title: A new mouse model with cochleo-saccular type inner ear defects.
abstract: We found a new inner ear mutant exhibiting abnormal behavior, such as circling and head shaking, in a breeding stock of SJL/J mice. The traits are inherited in a simple autosomal-recessive fashion. Animals homozygous for the responsible gene, designated cosa, show no startle response to sounds and an inability to swim. In the inner ears of cosa/cosa homozygous, but not +/cosa heterozygous adults, histopathological features of severe damage that are typical for 'cochleo-saccular' or 'spotting' mutants have been demonstrated. We suggest here that the abnormal mice carry a mutation of a gene that is developmentally switched on in the early stages of development and is involved in endolymph homeostasis.
authors.affiliation: Laboratory of Animal Management & Resources, Graduate School of Bioagricultural Sciences, Nagoya University, Nagoya 464-8601, Japan.
authors.forename: S
authors.initials: S
authors.lastname: Oda
authors.forename: A
authors.initials: A
authors.lastname: Hanai
authors.forename: S
authors.initials: S
authors.lastname: Masaki
authors.forename: S
authors.initials: S
authors.lastname: Yonezawa
mesh_terms: D000818:Animals; D003051:Cochlea / Q000002:abnormalities*; D003638:Deafness / Q000235:genetics* / Q000662:veterinary*; D004195:Disease Models, Animal; D005260:Female; D008297:Male; D051379:Mice; D017354:Point Mutation; D013216:Reflex, Startle; D012444:Saccule and Utricle / Q000002:abnormalities*; D013550:Swimming