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Correlation of otosclerotic foci and degenerative changes in the organ of Corti and spiral ganglion.

Statistical analyses of histopathologic findings in the cochlea and spiral ganglion of 37 temporal bones with otosclerosis, 12 controls of similar age, and seven controls with normal hearing were performed. In temporal bones with otosclerosis there was significant atrophy of the spiral ligament and stria vascularis in regions with endosteal involvement by otosclerosis, compared with regions without endosteal involvement (P less than .0001). There was more generalized atrophy of the stria vascularis in cochleae with two or more sites of endosteal involvement by otosclerosis than in cochleae with only one site of endosteal involvement (P less than .02), cochleae in temporal bones with otosclerosis but without endosteal involvement (P less than .05), or cochleae of controls of similar age (P less than .007). In addition, there was more atrophy of the spiral ligament in cochleae with two or more sites of endosteal involvement than in cochleae of similar age from the control group (P less than .03). In temporal bones with otosclerosis, there was no significant difference in counts of outer hair cells and density of spiral ganglion cells between regions demonstrating endosteal involvement by otosclerosis and regions without such involvement. However, total outer hair cell counts were lower in cochleae with two or more sites of endosteal involvement of otosclerosis than in cochleae with one site of endosteal involvement (P less than .04), cochleae in temporal bones with otosclerosis but without endosteal involvement (P less than .02), or cochleae from individuals of similar age but without otosclerosis (P = .05). Comparison of the mean bone conduction threshold, as measured in life, in temporal bones with otosclerosis compared with the air conduction threshold in aged-matched controls, demonstrated that only cochleae with two or more sites of endosteal involvement had a mild but statistically significant (P = .05) decrease in hearing. There was no evidence to support the concept that otosclerotic foci without stapedial fixation frequently cause significant degeneration of the cochlea or elevation of bone conduction thresholds.

Adolescent↗
PubMed2001

Correlations between computed tomography findings and family history in otosclerotic patients.

BACKGROUND: Otosclerosis is a heritable disease affecting the otic capsule. Its genetics have been studied since the 19th century, but several issues remain controversial. OBJECTIVE: The goals of this study were to assess the prevalence of sporadic and familial forms of otosclerosis in a population of otosclerotic patients and to compare the radiologic findings between both groups. STUDY DESIGN AND SETTING: This retrospective study was conducted in a single institution. PATIENTS: This study included 211 patients operated on for otosclerosis. MAIN OUTCOME MEASURES: Clinical data, including pure tone audiograms, were available from patients' charts. A questionnaire assessing family history of otosclerosis and deafness was mailed to the otosclerotic patients. A relative was considered otosclerotic if surgery confirmed the disease. The family history was correlated with the computed tomography results. This examination was performed before surgery in all patients. RESULTS: A family history of otosclerosis was found in 24.2% of the patients. The radiologic findings differed between patients with a sporadic form of otosclerosis and those with a familial form. The lesions were more often detectable, bilateral, and severe in the familial forms (p < 0.05). CONCLUSION: These findings lead to the assumption that fenestral radiologic otosclerosis occurs more in sporadic forms, whereas more extensive lesions on computed tomography seem to indicate the familial forms. Hereditary forms demonstrated to be familial seem to lead to more severe disease. The search for a genome locus of otosclerosis may be enlightened by these findings.

Adult↗