Otosclerosis and Dysplasias of the Temporal Bone

Sakai, Osamu; Curtin, Hugh D.; Hasso, Anton N.; Swartz, Joel D.

doi:10.1016/b978-0-323-05355-6.00019-7

Cited by 5 publications

(3 citation statements)

References 80 publications

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“…Inclusion criteria for treatment were progression of the sensorineural component of hearing loss in at least one ear, surgical confirmation of the diagnosis of otosclerosis in one or both ears, and evidence of retrofenestral otosclerosis on computed tomography (CT). Retrofenestral otosclerosis was defined as extension of low-density lucencies around the cochlear capsule[10]. …”

Section: Methodsmentioning

confidence: 99%

Third-Generation Bisphosphonates for Treatment of Sensorineural Hearing Loss in Otosclerosis

Quesnel

Seton²,

Merchant³

et al. 2012

Otology &Amp; Neurotology

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Objectives/Hypothesis Otosclerosis is a disease of abnormal bone remodeling in the otic capsule. Although several drugs that inhibit bone remodeling, including sodium fluoride and first generation bisphosphonates, have been tried, there remains no clearly effective treatment for otosclerosis-related sensorineural hearing loss (SNHL). Over the past several years, third generation bisphosphonates, with more powerful anti-resorptive properties and increased bone affinity, have been developed and have demonstrated effectiveness in the treatment of osteoporosis and other metabolic bone diseases. We hypothesized that newer generation bisphosphonates, such as risedronate and zoledronate, would be effective in slowing the progression of SNHL in patients with otosclerosis. Study design Retrospective review Setting Tertiary referral center, ambulatory care Interventions Risedronate or zoledronate administration Main outcome measures Bone conduction pure tone threshold averages (PTA) and word recognition (WR) scores were examined for each ear before and after bisphosphonate treatment. Criteria for significant change were defined as greater than 10 decibels in PTA or between 4 to 18% in WR based on binomial variance. Results All 10 patients had audiometric progression of SNHL in the pre-treatment monitoring interval and 12 ears met criteria for significant progression. All 10 patients (19 ears) showed at least no significant progression of SNHL (i.e. stabilization) at an average follow up of 13 months. Two patients (3 ears) showed improvement by defined audiometric criteria. There were no major complications. Conclusion Treatment with zoledronate or risedronate stabilized progressive SNHL related to otosclerosis in this small group of patients. Further evaluation of third generation bisphosphonate treatments is warranted.

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Section: Methodsmentioning

confidence: 99%

Third-Generation Bisphosphonates for Treatment of Sensorineural Hearing Loss in Otosclerosis

Quesnel

Seton²,

Merchant³

et al. 2012

Otology &Amp; Neurotology

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“…1 However, during the active or spongiotic phase of otosclerosis, the dense middle layer of enchondral bone is resorbed and replaced by spongy vascular bone, resulting in lower density on CT. 1, 5, 9 During the inactive phase, the affected areas of otosclerosis undergo new bone formation, thus mimicking the density of normal bone on CT. 2,9 Our study hypothesis is based on the pathophysiology that otosclerotic foci undergo continuous resorption and remodeling eventually resulting in production of more mature bone often larger than the original affected area, thus leading to focal thickening of the otic capsule. 1, 9 For this reason, our study included all consecutive patients with clinically diagnosed otosclerosis regardless of phase of disease. Because the otic capsule adjacent to the anterior margin of the oval window is expected to enlarge with remodeling, this bone should be thicker in otosclerosis patients compared to normal-hearing patients regardless of phase of disease.…”

Section: Discussionmentioning

confidence: 99%

“…Interestingly, 1.9% of otosclerosis patients had isolated round window lesions without associated disease anterior to the oval window, a higher percentage than reported in prior literature. 18 Although imaging may not be necessary in diagnosis of patients who present with characteristic clinical findings and typical audiometric tests, 9 imaging is helpful for diagnosis in cases of sensorineural or mixed hearing loss, evaluating for other differential diagnoses or coexisting diseases, and preoperative anatomical assessment. 2, 3, 6, 8 Most of our patients underwent imaging for preoperative evaluation prior to stapes prosthesis and cochlear implant insertion for fenestral and retrofenestral otosclerosis, respectively.…”

Section: Discussionmentioning

confidence: 99%

Retrospective Review of Otic Capsule Contour and Thickness in Patients with Otosclerosis and Individuals with Normal Hearing on CT

Sanghan

Chansakul

Kozin³

et al. 2018

AJNR Am J Neuroradiol

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Background and Purpose: Otosclerosis is commonly identified on CT as a focus of hypodensity in the otic capsule (OC) anterior to the oval window (OW). However, otosclerosis can have a sclerotic phase, approximating the density of normal bone making diagnosis challenging. This study assesses differences in OC contour and thickness anterolateral to the anterior margin of the oval window in otosclerosis compared to normal-hearing patients. Materials and Methods: Axial CT of 104 ears with clinically-diagnosed otosclerosis and 108 consecutive ears of audiometrically-normal individuals were retrospectively reviewed. Two radiologists independently evaluated the pattern of otosclerosis, OC contour and bone thickness on standardized axial images at the level of the OW and cochleariform process (CP). Measurements were made from the posterolateral margin of the cochlea to the apex of the OC convex contour just anterolateral to the anterior margin of the oval window. In the absence of a convex contour, the sulcus between the OW and CP was identified, and measurement to the depth of the sulcus was used. ROC analysis determined the best cutoff value of OC thickness. Results: Mean OC thickness (2 standard deviations) was 3.08 (0.93) mm and 1.82 (0.31) mm in otosclerosis and normal-hearing patients, respectively (p-value < 0.001) with excellent interobserver agreement. OC thickness > 2.3 mm had 96.2% sensitivity, 100% specificity, 100% PPV, and 96.4% NPV for otosclerosis. Bulging/convex contour of the OC had 68.3% sensitivity, 98.1% specificity, 97.3% PPV, and 76.3% NPV. Conclusion: Otosclerosis patients have significantly thicker bone abutting the OW than normal-hearing individuals.

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