title: Evaluation of hearing organ in patients with Turner syndrome.
abstract: Hearing loss and middle ear diseases are often reported in some of Turner patients. In most of the reports hearing organ was evaluated with the use of subjective methods. The aim of the work was subjective and objective evaluation of hearing organ with an attempt to set the correlation between the results and the genotype of the patients with Turner syndrome (Ts).
  
  
  METHODS
  51 Ts patients aged 14.3 years on average. There were 29 girls with monosomy X and 22 having mosaicism. A detailed medical history was taken in each case with attention given to the hearing loss risk factors.
  
  
  METHODS
  Physical ENT examination, hearing evaluation: pure tone audiometry, impedance audiometry, distortion products otoacoustic emissions (DPOAEs), brain auditory evoked potentials (BAEP). The control group consisted of 30 healthy patients.
  
  
  RESULTS
  Recurrent acute otitis media was reported by 19.6% of Ts patients. Pure tone audiometry was improper in 36.3% ears; conductive hearing loss was present in 11.7% ears, mixed hearing loss in 5.9% ears and the moderate sensorineural hearing loss in 18.6% ears. Impedance audiometry was impaired in 14.7% of the cases. DPOAE disturbances were present in 41.4% of Ts patients, BAEP was improper in 52.0%. The percentage of the disturbances in DOPAEs and in BAEP in patients with mosaicism was 45.4 and 40.9% while in patients with monosomy 68.9 and 62%.
  
  
  CONCLUSIONS
  Ts patients present predisposition to hearing disturbances. The disturbances seem to be connected with middle ear infections and with sensorineural hearing losses. Hearing loss in Ts women is not clinically apparent in most of the cases; this fact reflects the need of early evaluation and further monitoring of hearing organ in those patients. Sensorineural hearing loss seems to prevail in patients with 45,X genotype, so perhaps attention should be paid to this subgroup of Ts patients.
authors.affiliation: University of Wroclaw, Faculty of Medicine ENT Department, ul. Chałubinskiego 2, 50-368 Wroclaw, Poland. wgawron@interia.pl
authors.forename: Wojciech
authors.initials: W
authors.lastname: Gawron
authors.forename: Beata
authors.initials: B
authors.lastname: Wikiera
authors.forename: Beata
authors.initials: B
authors.lastname: Rostkowska-Nadolska
authors.forename: Krystyna
authors.initials: K
authors.lastname: Orendorz-Fraczkowska
authors.forename: Anna
authors.initials: A
authors.lastname: Noczyńska
mesh_terms: D000158:Acoustic Impedance Tests; D001301:Audiometry, Pure-Tone; D016057:Evoked Potentials, Auditory, Brain Stem; D005260:Female; D006309:Hearing; D006320:Hearing Tests; D006801:Humans; D010033:Otitis Media / Q000150:complications; D017084:Otoacoustic Emissions, Spontaneous; D014424:Turner Syndrome / Q000150:complications / Q000503:physiopathology*