Karyotype-specific ear and hearing problems in young adults with Turner syndrome and the effect of oxandrolone treatment

Otol Neurotol. 2014 Oct;35(9):1577-84. doi: 10.1097/MAO.0000000000000406.


Objective: To evaluate karyotype-specific ear and hearing problems in young-adult patients with Turner syndrome (TS) and assess the effects of previous treatment with oxandrolone (Ox).

Study design: Double-blind follow-up study.

Setting: University hospital.

Patients: Sixty-five TS patients (mean age, 24.3 yr) previously treated with growth hormone combined with placebo, Ox 0.03 mg/kg per day, or Ox 0.06 mg/kg per day from the age of 8 years and estrogen from the age of 12 years.

Intervention: Ear examination was performed according to standard clinical practice. Air- and bone conduction thresholds were measured in decibel hearing level.

Main outcome measures: We compared patients with total monosomy of the short arm of the X chromosome (Xp), monosomy 45,X and isochromosome 46,X,i(Xq), with patients with a partial monosomy Xp, mosaicism or other structural X chromosomal anomalies. We assessed the effect of previous Ox treatment.

Results: Sixty-six percent of the patients had a history of recurrent otitis media. We found hearing loss in 66% of the ears, including pure sensorineural hearing loss in 32%. Hearing thresholds in patients with a complete monosomy Xp were about 10 dB worse compared with those in patients with a partial monosomy Xp. Air- and bone conduction thresholds were not different between the placebo and Ox treatment groups.

Conclusion: Young-adult TS individuals frequently have structural ear pathology, and many suffer from hearing loss. This indicates that careful follow-up to detect ear and hearing problems is necessary, especially for those with a monosomy 45,X or isochromosome 46,X,i(Xq). Ox does not seem to have an effect on hearing.

Publication types

  • Randomized Controlled Trial
  • Research Support, Non-U.S. Gov't

MeSH terms

  • Adolescent
  • Adult
  • Anabolic Agents / administration & dosage
  • Anabolic Agents / adverse effects*
  • Double-Blind Method
  • Drug Therapy, Combination
  • Female
  • Follow-Up Studies
  • Hearing
  • Hearing Loss / epidemiology*
  • Hearing Loss / genetics
  • Hearing Loss, Sensorineural / genetics
  • Hearing Tests
  • Human Growth Hormone / administration & dosage
  • Humans
  • Karyotype
  • Karyotyping
  • Oxandrolone / administration & dosage
  • Oxandrolone / adverse effects*
  • Turner Syndrome / complications*
  • Turner Syndrome / drug therapy
  • Turner Syndrome / genetics
  • Young Adult


  • Anabolic Agents
  • Human Growth Hormone
  • Oxandrolone