Hearing loss in the laminin-deficient dy mouse model of congenital muscular dystrophy

De Ann M. Pillers, J. Beth Kempton, Nancy M. Duncan, Jiaqing Pang, Shannon J. Dwinnell, Dennis R. Trune

Research output: Contribution to journalArticle

14 Scopus citations

Abstract

Sensorineural hearing loss is found in many inherited forms of muscular dystrophy. We investigated the dy mouse model, which has congenital muscular dystrophy due to a defect in laminin α2, for evidence of cochlear dysfunction. Auditory brainstem response (ABR) audiometry to pure tones was used to evaluate 3-month-old homozygous dy/dy and age-matched C57 control mice. The average ABR thresholds to tone-burst stimuli for four frequencies (4, 8, 16, and 32 kHz) were determined and statistically compared by ANOVA. The dy/dy mice demonstrated elevated auditory thresholds ranging from 25 to 27 dB at each frequency tested (p < 0.0001). Anatomic evaluations of the ears revealed pathology ranging from extensive connective tissue infiltration within the inner ear to possible minor defects in the cells of the organ of Corti. These anatomic and physiologic observations suggest that the extracellular matrix protein laminin plays a crucial role in normal cochlear function. Furthermore, the dy congenital muscular dystrophy mouse offers a novel model for evaluation of sensorineural hearing loss associated with muscular dystrophy.

Original languageEnglish (US)
Pages (from-to)217-224
Number of pages8
JournalMolecular Genetics and Metabolism
Volume76
Issue number3
DOIs
StatePublished - Sep 27 2002

    Fingerprint

Keywords

  • Animal model
  • Auditory brainstem response
  • Cochlea
  • DFNA10
  • Laminin
  • Mouse
  • Muscular dystrophy
  • Sensorineural hearing loss

ASJC Scopus subject areas

  • Endocrinology, Diabetes and Metabolism
  • Biochemistry
  • Molecular Biology
  • Genetics
  • Endocrinology

Cite this