title: Further contributions to the genetic aspect of congenital sensorineural deafness in Dalmatians.
abstract: Sensorineural deafness is a common congenital disorder in Dalmatians and is genetically transmitted. Different modes of inheritance have been proposed and the objective of this study was to study these by segregation analyses using maximum likelihood procedures. Data from 33 complete Dalmatian families were collected and data from 56 single Dalmatians added. This resulted in a total of 575 dogs with 357 known phenotypes. All dogs were clinically evaluated and electrophysiologically tested with brainstem auditory evoked responses. The prevalence of deafness was 16.5% (9.4% unilaterally deaf, 7.1% bilaterally deaf). Females were 4.4% more affected than males but this difference was not significant. Within the same litter, different phenotypic expressions of deafness occurred, which suggested different expressions of the disease. In addition, two data sets were analysed: the first included normal, uni- and bilaterally deaf dogs, the second had normal and deaf Dalmatians. We found that a recessive allele at a single biallelic major locus fitted our data best, although an incomplete penetrance of the recessive homozygotes was observed.
authors.affiliation: Department for Clinical Veterinary Medicine, Section of Neurology, Institute of Animal Genetics, Nutrition and Housing, University of Berne, Switzerland. anne.muhle@itn.uni-be.ch
authors.forename: Anne C
authors.initials: AC
authors.lastname: Muhle
authors.forename: A
authors.initials: A
authors.lastname: Jaggy
authors.forename: C
authors.initials: C
authors.lastname: Stricker
authors.forename: F
authors.initials: F
authors.lastname: Steffen
authors.forename: G
authors.initials: G
authors.lastname: Dolf
authors.forename: A
authors.initials: A
authors.lastname: Busato
authors.forename: M
authors.initials: M
authors.lastname: Kornberg
authors.forename: M
authors.initials: M
authors.lastname: Mariscoli
authors.forename: P
authors.initials: P
authors.lastname: Srenk
authors.forename: C
authors.initials: C
authors.lastname: Gaillard
mesh_terms: D000818:Animals; D001933:Brain Stem / Q000473:pathology; D004283:Dog Diseases / Q000235:genetics*; D004285:Dogs; D005072:Evoked Potentials, Auditory; D005260:Female; D005808:Genes, Recessive; D006319:Hearing Loss, Sensorineural / Q000235:genetics / Q000662:veterinary*; D008297:Male; D010375:Pedigree; D015995:Prevalence; D012737:Sex Factors