Inner ear pathology and loss of hearing in estrogen receptor-beta deficient mice. 2009

Rusana Simonoska, and Annika E Stenberg, and Maoli Duan, and Konstantin Yakimchuk, and Anders Fridberger, and Lena Sahlin, and Jan-Ake Gustafsson, and Malou Hultcrantz
Department of Otorhinolaryngology, Karolinska Institutet and Karolinska University Hospital, Stockholm, Sweden. rusana.simonoska@karolinska.se

There are well known differences between males and females in hearing. In the present study, the role of estrogen receptor-beta (ER-beta; listed as ESR2 in the MGI Database) in hearing was investigated by comparing hearing and morphology of the inner ear in ER-beta knock-out mice (ER-beta(-/-)) with that of wild-type (WT) littermates. Hearing was analyzed with auditory brainstem response audiometry at 3 and 12 months. The ER-beta(-/-) 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-beta(-/-), 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-beta in the nuclei of some neurons in the inner ear in WT mice, but no ER-beta was found in the ER-beta(-/-) mice as expected. ER-alpha staining was predominant in the nuclei of large neurons and ER-beta 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-beta is important for the prevention of age-related hearing loss. These findings strengthen the hypothesis that estrogen has a direct effect on hearing functions.

UI MeSH Term Description Entries
D007758 Ear, Inner The essential part of the hearing organ consists of two labyrinthine compartments: the bony labyrinthine and the membranous labyrinth. The bony labyrinth is a complex of three interconnecting cavities or spaces (COCHLEA; VESTIBULAR LABYRINTH; and SEMICIRCULAR CANALS) in the TEMPORAL BONE. Within the bony labyrinth lies the membranous labyrinth which is a complex of sacs and tubules (COCHLEAR DUCT; SACCULE AND UTRICLE; and SEMICIRCULAR DUCTS) forming a continuous space enclosed by EPITHELIUM and connective tissue. These spaces are filled with LABYRINTHINE FLUIDS of various compositions. Labyrinth,Bony Labyrinth,Ear, Internal,Inner Ear,Membranous Labyrinth,Bony Labyrinths,Ears, Inner,Ears, Internal,Inner Ears,Internal Ear,Internal Ears,Labyrinth, Bony,Labyrinth, Membranous,Labyrinths,Labyrinths, Bony,Labyrinths, Membranous,Membranous Labyrinths
D007759 Labyrinth Diseases Pathological processes of the inner ear (LABYRINTH) which contains the essential apparatus of hearing (COCHLEA) and balance (SEMICIRCULAR CANALS). Inner Ear Disease,Ear Disease, Inner,Ear Diseases, Inner,Inner Ear Diseases,Labyrinth Disease
D008810 Mice, Inbred C57BL One of the first INBRED MOUSE STRAINS to be sequenced. This strain is commonly used as genetic background for transgenic mouse models. Refractory to many tumors, this strain is also preferred model for studying role of genetic variations in development of diseases. Mice, C57BL,Mouse, C57BL,Mouse, Inbred C57BL,C57BL Mice,C57BL Mice, Inbred,C57BL Mouse,C57BL Mouse, Inbred,Inbred C57BL Mice,Inbred C57BL Mouse
D002452 Cell Count The number of CELLS of a specific kind, usually measured per unit volume or area of sample. Cell Density,Cell Number,Cell Counts,Cell Densities,Cell Numbers,Count, Cell,Counts, Cell,Densities, Cell,Density, Cell,Number, Cell,Numbers, Cell
D005260 Female Females
D006309 Hearing The ability or act of sensing and transducing ACOUSTIC STIMULATION to the CENTRAL NERVOUS SYSTEM. It is also called audition. Audition
D000818 Animals Unicellular or multicellular, heterotrophic organisms, that have sensation and the power of voluntary movement. Under the older five kingdom paradigm, Animalia was one of the kingdoms. Under the modern three domain model, Animalia represents one of the many groups in the domain EUKARYOTA. Animal,Metazoa,Animalia
D013136 Spiral Ganglion The sensory ganglion of the COCHLEAR NERVE. The cells of the spiral ganglion send fibers peripherally to the cochlear hair cells and centrally to the COCHLEAR NUCLEI of the BRAIN STEM. Ganglion of Corti,Auditory Ganglion,Spiral Ganglia,Auditory Ganglions,Ganglia, Spiral,Ganglion, Auditory,Ganglion, Spiral,Ganglions, Auditory
D047629 Estrogen Receptor beta One of the ESTROGEN RECEPTORS that has greater affinity for ISOFLAVONES than ESTROGEN RECEPTOR ALPHA does. There is great sequence homology with ER alpha in the DNA-binding domain but not in the ligand binding and hinge domains. ERbeta,ERbetacx,Estrogen Receptor 2,Estrogen Receptors beta,Receptor beta, Estrogen
D051379 Mice The common name for the genus Mus. Mice, House,Mus,Mus musculus,Mice, Laboratory,Mouse,Mouse, House,Mouse, Laboratory,Mouse, Swiss,Mus domesticus,Mus musculus domesticus,Swiss Mice,House Mice,House Mouse,Laboratory Mice,Laboratory Mouse,Mice, Swiss,Swiss Mouse,domesticus, Mus musculus

Related Publications

Rusana Simonoska, and Annika E Stenberg, and Maoli Duan, and Konstantin Yakimchuk, and Anders Fridberger, and Lena Sahlin, and Jan-Ake Gustafsson, and Malou Hultcrantz
February 2008, FASEB journal : official publication of the Federation of American Societies for Experimental Biology,
Rusana Simonoska, and Annika E Stenberg, and Maoli Duan, and Konstantin Yakimchuk, and Anders Fridberger, and Lena Sahlin, and Jan-Ake Gustafsson, and Malou Hultcrantz
January 2021, Frontiers in cellular neuroscience,
Rusana Simonoska, and Annika E Stenberg, and Maoli Duan, and Konstantin Yakimchuk, and Anders Fridberger, and Lena Sahlin, and Jan-Ake Gustafsson, and Malou Hultcrantz
January 2008, Laboratory investigation; a journal of technical methods and pathology,
Rusana Simonoska, and Annika E Stenberg, and Maoli Duan, and Konstantin Yakimchuk, and Anders Fridberger, and Lena Sahlin, and Jan-Ake Gustafsson, and Malou Hultcrantz
June 2016, Laryngo- rhino- otologie,
Rusana Simonoska, and Annika E Stenberg, and Maoli Duan, and Konstantin Yakimchuk, and Anders Fridberger, and Lena Sahlin, and Jan-Ake Gustafsson, and Malou Hultcrantz
October 1984, HNO,
Rusana Simonoska, and Annika E Stenberg, and Maoli Duan, and Konstantin Yakimchuk, and Anders Fridberger, and Lena Sahlin, and Jan-Ake Gustafsson, and Malou Hultcrantz
December 1993, Voenno-meditsinskii zhurnal,
Rusana Simonoska, and Annika E Stenberg, and Maoli Duan, and Konstantin Yakimchuk, and Anders Fridberger, and Lena Sahlin, and Jan-Ake Gustafsson, and Malou Hultcrantz
October 1997, HNO,
Rusana Simonoska, and Annika E Stenberg, and Maoli Duan, and Konstantin Yakimchuk, and Anders Fridberger, and Lena Sahlin, and Jan-Ake Gustafsson, and Malou Hultcrantz
November 2009, PloS one,
Rusana Simonoska, and Annika E Stenberg, and Maoli Duan, and Konstantin Yakimchuk, and Anders Fridberger, and Lena Sahlin, and Jan-Ake Gustafsson, and Malou Hultcrantz
October 2001, The Journal of neuroscience : the official journal of the Society for Neuroscience,
Rusana Simonoska, and Annika E Stenberg, and Maoli Duan, and Konstantin Yakimchuk, and Anders Fridberger, and Lena Sahlin, and Jan-Ake Gustafsson, and Malou Hultcrantz
September 2000, The American journal of otology,
Copied contents to your clipboard!