Literature DB >> 18997489

Hearing loss in Turner syndrome: results of a multicentric study.

R Bergamaschi1, C Bergonzoni, L Mazzanti, E Scarano, F Mencarelli, F Messina, M Rosano, L Iughetti, A Cicognani.   

Abstract

UNLABELLED: The purpose of this article was to evaluate otological diseases in 173 patients (pts) with Turner syndrome (TS). STUDY
DESIGN: One hundred and seventy-three pts, mean chronological age (CA) 12+/-6.2 yr. Patients were submitted to different therapies: GH, estrogen therapy (EE), no therapy (no tx). Seventy-nine pts (CA 11 yr) had no otological diseases. Conductive hearing loss (CHL) occurred in 38.7% (CA 11 yr) and otoscopy was: persistent secretory otitis media in 55.2%, chronic otitis media in 10.4%, pars flaccida retraction pocket in 19.4%, mostly bilateral. Cholesteatoma was present in 15%. Sensorineurinal hearing loss (SNHL) occurred in 15.6% (CA 16 yr), 11 of whom were affected by high tone loss, and 15 by loss in midfrequencies (dip between 0.5-3 kHz), bilateral in 93%. Degree of hearing loss (HL) was mild [20-40 decibel hearing level (dBHL)] in 15%, moderate (45-60 dBHL) in 31%, severe (65-80 dBHL) in 8%, profound (dBHL>85) in 2%. We found a significant association between CHL and karyotype 45, X (p<0.025), congenital cranio-facial abnormalities, prevalently with low-set ears (p<0.04), narrow and/or high arched palate (p<0.018), and micrognathia (p<0.004). Our study confirms that the high prevalence of middle ear infections and CHL in TS are probably due to growth disturbances of the structures from the first and second branchial arches. We did not find any association between EE, GH, and HL. We recommend a regular audiological follow-up, especially during childhood, to prevent important middle ear anatomic sequele and to identify HL at an early stage, as the impact on social functioning may be significant.

Entities:  

Mesh:

Year:  2008        PMID: 18997489     DOI: 10.1007/BF03349257

Source DB:  PubMed          Journal:  J Endocrinol Invest        ISSN: 0391-4097            Impact factor:   4.256


  26 in total

1.  Final height of patients with Turner's syndrome treated with growth hormone (GH): indications for GH therapy alone at high doses and late estrogen therapy. Italian Study Group for Turner Syndrome.

Authors:  E Cacciari; L Mazzanti
Journal:  J Clin Endocrinol Metab       Date:  1999-12       Impact factor: 5.958

2.  Otitis media and hearing loss in Turner syndrome.

Authors:  N Sculerati; J Ledesma-Medina; D N Finegold; S E Stool
Journal:  Arch Otolaryngol Head Neck Surg       Date:  1990-06

3.  Hearing impairment in Turner's syndrome.

Authors:  H Anderson; R Filipsson; E Fluur; B Koch; J Lindsten; E Wedenberg
Journal:  Acta Otolaryngol       Date:  1969       Impact factor: 1.494

4.  The influence of karyotype on the auricle, otitis media and hearing in Turner syndrome.

Authors:  M L Barrenäs; O Nylén; C Hanson
Journal:  Hear Res       Date:  1999-12       Impact factor: 3.208

5.  Otologic disease in turner syndrome.

Authors:  Ingeborg J M Dhooge; E De Vel; C Verhoye; M Lemmerling; B Vinck
Journal:  Otol Neurotol       Date:  2005-03       Impact factor: 2.311

6.  Ear and hearing problems in 44 middle-aged women with Turner's syndrome.

Authors:  M Hultcrantz; L Sylvén; E Borg
Journal:  Hear Res       Date:  1994-06-01       Impact factor: 3.208

7.  Turner's syndrome and hearing disorders in women aged 16-34.

Authors:  M Hultcrantz; L Sylvén
Journal:  Hear Res       Date:  1997-01       Impact factor: 3.208

8.  Hearing in advanced age. A study of presbyacusis in 85-, 88- and 90-year-old people.

Authors:  R Jönsson; U Rosenhall
Journal:  Audiology       Date:  1998 Jul-Aug

9.  Otological problems in children with Turner's syndrome.

Authors:  A E Stenberg; O Nylén; M Windh; M Hultcrantz
Journal:  Hear Res       Date:  1998-10       Impact factor: 3.208

10.  Auditory brainstem responses after ovariectomy and estrogen replacement in rat.

Authors:  J R Coleman; D Campbell; W A Cooper; M G Welsh; J Moyer
Journal:  Hear Res       Date:  1994-11       Impact factor: 3.208

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  6 in total

Review 1.  Cognitive profile of Turner syndrome.

Authors:  David Hong; Jamie Scaletta Kent; Shelli Kesler
Journal:  Dev Disabil Res Rev       Date:  2009

Review 2.  Malformation syndromes associated with disorders of sex development.

Authors:  John M Hutson; Sonia R Grover; Michele O'Connell; Samuel D Pennell
Journal:  Nat Rev Endocrinol       Date:  2014-06-10       Impact factor: 43.330

3.  Multidisciplinary Approach to the Child with Sex Chromosomal Mosaicism Including a Y-Containing Cell Line.

Authors:  Bauke Debo; Marlies Van Loocke; Katya De Groote; Els De Leenheer; Martine Cools
Journal:  Int J Environ Res Public Health       Date:  2021-01-21       Impact factor: 3.390

4.  Association Between Endocrine Diseases and Serous Otitis Media in Children.

Authors:  Murat Koçyiğit; Taliye Çakabay; Safiye G Örtekin; Teoman Akçay; Güven Özkaya; Selin Üstün Bezgin; Melek Yıldız; Mustafa Kemal Adalı
Journal:  J Clin Res Pediatr Endocrinol       Date:  2016-09-09

5.  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

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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