Sensorineural hearing loss as the first manifestation of Sjögren's syndrome

Almeida, Raquel Sousa; Oliveira, Ana; Pego, Petra M.; Abuowda, Yahia; Gaspar, Iuri; Costa, José Jackson do Nascimento

doi:10.1590/1806-9282.63.01.7

Cited by 5 publications

(3 citation statements)

References 11 publications

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“…Since the inner ear is a small tissue, the biopsy is difficult and cannot be proved, but as inflammation progresses, necrosis and shedding of hair cells may lead to irreversible hearing loss. In adults, there are several reports of SS with hearing loss (Tables 1, 2) [4,[7][8][9][10][11][12][13][14]. It has been estimated that autoantigens released from one side of the damaged inner ear could trigger an autoimmune reaction in the other side of the inner ear, causing hearing loss in both ears with a variety of severity [15].…”

Section: Discussionmentioning

confidence: 99%

Unilateral Sensorineural Hearing Loss in Children Associated With Sjögren's Syndrome

Okawa

Okanari

Hirano

et al. 2021

Cureus

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The occurrence of unilateral sensorineural hearing loss (SNHL) during school age is relatively rare and accounts for approximately 6% of all deafness in childhood. We present two cases involving children who were diagnosed with SNHL associated with Sjögren's syndrome (SS). Case 1: An eight-year-old girl with an approximately two-year clinical history of gradual hearing loss was diagnosed with SNHL associated with SS based on histological findings of inflammation in the salivary glands and the presence of serum anti-Sjögren's syndrome-A antibody. Case 2: An eight-year-old boy with acute idiopathic thrombocytopenic purpura in whom unilateral hearing loss, which was not associated with any problems in daily life, was detected during hospitalization and who was finally diagnosed with SNHL and SS. Steroid treatment was ineffective for both patients. The previously unrecognized combination of SNHL with SS should be considered in the diagnosis of unilateral SNHL, even in children.

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Section: Discussionmentioning

confidence: 99%

Unilateral Sensorineural Hearing Loss in Children Associated With Sjögren's Syndrome

Okawa

Okanari

Hirano

et al. 2021

Cureus

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“…It is defined as a hearing loss of at least 30 dB, in three consecutive audiogram frequencies due to impairment of conduction in the VIII nerve, inner ear, or central brain processing centers [16,17]. The etiology of this syndrome is still unclear, but a good response to glucocorticosteroids treatment and recurrent nature suggests its association with immune system disorders [16,18]. The most commonly discussed hypothesis is based on the toxic activity of antibodies and cytotoxic apoptosis mediated by T cells in the inner ear.…”

Section: Discussionmentioning

confidence: 99%

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

et al. 2020

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Objectives Primary Sjögren's syndrome (pSS) is an autoimmune, multisystem exocrinopathy characterized by dysfunction of the exocrine glands. Central nervous system (CNS) involvement is estimated to be present in 10-60% patients with pSS. The present study aimed to evaluate brainstem auditory evoked potentials (BAEP) in pSS patients without central nervous system involvement, and without otolaryngological abnormalities. Method Thirty-six pSS patients (35 women, 1 man, mean age 48 years old) and 40 healthy volunteers were investigated. BAEP, brain imaging, laboratory parameters, and clinical neurological and otolaryngological examinations were performed. Results Abnormal BAEP were recorded in 16.7% patients. The mean wave BAEP I and V latency and mean wave III-V and I-V interpeak latencies were significantly longer in pSS patients than the controls. There were no statistically significant correlations between BAEP parameters and laboratory tests. None of the patients was found having the abnormalities on brain imaging. Conclusions This study confirms that in pSS patients without clinical central nervous system impairment, auditory pathway disturbances could be found. Disorders of brain bioelectrical activity may be a consequence of ongoing autoimmune process.

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“…Some other physiological changes such as atrophy of the spiral ganglion cells due to pSS cause sensorineural hearing loss; therefore, subjects with pSS may have neurological symptoms. The several autoantibodies that regulate the immune system suffer from autoimmune diseases; therefore, multiple problems like polyneuropathy may be revealed [22,23].…”

Section: Discussionmentioning

confidence: 99%

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

2019

Turk J Med Sci

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Background/aim: Autoimmune diseases are a remarkable issue for researchers due to their adverse effects on the auditory system, but for primary Sjögren's syndrome (pSS) there is little research on the comprehensive audiological findings in literature. The main objective of this study was to investigate the medial olivocochlear efferent functions of subjects with pSS and to examine the audiological findings. Materials and methods: The study included 36 subjects with pSS and 36 healthy subjects. All the subjects underwent testing including pure tone, speech, and high frequency audiometry; tympanometry and acoustic reflexes; distortion product otoacoustic emissions (DPOAE); and suppression of DPOAE. Results: The hearing thresholds of the pSS group were higher than those of the control at all frequencies (P < 0.001). Minimal to mild sensorineural hearing loss was observed in 52.77% of all the subjects with pSS. Additionally, all of the subjects had type A curve tympanograms, but the static compliances of the pSS group were lower and the acoustic reflex thresholds were higher than in the control (P < 0.001). In suppression levels of DPOAE, no statistically significant difference was found between the groups (P > 0.05). Conclusion: The study indicates that because of obtaining normal suppression levels in DPOAE, the medial olivocochlear efferent system is functional in pSS. However, there is a need for more tests, including auditory brainstem response, to evaluate the afferent auditory system in pSS.

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Sensorineural hearing loss as the first manifestation of Sjögren's syndrome

Cited by 5 publications

References 11 publications

Unilateral Sensorineural Hearing Loss in Children Associated With Sjögren's Syndrome

Unilateral Sensorineural Hearing Loss in Children Associated With Sjögren's Syndrome

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

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

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