Pathogenesis of hereditary inner ear abnormalities in animals

There are many different genetic syndromes in animals which result in deafness. Two major types of inherited deafness are known. The most common in animals, including man, is that in which there is early cell death of the neural epithelium. This is found in the cat, dog, mink, guinea pig, and mouse. The second type of inner ear deafness is associated with malformations of both the bony form of the inner ear and the neural epithelium. This type of deafness is that which is found in the Kreisler mouse. It is also found in man, but appears to be less common than the early cell death in which there is no malformation of the bony labyrinth. This form of inherited deafness has been associated and may be secondary to genetic abnormalities of the developing CNS.