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Deafness: hereditary

ISSN 2398-2942

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Synonym(s): Congenital sensorineural deafness (CSD), Early Adult Onset Deafness (EAOD)


  • Hereditary deafness.
  • Rare in total canine population but may occur commonly in specific breeds, eg Dalmatian, Border Collie, Great Dane, English Setter.
  • Most common form of deafness in dogs.
  • Cause: autosomal dominant or recessive gene. (X-linked occurs in humans. The mode of inheritance has not been demonstrated yet in dogs.)
  • Associated with coat color (white or merle) and blue irises.
  • Signs: bi- or unilateral deafness.
  • Diagnosis: brain stem auditory evoked potentials.
  • Treatment: none.
  • Prognosis: no cure but unilaterally deaf animals can lead normal life. Print off the owner factsheet on Living with a deaf dog Living with a deaf dog to give to your client.

Presenting signs

  • Difficult to rouse from sleep.
  • Difficult to train.

Age predisposition

  • From 14 days old (when ears should open).
  • Degeneration complete by 5 weeks old.

Breed/Species predisposition



  • Two genes for pigmentation are linked to hereditary deafness in dogs.
  • The first is the merle (M) locus, controlled by the premelanosome protein (PMEL) gene, previously called silver (SILV).
  • Merle is broadly characterized by the alleles ‘M’ and ‘m’, where m represents the wildtype allele for normal pigmentation and ‘M’ indicates the presence of the Merle variant in any form.
  • Merle coat colour has been associated with hearing loss in several breeds, proposedly owing to the non-survival or degeneration of melanocytes within the cochlear of the inner ear.
  • The dominant M allele is carried by many breeds, including (but not restricted to) the Border Collie, Australian Shepherd, Old English Sheepdog, Dalmatian, Great Dane and the Cardigan Welsh Corgi, and is associated with an increased risk of deafness.
  • Neither heterozygosity or homozygosity for the M allele (Mm and MM respectively) invariably results in deafness, although the risk increases with the number of M alleles carried.
  • The second gene is MITF (micropthalmia-associated transcription factor) with MITF-associated white markings linked to increased prevalence of pigment-associated congenital sensorineural deafness.
  • The white spotting alleles of MITF (Piebald, sᵖ) , and Extreme White (), common in breeds such as the Dalmatian, Bull Terrier and Beagle, have also been associated with higher risk deafness.
  • The inheritance of pigment-associated deafness is complex and the mechanism by which it occurs is not incompletely understood.

Forms of deafness not associated with pigment

  • Early Adult Onset Deafness in Border Collies is not associated with pigment and has been associated with a region on canine chromosome 6, although precise causal variants have not been published to date.
  • A non-syndromic form deafness in Beauceron dogs Beauceron is characterized by a bilateral hearing loss in puppies and is also not linked to coat color.
  • Rhodesian Ridgebacks can suffer from progressive hearing loss within 1-2 years after birth, that is not associated with pigment and dog is associated with an in-frame deletion in the EPS8L2 gene.
  • A form of congenital deafness has been described in the Australian Stumpy Tail Cattle that is associated with A missense mutation in the KLF7 Gene.
  • Bilateral deafness with concurrent vestibular dysfunction has been described in the Doberman pinscher is associated with a coding mutation in the MYO7A gene.

Predisposing factors


  • Genotype of parents.
  • Genotype of offspring.
  • Breed.


  • Neonatal puppies → absence of melanocytes (function unknown) in stria vascularis of cochlea → degeneration of stria vascularis → degeneration of hair cells of cochlear ducts → sensorineural deafness.
  • Dobermans → direct loss of cochlear hair cells → deafness with vestibular signs. Simple autosomal recessive.


  • Degeneration of inner ear structures completed by 5 weeks old.
  • For early adult onset forms of deafness (eg Border Collie and Rhodesian Ridgeback) dogs are born with normal hearing that degenerates later in life.


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Further Reading


Refereed papers

  • Recent references from PubMed and VetMedResource.
  • Abitbol M et al (2023) A CDH23 missense variant in Beauceron dogs with non-syndromic deafness. Anim Genet 54(1), 73-77 PubMed.
  • Brancalion L et al (2022) Canine coat pigmentation genetics: a review. Anim Genet 53(1), 3-34 (and references therein) PubMed.
  • Kawakami T et al (2022) Early onset adult deafness in the Rhodesian Ridgeback dog is associated with an in-frame deletion in the EPS8L2 gene. PLoS One 17(4), e0264365 PubMed.
  • Xu F et al (2021) A Missense Mutation in the KLF7 Gene Is a Potential Candidate Variant for Congenital Deafness in Australian Stumpy Tail Cattle Dogs. Genes (Basel) 12(4), 467 PubMed.
  • Webb A A et al (2019) A missense mutation in MYO7A is associated with bilateral deafness and vestibular dysfunction in the Doberman pinscher breed. Can J Vet Res 83(2), 142-148 PubMed.
  • Yokoyama J S et al (2012) Variation in genes related to cochlear biology is strongly associated with adult-onset deafness in border collies. PLoS Genet 8(9),  e1002898 PubMed DOI: 10.1371/journal.pgen.1002898.
  • Strain G M (2004) Deafness prevalence and pigmentation and gender associations in dog breeds at risk. Vet J 167, 23–32 PubMed DOI: 10.1016/s1090-0233(03)00104-7.
  • Coppens A G et al (2000) Bilateral deafness in a Maltese Terrier and a Great Pyrenean puppy - inner ear morphology. J Comp Pathol 122 (2-3), 223-228 PubMed.
  • Strain G M (1999) Congenital deafness and its recognition. Vet Clin North Am Small Animl Pract 29 (4), 895-907 PubMed.

Other sources of information

  • Knowles K (2000) Deafness in dogs and cats. In: Current Veterinary Therapy XII. Ed: R W Kirk. Philadelphia: W B Saunders. pp 971-974.