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Epidermal Growth Factor Receptor Expression in Human Fetal Cochlea With Turner Syndrome

Schwentner, Ilona*; Schmutzhard, Joachim*; Glueckert, Rudolf*; Charitidi, Konstantina; Falkeis, Christina‡§; Sergi, Consolato‡§; Canlon, Barbara; Schrott-Fischer, Annelies*

doi: 10.1097/MAO.0b013e3181b2880c
Basic Science

Hypothesis: Growth hormones have beneficial effects on increasing height in adults with Turner syndrome (TS) and may also affect auditory function.

Background: Turner syndrome is the most common sex-linked chromosomal abnormality in female conceptions. Epidermal growth factor and its receptor (EGFR) affect differentiation, proliferation, and migration of epithelial cells and function as survival factors. The expression of EGFR is found in the developing and juvenile inner ear of experimental animals but is absent in adults.

Methods: To determine whether EGFR plays a role in TS, its expression was analyzed in the cochlea of healthy fetus and fetus with TS and in healthy adults.

Results: In healthy fetuses, EGFR protein expression was localized to the inner and outer hair cells and the Reissner membrane. The fetuses with TS on the 13th gestational week (GW) showed a similar pattern of immunoreactivity as the normal 16th and 20th GW cochlea. By the 23rd GW, EGFR immunoreactivity was not detectable in the TS hair cells or the Reissner membrane, and less intensive staining was found in the surrounding fibrocytes of the spiral ganglion.

Conclusion: This is the first demonstration of EGFR immunoreactivity in the human cochlea and illustrates how EGFR expression is altered during development in TS. These findings indicate the importance of growth hormone receptors for inner ear development in humans.

*Department of Otorhinolaryngology, University Hospital, Medical University Innsbruck, Innsbruck, Austria; †Department of Physiology and Pharmacology, Karolinska Institutet, Stockholm, Sweden; ‡Institute of Pathology, Innsbruck Medical University, Innsbruck, Austria; and §Department of Laboratory Medicine, University of Alberta, Edmonton, Canada

Address correspondence and reprint requests to Joachim Schmutzhard, M.D., Department of Otorhinolaryngology, Medical University Innsbruck, Anichstraße 35, A-6020 Innsbruck, Austria; E-mail:

This study was supported by the Austrian Science Foundation (FWF Project B05 15958).

© 2009 Otology & Neurotology, Inc.