Alterações retrococleares na esclerose sistêmica: relato de casos

Valente, Julia de Souza Pinto; Corona, Ana Paula

doi:10.1590/2317-1782/20172016238

Cited by 3 publications

(4 citation statements)

References 14 publications

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“…On the audiometric testing, bilateral sensorineural hearing loss is the most common finding followed by mixed hearing loss. 24 These audiometric features are consistent with our findings of decreased spiral ganglion neurons in the cases of scleroderma. We also report vascular involvement of the stria vascularis in the lower basal cochlear turns, which is consistent with lesions reported in other vasculitides such as granulomatosis with polyangiitis.…”

Section: Discussionsupporting

confidence: 92%

“…Both patients in this study reported hearing loss and one reported tinnitus. On the audiometric testing, bilateral sensorineural hearing loss is the most common finding followed by mixed hearing loss 24 . These audiometric features are consistent with our findings of decreased spiral ganglion neurons in the cases of scleroderma.…”

Section: Discussionsupporting

confidence: 91%

Section: Discussionmentioning

confidence: 49%

“…Prior clinical studies have described a variety of otologic symptoms in scleroderma including hearing loss, hyperacusis, aural fullness, tinnitus, and vestibular disturbances. [22][23][24] Both patients in this study reported hearing loss and one reported tinnitus. On the audiometric testing, bilateral sensorineural hearing loss is the most common finding followed by mixed hearing loss.…”

Section: Discussionmentioning

confidence: 64%

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Human otopathology in scleroderma

Castillo‐Bustamante,

Roychowdhury,

Gandhi

et al. 2023

Laryngoscope Investig Oto

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ObjectiveScleroderma is a complex chronic progressive immune‐mediated disease that causes fibrosis of the skin and internal organs, and vasculopathy.Ear involvement has been poorly studied in patients with scleroderma. Vasculitic and autoimmune mechanisms are considered as possible etiologies on hearing impairment, however, this etiology still unclear.Herein, we reviewed three cases of scleroderma from a temporal bone repository.MethodsThe national temporal bone database was reviewed for cases with scleroderma. Clinical case review and correlative otopathologic analysis. Middle and inner ear otopathologic analysis was performed following hematoxylin and eosin staining under light microscopy. Findings were compared to three age‐matched controls.ResultsTwo patients (three cases) with a history of serologically confirmed scleroderma were identified. Both individuals reported tinnitus and demonstrated bilateral moderate to severe down‐sloping sensorineural hearing loss on audiometry. Histologically, the incudomallear joint space was diminished and ossicles appeared demineralized. A loss of hyaline cartilage, and obliteration of the incudomallear and incudostapedial joint synovial spaces was observed. Decreased caliber and intimal hyperplasia of arteries adjacent to ossicles was also identified. Mild diffuse atrophy of stria vascularis in the middle and apical turns of cochlea were found. Hair cell populations were normal. Total spiral ganglion neurons were lower in cases of scleroderma (range 29%–51%) compared to age‐matched controls.ConclusionFibrosis, inflammation, and vascular changes were observed in the middle and inner ear in patients with scleroderma. Findings have implications for understanding hearing and vestibular dysfunction in this patient population.Level of evidenceRetrospective study.

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Section: Discussionsupporting

confidence: 92%

Section: Discussionsupporting

confidence: 91%

Section: Discussionmentioning

confidence: 49%

Section: Discussionmentioning

confidence: 64%

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Human otopathology in scleroderma

Castillo‐Bustamante,

Roychowdhury,

Gandhi

et al. 2023

Laryngoscope Investig Oto

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Hearing Loss Secondary to Systemic Sclerosis Vasculopathy: Case Study with a Short Review

Bobeică¹,

Niculeț²,

Crăescu³

et al. 2022

CCID

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Systemic sclerosis (SSc) is a collagenosis with a substrate of chronic inflammation, which is determined by autoimmunity. The pathogenesis of this disease involves microvasculopathy (small vessel pathology) followed by excessive cutaneous and visceral fibrosis. Although acoustic and vestibular impairment is not classified as being a secondary pathology of SSc, several studies have identified cases of SSc that associate hearing loss and especially vertigo and tinnitus. This paper presents data from the medical literature that have identified vestibular and auditory symptoms among patients with SSc, associating the clinical case presentation of a patient suffering from SSc, which is associated with hearing loss. The need for additional studies on larger groups of patients is underlined, in order to clarify the impact of vasculopathy and fibrosis on the acoustic and vestibular analyzer in patients with SSc.

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Hearing impairment in systemic sclerosis patients—what do we really know?

Sieśkiewicz,

Rębacz,

Sieśkiewicz

2024

Front. Med.

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BackgroundSystemic sclerosis (SSc) is a disease of a very heterogeneous clinical picture and immunological profile with progression rate that varies between individuals. Although hearing deterioration is not a complaint that comes to the fore in SSc patients, as it is not life-threatening compared to many other more severe symptoms of this disease, it can significantly impair the quality of life. Medical literature concerning this problem is rather scarce.Materials and methodsIn this article we systematically reviewed the medical publications concerning hearing impairment in patients with systemic sclerosis to evaluate current understanding of this complex problem. Following PRISMA guidelines a total of 19 papers were found and analysed including 11 original studies and 8 case reports.ResultsAlthough it seems that hearing impairment in SSc patients is relatively more common than in the general population, based on the analysis of available literature, no firm conclusions regarding its frequency and pathomechanism can be drawn yet. Microangiopathy leading to damage to the sensory cells of the inner ear is suspected to be the main mechanism of hearing loss, although damage to the higher levels of the auditory pathway appears to be underestimated due to incomplete audiological diagnosis.ConclusionUndoubtedly, the reason for the difficulty in such an evaluation are the complex and still not fully elucidated pathomechanism of SSc, the individually variable dynamics of the disease and the unique heterogeneity of symptoms. Nevertheless, further studies in larger and appropriately selected groups of patients, focused more on the dynamics of microangiopathy and not solely on clinical symptoms could provide answers to many key questions in this regard.

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Alterações retrococleares na esclerose sistêmica: relato de casos

Cited by 3 publications

References 14 publications

Human otopathology in scleroderma

Human otopathology in scleroderma

Hearing Loss Secondary to Systemic Sclerosis Vasculopathy: Case Study with a Short Review

Hearing impairment in systemic sclerosis patients—what do we really know?

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