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Third Mobile Window Syndromes: New Insights in Pathomechanisms, Instrumental Diagnosis and Surgical Treatment

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A special issue of Audiology Research (ISSN 2039-4349).

Deadline for manuscript submissions: closed (31 July 2024) | Viewed by 14356

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Dr. Andrea Castellucci

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Guest Editor

ENT Unit, Department of Surgery, Azienda USL—IRCCS di Reggio Emilia, Reggio Emilia, Italy
Interests: neurotology; inner ear; vestibular disease; BPPV; Meniere’s disease; vestibular migraine; acute vestibular loss; canal dehiscence; sudden hearing loss; video-HIT; VEMPs
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Dr. Bryan Kevin Ward

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Guest Editor

Department of Otolaryngology-Head and Neck Surgery, Johns Hopkins University School of Medicine, Baltimore, MD, USA
Interests: vestibular; inner ear; third mobile window; bilateral vestibulopathy; eustachian tube; MRI; CT; imaging

Dr. Raymond Van De Berg

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Guest Editor

Department of Otorhinolaryngology and Head and Neck Surgery, Maastricht University Medical Centre, Maastricht, The Netherlands
Interests: vestibular; inner ear; third mobile window

Dr. Leonardo Manzari

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Co-Guest Editor

MSA ENT Academy Center, 03043 Cassino, Italy
Interests: otoneurology; vestibular system; ocular VEMPs; cervical VEMPs; vestibulo-ocular reflex (VOR); video-HIT; otolith system; eye movements (ocular torsion)
Special Issues, Collections and Topics in MDPI journals

Special Issue Information

Dear Colleagues,

Since the first description of superior canal dehiscence syndrome (SCDS) in 1998, an abundance of basic science and clinical research has supported modeling an additional opening in the inner ear as a third mobile window (TMW). Bony defects in the otic capsule bone such as over the superior semicircular canal result in a new low-impedance pathway in the inner ear for sound and pressure stimuli, accounting for several audio-vestibular symptoms and signs. The general term TMW syndrome has then been extended to a variety of abnormalities of the otic capsule sharing a similar clinical spectrum, such as enlarged vestibular aqueduct and dehiscences of the posterior and horizontal semicircular canals, cochlea and facial nerve. Beyond findings of sound/pressure-induced vertigo and nystagmus, low-frequency negative bone conduction thresholds on audiometry and enhanced vestibular-evoked myogenic potential (VEMP) responses, a wider range of atypical symptoms and abnormalities on clinical testing has been reported. Some physiologic and clinical aspects of TMW syndromes remain incompletely understood. Furthermore, consensus on the most effective surgical treatment is lacking.

The aim of this special Issue is to generate insights about clinical presentation, diagnosis and treatments strategies for TMW syndrome disorders, uncovering mechanisms of pathophysiology, and emphasizing differential diagnosis and outcomes following interventions. Original research manuscripts and reviews relevant to TMW syndromes are welcome.

Dr. Andrea Castellucci
Dr. Bryan Kevin Ward
Dr. Raymond Van De Berg
Dr. Leonardo Manzari
Guest Editors

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Keywords

third window syndrome
canal dehiscence
Tullio phenomenon
conductive hearing loss
enlarged vestibular aqueduct
labyrinthine fistula
vestibular-evoked myogenic potentials
video-head impulse test
vestibulo-ocular reflex

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Published Papers (8 papers)

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Research

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14 pages, 4263 KiB

Open AccessArticle

Otic Capsule Dehiscences Simulating Other Inner Ear Diseases: Characterization, Clinical Profile, and Follow-Up—Is Ménière’s Disease the Sole Cause of Vertigo and Fluctuating Hearing Loss?

by Joan Lorente-Piera, Carlos Prieto-Matos, Raquel Manrique-Huarte, Octavio Garaycochea, Pablo Domínguez and Manuel Manrique

Audiol. Res. 2024, 14(2), 372-385; https://doi.org/10.3390/audiolres14020032 - 12 Apr 2024

Cited by 3 | Viewed by 1279

Abstract

Introduction: We present a series of six cases whose clinical presentations exhibited audiovestibular manifestations of a third mobile window mechanism, bearing a reasonable resemblance to Ménière’s disease and otosclerosis. The occurrence of these cases in such a short period has prompted a review of the underlying causes of its development. Understanding the pathophysiology of third mobile window syndrome and considering these entities in the differential diagnosis of conditions presenting with vertigo and hearing loss with slight air-bone gaps is essential for comprehending this group of pathologies. Materials and Methods: A descriptive retrospective cohort study of six cases diagnosed at a tertiary center. All of them went through auditive and vestibular examinations before and after a therapeutic strategy was performed. Results: Out of 84 cases of dehiscences described in our center during the period from 2014 to 2024, 78 belonged to superior semicircular canal dehiscence, while 6 were other otic capsule dehiscences. Among these six patients with a mean age of 47.17 years (range: 18–73), all had some form of otic capsule dehiscence with auditory and/or vestibular repercussions, measured through hearing and vestibular tests, with abnormalities in the results in five out of six patients. Two of them were diagnosed with Ménière’s disease (MD). Another two had cochleo-vestibular hydrops without meeting the diagnostic criteria for MD. In two cases, the otic capsule dehiscence diagnosis resulted from an intraoperative complication due to a gusher phenomenon, while in one case, it was an accidental radiological finding. All responded well to the proposed treatment, whether medical or surgical, if needed. Conclusions: Otic capsule dehiscences are relatively new and unfamiliar entities that should be considered when faced with cases clinically suggestive of Ménière’s disease, with discrepancies in complementary tests or a poor response to treatment. While high-sensitivity and specificity audiovestibular tests exist, completing the study with imaging, especially petrous bone CT scans, is necessary to locate and characterize the otic capsule defect responsible for the clinical presentation. Full article

(This article belongs to the Special Issue Third Mobile Window Syndromes: New Insights in Pathomechanisms, Instrumental Diagnosis and Surgical Treatment)

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19 pages, 1854 KiB

Open AccessArticle

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

by Andrea Castellucci, Pasquale Malara, Salvatore Martellucci, Mohamad Alfarghal, Cristina Brandolini, Gianluca Piras, Enrico Armato, Rosanna Rita Ruberto, Pasquale Brizzi, Livio Presutti and Angelo Ghidini

Audiol. Res. 2023, 13(5), 802-820; https://doi.org/10.3390/audiolres13050071 - 20 Oct 2023

Cited by 3 | Viewed by 1597

Abstract

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. Full article

(This article belongs to the Special Issue Third Mobile Window Syndromes: New Insights in Pathomechanisms, Instrumental Diagnosis and Surgical Treatment)

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11 pages, 1690 KiB

Open AccessArticle

Cochlear Aqueduct Morphology in Superior Canal Dehiscence Syndrome

by Nimesh V. Nagururu, Diane Jung, Ferdinand Hui, Monica S. Pearl, John P. Carey and Bryan K. Ward

Audiol. Res. 2023, 13(3), 367-377; https://doi.org/10.3390/audiolres13030032 - 15 May 2023

Cited by 3 | Viewed by 1724

Abstract

The cochlear aqueduct (CA) connects the scala tympani to the subarachnoid space and is thought to assist in pressure regulation of perilymph in normal ears, however, its role and variation in inner ear pathology, such as in superior canal dehiscence syndrome (SCDS), is unknown. This retrospective radiographic investigation compared CA measurements and classification, as measured on flat-panel computerized tomography, among three groups of ears: controls, n = 64; anatomic superior canal dehiscence without symptoms (SCD), n = 28; and SCDS, n = 64. We found that in a multinomial logistic regression adjusted for age, sex, and BMI, an increase in CA length by 1 mm was associated with a lower odds for being in the SCDS group vs. control (Odds ratio 0.760 p = 0.005). Hierarchical clustering of continuous CA measures revealed a cluster with small CAs and a cluster with large CAs. Another multinomial logistic regression adjusted for the aforementioned clinical covariates showed an odds ratio of 2.97 for SCDS in the small CA cluster as compared to the large (p = 0.004). Further, no significant association was observed between SCDS symptomatology—vestibular and/or auditory symptoms—and CA structure in SCDS ears. The findings of this study lend support to the hypothesis that SCDS has a congenital etiology. Full article

(This article belongs to the Special Issue Third Mobile Window Syndromes: New Insights in Pathomechanisms, Instrumental Diagnosis and Surgical Treatment)

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Review

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9 pages, 222 KiB

Open AccessReview

Insidious Cases of Enlarged Vestibular Aqueduct (EVA) Syndrome Resembling Otosclerosis: Clinical Features for Differential Diagnosis and the Role of High-Resolution Computed Tomography in the Pre-Operative Setting

by Giovanni Motta, Salvatore Allosso, Ludovica Castagna, Ghita Trifuoggi, Tonia Di Meglio, Domenico Testa, Massimo Mesolella and Gaetano Motta

Audiol. Res. 2024, 14(4), 593-601; https://doi.org/10.3390/audiolres14040050 - 27 Jun 2024

Viewed by 1055

Abstract

Background: Enlarged vestibular aqueduct (EVA) syndrome can mimic otosclerosis in adults, presenting with an air–bone gap (ABG) and even absent stapedial reflexes. The ABG in inner-ear disorders is currently the object of several authors’ studies and seems to be related to a third mobile window (TMW) phenomenon. This can lead to misdiagnosis and inappropriate treatment. Given that it would be inappropriate and harmful to perform CT scans in all patients with a clinical diagnosis of otosclerosis, this study aims to highlight some clinical features useful for the differential diagnosis between otosclerosis and these rare cases of EVA presenting with an ABG, thus enabling the identification of suspected cases to be tested with CT scans. Methods: Between April and May 2024, a narrative review was conducted focusing on the differential diagnosis between some rare cases of EVA and otosclerosis. Clinical, audiological, and radiologic features of both conditions were investigated. Results: This review demonstrates the diagnostic challenge in differentiating atypical cases of EVA from otosclerosis in a subset of patients. Clinical and audiological features are important for differential diagnosis, but may not always be sufficient. Therefore, high-resolution computed tomography (HRCT) of the temporal bone plays a pivotal role in definitive diagnosis. Conclusions: In some specific cases, pre-operative imaging assessment using HRCT emerges as an essential tool for differentiating these two conditions and avoiding unnecessary stapes surgery. Full article

(This article belongs to the Special Issue Third Mobile Window Syndromes: New Insights in Pathomechanisms, Instrumental Diagnosis and Surgical Treatment)

20 pages, 6246 KiB

Open AccessReview

Skull Vibration-Induced Nystagmus in Superior Semicircular Canal Dehiscence: A New Insight into Vestibular Exploration—A Review

by Georges Dumas, Ian Curthoys, Andrea Castellucci, Laurent Dumas, Laetitia Peultier-Celli, Enrico Armato, Pasquale Malara, Philippe Perrin and Sébastien Schmerber

Audiol. Res. 2024, 14(1), 96-115; https://doi.org/10.3390/audiolres14010009 - 22 Jan 2024

Viewed by 1653

Abstract

The third window syndrome, often associated with the Tullio phenomenon, is currently most often observed in patients with a superior semicircular-canal dehiscence (SCD) but is not specific to this pathology. Clinical and vestibular tests suggestive of this pathology are not always concomitantly observed and have been recently complemented by the skull-vibration-induced nystagmus test, which constitutes a bone-conducted Tullio phenomenon (BCTP). The aim of this work was to collect from the literature the insights given by this bedside test performed with bone-conducted stimulations in SCD. The PRISMA guidelines were used, and 10 publications were included and analyzed. Skull vibration-induced nystagmus (SVIN), as observed in 55 to 100% of SCD patients, usually signals SCD with greater sensitivity than the air-conducted Tullio phenomenon (ACTP) or the Hennebert sign. The SVIN direction when the test is performed on the vertex location at 100 Hz is most often ipsilaterally beating in 82% of cases for the horizontal and torsional components and down-beating for the vertical component. Vertex stimulations are more efficient than mastoid stimulations at 100 Hz but are equivalent at higher frequencies. SVIN efficiency may depend on stimulus location, order, and duration. In SCD, SVIN frequency sensitivity is extended toward high frequencies, with around 400 Hz being optimal. SVIN direction may depend in 25% on stimulus frequency and in 50% on stimulus location. Mastoid stimulations show frequently diverging results following the side of stimulation. An after-nystagmus observed in 25% of cases can be interpreted in light of recent physiological data showing two modes of activation: (1) cycle-by-cycle phase-locked activation of action potentials in SCC afferents with irregular resting discharge; (2) cupula deflection by fluid streaming caused by the travelling waves of fluid displacement initiated by sound or vibration at the point of the dehiscence. The SVIN direction and intensity may result from these two mechanisms’ competition. This instability explains the SVIN variability following stimulus location and frequency observed in some patients but also discrepancies between investigators. SVIN is a recent useful insight among other bedside examination tests for the diagnosis of SCD in clinical practice. Full article

(This article belongs to the Special Issue Third Mobile Window Syndromes: New Insights in Pathomechanisms, Instrumental Diagnosis and Surgical Treatment)

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11 pages, 3035 KiB

Open AccessReview

Hearing Results after Transmastoid Superior Semicircular Canal Plugging for Superior Semicircular Canal Dehiscence: A Meta-Analysis

by Efterpi Michailidou, Pascal Oliver Rüegg, Tanya Karrer, Athanasia Korda, Stefan Weder, Martin Kompis, Marco Caversaccio and Georgios Mantokoudis

Audiol. Res. 2023, 13(5), 730-740; https://doi.org/10.3390/audiolres13050065 - 8 Oct 2023

Cited by 1 | Viewed by 1904

Abstract

Objective: The transmastoid plugging of a superior semicircular canal is considered a safe and effective technique for the management of superior semicircular canal dehiscence (SSCD). The aim of this meta-analysis is to assess the postoperative hearing outcomes after the transmastoid plugging of the superior semicircular canal. Search method and data sources: A systematic database search was performed on the following databases until 30 January 2023: MEDLINE, Embase, Cochrane Library, Web of Science, CINAHL, ICTRP, and clinicaltrials.gov. A systematic literature review and meta-analysis of the pooled data were conducted. We also included a consecutive case series with SCDS for those who underwent transmastoid plugging treatment at our clinic. Results: We identified 643 citations and examined 358 full abstracts and 88 full manuscripts. A total of 16 studies were eligible for the systematic review and 11 studies for the meta-analysis. Furthermore, 159 ears (152 patients) were included. The postoperative mean air conduction threshold remained unchanged (mean difference, 2.89 dB; 95% CI: −0.05, 5.84 dB, p = 0.58), while the mean bone conduction threshold was significantly worse (mean difference, −3.53 dB; 95% CI, −6.1, −0.95 dB, p = 0.9). Conclusion: The transmastoid plugging technique for superior semicircular canal dehiscence syndrome, although minimally worsening the inner ear threshold, is a safe procedure in terms of hearing preservation and satisfactory symptom relief. Full article

(This article belongs to the Special Issue Third Mobile Window Syndromes: New Insights in Pathomechanisms, Instrumental Diagnosis and Surgical Treatment)

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13 pages, 1501 KiB

Open AccessFeature PaperEditor’s ChoiceReview

A Review of Neural Data and Modelling to Explain How a Semicircular Canal Dehiscence (SCD) Causes Enhanced VEMPs, Skull Vibration Induced Nystagmus (SVIN), and the Tullio Phenomenon

by Ian S. Curthoys, Christopher M. Smith, Ann M. Burgess and Julia Dlugaiczyk

Audiol. Res. 2023, 13(3), 418-430; https://doi.org/10.3390/audiolres13030037 - 2 Jun 2023

Cited by 5 | Viewed by 2548

Abstract

Angular acceleration stimulation of a semicircular canal causes an increased firing rate in primary canal afferent neurons that result in nystagmus in healthy adult animals. However, increased firing rate in canal afferent neurons can also be caused by sound or vibration in patients after a semicircular canal dehiscence, and so these unusual stimuli will also cause nystagmus. The recent data and model by Iversen and Rabbitt show that sound or vibration may increase firing rate either by neural activation locked to the individual cycles of the stimulus or by slow changes in firing rate due to fluid pumping (“acoustic streaming”), which causes cupula deflection. Both mechanisms will act to increase the primary afferent firing rate and so trigger nystagmus. The primary afferent data in guinea pigs indicate that in some situations, these two mechanisms may oppose each other. This review has shown how these three clinical phenomena—skull vibration-induced nystagmus, enhanced vestibular evoked myogenic potentials, and the Tullio phenomenon—have a common tie: they are caused by the new response of semicircular canal afferent neurons to sound and vibration after a semicircular canal dehiscence. Full article

(This article belongs to the Special Issue Third Mobile Window Syndromes: New Insights in Pathomechanisms, Instrumental Diagnosis and Surgical Treatment)

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4 pages, 776 KiB

Open AccessComment

Comment on Castellucci et al. Impaired Vestibulo-Ocular Reflex on Video Head Impulse Test in Superior Canal Dehiscence: “Spontaneous Plugging” or Endolymphatic Flow Dissipation? Audiol. Res. 2023, 13, 802–820

by Eugen Constant Ionescu, Eugenia Mustea, Pierre Reynard and Hung Thai-Van

Audiol. Res. 2024, 14(5), 857-860; https://doi.org/10.3390/audiolres14050072 - 23 Sep 2024

Viewed by 463

Abstract

We read, with great interest, two recent articles by Castellucci and al [...] Full article

(This article belongs to the Special Issue Third Mobile Window Syndromes: New Insights in Pathomechanisms, Instrumental Diagnosis and Surgical Treatment)

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