Auditory Perception in Alport’s Syndrome

Viveiros, Carla Mherlyn; Pereira, Liliane Desgualdo; Kirsztajn, Gianna Mastroianni

doi:10.1016/s1808-8694(15)31049-1

Cited by 1 publication

(2 citation statements)

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“…The diagnosis of renal involvement in the Alport Syndrome is based on electron microscopy typical findings. Hearing loss is described as sensorioneural, bilateral, and symmetrical, even though its degree can vary, which we had already observed 13 .…”

Section: Discussionmentioning

confidence: 57%

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A Careful Interpretation of the Audiometry may contribute to the Diagnosis of Alport Syndrome: A Pilot Study

Cardoso¹,

Fernandes²,

Riguetti³

et al. 2020

J Rare Dis Res Treat

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Introduction: Hearing loss is a typical finding in Alport syndrome (sAlport) caused by a genetic defect in the type IV collagen synthesis. Depending on the criteria, these individuals may be diagnosed with normal hearing.Objective: To compare the prevalence of hearing loss according to different criteria -World Health Organization, WHO; Global Burden Disease, GBD and Clark -in patients with sAlport and segmental and focal glomeruloesclerosis (FSGS).Materials And Methods: This is a cross-sectional pilot study. Pure-tone audiometry was carried out in patients with sAlport and FSGS (glomerulopathy which was selected as a control group).Results: We assessed 13 patients (6 with sAlport and 7 with FSGS). Under the WHO criteria, no patient had hearing loss. The prevalence of hearing loss was similar according to GBD criteria (16.67% and 14.29% in the sAlport and FSGS groups, respectively). Clark's criteria, instead, revealed a higher prevalence of hearing loss in the sAlport group (66.67%) vs. FSGS (28.57%). Conclusion:The prevalence of hearing loss in the sAlport group varied depending on the criteria (from nonexistent to 67%). We consider that a critical evaluation of the hearing thresholds may help physicians to early detect minimal hearing impairment even though the report says, "normal hearing".

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Section: Discussionmentioning

confidence: 57%

“…If so, the patient has hearing loss 10 . In many studies the criteria for hearing loss remain unclear 9,[11][12][13][14][15] , others use WHO's recommendations 8 . Both cases can "hide" milder hearing losses.…”

Section: Introductionmentioning

confidence: 99%