scholarly journals Simultaneous, unilateral plugging of superior and posterior semicircular canal dehiscences to treat debilitating hyperacusis

2014 ◽  
Vol 128 (2) ◽  
pp. 174-178 ◽  
Author(s):  
P T Dang ◽  
T A Kennedy ◽  
S P Gubbels

AbstractObjective:To describe a case of bilateral superior and posterior semicircular canal dehiscences, and the use of a unilateral transmastoid approach to address both right-sided defects simultaneously.Case report:In a patient with right-sided hyperacusis, bilateral dehiscence of both the superior and the posterior semicircular canals was identified, located adjacent to the common crus, together with a right-sided, anterosuperiorly positioned sigmoid sinus and a high-riding jugular bulb. Results for audiography and cervical vestibular evoked myogenic potential testing were consistent with right-sided semicircular canal dehiscence. At surgery, a right-sided transmastoid approach provided access to plug both defects simultaneously, following posterior mobilisation of the sigmoid sinus. The patient's hyperacusis was completely resolved, with a 10–30 dB improvement in his right ear air conduction hearing, without decrement in bone conduction.Conclusion:In properly selected patients, a transmastoid approach can be used to effectively manage superior semicircular canal dehiscence and posterior semicircular canal dehiscence simultaneously. Pre-operative computed tomography is recommended to evaluate the dehiscence sites and to identify complicating vascular anatomy.

2012 ◽  
Vol 126 (9) ◽  
pp. 928-931 ◽  
Author(s):  
H W Lim ◽  
H J Park ◽  
J H Jung ◽  
J W Chung

AbstractObjective:We report a rare case of posterior semicircular canal dehiscence caused by a jugular diverticulum, and we describe its surgical treatment using a dehiscence resurfacing manoeuvre.Method:The clinical findings, surgical procedure and outcomes are presented.Results:A 66-year-old man presented with disequilibrium, sound-induced vertigo, a reduced ocular vestibular evoked myogenic potential threshold, and pressure-induced vertical and torsional nystagmus. Computed tomography revealed a right posterior semicircular canal dehiscence caused by a diverticulum of the jugular bulb. The defect in the posterior semicircular canal was localised and resurfaced with bone paté, temporalis muscle fascia and conchal cartilage, under direct visualisation. Post-operatively, the patient's symptoms disappeared and his ocular vestibular evoked myogenic potential threshold normalised.Conclusion:This case illustrates that posterior semicircular canal dehiscence can be surgically managed by resurfacing the defect site via a transmastoid approach.


2021 ◽  
pp. 1-10
Author(s):  
Bryan K. Ward ◽  
Raymond van de Berg ◽  
Vincent van Rompaey ◽  
Alexandre Bisdorff ◽  
Timothy E. Hullar ◽  
...  

This paper describes the diagnostic criteria for superior semicircular canal dehiscence syndrome (SCDS) as put forth by the classification committee of the Bárány Society. In addition to the presence of a dehiscence of the superior semicircular canal on high resolution imaging, patients diagnosed with SCDS must also have symptoms and physiological tests that are both consistent with the pathophysiology of a ‘third mobile window’ syndrome and not better accounted for by another vestibular disease or disorder. The diagnosis of SCDS therefore requires a combination of A) at least one symptom consistent with SCDS and attributable to ‘third mobile window’ pathophysiology including 1) hyperacusis to bone conducted sound, 2) sound-induced vertigo and/or oscillopsia time-locked to the stimulus, 3) pressure-induced vertigo and/or oscillopsia time-locked to the stimulus, or 4) pulsatile tinnitus; B) at least 1 physiologic test or sign indicating that a ‘third mobile window’ is transmitting pressure including 1) eye movements in the plane of the affected superior semicircular canal when sound or pressure is applied to the affected ear, 2) low-frequency negative bone conduction thresholds on pure tone audiometry, or 3) enhanced vestibular-evoked myogenic potential (VEMP) responses (low cervical VEMP thresholds or elevated ocular VEMP amplitudes); and C) high resolution CT imaging with multiplanar reconstruction in the plane of the superior semicircular canal consistent with a dehiscence. Thus, patients who meet at least one criterion in each of the three major diagnostic categories (symptoms, physiologic tests, and imaging) are considered to have SCDS.


2018 ◽  
Vol 158 (3) ◽  
pp. 534-540 ◽  
Author(s):  
Renee M. Banakis Hartl ◽  
Stephen P. Cass

Objectives (1) Evaluate changes in subjective symptoms in patients following transmastoid canal plugging for superior semicircular canal dehiscence (SSCD) syndrome. (2) Quantify changes in hearing in patients who have undergone transmastoid canal plugging for SSCD syndrome. Study Design Case series with chart review. Setting Single tertiary care institution. Subjects and Methods We retrospectively reviewed patients with SSCD who underwent repair with canal plugging via a transmastoid approach between January 2012 and January 2017. Symptom severity was assessed prospectively (autophony, sound/pressure-induced vertigo, disequilibrium, aural fullness, and pulsatile tinnitus) and after surgery. Pure-tone and speech audiometry were measured before and after surgery. Two-sided Wilcoxon rank-sum tests were used to evaluate changes in subjective symptoms and audiometric outcomes. Results Seventeen patients (19 ears) met inclusion criteria. The superior canal was successfully plugged via the transmastoid approach in all cases. Patients reported a statistically significant improvement in autophony, vertigo, aural fullness, and pulsatile tinnitus ( P < .01), without significant improvement in disequilibrium rating ( P = .06). There were no changes noted in pure-tone average or word recognition score; however, there was a statistically significant improvement in air-bone gap at 250 Hz of 10.9 dB ( P = .04) with 12.9-dB improvement in air conduction thresholds ( P = .02) and no difference (0.9 dB, P = .9) in bone conduction thresholds. Conclusion In our study, patients with SSCD demonstrated excellent hearing outcomes and resolution of most otologic symptoms after surgical repair. Transmastoid canal plugging, which has been described to date only in smaller case series, is a safe and effective alternative to the traditional middle cranial fossa approach.


2009 ◽  
Vol 24 (2) ◽  
pp. 6-13
Author(s):  
Scheherazade C. Ibrahim ◽  
Charlotte M. Chiong ◽  
Nathaniel W. Yang

Objective: This report aims to determine the clinical manifestations and management of patients with superior semicircular canal dehiscence syndrome (SSCDS). Methods: Study Design: Case series. Setting: Tertiary hospitals and private clinics Participants: Out of 30 patients with vestibular vertigo or otologic symptom, 14 patients were diagnosed with SSCDS based on high resolution computed tomographic scan (HRCT).  The demographic features, incidence of specific signs and symptoms and management of these patients were described, including the audiograms, vestibular evoked myogenic potential (VEMP) responses and ancillary tests. Results: Vertigo was the most common vestibular symptom of SSCDS. Tullio phenomenon was elicited in 50% of patients with confirmed dehiscence on HRCT scan. Low frequency (250 Hz and 500 Hz) air-bone gap was noted in 21.4% of patients. Lowered VEMP responses were also noted in 66.7% of patients with confirmed SSCDS. Severity of symptoms may determine its management. Conclusion: The diagnosis of SSCDS does not conform to a specific clinical presentation or audiologic result thus good clinical correlation is needed in order to raise suspicion of the disease and prompt the clinician to order confirmatory imaging by computed tomographic scan or magnetic resonance imaging. The presence of this syndrome in a proportion of children that is greater than previously reported needs further study as these children may be genetically predisposed to have thinned out superior semicircular canals that eventually become dehisced albeit at an earlier age. Key words: Superior semicircular canal dehiscence, pure tone audiometry, vestibular evoked myogenic potential


2020 ◽  
Vol 11 ◽  
Author(s):  
Giulia Mignacco ◽  
Lorenzo Salerni ◽  
Ilaria Bindi ◽  
Giovanni Monciatti ◽  
Alfonso Cerase ◽  
...  

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.


2003 ◽  
Vol 117 (7) ◽  
pp. 553-557 ◽  
Author(s):  
G. Michael Halmagyi ◽  
Swee T. Aw ◽  
Leigh A. McGarvie ◽  
Michael J. Todd ◽  
Andrew Bradshaw ◽  
...  

This is a report of a patient with an air-bone gap, thought 10 years ago to be a conductive hearing loss due to otosclerosis and treated with a stapedectomy. It now transpires that the patient actually had a conductive hearing gain due to superior semicircular canal dehiscence. In retrospect for as long as he could remember the patient had experienced cochlear hypersensitivity to bone-conducted sounds so that he could hear his own heart beat and joints move, as well as a tuning fork placed at his ankle. He also had vestibular hypersensitivity to air-conducted sounds with sound-induced eye movements (Tullio phenomenon), pressure-induced nystagmus and low-threshold, high-amplitude vestibular-evoked myogenic potentials. Furthermore some of his acoustic reflexes were preserved even after stapedectomy and two revisions. This case shows that if acoustic reflexes are preserved in a patient with an air-bone gap then the patient needs to be checked for sound- and pressure-induced nystagmus and needs to have vestibular-evoked myogenic potential testing. If there is sound- or pressure-induced nystagmus and if the vestibular-evoked myogenic potentials are also preserved, the problem is most likely in the floor of the middle fossa and not in the middle ear, and the patient needs a high-resolution spiral computed tomography (CT) of the temporal bones to show this.


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