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Vestibular Function in Pendred Syndrome: Intact High Frequency VOR and Saccular Hypersensitivity

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@article{d093d26a7975414a9777203b4f47d29a,
title = "Vestibular Function in Pendred Syndrome: Intact High Frequency VOR and Saccular Hypersensitivity",
abstract = "OBJECTIVE: Although Pendred syndrome involves anatomical abnormalities in the vestibular system and patient-perceived dizziness, the literature on vestibular function is scarce. The objective was to evaluate objective vestibular function in patients with PS using the video head impulse test (VHIT) and the cervical vestibular evoked myogenic potential (cVEMP) test, assessing the high frequency vestibulo-ocular reflex (VOR) and the saccular function, respectively.METHODS: Twenty-six subjects (52 ears) with Pendred Syndrome and severe-to-profound hearing loss (cochlear implant candidacy) were studied retrospectively. Main outcome measures were VHIT VOR gain, refixation saccades, cVEMP stimulus thresholds and amplitudes.RESULTS: In total, 4 of 52 ears (8%) had pathological VHIT outcomes (2 ears with low VHIT gain and 2 ears with saccades). The mean VHIT gain value was 0.96 (range 0.63-2.02). No patients had complete VOR loss. CVEMP responses were present in 76% of tested ears and absent in 24%. The mean cVEMP amplitude value was 192 μV. Absence of cVEMP response was associated with the presence of VHIT saccades (p = 0.038) and advanced age (rs = -0.34; p < 0.05). cVEMP amplitude was correlated with VHIT gain (rs = 0.46; p < 0.001).CONCLUSION: Despite dysmorphic vestibular anatomy and severe loss of hearing, the VHIT VOR function is normal in patients with Pendred Syndrome. However, saccular function appears to be abnormally sensitive, as documented by low cVEMP thresholds and high amplitudes, which agrees with a {"}third window{"} effect secondary to the large vestibular aqueduct.",
keywords = "Cochlear implant, Endolymphatic sac, Enlarged vestibular aqueduct, Hearing loss, Vestibular evoked myogenic potentials, Vestibular testing, Video head impulse test",
author = "West, {Niels Cramer} and Ryberg, {Alexander Christian} and Per Cay{\'e}-Thomasen",
note = "Copyright {\textcopyright} 2021, Otology & Neurotology, Inc.",
year = "2021",
month = oct,
day = "1",
doi = "10.1097/MAO.0000000000003270",
language = "English",
volume = "42",
pages = "e1327--e1332",
journal = "Otology and Neurotology",
issn = "1531-7129",
publisher = "Lippincott Williams & Wilkins",
number = "9",

}

RIS

TY - JOUR

T1 - Vestibular Function in Pendred Syndrome

T2 - Intact High Frequency VOR and Saccular Hypersensitivity

AU - West, Niels Cramer

AU - Ryberg, Alexander Christian

AU - Cayé-Thomasen, Per

N1 - Copyright © 2021, Otology & Neurotology, Inc.

PY - 2021/10/1

Y1 - 2021/10/1

N2 - OBJECTIVE: Although Pendred syndrome involves anatomical abnormalities in the vestibular system and patient-perceived dizziness, the literature on vestibular function is scarce. The objective was to evaluate objective vestibular function in patients with PS using the video head impulse test (VHIT) and the cervical vestibular evoked myogenic potential (cVEMP) test, assessing the high frequency vestibulo-ocular reflex (VOR) and the saccular function, respectively.METHODS: Twenty-six subjects (52 ears) with Pendred Syndrome and severe-to-profound hearing loss (cochlear implant candidacy) were studied retrospectively. Main outcome measures were VHIT VOR gain, refixation saccades, cVEMP stimulus thresholds and amplitudes.RESULTS: In total, 4 of 52 ears (8%) had pathological VHIT outcomes (2 ears with low VHIT gain and 2 ears with saccades). The mean VHIT gain value was 0.96 (range 0.63-2.02). No patients had complete VOR loss. CVEMP responses were present in 76% of tested ears and absent in 24%. The mean cVEMP amplitude value was 192 μV. Absence of cVEMP response was associated with the presence of VHIT saccades (p = 0.038) and advanced age (rs = -0.34; p < 0.05). cVEMP amplitude was correlated with VHIT gain (rs = 0.46; p < 0.001).CONCLUSION: Despite dysmorphic vestibular anatomy and severe loss of hearing, the VHIT VOR function is normal in patients with Pendred Syndrome. However, saccular function appears to be abnormally sensitive, as documented by low cVEMP thresholds and high amplitudes, which agrees with a "third window" effect secondary to the large vestibular aqueduct.

AB - OBJECTIVE: Although Pendred syndrome involves anatomical abnormalities in the vestibular system and patient-perceived dizziness, the literature on vestibular function is scarce. The objective was to evaluate objective vestibular function in patients with PS using the video head impulse test (VHIT) and the cervical vestibular evoked myogenic potential (cVEMP) test, assessing the high frequency vestibulo-ocular reflex (VOR) and the saccular function, respectively.METHODS: Twenty-six subjects (52 ears) with Pendred Syndrome and severe-to-profound hearing loss (cochlear implant candidacy) were studied retrospectively. Main outcome measures were VHIT VOR gain, refixation saccades, cVEMP stimulus thresholds and amplitudes.RESULTS: In total, 4 of 52 ears (8%) had pathological VHIT outcomes (2 ears with low VHIT gain and 2 ears with saccades). The mean VHIT gain value was 0.96 (range 0.63-2.02). No patients had complete VOR loss. CVEMP responses were present in 76% of tested ears and absent in 24%. The mean cVEMP amplitude value was 192 μV. Absence of cVEMP response was associated with the presence of VHIT saccades (p = 0.038) and advanced age (rs = -0.34; p < 0.05). cVEMP amplitude was correlated with VHIT gain (rs = 0.46; p < 0.001).CONCLUSION: Despite dysmorphic vestibular anatomy and severe loss of hearing, the VHIT VOR function is normal in patients with Pendred Syndrome. However, saccular function appears to be abnormally sensitive, as documented by low cVEMP thresholds and high amplitudes, which agrees with a "third window" effect secondary to the large vestibular aqueduct.

KW - Cochlear implant

KW - Endolymphatic sac

KW - Enlarged vestibular aqueduct

KW - Hearing loss

KW - Vestibular evoked myogenic potentials

KW - Vestibular testing

KW - Video head impulse test

UR - http://www.scopus.com/inward/record.url?scp=85116958609&partnerID=8YFLogxK

U2 - 10.1097/MAO.0000000000003270

DO - 10.1097/MAO.0000000000003270

M3 - Journal article

C2 - 34224544

VL - 42

SP - e1327-e1332

JO - Otology and Neurotology

JF - Otology and Neurotology

SN - 1531-7129

IS - 9

ER -

ID: 67832355