency
  • Melanocytes / cytology*
  • Melanocytes / pathology
  • Melanocytes / ultrastructure
  • Mice
  • Mice, Inbred C57BL
  • Microphthalmos / genetics
  • Microphthalmos / pathology*
  • Microphthalmos / physiopathology
  • Microscopy, Electron
  • Mutation / genetics
  • Sodium-Potassium-Exchanging ATPase / metabolism
  • Species Specificity
  • Stria Vascularis / drug effects
  • Stria Vascularis / pathology


  • Melanins
  • Dihydroxyphenylalanine
  • Calcium-Transporting ATPases
  • Sodium-Potassium-Exchanging ATPase