Otolith function in Usher type II syndrome.
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| Title: | Otolith function in Usher type II syndrome. |
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| Authors: | Amorim, Ana Margarida (AUTHOR), Rebelo, Diliana (AUTHOR), Ramada, Ana Beatriz (AUTHOR), Lopes, Ana Cristina (AUTHOR), Lemos, João (AUTHOR), Ribeiro, João Carlos (AUTHOR) |
| Source: | Acta Oto-Laryngologica. Sep2024, Vol. 144 Issue 9, p485-490. 6p. |
| Subjects: | Vestibular apparatus physiology, Vestibular apparatus, Vertigo, Statistical correlation, Descriptive statistics, Research, Usher's syndrome, Vestibular function tests, Auditory evoked response |
| Abstract (English): | Background: Usher's syndrome type II (USH2) is a rare genetic disorder encompassing hearing loss, vision impairment, and apparent intact vestibular function. Recent research suggests a potential involvement of the otolith vestibular receptors in USH2. Aims/Objectives: Evaluate otolith dynamic function in USH2. Material and Methods: Twenty-two USH2 (median age 53.9 ± 2.99) and age-matched controls underwent a complete battery vestibular testing including air conducted cervical and ocular vestibular evoked myogenic potentials (c-VEMPs and o-VEMPs). Vestibular function tests were correlated with Activities Balance Scale (ABC) and Dizziness Handicap Inventory (DHI) scores. Results: Fourteen USH2 reported previous vertigo (vs none control). Among 88 ears, c-VEMPs were absent in 15 USH2 cases and 4 controls (p = 0.034), while o-VEMPs were absent in 22 USH2 cases and 12 controls (p = 0.129). There were significant differences between USH2 vs controls in right ear o-VEMP N1 latencies (median 11.60/10.40, p < 0.010), N1-P1 amplitudes (median 5.15/10.10, p < 0.003) and in o-VEMP N1-P1 asymmetry ratio (median 24.78/40.50, p < 0.014). USH2 showed a strong correlation between o-VEMP amplitude and DHI score (p = 0.003, ρ = 0.769). No association was found between vertigo and VEMPs subgroups. Conclusions and Significance: Our findings suggest the presence of otolith dysfunction in USH2, which is independent from subjectively reported dizziness. Incorporating vestibular testing into USH2 evaluation and monitoring could enhance characterization of this multisensory disease. [ABSTRACT FROM AUTHOR] |
| Abstract (Chinese): | Usher 综合征 II 型 (USH2) 是一种罕见的遗传性疾病, 其特征包括听力丧失、视力障碍和看似完整的前庭功能。最新研究表明, 耳石前庭受体可能与 USH2有关联。 评估USH2 患者的耳石动态功能。 22 名 USH2(中位年龄 53.9 ± 2.99)和年龄匹配的对照者接受了完整的系列前庭测试, 包括空气传导的颈部和眼部前庭诱发的肌源性电位 (c-VEMPs 和 o-VEMPs)。前庭功能测试与活动平衡量表 (ABC) 和眩晕障碍量表 (DHI) 评分相关联。 14 名 USH2 患者报告先前患有眩晕(对照组无)。在 88 只耳朵中, 15 个 USH2 病例和 4 个对照组成员没有 c-VEMP(p = 0.034), 而 22 个USH2 病例和 12 个对照组成员没有 o-VEMP (p = 0.129)。USH2组 与对照组在右耳 o-VEMP N1 潜伏期(中位数 11.60/10.40, p < 0.010)、N1-P1 振幅(中位数 5.15/10.10, p < 0.003)和 o-VEMP N1-P1 不对称比率(中位数 24.78/40.50, p < 0.014)方面均存在显著差异。USH2 显示 o-VEMP 振幅与 DHI 评分之间存在很强的相关性(p = 0.003, ρ = 0.769)。没有发现眩晕和 VEMPs 亚组之间存在关联性。 我们的研究结果表明, USH2患者存在耳石功能障碍, 这与主观报告的头晕无关。将前庭测试纳入 USH2 评估和监测可以增强这种多感觉疾病的表征。 [ABSTRACT FROM AUTHOR] |
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| Database: | Psychology and Behavioral Sciences Collection |
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| Abstract: | Background: Usher's syndrome type II (USH2) is a rare genetic disorder encompassing hearing loss, vision impairment, and apparent intact vestibular function. Recent research suggests a potential involvement of the otolith vestibular receptors in USH2. Aims/Objectives: Evaluate otolith dynamic function in USH2. Material and Methods: Twenty-two USH2 (median age 53.9 ± 2.99) and age-matched controls underwent a complete battery vestibular testing including air conducted cervical and ocular vestibular evoked myogenic potentials (c-VEMPs and o-VEMPs). Vestibular function tests were correlated with Activities Balance Scale (ABC) and Dizziness Handicap Inventory (DHI) scores. Results: Fourteen USH2 reported previous vertigo (vs none control). Among 88 ears, c-VEMPs were absent in 15 USH2 cases and 4 controls (p = 0.034), while o-VEMPs were absent in 22 USH2 cases and 12 controls (p = 0.129). There were significant differences between USH2 vs controls in right ear o-VEMP N1 latencies (median 11.60/10.40, p < 0.010), N1-P1 amplitudes (median 5.15/10.10, p < 0.003) and in o-VEMP N1-P1 asymmetry ratio (median 24.78/40.50, p < 0.014). USH2 showed a strong correlation between o-VEMP amplitude and DHI score (p = 0.003, ρ = 0.769). No association was found between vertigo and VEMPs subgroups. Conclusions and Significance: Our findings suggest the presence of otolith dysfunction in USH2, which is independent from subjectively reported dizziness. Incorporating vestibular testing into USH2 evaluation and monitoring could enhance characterization of this multisensory disease. [ABSTRACT FROM AUTHOR] |
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| ISSN: | 00016489 |
| DOI: | 10.1080/00016489.2024.2390661 |
