Extended high-frequency audiometry as early detection of hearing loss in primary Sjögren syndrome

Abstract

The aim of the study is to evaluate hearing function by extended high-frequency audiometry in primary Sjögren Syndrome (pSS) and to correlate it with disease activity. All patients diagnosed with pSS were recruited within 1 year. Patients underwent otologic evaluation, extended high-frequency audiometry, tympanogram, and the ESSPRI scale evaluation. A total of 63 (60 female and 3 male) patients with mean age 49.5 ± 9.9 years with pSS were included. We detected a hearing loss prevalence of 95.2% (60 patients) in both ears in very high frequencies (10000-16,000 Hz). The hearing loss of all patients was sensorineural. There was no significant difference between the degree of disease activity and the hearing thresholds. We enrolled 188 subjects with a mean age of 47.20 ± 9.7 years as a healthy control group. We compared the hearing thresholds of both ears of patients with pSS versus the healthy control group and observed a significant difference in frequencies above 500 Hz. Sensorineural hearing loss was found in pSS patients in frequencies above 4000 Hz. Sensorineural hearing loss was highly prevalent in pSS patients, predominantly in very high frequencies. We claim that sensorineural hearing loss could be an associated symptom of primary Sjögren syndrome and suggest further research to support this conclusion.

Ulcerative colitis may be a risk factor for sensorineural hearing loss

“…AEID was first described in 1979 (15) and has since been associated with many autoimmune diseases including Wegener's granulomatosis, Sjogren's syndrome, Behcet's disease, rheumatoid arthritis, and systemic lupus erythematosus (16). In various autoimmune diseases, it has been determined that SNHL is seen especially at high frequencies (17,18).…”

Brainstem auditory evoked potentials in patients with primary Sjögren’s syndrome without central nervous system involvement

“…Hearing damage and neuropathy of the eighth cranial nerve are very rare manifestations of pSS. They can occur in up to 25% of patients with pSS, but the exact frequency of these abnormalities is unknown [3][4][5].…”

Evaluation of Medial Olivocochlear Efferent System and Hearing Loss in Patients with Primary Sjögren's Syndrome

“…There is little research on the pathophysiological impact that pSS may have on the human auditory system. While there are no robust findings about the effects of pSS, especially for the outer ear and middle ear, the possible reasons for auditory symptoms are thought to be dryness of the ear canal epidermis, middle and inner ear fluids, and dysfunction of the Eustachian tube [5,6]. On the other hand, there are many hypotheses about the pathophysiological impact of pSS on the inner ear.…”

“…The inner ear is defined as a sensory organ that contains perilymph and endolymph fluid and it transduces sound waves into senses of hearing to be used by the auditory neural parts. These neurosensory functions are tightly bound to the regulation of molecules in the perilymph and endolymph fluid volume in the inner ear [6]. Change in the volume of the inner ear fluids can disrupt the molecular structure of the fluids and cause hearing loss.…”

“…Additionally, ototoxic drugs used during the treatment of autoimmune inner ear diseases can cause the damage of intracellular fluids and change the molecular concentrations for physiological mechanisms. Furthermore, autoantibodies are produced in perilymph and endolymph fluids in normal hearing physiology, but in diseases such as pSS they are produced inadequately [5,6]. Some studies showed that the incidence of mild to severe hearing loss in patients with pSS was predicted as 78.38%, though many patients were unaware of their hearing loss [7].…”