PEDIATRICS: PUPPIES AND KITTENS 0195-5616/99 $8.00 + .

00

CONGENITAL DEAFNESS AND

ITS RECOGNITION
George M. Strain, PhD

Sensory function in neonatal dogs and cats is primarily tactile,

olfactory, and gustatory. The visual and auditory senses, although par-
tially functional at birth, exhibit significant postnatal development: in
the dog, the eyes do not open until a puppy is 8 to 10 days of age, the
ear canals do not open until it is 12 to 13 days of age, and mature system
function up through the cortex is not present until it is 3 months of age
or older. 18• 22 Similar delays are seen in the cat. As a result, disorders of
these systems frequently escape early detection.
Deafness can be described as (1) congenital or late onset, (2) heredi-
tary or acquired, and (3) conductive or sensorineural.14• 15 The most
commonly occurring deafness forms in dogs and cats are congenital
hereditary sensorineural deafness, late-onset acquired sensorineural
deafness, and late-onset acquired conductive deafness. Distinguishing
between hereditary and acquired deafness is generally not possible
without breeding trials, although an assumption of hereditary deafness
can be made in breeds with a high prevalence of deafness. The most
common form of deafness in young dogs and cats is congenital heredi-
tary sensorineural deafness, with acquired conductive and acquired sen-
sorineural deafness appearing on rare occasions.

This work was supported by Grant No 1R15DC01128-01 from the National Institutes
of Health and by a grant from the American Kennel Club.

From the Department of Veterinary Physiology, Pharmacology, and Toxicology, School of

Veterinary Medicine, and Office of Research and Graduate Studies, Louisiana State
University, Baton Rouge, Louisiana

VETERINARY CLINICS OF NORTH AMERICA: SMALL ANIMAL PRACTICE

VOLUME 29 • NUMBER 4 • JULY 1999 895

896 STRAIN

PATHOPHYSIOLOGY OF DEAFNESS

Perception of sound first requires transmission through the outer

and middle ears to the cochlea for transduction by neural hair cells.
Perception results from (1) transmission of transduced auditory informa-
tion from the cochlea by the eighth cranial nerve to the dorsal and
ventral cochlear nuclei, the inferior colliculus, the medial geniculate
nucleus of the thalamus, and the primary and secondary cortical audi-
tory areas on the temporal lobe; and (2) attention to the arriving informa-
tion. Commingling of ipsilateral and contralateral auditory information
occurs at multiple steps in the ascent up the auditory pathway. As a
result, unilateral hearing loss rarely results from lesions or disease affect-
ing auditory structures above the eighth nerve. Central deafness (unilat-
eral or bilateral) in the absence of severe neurological disease is clinically
unknown in veterinary medicine and is not considered further here.

Conduction Deafness

Conduction deafness results from blockage of sound transmission

to the cochlea as a consequence of occlusion of the ear canal or middle
ear cavity, or from developmental defects. Occlusion may result from
excess cerumen production, from otitis externa or media, or from foreign
objects. Developmental defects, which are uncommon, may include atre-
sia of the tympanum or ossicles, fusion of the ossicles, or collapse of
the ear canal from cartilaginous weakness or incomplete development.
Conduction deafness may be partial or complete and may be reduced by
intervention in some cases. Clearance by the body of the mucopurulent
discharge and detritus from otitis media may require weeks to months
after termination of the infection; hence, recovery of auditory function
is delayed. Hereditary forms of conduction deafness have not been
identified in domestic species, but the appearance of such a disorder
from a spontaneous genetic defect is possible.

Congenital Acquired Sensorineural Deafness

Congenital acquired sensorineural deafness, which is uncommon,

can result from in utero or perinatal exposure to ototoxic compounds
such as maternal treatment with aminoglycoside antibiotics,Z1 in utero
or perinatal otitis or meningitis, anoxia, or even trauma. Breeders with
animals belonging to breeds with a high prevalence of hereditary senso-
rineural deafness may suggest acquired causes of deafness rather than
confront the breeding and other implications of the presence of a heredi-
tary disorder. Sensorineural deafness, whether hereditary or acquired, is
the consequence of cochlear hair cell loss through primary or secondary
mechanisms (see below).
 CONGENITAL DEAFNESS 897

Congenital Hereditary Sensorineural Deafness

Congenital hereditary sensorineural deafness is usually seen in

breeds of dogs and cats with white pigmentation. In the small number
of canine breeds where it is not associated with white pigmentation
(Doberman Pinscher and other breeds not carrying piebald or merle
genes)/3 deafness results from the type of hair cell loss that is a primary
event with unknown cause. In breeds of dogs carrying piebald or merle
genes and in breeds of cats carrying the white gene, the hair cell loss is
secondary to degeneration of the cochlear blood supply. Figure 1 shows
a cross section of one turn of the cochlea, demonstrating the separation
of the cochlea into three parallel ducts: the scala vestibuli, the scala
media (or cochlear duct), and the scala tympani which joins at the apex
of the cochlea with the scala vestibuli. The outer margin of the scala
media is covered by a vascular bed, the stria vascularis. The stria is
responsible for secretion of endocochlear fluid and maintenance of its
high K + concentration which is essential to sound transduction by the
sensory hair cells. In pigment-associated hereditary deafness, this vascu-

Stria vascularis

Spiral prominence

Basilar
membrane

Figure 1. Cross-section of the cochlea. The organ of Corti rests on the basilar membrane,
with its hair cell cilia embedded in the tectorial membrane. The stria vascularis on the outer
margin of the scala media secretes the endocochlear fluid of the scala media and maintains
a high K+ concentration essential to sound transduction by the hair cells. Sensorineural
deafness can result from primary or secondary loss of cochlear hair cells. (From Bloom W,
Fawcett DW: A Textbook of Histology, ed 10. Philadelphia, WB Saunders, 1975; with
permission.)
898 STRAIN

Breeds of dogs with reported congenital deafness

Akita Italian Greyhound
American-Canadian Shepherd Jack Russell Terrier
American Eskimo Kuvasz
American Staffordshire Terrier Labrador Retriever
Australian Cattle Dog Maltese
Australian Shepherd Miniature Pinscher
Beagle Miniature Poodle
Bichon Frise mongrel
Border Collie Norwegian Dunkerhound
Borzoi Nova Scotia Duck Tolling.Retriever
Boston Terrier Old English Sheepdog
Boxer Papillon
Bulldog Pit Bull Terrier
Bull Terrier Pointer
Cardigan Welsh Corgi Puli
Catahoula Leopard Dog Rhodesian Ridgeback
Cavalier King Charles Spaniel Rottweiler
Chihuahua Saint Bernard
Chow Chow Schnauzer
Cocker Spaniel Scottish Terrier
Collie Sealyham Terrier
Dalmatian Shetland Sheepdog
Dappled Dachshund Shropshire Terrier
Doberman Pinscher Siberian Husky
Dogo Argentino Soft Coated Wheaten Terrier
English Bulldog Springer Spaniel
English Cocker Spaniel Sussex Spaniel
English Setter Tibetan Spaniel
Foxhound Tibetan Terrier
Fox Terrier Toy Poodle
French Bulldog Walker American Foxhound
German Shepherd West Highland White Terrier
Great Dane Whippet
Great Pyrenees Yorkshire Terrier
lbizan Hound

lar bed degenerates, resulting in secondary loss of hair cells and deaf-
ness. The cause for the strial degeneration is unknown, but histological
studies have demonstrated an absence of strial melanocytes, whose
presence or postnatal development is suppressed by piebald or merle
genes. The function of melanocytes in the stria is unknown, but they
appear to be critical to maintenance of elevated K+ levels in the scala
media and survival of the stria. Whether hair cell death is from primary
or secondary mechanisms, the loss is permanent, as mammals a:re unable
to regenerate cochlear neuronal tissue.
In the Dalmatian, postnatal auditory function development has been
 CONGENITAL DEAFNESS 899

shown to proceed normally up until 3 weeks of age, at which point the

strial degeneration produces rapid loss of hair cell function. 6 A similar
time course is likely in other breeds of dogs and cats with pigment-
associated deafness, but it has not been studied. Likewise, the time
course of congenital deafness in canine breeds not associated with white
pigmentation has not been documented, but it is likely that deafness is
present at birth or shortly thereafter. As a consequence of the docu-
mented 3 to 4 weeks of age at which time deafness appears, hearing
testing, as described below, is typically not performed until an animal
reaches at least 5 weeks of age.
A wide variety of breeds of dogs have been reported . to have
congenital deafness (see list on opposite page); not all of these cases of
deafness have been shown to result from hereditary causes. The breeds
for which the prevalence is known to be high are highlighted in bold,
although similar high rates may occur in other breeds that do not yet
routinely receive testing. Prevalence rates measured by the author are
shown in Table 1 for the canine breeds most often presented for hearing
testing services. The highest rates are seen in Dalmatians, of which 30%
are deaf in one or both ears; rates in other strongly affected breeds range
from 8% to 20%. Typically, there are two to three unilaterally deaf
animals for every bilaterally deaf animal. In breeds with white versus
nonwhite phenotypes (Bull Terrier, English Cocker Spaniel), there is a
clear increased prevalence in the white phenotype. Prevalence rates for
pure feline breeds have not been measured but are highest for the breeds
carrying the white gene (see list on this page), especially in cats with
blue eyes. Deafness in 256 mixed-breed white cats was reported as being
12% unilateral and 38% bilateral, for a total of 50% of cats being affected
(reviewed in Delack3). The prevalence of deafness increases as the num-
ber of blue eyes increases from zero to two, but not all blue-eyed white

Breeds of cats carrying the white coat pigment gene

White
European White
Foreign White
White Cornish Rex
White Devon Rex
White Manx
White Persian
White Scottish Fold
White Turkish Angora
White American Wirehair
White American Shorthair
White British Shorthair
White Exotic Shorthair
White Oriental Shorthair
1.0
g

Table 1. BREED-SPECIFIC DEAFNESS PREVALENCE IN DOGS

Breed Dogs Tested Bilaterally Hearing Unilaterally Deaf Bilaterally Deaf Total Deaf
Dalmatian 5009 70.2% (3510) 22.0% (1100) 8.0% (399) 30.0% (1499)
Bull Terrier 573 89.0% (510) 9.9% (57) 1.0% (6) 11.0% (63)
White 299 80.9% (242) 17.1% (51) 2.0% (6) 19.1% (57)
Colored 272 97.8% (266) 2.2% (6) 0.0% (0) 2.2% (6)
English Setter 530 85.7% (454) 12.1% (64) 2.3% (12) 14.3% (76)
English Cocker Spaniel 828 92.8% (768) 6.2% (51) 1.1% (9) 7.2% (60)
Particolor 794 92.6% (735) 6.5% (50) 1.1% (9) 7.4% (59)
Solid color 34 97.1% (33) 2.9% (1) 0.0% (0) 2.9% (1)
Australian Cattle Dog 238 87.4% (208) 10.5% (25) 2.1% (5) 12.6% (30)
Catahoula Leopard Dog 48 31.3% (15) 27.1% (13) 41.7% (20) 68.8% (33)
Jack Russell Terrier 47 80.9% (38) 8.5% (4) 10.6% (5) 19.1% (9)
 CONGENITAL DEAFNESS 901

cats are deaf. The prevalence of deafness (unilateral and bilateral) in

mixed-breed white cats was 17%, 40%, and 85% for zero, one, and two
blue eyes, respectively. 9

GENETICS OF DEAFNESS

Pigment-associated deafness has been reported since the last cen-

tury, 1, s, s-1o, 12 but the hereditary mechanisms are not yet fully understood.
The merle gene, which is responsible for the pattern of dark and light
hair in breeds such as the Collie and Shetland Sheepdog, is a simple
autosomal dominant gene. 7 Dogs that are homozygous for the merle
gene are usually deaf and frequently are solid white, blind, and sterile,
Heterozygotes have an increasing tendency to be deaf as the percentage
of white in the coat increases, Although the merle gene is a dominant
gene, the deafness associated with it is not inherited as a simple domi-
nant (or recessive) disorder.
The piebald and extreme piebald genes, which are responsible for
white hair in nonmerle gene breeds of dogs, are simple autosomal
recessive genes. 7 As a result, in breeds with major white areas on the
body, the animals are homozygous. An example is the Dalmatian, all of
which are homozygous for the extreme piebald gene. The underlying
coat color of black (dominant) or liver (recessive) is covered with white
by the extreme piebald gene, and spots are produced through the white
by the dominant ticking gene. The lightness or heaviness of the spotting
is thus controlled by the ticking gene and not the extreme piebald gene,
and it is not a factor in the prevalence of deafness. 19 Based on studies of
the Dalmatian, deafness in breeds carrying the piebald genes is neither
simple recessive nor dominant. Breeding of bilaterally hearing parents
routinely produces deaf offspring; thus, the mechanism is not simple
dominant. Breeding of two bilaterally deaf parents produces both deaf
and hearing offspring; the latter would not occur if the defect were
simple recessive and both parents were homozygous. There is no evi-
dence for X-linked or mitochondrial hereditary mechanisms. As a conse-
quence, inheritance of deafness associated with the piebald genes must
be polygenic or must involve incomplete expression or penetrance, or
some combination. Molecular genetic studies of deafness disorders in
humans and mice suggest that this deafness may result from a defect in
a gene responsible for regulation of the piebald genes such as one of the
homeobox class of genes. Evidence for this comes from further findings
in Dalmatians. A patch in Dalmatians is a large black or liver area
present at birth when the puppy is otherwise solid white; patches are
disallowed in the Dalmatian breed standard. The patch appears to result
from weak expression of the extreme piebald gene, resulting in a failure
to cover the underlying coat color; patched Dalmatians are statistically
less likely to be deaf than unpatched animals. 19 When the extreme
piebald gene is strongly expressed, brown pigment is suppressed in the
iris, resulting in blue eyes (and frequently an absence of pigment in1he
902 S1RAIN

tapetum lucidum), and melanocytes are suppressed in the cochlear stria

vascularis, resulting in deafness. Blue-eyed Dalmatians are statistically
more likely to be deaf than brown-eyed animals. 19 The blue eye is
permitted in the breed standard in the United States but not in Canada,
Mexico, or Europe. The prevalence of deafness is lower in Europe
(combined unilateral and bilateral deafness is 21% in the United King-
dom and 18% in Holland compared with 30% in the United States), and
breeding away from blue eyes was shown to reduce deafness in Nor-
way.4 It is not known if differences exist for deafness associated with the
two different piebald genes.
Deafness in the Doberman Pinscher, which is accompanied by tran-
sient vestibular dysfunction, is transmitted by a simple recessive mecha-
nism.23 -
Pigment-associated congenital hereditary sensorineural deafness in
the cat is linked to the white gene, which is dominant over color and is
unrelated to albinismY On occasion, these cats have a head spot and
usually have one or two blue eyes. Although the white gene is dominant,
not all carriers are deaf; thus, deafness is not simply inherited.

BEHAVIORAL INDICATORS OF DEAFNESS

Newborn puppies and kittens with undeveloped auditory and vi-

sual function use other sensory cues for their feeding,· elimination, and
locomotion behaviors. As auditory development proceeds, they can de-
tect loud noises, despite the unopened ear canal. Breeders relying on
this for home testing may find themselves to have been in error at a
later date. Behavioral testing of hearing after opening of the canal relies
on the detection of a response to sound stimuli in the absence of other
detectable sensory signals. These noises should be produced outside of
the visual fields, avoiding visual cues, vibratory cues, touch, and air
movements. Behavioral testing has limited value; animal responses rap-
idly adapt even when hearing is present, stressed animals with intact
hearing may fail to respond, and unilateral deafness cannot be detected.
In unilaterally deaf animals, the only behavioral sign of deafness is a
difficulty in localizing the source of a sound, and many animals adapt
to that also. Behavioral deafness detection with young animals in the
home is difficult, as the deaf young cue off the behavior of their lit-
termates. A puppy or kitten that does not awaken in response to a loud
noise is almost certainly bilaterally deaf, but the unilaterally deaf animal
cannot be detected with any reliability. As a consequence, behavioral
hearing assessment of animals in the clinic or home is of limited reliabil-
ity, and electrodiagnostic tests are used for objective assessment.

ELECTRODIAGNOSIS OF DEAFNESS

The most widely used electrodiagnostic test of hearing is the brain

stem auditory evoked response (BAER), also known as the brain stem
 CONGENITAL DEAFNESS 903

auditory evoked potential or the auditory brain stem response. This test
was first used in veterinary research applications in the 1970s and in
clinical veterinary applications in the early 1980s. The BAER detects
electrical activity in the cochlea and auditory pathways in the brain in
much the same way that an electrocardiogram detects electrical activity
in the heart. 17 The response waveform consists of a series of peaks
labeled with Roman numerals: peak I is produced by the cochlea and
auditory nerve, and later peaks are produced within the brain. The
response from an ear that is deaf is an essentially flat line. In the sample
recordings in Figure 2, the Dalmatian puppy in tracing A could hear in
the tested ear, although the Dalmatian puppy in tracing B was deaf in
the tested ear, with an essentially flat response. Because the response
amplitude is quite small, it is necessary to average the responses to
multiple stimuli (clicks) to unmask them from the other unrelated electri-
cal activity that is also present on the scalp (e.g., electroencephalographic
activity, muscle activity).
The response is collected with a special computer through small
subdermal needle electrodes: one is placed in front of each ear, one is
placed at the top of the head, and a ground electrode is placed either
between and behind the eyes or on the neck. It is rare for a dog to show
any evidence of pain from the placement of the electrodes-if anything,
the dog objects to the gentle restraint and the presence of wires hanging
in front of its face. A stimulus click (air-conducted) produced by the

A
~
~.
~ B
~
Q)
-c

tE
<(

0 2 4 6 8 10

Latency(ms)

Figure 2. Brainstem auditory evoked responses (BAER) recorded from puppies; cat BAER
appear similar. A, A BAER from a normal dog in response to an air-conducted click
stimulus. Peak I in the response is generated by the cochlea and Vlllth cranial nerve, while
later peaks are generated in brainstem structures. B, A BAER from a deaf Dalmatian. C, A
BAER from a normal dog in response to a bone-conducted click stimulus.
904 STRAIN

computer is directed into the ear with a foam insert earphone. Each ear
is tested individually, and the test is usually completed in 10 to 15
minutes. Sedation or anesthesia is unnecessary unless the dog becomes
extremely agitated, which can usually be avoided with patient and
gentle handling. Sedation or anesthesia does not materially affect the
BAER.
The click stimulus used contains most of the audible frequencies of
the dog and cat, with the exception of the highest perceived frequencies.
Accordingly, the BAER is a frequency nonspecific test that is more useful
for detecting the presence or total absence of auditory function without .
quantifying hearing loss in decibels. Assessment of the normalcy of a
response is based on identification of the presence of peak I within a
narrow expected time frame (which varies based on the equipment
used) and the presence of the expected pattern of peaks. With progres-
sive hearing loss, there is a reduction in the amplitude of the BAER
peaks and an increase in peak latencies; thus, a subjective assessment of
partial hearing loss can be made but not quantified, and differing de-
grees of loss in different frequency ranges cannot be determined. Diagno-
sis of partial hearing loss based on the BAER is done only with great
caution, as a number of technical factors can affect peak amplitude and
latency in subjects with normal hearingP Fortunately, partial hearing
loss is rare in puppies or kittens.
The BAER demonstrates maturational changes.22 Because the greater
portion of the BAER originates in the brain stem, there is less postnatal
development than is seen in tests of other sensory modalities; however,
postnatal development is greater in altricial species like the dog and cat
than in precocial species like the horse and cow. Full maturation of the
BAER occurs by 40 days in the dog and the cat. The BAER can be
recorded in response to loud stimuli prior to the opening of the ear
canal, but this is not of use as it predates the age at which deafness is
manifested.
In some circumstances, it is useful to be able to differentiate between
sensorineural and conductive deafness, as this can affect breeding deci-
sions and whether a young animal is placed in a show home or a pet
home. When a BAER indicates deafness in an animal in which conduc-
tion deafness might be suspected (i.e., long-eared breed, recent ear
infection), the test is repeated with a mechanical transducer that trans-
mits the stimulus click as a vibration through bone rather than through
air conduction. 20 Because the cochlea is imbedded in bone, the bone-
conducted BAER bypasses the outer and middle ears, the sites of con-
duction blockade, and directly activates the cochlea. The response ap-
pearance is the same as an air-conducted BAER, but the peaks occur at
a shorter latency due to the shorter path traversed by the stimulus (see
Fig. 2).
A limited availability of BAER testing sites blocks some potential
users from access, but the number of test locations is increasing beyond
the original veterinary school sites. The equipment cost of approximately
$20,000 and an absence of formal veterinary training programs outside of
 CONGENITAL DEAFNESS 905

neurology residencies have impeded ready access. A listing of available

national and international sites is maintained at the author's web site/6
which is also a resource of additional information on deafness.

CLIENT COUNSELING ISSUES

Advice to clients faced with a deaf puppy or kitten varies based on

breed, animal age, home environment, unilateral versus bilateral deaf-
ness, and other factors. Bilaterally deaf animals present a variety of
liabilities and emotional land mines, more so for dogs than for cats. Deaf
animals are at risk of injury or death from undetected dangers such as
motor vehicles. When startled, they may reflexly bite, which is a special
concern around infants and toddlers. Anxious or aggressive personalities
may develop in deaf dogs from constantly being subjected to startle,
and familiar family members and friends may be attacked without
warning or cause. Not all deaf dogs develop these problems, and no
data exist on prevalence rates for such events, but there is no way to
predict which animals may or may not have these experiences. Based on
inherent temperament differences, there may also be variations between
breeds in the likelihood of such problems. Bilaterally deaf dogs are quite
difficult to raise and train; as a result, they often end up in animal
control shelters. From there, they may be reclaimed by breed rescue
groups, after which the cycle may begin again. House cats present
fewer problems, but outdoor cats may fall victim to motor vehicles. An
emotional cost is invariably paid by the owners of those animals that
cannot cope or adjust to their disability both from the perspective of
management and from that of facing the decision as to whether to
euthanatize an animal with which an emotional attachment has formed.
Unilateral deafness does not pose such problems.
Because of the many problems associated with bilaterally deaf dogs
and the surplus of available puppies, the Dalmatian Club of America
has an official position calling for the euthanasia of deaf puppies, with
the emphasis on breeders rather than on new owners. A similar senti-
ment is held by the official organizations of other breeds with high
deafness prevalence rates, but these groups have not adopted such an
official policy. This position has generated considerable controversy
within the purebred dog community, the general dog-owning commu-
nity, the veterinary community, and the human deaf community, but it
is probably appropriate when divorced from emotional considerations.
Resources exist for those owners opting to keep a deaf dog. Books
have been written on living with a deaf dog/ including directions for
training deaf dogs to respond to American Sign Language signs, and
web pages have been posted with information and support content.
Deaf dogs and cats learn to respond to flashing porch lights and vibrat-
ing collars 16 and can cue off the behavior of other animals in the house-
hold. In such circumstances, the owner should be instructed in protecting
the animal from the inherent dangers associated with deafness: both
906 STRAIN

dangers to the deaf animal and dangers to people around the deaf ani-
mal.
Genetic counseling for owners of deaf dogs and cats cannot be
presented with total assurance because of the incomplete knowledge of
mechanisms of deafness inheritance. It can be stated that deaf animals
.in breeds with a high known prevalence of either unilateral or bilateral
deafness should not be bred; unilaterally deaf animals have the genetic
defect but have one ear spared. Over the long run, such breedings have
the probability of producing more deaf animals. In addition, it may not
be advisable to breed to animals from litters that have a high percentage
of deaf animals or from lines with a history of producing high percent-
ages of deaf animals. The most conservative approach avoids any possi-
ble introduction of defective genes but may be difficult in certain breeds.
A deaf animal from one of these breeds must be assumed to have
hereditary deafness instead of acquired deafness unless the clinical his-
tory convincingly indicates otherwise. Because of the association ob-
served between deafness and blue eyes in the Dalmatian, it is also
advisable not to breed blue-eyed dogs from those breeds in which the
blue eye is not a standard part of the breed phenotype.
If a deaf animal is presented from a breed with no history of notable
numbers of deaf animals, the guidelines for advice are murkier. The most
conservative approach is not to breed any affected animal, especially if
the breed is one carrying piebald or merle genes, unless the clinical
history suggests a likely acquired cause such as otitis or drug ototoxicity.
Even these latter cases are not without risk unless precedent documenta-
tion of normal hearing exists.
In an effort to promote research to reduce deafness and provide
data for potential breeders, several national breed organizations in this
country have set up hearing registries either managed by the breed
organization (e.g., English Setter Association of America) or by a con-
tracted second party (e.g., Dalmatian Club of America managed by the
Institute for Genetic Disease Control in Animals, Bull Terrier Club of
America managed by the Orthopedic Foundation of America). The first
registry is closed, although the second is open, and the third offers the
option of being either closed or open to qualified members of the public.
Current canine deafness research focuses on determining mechanisms of
inheritance from extended pedigrees and on identifying the responsible
defective genes using molecular biological techniques.

References

1. Anderson H, Henricson B, Lundquist P-G, et al: Genetic hearing impairment in the

Dalmatian dog. Acta Otolaryngol Suppl (Stockh) 23:1, 1968
2. Becker SC: Living With a Deaf Dog. Cincinnati, Susan Cope Becker, 1998
3. Delack JB: Hereditary deafness in the white cat. Compend Contin Educ Pract Vet
6:609, 1984
4. Greibrokk T: Hereditary deafness in the Dalmatian: Relationship to eye and coat color.
JAm Anim Hosp Assoc 30:170, 1994
 CONGENITAL DEAFNESS 907

5. Hudson WR, Ruben RJ: Hereditary deafness in the Dalmatian dog. Arch Otolaryngol
75:213, 1962
6. Johnsson LG, Hawkins JE, Jr, Muraski AA, et al: Vascular anatomy and pathology of
the cochlea in Dalmatian dogs. In de Lorenzo AJD (ed): Vascular Disorders and
Hearing Defects. Baltimore, University Park Press, 1973, p 249
7. Little CC: The Inheritance of Coat Color in Dogs. New York, Howell, 1957
8. Lurie MH: The membranous labyrinth in the congenitally deaf collie and Dalmatian
dog. Laryngoscope 58:279, 1948
9. Mair IWS: Hereditary deafness in the white cat. Acta Otolaryngol Suppl (Stockh)
314:1, 1973
10. Mair IWS: Hereditary deafness in the Dalmatian dog. Archives of Otorhinolaryngology
212:1, 1976
11. Pujol R, Hilding D: Anatomy and physiology of the onset of auditory function. Acta
Otolaryngol (Stockh) 76:1, 1973
12. Rawitz B: Gehororgan und Gehirn eines Weissen Hundes mit blauen Augen. Morpho-
logische Arbeiten 6:545, 1896
13. Searle AG: Comparative Genetics of Coat Color in Mammals. London, Logos Press,
1968
14. Strain GM: Aetiology, prevalence, and diagnosis of deafness in dogs and cats. Br Vet J
152:17, 1996
15. Strain GM: Congenital deafness in dogs and cats. Compend Contin Educ Pract Vet
13:245, 1991
16. Strain GM: Deafness in dogs and cats. Located at: http:/ /www.lsu.edu/guests/senate/
public_html/ deaf.htrn
17. Strain GM: Electrophysiological assessment of auditory function. In Proceedings of the
15th American College of Veterinary Internal Medicine Forum, Orlando, FL, 1997, p 15
18. Strain GM, Jackson RM, Tedford BL: Postnatal development of the visual-evoked
potential in dogs. Am J Vet Res 52:231, 1991
19. Strain GM, Kearney MT, Gignac IJ, et al: Brainstem auditory evoked potential assess-
ment of congenital deafness in Dalmatians: Associations with phenotypic markers. J
Vet Intern Med 6:175, 1992
20. Strain GM, Green KD, Twedt AC, et al: Brain stem auditory evoked potentials from
bone stimulation in dogs. Am J Vet Res 54:1817, 1993
21. Strain GM, Merchant SR, Neer TM, et al: Ototoxicity assessment of a gentamicin
sulfate otic preparation in dogs. Am J Vet Res 56:532, 1995
22. Strain GM, Tedford L, Jackson RM: Postnatal development of the brainstem auditory-
evoked potential in dogs. Am J Vet Res 52:410, 1994
23. Wilkes MK, Palmer AC: Congenital deafness and vestibular deficit in the doberman. J
Small Anim Pract 33:218, 1992

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