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Generation of Induced Pluripotent Stem Cells from Turner Syndrome 45XO Fetal Cells for Downstream Modelling of Neurological Deficits Associated with the Syndrome
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Generation of Induced Pluripotent Stem Cells from Turner Syndrome 45XO Fetal Cells for Downstream Modelling of Neurological Deficits Associated with the Syndrome

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Otologic disorders in Turner syndrome

E Bois1, M Nassar1, D Zenaty2

  • 1Service d'ORL pédiatrique, hôpital Robert-Debré, Paris, université Diderot Paris 7, AP-HP, 48, boulevard Sérurier, 75019 Paris, France.

European Annals of Otorhinolaryngology, Head and Neck Diseases
|September 25, 2017
PubMed

Insights

Patients with Turner syndrome (TS) experience frequent ear problems, including hearing loss and recurrent ear infections. Early and ongoing Ear, Nose, and Throat (ENT) care is essential for managing these pediatric otologic disorders in TS patients.

Area of Science:

  • Otolaryngology
  • Genetics
  • Pediatrics

Background:

  • Turner syndrome (TS) is associated with craniofacial abnormalities affecting Eustachian tube and velar function.
  • These anatomical differences increase the risk of acute otitis media in pediatric patients with TS.

Purpose of the Study:

  • To document the spectrum of pediatric otologic disorders in patients with Turner syndrome.
  • To analyze these disorders at their initial Ear, Nose, and Throat (ENT) consultation.

Main Methods:

  • Retrospective review of ENT consultation data for pediatric TS patients (2005-2015).
  • Evaluated otoscopy, hearing thresholds, and history of otitis media or ENT surgery.
  • Compared data across different TS karyotypes (e.g., 45,X, mosaic, isochromosome).

Main Results:

  • Ninety TS patients (mean age 11.9 years) were analyzed.
  • High prevalence of tympanic abnormalities (29%), hearing loss (21%), and recurrent acute otitis media (24%).
  • Common ENT surgeries included adenoidectomy (18%) and T-tube insertion (24%).

Conclusions:

  • Pediatric patients with Turner syndrome exhibit a high incidence of otologic conditions.
  • Continuous and vigilant ENT monitoring is crucial for this population.
Abstract

Keywords:
CholesteatomaHearing lossOtitisTurner syndrome

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