Risk of progressive hearing loss in untreated superior semicircular canal dehiscence

Patel, Neil; Hunter, Jacob B.; O’Connell, Brendan P.; Bertrand, Natalie M.; Wanna, George B.; Carlson, Matthew L.

doi:10.1002/lary.26322

Cited by 15 publications

(9 citation statements)

References 25 publications

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“…The reason for a mixed or conductive loss can be explained by the third window effect. Sensorineural hearing loss is not unknown either (16, 17, 24, 25) and in this series it was observed in 2 children. These children did not show any balance symptoms and the reason they were requested for a high resolution CT was because their MRI was normal as suggested in the aetiological diagnosis algorithm.…”

Section: Discussionmentioning

confidence: 49%

Functional and Objective Audiovestibular Evaluation of Children With Apparent Semicircular Canal Dehiscence–A Case Series in a Pediatric Vestibular Center

Dasgupta

Ratnayake

2019

Front. Neurol.

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Semicircular canal dehiscence is a bony abnormality in the otic capsule especially involving the superior semicircular canal. Since its identification in 1998, there is significant research regarding the pathology in the adult population. This condition generates a third window effect that is well–described in the literature. However, the entity is rare in the pediatric population with limited research. Difficulties encountered in children are obtaining a direct history that is essential for the diagnosis followed by neurovestibular tests that may be difficult to perform. This study presents observations regarding different clinical and diagnostic aspects of semicircular canal dehiscences in children as a retrospective audit in a tertiary pediatric vestibular center. Of 580 children assessed in a 30 months period undergoing comprehensive functional and objective audiovestibular assessment, 13 children (2.2%) were detected to possess radiological semicircular canal dehiscences (high resolution computed tomography scans at 0.625 mm slices reformatted in the axial, coronal and sagittal planes). The right superior semicircular canal was most commonly affected (66.6%). There were 4 bilateral semicircular canal dehiscences. Clinical suspicion of the condition was raised with reliable surrogate history from carers or from older children (100%), a mixed or conductive hearing loss (80% of hearing losses) in the presence of normal impedance audiometry (92.3%), normal transient otoacoustic emissions (84.6%) on the side of the dehiscence and the presence of replicable pathological saccades in the video head impulse test (76.9%). Disequilibrium symptoms and typical third window symptoms were absent or difficult to elicit in children (46.15 and 30.76% respectively). Only 3 (0.5%) fulfilled the adult criteria of a superior semicircular canal dehiscence syndrome. The abnormal video head impulse test characterized by pathological saccades may affect other non-dehisced ipsilateral canals. Semicircular canal dehiscences are rare in children but may be considered as an etiology for hearing losses and imbalance. Children with semicircular canal dehiscence may present differently from the classical superior semicircular canal dehiscence syndrome found in adults.

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Section: Discussionmentioning

confidence: 49%

Functional and Objective Audiovestibular Evaluation of Children With Apparent Semicircular Canal Dehiscence–A Case Series in a Pediatric Vestibular Center

Dasgupta

Ratnayake

2019

Front. Neurol.

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“…Among a series of patients with incidental or minimally symptomatic SSCD described by Patel et al, it was found that the median AC PTA was 18.5 dB, while the mean AC PTA was 38.0 dB among this cohort of patients, when matched for age. 16 When evaluating the BC PTA among this cohort of patients, the mean was 24.3, compared to the BC PTA from Patel et al that was 13.3 dB. Thus, this cohort of patients is found to have a worse bone line than in previously published cohorts.…”

Section: Discussionmentioning

confidence: 59%

“…15 If symptoms are mild, many patients elect to defer surgery, owing to the lack of demonstrated hearing decline with observation. 16 In the present study, we describe a series of patients who presented with symptoms and imaging consistent with SSCD syndrome after trauma, suggesting that a patient's trauma could push them "over the edge" in terms of developing SSCD syndrome symptoms. We theorize that these patients likely have a thin layer of endosteum still covering the dehiscence that is compromised with an abrupt increase in intra-vestibular or intracranial pressure-a so-called, "unmasking" of the condition in a patient who is already anatomically predisposed (Fig.…”

Section: Discussionmentioning

confidence: 83%

Superior Semicircular Canal Dehiscence Syndrome Following Head Trauma: A Multi‐institutional Review

et al. 2021

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Objective: To evaluate patients who become symptomatic from superior semicircular canal dehiscence (SSCD) following head trauma.Study Design: Case series assessing patients presenting with SSCD after a trauma. Methods: A case series was completed assessing patients presenting with SSCD after trauma. Data from three academic medical centers were evaluated, including the following: imaging, videonystagmography (VNG)/vestibular evoked myogenic potential (VEMP) testing, audiometric assessment, and surgical repair. Outcome measures included the following: 1) Description of audio-vestibular symptoms, 2) mean pre-and post-operative pure tone average (PTA), word recognition score (WRS), and air bone gap (ABG).Results: A total of 14 patients were included; 86% were male. Approximately 43% were found to have bilateral SSCD on imaging, with 57% of patients pursuing surgical management. The most common presenting symptoms included pulsatile tinnitus (93%), autophony (79%), and hearing loss (64%). Approximately 36% of patients underwent VNG/VEMP testing, with 83.3% of those demonstrating abnormal results. The mean audiometric findings on the symptomatic side included an air-conduction PTA of 38.0 dB, bone-conduction PTA of 24.3 dB, WRS of 81%, and ABG of 17.9 dB. Among patients who underwent surgery (57%), there was no significant change in the air-conduction PTA, bone-conduction PTA, or WRS (P > .05). However, there was an improvement in the ABG (preoperative = 22.8 dB versus postoperative = 9.7 dB; P = .005).Conclusion: Head trauma may be a potentiating event for SSCD syndrome. This study advances the hypothesis that these patients likely have underlying radiographic SSCD prior to their trauma, and a traumatic event increases in intra-vestibular or intracranial pressures, unmasking SSCD syndrome.

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“…It appears that the hearing loss does not significantly change over time (68). There have been reported cases of worsening conductive hearing loss over time and cases have been reported in which symptoms progressed over many years (35, 47, 69), while at least one case developed rapid mixed hearing loss (70).…”

Section: Symptomsmentioning

confidence: 99%

Superior Canal Dehiscence Syndrome: Lessons from the First 20 Years

2017

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Superior semicircular canal dehiscence syndrome was first reported by Lloyd Minor and colleagues in 1998. Patients with a dehiscence in the bone overlying the superior semicircular canal experience symptoms of pressure or sound-induced vertigo, bone conduction hyperacusis, and pulsatile tinnitus. The initial series of patients were diagnosed based on common symptoms, a physical examination finding of eye movements in the plane of the superior semicircular canal when ear canal pressure or loud tones were applied to the ear, and high-resolution computed tomography imaging demonstrating a dehiscence in the bone over the superior semicircular canal. Research productivity directed at understanding better methods for diagnosing and treating this condition has substantially increased over the last two decades. We now have a sound understanding of the pathophysiology of third mobile window syndromes, higher resolution imaging protocols, and several sensitive and specific diagnostic tests. Furthermore, we have a treatment (surgical occlusion of the superior semicircular canal) that has demonstrated efficacy. This review will highlight some of the fundamental insights gained in SCDS, propose diagnostic criteria, and discuss future research directions.

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Risk of progressive hearing loss in untreated superior semicircular canal dehiscence

Abstract: 4. Laryngoscope, 127:1181-1186, 2017.

Cited by 15 publications

References 25 publications

Functional and Objective Audiovestibular Evaluation of Children With Apparent Semicircular Canal Dehiscence–A Case Series in a Pediatric Vestibular Center

Functional and Objective Audiovestibular Evaluation of Children With Apparent Semicircular Canal Dehiscence–A Case Series in a Pediatric Vestibular Center

Superior Semicircular Canal Dehiscence Syndrome Following Head Trauma: A Multi‐institutional Review

Superior Canal Dehiscence Syndrome: Lessons from the First 20 Years

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