Deafness prevalence and pigmentation and gender associations in dog breeds at risk

Hearing function was tested in dogs from breeds at risk for pigment-associated congenital sensorineural deafness - Dalmatian, English setter (ES), English cocker spaniel (ECS), bull terrier (BT), Australian cattle dog (ACD), whippet, Catahoula leopard dog, and Jack Russell terrier. Deafness prevalence was highest in Dalmatians and lowest in ECS. Phenotype correlation studies were performed in breeds with >100 brainstem auditory evoked responses (BAER) tested subjects. No gender differences were observed. No differences were seen between black- and liver-spotted Dalmatians, among the ES roan colour varieties, among the ECS parti varieties, or among the ACD colour varieties. Blue eyes were positively associated and patches were negatively associated with deafness in the Dalmatian. Blue eyes were also associated with deafness in the ES and ECS. White BT were more likely than coloured BT to be deaf. Having one or more parent's ear deaf was positively associated with deafness in Dalmatians, ES, and ECS.     

Prevalence of Deafness in Dogs Heterozygous or Homozygous for the Merle Allele

Background: Deafness in dogs is frequently associated with the pigment genes piebald and merle. Little is known about the prevalence of deafness in dogs carrying the merle allele.
Objective: To determine the prevalence of deafness in dogs heterozygous and homozygous for the merle allele of the mouse Silver pigment locus homolog (SILV) gene.
Animals: One hundred and fifty-three privately owned merle dogs of different breeds and both sexes.
Methods: Hearing was tested by brainstem auditory-evoked response and classified as bilaterally hearing, unilaterally deaf, or bilaterally deaf. DNA from buccal cells was genotyped as either heterozygous or homozygous for the merle allele. Deafness association tests among merle genotype, eye color, and sex were performed by the χ² test.
Results: Deafness prevalence in merles overall was 4.6% unilaterally deaf and 4.6% bilaterally deaf. There was a significant association between hearing status and heterozygous versus homozygous merle genotype. For single merles ( Mm ), 2.7% were unilaterally deaf and 0.9% were bilaterally deaf. For double merles ( MM ), 10% were unilaterally deaf and 15% were bilaterally deaf. There was no significant association with eye color or sex.
Conclusions: Deafness prevalence in merle dogs was greater than that in some dog breeds homozygous for the piebald gene, such as the English Cocker Spaniel, but comparable to, or lower than, that in the Dalmatian and white Bull Terrier. Dogs homozygous for the merle allele were significantly more likely to be deaf than heterozygotes.     

Complex segregation analysis of deafness in Dalmatians

OBJECTIVE: To use pedigree analysis to evaluate the feasibility of a major locus model for deafness in Dalmatians. ANIMALS: 605 purebred Dalmatians from 42 families. PROCEDURE: Hearing loss was evaluated through the brainstem auditory-evoked response. Dogs were classified into mutually exclusive categories: normal hearing, unilaterally deaf, or bilaterally deaf. Information was collected on sex, coat color, presence or absence of a color patch at birth, and eye color. Statistical analyses were performed by use of regressive logistic models designed for complex segregation analysis. Genetic correlations among eye color, deafness, and color patch were estimated. RESULTS: Prevalence of hearing loss was 11% for dogs classified as unilaterally deaf and 5% for dogs that were bilaterally deaf. Complex segregation analysis detected statistical evidence of a single allele with an expected frequency of 0.21 that had an effect on the prevalence of deafness. Results of analyses suggested that this locus cannot completely explain the inheritance and incidence of deafness in Dalmatians. Genetic correlation estimates among deafness, eye color, and color patch revealed strong interrelationships among these characteristics. CONCLUSIONS AND CLINICAL RELEVANCE: To reduce the incidence of hearing loss in Dalmatians, unilaterally deaf, blue-eyed dogs should not be considered as potential parents.     

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.     

Use of brain stem auditory-evoked response to evaluate deafness in a group of Dalmatian dogs

A group of 18 adult and 28 six-week-old purebred Dalmatian dogs was tested for hearing by brain stem auditory-evoked responses. Each ear was tested independently. Absence of a response was considered evidence of deafness in that ear. Responses from bilaterally hearing adults and pups and unilaterally hearing dogs were compared with each other and with responses from normally hearing, adult, mixed-breed dogs. Of the 18 adults tested, 5 had normal responses from both ears, 6 were deaf in both ears, and 7 had a response from only one side. Of 28 pups tested, 14 had normal responses from both ears, 9 had a response from only one ear, and 5 were bilaterally deaf. Wave peak latencies from all ears that responded were within normal limits, except that the 5th and 6th waves from unilaterally hearing adults had significantly longer latencies. There were no differences between males and females.     

The use of contralateral masking noise in the detection of unilateral deafness in Dalmatian puppies

BACKGROUND: The brainstem auditory-evoked response (BAER) is currently the standard evaluation method of hearing in dogs. In asymmetrical hearing loss in human patients, simultaneous presentation of masking noise to the nontest ear is routinely performed during BAER to eliminate the crossover effect. HYPOTHESIS: The crossover effect occurs during canine BAER, and masking noise of 20 decibels (dB) below click stimulus intensity is sufficient to abolish this effect. ANIMALS: Fifty-six Dalmatian puppies with confirmed unilateral deafness. METHODS: The BAER was elicited with 80 and 100 dB normalized hearing level (dBnHL) stimulus intensity in the deaf ear. The 100 dBnHL stimulus was repeated while simultaneously applying 80 dBnHL white masking noise to the nontest ear. RESULTS: Ten dogs were excluded because of BAER trace baseline fluctuation. In the remaining 46 dogs, 8 dogs had no waveforms, but 38 dogs had an identifiable wave-V in the deaf ear BAER at 80 dBnHL intensity stimulus. At 100 dBnHL intensity stimulus, all but 1 dog had a discernible wave-V in the deaf ear BAER. The deaf ear BAER waveforms were abolished by white masking noise at 80 dBnHL in the nontest ear in all dogs. CONCLUSIONS AND CLINICAL IMPORTANCE: Abolition of BAER wave-V in the deaf ear by white masking noise in the nontest ear suggests that this wave is caused by the crossover effect. beta distribution indicates 95% confidence that white masking noise, at 20 dB below click stimulus intensity, would abolish this crossover effect in over 90% of the dogs. This supports using masking noise in the nontest ear during canine BAER.     

Unilateral and Bilateral Congenital Sensorineural Deafness in Client-Owned Pure-Breed White Cats

Background: Congenital sensorineural deafness has been reported frequently in experimental mixed-breed white cats but there is a paucity of data on occurrence of deafness in client-owned pure-breed white cats.
Objective: To describe hearing status in client-owned pure-breed white cats.
Animals: Eighty-four pure-breed client-owned cats with white coat color of 10 registered breeds presented for routine hearing evaluation before breeding (1995–2008).
Methods: Hearing was assessed by click-evoked brainstem auditory evoked response.
Results: Overall deafness prevalence was 20.2%; 9 cats (10.7%) were bilaterally deaf and 8 cats (9.5%) were unilaterally deaf. There was no association between sex and deafness status ( P = .85). Deafness status was associated with iris color ( P = .04).
Conclusions and Clinical Importance: Congenital sensorineural deafness frequently occurs in pure-breed cats with white coat color. Unilateral sensorineural deafness was as common as bilateral deafness.     

Post‐anesthesia deafness in dogs and cats following dental and ear cleaning procedures

Objective The present study was performed to document hearing loss in dogs and cats following procedures performed under anesthesia. Most cases of reported hearing loss were subsequent to dental and ear cleaning procedures. Study design Prospective and retrospective case survey. Animals Subjects were dogs and cats with deafness, personally communicated to one author, cases discussed on a veterinary information web site, and cases communicated through a survey of general practice and dental specialist veterinarians. Methods Reported deafness cases were characterized by species (dog, cat), breed, gender, age, and dog breed size. Results Sixty‐two cases of hearing loss following anesthesia were reported between the years 2002 and 2009. Five additional cases were reported by survey respondents. Forty‐three cases occurred following dental procedures. Sixteen cases occurred following ear cleaning. No relationship was observed between deafness and dog or cat breed, gender, anesthetic drug used, or dog size. Geriatric animals appeared more susceptible to post‐anesthetic, post‐procedural hearing loss. Conclusions Deafness may occur in dogs and cats following anesthesia for dental and ear cleaning procedures, but the prevalence is low. The hearing loss appears to be permanent. Clinical relevance Deafness can be a consequence following anesthesia for dental or ear cleaning procedures. Older animals may have greater susceptibility.     

Does a pleiotropic gene explain deafness and blue irises in white cats?

The prevalence of deafness is high in cat populations in which the dominant white gene is segregating. The objective of this study was to investigate whether there is a gene that is responsible for deafness as well as for blue eyes and to establish a plausible mode of inheritance. For this purpose, data from an experimental colony with deaf cats were analyzed. The hearing status was determined by acoustically evoked brain stem responses (BAER). Complex segregation analyses were conducted to find out the most probable mode of inheritance using maximum likelihood procedures. The prevalence of deafness and partial hearing in the experimental colony was 67% and 29%, respectively. The results of the bivariate segregation analysis support the hypothesis of a pleiotropic major gene segregating for deafness and blue iris colour. The high heritability coefficients for both traits, 0.55 and 0.75 respectively, indicate that beside the major gene there is an important influence of polygenic effects.     

A comparison of the brainstem auditory evoked response in healthy ears of unilaterally deaf dogs and bilaterally hearing dogs

Aims

Auditory plasticity in response to unilateral deafness has been reported in various animal species. Subcortical changes occurring in unilaterally deaf young dogs using the brainstem auditory evoked response have not been evaluated yet. The aim of this study was to assess the brainstem auditory evoked response findings in dogs with unilateral hearing loss, and compare them with recordings obtained from healthy dogs.

Methods

Brainstem auditory evoked responses (amplitudes and latencies of waves I, II, III, V, the V/I wave amplitude ratio, wave I-V, I-III and III-V interpeak intervals) were studied retrospectively in forty-six privately owned dogs, which were either unilaterally deaf or had bilateral hearing. The data obtained from the hearing ears in unilaterally deaf dogs were compared to values obtained from their healthy littermates.

Results

Statistically significant differences in the amplitude of wave III and the V/I wave amplitude ratio at 75 dB nHL were found between the group of unilaterally deaf puppies and the control group. The recordings of dogs with single-sided deafness were compared, and the results showed no statistically significant differences in the latencies and amplitudes of the waves between left- (AL) and right-sided (AR) deafness.

Conclusions

The recordings of the brainstem auditory evoked response in canines with unilateral inborn deafness in this study varied compared to recordings from healthy dogs. Future studies looking into electrophysiological assessment of hearing in conjunction with imaging modalities to determine subcortical auditory plasticity and auditory lateralization in unilaterally deaf dogs are warranted.

     

Prevalence of unilateral and bilateral deafness in border collies and association with phenotype

BACKGROUND: Congenital sensorineural deafness (CSD) occurs in Border Collies, but its prevalence and inheritance are unknown. This study estimated the prevalence of CSD in Border Collies and investigated its association with phenotypic attributes linked to the merle gene, including coat pigmentation and iris color. HYPOTHESIS: Deafness in Border Collies is associated with pigmentation patterns linked to the merle gene. ANIMALS: A total of 2597 Border Collies from the United Kingdom. METHODS: A retrospective study of Border Collies tested, during 1994-2002, by using brainstem auditory evoked responses. Associations between deafness and phenotypic attributes were assessed by using generalized logistic regression. RESULTS: The prevalence of CSD in puppies was estimated as 2.8%. The corresponding rates of unilateral and bilateral CSD were 2.3 and 0.5%, respectively. Adjustment for clustering of hearing status by litter reduced the overall prevalence estimate to 1.6%. There was no association between CSD and sex (P = .2). Deaf Border Collies had higher rates of merle coat pigmentation, blue iris pigment, and excess white on the head than normal hearing Border Collies (all P < .001). The odds of deafness were increased by a factor of 14 for Border Collies with deaf dams, relative to the odds for dogs with normal dams (P = .007), after adjustment for phenotypic attributes. CONCLUSIONS AND CLINICAL IMPORTANCE: Associations between CSD and pigmentation patterns linked to the merle gene were demonstrated for Border Collies. Evidence for an inherited component to CSD in Border Collies supports selective breeding from only tested and normal parents to reduce the prevalence of this disease.     

Effects of otitis on hearing in dogs characterised by brainstem auditory evoked response testing

Hearing function was measured in normal dogs and in dogs with otitis using brainstem auditory evoked response testing. Data were obtained from 86 normal ears and from 105 ears with otitis, cntegorised into four degrees of severity. The data were analysed to illustrate the differences between the hearing function in the normal and abnormal ears and to estimate the degree of impairment associated with differing degrees of pathology. While severe hearing loss seemed to be present in the dogs with more severe otitis, only two individuals were identified as being totally deaf in the affected ears and no dogs were identified in which the cleaning and examination processes had caused damage to hearing function. Cleaning the ear canal produced measurable improvements in hearing in several dogs, indicating the profound effect of physical obstruction of the external ear canal by debris. It is concluded that most dogs with chronic otitis externa are not totally deaf and that the hearing impairment that does occur has the characteristics of conductive hearing loss.     

Congenital sensorineural deafness in dogs: a molecular genetic approach toward unravelling the responsible genes

Deafness is often diagnosed in different dog breeds and has been identified as a significant problem for breeders, owners and clinicians. The aetiology can be inherited or acquired, and a distinction must be made between sensorineural and conductive forms of deafness. This paper provides a brief overview of the varieties of findings in different dog breeds and in one breed in particular including prevalence, phenotypic and gender associations, histology, modes of inheritance and the number of contributing genes in congenital sensorineural deafness. We have also described molecular genetic approaches to canine hearing loss and discuss how comparative genomics could help reduce the prevalence of deafness in affected breeds leading to new insights into the molecular mechanisms of auditory function in both dogs and humans.     

Congenital deafness in Jack Russell terriers: Prevalence and association with phenotype

Congenital hereditary sensorineural deafness is the most common form of deafness in dogs. The objectives of this study were to determine a reliable measure of the prevalence of deafness in Jack Russell terriers, an affected breed, and associations between deafness and phenotypic characteristics. Brainstem auditory evoked response recordings and phenotypic parameters (coat color, coat texture, sex, eye color, sire and dam hearing status) were recorded for 1009 Jack Russell terriers. The prevalence of unilateral and bilateral deafness was 3.57% and 0.50%, respectively, lower by a factor of three to four than in earlier reports based on smaller and closely related kindreds. Significant association with deafness was identified with white coat color and parental hearing status, but not with sex or coat type. Lack of significant sex or coat type associations and the significant association with white coat color are consistent with previous reports. In conclusion the prevalence of deafness in Jack Russell terriers is lower than initially reported. Deafness was associated with white coat color and parental hearing status. The association with parental hearing status supports this form of deafness being a heritable trait in the breed and the association with white coat color supports an inheritance linked to pigmentation genes.     

Brainstem auditory-evoked potential assessment of auditory function and congenital deafness in llamas (Lama glama) and alpacas (L. pacos)

Auditory function of llamas and alpacas was assessed objectively by means of brainstem auditory-evoked response audiometry (BAER) to establish the normal hearing range and to test the hypothesis of a correlation between blue eyes, white coat, and deafness. Sixty-three camelids were available for the study. Thirteen animals had blue irides; 1 animal had 1 blue and 1 pigmented iris. Wave latencies, amplitudes, and interpeak latencies were measured under general anesthetic. Click stimuli (dB [HL]) were delivered by an insert earphone. Four to five positive peaks could be detected; waves I, II, and V were reproducible; wave II appeared infrequently; and wave IV generally merged with wave V to form a complex. Peak latencies decreased and peak amplitudes increased as stimulus intensity increased. A hearing threshold level of 10-20 dB (HL) was proposed as the normal range in llamas and alpacas. None of the animals with pigmentation of coat and iris showed any degree of hearing impairment. Seven of the 10 blue-eyed, pure-white animals were bilaterally deaf and one of them was unilaterally deaf. However, 2 blue-eyed, white animals exhibited normal hearing ability. Three blue-eyed animals with pigmented coat did not show any hearing impairment. All white animals with normal iris pigmentation had normal auditory function; so did the 1 animal with 1 normal and 1 blue iris. The high frequency (78%) of bilaterally deaf animals with pure white coat and blue iris pigmentation supports the hypothesis of a correlation between pigmentation anomalies and congenital deafness in llamas and alpacas.     

N3 potentials in response to high intensity auditory stimuli in animals with suspected cochleo-saccular deafness

We describe a previously un-reported vertex-negative potential evoked by high intensity click auditory stimuli in some dogs and cats with suspected cochleo-saccular deafness. Brainstem auditory evoked potential tracings from 24 unilaterally or bilaterally deaf animals, 22 dogs and 2 cats, among which 21 belonged to breeds with high prevalence of suspected or histologically confirmed cochleo-saccular deafness, were studied retrospectively. Values for latency, amplitude and threshold of this potential in dogs were 2.15+/-0.23 ms, 0.49+/-0.25 microV, and 91.9+/-4.7 dB NHL, respectively (mean+/-SD). Latency and threshold values in cats were in the mean+/-2 SD range of the dog values. Sensitivity to click stimulus polarity and to click stimulus delivery rate pointed towards a neural potential instead of a receptor potential. The vertex-negative wave observed in these animals shares all characteristics with the N3 potential described in some deaf humans with cochlear deafness, where it is presumed to arise from saccular stimulation. The combined degeneration of cochlea and sacculus usually reported in deaf white dogs and cats suggest that N3 may have a different origin in these species.     

Genetic predisposition to purine uroliths in Dalmatian dogs

Homozygosity for a defective recessive gene results in all Dalmatians having elevated concentrations of urine uric acid and predisposes them to formation of purine uroliths. Why some animals form uroliths and others do not is not known. In a pilot breeding experiment, the incidence of sensorineural deafness among backcrossed, high urine uric acid Dalmatians was significantly higher (probability less than 0.05) than among low urine uric acid siblings. Urine uric acid values for Dalmatians reported in the literature are not likely to be comparable, because uric acid appears to be more likely to precipitate in Dalmatian urine than in the urine of other dogs and man. Heating, alkalinization, and thorough mixing are required whenever specimens are transferred from one container to another or an aliquot is removed for assay.     

Brainstem auditory evoked potential wave V latency-intensity function in normal Dalmatian and Beagle puppies

This study investigated whether Dalmatian puppies with normal hearing bilaterally had the same click-evoked brainstem auditory potential characteristics as age-matched dogs of another breed. Short-latency brainstem auditory potentials evoked by condensation and rarefaction clicks were recorded in 23 1.5- to 2-month-old Dalmatian puppies with normal hearing bilaterally by a qualitative brainstem auditory evoked potential test and in 16 Beagle dogs of the same age. For each stimulus intensity, from 90 dB normal hearing level down to the wave V threshold, the sum of the potentials evoked by the 2 kinds of stimuli were added, giving an equivalent to the alternate click polarity stimulation. The slope of the L segment of the wave V latency-intensity curve was steeper in Dalmatian (-40 +/- 10 micros/dB) than in Beagles (-28 +/- 5 micros/dB, P < .001) puppies. The hearing threshold was lower in the Beagle puppies (P < .05). These results suggest that interbreed differences may exist at the level of cochlear function in this age class. The wave V latency and wave V-wave I latencies differences at high stimulus intensity were different between the groups of puppies (4.3 +/- 0.2 and 2.5 +/- 0.2 milliseconds, respectively, for Beagles; and 4.1 +/- 0.2 and 2.3 +/- 0.2 milliseconds for Dalmatians, P < .05). A different maturation speed of the neural pathways is one possible explanation of this observation.     

Unilateral and Bilateral Brainstem Auditory-Evoked Response Abnormalities in 900 Dalmatian Dogs

In a survey of 900 Dalmatian dogs, brainstem auditory-evoked responses (BAER) and clinical observations were used to determine the incidence and sex distribution of bilateral and unilateral BAER abnormalities and their association with heterochromia iridis (HI). To assess the efficacy of BAER testing in guiding breeding programs, data from 749 dogs (subgroup A), considered to be a sample of the population at large, were compared with data from a subgroup (subgroup B; n = 151) in which selection of breeding stock had been based on BAER testing from the beginning of the 4-year survey. Brainstem auditory-evoked responses were elicited by applying click stimuli unilaterally, while applying a white noise masking sound to the contralateral ear. Under these conditions, BAER were either normal, unilaterally absent, or bilaterally absent. Dogs with bilaterally absent BAER were clinically deaf; dogs with unilaterally absent BAER were not clinically deaf but appeared dependent on their BAER-normal ears for their auditory-cued behavior. Dogs with unilaterally absent BAER often were misidentified as normal by uninformed observers. Among the 900 dogs, 648 (72.0%) were normal, 189 (21.0%) had unilateral absence of BAER, and 63 (7.0%) had bilateral absence of BAER or were clinically deaf and assumed to have bilaterally absent BAER (n = 4). Total incidence in the population sampled was assumed to be higher, because some bilaterally affected dogs that would have been members of subgroup A undoubtedly did not come to our attention. Among females, 24.0% were unilaterally abnormal and 8.2% were bilaterally abnormal whereas, among males, 17.8% were unilaterally abnormal and 5.7% were bilaterally abnormal.(ABSTRACT TRUNCATED AT 250 WORDS)