Literature DB >> 18343510

Evaluation of hearing organ in patients with Turner syndrome.

Wojciech Gawron1, Beata Wikiera, Beata Rostkowska-Nadolska, Krystyna Orendorz-Fraczkowska, Anna Noczyńska.   

Abstract

UNLABELLED: 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). MATERIAL: 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.
METHOD: 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.

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Year:  2008        PMID: 18343510     DOI: 10.1016/j.ijporl.2008.01.021

Source DB:  PubMed          Journal:  Int J Pediatr Otorhinolaryngol        ISSN: 0165-5876            Impact factor:   1.675


  6 in total

Review 1.  Current best practice in the management of Turner syndrome.

Authors:  Roopa Kanakatti Shankar; Philippe F Backeljauw
Journal:  Ther Adv Endocrinol Metab       Date:  2017-12-18       Impact factor: 3.565

2.  Complex X chromosome rearrangement associated with multiorgan autoimmunity.

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Journal:  Mol Cytogenet       Date:  2015-07-19       Impact factor: 2.009

3.  Cholesteatoma in Children with Sotos Syndrome.

Authors:  Colin Gerald Leonard; Sebastian Ranguis; Sharon Lynn Cushing; Susan Blaser; Adrian James
Journal:  J Int Adv Otol       Date:  2022-03       Impact factor: 1.316

4.  Surgical management of middle ear cholesteatoma in children with Turner syndrome: a multicenter experience.

Authors:  Diego Zanetti; Filippo Di Lella; Maurizio Negri; Vincenzo Vincenti
Journal:  Acta Biomed       Date:  2018-10-08

5.  Association between cytogenetic alteration and the audiometric profile of individuals with Turner syndrome.

Authors:  Martha Marcela de Matos Bazilio; Adriana Fernandes Duarte Dos Santos; Fernanda Gomes de Almeida; Silvana Frota; Marília Guimarães; Márcia Gonçalves Ribeiro
Journal:  Braz J Otorhinolaryngol       Date:  2020-04-27

Review 6.  Hearing loss among patients with Turner's syndrome: literature review.

Authors:  Cresio Alves; Conceição Silva Oliveira
Journal:  Braz J Otorhinolaryngol       Date:  2014 May-Jun
  6 in total

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