Back

Early Onset Adult Deafness (Rhodesian Ridgeback Type)

Early Onset Adult Deafness (EOAD) in Rhodesian Ridgebacks is an inherited disorder causing progressive hearing loss typically between 1-2 years of age, confirmed by a BAER test, without any other associated abnormalities.




Affected Genes: EPS8L2

Inheritance: Autosomal Recessive

Variant(canFam6):
chr18:25423563-25423575: 13 bp deletion GTGCACTTCCTCT

Breed: Rhodesian Ridgeback

General Information: Early Onset Adult Deafness (EOAD) is an inherited disorder in dogs, causing progressive hearing loss typically between 1-2 years of age, though it can present as early as 4 months. Diagnosis is confirmed with a Brainstem Auditory Evoked Response (BAER) test. This form of deafness is unique and not associated with pigment loss or other causes. Affected dogs do not exhibit any other abnormalities.

How to Read Your Dog's Test Results for this Genetic Variant:

Two Variants Detected: Dog Likely Affected

One Variant Detected: Dog Unlikely Affected

No Variants Detected: No Effect

Gene / Testing Information: Genetic testing of the EPS8L2 gene can identify carriers of Early Onset Adult Deafness (EOAD) in dogs. This autosomal recessive disorder requires two copies of the mutated gene for a dog to be affected. Carrier dogs typically do not show symptoms, but breeding two carriers can result in affected pups, with a 25% chance of developing EOAD. Reliable genetic testing is essential for informed breeding practices to prevent this disorder. Testing should be done before breeding, as symptoms may not appear until adulthood.

References:
Kawakami T, Raghavan V, Ruhe AL, Jensen MK, Milano A, Nelson TC, Boyko AR. Early onset adult deafness in the Rhodesian Ridgeback dog is associated with an in-frame deletion in the EPS8L2 gene. PLoS One 2022 17(4):e0264365.

Strain GM. The Genetics of Deafness in Domestic Animals. Front Vet Sci. 2015 2:29.