Document Detail

Audiovestibular manifestations in patients with limited systemic sclerosis and centromere protein-B (CENP-B) antibodies.
MedLine Citation:
PMID:  18520322     Owner:  NLM     Status:  MEDLINE    
Audiovestibular dysfunction has been reported in patients with connective tissue disease. Systemic sclerosis (SSc; scleroderma) is a rare connective tissue disease of unknown etiology. In the current study we assess whether audiovestibular involvement is present in patients with limited scleroderma (lSSc). To answer this question we studied a series of 35 consecutive patients who fulfilled well-established classification criteria for lSSc and had antibodies against the major centromere protein-B (CENP-B), and 59 matched controls. Individuals with a history of cerebrovascular complications, syphilis, Ménière and other vestibular syndromes, infections involving the inner ear, barotrauma, or in treatment with ototoxic drugs were excluded. The majority of patients with lSSc were women (94%). The mean age at time of study was 64.5 years, and the mean age at time of disease diagnosis was 56.9 years. Besides Raynaud phenomenon, most patients with lSSc had other typical features of CREST (calcinosis, Raynaud phenomenon, esophageal hypomotility, sclerodactyly, and telangiectasia) syndrome. Twenty-seven (77%) patients showed abnormal hearing loss in the audiogram compared with only 15 (26%) of the controls (p < 0.001). Values of audiometric tests (pure-tone average and speech reception threshold) yielded significant differences between patients and controls (p < 0.001). The typical pattern of hearing impairment in our series of lSSc patients was a bilateral and symmetrical sensorineural hearing loss with a flat pattern in the audiogram. Abnormal tympanogram and abnormal stapedial reflex were more commonly observed in patients than controls (p < or = 0.001). Similarly, a significantly increased frequency of abnormal oculocephalic response (10 patients, 29%) and head-shaking nystagmus (9 patients, 26%) was observed in patients compared with controls (p < 0.001 for both comparisons). Finally, a significantly increased frequency of abnormal caloric test and clinical test of sensory integration and balance was observed in lSSc patients (31% and 46% of patients, respectively) compared with controls (0% and 12%, respectively) (p < 0.001 for both comparisons). The current study demonstrates strong evidence for inner ear compromise in patients with lSSc.
Juan C Amor-Dorado; Maria C Arias-Nuñez; Jose A Miranda-Filloy; Carlos Gonzalez-Juanatey; Javier Llorca; Miguel A Gonzalez-Gay
Publication Detail:
Type:  Journal Article    
Journal Detail:
Title:  Medicine     Volume:  87     ISSN:  0025-7974     ISO Abbreviation:  Medicine (Baltimore)     Publication Date:  2008 May 
Date Detail:
Created Date:  2008-06-03     Completed Date:  2008-07-17     Revised Date:  2009-11-03    
Medline Journal Info:
Nlm Unique ID:  2985248R     Medline TA:  Medicine (Baltimore)     Country:  United States    
Other Details:
Languages:  eng     Pagination:  131-41     Citation Subset:  AIM; IM    
Division of Otolaryngology, School of Medicine, University of Cantabria, Santander, Spain.
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MeSH Terms
Autoantibodies / blood*
CREST Syndrome / complications,  diagnosis
Centromere Protein B / immunology*
Hearing Loss, Sensorineural / complications*,  diagnosis
Middle Aged
Nystagmus, Pathologic / complications,  diagnosis
Scleroderma, Systemic / complications*,  immunology
Vestibular Diseases / complications*,  diagnosis
Reg. No./Substance:
0/Autoantibodies; 0/Centromere Protein B

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