Pathology of the cochlea following a spontaneous mutation in DBA/2 mice

The DBA/2 strain of mice usually presents with noise-induced epileptic seizures and hearing disorders. After a spontaneous mutation a strain with early hearing loss and circling behaviour was produced. This strain presents with clinical symptoms found in diseases connected to inner ear disorders. These animals do not suffer from periodical disorders, however, but have functional disturbances continuously and can therefore serve as an animal model for diseases originating from both parts of the inner ear. The genetic inheritance appears to be autosomal recessive. Offspring showed circling behaviour and severe pathology in the vestibular part of the inner ear. In the present study pathology of the cochlear part of the inner ear was visualized using conventional microscopical techniques. The content of actin and fodrin was labelled immunohistochemically, and hearing was assessed with auditory brainstem recordings. After 1 month the animals showed deterioration of the cochlear part of the inner ear. At 6 months no organ of Corti remained and the animals were deaf. Transmission and scanning electron microscopy revealed severe apical hair cell changes. The content of alpha-actinin and fodrin in the DBA/2 mouse was already fainter than that in age-matched CBA control mice at the age of 1 month. Labelling of antibodies against fodrin increased in the supporting cells of the older animals, probably owing to the replacement of hair cells.