Audiometric and cVEMP Thresholds Show Little Correlation With Symptoms in Superior Semicircular Canal Dehiscence Syndrome

Noij, Kimberley S.; Wong, Kevin; Duarte, Maria J.; Masud, Salwa; Dewyer, Nicholas A.; Herrmann, Barbara S.; Guinan, John J.; Kozin, Elliott D.; Jung, David; Rauch, Steven D.; Lee, Dong Hoon

doi:10.1097/mao.0000000000001910

Cited by 18 publications

(12 citation statements)

References 43 publications

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“…There was also variability of amplitude in the CFD ear relative to the ear without CFD. Noij et al found that in SSCD patients, the threshold audiometry and cVEMP data were useful diagnostically and for monitoring outcomes post-operatively, these measures showed no significant correlation with vestibular and most auditory symptoms or their severity (71).…”

Section: Cervical Vestibular Evoked Myogenic Potentialsmentioning

confidence: 99%

Third Window Syndrome: Surgical Management of Cochlea-Facial Nerve Dehiscence

et al. 2019

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Objective: This communication is the first assessment of outcomes after surgical repair of cochlea-facial nerve dehiscence (CFD) in a series of patients. Pre-and post-operative quantitative measurement of validated survey instruments, symptoms, diagnostic findings and anonymous video descriptions of symptoms in a cohort of 16 patients with CFD and third window syndrome (TWS) symptoms were systematically studied. Study design:Observational analytic case-control study.Setting: Quaternary referral center.Patients: Group 1 had 8 patients (5 children and 3 adults) with CFD and TWS who underwent surgical management using a previously described round window reinforcement technique. Group 2 had 8 patients (2 children and 6 adults) with CFD who did not have surgical intervention.Interventions: The Dizziness Handicap Inventory (DHI) and Headache Impact Test (HIT-6) were administered pre-operatively and post-operatively. In addition, diagnostic findings of comprehensive audiometry, cervical vestibular evoked myogenic potential (cVEMP) thresholds and electrocochleography (ECoG) were studied. Symptoms before and after surgical intervention were compared.Main outcome measures: Pre-vs. post-operative DHI, HIT-6, and audiometric data were compared statistically. The thresholds and amplitudes for cVEMP in symptomatic ears, ears with cochlea-facial nerve dehiscence and ears without CFD were compared statistically.Results: There was a highly significant improvement in DHI and HIT-6 at pre-vs. post-operative (p < 0.0001 and p < 0.001, respectively). The age range was 12.8-52.9 years at the time of surgery (mean = 24.7 years). There were 6 females and 2 males. All 8 had a history of trauma before the onset of their symptoms. The mean cVEMP threshold was 75 dB nHL (SD 3.8) for the operated ear and 85.7 dB (SD 10.6) for the unoperated ear. In contrast to superior semicircular canal dehiscence, where most ears have abnormal ECoG findings suggestive of endolymphatic hydrops, only 1 of 8 operated CFD ears (1 of 16 ears) had an abnormal ECoG study. Wackym et al. Surgery for Cochlea-Facial Nerve DehiscenceConclusions: Overall there was a marked improvement in DHI, HIT-6 and symptoms post-operatively. Statistically significant reduction in cVEMP thresholds was observed in patients with radiographic evidence of CFD. Surgical management with round window reinforcement in patients with CFD was associated with improved symptoms and outcomes measures.

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Section: Cervical Vestibular Evoked Myogenic Potentialsmentioning

confidence: 99%

Third Window Syndrome: Surgical Management of Cochlea-Facial Nerve Dehiscence

et al. 2019

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“…Superior semi-circular canal dehiscence syndrome is associated with multiple symptoms, the severity of which do not correlate well with objective measures such as pure tone audiometry or vestibular evoked myogenic potentials. 14 Yet there are still no validated disease-specific quality of life measures. To the best of our knowledge, there is only a single previous study that has measured quality of life in patients with superior semi-circular canal dehiscence syndrome, and this study used a generic quality of life measure (36-Item Short Form survey).…”

Section: Introductionmentioning

confidence: 99%

Superior semi-circular canal dehiscence syndrome: quantifying the effectiveness of treatment from the patient's perspective

et al. 2021

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Background Superior semi-circular canal dehiscence syndrome is a disorder characterised by auditory and vestibular symptoms that can significantly impact quality of life, and yet it has no disease-specific quality of life instrument. Method Thirty-six patients who underwent transmastoid superior semicircular canal resurfacing and plugging were included from an initial cohort of 60 surgically managed patients. A sub-cohort of 19 consecutive patients completed validated symptom and quality of life questionnaires before and after surgery. Of the 36 patients, 31 participated in a telephone semi-structured interview post-operatively. Results Following surgery, there was a statistically significant improvement in autophony index score (p = 0.02), symptom severity score (p < 0.001) and sound hypersensitivity (p = 0.01). Thematic analysis of telephone interviews suggested three main symptom themes: auditory hypersensitivity, dysequilibrium, headache and concentration difficulties. Dysequilibrium was found to persist post-operatively. Conclusion Surgery improves overall symptoms and quality of life. However, important symptom themes may be overlooked using the outcome measures that are currently available. A unified disease-specific outcome measure is urgently required to better understand the impact of symptoms and measure treatment effects.

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“…A discrepancy between the symptoms and the physiological measurements in superior canal dehiscence syndrome 4 is the rule, probably because the two manifestations have different dynamics. There is the possibility of an early phase, in which the physiological measures change, in the absence of symptomatic correlates.…”

Section: Discussionmentioning

confidence: 96%

Asymptomatic superior semicircular canal dehiscence

2021

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Objective To describe a case of asymptomatic superior semicircular canal dehiscence. Method Clinical case report. Results A 50-year-old man presenting with right-sided Ménière´s disease also showed an enhanced response on vestibular evoked myogenic potential testing for the left ear. Unilateral left-sided superior semicircular canal bone dehiscence was clearly visualised on a subsequent temporal bone computed tomography scan. These findings were consistent with superior canal dehiscence syndrome. However, the patient did not complain of any specific superior canal dehiscence syndrome symptoms. Given that vestibular evoked myogenic potential testing may detect asymptomatic forms of superior canal dehiscence, as noted in this case, such testing seems to exhibit reduced specificity for superior canal dehiscence syndrome. Conclusion An enhanced response on vestibular evoked myogenic potential testing in isolation appears to be a weaker indicator of superior canal dehiscence syndrome, and rather a marker of superior semicircular canal dehiscence.

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Audiometric and cVEMP Thresholds Show Little Correlation With Symptoms in Superior Semicircular Canal Dehiscence Syndrome

Abstract: While threshold audiometry and cVEMP are important tools to diagnose SCD and monitor surgical outcomes, these measures showed no significant correlation with vestibular and most auditory symptoms or their severity.

Cited by 18 publications

References 43 publications

Third Window Syndrome: Surgical Management of Cochlea-Facial Nerve Dehiscence

Third Window Syndrome: Surgical Management of Cochlea-Facial Nerve Dehiscence

Superior semi-circular canal dehiscence syndrome: quantifying the effectiveness of treatment from the patient's perspective

Asymptomatic superior semicircular canal dehiscence

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