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Related Experiment Video

Updated: Jul 18, 2026

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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Hearing loss in Turner syndrome.

Noriko Morimoto1, Toshiaki Tanaka, Hidenobu Taiji

  • 1Department of Otolaryngology, National Center for Child Health and Development, Tokyo, Japan. morimoto-n@ncchd.go.jp

The Journal of Pediatrics
|November 11, 2006
PubMed
Summary

High-frequency sensory neural hearing loss (HFQ-SNHL) affects over 60% of Turner syndrome (TS) patients. Hearing loss progression is linked to aging and karyotype, necessitating regular ear examinations.

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Area of Science:

  • Otolaryngology
  • Genetics
  • Audiology

Background:

  • Turner syndrome (TS) is a genetic condition affecting females.
  • Hearing impairment is a recognized complication in TS.
  • Understanding the specific characteristics of hearing loss in TS is crucial for management.

Purpose of the Study:

  • To characterize hearing loss in patients with Turner syndrome (TS).
  • To identify potential causative factors associated with hearing impairment in TS.
  • To analyze the relationship between hearing loss, karyotype, and aging in TS.

Main Methods:

  • Audiological measurements were performed on 33 patients with TS (ages 8-40).
  • Otoacoustic emissions (DPOAE and TEOAE) were used to assess cochlear function.
  • Correlation analysis was conducted to explore relationships between hearing thresholds, age, body height, karyotype, and clinical history.

Main Results:

  • Over 60% of TS patients exhibited high-frequency sensory neural hearing loss (HFQ-SNHL) at 8 kHz.
  • HFQ-SNHL was not strongly related to middle ear infections or puberty history.
  • Hearing thresholds at high frequencies correlated significantly with age and body height.
  • Age-dependent hearing decline was more pronounced in TS patients with monosomic 45,X karyotype compared to mosaic types.

Conclusions:

  • High-frequency sensory neural hearing loss is prevalent in Turner syndrome.
  • Karyotype and aging are significant factors influencing the progression of hearing loss in TS.
  • Regular otological examinations are recommended for early detection and management of hearing loss in TS patients.