Eya4-deficient mice are a model for heritable otitis media
Frederic F.S. Depreux, Keith Darrow, David A. Conner, Roland D. Eavey, M. Charles Liberman, Christine E. Seidman, J.G. Seidman
Frederic F.S. Depreux, Keith Darrow, David A. Conner, Roland D. Eavey, M. Charles Liberman, Christine E. Seidman, J.G. Seidman
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Research Article

Eya4-deficient mice are a model for heritable otitis media

Abstract

Otitis media is an extremely common pediatric inflammation of the middle ear that often causes pain and diminishes hearing. Vulnerability to otitis media is due to eustachian tube dysfunction as well as other poorly understood factors, including genetic susceptibility. As EYA4 mutations cause sensorineural hearing loss in humans, we produced and characterized Eya4-deficient (Eya4–/–) mice, which had severe hearing deficits. In addition, all Eya4–/– mice developed otitis media with effusion. Anatomic studies revealed abnormal middle ear cavity and eustachian tube dysmorphology; thus, Eya4 regulation is critical for the development and function of these structures. We suggest that some human otitis media susceptibility reflects underlying genetic predisposition in genes like EYA4 that regulate middle ear and eustachian tube anatomy.

Authors

Frederic F.S. Depreux, Keith Darrow, David A. Conner, Roland D. Eavey, M. Charles Liberman, Christine E. Seidman, J.G. Seidman

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Figure 4

Morphometry of wild-type (+/+) and Eya4–/– (–/–) middle ear cavity in azithromycin-treated mice.

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Morphometry of wild-type (+/+) and Eya4–/– (–/–) middle ear cavity in az...
(A) The OpTC of Eya4–/– mice was smaller than that in wild-type. Scale bars: 200 μm. (B) A 3D reconstruction of portions of the middle ear cavity, eustachian tube, and nasopharynx (NP). The moET, isthmus, cartilaginous segment (cartET), and opening of the eustachian tube within the nasopharynx (OpNP) are indicated on the wild-type 3D reconstruction. The location of the OpTC is denoted (white asterisk). Eustachian tube dimensions (see Table 2) were obtained by measurement of the structures identified. Scale bar: 300 μm.