Evidence of Osteoclastic Activity in the Human Temporal Bone

Kamakura, Takefumi; Nadol, Joseph B.

doi:10.1159/000481279

Cited by 13 publications

(10 citation statements)

References 33 publications

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“…The presence of microcavitations in the temporal bone is consistent with osteoclastic activity, which may play a role in the development of SCD. 3 There were also microdehiscences of the mastoid tegmen and over the geniculate ganglion in this case, which is consistent with the hypothesis that superior canal dehiscence may be secondary to failure of postnatal bone development. 2 The visual depiction of the exposed geniculate ganglion is also a reminder to surgeons that care must be taken with elevation of the dura anteriorly over the geniculate ganglion during middle fossa surgery.…”

Section: Discussionsupporting

confidence: 89%

“…In the present case, microcavitations of bone suggesting osteoclastic activity were seen at the left ear common crus but not adjacent to the right‐sided superior canal dehiscence. The presence of microcavitations in the temporal bone is consistent with osteoclastic activity, which may play a role in the development of SCD . There were also microdehiscences of the mastoid tegmen and over the geniculate ganglion in this case, which is consistent with the hypothesis that superior canal dehiscence may be secondary to failure of postnatal bone development .…”

Section: Discussionsupporting

confidence: 84%

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Temporal bone histopathology: Superior semicircular canal dehiscence

Lin

Reinshagen

Nadol

et al. 2019

Laryngoscope Investig Oto

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Objectives To present a histopathological case of a 91‐year‐old woman who was diagnosed with superior semicircular canal dehiscence postmortem. Methods The patient was a registered donor with the National Temporal Bone Donor Program at the NIDCD National Temporal Bone, Hearing and Balance Pathology Resource Registry. Computed tomography imaging was performed on each temporal bone. The temporal bones were decalcified with ethylenediaminetetracetate and embedded in celloidin, and tissue sections were stained with hematoxylin and eosin. Horizontal sections were taken through the left temporal bone, and vertical sections were taken through the right temporal bone. Results Histopathological sections taken through the right temporal bone demonstrated no bone between the membranous wall of the superior semicircular canal and the middle fossa dura. There was no histopathological evidence of superior semicircular canal dehiscence in the left temporal bone; however, a small dehiscence would not be identified on horizontal sections. Microcavitations were observed in the common crus of the left temporal bone. Conclusion This reports describes the case of a woman who was diagnosed with superior semicircular canal dehiscence postmortem. The presence of microcavitations in the temporal bone is consistent with osteoclastic activity, which may play a role in the development of superior canal dehiscence.

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Section: Discussionsupporting

confidence: 89%

Section: Discussionsupporting

confidence: 84%

Temporal bone histopathology: Superior semicircular canal dehiscence

Lin

Reinshagen

Nadol

et al. 2019

Laryngoscope Investig Oto

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“…The only other published radiologic abnormality was reported in one patient with DFNA9 mutation presenting with bilateral superior semicircular canal dehiscence (SSCD) on CT imaging (4). While another case of SSCD was identified on histopathology in a DFNA9 patient, probably a circumstantial finding unrelated to DFNA9 (15).…”

Section: Discussionmentioning

confidence: 97%

Correlations Between Vestibular Function and Imaging of the Semicircular Canals in DFNA9 Patients

et al. 2020

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Background and Purpose: Radiologic abnormalities on computed tomography (CT), including narrowing or sclerosis of the semicircular canals (SCCs), and T2-weighted magnetic resonance imaging (MRI), including signal loss in the SCC, have been reported as potential biomarkers in patients with P51S mutations in the COCH gene (i.e., DFNA9). The aim of our study was to correlate caloric responses through electronystagmography (ENG) data with imaging results in DFNA9 patients. Materials and Methods: A retrospective study was performed in 45 patients; therefore, 90 ears with P51S mutations in the COCH gene were tested. Caloric responses and CT and MRI data were analyzed from June 2003 until May 2014. More than half of patients (54%) were candidates for cochlear implantation. Results: In our population, 91% of tested ears had sclerotic lesions and/or narrowing in one or more SCCs on CT scan. All tested ears had narrowing or signal loss in at least one SCC on T2-weighted MRI. The lateral SCC was affected in 87% on CT scan and 92% on MRI. However, in 83% of tested ears, all three SCCs were affected on MRI. Furthermore, in 77% of tested ears, caloric responses were reduced bilaterally, while 11.5% showed unilateral hypofunction and the other 11.5% had normal caloric responses. CT abnormalities correlated with hypofunction of caloric responses. This statistically significant difference was present if abnormalities were observed in at least one of the SCCs as well as in ipsilateral lateral SCC function loss. MRI abnormalities in at least one of the SCCs correlated with ENG hypofunction, but there was no direct correlation between lateral SCC abnormalities on MRI and caloric responses of the investigated lateral canal. Conclusion: Our retrospective analysis confirms the presence of CT and MRI abnormalities in DFNA9 patients with the P51S mutation in the COCH gene. A correlation between these radiologic features and vestibular function (tested by means of caloric response) was found in this population.

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“…Others have argued for an acquired etiology. The finding of osteoclastic activity near the superior semicircular canal has been noted in one temporal bone study [24]. The authors suggested that this indicates that increased bone turnover may lead to reabsorption of previously developed bone and the initiation of a third window.…”

Section: Introductionmentioning

confidence: 84%

Superior semicircular canal dehiscence syndrome: Diagnostic criteria consensus document of the committee for the classification of vestibular disorders of the Bárány Society

Ward

Berg

Rompaey

et al. 2021

VES

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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.

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Evidence of Osteoclastic Activity in the Human Temporal Bone

Cited by 13 publications

References 33 publications

Temporal bone histopathology: Superior semicircular canal dehiscence

Temporal bone histopathology: Superior semicircular canal dehiscence

Correlations Between Vestibular Function and Imaging of the Semicircular Canals in DFNA9 Patients

Superior semicircular canal dehiscence syndrome: Diagnostic criteria consensus document of the committee for the classification of vestibular disorders of the Bárány Society

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