MP:0000043 | organ of Corti degeneration | "a retrogressive impairment of function or destruction of the highly specialized epithelium in the floor of the ductus cochlearis" [cwg:Carroll-Ann W. Goldsmith , Mouse Genome Informatics curator] |
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Allelic Composition: Prox1tm1Gco/Prox1+ Genetic Background: involves: 129S1/Sv * 129X1/SvJ
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MP:0001262 | decreased body weight | "lower than normal average weight " [J:61295] |
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Allelic Composition: Prox1tm1Gco/Prox1+ Genetic Background: involves: 129S1/Sv * 129X1/SvJ
Allelic Composition: Slc26a5tm1Jnz/Slc26a5+ Genetic Background: involves: 129S7/SvEvBrd * C57BL/6J
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MP:0001967 | deafness | "inability to hear" [J:57651] |
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Allelic Composition: Prox1tm1Gco/Prox1+ Genetic Background: involves: 129S1/Sv * 129X1/SvJ
Allelic Composition: Slc26a5mpc234H/Slc26a5mpc234H Genetic Background: involves: BALB/c * C3H/HeH * C57BL/6J
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MP:0002857 | cochlear ganglion degeneration | "loss of neural cell bodies in the group of nerve cell bodies that conveys auditory sensation from the organ of Corti to the hindbrain" [Stedman s Medical Dictionary , 27th edition:ISBN 0-683-40008-8] |
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Allelic Composition: Prox1tm1Gco/Prox1+ Genetic Background: involves: 129S1/Sv * 129X1/SvJ
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MP:0004362 | cochlear hair cell degeneration | "degeneration or loss of the sensory epithelial cells of the cochlea; these cells are in synaptic contact with the auditory nerve" [ISBN:0-683-40008-8 "Stedman s Medical Dictionary, 27th edition", MGI:csmith "Cynthia L. Smith, Mouse Genome Informatics Curator"] |
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Allelic Composition: Prox1tm1Gco/Prox1+ Genetic Background: involves: 129S1/Sv * 129X1/SvJ
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MP:0004398 | cochlear inner hair cell degeneration | "degeneration or loss of the single row of flask-shaped inner hair sensory cells of the organ of Corti" [MGI:anna "Anna V. Anagnostopoulos, Mouse Genome Informatics Curator"] |
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Allelic Composition: Prox1tm1Gco/Prox1+ Genetic Background: involves: 129S1/Sv * 129X1/SvJ
Allelic Composition: Slc26a5tm1Jnz/Slc26a5+ Genetic Background: involves: 129S7/SvEvBrd * C57BL/6J
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MP:0004399 | abnormal cochlear outer hair cell morphology | "any structural abnormality in the sensory cells of the organ of Corti which are in synaptic contact with sensory as well as efferent fibers of the auditory nerve; there are three rows of columnar outer hair cells largely surrounded by cortilymph and supporting phalangeal cells; the apical end of each cell is anchored to the cuticular plate and about 100 stereocilia arranged in a V-shaped pattern extend from the cell s surface through the cuticular plate into the tectorial membrane" [MGI:anna "Anna V. Anagnostopoulos, Mouse Genome Informatics Curator"] |
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Allelic Composition: Slc26a5tm1Jnz/Slc26a5+ Genetic Background: involves: 129S7/SvEvBrd * C57BL/6J
Allelic Composition: Slc26a5tm1Jnz/Slc26a5tm1Jnz Genetic Background: involves: 129S7/SvEvBrd
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MP:0004402 | decreased cochlear outer hair cell number | "decreased number (or less than the expected 3 rows) of the columnar outer hair sensory cells of the organ of Corti" [MGI:anna "Anna V. Anagnostopoulos, Mouse Genome Informatics Curator"] |
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Allelic Composition: Ctnnb1tm2Kem/Ctnnb1tm2Kem,Rac1tm1Djk/Rac1+,Tg(Msx2-cre)5Rem/0 Genetic Background: involves: 129S4/SvJae
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MP:0004404 | cochlear outer hair cell degeneration | "degeneration or loss of the columnar outer hair sensory cells of the organ of Corti" [MGI:anna "Anna V. Anagnostopoulos, Mouse Genome Informatics Curator"] |
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Allelic Composition: Prox1tm1Gco/Prox1+ Genetic Background: involves: 129S1/Sv * 129X1/SvJ
Allelic Composition: Slc26a5tm1Jnz/Slc26a5+ Genetic Background: involves: 129S7/SvEvBrd * C57BL/6J
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MP:0004413 | absent cochlear microphonics | "absence or loss of the bioelectric potentials produced by the hair cells of the organ of Corti in response to sound" [MGI:anna "Anna V. Anagnostopoulos, Mouse Genome Informatics Curator"] |
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Allelic Composition: Prox1tm1Gco/Prox1+ Genetic Background: involves: 129S1/Sv * 129X1/SvJ
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MP:0004414 | decreased cochlear microphonics | "reduction of the bioelectric potentials produced by the hair cells of the organ of Corti in response to sound" [MGI:anna "Anna V. Anagnostopoulos, Mouse Genome Informatics Curator"] |
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Allelic Composition: Prox1tm1Gco/Prox1+ Genetic Background: involves: 129S1/Sv * 129X1/SvJ
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MP:0004417 | decreased cochlear nerve compound action potential | "reduction of the combined potentials resulting from activation of the auditory division of the eighth cranial nerve" [MGI:anna "Anna V. Anagnostopoulos, Mouse Genome Informatics Curator"] |
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Allelic Composition: Prox1tm1Gco/Prox1+ Genetic Background: involves: 129S1/Sv * 129X1/SvJ
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MP:0004430 | abnormal Claudius cell morphology | "any structural abnormality in the columnar cells located on the floor of the ductus cochlearis external to the spiral organ" [MGI:anna "Anna V. Anagnostopoulos, Mouse Genome Informatics Curator"] |
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Allelic Composition: Prox1tm1Gco/Prox1+ Genetic Background: involves: 129S1/Sv * 129X1/SvJ
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MP:0004434 | abnormal cochlear outer hair cell physiology | "anomalies in processes pertinent to the function of cochlear OHCs which are known to enhance the performance of the cochlea, both qualitatively (increased selectivity) and quantitatively (increased sensitivity); in addition to the mechanoelectric transduction required for auditory sensation, OHCs also perform electromechanical transduction, whereby transmembrane voltage drives rapid changes in the length and stiffness of OHCs at audio frequencies in vitro (electromotility)" [MGI:anna "Anna V. Anagnostopoulos, Mouse Genome Informatics Curator"] |
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Allelic Composition: Ctnnb1tm2Kem/Ctnnb1tm2Kem,Rac1tm1Djk/Rac1+,Tg(Msx2-cre)5Rem/0 Genetic Background: involves: 129S4/SvJae
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MP:0004435 | abnormal cochlear outer hair cell electromotility | "anomalous motility of the cochlear OHCs in response to electrical stimulation; normally, the motile response (i.e. rapid change in length and stiffness of OHCs) is closely coupled to the changing transduction current and provides a region-specific amplification in the movement of the spiral organ that enhances transduction at the IHCs in that specific region of the cochlear spiral, increasing both sensitivity and specificity; electromotility is believed to arise through voltage-gated conformational changes in motor protein (prestin) or proteins located in the lateral wall of the OHC" [MGI:anna "Anna V. Anagnostopoulos, Mouse Genome Informatics Curator"] |
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Allelic Composition: Dicer1Gt(RRF266)Byg/Dicer1Gt(RRF266)Byg Genetic Background: involves: 129P2/OlaHsd * C57BL/6
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MP:0004436 | absent cochlear outer hair cell electromotility | "absence or loss of motility of the cochlear OHCs in response to electrical stimulation" [MGI:anna "Anna V. Anagnostopoulos, Mouse Genome Informatics Curator"] |
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Allelic Composition: Prox1tm1Gco/Prox1+ Genetic Background: involves: 129S1/Sv * 129X1/SvJ
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MP:0004437 | decreased cochlear outer hair cell electromotility | "reduced motility of the cochlear OHCs in response to electrical stimulation" [MGI:anna "Anna V. Anagnostopoulos, Mouse Genome Informatics Curator"] |
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Allelic Composition: Slc26a5tm1Jnz/Slc26a5+ Genetic Background: involves: 129S7/SvEvBrd * C57BL/6J
Allelic Composition: Slc26a5tm3Jnz/Slc26a5tm3Jnz Genetic Background: involves: 129S6/SvEvTac * C57BL/6 * FVB/N
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MP:0004465 | degeneration of supporting cells | "degeneration or loss of the highly differentiated epithelial cells with distinctive morphological features that surround all hair cells in the organ of Corti" [MGI:anna "Anna V. Anagnostopoulos, Mouse Genome Informatics Curator"] |
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Allelic Composition: Prox1tm1Gco/Prox1+ Genetic Background: involves: 129S1/Sv * 129X1/SvJ
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MP:0004466 | short cochlear outer hair cells | |
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Allelic Composition: Prox1tm1Gco/Prox1+ Genetic Background: involves: 129S1/Sv * 129X1/SvJ
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MP:0004736 | abnormal distortion product otoacoustic emission | "any abnormality in the sound produced by the cochlea in response to stimulation with 2 simultaneous tones of different frequencies; this measurement is particularly useful in assessing the functional state of OHCs" [eMedicine:http://www.emedicine.com/ent/topic372.htm, MGI:anna "Anna V. Anagnostopoulos, Mouse Genome Informatics Curator", MGI:csmith "Cynthia L. Smith, Mouse Genome Informatics Curator"] |
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Allelic Composition: Prox1tm1Gco/Prox1+ Genetic Background: involves: 129S1/Sv * 129X1/SvJ
Allelic Composition: Slc26a5tm1Jnz/Slc26a5+ Genetic Background: involves: 129S7/SvEvBrd * C57BL/6J
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MP:0004737 | absent distortion product otoacoustic emissions | "failure to create mechanical distortions in the inner ear when two primary tones are presented, indicating failure of outer hair cells to amplify basilar membrane motion" [MGI:anna "Anna V. Anagnostopoulos, Mouse Genome Informatics Curator"] |
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Allelic Composition: Prox1tm1Gco/Prox1+ Genetic Background: involves: 129S1/Sv * 129X1/SvJ
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MP:0004767 | increased cochlear nerve compound action potential | "greater combined potentials resulting from activation of the auditory division of the eighth cranial nerve" [MGI:anna "Anna V. Anagnostopoulos, Mouse Genome Informatics Curator"] |
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Allelic Composition: Slc26a5tm3Jnz/Slc26a5tm3Jnz Genetic Background: involves: 129S6/SvEvTac * C57BL/6 * FVB/N
Allelic Composition: Slc26a5tm1Jnz/Slc26a5tm1Jnz Genetic Background: involves: 129S7/SvEvBrd
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MP:0006325 | impaired hearing | "reduced ability to percieve auditory stimuli" [MGI:smb "Susan M. Bello, Mouse Genome Informatics Curator"] |
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Allelic Composition: Prox1tm1Gco/Prox1+ Genetic Background: involves: 129S1/Sv * 129X1/SvJ
Allelic Composition: Slc26a5tm1Jnz/Slc26a5+ Genetic Background: involves: 129S7/SvEvBrd * C57BL/6J
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MP:0006336 | abnormal otoacoustic response | "anomaly in the acoustic energy produced by the cochlea in the presence or absence of sound stimulation" [MGI:smb "Susan M. Bello, Mouse Genome Informatics Curator"] |
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Allelic Composition: Slc26a5tm1Jnz/Slc26a5tm1Jnz Genetic Background: involves: 129S7/SvEvBrd
Allelic Composition: Slc26a5tm1Jnz/Slc26a5tm1Jnz,Tectatm1.1Ogha/Tectatm1.1Ogha Genetic Background: involves: 129S7/SvEvBrd * CBA
Allelic Composition: Slc26a5tm1Jnz/Slc26a5tm1Jnz,Tectatm1.1Ogha/Tecta+ Genetic Background: involves: 129S7/SvEvBrd * CBA
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MP:0011967 | increased or absent threshold for auditory brainstem response | "increase in the value at which one or more sound frequencies or broadband clicks first elicits a recordable response generated by electrical activity of neurons in the ascending auditory system, or complete lack of a recordable response at any frequency or broadband click" [MGI:csmith] |
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Allelic Composition: Prox1tm1Gco/Prox1+ Genetic Background: involves: 129S1/Sv * 129X1/SvJ
Allelic Composition: Slc26a5tm1Jnz/Slc26a5+ Genetic Background: involves: 129S7/SvEvBrd * C57BL/6J
Allelic Composition: Slc26a5tm4.1(cre/ERT2)Jnz/Slc26a5tm4.1(cre/ERT2)Jnz Genetic Background: involves: 129S6/SvEvTac * C57BL/6
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