Abstract #326, Date 2/15/99, Session K3, Poster (B73)

Early presbyacusis in Turner's Syndrome: Mapping of estrogen receptor alfa and beta in the inner ear

*A. Elmqvist Stenberg (Otorhinolaryng., Karolinska Hosp.); L. Sahlin, H. Wang (Reproduct. Endocrin., Karolinska Hosp.); A. Schrott-Fischer, J. Fish (Otorhinolaryng., Univ. Hosp., Innsbruck, Austria); M. Hultcrantz (Otorhinolaryng., Karolinska Hosp., Sweden)

Background: Turner's Syndrome (TS) (45,X) affects approximately 1:2000 new-born girls and leads to short stature, failure to enter puberty spontaneously and infertility due to streak ovaries and estrogen deficiency. Ear and hearing problems are common problems among these patients. The young Turner girls often suffer from repeated otitis media and the middle aged Turner women frequently complain of a rapid onset of social hearing problems, due to a premature ageing of the ear (presbyacusis) with a high frequency sensorineural hearing loss added to an early appearing dip in the mid-frequencies. Aim: One of the main characteristics of TS is lack of endogenous estrogen production. In order to investigate the possible influence of estrogen on the inner ear and hearing, we wanted to map eventual estrogen receptors in the inner ear. Methods: Specimens of healthy mouse and rat inner ear and specimens of inner ear from a "Turner mouse" was used. Also inner ear tissue from human fetuses (all spontaneously aborted) and adults were stained immunohistologically with antibodies against estrogen receptor alfa and beta. Results: We found staining of estrogen receptors in the organ of corti at specific locations, which are described. Conclusion: Estrogen receptors are present in the inner ear proposing that estrogen may affect hearing, possibly in a protective way.