Skip to main content
SpringerLink
Log in
Book cover

Turner Syndrome pp 185–197Cite as

Ear and Hearing Problems in Turner Syndrome

  • Chapter
  • First Online:

  • 749 Accesses

Abstract

Ear and hearing problems are frequent in women with Turner syndrome and have a negative effect on well-being and quality of life. Throughout life different periods of ear and hearing problems can be seen in girls and women with Turner syndrome. During childhood, there is a high incidence of otitis media resulting in a conductive hearing loss, frequent tympanostomy tube insertions, and antibiotic treatment. Women with Turner syndrome also have a higher incidence of chronic otitis and cholesteatoma over time. Sensorineural hearing loss in the form of a mid-frequency dip can be present as early as age 5 and progresses over the years. A high-frequency sensorineural hearing loss that develops in early adulthood is often present, and this can have a devastating effect on the hearing situation for the individual. In such cases, hearing aids are often required earlier than in the normal population. There is an increased risk for ear and hearing problems in all women with Turner syndrome, but it is more common in karyotype 45,X and 46,X,i(Xq). On an individual level, however, the phenotype penetration can vary which is why medical advice based on karyotype has to be carefully planned.

This is a preview of subscription content, log in via an institution.

Chapter
USD   29.95
Price excludes VAT (USA)
  • Available as PDF
  • Read on any device
  • Instant download
  • Own it forever
eBook
USD   109.00
Price excludes VAT (USA)
  • Available as EPUB and PDF
  • Read on any device
  • Instant download
  • Own it forever
Hardcover Book
USD   139.99
Price excludes VAT (USA)
  • Durable hardcover edition
  • Dispatched in 3 to 5 business days
  • Free shipping worldwide - see info

Tax calculation will be finalised at checkout

Purchases are for personal use only

Learn about institutional subscriptions

Abbreviations

Tfh cells:

T follicular helper cells

References

  1. Hultcrantz M. Ear and hearing problems in Turner’s syndrome. Acta Otolaryngol. 2003;123(2):253–7.

    Article  CAS  Google Scholar 

  2. Carel JC, Ecosse E, Bastie-Sigeac I, Cabrol S, Tauber M, Leger J, et al. Quality of life determinants in young women with Turner’s syndrome after growth hormone treatment: results of the StaTur population-based cohort study. J Clin Endocrinol Metab. 2005;90(4):1992–7.

    Article  CAS  Google Scholar 

  3. Verver EJ, Freriks K, Thomeer HG, Huygen PL, Pennings RJ, Alfen-van der Velden AA, et al. Ear and hearing problems in relation to karyotype in children with Turner syndrome. Hear Res. 2011;275(1–2):81–8.

    Article  CAS  Google Scholar 

  4. Stenberg AE, Nylen O, Windh M, Hultcrantz M. Otological problems in children with Turner’s syndrome. Hear Res. 1998;124(1–2):85–90.

    Article  CAS  Google Scholar 

  5. Sculerati N, Ledesma-Medina J, Finegold DN, Stool SE. Otitis media and hearing loss in Turner syndrome. Arch Otolaryngol Head Neck Surg. 1990;116(6):704–7.

    Article  CAS  Google Scholar 

  6. Hall JE, Richter GT, Choo DI. Surgical management of otologic disease in pediatric patients with Turner syndrome. Int J Pediatr Otorhinolaryngol. 2009;73(1):57–65.

    Article  Google Scholar 

  7. Lim DBN, Gault EJ, Kubba H, Morrissey MSC, Wynne DM, Donaldson MDC. Cholesteatoma has a high prevalence in Turner syndrome, highlighting the need for earlier diagnosis and the potential benefits of otoscopy training for paediatricians. Acta Paediatrica. 2014;103(7):e282–7.

    CAS  PubMed  Google Scholar 

  8. Davenport ML, Roush J, Liu C, Zagar AJ, Eugster E, Travers S, et al. Growth hormone treatment does not affect incidences of middle ear disease or hearing loss in infants and toddlers with Turner syndrome. Horm Res Paediatr. 2010;74(1):23–32.

    Article  CAS  Google Scholar 

  9. Roush J, Davenport ML, Carlson-Smith C. Early-onset sensorineural hearing loss in a child with Turner syndrome. J Am Acad Audiol. 2000;11(8):446–53.

    CAS  PubMed  Google Scholar 

  10. Cameron-Pimblett A, La Rosa C, King TFJ, Davies MC, Conway GS. The Turner syndrome life course project: karyotype-phenotype analyses across the lifespan. Clin Endocrinol. 2017;87(5):532–8.

    Article  CAS  Google Scholar 

  11. Hultcrantz M, Sylven L, Borg E. Ear and hearing problems in 44 middle-aged women with Turner’s syndrome. Hear Res. 1994;76(1–2):127–32.

    Article  CAS  Google Scholar 

  12. King KA, Makishima T, Zalewski CK, Bakalov VK, Griffith AJ, Bondy CA, et al. Analysis of auditory phenotype and karyotype in 200 females with Turner syndrome. Ear Hear. 2007;28(6):831–41.

    Article  Google Scholar 

  13. Hederstierna C, Hultcrantz M, Rosenhall U. Estrogen and hearing from a clinical point of view; characteristics of auditory function in women with Turner syndrome. Hear Res. 2009;252(1–2):3–8.

    Article  CAS  Google Scholar 

  14. Barrenas M, Landin-Wilhelmsen K, Hanson C. Ear and hearing in relation to genotype and growth in Turner syndrome. Hear Res. 2000;144(1–2):21–8.

    Article  CAS  Google Scholar 

  15. Anderson S, White-Schwoch T, Choi HJ, Kraus N. Training changes processing of speech cues in older adults with hearing loss. Front Syst Neurosci. 2013;7:97.

    Article  Google Scholar 

  16. Bonnard A, Hederstierna C, Bark R, Hultcrantz M. Audiometric features in young adults with Turner syndrome. Int J Audiol. 2017;56(9):650–6.

    Article  Google Scholar 

  17. Hederstierna C, Hultcrantz M, Collins A, Rosenhall U. Hearing in women at menopause. Prevalence of hearing loss, audiometric configuration and relation to hormone replacement therapy. Acta Otolaryngol. 2007;127(2):149–55.

    Article  Google Scholar 

  18. Hederstierna C, Hultcrantz M, Collins A, Rosenhall U. The menopause triggers hearing decline in healthy women. Hear Res. 2010;259(1–2):31–5.

    Article  CAS  Google Scholar 

  19. Johansson MSK, Arlinger SD. Hearing threshold levels for an otologically unscreened, non-occupationally noise-exposed population in Sweden: umbrales auditivos en una población no estudiada, sin exposición a ruido ocupacional en Suecia. Int J Audiol. 2009;41(3):180–94.

    Article  Google Scholar 

  20. Sculerati N, Oddoux C, Clayton CM, Lim JW, Oster H. Hearing loss in turner syndrome. Laryngoscope. 1996;106(8):992–7.

    Article  CAS  Google Scholar 

  21. Dhooge IJ, De Vel E, Verhoye C, Lemmerling M, Vinck B. Otologic disease in Turner syndrome. Otol Neurotol. 2005;26(2):145–50.

    Article  Google Scholar 

  22. Bois E, Nassar M, Zenaty D, Leger J, Van Den Abbeele T, Teissier N. Otologic disorders in Turner syndrome. Eur Ann Otorhinolaryngol Head Neck Dis. 2018;135(1):21–4.

    Article  CAS  Google Scholar 

  23. Bergamaschi R, Bergonzoni C, Mazzanti L, Scarano E, Mencarelli F, Messina F, et al. Hearing loss in Turner syndrome: results of a multicentric study. J Endocrinol Investig. 2008;31(9):779–83.

    Article  CAS  Google Scholar 

  24. Hall AJ, Maw R, Midgley E, Golding J, Steer C. Glue ear, hearing loss and IQ: an association moderated by the child’s home environment. PLoS One. 2014;9(2):e87021.

    Article  Google Scholar 

  25. Morimoto N, Tanaka T, Taiji H, Horikawa R, Naiki Y, Morimoto Y, et al. Hearing loss in Turner syndrome. J Pediatr. 2006;149(5):697–701.

    Article  Google Scholar 

  26. Barrenas ML, Nylen O, Hanson C. The influence of karyotype on the auricle, otitis media and hearing in Turner syndrome. Hear Res. 1999;138(1–2):163–70.

    Article  CAS  Google Scholar 

  27. Hultcrantz M, Sylvén L. Turner’s syndrome and hearing disorders in women aged 16–34. Hear Res. 1997;103(1–2):69–74.

    Article  CAS  Google Scholar 

  28. Anderson H, Filipsson R, Fluur E, Koch B, Lindsten J, Wedenberg E. Hearing impairment in Turner’s syndrome. Acta Otolaryngol. 1969;247(Suppl):1–26.

    Google Scholar 

  29. Rizell S, Barrenas ML, Andlin-Sobocki A, Stecksen-Blicks C, Kjellberg H. 45,X/46,XX karyotype mitigates the aberrant craniofacial morphology in Turner syndrome. Eur J Orthod. 2013;35(4):467–74.

    Article  Google Scholar 

  30. Cook KD, Shpargel KB, Starmer J, Whitfield-Larry F, Conley B, Allard DE, et al. T follicular helper cell-dependent clearance of a persistent virus infection requires T cell expression of the histone demethylase UTX. Immunity. 2015;43(4):703–14.

    Article  CAS  Google Scholar 

  31. Gravholt CH, Andersen NH, Conway GS, Dekkers OM, Geffner ME, Klein KO, et al. Clinical practice guidelines for the care of girls and women with Turner syndrome: proceedings from the 2016 Cincinnati International Turner Syndrome Meeting. Eur J Endocrinol. 2017;177(3):G1–g70.

    Article  CAS  Google Scholar 

  32. Simonoska R, Stenberg AE, Duan M, Yakimchuk K, Fridberger A, Sahlin L, et al. Inner ear pathology and loss of hearing in estrogen receptor-beta deficient mice. J Endocrinol. 2009;201(3):397–406.

    Article  CAS  Google Scholar 

  33. Meltser I, Tahera Y, Simpson E, Hultcrantz M, Charitidi K, Gustafsson JA, et al. Estrogen receptor beta protects against acoustic trauma in mice. J Clin Invest. 2008;118(4):1563–70.

    Article  CAS  Google Scholar 

  34. Stenberg AE, Wang H, Fish J 3rd, Schrott-Fischer A, Sahlin L, Hultcrantz M. Estrogen receptors in the normal adult and developing human inner ear and in Turner’s syndrome. Hear Res. 2001;157(1–2):87–92.

    Article  CAS  Google Scholar 

  35. Ross J, Roeltgen D, Zinn A. Cognition and the sex chromosomes: studies in Turner syndrome. Horm Res. 2006;65(1):47–56.

    CAS  PubMed  Google Scholar 

  36. Monzani D, Galeazzi GM, Genovese E, Marrara A, Martini A. Psychological profile and social behaviour of working adults with mild or moderate hearing loss. Acta Otorhinolaryngol Ital. 2008;28(2):61–6.

    CAS  PubMed  PubMed Central  Google Scholar 

  37. Gopinath B, Hickson L, Schneider J, McMahon CM, Burlutsky G, Leeder SR, et al. Hearing-impaired adults are at increased risk of experiencing emotional distress and social engagement restrictions five years later. Age Ageing. 2012;41(5):618–23.

    Article  Google Scholar 

  38. Laplante-Levesque A, Hickson L, Worrall L. Factors influencing rehabilitation decisions of adults with acquired hearing impairment. Int J Audiol. 2010;49(7):497–507.

    Article  Google Scholar 

  39. Phillips JS, Yung MW, Nunney I. Myringoplasty outcomes in the UK. J Laryngol Otol. 2015;129(9):860–4.

    Article  CAS  Google Scholar 

Download references

Author information

Authors and Affiliations

  1. Department of Clinical Science, Intervention and Technology, Division of Otorhinolaryngology, Karolinska Institutet, Stockholm, Sweden

    Åsa Bonnard & Malou Hultcrantz

  2. Department of Otorhinolaryngology, Karolinska University Hospital, Stockholm, Sweden

    Åsa Bonnard & Malou Hultcrantz

Authors
  1. Åsa Bonnard
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Malou Hultcrantz
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to Åsa Bonnard .

Editor information

Editors and Affiliations

  1. Division of Endocrinology, Seattle Children’s Hospital, University of Washington, Seattle, WA, USA

    Patricia Y. Fechner

Rights and permissions

Reprints and permissions

Copyright information

© 2020 Springer Nature Switzerland AG

About this chapter

Check for updates. Verify currency and authenticity via CrossMark

Cite this chapter

Bonnard, Å., Hultcrantz, M. (2020). Ear and Hearing Problems in Turner Syndrome. In: Fechner, P. (eds) Turner Syndrome. Springer, Cham. https://doi.org/10.1007/978-3-030-34150-3_10

Download citation

  • DOI: https://doi.org/10.1007/978-3-030-34150-3_10

  • Published:

  • Publisher Name: Springer, Cham

  • Print ISBN: 978-3-030-34148-0

  • Online ISBN: 978-3-030-34150-3

  • eBook Packages: MedicineMedicine (R0)

Publish with us

Policies and ethics

Search

Navigation