How significant is sensorineural hearing loss in primary Sjogren's syndrome? An individually matched case-control study

Objective. We evaluated whether sensorineural loss and vestibular abnormali ties are common in patients with primary Sjogren's syndrome (pSS) and wheth er such abnormalities: are clinically significant. Methods. In an individually matched case-control design, 48 patients with p SS underwent complete audiovestibular evaluation along with 48 age and sex matched individuals without otologic problems., Differences of > 20 dB betw een patient and control ears at any frequency tested were considered to be significant. Results. Significant differences in hearing loss were seen at 4000 Hz (6 vs 0 ears: p = 0.03) and at 8000 Hz (9 vs 0 ears; p = 0.003). Small differenc es in hearing acuity were also observed in the lower frequencies, but the a bsolute mean difference was < 3 dB. A decrease of at least 60 dB in hearing acuity at any frequency up to 4000 Hz was seen only in 3 elderly pSS patie nts. Abnormal brainstem auditory evoked responses were recorded in 7 patien ts and 5 controls. but no patient had retrocochlear lesions identified on m agnetic resonance imaging. Four patients in each group had abnormalities on electronystagmography. Conclusion. pSS is associated with sensorineural hearing loss affecting pre ferentially the high frequencies, but clinically significant defects are no t common. There is no evidence of retrocochlear disease or increased vestib ular involvement in pSS.