Hereditary deafness occurring in cd/1 mice

Shone, G. R.; Raphael, Yehoash; Miller, Josef M.

doi:10.1016/0378-5955(91)90084-m

Cited by 43 publications

(34 citation statements)

References 7 publications

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“…A complete degeneration of the organ of Corti was also observed at the basal turn of old CD-1 mice (Fig. 6F), as reported previously (Shone et al 1991). The whole organ of Corti was replaced by simple epithelial cells lacking any distinguishable morphology of hair cells.…”

Section: Cd-1 Mouse Strainsupporting

confidence: 88%

Section: Introductionmentioning

confidence: 99%

“…The CD-1 mouse strain is also a special example of AHL, exhibiting early sensorineural hearing loss (Le Calvez et al 1998a;Shone et al 1991) progressing from 3 weeks after birth. Concurrent with the hearing loss is a progressive loss of both inner and outer hair cells (Shone et al 1991). CD-1 mice older than 4 months consistently had extensive hair cell loss throughout the cochlea: from 80% to 100% loss of outer hair cells (OHCs) and 7-15% loss of inner hair cells (IHCs) (Le Calvez et al 1998a, 1998bShone et al 1991).…”

Section: Introductionmentioning

confidence: 99%

“…Concurrent with the hearing loss is a progressive loss of both inner and outer hair cells (Shone et al 1991). CD-1 mice older than 4 months consistently had extensive hair cell loss throughout the cochlea: from 80% to 100% loss of outer hair cells (OHCs) and 7-15% loss of inner hair cells (IHCs) (Le Calvez et al 1998a, 1998bShone et al 1991). In 6-month-old CD-1 mice, no hair cells were present in the basal cochlear turn and the basilar membrane was covered with a simple epithelium.…”

Section: Introductionmentioning

confidence: 99%

“…In 6-month-old CD-1 mice, no hair cells were present in the basal cochlear turn and the basilar membrane was covered with a simple epithelium. In the apical turn, about 50% of the IHCs and 80% of the OHCs were missing (Shone et al 1991).…”

Section: Introductionmentioning

confidence: 99%

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Age-Related Changes in Cochlear Endolymphatic Potassium and Potential in CD-1 and CBA/CaJ Mice

Marcus

2003

JARO - Journal of the Association for Research in Otolaryngolog

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The CD-1 mouse strain is known to have early onset of hearing loss that is progressive with aging. We sought to determine whether a disturbance of K + homeostasis and pathological changes in the cochlear lateral wall were involved in the age-related hearing loss (AHL) of CD-1 as compared to the CBA/CaJ strain which has minimal AHL. In the present study, the endocochlear potential (EP) and endolymphatic K + concentration ([K + ] e ) were measured in both strains of mice with double-barrel microelectrodes at ''young'' (1-2 mo) and ''old'' (5-9 mo) ages. CBA/ CaJ mice displayed no changes with aging in EP and [K + ] e of the basal turn. In the apical turn, there was a small positive shift of the EP (10 mV) with aging under both normoxic and acute anoxic conditions ()EP), without any change of [K + ] e . Further, there were no obvious pathological changes in the lateral wall of CBA/CaJ mice. By contrast, old CD-1 mice displayed a significantly reduced [K + ] e by 30% in both basal and apical turns with no significant changes in normoxic EP. The )EP in the apical turn was significantly reduced in magnitude by 6 mV. A severe loss of cells with aging was observed in the region of type IV fibrocytes of the apical and basal turns and of type II fibrocytes in the basal turn. A complete degeneration of organ of Corti was also observed at the basal turn of old CD-1 mice, as well as a basalward decline of spiral ganglion neuron density. The pathological changes in spiral ligament of CD-1 mice were similar to those of an inbred mouse strain C57BL/6J that expresses an AHL gene (ahl) and might be a primary etiology of AHL of CD-1 mice. These findings have ramifications for our understanding of AHL and for interpretation of genetic mutations in a CD-1 background.

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Section: Cd-1 Mouse Strainsupporting

confidence: 88%

Section: Introductionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

See 3 more Smart Citations

Age-Related Changes in Cochlear Endolymphatic Potassium and Potential in CD-1 and CBA/CaJ Mice

Marcus

2003

JARO - Journal of the Association for Research in Otolaryngolog

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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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Cu/Zn SOD deficiency potentiates hearing loss and cochlear pathology in aged 129,CD-1 mice

et al. 1999

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Hereditary deafness occurring in cd/1 mice

Cited by 43 publications

References 7 publications

Age-Related Changes in Cochlear Endolymphatic Potassium and Potential in CD-1 and CBA/CaJ Mice

Age-Related Changes in Cochlear Endolymphatic Potassium and Potential in CD-1 and CBA/CaJ Mice

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

Cu/Zn SOD deficiency potentiates hearing loss and cochlear pathology in aged 129,CD-1 mice

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