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. 2018 Apr 3;8:5482. doi: 10.1038/s41598-018-23856-8

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© The Author(s) 2018

Open Access This article is licensed under a Creative Commons Attribution 4.0 International License, which permits use, sharing, adaptation, distribution and reproduction in any medium or format, as long as you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons license, and indicate if changes were made. The images or other third party material in this article are included in the article’s Creative Commons license, unless indicated otherwise in a credit line to the material. If material is not included in the article’s Creative Commons license and your intended use is not permitted by statutory regulation or exceeds the permitted use, you will need to obtain permission directly from the copyright holder. To view a copy of this license, visit http://creativecommons.org/licenses/by/4.0/.

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Trooper mice have ossicle and semicircular canal malformations. (A–C) X-ray μCT images of the left middle and inner ears of Chd7^+/+and Chd7^+/Trooper mice including the vestibular apparatus (V) and cochlea (C). The anterior canal (red arrowhead) is hypoplastic and the intersection of the posterior and superior canals (green arrowhead) is enlarged and misshapen in both Chd7^+/Trooper mice when compared with a Chd7^+/+control. (B) In the non-circling Chd7^+/Trooper mouse the lateral semicircular canal (black arrowhead) is complete but hypoplastic. (C) In the circling Chd7^+/Trooper mouse the lateral semicircular canal (black arrowhead) is incomplete. The missing section in each cochlea (*) is an artifact of the imaging/reconstruction process. n = 1 Chd7^+/+ and 2 Chd7^+/Trooper mice. (D,E) An alternate view of the above middle ears. The Chd7^+/Trooper stapes was rounded and small with an unusual bone growth extending from the tubercle to the otic capsule (grey arrow) and the stapes footplate (purple arrow) was also deformed. In the non-circling Trooper, the footplate was under developed and not contacting the oval window however the more severely affected Chd7^+/Trooper (with circling behavior) has stapedial fusion with the oval window.