Ear and Hearing Problems in Turner Syndrome

  • Åsa BonnardEmail author
  • Malou Hultcrantz


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.


Turner syndrome Hearing Hearing impairment Hearing loss Otitis media Chronic otitis Audiologic rehabilitation 


Tfh cells

T follicular helper cells


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© Springer Nature Switzerland AG 2020

Authors and Affiliations

  1. 1.Department of Clinical Science, Intervention and Technology, Division of OtorhinolaryngologyKarolinska InstitutetStockholmSweden
  2. 2.Department of OtorhinolaryngologyKarolinska University HospitalStockholmSweden

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