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

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

Author information

1
*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.

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).

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.

PMID:
25133471
DOI:
10.1097/MAO.0000000000000406
[Indexed for MEDLINE]

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