TY - JOUR
T1 - Karyotype-specific ear and hearing problems in young adults with turner syndrome and the effect of oxandrolone treatment
AU - Verver, Eva J.J.
AU - Freriks, Kim
AU - Sas, Theo C.J.
AU - Huygen, Patrick L.M.
AU - Pennings, Ronald J.E.
AU - Smeets, Dominique F.C.M.
AU - Hermus, Ad R.M.M.
AU - Menke, Leonie A.
AU - Wit, Jan M.
AU - Otten, Barto J.
AU - Van Alfen-Vander Velden, Janiëlle A.E.M.
AU - De Muinck Keizer-Schrama, Sabine M.P.F.
AU - Topsakal, Vedat
AU - Admiraal, Ronald J.C.
AU - Timmers, Henri J.L.M.
AU - Kunst, Henricus P.M.
PY - 2014
Y1 - 2014
N2 - 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.
AB - 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.
KW - Adult
KW - Cholesteatoma
KW - Hearing impairment
KW - Karyotype
KW - Oxandrolone
KW - Turner syndrome
UR - http://www.scopus.com/inward/record.url?scp=84914115018&partnerID=8YFLogxK
U2 - https://doi.org/10.1097/MAO.0000000000000406
DO - https://doi.org/10.1097/MAO.0000000000000406
M3 - Article
C2 - 25133471
SN - 1531-7129
VL - 35
SP - 1577
EP - 1584
JO - Otology and Neurotology
JF - Otology and Neurotology
IS - 9
ER -