Further contributions to the genetic aspect of congenital sensorineural deafness in Dalmatians

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.     

Gender effects in hearing loss in Dalmatians

Brainstem auditory-evoked-response data were collected from 3101 Dalmatian dogs from 1984 to 1998 at the Veterinary Medicine Teaching Hospital at the University of California, Davis. Also collected were data on eye color and the presence or absence of a color-patch at birth. Our objective was to evaluate the role of gender in hearing loss, including the possibility that the probability of suffering unilateral or bilateral deafness was greater if the dam was hearing impaired than if the sire was hearing impaired.Results of a multiple-trait threshold-model analysis support the commonly held observation that females were more likely to be deaf than males. In addition, females were also more likely to have two blue eyes (a condition associated with an increased prevalence of deafness). However, gender differences in hearing loss were limited to these direct observations. There was no detectable difference in the prevalence of hearing loss between offspring of deaf mothers and the offspring of deaf fathers. Finally, there was no detectable decrease in the prevalence of hearing loss over the years covered in the data set - suggesting that Dalmatian breeders are not yet selecting against hearing problems.     

Pedigree study of the heredity of copper-associated hepatitis in Dalmatians in Japan

The pedigrees of 3 Dalmatian dogs afflicted with copper-associated hepatitis were investigated to discover the mode of inheritance. A composite family pedigree showed that the 3 affected Dalmatians were related. None of the parents of the affected dogs showed clinical symptoms of liver disease, and the disease had no sex predisposition. The estimated segregation ratio was approximately 3:1 based on surviving littermates. These findings suggested that the copper-associated hepatitis in these Dalmatians was an autosomal recessive mode of inheritance. In addition, some male Dalmatians imported from abroad might have been involved in the occurrence of this disease in Japan.     

Brainstem Auditory-Evoked Potential Assessment of Congenital Deafness in Dalmatians: Associations With Phenotypic Markers

To screen for congenital deafness, brainstem auditory-evoked potential (BAEP) testing was performed on 1031 Dalmatians from three geographically separated areas. Phenotypic marker assessment was done to determine markers possibly associated with deafness. Markers included sex, hair coat color, pigmentation of different areas of skin (eye rims, nose, and ears), presence of a patch, spot size and marking (density of spotting), sire and dam BAEP status, and presence of iris and retinal tapetal pigmentation. Combined data from all test sites showed 8.1% bilateral deafness (N = 83 dogs) and 21.6% unilateral deafness (N = 223), or an overall 29.7% incidence of hearing disorders. Significant (P less than 0.05) associations with deafness for the data from all test sites combined were seen for patch, sire and dam BAEP, iris pigment, and retinal pigment. However, results differed for several of the significant phenotypic markers when analyses were done on the data from the individual test sites; changes from significant to not significant were found. This suggested the existence of multiple populations of deafness patterns, and reinforced the precautionary conclusion that associations of phenotypic markers with deafness are not necessarily functionally significant.     

Canine Dal blood type: A red cell antigen lacking in some Dalmatians

BACKGROUND: Based upon alloantibodies produced after sensitizing dogs with transfused blood, more than a dozen blood group systems have been recognized thus far, and some have been classified as dog erythrocyte antigens (DEA). HYPOTHESIS: A new canine red cell antigen was suspected, based on the development of specific alloantibodies in a Dalmatian previously sensitized by blood transfusions. ANIMALS: Twenty-six Dalmatians (including 1 Dalmatian in need of blood compatibility studies); 55 canine blood donors. METHODS: Serologic tests, including blood typing, crossmatching, and direct Coombs' test were performed by standard tube techniques and a novel gel column technology adapted from human blood banking. RESULTS: By day 40 after transfusion of an anemic Dalmatian, all major crossmatch tests to 55 non-Dalmatian dogs were incompatible. The 2 initial donors, who were compatible before transfusion, were also now incompatible, suggesting the development of an alloantibody to a common red cell antigen. No siblings were available, but 4 of 25 unrelated Dalmatians were crossmatch compatible, suggesting that they were missing the same red cell antigen. The patient was blood typed DEA 1.1, 3, 4, and 5 positive, but DEA 7 negative. Further blood typing and crossmatching results did not support an association to any of these known blood types. The alloantibodies produced were determined to be of the immunoglobulin G class. CONCLUSIONS AND CLINICAL IMPORTANCE: Based upon the identification of an acquired alloantibody in a Dalmatian, a presumably new common blood type named Dal was identified. Dalmatians lacking the Dal antigen are likely at risk of delayed and acute hemolytic transfusion reactions.     

Heritability and complex segregation analysis of hypoadrenocorticism in the standard poodle

The heritability of hypoadrenocorticism (Addison's disease) was evaluated in 778 standard poodles with known Addisonian phenotypes. Addisonian status was confirmed clinically by adrenocorticotropic hormone (ACTH) challenge and 8.6 per cent of the poodles enrolled in the study were classified as being Addisonian. Hypoadrenocorticism affected both sexes with equal probability (P > 0.1). The most common coat colours had a negligible effect on the incidence of hypoadrenocorticism (P > 0.09), although red coat colour had a significant impact on the disease, probably due to the relatively small numbers of dogs with that coat colour. The heritability of hypoadrenocorticism in the standard poodle was estimated to be 0.75. Complex segregation analyses suggested that hypoadrenocorticism in the breed is influenced by an autosomal recessive locus. Clarification of both the heritability and mode of inheritance of hypoadrenocorticism in the standard poodle allows for better-informed breeding decisions.     

Copper-associated liver disease in Dalmatians: a review of 10 dogs (1998-2001)

This retrospective study summarizes 10 Dalmatians suspected of having hepatic copper toxicosis. Hepatic copper toxicosis can result from either a primary metabolic defect in hepatic copper metabolism or from altered hepatic biliary excretion of copper. An inherited copper-associated hepatopathy has been documented in Bedlington Terriers, and there is evidence for familial copper-associated liver disease in West Highland White (WHW) Terriers and Skye Terriers. Nine of the 10 Dalmatians in this study presented for gastrointestinal clinical signs, including anorexia and vomiting. All animals had increased alanine aminotransferase (ALT) enzyme activity, and 9 of 10 had increased alkaline phosphatase (ALP) enzyme activity. The relative increase in ALT activity was much greater than the relative increase in ALP activity, suggesting a predominantly hepatocellular rather than cholestatic liver disease. The mean hepatic copper concentration for 9 Dalmatians was 3,197 microg/g dry weight liver (dwl) (normal, <450 microg/g). In 5 of these 9 dogs, hepatic copper concentrations exceeded 2,000 microg/g dwl. Necroinflammatory alterations associated with copper-laden parenchymal cells were the notable histopathologic finding. The inflammatory infiltrate was either primarily lymphocytic or neutrophilic. Morphologic features of cholestasis generally were not prominent except in those dogs with severe pathology. These findings lend support to the hypothesis that a primary metabolic defect in hepatic copper metabolism occurs in the Dalmatian breed. The mechanism and genetic basis of this condition require further study.     

Electrodiagnostic evaluation of deafness in two English setter littermates

Two English Setter littermates (male and female) were evaluated for hearing difficulties at 4, 16, and 24 months of age. Auditory function was evaluated by behavioral response to pure-tone sounds, tympanometry, brain stem auditory evoked response, and acoustic reflex testing. Hereditary sensorineural deafness was diagnosed in both dogs. The extent of the hearing deficit, as characterized by these tests, was slightly different between the dogs, as well as between the ears in 1 dog.     

Congenital deafness and vestibular deficit in the dobermann

A condition characterised by the early onset of vestibular deficit and hearing loss was investigated in the dobermann breed of dog. Affected pups showed behavioural signs of head tilt, circling and ataxia and there was a total absence of vestibular response to rotation or caloric stimulation. Severe deafness, as assessed by brainstem auditory evoked response testing, was present by three weeks of age in all affected animals. The inner ears showed a progressive neuroepithelial type of cochlear degeneration with loss of the auditory sensory cells. In the vestibular system, however, there was no equivalent sensory cell loss and the only abnormal feature was the absence or abnormality of the otoconia in some of the affected animals. Pedigree analysis suggested that the condition was inherited as an autosomal recessive trait.     

“杜长嘉”商品猪毛色分离现象的遗传分析

通过对504窝5828头杜长嘉商品猪毛色分离的调查,发现杜长嘉商品猪群体中通常有全白色、棕红色、白黑花斑、棕白花斑、黑棕花斑和全黑色6种毛色[5],且不同毛色长嘉母本的后代毛色的构成比不同。其结果如下:①纯白长嘉母猪后代:全白色0 4486±0 1868、棕红色0 1842±0 1426、白黑花斑0 0708±0 1175、棕白花斑0 0264±0 0706、黑棕花斑0 0844±0 1203、全黑色0 1856±0 1240;②黑斑长嘉母猪后代:全白色0 3685±0 2191、棕红色0 0899±0 1069、白黑花斑0 1773±0 1482、棕白花斑0 0546±0 0874、黑棕花斑0 1045±0 1236、全黑色0 2052±0 1651。     

Diagnosis of deafness in a horse by brainstem auditory evoked potential

Deafness was confirmed in a blue-eyed, 3-year-old, overo paint horse by brainstem auditory evoked potential. Congenital inherited deafness associated with lack of facial pigmentation was suspected. Assessment of hearing should be considered, especially in paint horses, at the time of pre-purchase examination. Brainstem auditory evoked potential assessment is well tolerated and accurate.     

Disseminated protothecosis causing acute blindness and deafness in a dog

A 3-year-old Collie bitch was euthanatized two weeks after the onset of blindness and deafness. The hearing deficit had been localized by clinical signs, brain stem auditory evoked responses, and impedance audiometry. Protothecosis was diagnosed by cytologic and histologic examinations. The organism was identified as Prototheca zopfii . Organisms and granulomatous lesions were found in kidney, heart, liver, skeletal muscle, thyroid gland, colon, bronchial lymph node, brain, and cochlea.     

Abnormal segregation of a haemagglutinating gene in a control flock

The frequency of a dominant autosomal gene Ka + was observed during two generations in a pedigree control flock of Australorps. The red blood cells of birds carrying the Ka + gene were agglutinated by Kahn's antigen.

Male parents heterozygous at the haemagglutination locus transmitted the Ka ⁺gene and its alternative the Ka～ gene to their offspring in the ratio of 0.6 to 1. Female parents had normal transmission ratios.

Eggs from matings with sires carrying the Ka ⁺ gene showed reduced fertility and there was an increased mortality of embryos early in development. These eggs had a 14.6 per cent lower hatchability than eggs from Ka ^‐sire matings in one generation and a 5.2 per cent lower hatchability in the next.

The frequency of the Ka ⁺ gene in the flock declined from 0.23 to 0.16 between the parents of one generation and the offspring of the next despite the restraint of the pedigree control system.