Skip to main content

Prenatal Androgenization Affects Gender-Related Behavior But Not Gender Identity in 5–12-Year-Old Girls with Congenital Adrenal Hyperplasia

Abstract

Gender assignment of children with intersexuality and related conditions has recently become highly controversial. On the basis of extensive animal research and a few human case reports, some authors have proposed the putative masculinization of the brain by prenatal hormones—indicated by the degree of genital masculinization—as the decisive criterion of gender assignment and have derived the recommendation that 46,XX newborns with congenital adrenal hyperplasia (CAH) and full genital masculinization should be assigned to the male gender. The purpose of this study was to test in CAH girls of middle childhood the assumption that prenatal androgens determine the development of gender identity. Fifteen girls with CAH (range of genital Prader stage, 2–4/5), 30 control girls, and 16 control boys (age range, 5–12 years) underwent 2 gender-play observation sessions, and a gender identity interview yielding scales of gender confusion/dysphoria. About half a year earlier, mothers had completed 2 questionnaires concerning their children's gender-related behavior. The results showed that, as expected, CAH girls scored more masculine than control girls on all scales measuring gender-related behavior, with robust effect sizes. By contrast, neither conventionally significant differences nor trends were found on the 3 scales of the gender identity interview. We conclude that prenatal androgenization of 46,XX fetuses leads to marked masculinization of later gender-related behavior, but the absence of any increased gender-identity confusion/dysphoria does not indicate a direct determination of gender identity by prenatal androgens and does not, therefore, support a male gender assignment at birth of the most markedly masculinized girls.

This is a preview of subscription content, access via your institution.

References

  1. Arnold, A. P. (2002). Concepts of genetic and hormonal induction of vertebrate sexual differentiation in the twentieth century, with special reference to the brain. In D. W. Pfaff, A. P. Arnold, A. M. Etgen, S. E. Fahrbach, & R. T. Rubin (Ed.),Hormones, brain, and behavior (Vol. 4, pp. 105-135). Amsterdam: Academic Press (Elsevier Science).

    Google Scholar 

  2. Berenbaum, S. A., & Bailey, J. M. (2003). Effects on gender identity of prenatal androgens and genital appearance: Evidence from girls with congenital adrenal hyperplasia.Journal of Clinical Endocrinology and Metabolism, 88, 1102-1106.

    Google Scholar 

  3. Bin-Abbas, B., Conte, F. A., Grumbach, M. M., & Kaplan, S. L. (1999). Congenital hypogonadotropic hypogonadism and micropenis: Effect of testosterone treatment on adult penis size. Why sex reversal is not indicated.Journal of Pediatrics, 134, 579-583.

    Google Scholar 

  4. Chan-Cua, S., Freidenberg, G., & Jones, K. L. (1989). Occurrence of male phenotype in genotypic females with congenital virilizing adrenal hyperplasia.American Journal of Medical Genetics, 34, 406-412.

    Google Scholar 

  5. Dasgupta, R., Schnitzer, J. J., Hendren, W. H., & Donahoe, P. K. (2003). Congenital adrenal hyperplasia: Surgical considerations required to repair a 46,XX patient raised as a boy.Journal of Pediatric Surgery, 38, 1269-1273.

    Google Scholar 

  6. De Vries, G. J., & Simerly, R. B. (2002). Anatomy, development, and function of sexually dimorphic neural circuits in the mammalian brain. In D. W. Pfaff, A. P. Arnold, A. M. Etgen, S. E. Fahrbach, & R. T. Rubin (Ed.),Hormones, brain, and behavior (Vol. 4, pp. 137-191). Amsterdam: Academic Press (Elsevier Science).

    Google Scholar 

  7. Diamond, M. (1997). Sexual identity and sexual orientation in children with traumatized or ambiguous genitalia.Journal of Sex Research, 34, 199-211.

    Google Scholar 

  8. Diamond, M., & Sigmundson, H. K. (1997). Management of intersexuality: Guidelines for dealing with persons with ambiguous genitalia.Archives of Pediatrics and Adolescent Medicine, 151, 1046-1050.

    Google Scholar 

  9. Ehrhardt, A. A., & Baker, S. W. (1974). Fetal androgens, human central nervous system differentiation, and behavior sex differentiation. In R. C. Friedman, R. M. Richart, & R. L. Van De Wiele (Ed.),Sex differences in behavior (pp. 33-51). New York: Wiley.

    Google Scholar 

  10. Ehrhardt, A. A., Epstein, R., & Money, J. (1968). Fetal androgens and female gender identity in the early treated adrenogenital syndrome.Johns Hopkins Medical Journal, 122, 160-167.

    Google Scholar 

  11. Goy, R. W., & McEwen, B. S. (1980).Sexual differentiation of the brain. Cambridge, MA: MIT.

    Google Scholar 

  12. Grumbach, M. M., Hughes, I. A., & Conte, F. A. (2003). Disorders of sex differentiation. In P. R. Larsen, H. M. Kronenberg, S. Melmed, & K. S. Polonsky (Ed.),Williams textbook of endocrinology (10th ed., pp. 842-1002). Philadelphia: W. B. Saunders.

    Google Scholar 

  13. Hines, M. (2002). Sexual differentiation of human brain and behavior. In D. W. Pfaff, A. P. Arnold, A. M. Etgen, S. E. Fahrbach, & R. T. Rubin (Ed.),Hormones, brain, and behavior (Vol. 4, pp. 452-462). Amsterdam: Academic Press (Elsevier Science).

    Google Scholar 

  14. Kandemir, N., & Yordam, N. (1997). Congenital adrenal hyperplasia in Turkey: A review of 273 patients.Acta Paediatrica, 86, 22-25.

    Google Scholar 

  15. Kruijver, F. P. M., Zhou, J.-N., Pool, C. W., Hofman, M. A., Gooren, L. J. G., & Swaab, D. F. (2000). Male-to-female transsexuals have female neuron numbers in a limbic nucleus.Journal of Clinical Endocrinology and Metabolism, 85, 2034-2041.

    Google Scholar 

  16. Madsen, P. O. (1963). Familial female pseudohermaphroditism with hypertension and penile urethra.Journal of Urology, 90, 466-469.

    Google Scholar 

  17. Meyer-Bahlburg, H. F. L. (1999). Gender assignment and reassignment in 46,XY pseudohermaphroditism and related conditions.Journal of Clinical Endocrinology and Metabolism, 84, 3455-3458.

    Google Scholar 

  18. Meyer-Bahlburg, H. F. L. (2001). Gender and sexuality in classic congenital adrenal hyperplasia.Endocrinology and Metabolism Clinics of North America, 20(1), 155-171.

    Google Scholar 

  19. Meyer-Bahlburg, H. F. L., Dolezal, C., Baker, S. W., Carlson, A. D., Obeid, J. S., & New, M. I. (in press). Cognitive and motor development of children with and without congenital adrenal hyperplasia after early-prenatal dexamethasone.Journal of Clinical Endocrinology and Metabolism.

  20. Meyer-Bahlburg, H. F. L., Gruen, R. S., New, M. I., Bell, J. J., Morishima, A., Shimshi, M., et al. (1996). Gender change from female to male in classical congenital adrenal hyperplasia.Hormones and Behavior, 30, 319-332.

    Google Scholar 

  21. Meyer-Bahlburg, H. F. L., Sandberg, D. E., Dolezal, C. L., & Yager, T. J. (1994). Gender-related assessment of childhood play.Journal of Abnormal Child Psychology, 22, 643-660.

    Google Scholar 

  22. Meyer-Bahlburg, H. F. L., Sandberg, D. E., Yager, T. J., Dolezal, C. L., & Ehrhardt, A. A. (1994). Questionnaire scales for the assessment of atypical gender development in girls and boys.Journal of Psychology and Human Sexuality, 6, 19-39.

    Google Scholar 

  23. Money, J. (1991).Biographies of gender and hermaphroditism in paired comparisons. Amsterdam: Elsevier.

    Google Scholar 

  24. Money, J., & Daléry, J. (1976). Iatrogenic homosexuality: Gender identity in seven 46,XX chromosomal females with hyperadrenocortical hermaphroditism born with a penis, three reared as boys, four reared as girls.Journal of Homosexuality, 1, 357-371.

    Google Scholar 

  25. Money, J., & Ehrhardt, A. A. (1972).Man and woman, boy and girl: The differentiation and dimorphism of gender identity from conception to maturity. Baltimore: The Johns Hopkins University Press.

    Google Scholar 

  26. Money, J., Hampson, J. G., & Hampson, J. L. (1955). Hermaphroditism: Recommendations concerning assignment of sex, change of sex, and psychologic management.Bulletin of the Johns Hopkins Hospital, 97, 284-300.

    Google Scholar 

  27. Mulaikal, R. M., Migeon, C. J., & Rock, J. A. (1987). Fertility rates in female patients with congenital adrenal hyperplasia due to 21-hydroxylase deficiency.New England Journal of Medicine, 316, 178-182.

    Google Scholar 

  28. New, M. I., Carlson, A., Obeid, J., Marshall, I., Cabrera, M. S., Goseco, A., et al. (2001). Prenatal diagnosis for congenital adrenal hyperplasia in 532 pregnancies.Journal of Clinical Endocrinology and Metabolism, 86, 5651-5657.

    Google Scholar 

  29. Ozbey, H. (1998). Gender assignment in female congenital adrenal hyperplasia.British Journal of Urology, 81, 178-179.

    Google Scholar 

  30. Ravichandran, R., Lafferty, F., McGinniss, M. J., & Taylor, H. C. (1996). Congenital adrenal hyperplasia presenting as massive adrenal incidentalomas in the sixth decade of life: Report of two patients with 21-hydroxylase deficiency.Journal of Clinical Endocrinology and Metabolism, 81, 1776-1779.

    Google Scholar 

  31. Reiner, W. (1997). To be male or female: That is the question.Archives of Pediatrics and Adolescent Medicine, 151, 224-225.

    Google Scholar 

  32. Slijper, F. M. E., Drop, S. L. S., Molenaar, J. C., & de Muinck Keizer-Schrama, S. M. P. F. (1998). Long-term psychological evaluation of intersex children.Archives of Sexual Behavior, 27, 125-144.

    Google Scholar 

  33. Sripathi, V., Ahmed, S., Sakati, N., & Al-Ashwal, A. (1997). Gender reversal in 46XX congenital virilizing adrenal hyperplasia.British Journal of Urology, 79, 785-789.

    Google Scholar 

  34. Stoller, R. J. (1964). The hermaphroditic identity of hermaphrodites.Journal of Nervous and Mental Disease, 139, 453-457.

    Google Scholar 

  35. Wallen, K., & Baum, M. J. (2002). Masculinization and defeminization in altricial and precocial mammals: Comparative aspects of steroid hormone action. In D. W. Pfaff, A. P. Arnold, A. M. Etgen, S. E. Fahrbach, & R. T. Rubin (Ed.),Hormones, brain, and behavior (Vol. 4, pp. 385-423). Amsterdam: Academic Press (Elsevier Science).

    Google Scholar 

  36. Wilson, J. D. (1999). The role of androgens in male gender role behavior.Endocrine Reviews, 20, 726-737.

    Google Scholar 

  37. Wisniewski, A. B., Migeon, C. J., Gearhart, J. P., Rock, J. A., Berkovitz, G. D., Plotnick, L. P., et al. (2001). Congenital micropenis: Long-term medical, surgical and psychosexual follow-up of individuals raised male or female.Hormone Research, 56, 3-11.

    Google Scholar 

  38. Woelfle, J., Hoepffner, W., Sippell, W. G., Brämswig, J. H., Heidemann, P., Deiß, D., et al. (2002). Complete virilization in congenital adrenal hyperplasia: Clinical course, medical management and disease-related complications.Clinical Endocrinology, 56, 231-238.

    Google Scholar 

  39. Zucker, K. J. (1999). Intersexuality and gender identity differentiation.Annual Review of Sex Research, 10, 1-69.

    Google Scholar 

  40. Zucker, K. J., & Bradley, S. J. (1995).Gender identity disorder and psychosexual problems in children and adolescents. New York: Guilford.

    Google Scholar 

  41. Zucker, K. J., Bradley, S. J., Kuksis, M., Pecore, K., Birkenfeld-Adams, A., Doening, R. W., et al. (1999). Gender constancy judgments in children with gender identity disorder: Evidence for a developmental lag.Archives of Sexual Behavior, 28, 475-502.

    Google Scholar 

  42. Zucker, K. J., Bradley, S. J., Lowry Sullivan, C. B., Kuksis, M., Birkenfeld-Adams, A., & Mitchell, J. N. (1993). A gender identity interview for children.Journal of Personality Assessment, 61, 443-456.

    Google Scholar 

  43. Zucker, K. J., Bradley, S. J., Oliver, G., Blake, J., Fleming, S., & Hood, J. (1996). Psychosexual development of women with congenital adrenal hyperplasia.Hormones and Behavior, 30, 300-318.

    Google Scholar 

Download references

Author information

Affiliations

Authors

Corresponding author

Correspondence to Heino F. L. Meyer-Bahlburg.

Rights and permissions

Reprints and Permissions

About this article

Cite this article

Meyer-Bahlburg, H.F.L., Dolezal, C., Baker, S.W. et al. Prenatal Androgenization Affects Gender-Related Behavior But Not Gender Identity in 5–12-Year-Old Girls with Congenital Adrenal Hyperplasia. Arch Sex Behav 33, 97–104 (2004). https://doi.org/10.1023/B:ASEB.0000014324.25718.51

Download citation

  • hermaphroditism
  • congenital adrenal hyperplasia
  • androgens
  • gender identity