Concurrent superior semicircular canal dehiscence and endolymphatic hydrops: A novel case series

Johanis, Michael; Jong, Russell De; Miao, Tyler; Hwang, Leslie; Lum, Meachelle; Kaur, Taranjit; Willis, Shelby; Arsenault, John J.; Duong, Courtney; Yang, Isaac; Gopen, Quinton

doi:10.1016/j.ijscr.2020.12.074

Cited by 7 publications

(4 citation statements)

References 20 publications

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“…Thus, in the case of ignored multiple OCDs, performing surgery for an SSCD in a patient without clear and specific vestibular symptoms evocative of TMWS (e.g., Tullio phenomenon or pressure sensitivity in the EAC) could worsen the postoperative clinical status of an undiagnosed OCD. This seems obvious because the volume of the endolymphatic system after a plugging procedure is diminished by the surgical procedure, favoring the appearance or persistence of concomitant EH ( 41 ). Regarding the EH associated with OCD, it should be added that some authors have reported this entanglement between two apparently distinct pathologies ( 41 – 43 ).…”

Section: Discussionmentioning

confidence: 99%

“…This seems obvious because the volume of the endolymphatic system after a plugging procedure is diminished by the surgical procedure, favoring the appearance or persistence of concomitant EH ( 41 ). Regarding the EH associated with OCD, it should be added that some authors have reported this entanglement between two apparently distinct pathologies ( 41 – 43 ). Future studies should verify whether the simultaneous presence of a an EH (confirmed by electrocochleography and dedicated imaging), it does not constitute a risk factor for the postoperative results in case of SSCD plugging-type surgery.…”

Section: Discussionmentioning

confidence: 99%

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Why should multiple dehiscences of the otic capsule be considered before surgically treating patients with superior semicircular canal dehiscence? A radiological monocentric review and a case series

et al. 2023

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This review aims to draw attention to the multiple ipsilateral otic capsule dehiscences (OCDs), which may cause therapeutic failure in operated patients. A series of six severely disabled patients with symptoms and signs consistent with a superior semicircular canal dehiscence (SSCD) diagnosis, confirmed by a high-resolution CT scan, is presented here. Five of the patients underwent surgery, and in four of the cases, the postoperative results were poor and/or disappointing. The ethical principles underlying modern medicine encourage medical staff to learn from past experience even when the results are modest despite the accuracy of the treatment applied to a patient. Consequently, we reviewed the radiological records of symptomatic and asymptomatic patients diagnosed or referred to our center for confirmation over the past 5 years to determine the incidence of multiple OCD in this population. Multiple localizations of suspected OCD in the ipsilateral ear did not appear to be rare and were found in 29 of 157 patients (18.47%) in our retrospective review using high-resolution thin-sliced CT scans. The decision to perform surgery for a documented symptomatic superior SSCD should be made with caution only after ruling out concomitant lesser-known variants of OCD in the ipsilateral ear.

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Why should multiple dehiscences of the otic capsule be considered before surgically treating patients with superior semicircular canal dehiscence? A radiological monocentric review and a case series

et al. 2023

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“…As for case 2, given the history consistent with ipsilesional MD, it could also be hypothesized that a hydropic distension of the undermost part of the labyrinth (PSC and saccule) might lead to a selective PSC loss, either directly or indirectly, through a distension of the otolith structure herniating into the PSC [64]. This assumption could be supported by the detection of signs of endolymphatic hydrops in patients with TMW disorders [65][66][67]. Nevertheless, the instrumental profile of the patient was fully consistent with a TMWM and symptoms improved after PSC plugging, while standard treatment attempts for MD were ineffective, making the aforementioned mechanism less likely.…”

Section: Discussionmentioning

confidence: 94%

Posterior Semicircular Canal Dehiscence with Vestibulo-Ocular Reflex Reduction for the Affected Canal at the Video-Head Impulse Test: Considerations to Pathomechanisms

Castellucci,

Dumas,

Abuzaid

et al. 2024

Audiology Research

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Posterior semicircular canal dehiscence (PSCD) has been demonstrated to result in a third mobile window mechanism (TMWM) in the inner ear similar to superior semicircular canal dehiscence (SSCD). Typical clinical and instrumental features of TMWM, including low-frequency conductive hearing loss (CHL), autophony, pulsatile tinnitus, sound/pressure-induced vertigo and enhanced vestibular-evoked myogenic potentials, have been widely described in cases with PSCD. Nevertheless, video-head impulse test (vHIT) results have been poorly investigated. Here, we present six patients with PSCD presenting with a clinical scenario consistent with a TMWM and an impaired vestibulo-ocular reflex (VOR) for the affected canal on vHIT. In two cases, an additional dehiscence between the facial nerve and the horizontal semicircular canal (HSC) was detected, leading to a concurrent VOR impairment for the HSC. While in SSCD, a VOR gain reduction could be ascribed to a spontaneous “auto-plugging” process due to a dural prolapse into the canal, the same pathomechanism is difficult to conceive in PSCD due to a different anatomical position, making a dural herniation less likely. Alternative putative pathomechanisms are discussed, including an endolymphatic flow dissipation during head impulses as already hypothesized in SSCD. The association of symptoms/signs consistent with TMWM and a reduced VOR gain for the posterior canal might address the diagnosis toward PSCD.

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“…On the other hand, according to the inclusion criteria adopted in our study, we excluded from the correlation analysis the patients with SCD presenting with a clinical scenario consistent with MD, aiming to avoid possible interference in data collection due to additional pathologies other than SCD itself. Nevertheless, the literature reporting how third window disorders might result in MD is increasing, broadening the range of the clinical phenotypes related to TMWM [63][64][65][66][67]. In fact, it could be speculated that an intermittent spontaneous prolapse of the dura within the canal might occur in this subgroup of patients, resulting in a transient displacement of the contained volume of the endolymph from the SSC into the contiguous areas, thus developing a transitory distension of the residual membranous labyrinth in a manner comparable to endolymphatic hydrops.…”

Section: Discussionmentioning

confidence: 99%

Impaired Vestibulo-Ocular Reflex on Video Head Impulse Test in Superior Canal Dehiscence: “Spontaneous Plugging” or Endolymphatic Flow Dissipation?

Castellucci,

Malara,

Martellucci

et al. 2023

Audiology Research

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Surgical plugging of the superior semicircular canal (SSC) represents an effective procedure to treat disabling symptoms in superior canal dehiscence (SCD), despite resulting in an impaired vestibulo-ocular reflex (VOR) gain for the SSC. On the other hand, SSC hypofunction on video head impulse test (vHIT) represents a common finding in patients with SCD exhibiting sound/pressure-induced vertigo, a low-frequency air–bone gap (ABG), and enhanced vestibular-evoked myogenic potentials (VEMPs). “Spontaneous canal plugging” has been assumed as the underlying process. Nevertheless, missing/mitigated symptoms and/or near-normal instrumental findings would be expected. An endolymphatic flow dissipation has been recently proposed as an alternative pathomechanism for SSC VOR gain reduction in SCD. We aimed to shed light on this debate by comparing instrumental findings from 46 ears of 44 patients with SCD exhibiting SSC hypofunction with post-operative data from 10 ears of 10 patients with SCD who underwent surgical plugging. While no difference in SSC VOR gain values was found between the two groups (p = 0.199), operated ears developed a posterior canal hypofunction (p = 0.002). Moreover, both ABG values (p = 0.012) and cervical/ocular VEMP amplitudes (p < 0.001) were significantly higher and VEMP thresholds were significantly lower (p < 0.001) in ears with SCD compared to operated ears. According to our data, canal VOR gain reduction in SCD should be considered as an additional sign of a third window mechanism, likely due to an endolymphatic flow dissipation.

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Concurrent superior semicircular canal dehiscence and endolymphatic hydrops: A novel case series

Cited by 7 publications

References 20 publications

Why should multiple dehiscences of the otic capsule be considered before surgically treating patients with superior semicircular canal dehiscence? A radiological monocentric review and a case series

Why should multiple dehiscences of the otic capsule be considered before surgically treating patients with superior semicircular canal dehiscence? A radiological monocentric review and a case series

Posterior Semicircular Canal Dehiscence with Vestibulo-Ocular Reflex Reduction for the Affected Canal at the Video-Head Impulse Test: Considerations to Pathomechanisms

Impaired Vestibulo-Ocular Reflex on Video Head Impulse Test in Superior Canal Dehiscence: “Spontaneous Plugging” or Endolymphatic Flow Dissipation?

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