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Journal of Endocrinology (2009) 201, 397-406       DOI: 10.1677/JOE-09-0060
© 2009 Society for Endocrinology
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Inner ear pathology and loss of hearing in estrogen receptor-β deficient mice

Rusana Simonoska, Annika E Stenberg, Maoli Duan1, Konstantin Yakimchuk2, Anders Fridberger1, Lena Sahlin3, Jan-Åke Gustafsson2,4 and Malou Hultcrantz

Department of Otorhinolaryngology, Karolinska Institutet and Karolinska University Hospital, 171 76 Stockholm, Sweden1 Center for Hearing and Communication Research, Karolinska Institutet, 171 76 Stockholm, Sweden2 Department of Biosciences and Nutrition, Karolinska Institutet, NOVUM, 141 86 Huddinge, Sweden3 Department of Woman and Child Health, Karolinska Institutet and Karolinska University Hospital, 171 76 Stockholm, Sweden4 Department of Biology and Biochemistry, Center for Nuclear Receptors and Cell Signalling, University of Houston, Houston, Texas 77 204, USA

(Correspondence should be addressed to R Simonoska; Email: rusana.simonoska{at}karolinska.se)

There are well known differences between males and females in hearing. In the present study, the role of estrogen receptor-β (ER-β; listed as ESR2 in the MGI Database) in hearing was investigated by comparing hearing and morphology of the inner ear in ER-β knock-out mice (ER-β–/–) with that of wild-type (WT) littermates. Hearing was analyzed with auditory brainstem response audiometry at 3 and 12 months. The ER-β–/– mice were deaf at 1 year of age, and the morphological analysis showed absence of hair cells and loss of the whole organ of Corti initiated in the basal turn of the cochlea. Furthermore, in ER-β–/–, but not in WT mice, the spiral ganglion was lacking many of its neurons. Immunostaining showed the presence of both ER-{alpha} (listed as ESR1 in the MGI Database) and ER-β in the nuclei of some neurons in the inner ear in WT mice, but no ER-β was found in the ER-β–/– mice as expected. ER-{alpha} staining was predominant in the nuclei of large neurons and ER-β in nuclei of small neurons and fibroblasts. These results reveal that both ERs are present in the inner ear at specific localizations suggesting subtype-specific functions. It is concluded that ER-β is important for the prevention of age-related hearing loss. These findings strengthen the hypothesis that estrogen has a direct effect on hearing functions.







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