Sexual function in women with Turner syndrome
Abstract
BACKGROUND: Turner syndrome (TS) (45,X) is associated with premature ovarian failure, usually occurring during childhood. Estrogen treatment is required for the development of secondary sexual characteristics and to maintain feminization. The present study aimed to determine the level of sexual activity in adult women with TS participating as volunteers in the NIH natural history study. METHODS: The Derogatis Interview for Sexual Functioning was administered to 98 women, average age 37 +/- 10 years (range 18-59) with karyotype-proven TS. Results were compared to gender-based norms, expressed as standardized area T-scores. RESULTS: The average total score was 32 +/- 12, approximately at the 10th centile, based on nonpatient community respondents. The survey included independent sections on fantasy, arousal, experience, orgasm, and relationship. Scores on sexual fantasy were in the normal range and were significantly greater than scores on all the other sections (p < 0.001). Approximately 30% of the study group were involved in a partner relationship, and this group scored within the average range on all sections. We examined factors that might influence sexual function, including age; parental origin of the single normal X chromosome; physical stigmata, such as neck webbing; estrogen use; testosterone levels; age of puberty; and hearing loss, but none of these appeared to contribute. Of all factors investigated, only height and years of education were positively correlated with partnership status and sexual function. In summary, women with TS in a partner relationship report relatively normal overall sexual function, but the majority of unpartnered women reported very low level sexual functioning. CONCLUSIONS: Further study is needed to elucidate the nature of psychosocial impediments affecting establishment of partner relations and sexual functioning in women with TS.
Integrity signals
Registry
Cites
Statistics
Claims the paper makes
Claims other papers make about this one
Each is checked against this paper’s own text: does it say what the citing paper says it does?
Despite having normal sex drive/libido, individuals with TS are less likely to be married or in relationships compared to women of the same age.
“Despite having normal sex drive/libido, individuals with TS are less likely to be married or in relationships as compared to women of the same age.371,372,569,570”
From Clinical practice guidelines for the care of girls and women with Turner syndrome. (2024) · cites this paper as [372]
Cites
Mattick, R. P., & Clarke, J. C. (1998) · Behaviour Research and Therapy · doi:10.1016/s0005-7967(97)10031-6 · Cited by 3,367 · see citing works
Nielsen, J., & Wohlert, M. (1991) · Human Genetics · doi:10.1007/bf01213097 · Cited by 1,193 · see citing works
Derogatis, L. R., Rosen, R. C., Leiblum, S. R., Burnett, A. L. “., & Heiman, J. R. (2002) · Journal of Sex & Marital Therapy · doi:10.1080/00926230290001448 · Cited by 795 · see citing works
Evidence from Turner's syndrome of an imprinted X-linked locus affecting cognitive function
Skuse, D. H., James, R. S., Bishop, D. V. M., Coppin, B., Dalton, P., Aamodt-Leeper, G., Bacarese-Hamilton, M., Creswell, C., McGurk, R., & Jacobs, P. A. (1997) · Nature · doi:10.1038/42706 · Cited by 747 · see citing works
Rosenfeld, R. G. (1990) · Cited by 505 · see citing works
Spontaneous Pubertal Development in Turner's Syndrome
Pasquino, A. M., Passeri, F., Pucarelli, I., Segni, M., & Municchi, G. (1997) · The Journal of Clinical Endocrinology & Metabolism · doi:10.1210/jcem.82.6.3970 · Cited by 385 · see citing works
Goldstat, R., Briganti, E. M., Tran, J., Wolfe, R. S., & Davis, S. R. (2003) · Menopause The Journal of The North American Menopause Society · doi:10.1097/01.gme.0000060256.03945.20 · Cited by 296 · see citing works
The derogatis interview for sexual functioning (DISF/DISF-SR): An introductory report
Derogatis, L. R. (1997) · Journal of Sex & Marital Therapy · doi:10.1080/00926239708403933 · Cited by 287 · see citing works
Lippe, B. M. (1991) · Endocrinology and Metabolism Clinics of North America · doi:10.1016/s0889-8529(18)30284-6 · Cited by 252 · see citing works
Dennerstein, L., Lehert, P., & Burger, H. (2005) · Fertility and Sterility · doi:10.1016/j.fertnstert.2005.01.119 · Cited by 235 · see citing works
Follicles Are Found in the Ovaries of Adolescent Girls with Turner's Syndrome
Hreinsson, J. G., Otala, M., Fridström, M., Borgström, B., Rasmussen, C., Lundqvist, M., Tuuri, T., Simberg, N. H., Mikkola, M., Dunkel, L., et al. (2002) · The Journal of Clinical Endocrinology & Metabolism · doi:10.1210/jcem.87.8.8753 · Cited by 192 · see citing works
Persistent cognitive deficits in adult women with Turner syndrome
ROSS, J. L., Stefanatos, G. A., Kushner, H., Zinn, A. R., Bondy, C. A., & Roeltgen, D. P. (2002) · Neurology · doi:10.1212/wnl.58.2.218 · Cited by 188 · see citing works
Self-citationShyness, social anxiety, and impaired self-esteem in Turner syndrome and premature ovarian failure
Schmidt, P. J., Cardoso, G. M. P., Ross, J. L., Haq, N., Rubinow, D. R., & Bondy, C. A. (2006) · JAMA · doi:10.1001/jama.295.12.1374 · Cited by 163 · see citing works
Self-citationPsychosocial and sexual functioning in women with Turner syndrome
Pavlidis, K., McCauley, E., & Sybert, V. P. (1995) · Clinical Genetics · doi:10.1111/j.1399-0004.1995.tb03929.x · Cited by 154 · see citing works
Shyness: relationship to social phobia and other psychiatric disorders
Heiser, N. A., Turner, S. M., & Beidel, D. C. (2003) · Behaviour Research and Therapy · doi:10.1016/s0005-7967(02)00003-7 · Cited by 149 · see citing works
Cognitive profile of Turner syndrome
Hong, D. S., Kent, J. S., & Kesler, S. R. (2009) · Developmental Disabilities Research Reviews · doi:10.1002/ddrr.79 · Cited by 131 · see citing works
McCauley, E., Ito, J. R., & KAY, T. (1986) · Journal of the American Academy of Child Psychiatry · doi:10.1016/s0002-7138(09)60606-3 · Cited by 112 · see citing works
Gravholt, C. H., Svenstrup, B., Bennett, P., & Christiansen, J. S. (1999) · Clinical Endocrinology · doi:10.1046/j.1365-2265.1999.00720.x · Cited by 103 · see citing works
Dimensions of Rosenburg's Self-Esteem Scale
Hensley, W. E., & Roberts, M. K. (1976) · Psychological Reports · doi:10.2466/pr0.1976.38.2.583 · Cited by 99 · see citing works
The Turner Syndrome–Associated Neurocognitive Phenotype Maps to Distal Xp
ROSS, J. L., Roeltgen, D. P., Kushner, H., Wei, F., & Zinn, A. R. (2000) · The American Journal of Human Genetics · doi:10.1086/303039 · Cited by 96 · see citing works
Sex determination and the Y chromosome
McElreavey, K., & Fellous, M. (1999) · American Journal of Medical Genetics · doi:10.1002/(sici)1096-8628(19991229)89:4 · Cited by 93 · see citing works
Current and lifetime psychiatric illness in women with Turner syndrome
Cardoso, G., Daly, R. J., Haq, N., Hanton, L., Rubinow, D. R., Bondy, C. A., & Schmidt, P. J. (2004) · Gynecological Endocrinology · doi:10.1080/09513590400021227 · Cited by 80 · see citing works
Self-citationMaternal X Chromosome, Visceral Adiposity, and Lipid Profile
Van, P. L., Bakalov, V. K., Zinn, A. R., & Bondy, C. A. (2006) · JAMA · doi:10.1001/jama.295.12.1373 · Cited by 75 · see citing works
Self-citationLawrence, K., Campbell, R., Swettenham, J., Terstegge, J., Akers, R., Coleman, M., & Skuse, D. (2003) · Neuropsychologia · doi:10.1016/s0028-3932(03)00002-2 · Cited by 74 · see citing works
The Importance of Estrogen Replacement in Young Women with Turner Syndrome
Hanton, L., Axelrod, L., Bakalov, V. K., & Bondy, C. A. (2003) · Journal of Women s Health · doi:10.1089/154099903322643893 · Cited by 68 · see citing works
Self-citationLack of personality pathology in Turner's syndrome: Relation to cytogenetics, hormones and physique
Money, J., & Mittenthal, S. (1970) · Behavior Genetics · doi:10.1007/bf01067370 · Cited by 65 · see citing works
Verlinde, F., Massa, G., Lagrou, K., Froidecoeur, C., Bourguignon, J.-P., Craen, M., Schepper, J. D., Caju, M. D., Heinrichs, C., François, I., et al. (2004) · Hormone Research in Paediatrics · doi:10.1159/000080099 · Cited by 65 · see citing works
ROSS, J. L., Stefanatos, G. A., Kushner, H., Bondy, C. A., Nelson, L. M., Zinn, A. R., & Roeltgen, D. P. (2004) · The Journal of Clinical Endocrinology & Metabolism · doi:10.1210/jc.2003-031463 · Cited by 56 · see citing works
Self-citationPsychological well-being in women with Turner syndrome: Somatic and social correlates
Boman, U. W., Bryman, I., & Möller, A. (2004) · Journal of Psychosomatic Obstetrics & Gynecology · doi:10.1080/01674820400017855 · Cited by 41 · see citing works
The Quality of Life of Turner Women in Comparison with Grown-Up GH-Deficient Women.
Okada, Y. (1994) · Endocrine Journal · doi:10.1507/endocrj.41.345 · Cited by 22 · see citing works
Declining Estrogen Use in Young Women With Turner Syndrome
Bondy, C. A., Ceniceros, I., Lange, E., & Bakalov, V. K. (2006) · Archives of Internal Medicine · doi:10.1001/archinte.166.12.1322-a · Cited by 16 · see citing works
Self-citationShyness Situations: Perspectives of a Diverse Sample of Shy Females
Watson, A. K., & Cheek, J. M. (1986) · Psychological Reports · doi:10.2466/pr0.1986.59.3.1040 · Cited by 10 · see citing works
Psychiatric Phenotypes Associated with Neurogenetic Disorders
Feinstein, C., & Chahal, L. (2009) · Psychiatric Clinics of North America · doi:10.1016/j.psc.2008.12.001 · Cited by 7 · see citing works
Pines, A., Weisman, Y., Ayalon, D., & Shapira, I. (2005) · PubMed · Cited by 2 · see citing works
Flagged first, then by citations
Cited by
Sorted by citations
Gravholt, C. H., Andersen, N. H., Conway, G. S., Dekkers, O. M., Geffner, M. E., Klein, K. O., Lin, A. E., Mauras, N., Quigley, C. A., Rubin, K., et al. (2017) · European Journal of Endocrinology · doi:10.1530/eje-17-0430 · Cited by 1,101 · see citing works
Psychological and Interpersonal Dimensions of Sexual Function and Dysfunction
McCabe, M. P., Althof, S. E., Assalian, P., Chevret‐Méasson, M., Leiblum, S. R., Simonelli, C., & Wylie, K. R. (2010) · The Journal of Sexual Medicine · doi:10.1111/j.1743-6109.2009.01618.x · Cited by 594 · see citing works
Psychological and interpersonal dimensions of sexual function and dysfunction
Brotto, L., Atallah, S., Johnson-Agbakwu, C., Rosenbaum, T., Abdo, C., Byers, E. S., Graham, C., Nobre, P., & Wylie, K. (2016) · The Journal of Sexual Medicine · doi:10.1016/j.jsxm.2016.01.019 · Cited by 439 · see citing works
Turner syndrome: mechanisms and management
Gravholt, C. H., Viuff, M. H., Brun, S., Stochholm, K., & Andersen, N. H. (2019) · Nature Reviews Endocrinology · doi:10.1038/s41574-019-0224-4 · Cited by 356 · see citing works
Clinical practice guidelines for the care of girls and women with Turner syndrome.
Gravholt, C. H., Andersen, N. H., Christin-Maitre, S., Davis, S. M., Duijnhouwer, A., Gawlik, A., Maciel-Guerra, A. T., Gutmark-Little, I., Fleischer, K., Hong, D., et al. (2024) · European Journal of Endocrinology · doi:10.1093/ejendo/lvae050 · Cited by 224 · see citing works
Estrogen replacement in Turner syndrome: literature review and practical considerations
Klein, K. O., Rosenfield, R. L., Santen, R. J., Gawlik, A. M., Backeljauw, P. F., Gravholt, C. H., Sas, T. C. J., & Mauras, N. (2018) · The Journal of Clinical Endocrinology & Metabolism · doi:10.1210/jc.2017-02183 · Cited by 151 · see citing works
Cognitive profile of Turner syndrome
Hong, D. S., Kent, J. S., & Kesler, S. R. (2009) · Developmental Disabilities Research Reviews · doi:10.1002/ddrr.79 · Cited by 131 · see citing works
Davis, S. R., Worsley, R., Miller, K. K., Parish, S. J., & Santoro, N. F. (2016) · The Journal of Sexual Medicine · doi:10.1016/j.jsxm.2015.12.033 · Cited by 124 · see citing works
Davis, S. R., & Braunstein, G. D. (2012) · The Journal of Sexual Medicine · doi:10.1111/j.1743-6109.2011.02634.x · Cited by 121 · see citing works
The role of steroid hormones in the sexual differentiation of the human brain
Bakker, J. (2021) · Journal of Neuroendocrinology · doi:10.1111/jne.13050 · Cited by 87 · see citing works
Lin, A. E., Prakash, S. K., Andersen, N. H., Viuff, M. H., Levitsky, L. L., Rivera‐Davila, M., Crenshaw, M. L., Hansen, L., Colvin, M. K., Hayes, F. J., et al. (2019) · American Journal of Medical Genetics Part A · doi:10.1002/ajmg.a.61310 · Cited by 50 · see citing works
Care of girls and women with Turner syndrome: beyond growth and hormones
Culen, C., Ertl, D., Schubert, K., Bartha, L., & Haeusler, G. (2017) · Endocrine Connections · doi:10.1530/ec-17-0036 · Cited by 49 · see citing works
The Sexual Differentiation of the Human Brain: Role of Sex Hormones Versus Sex Chromosomes
Bakker, J. (2018) · Current topics in behavioral neurosciences · doi:10.1007/7854_2018_70 · Cited by 49 · see citing works
Kreukels, B. P. C., Cohen‐Kettenis, P. T., Roehle, R., van de Grift, T. C., Słowikowska‐Hilczer, J., der Grinten, H. L. C., Hirschberg, A. L., de Vries, A. L. C., Reisch, N., Bouvattier, C. M., et al. (2019) · Journal of Sex & Marital Therapy · doi:10.1080/0092623x.2019.1610123 · Cited by 49 · see citing works
Ros, C., Alobid, I., Balasch, J., Mullol, J., & Castelo-Branco, C. (2013) · American Journal of Obstetrics and Gynecology · doi:10.1016/j.ajog.2013.01.011 · Cited by 47 · see citing works
A 6‐year Follow‐up survey of health status in middle‐aged women with Turner syndrome
Fjermestad, K. W., Næss, E. E., Bahr, D., & Gravholt, C. H. (2016) · Clinical Endocrinology · doi:10.1111/cen.13068 · Cited by 33 · see citing works
Depression in Turner Syndrome: A Systematic Review
Morris, L. A., Tishelman, A. C., Kremen, J., & Ross, R. (2019) · Archives of Sexual Behavior · doi:10.1007/s10508-019-01549-1 · Cited by 30 · see citing works
Guntram, L. (2013) · Social Science & Medicine · doi:10.1016/j.socscimed.2013.09.018 · Cited by 26 · see citing works
Menke, L. A., Sas, T., Visser, M., Kreukels, B. P. C., Stijnen, T., Zandwijken, G. R. J., Keizer‐Schrama, S. M. P. F. D. M., Otten, B. J., Wit, J. M., & Cohen‐Kettenis, P. T. (2010) · Hormones and Behavior · doi:10.1016/j.yhbeh.2009.12.011 · Cited by 16 · see citing works
Sexuality and quality of life in congenital hypogonadisms
Oyarzún, M. F. G., & Castelo‐Branco, C. (2016) · Gynecological Endocrinology · doi:10.1080/09513590.2016.1241229 · Cited by 14 · see citing works
Dowlut-McElroy, T., & Shankar, R. K. (2022) · Journal of Pediatric and Adolescent Gynecology · doi:10.1016/j.jpag.2022.02.002 · Cited by 11 · see citing works
Davies, W. (2021) · European Journal of Medical Genetics · doi:10.1016/j.ejmg.2021.104169 · Cited by 11 · see citing works
Sex Differentiation: Organizing Effects of Sex Hormones
Bakker, J. (2013) · Focus on sexuality research · doi:10.1007/978-1-4614-7441-8_1 · Cited by 10 · see citing works
Attard, C. C., Cameron‐Pimblett, A., Puri, D., Rosa, C. L., Talaulikar, V. S., Davies, M. C., Learner, H. I., Liao, L., & Conway, G. S. (2020) · Clinical Endocrinology · doi:10.1111/cen.14271 · Cited by 10 · see citing works
Guntram, L. (2017) · Health An Interdisciplinary Journal for the Social Study of Health Illness and Medicine · doi:10.1177/1363459317693403 · Cited by 9 · see citing works
Psychiatric Phenotypes Associated with Neurogenetic Disorders
Feinstein, C., & Chahal, L. (2009) · Psychiatric Clinics of North America · doi:10.1016/j.psc.2008.12.001 · Cited by 7 · see citing works
Backeljauw, P. F., Chernausek, S. D., Gravholt, C. H., & Kruszka, P. S. (2021) · Elsevier eBooks · doi:10.1016/b978-0-323-62520-3.00017-8 · Cited by 5 · see citing works
Guntram, L. (2014) · Linköping University Electronic Press eBooks · doi:10.3384/diss.diva-1111100 · Cited by 4 · see citing works
Saenger, P. H., & Bondy, C. A. (2014) · Elsevier eBooks · doi:10.1016/b978-1-4557-4858-7.00025-1 · Cited by 4 · see citing works
Self-citationTarantino, C., Vincenzi, L., Angelini, F., Tomaselli, A., Carlomagno, F., Rosato, E., Pofi, R., Lenzi, A., Pozza, C., Minnetti, M., et al. (2025) · Journal of Endocrinological Investigation · doi:10.1007/s40618-024-02521-0 · Cited by 2 · see citing works
The Role of Pubertal Hormones in the Development of Gender Identity: fMRI Studies
Burke, S., & Bakker, J. (2014) · Research and perspectives in endocrine interactions · doi:10.1007/978-3-319-09168-6_3 · Cited by 2 · see citing works
Hormone replacement in disorders of sex development, and long-term effects
Nordenström, A., Mangone, A., & Mantovani, G. (2025) · Best Practice & Research Clinical Endocrinology & Metabolism · doi:10.1016/j.beem.2025.102022 · Cited by 1 · see citing works
Turner Syndrome: Primary Amenorrhea from Adolescence to Aging
Castelo‐Branco, C., & Naumova, I. (2019) · Springer eBooks · doi:10.1007/978-3-030-13936-0_2 · Cited by 1 · see citing works
Bondy, C. A. (2010) · Springer eBooks · doi:10.1007/978-1-4419-1436-1_19 · Cited by 1 · see citing works
Self-citationPregnancy, Childbirth, Puerperium, Breastfeeding, and Sexuality in the World of Rare Diseases
Rodríguez-Blanque, R., Sánchez-García, J. C., & Cortés‐Martín, J. (2023) · Healthcare · doi:10.3390/healthcare11091351 · Cited by 1 · see citing works
Jenseits von Hormonen und Wachstum – psychosoziale Hürden bei Turner-Syndrom
Culen, C., Ertl, D., & Haeusler, G. (2018) · Journal für Klinische Endokrinologie und Stoffwechsel · doi:10.1007/s41969-018-0019-6
Estrogen Replacement in Turner Syndrome
Klein, K. O., Rosenfield, R. L., Santen, R. J., Gawlik, A., Backeljauw, P. F., Gravholt, C. H., Sas, T., & Mauras, N. (2020) · Springer eBooks · doi:10.1007/978-3-030-34150-3_5
Turner Sendromunda Fertilite Durumu ve İnfertilite Hemşiresinin Rolleri
Öngen, M., & BAŞAR, F. (2020) · Bandırma Onyedi Eylül Üniversitesi Sağlık Bilimleri ve Araştırmaları Dergisi · doi:10.46413/boneyusbad.792458
Bartel, W. P., Accordino, R. E., Awidi, B., & Prager, L. M. (2024) · Springer eBooks · doi:10.1007/978-3-031-76269-7_11
Dowlut‐McElroy, T., Hyunh, V. O., Roth, L. W., Ross, M. E., Priebe, A., Maher, J. Y., & Gomez‐Lobo, V. (2025) · Journal of Pediatric and Adolescent Gynecology · doi:10.1016/j.jpag.2025.09.002
Endocrine Disorders in Adolescence
Ranke, M. B. (2013) · Springer eBooks · doi:10.1007/978-1-4614-6633-8_41
Alteracions neurosensorials en dones adultes amb Síndrome de Turner
Cerro, C. R. (2013) · LA Referencia (Red Federada de Repositorios Institucionales de Publicaciones Científicas)
Fertility preservation in patients with Turner syndrome: a SWOT analysis
Ferro, R., Vento, F. D., & Streuli, I. (2025) · Reproductive BioMedicine Online · doi:10.1016/j.rbmo.2025.105438
de Paillette, E., Karila, D., Rousseau, A., Dubost, E., Meykiechel, T., Lamothe, S., Vatier, C., Jaillard, S., Nedelcu, M., Bourcigaux, N., et al. (2026) · Sexual Medicine · doi:10.1093/sexmed/qfag074
- Authors
- Sheaffer, A. T., Lange, E., & Bondy, C. A.
- Affiliations
- National Institutes of Health (US)
- ORCID
- Lange, Eileen
- Venue
- Journal of Women s Health
- Publisher
- SAGE Publications
- Published
- 2008-01
- Volume
- 17
- Issue
- 1
- Pages
- 27-33
- ISSN
- 1059-7115 (ISSN-L)
- 1540-9996
- 1931-843X
- OpenAlex
- W2062261334
- Cited by
- 45
- References
- 34
- Citations by year
- 2026: 1 · 2025: 4 · 2024: 2 · 2023: 1 · 2022: 1 · 2021: 3 · 2020: 3 · 2019: 5 · 2018: 3 · 2017: 3 · 2016: 4 · 2014: 3 · 2013: 6 · 2012: 1
- FWCI
- 0.95
- Type
- journal article
- Language
- en
- Open access
- closed
- Locations
- Journal of Women s Health (publishedVersion)
- PubMed (publishedVersion)
- Funders
- National Institutes of Health
- Topics
- Life Sciences › Biochemistry, Genetics and Molecular Biology › Genetics › Genetic and Clinical Aspects of Sex Determination and Chromosomal Abnormalities
- Health Sciences › Medicine › Psychiatry and Mental health › Sexual function and dysfunction studies
- Life Sciences › Biochemistry, Genetics and Molecular Biology › Molecular Biology › Sexual Differentiation and Disorders
- Keywords
- Turner syndrome, sexual function, sexual activity, sexual fantasies, orgasm, couple relationships, premature ovarian failure, estrogen therapy, testosterone levels, psychosocial factors, adult women
- MeSH terms
- Adult, Anxiety, Female, Humans, Libido, Middle Aged, Surveys and Questionnaires, Self Concept, Sexual Dysfunction, Physiological, Sexual Partners, Shyness, Testosterone, Turner Syndrome, United States, Comorbidity, Women's Health, Sexual Dysfunctions, Psychological
About these checks
Registry, retraction, Cites and statistics results are computed, not judged, with one exception: a printed reference that no index lists is read once by a model before it counts as a citation error. Claims are extracted by a model and kept only when their quote appears verbatim in the text; each cited source is then checked, with a model’s help, for whether it says what the claim relies on. Those verdicts can be wrong — read the claim’s quote and the cited source before relying on one.
Statistics and arithmetic are recomputed, not judged.
Confidence bands are shown only for fields calibrated on our annotated test set. None is calibrated yet, so no band is shown.
What counts as a claim ›How we measure confidence ›How we check this ›
Are you an author, or do you see a problem with this page? Request a review
