Severe hearing loss in Dlxl mutant mice

Hearing Research
Daniel B PolleyJohn L R Rubenstein

Abstract

The Dlx homeobox gene family participates in regulating middle and inner ear development. A significant role for Dlxl, in particular,has been demonstrated in the development of the middle ear ossicles, but the functional consequences of Dlx.l gene mutation on hearing thresholds has not been assessed. The present study characterizes auditory brainstem responses to click and tonal stimuli in a non-lethal variant of a Dlxl gene knockout. We found that peripheral hearing thresholds for click and tonal stimuli were significantly elevated in homozygous Dlxl knockout (Dlxl-/ ) compared to both heterozygous (Dlxl+/ ) and wild type (Dlxl+/+) mice. Thus, abnormal mor-phogenesis of the incus and stapes that has been documented previously with histological measures is now known to result in a severe peripheral hearing deficit.

References

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Citations

Jul 4, 2012·Proceedings of the National Academy of Sciences of the United States of America·Bryan A SeyboldChristoph E Schreiner
Aug 7, 2012·Proceedings of the National Academy of Sciences of the United States of America·Jennifer F Linden
Aug 26, 2010·Journal of Child Psychology and Psychiatry, and Allied Disciplines·John L R Rubenstein
Oct 10, 2009·Journal of Neurodevelopmental Disorders·Rong MaoMriganka Sur

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