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Thyroid hormone receptor β-dependent expression of a potassium conductance in inner hair cells at the onset of hearing

By Alfons Rüsch, Lawrence C. Erway, Dominik Oliver, Björn Vennström and Douglas Forrest


To elucidate the role of thyroid hormone receptors (TRs) α1 and β in the development of hearing, cochlear functions have been investigated in mice lacking TRα1 or TRβ. TRs are ligand-dependent transcription factors expressed in the developing organ of Corti, and loss of TRβ is known to impair hearing in mice and in humans. Here, TRα1-deficient (TRα1(−/−)) mice are shown to display a normal auditory-evoked brainstem response, indicating that only TRβ, and not TRα1, is essential for hearing. Because cochlear morphology was normal in TRβ(−/−) mice, we postulated that TRβ regulates functional rather than morphological development of the cochlea. At the onset of hearing, inner hair cells (IHCs) in wild-type mice express a fast-activating potassium conductance, I(K,f), that transforms the immature IHC from a regenerative, spiking pacemaker to a high-frequency signal transmitter. Expression of I(K,f) was significantly retarded in TRβ(−/−) mice, whereas the development of the endocochlear potential and other cochlear functions, including mechanoelectrical transduction in hair cells, progressed normally. TRα1(−/−) mice expressed I(K,f) normally, in accord with their normal auditory-evoked brainstem response. These results establish that the physiological differentiation of IHCs depends on a TRβ-mediated pathway. When defective, this may contribute to deafness in congenital thyroid diseases

Topics: Biological Sciences
Publisher: The National Academy of Sciences
Year: 1998
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Provided by: PubMed Central
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