The Association Between Semicircular Canal Dehiscence and Chiari Type I Malformation

Kuhn, Jeffery J.; Clenney, Timothy L.

doi:10.1001/archoto.2010.169

Cited by 15 publications

(5 citation statements)

References 43 publications

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“…The audiological symptomatology and investigation findings are no different to patients with superior canal dehiscence, although the authors did not comment or record the eye movements to sound exposure. A further retrospective case series of 32 patients diagnosed with semicircular canal dehiscence found a Chiari type I malformation in six patients, 83% of these in association with unilateral or bilateral posterior SCD, suggesting a high prevalence of Chiari type I malformation in SCD, particularly of the posterior canal variety [53]. We found no cases of Chiari malformation in our case series.…”

Section: Pathophysiologysupporting

confidence: 41%

The Tullio phenomenon: a neurologically neglected presentation

et al. 2011

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The Tullio phenomenon refers to sound-induced disequilibrium or oscillopsia. Patients with this condition frequently present to neurologists, many of whom are unfamiliar with the condition and its diagnostic criteria. Indeed, due to the unusual nature of the symptoms patients are often misdiagnosed as having psychiatric disturbances. Tullio patients describe disequilibrium, auditory and visual symptoms, which are recurrent, brief, and often triggered by loud noises or middle ear pressure changes, e.g. the Valsalva manoeuvre. Many cases are associated with superior semicircular canal dehiscence (SCCD). Early work suggested that the presence of sound-induced torsional eye movements and visual field tilts were consequent upon a utricular-mediated ocular tilt reaction. However, more recent evidence from imaging and oculographic research, as well as data from our patient series indicates that these ocular abnormalities are usually the result of superior semicircular canal stimulation. The clinical history and a focussed examination are often sufficient to make the diagnosis, which can be confirmed with high resolution CT imaging of the temporal bones. In some patients, surgical occlusion or resurfacing of the affected canal can ameliorate symptoms and signs. The aim of this paper is two-fold: Firstly, to review the clinical features of the Tullio phenomenon, and secondly, to highlight our own observations in three cases with a new clinical syndrome consisting of Tullio's phenomenon with bilateral vestibular failure, a pure horizontal nystagmus in response to sound, and no evidence of canal dehiscence.

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Section: Pathophysiologysupporting

confidence: 41%

The Tullio phenomenon: a neurologically neglected presentation

et al. 2011

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“…Evidence supporting a congenital etiology includes the observations that SSCD syndrome exists in children (9) and that SSCD has been identified in patients with congenital anomalies such as malformations of the labyrinth (10) and brainstem (11). However, the findings that SSCD is associated with increased age (7), osteopenia (12), and trauma (2) support an acquired cause of SSCD.…”

Section: Introductionmentioning

confidence: 99%

Radiographic Features of Superior Semicircular Canal Dehiscence in the Setting of Chronic Ear Disease

Gartrell

Gentry

Kennedy³

et al. 2014

Otology &Amp; Neurotology

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Objective To determine if radiologic chronic otitis media (COM), both with and without cholesteatoma, is associated with superior semicircular canal dehiscence (SSCD). Study Design Retrospective review of consecutive high-resolution computed tomography (HRCT) scans of the temporal bone. Setting Tertiary care medical center Patients Two-hundred consecutive patients undergoing HRCT of the temporal bone beginning January 1st, 2012. Intervention Imaging was evaluated by three reviewers (two neuroradiologists and one neurotologist). All scans were assessed for the presence of SSCD, cholesteatoma, chronic otomastoiditis, tegmen dehiscence, and for abnormalities of the cochlea, vestibule, facial nerve, and temporal bone vasculature. Main Outcome Measure Ears with COM associated with chronic otomastoiditis or cholesteatoma were compared to those without COM with respect to the presence of SSCD or other temporal bone abnormalities. Statistical analysis was performed to assess for differences between the groups studied. Results 194 patients (388 ears) were included. Cholesteatoma was identified in 48 ears (12.4%) and chronic otomastoiditis in 62 ears (16%). Ten ears with cholesteatoma had ipsilateral SSCD and 8 ears with chronic otomastoiditis had ipsilateral SSCD. In 340 ears without either cholesteatoma or chronic otomastoiditis, SSCD was found in 18 (5.3%). SSCD was found to occur significantly more often in patients with ipsilateral radiologic cholesteatoma. No cases of SSCD were associated with cochlear, facial nerve, or vascular abnormalities. Conclusion Our findings suggest that COM with cholesteatoma is associated with the presence of SSCD, although the nature of this association is unclear.

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“…[14][15][16][17] An increased incidence of Chiari type I malformations, another condition associated with increased intracranial pressure, has been described in patients with SCD. 18 This indicates a possible role of increased intracranial pressure in the development SCD. Because of similar etiologies and their possible association with elevated intracranial pressure, patients with spontaneous CSF otorrhea may have an increased prevalence of SCD.…”

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confidence: 95%

“…Conditions associated with increased intracranial pressure, such as obesity or benign intracranial hypertension, increase the risk of developing spontaneous CSF otorrhea 14–17 . An increased incidence of Chiari type I malformations, another condition associated with increased intracranial pressure, has been described in patients with SCD 18 . This indicates a possible role of increased intracranial pressure in the development SCD.…”

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confidence: 99%

Superior Semicircular Canal Dehiscence in Patients with Spontaneous Cerebrospinal Fluid Otorrhea

Allen

Perez

Isaacson

et al. 2012

Otolaryngol.--head neck surg.

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The Association Between Semicircular Canal Dehiscence and Chiari Type I Malformation

Abstract: Among patients with SCD, the prevalence of CM-I is elevated. This association is especially marked in patients with posterior SCD. This finding suggests a relationship between CM-I and SCD, particularly with posterior SCD.

Cited by 15 publications

References 43 publications

The Tullio phenomenon: a neurologically neglected presentation

The Tullio phenomenon: a neurologically neglected presentation

Radiographic Features of Superior Semicircular Canal Dehiscence in the Setting of Chronic Ear Disease

Superior Semicircular Canal Dehiscence in Patients with Spontaneous Cerebrospinal Fluid Otorrhea

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