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.