Ear and hearing problems in relation to karyotype in children with Turner syndrome

被引:28
作者
Verver, E. J. J. [1 ]
Freriks, K. [2 ]
Thomeer, H. G. X. M. [1 ]
Huygen, P. L. M. [1 ]
Pennings, R. J. E. [1 ]
Alfen-van der Velden, A. A. E. M. [3 ]
Timmers, H. J. [2 ]
Otten, B. J. [3 ]
Cremers, C. W. R. J. [1 ]
Kunst, H. P. M. [1 ]
机构
[1] Radboud Univ Nijmegen Med Ctr, Dept Otorhinolaryngol, NL-6500 HB Nijmegen, Netherlands
[2] Radboud Univ Nijmegen Med Ctr, Dept Endocrinol, NL-6500 HB Nijmegen, Netherlands
[3] Radboud Univ Nijmegen Med Ctr, Dept Pediat Endocrinol, NL-6500 HB Nijmegen, Netherlands
关键词
RECURRENCE RATES; OTOLOGIC DISEASE; OTITIS-MEDIA; CHOLESTEATOMA; WOMEN; PREVALENCE; FEATURES; ESTROGEN;
D O I
10.1016/j.heares.2010.12.007
中图分类号
R36 [病理学]; R76 [耳鼻咽喉科学];
学科分类号
100104 ; 100213 ;
摘要
The aim of the study was to report otologic and audiologic characteristics in a group of children with Turner syndrome (TS) and correlate these findings to karyotype. Additionally, we give recommendations for the otologic care of these children. Sixty children (age 1.7-21.2 years) were included in this retrospective study. Medical history and karyotypes were recorded and otologic and audiologic evaluation was performed. A history of recurrent otitis media was reported in 41/60 (68%) children and 3/60 (5%) had suffered from cholesteatoma. Audiometric data in 56 children revealed that normal hearing was only present in 33/112 (29%) ears. All other ears 79/112 (71%) were classified in five different audiometric categories for hearing loss. Hearing thresholds in general appeared to be about 10-11 dB worse in children with a monosomy 45,X or isochromosome (both have a total deletion of the short (p) arm of the X-chromosome) compared to those having a mosaicism or structural anomaly (partial deletion, or total deletion in only a few cells). Our findings support the hypothesis that hearing can be affected by loss of the p-arm of the X-chromosome. It is for the first time that a relation between hearing problems and karyotype is statistically confirmed in a large group of children with TS. (C) 2010 Elsevier B.V. All rights reserved.
引用
收藏
页码:81 / 88
页数:8
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