Audiological Abnormalities in Patients With Turner Syndrome Purpose The purpose of this study was to evaluate the prevalence, type, and severity of hearing impairment in patients with Turner syndrome (TS) and to determine whether these characteristics correlated with the patient karyotype and age. Method This was a cross-sectional prospective study. The participants underwent an audiological ... Research Article
Research Article  |   December 01, 2013
Audiological Abnormalities in Patients With Turner Syndrome
 
Author Affiliations & Notes
  • Conceição Silva Oliveira
    Institute of Health Sciences, Federal University of Bahia, Salvador-Bahia, Brazil
  • Francine Mendonça Ribeiro
    Centro de Endocrinologia e Diabetes do Estado da Bahia, Salvador-Bahia, Brazil
  • Renata Lago
    Genetics Services Unit, Hospital Universitário Prof. Edgard Santos, Federal University of Bahia, Salvador-Bahia, Brazil
  • Crésio Alves
    Pediatric Endocrinology Unit, Hospital Universitário Prof. Edgard Santos, Faculty of Medicine, Federal University of Bahia, Salvador-Bahia, Brazil
  • Disclosure: The authors have declared that no competing interests existed at the time of publication.
    Disclosure: The authors have declared that no competing interests existed at the time of publication.×
  • Correspondence to Crésio Alves: cresio.alves@uol.com.br
  • Editor: Sheila Pratt
    Editor: Sheila Pratt×
  • Associate Editor: Dana Boatman
    Associate Editor: Dana Boatman×
Article Information
Hearing Disorders / Special Populations / Genetic & Congenital Disorders / Research Articles
Research Article   |   December 01, 2013
Audiological Abnormalities in Patients With Turner Syndrome
American Journal of Audiology, December 2013, Vol. 22, 226-232. doi:10.1044/1059-0889(2013/11-0027)
History: Received August 23, 2011 , Revised December 8, 2011 , Accepted March 18, 2013
 
American Journal of Audiology, December 2013, Vol. 22, 226-232. doi:10.1044/1059-0889(2013/11-0027)
History: Received August 23, 2011; Revised December 8, 2011; Accepted March 18, 2013
Web of Science® Times Cited: 3

Purpose The purpose of this study was to evaluate the prevalence, type, and severity of hearing impairment in patients with Turner syndrome (TS) and to determine whether these characteristics correlated with the patient karyotype and age.

Method This was a cross-sectional prospective study. The participants underwent an audiological evaluation, including pure-tone audiometry, speech audiometry, and acoustic immittance measures.

Results This study included 52 patients with TS (mean age = 19 years, SD = 6 years, range = 7–37 years). The majority of these patients had a decline in pure-tone thresholds at 8000 Hz, and most also had mild to moderate hearing loss. Most patients had normal tympanometric results. More than 80% of patients had acoustic reflexes present at 500, 1000, and 2000 Hz frequencies bilaterally, but less than 50% of the patients displayed an acoustic reflex at 4000 Hz bilaterally. The hearing loss was more prevalent in patients with a 45,X karyotype and isochromosomes as compared to patients with mosaicism.

Conclusion The results showed that TS patients with a 45,X karyotype and isochromosomes with loss of the p-arm of the X chromosome had a greater risk of developing hearing loss than patients with mosaicism. There was a linear association between hearing loss and age in these patients.

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