Bilateral deafness in a maltese terrier and a great pyrenean puppy: inner ear morphology

Two puppies, a 4-month-old female Maltese terrier and a 6-week-old male Great Pyrenean, were presented for confirmation of bilateral deafness by electrophysiological testing. In both puppies, brainstem auditory potentials were not evoked by 90 dB NHL click stimulation of each ear. Examination of the inner ear revealed a bilateral cochleo-saccular degeneration in both animals. The lesions were characterized by generalized atrophy of the stria vascularis, collapse of the cochlear duct, degeneration of the organ of Corti, an abnormal tectorial membrane, and saccular collapse, with a normal spiral ganglion. The cochlear duct was entirely obliterated throughout the cochleae in the Maltese terrier puppy, but was locally and asymmetrically affected in the Great Pyrenean. The abnormalities observed in the Maltese terrier puppy were identical with those previously described in deaf Dalmatian puppies; the lesions observed in the Great Pyrenean, however, were less typical. This is the first histopathological description of cochleo-saccular degeneration in the Maltese terrier and Great Pyrenean breeds. In both puppies the defect was probably congenital.