title: A guinea pig strain with recessive heredity of deafness, producing normal-hearing heterozygotes with resistance to noise trauma.
abstract: A new strain of waltzing guinea pigs arose spontaneously in a guinea pig breeding facility in Germany in 1996. In addition to obvious vestibular dysfunction, the waltzing animals appear deaf already at birth. Histological analysis revealed that the waltzers lack an open scala media due to the collapse of Reissner's membrane onto the surface of the hearing organ. Subsequent breeding has shown that this strain has a recessive mode of inheritance. The homozygotes are deaf and display a waltzing behaviour throughout their lives while the heterozygotes show no significant signs of inner ear injury despite being carriers of this specific mutated gene of hearing impairment. However, the heterozygous animals offer the opportunity to study how hereditary factors interact with auditory stress. In the present study, the susceptibility of the carriers to noise was investigated. Auditory brainstem responses were obtained prior to and after noise exposure (4 kHz, 110 dB, 6 h). The carriers were significantly less affected by the noise as compared to control animals. This difference was still significant at 4 weeks following noise exposure. It is suggested that the heterozygous animals have an endogenous resistance to auditory stress.
authors.affiliation: Center for Hearing and Communication Research and Department of Clinical Neuroscience, Karolinska Institutet, Stockholm, Sweden.
authors.forename: Asa
authors.initials: A
authors.lastname: Skjönsberg
authors.forename: Petra
authors.initials: P
authors.lastname: Herrlin
authors.forename: Maoli
authors.initials: M
authors.lastname: Duan
authors.forename: Ann-Christin
authors.initials: AC
authors.lastname: Johnson
authors.forename: Mats
authors.initials: M
authors.lastname: Ulfendahl
mesh_terms: D000818:Animals; D001309:Auditory Threshold; D001522:Behavior, Animal / Q000502:physiology; D003051:Cochlea / Q000473:pathology; D004195:Disease Models, Animal; D016057:Evoked Potentials, Auditory, Brain Stem / Q000502:physiology; D005260:Female; D006168:Guinea Pigs / Q000235:genetics; D034381:Hearing Loss / Q000235:genetics*; D006317:Hearing Loss, Noise-Induced / Q000235:genetics / Q000503:physiopathology / Q000517:prevention & control*; D006579:Heterozygote; D006720:Homozygote; D009622:Noise / Q000009:adverse effects*; D011247:Pregnancy