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Eya4-deficient mice are a model for heritable otitis media
Frederic F.S. Depreux, … , Christine E. Seidman, J.G. Seidman
Frederic F.S. Depreux, … , Christine E. Seidman, J.G. Seidman
Published January 24, 2008
Citation Information: J Clin Invest. 2008;118(2):651-658. https://doi.org/10.1172/JCI32899.
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Research Article

Eya4-deficient mice are a model for heritable otitis media

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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 2

Middle ear pathology in Eya4–/– mice.

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Middle ear pathology in Eya4–/– mice.
               
(A) Macroscopic vi...
(A) Macroscopic views of tympanic membrane from wild-type (+/+) and Eya4–/– (–/–) mice. Eya4–/– mouse ears had air bubbles (asterisk), indicative of middle ear effusion, and dilated capillaries (black arrow) as well as tympanic membrane retraction (see also Figure 3). Scale bars: 1 mm. (B and C) PAS-stained paraffin sections of mice, age 3 weeks. (B) The mesotympanum of middle ear cavity shows inflammatory cells (red arrow) in Eya4–/– mice, and the mucoperiosteum (black arrowheads) is thickened. A, stapedial artery; I, incus; M, malleus; OW, bony niche of the oval window; T, tympanic membrane. Scale bars: 200 μm. (C) The hypotympanum regions of Eya4–/– mice show effusion (asterisk), inflammatory cells (red arrow), hyperplastic ciliated epithelial cells (black arrows), and expanded lamina propria connective tissue in the mucoperiosteum (arrowhead; corresponding regions denoted by gray arrow/arrowhead in wild-type). MEC, middle ear cavity.

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