See also deafness for information about loss of hearing with no other associated clinical signs.

related terms: congenital peripheral vestibular disease

What is congenital deafness and vestibular disease?

The vestibule is an oval cavity in the inner ear containing tiny bones (bony labyrinth) and sensory cells that affect locomotion and are part of the hearing system. In dogs with this condition, some of these structures begin to degenerate shortly after birth. Affected dogs show early signs of a vestibular deficit (eg. a head tilt, lack of coordination, and circling) and become completely deaf by about 3 weeks of age, although this is often not noticed until later.

How is congenital deafness and vestibular disease inherited?

This disorder has been most studied in the Doberman pinscher, in which it is believed to be an autosomal recessive trait.

What breeds are affected by congenital deafness and vestibular disease?

Doberman pinscher. Deafness with vestibular signs is also reported in the beagle, Akita, English cocker spaniel, German shepherd dog, Shetland sheepdog, and Tibetan terrier.

For many breeds and many disorders, the studies to determine the mode of inheritance or the frequency in the breed have not been carried out, or are inconclusive. We have listed breeds for which there is a consensus among those investigating in this field and among veterinary practitioners, that the condition is significant in this breed.

What does congenital deafness and vestibular disease mean to your dog & you?

Although affected puppies are lively and alert, owners or breeders will usually see signs such as a head tilt, circling behaviour, or a tendency to roll over or fall by 10 to 16 weeks of age. Pups lose their hearing a few to several weeks after birth. Signs of bilateral deafness may include aggressive play because the deaf pup does not hear cries of pain from littermates, and having to be jostled to waken. 

The clinical signs such as circling, head tilt, and lack of coordination usually improve with age. This is a result of compensation by other parts of the brain. However specific tests show that the underlying vestibular deficit remains the same.

Deafness is permanent. Although dogs can accommodate remarkably well in familiar surroundings, dogs that are deaf in both ears startle easily, are difficult to train, and are prone to accidents. For these reasons, they are usually euthanized as pups if the condition is recognized early. As an alternative, an owner may train the dog to respond to hand signals or other visual cues, always restrain him/her on a leash or in a fenced yard, and be alert to the possibility of the dog biting someone if startled.

How is congenital deafness and vestibular disease diagnosed?

The clinical signs (head tilt, circling, etc.) point to a vestibular deficit. Your veterinarian will do blood tests to rule out various possible causes, since this particular disorder is fairly rare. A BAER (Brainstem Auditory-Evoked Response) test is required to evaluate hearing, as hearing loss can be very difficult to assess by clinical examination (ie. behavioural response to sounds). The BAER test is a painless and reliable means of detecting hearing loss in one or both ears, that is available at veterinary schools and referral centres. It can be used in puppies of 5 weeks of age on. Older pups and adult dogs may need to be sedated for the test.

How is congenital deafness and vestibular disease treated?

There is no treatment or cure. The clinical signs of a vestibular deficit are likely to improve with age; deafness is permanent.

Breeding advice

Although little is known of the inheritance of this condition in breeds other than the doberman, affected dogs should definitely not be bred. It is preferable not to breed their close relatives (parents or siblings) either.

FOR MORE INFORMATION ABOUT THIS DISORDER, PLEASE SEE YOUR VETERINARIAN.

Resources

Wilkes, M.K., Palmer, A.C. 1992. Congenital deafness and vestibular deficit in the doberman. J. of Small Animal Practice. 33: 218-224. This reference has detailed information on the pathological changes seen with this disorder.

Copyright 1998 Canine Inherited Disorders Database. All rights reserved.
Revised: October 30, 2001.

This database is a joint initiative of the Sir James Dunn Animal Welfare Centre at the Atlantic Veterinary College, University of Prince Edward Island, and the Canadian Veterinary Medical Association.