Intersex and the Olympic Games

¹ Department of Urology, Churchill Hospital Oxford OX3 7LJ, UK
² Nuffield Department of Surgery, John Radcliffe Hospital Oxford OX3 9DU, UK
³ IMG Media McCormack House, Burlington Lane, London W4 2TH, UK

Correspondence to: Mr R Ritchie robritchie{at}doctors.org.uk

Introduction

The assignment of sex at birth determines the manner in which a child is raised. It determines their appearance and governs their development.¹ Sexual ambiguity – intersex – may result in a broad spectrum of physical problems. Certain anomalies may be detectable only by the astute clinician and would otherwise go unnoticed, whereas more profound anomalies may result in considerable developmental and psychological difficulties for both the child and his or her parents and family. Sex determination in individuals with sexual ambiguity has created difficulties in the arena of international sports for many years. While Hitler's Olympic Games in Berlin in 1936 generated much controversy around the issue of racial and religious discrimination, it also brought the complexities of gender verification to the forefront of competitive sport for the first time.

In this paper we review the historical impact of intersex in the area of international sports and discuss current thinking with regard to determination of sex in sports men and women.

Intersex

Intersex refers to the atypical appearance of the external genitalia at birth where they differ from the usual development of either sex and create difficulty in sex assignment.² This condition is uncommon, with an estimated incidence of 0.018%. Whether conditions such as Klinefelter's syndrome and Turner's syndrome should be included in the definition of intersex is debatable – they are not traditionally regarded as true intersex. If included, the incidence may be as high as 1.7%.³ Classification of intersex is challenging and controversial; some authorities suggest three broad groups – conditions resulting in the masculinized female, the under-masculinized male and true hermaphroditism.² This contrasts with other classifications which include male and female pseudohermaproditism.⁴ A recent consensus statement from the International Intersex Consensus Conferences recommends using the term Disorder(s) of Sex Development (DSD) to encompass the myriad of conditions featuring varying types of sexual ambiguity.⁵ Table 1, adapted from this consensus statement, gives an example of the classification of DSD.

Sport

Historically, sport's first ‘encounter’ with DSD was in 1936 at the infamous Berlin Olympics, commonly known as Hitler's Olympics. The lead up to the Berlin Olympics had been blighted by Hitler's attempts to disadvantage Jewish competitors, preventing them from training and issuing them with threats to discourage their entry to the competition. Controversy continued during the games, most notably in the women's 100-metre sprint. Stella Walsh, a Polish-born athlete with US citizenship, and Helen Stephens, an American sprinter born in Missouri, had competed fiercely in previous competitions – Walsh won the 100 metre sprint in 1932 and therefore attended Berlin as defending champion. Stephens finished just in front of Walsh, posting a world record time of 11.5 seconds; Walsh completed the sprint in 11.7 seconds. Physically, both women appeared virilized, with muscle patterns and facial features more characteristic of the male sex (Figure 1). The sporting press nicknamed Stella Walsh ‘Stella the Fella’. Indeed, fellow athletes noted that she always changed by herself and isolated herself from her competitors.

Figure 1. Stella Walsh and Helen Stephens

 
Rumours circulated the Games that both Walsh and Stephens were men, competing with the wrong sex to gain unfair advantage. Walsh, in fact, publicly accused Stephens of being male after she won the 100-metre sprint in 1936. Since no formal gender verification program existed at this time, the Olympic committee felt compelled to perform a sex check on Stephens. This sex test was a crude physical examination involving the gross inspection of the external genitalia; it confirmed Stephens possessed female external genitalia.

The truth of their rivalry only came to light decades later following Walsh's murder – she was shot and killed in the cross-fire of an armed bank robbery in Los Angeles in 1980. A post-mortem examination confirmed that Walsh possessed ambiguous genitalia and abnormal sex chromosomes, although the exact DSD was not established.

In the following 30 years, the sporting media speculated that several other female athletes had DSDs because they possessed physical attributes which would generally be associated with the male sex. Still without formal gender verification, these rumours remained as such, fuelled by the media who were fully aware that there would be never be any scientific evidence to disprove them. Thus, journalists reported that genetically male Eastern Bloc athletes were binding their genitals and competing as females. Gender controversy also surrounded Irina and Tamara Press, two Russian sisters (Figure 2) who were dominant in a variety of female track and field events during the 1950s and 1960s. They won 26 world records and six Olympic gold medals.

Figure 2. Irina and Tamara Press

 
As media hype reached fever pitch, compulsory gender verification in the form of a gynaecological examination was introduced prior to the 1966 European athletics championship. In these so-called ‘nude parades’, athletes were forced to stand naked in front of a committee and were subjected to an inspection of their external gentalia. 243 women attended for examination and no abnormalities were reported. Neither of the Press sisters attended and they were never to appear in athletic competition again. Their absence was widely interpreted as evidence they both possessed abnormal external genitalia. It is still not known whether the Press sisters deliberately misrepresented their gender or, as seems more likely, they both had a DSD.

Gender verification for female athletes

Following the introduction of gender verification in 1960, it became increasingly clear that inspection of external genitalia was not a satisfactory or acceptable process. For the 1968 Olympics, Barr body detection was introduced and was widely proclaimed to be the solution to gender misrepresentation in sport. This reportedly ‘simpler, objective and more dignified’⁶ test involved the cytological analysis of a buccal smear. The Barr body was first detected by Murray Barr in 1948 during research on the nervous system of cats – cells were analysed following electrical stimulation and a dark staining body was found in the nucleus of some animals and not others. The distinction was found to be related to sex and a similar finding was noted in human autopsies. The findings were published in Nature in 1949 and the nuclear marking became known as the Barr Body.⁷ This Barr body is only found in cells with XX sex chromosomes, and represents a chromatin clump which occurs as a consequence of deactivation of one of the paired sex chromosomes.

Ewa Klobukowska, a Polish sprinter, became the first woman to be disqualified from sport following the introduction of the Barr body test. She was stripped of her medals and publicly chastised. It is believed that she had XX/XXY mosaicism. In the eyes of the regulatory bodies, the case of Klobukowska and others justified gender verification and enthusiasm for compulsory testing continued. In reality, however, the introduction of Barr body analysis created more problems than it solved – confirming or refuting sex purely via a chromosomal test fails to take account of the complexities of sex determination itself. Women with complete androgen insensitivity syndrome would have been barred from competing due to the presence of XY sex chromosomes despite an entirely female phenotype. Men with Klinefelter's (XXY) would be eligible to compete as females due to the presence of the Barr body on cytological analysis and would potentially gain clear sporting advantage because of their physique.

Advances in technology introduced polymerase chain reaction (PCR) amplification into the arena of gender verification. In 1991, Barr body analysis was replaced with PCR analysis for the SRY locus. The SRY gene was previously considered essential for differentiation of the indifferent foetal gonad into the male testis ( Figure 3).² It is now accepted that other genes are prerequisites for the normal development of the testes and male internal and external genitalia.⁸ Indeed, certain 46XX individuals have testes but do not possess the SRY gene, implying that male differentiation of the indifferent foetal gonad can be induced by alternative sex determining genes alone.⁸ Ovarian differentiation is also likely to be an active rather than passive process, induced by genes that have not yet been determined.⁹

Figure 3. Differentiation of the foetal gonad. Adapted from Hughes.2

 
The PCR analysis for the SRY locus was performed on DNA extracted from buccal smears. All women were screened in Olympic competition from 1992 onwards, with over 2000 tests performed at the 1992 Barcelona games. Fifteen tests were reported positive, with a further eight out of over 3000 positive tests at the Atlanta games in 1996. Of the eight athletes found to have the SRY locus in Atlanta, seven had androgen insensitivity syndrome and the final athlete had 5-alpha-reductase deficiency. All athletes were allowed to compete in their respective competition. The morphological status of those whose tested positive in Barcelona is strictly confidential and it is not known whether these athletes were allowed to compete.¹⁰

Notably, gender testing in athletics has never identified an individual deliberately misrepresenting their gender.^11,12 Testing has, however, created controversy and embarrassment for a significant number of female athletes competing, often unknowingly, with some form of intersex disorder. Indeed, there is no evidence that female athletes with DSDs have displayed any sports-relevant physical attributes which have not been seen in biologically normal female athletes.^6,12 However, numerous female athletes have been unfairly barred from competing. Concerns regarding gender verification after the introduction of DNA extraction techniques for the 1992 Barcelona Olympics culminated in a renowned Spanish geneticist refusing to assist with gender verification at the 1992 Olympics for this reason.^11,13 Despite this, the International Olympic Committee (IOC) were determined to persist with gender verification in the 1992 Winter Olympics in Albertville, citing the rarity of disorders such as XX men as justification for ongoing DNA testing.¹⁴

Suspension of gender verification

The decision to abandon compulsory gender verification in Olympic competition was taken in 1999, following many years of debate. Although initially taken as a temporary stance, this policy continues at present. The IOC has the authority to request gender verification on an individual basis if there is suspicion of masquerading. No complaints have been voiced by competing nations regarding possible gender misrepresentation.

As our understanding of gender and sexual identify increased in the late 20th century, it became increasingly apparent to scientists and athletes alike that determination of sex is derived from far more than our genotype. In the words of Eric Vilain:

Gender testing was initially welcomed by female athletes as a method of preventing ‘cheaters’. However, it has become apparent that the discrimination against those with DSD was unfair and detrimental to the sport.

Conclusion

DSDs are a hugely complex group of conditions. These abnormalities challenge both our scientific and social understanding of what ‘sex’ and ‘sexual differentiation’ are. The management of DSDs is challenging; the traditional approach bases sex assignment around future reproductive potential, future sexual potential and the cosmetic appearance of the external genitalia.¹⁶ Recent neuroscience research suggests that sexual dimorphism of the brain may occur prenatally, implying that gender-typical behaviour may be determined prior to sex assignment at birth. A more flexible approach to DSD management, involving parental decision making and close liaison with a child psychiatrist, is currently suggested.¹⁶

Sport has struggled with the issue of gender anomalies for years and the controversy regarding how to ‘test’ for DSD remains. Chromosomes can be tested but sex is not so easily determined – our upbringing and society's attitude towards us plays a crucial role in defining sex. For those female athletes with DSD, it seems far more likely that they are doing their best to compete as the sex chosen for them at birth rather than attempting to attain unfair advantage through masquerading their gender. As such, compulsory gender verification seems unfair, humiliating and unproductive in the majority of situations, although vigilance must remain to identify those whose aim is to win no matter what the cost.

Footnotes

Funding No funding or sponsorship was received for this article

Ethical approval Not applicable

Guarantor RR will act as guarantor for this article, accepting full responsibility for the work and conduct of the article

Contributorship TL provided the original idea for the article, provided specific information on athletes with DSDs and provided the images. RR wrote the article with assistance from JR. JR reviewed and corrected the article

Acknowledgements

This article arose as a result of a presentation at the Sir Geoffrey Chisholm Communication Prize (May 2007) of the Urology section of the RSM: The presentation was awarded the runner-up prize

References

2. Hughes I. Intersex. BJU Int 2002;90:769–76 [Medline]

3. Sax L. How common is intersex? a response to Anne Fausto-Sterling. J Sex Res 2002;39:174–8 [Medline]

4. Frimberger D, Gearhart J. Ambiguous genitalia and intersex. Urol Int 2005;75:291–7 [Medline]

5. Lee P, Houk C, Ahmed S, Hughes I. Consensus statement on management of intersex disorders. International Consensus Conference on Intersex. Pediatrics 2006;118:e488–500 [Free Full Text]

6. Elsas L, Ljungqvist A, Ferguson-Smith M, et al. Gender verification of female athletes. Genet Med 2000;2:249–54 [Medline]

7. Barr M, Eg B. A morphological distinction between neurones of the male and female, and the behaviour of the nucleolar satellite during accelerated nucleoprotein synthesis. Nature 1949;163:676–7 [Medline]

8. Cotinot C, Pailhoux E, Jaubert F, Fellous M. Molecular genetics of sex determination. Semin Reprod Med 2002;20:157–68 [Medline]

9. Yao H. The pathway to femaleness: current knowledge on embryonic development of the ovary. Mol Cell Endocrinol 2005;230:87–93 [Medline]

10. Serrat A, García de Herreros A. Determination of genetic sex by PCR amplification of Y-chromosome-specific sequences. Lancet 1993;341:1593 [Medline]

11. Ferguson-Smith M. Olympic row over sex testing. Nature 1992;355:10 [Medline]

12. Dickinson B, Genel M, Robinowitz C, Turner P, Woods G. Gender verification of female Olympic athletes. Med Sci Sports Exerc 2002;34:1539–42 Discussion 2002;34:43 [Medline]

13. Anderson C. Genetic testing. Olympic row over sex testing. Nature 1991;353:784

14. Dingeon B, Hamon P, Robert M, Schamasch P, Pugeat M. Sex testing at the Olympics. Nature 1992;358:447

15. Vilain E. Gender Blender: Intersexual? Transsexual? Male, female aren't so easy to define. Los Angeles Times 2004

16. Reiner W. Assignment of sex in neonates with ambiguous genitalia. Curr Opin Pediatr 1999;11:363–5 [Medline]

Source - Journal of The Royal Society of Medicine

***********************

Sexual development of the humans

Why Two Sexes Are Better Than One? >>
Concept about sexual dimorphism >>
Physiological process of the formation of the male's and female individuals >>
Human pathologies of sexual development >>

The Evolutionary Theory of Sex - Why Two Sexes Are Better Than One?

First organisms on the Earth were propagating itself vegetativelly or by gemmation. The Bacteria continue so and today. Plants have saved such way of reproduction. However, there are a sexual reproduction at all highest plants, and all highest animals, reptilies, fishes and birds, as it has a number of advantages which are necessary for organisms of a higher level of evolution.

It is necessary to explain. As was said in item 2, mutations in process of the genes recombination are excluded. But it is executed only in a case when a foetus is female's, as both cooperating sexual chromosomes are X ("female's"). At males of the class Mammalia the second chromosome has a type Y, and consequently she is not capable to compensate all mutations in X-chromosome. And when male appear, his genotype has the mutated genes. The majority of mutations are harmful, and such males perish, not passing a poor-quality genotype to posterity. But some mutations are useful, they have an adaptative characteristic, i.e. they cause such new properties, which help the individual to adapt better to the changed conditions of environment. Such males survive and pass the improved genotype to posterity. When female posterity receive such genes, they are fixed in a population.

Concept about sexual dimorphism

Thus, females are a stable conservative part of a population, her basic part, and males are an operative subsystem of a species, a vanguard of the evolution of the biological species. Females and males performs the different functions in a nature. For example, the destruction even plenties of males will not result in destruction of all population, while the destruction of a plenty of females will result in a crisis of population . Therefore nature has disposed so, that males instinctively sacrifice oneself for the sake of a population, for example, males first meet external danger, protect herd from predators and first perish. For realization of this function the males have abilities distinguishing them from females. It is called "a sexual dimorphism". These abilities are mental, physical, physiological. For example, males should be strong and courageous that they could perform this function.
Each animal species has the specificity, and sexual dimorphism at different species can qualitative and quantitatively differ.

At primates the sexual dimorphism is expressed strongly. Besides, the human children have the large brain and the large head, therefore human female should have a wide pelvis, that the foetus has not hurt a brain at birth.

Psychological differences depend from social learning (from the gender stereotypes) and inherent potentials. The gender stereotypes in the majority have an origin from inherent potentials of males and females. In a nature the males and females of primates differ, and the same differences have parallels at the humans. For example, it is known that at the women the speech abilities are more advanced than at the men. At the primate females also have the greater talkativeness [see Sex differences in vocal communication among adult rhesus macaques - Evolution and Human Behavior (official Journal)]. The frequent dialogue with others females helps them to nurse for posterity. But the men have more mathematical abilities (this specificity more begins to be shown at the beginning of puberty). It is more strongly shown at the decision of the complicated tasks and tasks demanding the non-trivial decision. The boys older 8-10 y.o. (when the sexual glands begin to secrete /to produce/ sexual hormones slightly more) have propensity to physically active pastime, but the girls prefer dolls, the male youngs of primates are inclined to power (force) commotion too, and the female youngs of primates help the adults females to look after for younger. Males are more mobile than females, the research instinct is shown in it. The infantile age boys cry at occurrence of novelty of a situation, it is shown research ability to distinguish new stimulus (i.e. reaction of the boys is "research"). The infantile age girls cry when there is a threat of deprivation of communication, it is shown a "communicative reaction. It corresponds tool and expressive styles which are gender-typical for men and women (Bendas T. The gender psychology, 2006).

Males of primates are interested in toys, by which does the boys are interested too, and females of primates are interested in toys, by which does the girl are interested. [see Sex differences in response to children's toys in nonhuman primates - Evolution and Human Behavior (official Journal)]. It are the inherent programs of behaviour, caused by natural functions of the males and females.

The presence of androgenes makes the men more vigorous and results in the large charge of energy. In time unit the man's organism even of the same weight will execute more vigorous physical work than women. But the female organism has more fatty fabric and spends less energy, therefore at very long moderate physical loadings the endurance of the female organism is higher. The fatty fabric also helps the woman to swim on water more long period of time.

Physiological process of the formation of the male's and female individuals

The male's foetus has karyotype XY, the female foetus has karyotype XX. Both foetuss at first execute the female program of the development. If at this time at blood the sexual hormones will not be present, foetus will be a female's. However at some moment at the male's foetus the chromosome Y begin to give an effect which begins to form male's glands at the foetus. The male's sexual hormones (androgenes), which are secreted by these glands, act on all developing organism and brain and develop a foetus for a male's type. They form a male's brain and his specificity, their lack results to the unsufficient masculinity. Even if the foetus will have karyotype XX, the presence of the androgenes will result in formation at him of a male's brain, physiology and psychology, and partially will generate foetus male primary sexual attributes at a genetic female.

Human pathologies of sexual development

As the statement indicates, sexual hormones have determinative meaning (importance) in development human organism. The researches have shown that the gender identification at the human has the inherent mechanism and is adjusted hormonally. [see Clinical case of David Reimer and Developmental Endocrine Influences on Gender Identity]. The heightened contents of andronenes in the girl organism results in formation of the male's somatictype, which is characterized by increase of length of the lower extremities , by increase of the shoulders width and by reduction of the width of a pelvis, and also reduction of fatty weight of a body and increase of muscular weight. The puberty is late (per 14 years lingers over are absent mammary gland and menstruations).

If the pregnant woman will have any infringements of a background of the sexual hormones, it can pathologically affect her future child. Female hormones (aestrogenes) and androgenes chemically differ from each other by one aromatic ring and can turn each other in tissues, therefore the increase of a level aestrogenes too works adversely on a female foetus, mascilinizating it. That is why nature has provided at a female foetus a very low level of the sexual hormones.

The taking of some medicines results in infringement of a hormone background at the mother. (M. Collaer, M. Hines, 1995).

There exists "complete androgen insensitivity syndrome" - cAIS). He is caused by infringement of several genes, which form the androgene receptors. In this case in spite of the fact that the foetus has karyotype XY, androgenes do not work on a body, then a body and brain are not developing as a male's type organs.

Most often the inherent hyperplasia of cortex of adrenal glands ("inherent adrenal glands cortex hyperplasia", now: "inherent adrenal glands cortex dysfunction") happens. He also is caused by defect of some genes. There is a virilizating form of this syndrom ("androgenital syndrom"). There are a lots of aestrogenes in female organismе, but also there are a small quantity of the andronenes. Androgenes is secreted by ovary(es) and by the adrenal glands cortex. At case of moderate lack of ferment "21-hydroxylase" the amount of andronenes at the girl is increased. Adrenal androgenes do not have an influence on internal female sexual organes, but only on external ones. Because of an anabolic action of the adrenal andronenes there is such clinical picture: the girl is fast (early) mature, early occurs sexual pilosis (for a male's type) or hirsutism, they have an advanced (full-blown) muscular system, wide shoulders and the relatively narrow pelvis. Sometimes such influence is almost imperceptible, but sometimes very strong (up to that the genetic girls accept on the boys, as their external genitals are similar to penis). (The textbook "Clinical endocrinology")

The hormone infringement at such girls results that they prefer of the boy's game (infringement of the program of a brain), are very physically active. Such girls often be "SheMales" and transsexuals, very often have a homosexual orientation. Outstripping the young persons of the same age in physical development, they often early become the outstanding sportswomen. In highest hogorts of the sports the masculinizated type of the women are found at 70-90% (Soboleva T. Problems of female sports). Under the physical characteristics they approximate to the men, but be not above than the sport men's average level.

see at greater length Sexual differentiation >>