当前位置： SCI文献检索 > INTERNATIONAL JOURNAL OF PEDIATRIC OTORHINOLARYNGOLOGY期刊下所有文献 > Fluctuant, progressive hearing loss associated with Menière like vertigo in three patients with the Pendred syndrome.

Fluctuant, progressive hearing loss associated with Menière like vertigo in three patients with the Pendred syndrome.

Abstract：

OBJECTIVE:To evaluate vestibular and long-term audiometric findings in patients with Pendred syndrome. STUDY DESIGN:Retrospective analysis of long-term clinical data. SETTING:University hospital department. PATIENTS:Three patients with Pendred syndrome caused by a mutation in the SLC26A4 gene. METHODS:Perchlorate discharge test, mutation analysis of the SLC26A4 gene, MR imaging of temporal bones, vestibular function test (in two cases) and serial audiometry. A saturation hyperbola with onset age was fitted to the audiometric threshold-on-age data using a nonlinear regression method. The residues remaining after regression were analyzed in a correlation analysis to detect significant ipsilateral or contralateral cofluctuation. RESULTS:All three patients had a mutation in the SLC26A4 gene and bilateral enlarged vestibular aqueduct; two of them had a positive perchlorate discharge test but in one of two siblings this test was negative. Hearing loss was significantly progressive with significant ipsilateral and contralateral cofluctuation in all evaluable cases, combined with episodes of Menière like vertigo in two cases. The episodes of vertigo are as seen in Menière disease. One case had unilateral caloric areflexia and one had bilateral vestibular hyporeflexia, proven to be progressive in a repeat examination. CONCLUSIONS:Patients with Pendred syndrome may exhibit progressive and fluctuant hearing loss with episodes of vertigo.

journal_name

Int J Pediatr Otorhinolaryngol

journal_title

International journal of pediatric otorhinolaryngology

authors

Stinckens C,Huygen PL,Joosten FB,Van Camp G,Otten B,Cremers CW

doi

10.1016/s0165-5876(01)00573-0

subject

Has Abstract

pub_date

2001-12-01 00:00:00

pages

207-15

issue

3

eissn

0165-5876

issn

1872-8464

pii

S0165587601005730

journal_volume

61

pub_type

杂志文章

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