Normal cochlear function in mdx and mdx(Cv3) Duchenne muscular dystrophy mouse models

De Ann M Pillers, Nancy M. Duncan, Shannon J. Dwinnell, Sean M. Rash, J. Beth Kempton, Dennis Trune

Research output: Contribution to journalArticle

6 Citations (Scopus)

Abstract

Objectives/Hypothesis: Sensorineural hearing loss has been found in association with inherited muscular dystrophies in humans and in mouse models. An increased brainstem auditory evoked response threshold has been previously reported in the dystrophin-deficient mdx mouse model for Duchenne muscular dystrophy, suggesting that full-length dystrophin (Dp427) is involved in hearing. The objective of the present study was to confirm cochlear dysfunction with this gene defect and determine whether the shorter carboxyl terminus isoforms of dystrophin are also critical in maintaining normal hearing. Study Design: Case controlled. Animal model. Methods: Auditory brainstem response (ABR) audiometry to pure tones was used to evaluate cochlear function. Fourteen mdx, 4 mdx(Cv3), and 13 age-matched control (C57BL/6J and C57BL/10ScSn) male mice were tested at 5 weeks and 11 weeks of age. The ABR thresholds to tone-burst stimuli at 4, 8, 16, and 32 kHz were obtained for each ear and statistically compared (ANOVA) for potential group differences. Results: Both mdx and mdx(Cv3) mice demonstrated normal ABR thresholds when compared with controls. Conclusions: Both mdx and mdx(Cv3) mouse models have normal hearing by ABR. The authors' data suggest that dystrophin and its carboxyl terminus isoforms do not play a critical role in hearing in the mouse. This was unexpected, as previous studies using the brainstem auditory evoked response method suggested that the mdx mouse has an increased threshold for hearing.

Original languageEnglish (US)
Pages (from-to)1310-1312
Number of pages3
JournalLaryngoscope
Volume109
Issue number8
DOIs
StatePublished - Aug 1999

Fingerprint

Duchenne Muscular Dystrophy
Brain Stem Auditory Evoked Potentials
Cochlea
Inbred mdx Mouse
Dystrophin
Hearing
Protein Isoforms
Pure-Tone Audiometry
Muscular Dystrophies
Sensorineural Hearing Loss
Ear
Analysis of Variance
Animal Models
Genes

Keywords

  • Animal model
  • Cochlea
  • Dystrophin
  • Hearing
  • Muscular dystrophy

ASJC Scopus subject areas

  • Otorhinolaryngology

Cite this

Pillers, D. A. M., Duncan, N. M., Dwinnell, S. J., Rash, S. M., Kempton, J. B., & Trune, D. (1999). Normal cochlear function in mdx and mdx(Cv3) Duchenne muscular dystrophy mouse models. Laryngoscope, 109(8), 1310-1312. https://doi.org/10.1097/00005537-199908000-00023

Normal cochlear function in mdx and mdx(Cv3) Duchenne muscular dystrophy mouse models. / Pillers, De Ann M; Duncan, Nancy M.; Dwinnell, Shannon J.; Rash, Sean M.; Kempton, J. Beth; Trune, Dennis.

In: Laryngoscope, Vol. 109, No. 8, 08.1999, p. 1310-1312.

Research output: Contribution to journalArticle

Pillers, DAM, Duncan, NM, Dwinnell, SJ, Rash, SM, Kempton, JB & Trune, D 1999, 'Normal cochlear function in mdx and mdx(Cv3) Duchenne muscular dystrophy mouse models', Laryngoscope, vol. 109, no. 8, pp. 1310-1312. https://doi.org/10.1097/00005537-199908000-00023
Pillers, De Ann M ; Duncan, Nancy M. ; Dwinnell, Shannon J. ; Rash, Sean M. ; Kempton, J. Beth ; Trune, Dennis. / Normal cochlear function in mdx and mdx(Cv3) Duchenne muscular dystrophy mouse models. In: Laryngoscope. 1999 ; Vol. 109, No. 8. pp. 1310-1312.
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