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

Autor: Dominique Smeets, Henri J L M Timmers, Vedat Topsakal, Sabine M.P.F. de Muinck Keizer-Schrama, Ronald J.E. Pennings, Ronald J.C. Admiraal, Jan M. Wit, Leonie A. Menke, K. Freriks, Henricus P. M. Kunst, Ad R. M. M. Hermus, Patrick L. M. Huygen, Theo C J Sas, Janiëlle A E M van Alfen-van der Velden, Eva J J Verver, Barto J. Otten
Přispěvatelé: General Paediatrics, Amsterdam Reproduction & Development (AR&D), Surgical clinical sciences, Ear, nose & throat
Jazyk: angličtina
Rok vydání: 2014
Předmět:
Human Growth Hormone/administration & dosage
Pediatrics
Turner Syndrome/complications
Vascular damage Radboud Institute for Health Sciences [Radboudumc 16]
Sensory disorders Radboud Institute for Health Sciences [Radboudumc 12]
Audiology
Sensory disorders Donders Center for Medical Neuroscience [Radboudumc 12]
surgery
Oxandrolone
Anabolic Agents
Hearing
Turner syndrome
Cholesteatoma
Human Growth Hormone
Hearing Tests
Vascular damage Radboud Institute for Molecular Life Sciences [Radboudumc 16]
Sensory Systems
young adult
Sensorineural hearing loss
Drug Therapy
Combination
Female
medicine.symptom
Hearing Loss
Sensorineural/genetics
medicine.drug
Adult
medicine.medical_specialty
Monosomy
Adolescent
Hearing loss
Hearing Loss/epidemiology
Hearing Loss
Sensorineural
Oxandrolone/administration & dosage
Isochromosome
Karyotype
Other Research Donders Center for Medical Neuroscience [Radboudumc 0]
Hearing impairment
Bone conduction
Double-Blind Method
medicine
otorhinolaryngologic diseases
Humans
Hearing Loss
business.industry
Other Research Radboud Institute for Health Sciences [Radboudumc 0]
Anabolic Agents/administration & dosage
medicine.disease
Otorhinolaryngology
Karyotyping
Neurology (clinical)
business
Follow-Up Studies
Zdroj: Otology and Neurotology, 35(9), 1577-1584. Lippincott Williams and Wilkins
Otology and Neurotology, 35(9), 1577-1584
Otology & Neurotology, 35, 9, pp. 1577-84
Otology & Neurotology, 35, 1577-84
ISSN: 1531-7129
Popis: Item does not contain fulltext 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.
Databáze: OpenAIRE
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