Mice homozygous for the <i>Ush1c<sup>tm1Xzl</sup></i> mutation have circling, head-tossing, and hyperactive behaviors typical of vestibular mutants.  Auditory-evoked brainstem assessment shows that homozygotes are completely deaf and scanning electron microscopy shows that the hair cell stereocilia become progressively disorganized with age, with the outer hair cells more affected than the inner hair cells.  Although histology at 12 months of age does not reveal any abnormalities, there is a decline in electrotreinogram readings for both rods and cones by 11 months of age.  Because the first four exons were replaced by the beta galactosidase reporter cassette, endogenous expression of this gene in phenotypically normal mice can be traced by assessing lacZ expression in heterozygotes.

Mice homozygous for this knock-out mutation are deaf and exhibit movement anomalies such as circling and head-tossing.

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