Congenital Sensorineural Deafness In Cats
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Congenital sensorineural deafness occurs commonly in domestic
cat The cat (''Felis catus''), also referred to as the domestic cat or house cat, is a small domesticated carnivorous mammal. It is the only domesticated species of the family Felidae. Advances in archaeology and genetics have shown that the ...
s with a white coat. It is a
congenital deafness Hearing loss is a partial or total inability to hear. Hearing loss may be present at birth or acquired at any time afterwards. Hearing loss may occur in one or both ears. In children, hearing problems can affect the ability to acquire spok ...
caused by a degeneration of the
inner ear The inner ear (internal ear, auris interna) is the innermost part of the vertebrate ear. In vertebrates, the inner ear is mainly responsible for sound detection and balance. In mammals, it consists of the bony labyrinth, a hollow cavity in the ...
. Deafness is far more common in white cats than in those with other coat colours.


Occurrence

Domesticated cats with blue eyes and white coats are often completely deaf.
Charles Darwin Charles Robert Darwin ( ; 12 February 1809 – 19 April 1882) was an English Natural history#Before 1900, naturalist, geologist, and biologist, widely known for his contributions to evolutionary biology. His proposition that all speci ...
mentions this phenomenon in his book, ''
On the Origin of Species ''On the Origin of Species'' (or, more completely, ''On the Origin of Species by Means of Natural Selection, or the Preservation of Favoured Races in the Struggle for Life'')The book's full original title was ''On the Origin of Species by M ...
'', to explain correlated variation. Deafness can occur in white cats with yellow, green or blue irises, although it is mostly likely in white cats with blue irises. In white cats with one blue eye and one eye of a different color ( odd-eyed cats), deafness is more likely to affect the ear on the blue-eyed side. Approximately 50% of white cats have one or two blue eyes. According to the ASPCA Complete Guide to Cats, "17 to 20 percent of white cats with non-blue eyes are deaf; 40 percent of "odd-eyed" white cats with one blue eye are deaf; and 65 to 85 percent of blue-eyed white cats are deaf." In one 1997 study of white cats, 72% of the animals were found to be totally deaf. The entire
organ of Corti The organ of Corti, or spiral organ, is the receptor organ for hearing and is located in the mammalian cochlea. This highly varied strip of epithelial cells allows for transduction of auditory signals into nerve impulses' action potential. Trans ...
in the
cochlea The cochlea is the part of the inner ear involved in hearing. It is a spiral-shaped cavity in the bony labyrinth, in humans making 2.75 turns around its axis, the modiolus (cochlea), modiolus. A core component of the cochlea is the organ of Cort ...
was found to have degenerated in the first few weeks after birth; however, even during these weeks no brain stem responses could be evoked by auditory stimuli, suggesting that these animals had never experienced any auditory sensations. It was found that some months after the organ of Corti had degenerated, the
spiral ganglion The spiral (cochlear) ganglion is a group of neuron cell bodies in the modiolus, the conical central axis of the cochlea. These bipolar neurons innervate the hair cells of the organ of Corti. They project their axons to the ventral and dorsa ...
of the cochlea also began to degenerate. BAER-testing ( Brainstem Auditory Evoked Response) is used to test deafness in cats.


Genetics

Although few studies have been done to link this to genes known to be involved in human
Waardenburg syndrome Waardenburg syndrome is a group of rare genetic conditions characterised by at least some degree of congenital hearing loss and pigmentation deficiencies, which can include bright blue eyes (or Heterochromia iridum, one blue eye and one brown ey ...
, a syndrome of hearing loss and depigmentation caused by a genetic disruption to
neural crest The neural crest is a ridge-like structure that is formed transiently between the epidermal ectoderm and neural plate during vertebrate development. Neural crest cells originate from this structure through the epithelial-mesenchymal transition, ...
cell development, such a disruption would lead to this presentation in cats as well. Waardenburg syndrome type 2A (caused by a mutation in '' MITF'') has been found in many other small mammals including dogs, minks and mice, and they all display at least patchy white depigmentation and some degeneration of the cochlea and
saccule The saccule (Latin: sacculus) is a bed of sensory cells in the inner ear that detects linear acceleration and head tilting in the vertical plane, and converts these vibrations into electrical impulses to be interpreted by the brain. When the he ...
, as in deaf white cats. A major gene that causes a cat to have a white coat is a dominant masking gene, an
allele An allele is a variant of the sequence of nucleotides at a particular location, or Locus (genetics), locus, on a DNA molecule. Alleles can differ at a single position through Single-nucleotide polymorphism, single nucleotide polymorphisms (SNP), ...
of ''KIT'' which suppresses pigmentation and hearing. The cat would have an underlying coat colour and pattern, but when the dominant white gene is present, that pattern will not be expressed, and the cat will be deaf. A cat that is homozygous (WW) or heterozygous (Ww) for this gene will have a white coat despite the underlying pattern/colour. A cat that lacks this dominant masking gene (ww) will exhibit a coat colour/pattern. ''KIT'' mutations have also led to patchy depigmentation and different coloured irises in humans, and ''KIT'' has been found to increase ''MITF'' expression, the gene involved in human Waardenburg syndrome type 2A.


Non-deaf blue-eyed white cats

The established link between deafness and blue eyes is found in the link between deafness, blue eyes, and solid white coats. However, it is a common misconception that all white cats with blue eyes are deaf. It is possible to have a cat with a white coat without this gene as an extreme form of white spotting, though this is rare; some small non-white patch usually remains. Furthermore, there are multiple genes responsible for blue eyes, and several of these genes are not linked to masking, white coats or deafness, such as the dominant blue eye (DBE) gene carried by Ojos Azules. Another example is a mutation in the TYR gene causing
point coloration Points are specific areas of an animal coat that are colored differently from the main body colorations. Point coloration may be represented by a pale body color and relatively darker extremities, such as face, ears, feet, tail, and external sex ...
, a form of partial albinism seen in Siamese cats and related breeds. Foreign White cats, a white variation of Siamese, are also not prone to deafness.


In popular culture

* The character Snowkit in the Warriors series book '' A Dangerous Path'' has congenital sensorineural deafness. His condition leads to his death as he fails to hear his mother's warning of the appearance of a predator.


See also

*
Cat coat genetics Cat coat genetics determine the coloration, pattern, length, and texture of feline fur. The variations among cat coats are physical properties and should not be confused with cat breeds. A cat may display the coat of a certain breed without actu ...
*
Merle (dog coat) Merle is a genetic pattern in a dog's coat and alleles of the PMEL gene. It results in different colors and patterns and can affect any coats. The allele creates mottled patches of color in a solid or piebald coat, blue or odd-colored eye ...
*
Pleiotropy Pleiotropy () is a condition in which a single gene or genetic variant influences multiple phenotypic traits. A gene that has such multiple effects is referred to as a ''pleiotropic gene''. Mutations in pleiotropic genes can impact several trait ...
* Van cat *
Waardenburg syndrome Waardenburg syndrome is a group of rare genetic conditions characterised by at least some degree of congenital hearing loss and pigmentation deficiencies, which can include bright blue eyes (or Heterochromia iridum, one blue eye and one brown ey ...


References

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