The objective of this study was to evaluate any otopathologic changes in temporal bone specimens from dogs with deafness related to cochleosaccular (Scheibe) dysplasia (CSD). We used the canine temporal bone collections of the Otopathology Laboratory at the University of Minnesota and of the Massachusetts Eye and Ear Infirmary at Harvard University in Boston. Our morphometric analysis included measuring the areas of the stria vascularis and the spiral ligament and counting the number of spiral ganglion cells. In addition, we noted the presence of the organ of Corti and cochlear hair cells, assessed the location of Reissner’s membrane and the saccular membrane, and counted the number of both Type I and Type II vestibular hair cells in the macule of the saccule and vestibular ganglion cells. In the group of specimens from dogs with cochleosaccular dysplasia, we observed generalized degeneration in the cochlea and a significantly decreased number of Type I and Type II vestibular hair cells and vestibular ganglion cells. As hereditary deafness is presently untreatable with known therapeutic methods, dogs with cochleosaccular dysplasia should not be considered for breeding. Future therapeutic approaches, such as stem cell therapies, should be designed to target all the elements of the cochlea in addition to the saccule as it was found that both are affected in dogs with CSD.
|Original language||English (US)|
|Number of pages||6|
|Journal||Canadian Journal of Veterinary Research|
|State||Published - Jan 2019|
Bibliographical noteFunding Information:
The authors thank the International Hearing Foundation; 5M Lions International; Starkey Hearing Foundation; the Scientific and Technological Research Council of Turkey (TUBITAK); and the Otopathology Laboratory, Department of Otolaryngology, Massachusetts Eye and Ear Infirmary and the Harvard Medical School for their support. The authors also thank Mary E. Knatterud for editing the manuscript.