Mouse Genome Informatics
hm1
    Strctm1Ugds/Strctm1Ugds
involves: 129S2/SvPas * BALB/c * C57BL/6 * C57BL/6J
Key:
phenotype observed in females WTSI Wellcome Trust Sanger Institute
phenotype observed in males EuPh Europhenome
N normal phenotype
hearing/vestibular/ear
• hair-bundle imprints corresponding to the anchoring points of the tallest outer hair cell stereocilia are absent
• the tops of the stereocilia rows in all outer hair cells, but not inner hair cells, are not as clearly aligned as in wild-type mice
• at P14, simultaneous masking of CAP is weakened compared to in wild-type mice
• at P60, mice exhibit an increase in compound auditory potential (CAP) threshold compared to in wild-type mice due to loss of functional mechanoelectrical transducer channels
• from P15 to P60, the amplitude of cochlear microphonic response decreased compared to in wild-type mice indicating a loss of mechanoelectrical transducer channels
• however, cochlear microphonic waves have the same phase as in wild-type mice
• up to 80 db from P14 onward
• from P15

nervous system
• hair-bundle imprints corresponding to the anchoring points of the tallest outer hair cell stereocilia are absent
• the tops of the stereocilia rows in all outer hair cells, but not inner hair cells, are not as clearly aligned as in wild-type mice
• from P15 to P60, the amplitude of cochlear microphonic response decreased compared to in wild-type mice indicating a loss of mechanoelectrical transducer channels
• however, cochlear microphonic waves have the same phase as in wild-type mice

Mouse Models of Human Disease
OMIM IDRef(s)
Deafness, Autosomal Recessive 16; DFNB16 603720 J:141421