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Accepted Preprint first posted online on 17 March 2009

Journal of Endocrinology 2009;201:397.

Journal of Endocrinology (2009) In press
DOI: 10.1677/JOE-09-0060
© 2009 Society for Endocrinology
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RESEARCH

Inner ear pathology and loss of hearing in estrogen receptor beta deficient mice

Rusana Simonoska, Annika Stenberg, Maoli Duan, Konstantin Yakimchuk, Anders Fridberger, Lena Sahlin, Jan-Ake Gustaffson and Malou Hultcrantz

R Simonoska, Dept of Otorhinolaryngology, Karolinska Institutet, Stockholm, Sweden
A Stenberg, Dept of Otorhinolaryngology, Karolinska Institutet, Stockholm, Sweden
M Duan, Center for Hearing Communication Research, Karolinska Institutet, Stockholm, Sweden
K Yakimchuk, Dept of Biosciences and Nutrition, Karolinska Institutet, Stockholm, Sweden
A Fridberger, Center for Hearing Communication Research, Karolinska Institutet, Stockholm, Sweden
L Sahlin, Dept of Woman and Child Health, Karolinska institutet, Stockholm, Sweden
J Gustaffson, Dept of Biosciences and Nutrition, Karolinska Institutet, Stockholm, Sweden
M Hultcrantz, Dept of Otorhinolaryngology, Karolinska Institutet, Stockholm, Sweden

Correspondence: Rusana Simonoska, Email: rusana.simonoska{at}karolinska.se

Abstract

There are well known differences between males and females in hearing. In the present study, the role of estrogen receptor beta (ERbeta) in hearing was investigated by comparing hearing and morphology of the inner ear in ERbeta knock-out mice (ERbeta-/-) with that of wild type (WT) littermates. Hearing was analyzed with auditory brainstem response audiometry at 3 and 12 months. The ERbeta-/- mice were deaf at one 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 ERbeta-/-, but not in WT mice, the spiral ganglion was lacking many of its neurons. Immunostaining showed the presence of both ERalpha and ERbeta in the nuclei of some neurons in the inner ear in WT mice, but no ERbeta was found in the ERbeta-/- mice as expected. ERalpha staining was predominant in the nuclei of large neurons and ERbeta in nuclei of small neurons and fibroblasts. These results reveal that both estrogen receptors are present in the inner ear at specific localizations suggesting subtype-specific functions. It is concluded that ERbeta 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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