Assessment of Hearing in 80 Inbred Strains of Mice by ABR Threshold Analyses

Overview

Journal Hear Res

Specialty Otorhinolaryngology

Date 1999 May 13

PMID 10320101

Citations 436

Authors

Q Y Zheng

K R Johnson

L C Erway

Affiliations

Soon will be listed here.

Abstract

The common occurrence of hearing loss in both humans and mice, and the anatomical and functional similarities of their inner ears, attest to the potential of mice being used as models to study inherited hearing loss. A large-scale, auditory screening project is being undertaken at The Jackson Laboratory (TJL) to identify mice with inherited hearing disorders. To assess hearing sensitivity, at least five mice from each inbred strain had auditory brainstem response (ABR) thresholds determined. Thus far, we have screened 80 inbred strains of mice; 60 of them exhibited homogeneous ABR threshold values not significantly different from those of the control strain CBA/CaJ. This large database establishes a reliable reference for normal hearing mouse strains. The following 16 inbred strains exhibited significantly elevated ABR thresholds before the age of 3 months: 129/J, 129/ReJ, 129/SvJ, A/J, ALR/LtJ, ALS/LtJ, BUB/BnJ, C57BLKS/J, C57BR/cdJ, C57L/J, DBA/2J, I/LnJ, MA/MyJ, NOD/LtJ, NOR/LtJ, and SKH2/J. These hearing impaired strains may serve as models for some forms of human non-syndromic hearing loss and aid in the identification of the underlying genes.

Citing Articles

Limiting Hearing Loss in Transgenic Mouse Models.

Babola T, Donovan N, Darcy S, Spjut C, Kanold P eNeuro. 2025; 12(2).

PMID: 39900508 PMC: 11875052. DOI: 10.1523/ENEURO.0465-24.2025.

The Use of Event-Related Potentials in the Study of Schizophrenia: An Overview.

Crown L, Featherstone R, Sobell J, Parekh K, Siegel S Adv Neurobiol. 2024; 40:285-319.

PMID: 39562449 DOI: 10.1007/978-3-031-69491-2_11.

Resistance to age-related hearing loss in the echolocating big brown bat ().

Capshaw G, Diebold C, Adams D, Rayner J, Wilkinson G, Moss C Proc Biol Sci. 2024; 291(2034):20241560.

PMID: 39500378 PMC: 11708781. DOI: 10.1098/rspb.2024.1560.

Morphological phenotyping of the aging cochlea in inbred C57BL/6N and outbred CD1 mouse strains.

Attanasio C, Palladino A, Giaquinto D, Scavizzi F, Raspa M, Peres C Aging Cell. 2024; 24(1):e14362.

PMID: 39482905 PMC: 11709085. DOI: 10.1111/acel.14362.

Effects of aging on otolith morphology and functions in mice.

Ueda K, Imai T, Ito T, Okayasu T, Harada S, Kamakura T Front Neurosci. 2024; 18:1466514.

PMID: 39479359 PMC: 11521974. DOI: 10.3389/fnins.2024.1466514.

References

Steel K . Inherited hearing defects in mice. Annu Rev Genet. 1995; 29:675-701. DOI: 10.1146/annurev.ge.29.120195.003331. View

Simpson E, Linder C, Sargent E, Davisson M, Mobraaten L, Sharp J . Genetic variation among 129 substrains and its importance for targeted mutagenesis in mice. Nat Genet. 1997; 16(1):19-27. DOI: 10.1038/ng0597-19. View

Liu X, Walsh J, Mburu P, Kendrick-Jones J, Cope M, Steel K . Mutations in the myosin VIIA gene cause non-syndromic recessive deafness. Nat Genet. 1997; 16(2):188-90. DOI: 10.1038/ng0697-188. View

Hall 3rd J, Rupp K . Auditory brainstem response: recent developments in recording and analysis. Adv Otorhinolaryngol. 1997; 53:21-45. DOI: 10.1159/000059038. View

Martini A, Milani M, Rosignoli M, Mazzoli M, Prosser S . Audiometric patterns of genetic non-syndromal sensorineural hearing loss. Audiology. 1997; 36(4):228-36. DOI: 10.3109/00206099709071975. View

Noben-Trauth K, Zheng Q, Johnson K, Nishina P . mdfw: a deafness susceptibility locus that interacts with deaf waddler (dfw). Genomics. 1997; 44(3):266-72. DOI: 10.1006/geno.1997.4869. View

Liu X, Walsh J, Tamagawa Y, Kitamura K, Nishizawa M, Steel K . Autosomal dominant non-syndromic deafness caused by a mutation in the myosin VIIA gene. Nat Genet. 1997; 17(3):268-9. DOI: 10.1038/ng1197-268. View

Ohlemiller K, McGee J, Walsh E, Kimberling W . Hearing loss in the RBF/DnJ mouse, a proposed animal model of Usher syndrome type IIa. Hear Res. 1997; 112(1-2):1-12. DOI: 10.1016/s0378-5955(97)00087-7. View

Parham K . Distortion product otoacoustic emissions in the C57BL/6J mouse model of age-related hearing loss. Hear Res. 1997; 112(1-2):216-34. DOI: 10.1016/s0378-5955(97)00124-x. View

10.

Johnson K, Erway L, Cook S, Willott J, Zheng Q . A major gene affecting age-related hearing loss in C57BL/6J mice. Hear Res. 1998; 114(1-2):83-92. DOI: 10.1016/s0378-5955(97)00155-x. View

11.

Petit C . Genes responsible for human hereditary deafness: symphony of a thousand. Nat Genet. 1996; 14(4):385-91. DOI: 10.1038/ng1296-385. View

12.

Sun X, Jung M, Kim D, Randolph K . Distortion product otoacoustic emission test of sensorineural hearing loss in humans: comparison of unequal- and equal-level stimuli. Ann Otol Rhinol Laryngol. 1996; 105(12):982-90. DOI: 10.1177/000348949610501209. View

13.

Kim D, Paparello J, Jung M, Smurzynski J, Sun X . Distortion product otoacoustic emission test of sensorineural hearing loss: performance regarding sensitivity, specificity and receiver operating characteristics. Acta Otolaryngol. 1996; 116(1):3-11. DOI: 10.3109/00016489609137705. View

14.

Steel K, Brown S . Genetics of deafness. Curr Opin Neurobiol. 1996; 6(4):520-5. DOI: 10.1016/s0959-4388(96)80059-6. View

15.

Erway L, Shiau Y, Davis R, Krieg E . Genetics of age-related hearing loss in mice. III. Susceptibility of inbred and F1 hybrid strains to noise-induced hearing loss. Hear Res. 1996; 93(1-2):181-7. DOI: 10.1016/0378-5955(95)00226-x. View

16.

Erkman L, McEvilly R, Luo L, Ryan A, Hooshmand F, OConnell S . Role of transcription factors Brn-3.1 and Brn-3.2 in auditory and visual system development. Nature. 1996; 381(6583):603-6. DOI: 10.1038/381603a0. View

17.

Willott J, Erway L, Archer J, Harrison D . Genetics of age-related hearing loss in mice. II. Strain differences and effects of caloric restriction on cochlear pathology and evoked response thresholds. Hear Res. 1995; 88(1-2):143-55. DOI: 10.1016/0378-5955(95)00107-f. View

18.

Walton J, Frisina R, Meierhans L . Sensorineural hearing loss alters recovery from short-term adaptation in the C57BL/6 mouse. Hear Res. 1995; 88(1-2):19-26. DOI: 10.1016/0378-5955(95)00093-j. View

19.

Keats B, Nouri N, Huang J, Money M, Webster D, Berlin C . The deafness locus (dn) maps to mouse chromosome 19. Mamm Genome. 1995; 6(1):8-10. DOI: 10.1007/BF00350886. View

20.

Gibson F, Walsh J, Mburu P, Varela A, Brown K, Antonio M . A type VII myosin encoded by the mouse deafness gene shaker-1. Nature. 1995; 374(6517):62-4. DOI: 10.1038/374062a0. View