title: Karyotype-specific ear and hearing problems in young adults with Turner syndrome and the effect of oxandrolone treatment.
abstract: 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).
  
  
  METHODS
  Double-blind follow-up study.
  
  
  METHODS
  University hospital.
  
  
  METHODS
  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.
  
  
  METHODS
  Ear examination was performed according to standard clinical practice. Air- and bone conduction thresholds were measured in decibel hearing level.
  
  
  METHODS
  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.
  
  
  CONCLUSIONS
  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.
authors.affiliation: *Department of Otorhinolaryngology and Head and Neck Surgery, Rudolf Magnus Institute of Neuroscience, University Medical Center Utrecht, Utrecht; †Departments of Otorhinolaryngology, Head and Neck Surgery, and ‡Medicine, Division of Endocrinology, Radboud University Nijmegen Medical Centre, Nijmegen; §Department of Pediatrics, Erasmus Medical Center/Sophia Children's Hospital, Rotterdam; ∥Department of Pediatrics, Albert Schweitzer Hospital, Dordrecht; ¶Department of Human Genetics, Radboud University Nijmegen Medical Centre, Nijmegen; #Department of Pediatrics, Leiden University Medical Center, Leiden; and **Department of Pediatrics, Radboud University Nijmegen Medical Centre, Nijmegen, The Netherlands.
authors.forename: Eva J J
authors.initials: EJ
authors.lastname: Verver
authors.forename: Kim
authors.initials: K
authors.lastname: Freriks
authors.forename: Theo C J
authors.initials: TC
authors.lastname: Sas
authors.forename: Patrick L M
authors.initials: PL
authors.lastname: Huygen
authors.forename: Ronald J E
authors.initials: RJ
authors.lastname: Pennings
authors.forename: Dominique F C M
authors.initials: DF
authors.lastname: Smeets
authors.forename: Ad R M M
authors.initials: AR
authors.lastname: Hermus
authors.forename: Leonie A
authors.initials: LA
authors.lastname: Menke
authors.forename: Jan M
authors.initials: JM
authors.lastname: Wit
authors.forename: Barto J
authors.initials: BJ
authors.lastname: Otten
authors.forename: Janiëlle A E M
authors.initials: JA
authors.lastname: van Alfen-van der Velden
authors.forename: Sabine M P F
authors.initials: SM
authors.lastname: de Muinck Keizer-Schrama
authors.forename: Vedat
authors.initials: V
authors.lastname: Topsakal
authors.forename: Ronald J C
authors.initials: RJ
authors.lastname: Admiraal
authors.forename: Henri J L M
authors.initials: HJ
authors.lastname: Timmers
authors.forename: Henricus P M
authors.initials: HP
authors.lastname: Kunst
mesh_terms: D000293:Adolescent; D000328:Adult; D045930:Anabolic Agents / Q000008:administration & dosage / Q000009:adverse effects*; D004311:Double-Blind Method; D004359:Drug Therapy, Combination; D005260:Female; D005500:Follow-Up Studies; D006309:Hearing; D034381:Hearing Loss / Q000453:epidemiology* / Q000235:genetics; D006319:Hearing Loss, Sensorineural / Q000235:genetics; D006320:Hearing Tests; D019382:Human Growth Hormone / Q000008:administration & dosage; D006801:Humans; D059785:Karyotype; D007621:Karyotyping; D010074:Oxandrolone / Q000008:administration & dosage / Q000009:adverse effects*; D014424:Turner Syndrome / Q000150:complications* / Q000188:drug therapy / Q000235:genetics; D055815:Young Adult
chemical_list: D045930:Anabolic Agents; D019382:Human Growth Hormone; D010074:Oxandrolone