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Archives of Sexual Behavior

, Volume 35, Issue 6, pp 667–684 | Cite as

Gender Development in Women with Congenital Adrenal Hyperplasia as a Function of Disorder Severity

  • Heino F. L. Meyer-Bahlburg
  • Curtis Dolezal
  • Susan W. Baker
  • Anke A. Ehrhardt
  • Maria I. New
Original Paper

Abstract

Prenatal-onset classical congenital adrenal hyperplasia (CAH) in 46,XX individuals is associated with variable masculinization/defeminization of the genitalia and of behavior, presumably both due to excess prenatal androgen production. The purpose of the current study was threefold: (1) to extend the gender-behavioral investigation to the mildest subtype of 46,XX CAH, the non-classical (NC) variant, (2) to replicate previous findings on moderate and severe variants of 46,XX CAH using a battery of diversely constructed assessment instruments, and (3) to evaluate the utility of the chosen assessment instruments for this area of work. We studied 63 women with classical CAH (42 with the salt wasting [SW] and 21 with the simple virilizing [SV] variant), 82 women with the NC variant, and 24 related non-CAH sisters and female cousins as controls (COS). NC women showed a few signs of gender shifts in the expected direction, SV women were intermediate, and SW women most severely affected. In terms of gender identity, two SW women were gender-dysphoric, and a third had changed to male in adulthood. All others identified as women. We conclude that behavioral masculinization/defeminization is pronounced in SW-CAH women, slight but still clearly demonstrable in SV women, and probable, but still in need of replication in NC women. There continues a need for improved instruments for gender assessment.

Keywords

Congenital adrenal hyperplasia Gender-related behavior Gender identity Gender dysphoria Androgen effects Disorders of sex development Hermaphroditism 

Notes

Acknowledgments

The project described was supported in part by USPHS Grants HD-38409, RR06020 (GCRC), and by Grant Number U54 RR01-9484 from the National Center for Research Resources (NCRR), a component of the National Institutes of Health (NIH). Its contents are solely the responsibilities of the authors and do not necessarily represent the official views of NCRR or NIH. We thank the study women for their participation in this research and Drs. Sheri Berenbaum and Richard Lippa for making their respective questionnaires available to us. Ms. Rhoda Gruen served as interviewer trainer. Ms. Patricia Connolly assisted in word processing.

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Copyright information

© Springer Science+Business Media, Inc. 2006

Authors and Affiliations

  • Heino F. L. Meyer-Bahlburg
    • 1
  • Curtis Dolezal
    • 1
  • Susan W. Baker
    • 2
  • Anke A. Ehrhardt
    • 1
  • Maria I. New
    • 2
  1. 1.NYS Psychiatric Institute/Department of PsychiatryColumbia UniversityNew YorkUSA
  2. 2.Department of PediatricsMount Sinai School of MedicineNew YorkUSA

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