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The Journal of Neuroscience, September 9, 2009, 29(36):11123-11133; doi:10.1523/JNEUROSCI.2232-09.2009

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Neurobiology of Disease
Atoh1-Lineal Neurons Are Required for Hearing and for the Survival of Neurons in the Spiral Ganglion and Brainstem Accessory Auditory Nuclei

Stephen M. Maricich,¹ Anping Xia,² Erin L. Mathes,¹ Vincent Y. Wang,³ John S. Oghalai,⁵ Bernd Fritzsch,⁶ and Huda Y. Zoghbi⁴

¹Department of Pediatrics and Neurosciences, Case Western Reserve University, Cleveland, Ohio 44106, ²Department of Otolaryngology, Stanford University, Stanford, California 94305, ³Department of Neurosurgery, University of California, San Francisco, San Francisco, California 94143, ⁴Departments of Pediatrics, Molecular and Human Genetics, and Neuroscience, and Howard Hughes Medical Institute, and ⁵Department of Otolaryngology, Baylor College of Medicine, Houston, Texas 77030, and ⁶Department of Biology, University of Iowa, Iowa City, Iowa 52242

Correspondence should be addressed to either of the following: Stephen M. Maricich, Department of Neuroscience, Room E750i, Case Western Reserve University, 10900 Euclid Avenue, Cleveland, OH 44106, Email: smm18{at}case.edu; or Huda Y. Zoghbi, Department of Pediatrics, Baylor College of Medicine, One Baylor Plaza, Room T807, Houston, TX 77030, Email: hzoghbi{at}bcm.edu

Atoh1 is a basic helix–loop–helix transcription factor necessary for the specification of inner ear hair cells and central auditory system neurons derived from the rhombic lip. We used the Cre–loxP system and two Cre-driver lines (Egr2^Cre and Hoxb1^Cre) to delete Atoh1 from different regions of the cochlear nucleus (CN) and accessory auditory nuclei (AAN). Adult Atoh1-conditional knock-out mice (Atoh1^CKO) are behaviorally deaf, have diminished auditory brainstem evoked responses, and have disrupted CN and AAN morphology and connectivity. In addition, Egr2; Atoh1^CKO mice lose spiral ganglion neurons in the cochlea and AAN neurons during the first 3 d of life, revealing a novel critical period in the development of these neurons. These new mouse models of predominantly central deafness illuminate the importance of the CN for support of a subset of peripheral and central auditory neurons.

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Received May 12, 2009; revised July 8, 2009; accepted July 14, 2009.

Correspondence should be addressed to either of the following: Stephen M. Maricich, Department of Neuroscience, Room E750i, Case Western Reserve University, 10900 Euclid Avenue, Cleveland, OH 44106, Email: smm18{at}case.edu; or Huda Y. Zoghbi, Department of Pediatrics, Baylor College of Medicine, One Baylor Plaza, Room T807, Houston, TX 77030, Email: hzoghbi{at}bcm.edu

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