Download PDF
Horm Metab Res 2015; 47(05): 361-366
DOI: 10.1055/s-0035-1548792
Review
© Georg Thieme Verlag KG Stuttgart · New York

Gender Development and Sexuality in Disorders of Sex Development

S. A. Berenbaum
1   Departments of Psychology & Pediatrics, The Pennsylvania State University, Pennsylvania, USA
,
H. F. L. Meyer-Bahlburg
2   New York State Psychiatric Institute & Department of Psychiatry, College of Physicians & Surgeons, Columbia University New York, New York, USA
› Author Affiliations
Further Information

Publication History

received 02 October 2014

accepted 02 March 2015

Publication Date:
08 April 2015 (online)

Also available at
    Permissions and Reprints

Abstract

Understanding psychological development in individuals with disorders of sex development (DSD) is important for optimizing their clinical care and for identifying paths to competence and health in all individuals. In this paper, we focus on psychological outcomes likely to be influenced by processes of physical sexual differentiation that may be atypical in DSD, particularly characteristics related to being male or female (those that show sex differences in the general population, gender identity, and sexuality). We review evidence suggesting that (a) early androgens facilitate several aspects of male-typed behavior, with large effects on activity interests, and moderate effects on some social and personal behaviors (including sexual orientation) and spatial ability; (b) gender dysphoria and gender change occur more frequently in individuals with DSD than in the general population, with rates varying in relation to syndrome, initial gender assignment, and medical treatment; and (c) sexual behavior may be affected by DSD through several paths related to the condition and treatment, including reduced fertility, physical problems associated with genital ambiguity, social stigmatization, and hormonal variations. We also consider limitations to current work and challenges to studying gender and sexuality in DSD. We conclude with suggestions for a research agenda and a proposed research framework.

Key words

gendered activities - gender roles - gender identity - gender dysphoria - sexual orientation - sex hormones
 

  • References

  • 1 McCarthy MM, Arnold AP. Reframing sexual differentiation of the brain. Nature Neuroscience 2011; 14: 677-683
    CrossrefPubMedGoogle Scholar
  • 2 Berenbaum SA, Beltz AM. Sexual differentiation of human behavior: Effects of prenatal and pubertal organizational hormones. Front Neuroendocrinol 2011; 32: 183-200
    CrossrefPubMedGoogle Scholar
  • 3 Arnold AP, Breedlove SM. Organizational and activational effects of sex steroids on brain and behavior: A reanalysis. Horm Behav 1985; 19: 469-498
    CrossrefPubMedGoogle Scholar
  • 4 Bukowski WM, McCauley E, Mazur T. DSD, peers and psychosocial well-being. Horm Metab Res 2015; in press
    PubMedGoogle Scholar
  • 5 Blakemore JEO, Berenbaum SA, Liben LS. Gender development. New York: Psychology Press/Taylor & Francis; 2009
    Google Scholar
  • 6 Hines M. Gender development and the human brain. Ann Rev Neurosci 2011; 34: 69-88
    CrossrefPubMedGoogle Scholar
  • 7 Jürgensen M, Hiort O, Holterhus P-M, Thyen U. Gender role behavior in children with XY karyotype and disorders of sex development. Horm Behav 2007; 51: 443-453
    CrossrefPubMedGoogle Scholar
  • 8 Berenbaum SA, Bryk KLK, Beltz AM. Early androgen effects on spatial and mechanical abilities: Evidence from congenital adrenal hyperplasia. Behav Neurosci 2012; 126: 86-96
    CrossrefPubMedGoogle Scholar
  • 9 Meyer-Bahlburg HFL. Sex steroids and variants of gender identity. Endocrinol Metab Clin North Am 2013; 42: 435-452
    CrossrefPubMedGoogle Scholar
  • 10 Meyer-Bahlburg HFL, Dolezal C, Baker SW, Ehrhardt AA, New MI. Gender development in women with congenital adrenal hyperplasia as a function of disorder severity. Arch Sex Behav 2006; 35: 667-684
    CrossrefPubMedGoogle Scholar
  • 11 Meyer-Bahlburg HFL, Migeon CJ, Berkovitz GD, Gearhart JP, Dolezal C, Wisniewski AB. Attitudes of adult 46,XY intersex persons to clinical management policies. J Urol 2004; 171: 1615-1619
    CrossrefPubMedGoogle Scholar
  • 12 Hughes IA, Houk C, Ahmed SF, Lee PA. LWPES Consensus Group, ESPE Consensus Group . Consensus statement on management of intersex disorders. Arch Dis Child 2006; 91: 554-563
    PubMedGoogle Scholar
  • 13 Meyer-Bahlburg HFL. Psychoendocrinology of congenital adrenal hyperplasia. In: New MI, Lekarev O, Parsa A, Yuen TT, O’Malley B, Hammer GD. (eds.) Genetic steroid disorders. London, Waltham, San Diego: Academic Press/Elsevier; 2014: 285-300
    CrossrefGoogle Scholar
  • 14 Wood H, Sasaki S, Bradley SJ, Singh D, Fantus S, Owen-Anderson A, Di Giacomo A, Bain J, Zucker KJ. Patterns of referral to a gender identity service for children and adolescents (1976–2011): Age, sex ratio, and sexual orientation. J Sex Marital Ther 2013; 39: 1-6
    PubMedGoogle Scholar
  • 15 Coleman E, Bockting W, Botzer M, Cohen-Kettenis P, DeCuypere G, Feldman J, Fraser L, Green L, Knudson G, Meyer III WJ, Monstrey S, Adler RK, Brown GR, Devor AH, Ehrbar R, Ettner R, Eyler E, Garofalo R, Karasic DH, Lev AI, Mayer G, Meyer-Bahlburg HFL, Hall BP, Pfaefflin F, Rachlin K, Robinson B, Schechter LS, Tangpricha V, van Trotsenburg M, Vitale A, Winter S, Whittle S, Wylie KR, Zucker K. Standards of Care for the Health of Transsexual, Transgender, and Gender-Nonconforming People, Version 7. Int J Transgend 2012; 13: 165-232
    PubMedGoogle Scholar
  • 16 Schweizer K, Brunner F, Schützmann K, Schönbucher V, Richter-Appelt H. Gender identity and coping in female 46,XY adults with androgen biosynthesis deficiency (intersexuality/DSD). J Counsel Psychol 2009; 56: 189-201
    CrossrefPubMedGoogle Scholar
  • 17 Meyer-Bahlburg HFL, Dolezal C, Baker SW, New MI. Sexual orientation in women with classical or non-classical congenital adrenal hyperplasia as a function of degree of prenatal androgen excess. Arch Sex Behav 2008; 37: 85-99
    CrossrefPubMedGoogle Scholar
  • 18 Hines M, Ahmed SF, Hughes IA. Psychological outcome and gender-related development in complete androgen insensitivity syndrome. Arch Sex Behav 2003; 32: 93-101
    CrossrefPubMedGoogle Scholar
  • 19 Pfaus JG, Kippin TE, Coria-Avila GA, Gelez H, Afonso VM, Ismail N, Parada M. Who, what, where, when (and maybe even why)? How the experience of sexual reward connects sexual desire, preference, and performance. Arch Sex Behav 2012; 41: 31-62
    CrossrefPubMedGoogle Scholar
  • 20 Dawson SJ, Chivers ML. Gender-specificity of solitary and dyadic sexual desire among gynephilic and androphilic women and men. J Sex Med 2014; 11: 980-994
    CrossrefPubMedGoogle Scholar
  • 21 Diamond LM. Sexual fluidity. Understanding women’s love and desire. Cambridge, MA: Harvard University Press; 2008
    Google Scholar
  • 22 McCullough LB, Chervenak FA, Brent RL, Hippen B. A case study in unethical transgressive bioethics: “Letter of Concern from Bioethicists” about the prenatal administration of dexamethasone. Am J Bioethic 2010; 10: 35-45
    PubMedGoogle Scholar
  • 23 Köhler B, Kleinemeier E, Lus A, Hiort O, Grüters A, Thyen U. DSD Network Working Group . Satisfaction with genital surgery and sexual life of adults with XY disorders of sex development: results from the German clinical evaluation study. J Clin Endocrinol Metab 2012; 97: 577-588
    CrossrefPubMedGoogle Scholar
  • 24 Arnold AP. The end of gonad-centric sex determination in mammals. Trends Genet 2012; 28: 55-61
    CrossrefPubMedGoogle Scholar
  • 25 Beltz AM, Blakemore JEO, Berenbaum SA. Sex differences in brain and behavioral development. In: Rakic P, Rubenstein J. (eds.) Comprehensive Developmental Neuroscience. Oxford: Elsevier; 2013
    CrossrefGoogle Scholar
  • 26 Merke DP, Fields JD, Keil MF, Vaituzis AC, Chrousos GP, Giedd JN. Children with classic congenital adrenal hyperplasia have decreased amygdala volume: potential prenatal and postnatal hormonal effects. J Clin Endocrinol Metab 2003; 88: 1760-1765
    CrossrefPubMedGoogle Scholar
  • 27 Guillamon A. The brain of transsexual persons. Invited Lecture, WPATH Symposium. Bangkok, Thailand, February 14–18 2014
    PubMedGoogle Scholar
  • 28 DSD-TRN (DSD Translational Research Network) https://dsdtrn.genetics,ucla.edu Accessed 8-21-14
    PubMed
  • 29 I-DSD (International Disorders of Sex Development) www.gla.ac.uk/schools/medicine/medicine/childhealth/i-dsdproject/ Accessed 8-21-14
    PubMed
  • 30 dsd-LIFE www.dsd-life.eur Accessed 8-21-14
    PubMed