Systematic Review of Round Window Operations for the Treatment of Superior Semicircular Canal Dehiscence

Superior semicircular canal dehiscence (SSCD) syndrome is an increasingly recognized cause of vestibular and/or auditory symptoms in both adults and children. These symptoms are believed to result from the presence of a pathological mobile “third window” into the labyrinth due to deficiency in the osseous shell, leading to inadvertent hydroacoustic transmissions through the cochlea and labyrinth. The most common bony defect of the superior canal is found over the arcuate eminence, with rare cases involving the posteromedial limb of the superior canal associated with the superior petrosal sinus. Operative intervention is indicated for intractable or debilitating symptoms that persist despite conservative management and vestibular sedation. Surgical repair can be accomplished by reconstruction or plugging of the bony defect or reinforcement of the round window through a variety of operative approaches. The authors review the etiology, pathophysiology, presentation, diagnosis, surgical options, and outcomes in the treatment of this entity, with a focus on potential pitfalls that may be encountered during clinical management.

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Case Report: Local Anesthesia Round Window Plugging and Simultaneous Vibrant Soundbridge Implant for Superior Semicircular Canal Dehiscence

Frontiers in Neurology ◽

10.3389/fneur.2020.581783 ◽

2020 ◽

Vol 11 ◽

Author(s):

Giulia Mignacco ◽

Lorenzo Salerni ◽

Ilaria Bindi ◽

Giovanni Monciatti ◽

Alfonso Cerase ◽

...

Keyword(s):

Semicircular Canal ◽

Round Window ◽

Bone Conduction ◽

Hearing Threshold ◽

Vestibular Evoked Myogenic Potentials ◽

Vibrant Soundbridge ◽

Superior Semicircular Canal Dehiscence ◽

Superior Semicircular Canal ◽

The Right ◽

Canal Dehiscence

The aim of the present study is to report the outcomes of round window reinforcement surgery performed with the application of a Vibrant Soundbridge middle ear implant (VSB; MED-EL) in a patient with superior semicircular canal dehiscence (SSCD) who presented with recurrent vertigo, Tullio phenomenon, Hennebert's sign, bone conduction hypersensitivity, and bilateral moderate to severe mixed hearing loss. Vestibular evoked myogenic potentials (VEMPs) and high-resolution computed tomography (HRCT) confirmed bilateral superior semicircular canal dehiscence while this was not seen in magnetic resonance imaging. The surgical procedure was performed in the right ear as it had worse vestibular and auditory symptoms, a poorer hearing threshold, and greatly altered HRCT and VEMPs findings. With local-assisted anesthesia, round window reinforcement surgery (plugging) with perichondrium was performed with simultaneous positioning of a VSB on the round window niche. At the one and 3 months follow-up after surgery, VSB-aided hearing threshold in the right ear improved to mild, and loud sounds did not elicit either dizziness or pain in the patient.

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Age and gender considerations on the symptomology in patients with superior semicircular canal dehiscence: A systematic review and case illustration

Journal of Clinical Neuroscience ◽

10.1016/j.jocn.2019.04.006 ◽

2019 ◽

Vol 65 ◽

pp. 112-120 ◽

Cited By ~ 1

Author(s):

Thien Nguyen ◽

John P. Sheppard ◽

Courtney Duong ◽

Kevin Ding ◽

Dillon Dejam ◽

...

Keyword(s):

Systematic Review ◽

Semicircular Canal ◽

Case Illustration ◽

Age And Gender ◽

Superior Semicircular Canal Dehiscence ◽

Superior Semicircular Canal ◽

And Gender ◽

Canal Dehiscence

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The influence of the subarcuate artery in the superior semicircular canal dehiscence and its frequency on stillbirths: illustrative cases and systematic review

Acta Oto-Laryngologica ◽

10.1080/00016489.2017.1416169 ◽

2017 ◽

Vol 138 (5) ◽

pp. 437-442 ◽

Cited By ~ 1

Author(s):

Gabriela Pereira Bom Braga ◽

Jack H. Noble ◽

Eloisa Maria Mello Santiago Gebrim ◽

Robert F. Labadie ◽

Ricardo Ferreira Bento

Keyword(s):

Systematic Review ◽

Semicircular Canal ◽

Superior Semicircular Canal Dehiscence ◽

Superior Semicircular Canal ◽

Subarcuate Artery ◽

Canal Dehiscence

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Occlusion of the round window: a novel way to treat hyperacusis symptoms in superior semicircular canal dehiscence syndrome

The Journal of Laryngology & Otology ◽

10.1017/s0022215113001096 ◽

2013 ◽

Vol 127 (7) ◽

pp. 705-707 ◽

Cited By ~ 20

Author(s):

A Nikkar-Esfahani ◽

D Whelan ◽

A Banerjee

Keyword(s):

Semicircular Canal ◽

Round Window ◽

Round Window Membrane ◽

Superior Semicircular Canal Dehiscence ◽

Tullio Phenomenon ◽

Superior Semicircular Canal ◽

Case Surgery ◽

Post Operation ◽

Round Window Niche ◽

Canal Dehiscence

AbstractBackground:Conductive hyperacusis in superior semicircular canal dehiscence syndrome occurs due to the presence of a ‘third window’ created by the dehiscence. Reversible blocking of the round window can, in theory, cause a reduction in the compression-related volume displacement, and thereby minimise symptoms of conductive hyperacusis. This study describes a technique of permeatal blocking of the round window.Method:The tympanomeatal flap is elevated and the round window niche is identified. The round window membrane is subsequently identified and occluded with bone wax, muscle and fascia, in three separate layers. Finally, the tympanomeatal flap is reflected, and an ear wick is inserted.Results:Two patients who underwent the procedure reported a reduction in symptoms. Importantly, no Tullio phenomenon was reported post-operation.Conclusion:Blocking of the round window can be used to control symptoms of superior semicircular canal dehiscence syndrome in patients who present solely with symptoms of conductive hyperacusis. This technique provides an alternative to resurfacing techniques. The procedure is simple to perform, reversible and can be undertaken as day-case surgery.

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Complete round Window Niche Occlusion for Superior Semicircular Canal Dehiscence Syndrome: A Minimally Invasive Approach

Ear Nose & Throat Journal ◽

10.1177/014556130908800808 ◽

2009 ◽

Vol 88 (8) ◽

pp. 1042-1056 ◽

Cited By ~ 24

Author(s):

Herbert Silverstein ◽

Mark J. Van Ess

Keyword(s):

Semicircular Canal ◽

Round Window ◽

Surgical Techniques ◽

Invasive Approach ◽

Superior Semicircular Canal Dehiscence ◽

Superior Semicircular Canal ◽

Auditory Dysfunction ◽

Round Window Niche ◽

Canal Dehiscence

Superior semicircular canal dehiscence (SCD) syndrome is a recognized condition associated with varying degrees of vestibular and auditory dysfunction. The authors present a case study of disabling SCD syndrome in a 37-year-old man who was successfully treated with a complete round window niche occlusion via a transcanal approach. This case represents the first report of a transcanal complete round window niche occlusion for the treatment of SCD syndrome. A brief literature review and discussion of surgical techniques follow.

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