Congenital sensorineural deafness in English setters in the United Kingdom: prevalence and association with phenotype and sex.

BACKGROUND

The English setter (ES) is predisposed to congenital sensorineural deafness (CSD). CSD prevalence and association with phenotype in the UK ES population are previously unreported.

METHODS

The database of the authors' institution was searched for ES puppies undergoing brainstem auditory evoked response (BAER) testing for CSD screening (2000-2018). Inclusion criteria were BAER performed at 5-10 weeks of age, testing of complete litters and available phenotypic data. The age, sex, presence of patches at birth, coat colour, iris colour, hearing status and BAER-determined parental hearing status of each puppy were recorded. Multivariable binary logistic regression was performed to determine the significance of these variables as predictors for the likelihood of puppies being unilaterally or bilaterally deaf.

RESULTS

Inclusion criteria were met for 447 puppies. Hearing was bilaterally normal in 427 (95.5 per cent) puppies. The prevalence of unilateral and bilateral CSD was 3.6 per cent and 0.9 per cent, respectively. Females were 3.3 times more likely to be deaf than males, and puppies with both parents of unknown hearing status were 4.6 times more likely to be deaf than those with at least one normal parent.

CONCLUSION

The prevalence of CSD was 4.5 per cent, with female puppies and those with two parents of unknown hearing status at greatest risk.