Incomplete Maturation of Brainstem Auditory Nuclei in Genetically Induced Early Postnatal Cochlear Degeneration

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Abstract
In the Shaker-2 mouse mutant, a cochleo-saccular type of genetically induced inner ear degeneration occurs. Morphological signs of degeneration are evident in the 3rd postnatal week and a severe and almost total degeneration has occurred by the age of 6-9 weeks. There are no qualitative differences between the brain of the normal CBA/J mouse and that of the Shaker-2 mouse. The growth of the auditory brainstem nuclei (dorsal cochlear nucleus and ventral cochlear nucleus) in the mutant Sh-2 mouse has stopped by 14 days of age-but not in the normal CBA/J mouse. The brainstem as a whole continues to grow between 14 and 140 days of age in both strains. The early cochlear degeneration coincides with the critical period when a normal input from the peripheral receptor organ is necessary for the maturation of the central auditory pathway.