Knock-in mouse model for resistance to thyroid hormone (RTH): an RTH mutation in the thyroid hormone receptor beta gene disrupts cochlear morphogenesis.

Thyroid hormone and the beta isoform of its receptor, Trb, are essential for normal development of the mammalian auditory system. We have analyzed auditory system function and structure in a mouse strain with a targeted Thrb mutation, Thrb(PV), which leads to the loss of binding of thyroid hormone (T3) to the Trb protein. Heterozygosity for the orthologous human THRB(PV) mutation and other similar mutations in human THRB cause resistance to thyroid hormone (RTH), which is occasionally associated with mild sensorineural hearing impairment.

Enhanced cochlear responses after sound exposure.

Alternating potentials produced in Hensen's cells of Mongolian gerbils by sinusoidal stimuli were enhanced or depressed after exposure to broad-band sound of moderately high intensity, depending on exposure- and stimulus intensities. Since Hensen's cell responses have been shown to be identical in phase and directly proportional in magnitude to outer hair cell (OHC) responses (Oesterle, E.C.

Stiffness changes in chick hair bundles following in vitro overstimulation.

1. The in vitro effect of intense stimulation on the micromechanical stiffness of hair cell sensory hair bundles was studied at three locations on the chick basilar papilla. Threshold levels of hair bundle motion, produced by a water jet stimulus, were examined before and after exposure to a 300 Hz water jet stimulus set at 25 dB above the pre-exposure threshold level.

Stiffness of hair bundles in the chick cochlea.

The stiffness of hair bundles from isolated chick cochlear hair cells was measured in tissue culture medium. A water jet was used to deflect fiberglass fibers, quartz fibers, and hair bundles of isolated hair cells. A voltage-displacement curve was generated for a water jet ramp stimulus applied to miniature fiberglass and quartz fibers.