Audiovestibular Manifestations in Patients With Limited Systemic Sclerosis and Centromere Protein-B (CENP-B) Antibodies

Abstract

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.

Auditory and vestibular dysfunctions in systemic sclerosis: literature review

“…Of the eight studies that investigated changes, all identified hearing loss in most individuals with SS, and the prevalence in this finding ranged from 20 to 77%. The type of hearing loss was bilateral sensorineural (6,(9)(10)(11)13,16,17) in most cases, corroborating the physiopathology of the systemic disease. However, mixed (11,15,17) and unilateral (13,17) hearing loss were also described in few cases.…”

“…Amor-Dorado et al (12,17) and Maciaszczyk et al (13) had differences in the study population with regard to the form of disease (limited or diffuse); however, only in the analysis by Amor-Dorado et al (12) results were presented by considering the type of disease. Prevalence of changes in the vestibular system was higher for patients with diffuse SS (Charts 1 and 2).…”

“…Only the studies by Berrettini et al (10) and Amor-Dorado et al (17) investigated auditory and vestibular involvement in patients with SS. However, six studies aimed at assessing or reporting only auditory changes in these patients (6,9,11,13,15,16) and only two (12,14) investigated vestibular dysfunctions (Chart 1).…”

“…As this information is very important because it will guide the auditory rehabilitation process among these individuals, it should be included while conducting new studies. AmorDorado et al (17) described the flat hearing loss configuration in 13 individuals and high-frequency hearing loss in 14, accounting for 27 patients with sensorineural hearing loss (SNHL). Deroee et al (16) observed high-frequency hearing loss in one of the ears and severe loss in another.…”

“…The most used study design for describing auditory and vestibular changes in individuals with SS was the crosssectional design, observed in five analyses (10)(11)(12)(13)(14) , followed by four case reports (6,9,15,16) , and one case-control study (17) . Because cross-sectional studies reported findings of a single moment, it was difficult to determine a temporal sequence between exposure to a specific factor and the subsequent development of the disease.…”