Medicine and Health Sciences Commons^™

Cib2 Interacts With Tmc1 And Tmc2 And Is Essential For Mechanotransduction In Auditory Hair Cells, Arnaud P. J. Giese, Yi-Quan Tang, Ghanshyam P. Sinha, Michael R. Bowl, Adam C. Goldring, Andrew Parker, Mary J. Freeman, Steve D. M. Brown, Saima Riazuddin, Robert Fettiplace, William R. Schafer, Gregory I. Frolenkov, Zubair M. Ahmed

Physiology Faculty Publications

Inner ear hair cells detect sound through deflection of stereocilia, the microvilli-like projections that are arranged in rows of graded heights. Calcium and integrin-binding protein 2 is essential for hearing and localizes to stereocilia, but its exact function is unknown. Here, we have characterized two mutant mouse lines, one lacking calcium and integrin-binding protein 2 and one carrying a human deafness-related Cib2 mutation, and show that both are deaf and exhibit no mechanotransduction in auditory hair cells, despite the presence of tip links that gate the mechanotransducer channels. In addition, mechanotransducing shorter row stereocilia overgrow in hair cell bundles of …

Pre-Pulse Inhibition Assessment Of Sound Localization In Mice, Kathryn C. Brooks

Senior Honors Projects, 2010-2019

The purpose of my Honors Research Project was to assist Dr. Megan

Klingenberg in completing part of the research in her final dissertation project. Dr.

Klingenberg’s project was based off of a study done by Allen and Ison in 2010.

This study tested the auditory spatial acuity of mice using pre-pulse inhibition of

the startle reflex as the response for detecting the sound stimulus. The goal of

Dr. Klingenberg’s AuD project was “to explore the methodological, functional, and

genetic influences on sound localization using pre-pulse inhibition of the acoustic

startle response in mice”. Dr. Klingenberg’s project was broken into three …

Pre-Pulse Inhibition Assessment Of Sound Localization In Mice: Methodological, Functional, And Genetic Considerations, Megan Klingenberg

Dissertations, 2014-2019

Sound localization is an important aspect of normal hearing. The Eph/ephin family of signaling proteins, studied here, is known to guide the formation of central auditory connections in early development, particularly topographic inputs from the lateral superior olive (LSO) to the inferior colliculus (IC). Processing in the LSO and its influences on the IC are known to be heavily involved in sound localization tasks. One way to study sound localization in mice is through pre-pulse inhibition (PPI). PPI is the phenomenon by which a weak prestimulus inhibits the response to a subsequent startle stimulus. In studying sound localization, the prestimulus …

Hearing Loss And Retarded Cochlear Development In Mice Lacking Type 2 Iodothyronine Deiodinase, Lily Ng, Richard J. Goodyear, Chad A. Woods, Mark J. Schneider

Dartmouth Scholarship

The later stages of cochlear differentiation and the developmental onset of hearing require thyroid hormone. Although thyroid hormone receptors (TRs) are a prerequisite for this process, it is likely that other factors modify TR activity during cochlear development. The mouse cochlea expresses type 2 deiodinase (D2), an enzyme that converts thyroxine, the main form of thyroid hormone in the circulation, into 3,5,3'-triiodothyronine (T3) the major ligand for TRs. Here, we show that D2-deficient mice have circulating thyroid hormone levels that would normally be adequate to allow hearing to develop but they exhibit an auditory phenotype similar to that caused by …