The Sound of Silence: Mouse Models for Hearing Loss
Figure 1
Assays to detect phenotypes of deafness-related mutations in mice. ((a)–(c)) Light microscopy analysis of sections of cochlear duct from (a) wild type, (b) , and (c) . LZ: limbal zone, and MB: marginal band, HS: Hensen’s stripe. Arrowhead in (b) Kimura’s membrane, arrow in (c) tectorial membrane. Reprinted with permission from [17]. ((d)–(f)) Transmission electron micrograph of the organ of Corti in wild type versus when Cx26 was deleted (e-f). D: deiters cell, P: outer pilla cells. Reprinted with permission from [18]. ((g)-(h)) Scanning electron micrograph of Organ of Corti basal portion at P0 from yellow submarine (Ysb) homozygous. Reprinted with permission from [19]. (i) In vitro analysis of OHC electromotility in wild type and mutant (Slc26a5) mice. It shows length changes of OHC in response to voltage steps (−120–60 mV in 20 mV steps) in whole-cell, voltage-clamp recordings. Reprinted with permission from [20]. (j) In situ hybridization detects expression of Cdh23 in the neurosensory epithelium of 4-day neonates. Antisense probe specifically labels the neuroepithelium along the cochlea duct (arrowheads). Cross-section through the cochlear duct identifies specific labeling in three outer (OHC) and one inner hair cells (IHCs). Reprinted with permission from [21]. (k) Immunohistochemistry on whole mount shows Cdh2 (green) is detected at E16.5 in stereocilia (stained for F-actin, red) of hair cells from the basal turn of the cochlea in wild-type mice. (l) Immunolocalization of cadherin 23 (shown in green) in the inner ear of homozygous waltzer mice. Cadherin 23 (green) was not detected in utricle stereocilia (stained for F-actin, red), but was detected in the cuticular plate of hair cells (arrows). Reprinted with permission from [22].
