Congenital Deafness in White Cats

Wilson, T. G.; Kane, Francis J.

doi:10.3109/00016485909129195

Cited by 51 publications

(10 citation statements)

References 6 publications

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“…That is, cell changes in various centers in the central auditory pathway have provided evidence for transneuronal pathology. There is a marked but variable reduction in number of spiral ganglion neurons (Wilson and Kane, 1959;Rebillard et al, 1981a,b), and additional alterations are expressed higher in the auditory neuraxis to include the cochlear nucleus (West and Harrison, 1973;Larsen and Kirchhoff, 1992;Saada et al, 1996;Huchton et al, 1997) and superior olivary complex (Schwartz and Higa, 1982). Compared to normal-hearing cats, there is a 38-50% reduction in the size of spherical bushy cells in the anteroventral cochlear nucleus and a 31% reduction in the size of pyramidal cells in the dorsal cochlear nucleus (West and Harrison, 1973;Larsen and Kirchhoff, 1992;Saada et al, 1996).…”

mentioning

confidence: 99%

Single unit recordings in the auditory nerve of congenitally deaf white cats: Morphological correlates in the cochlea and cochlear nucleus

et al. 1998

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It is well known that experimentally induced cochlear damage produces structural, physiological, and biochemical alterations in neurons of the cochlear nucleus. In contrast, much less is known with respect to the naturally occurring cochlear pathology presented by congenital deafness. The present study attempts to relate organ of Corti structure and auditory nerve activity to the morphology of primary synaptic endings in the cochlear nucleus of congenitally deaf white cats. Our observations reveal that the amount of sound-evoked spike activity in auditory nerve fibers influences terminal morphology and synaptic structure in the anteroventral cochlear nucleus. Some white cats had no hearing. They exhibited severely reduced spontaneous activity and no sound-evoked activity in auditory nerve fibers. They had no recognizable organ of Corti, presented >90% loss of spiral ganglion cells, and displayed marked structural abnormalities of endbulbs of Held and their synapses. Other white cats had partial hearing and possessed auditory nerve fibers with a wide range of spontaneous activity but elevated sound-evoked thresholds (60-70 dB SPL). They also exhibited obvious abnormalities in the tectorial membrane, supporting cells, and Reissner's membrane throughout the cochlear duct and had complete inner and outer hair cell loss in the base. The spatial distribution of spiral ganglion cell loss correlated with the pattern of hair cell loss. Primary neurons of hearing-impaired cats displayed structural abnormalities of their endbulbs and synapses in the cochlear nucleus which were intermediate in form compared to normal and totally deaf cats. Changes in endbulb structure appear to correspond to relative levels of deafness. These data suggest that endbulb structure is significantly influenced by sound-evoked auditory nerve activity.

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confidence: 99%

Single unit recordings in the auditory nerve of congenitally deaf white cats: Morphological correlates in the cochlea and cochlear nucleus

et al. 1998

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“…A discriminant analysis revealed that the inner intercanthal distance, philtrum length, lower facial height, and nasal bone length were discriminating parameters of WS type I 30 . WS is caused by a gene alteration, mainly by mutations 13 , 31–92 …”

Section: Clinical Manifestationsmentioning

confidence: 99%

“…Piebaldism has also been reported in association with neurologic impairment such as megacolon (HD), 70 as well as WS type IV. Many animals have been observed with clinical manifestations of WS, including cats, 71 , 72 dogs, 73 horses, 27 and mice 74 …”

Section: Differential Diagnosismentioning

confidence: 99%

Waardenburg syndrome

Dourmishev¹,

Dourmishev²,

Schwartz³

et al. 1999

Int J Dermatology

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“…Possible animal models are known. The association of deafness with hypopigmentation in animals has been known since 1769 [Wolf, 19421. It has been reported mainly in cats, dogs, rabbits, mice, cattle, and horses [Wilson and Kane, 1959;Pantke and Cohen, 19711. The best known models are the Ankora cats which are white, blue-eyed, and deaf.…”

Section: Animal Modelsmentioning

confidence: 99%

Waardenburg I syndrome: A clinical and genetic study of two large Brazilian kindreds, and literature review

da-Silva

1991

Am. J. Med. Genet.

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Two large kindreds with Waardenburg I syndrome are described. The total number of affected individuals is 73. The major manifestations are telecanthus (the only constant anomaly in all cases), prominent nasal root, round or square tip of nose, hypoplastic alae, smooth philtrum, bushy eyebrows with synophrys, sensorineural deafness, heterochromia or hypoisochromia iridis, hypopigmented ocular fundus, white forelock, premature greying, and hypopigmented skin lesions. These and other aspects of the syndrome, associated findings, frequency, genetic heterogeneity, pathogenesis, animal models, and gene linkage and mapping are reviewed briefly.

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Congenital Deafness in White Cats

Cited by 51 publications

References 6 publications

Single unit recordings in the auditory nerve of congenitally deaf white cats: Morphological correlates in the cochlea and cochlear nucleus

Single unit recordings in the auditory nerve of congenitally deaf white cats: Morphological correlates in the cochlea and cochlear nucleus

Waardenburg syndrome

Waardenburg I syndrome: A clinical and genetic study of two large Brazilian kindreds, and literature review

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