Affected puppies can exhibit clinical signs of vestibular defects (head tilt and possible loss of balance) as early as one to two weeks of age - though the presentation is commonly mild at a young age and becomes more pronounced as the dog ages. The affected dog can appear normal by a routine physical examination, however a neurological examination often reveals signs of bilateral vestibular disease and auditory testing (BAER exam) is critical to identify deafness. Pathological findings reveal degeneration with the cochlear portion of the inner ear. This disease was first reported in the Doberman Pinscher breed in the early 1980’s, and later clinically characterized in 1992*. More recently the genetic basis of this recessively inherited disorder was resolved through the successful collaboration efforts of Dr. Mark Neff, Head of the Laboratory of Canine Genetics & Genomics at the Van Andel Research Institute, in Grand Rapids, MI, and Dr. Aubrey Webb of the CullenWebb Animal Neurology & Ophthalmology Center in NB, Canada. A causative genetic mutation was identified - enabling the development of the now available DNA test. As the disorder is an autosomal recessive disease, breeders can now judiciously choose to reduce the prevalence of the disease by only crossing known heterozygote (carrier dogs) to known unaffected dogs or choose to only mate unaffected dogs.