Effect of adult-onset deafness on the human central auditory system.

Degenerative change in the central auditory system was assessed in seven subjects with profound bilateral adult-onset deafness. The degree of transneuronal atrophy was determined by measuring cell size at three levels of the brain stem auditory pathway (anteroventral cochlear nucleus, medial superior olivary nucleus, and inferior colliculus). Within subjects, the relative degree of cell shrinkage was similar across all levels of the central pathway. Across subjects, the best neuronal preservation was seen in a case of viral labyrinthitis with 1 year of bilateral dearness and a near-normal population of cochlear ganglion cells. Reduction in cell size was greatest in cases of bacterial labyrinthitis or Scheibe degeneration with reduced populations of ganglion cells and longer periods of deafness. At the level of the cochlear nucleus, there was no consistent difference in cell size between the side stimulated by a functioning prosthetic device and the nonstimulated side.