Inner ear disease is extremely rare in the old English sheepdog. The present case would constitute the second known case in Scandinavia and the first one observed in Norway. The morphological investigation revealed complete degeneration of the organ of Corti. A single layer of cells was covering the anatomical location of the stria vascularis. The dog did not show clinical signs of vestibular dysfunction. Hair cells were present in the cristae ampullares and macula utriculi. The histopathological features support a genetic basis for the sensorineural hearing loss, though other (rare) causes of exogenically induced inner ear degeneration cannot be completely excluded on the morphological findings alone. The brain revealed signs of chronic meningitis.