Separate forms of pathology in the cochlea of congenitally deaf white cats.

Congenital deafness due to cochlear pathology can have an immediate or progressive onset. The timing of this onset could have a significant impact on the development of structures in the central auditory system, depending on the animal's hearing status during its critical period. In order to determine whether cats in our deaf white cat colony suffered from progressive hearing loss, they were tested repeatedly in 30-day intervals using standard auditory evoked brainstem response (ABR) methodology. ABR thresholds did not change over time, indicating that deafness in our colony was not progressive. Moreover, different forms of cochlear pathology were associated with deafness. One form (67% of the deaf ears) had a collapsed Reissner's membrane that obliterated the scala media, resembling what is called the Scheibe deformity in humans. A second form (18%) exhibited excessive epithelial growth within the bony labyrinth. A third form (15%) combined excessive epithelial growth in the apex and a collapsed Reissner's membrane in the base. Cochleae having an abnormally thin tectorial membrane and an outward bulging Reissner's membrane were associated with elevated thresholds (poor hearing).