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Identification of 17 hearing impaired mouse strains in the TMGC ENU-mutagenesis screen
- Source :
- Hearing research. 220(1-2)
- Publication Year :
- 2006
-
Abstract
- The Tennessee Mouse Genome Consortium (TMGC) employed an N-ethyl-N-nitrosourea (ENU)-mutagenesis scheme to identify mouse recessive mutants with hearing phenotypes. We employed auditory brainstem responses (ABR) to click and 8, 16, and 32 kHz stimuli and screened 285 pedigrees (1819 mice of 8-11 weeks old in various mixed genetic backgrounds) each bred to carry a homozygous ENU-induced mutation. To define mutant pedigrees, we measured P12 mice per pedigree in P2 generations and used a criterion where the mean ABR threshold per pedigree was two standard deviations above the mean of all offspring from the same parental strain. We thus identified 17 mutant pedigrees (6%), all exhibiting hearing loss at high frequencies (P16 kHz) with an average threshold elevation of 30-35 dB SPL. Interestingly, four mutants showed sex-biased hearing loss and six mutants displayed wide range frequency hearing loss. Temporal bone histology revealed that six of the first nine mutants displayed cochlear morphological defects: degeneration of spiral ganglia, spiral ligament fibrocytes or inner hair cells (but not outer hair cells) mostly in basal turns. In contrast to other ENU-mutagenesis auditory screens, our screen identified high-frequency, mild and sex-biased hearing defects. Further characterization of these 17 mouse models will advance our understanding ofmore » presbycusis and noise-induced hearing loss in humans.« less
- Subjects :
- medicine.medical_specialty
Hearing loss
Mutant
Mutagenesis (molecular biology technique)
Presbycusis
Audiology
Biology
Mice
Sex Factors
otorhinolaryngologic diseases
medicine
Evoked Potentials, Auditory, Brain Stem
Animals
Inner ear
Genetic Testing
Cochlea
Genetics
medicine.disease
Sensory Systems
Pedigree
Mice, Inbred C57BL
Disease Models, Animal
Auditory brainstem response
medicine.anatomical_structure
Phenotype
Hearing Loss, Noise-Induced
Mutagenesis
Ethylnitrosourea
Spiral ligament
sense organs
medicine.symptom
Noise
Mutagens
Subjects
Details
- ISSN :
- 03785955
- Volume :
- 220
- Issue :
- 1-2
- Database :
- OpenAIRE
- Journal :
- Hearing research
- Accession number :
- edsair.doi.dedup.....71ff0fe80e6a18b9282f49314821d9af