The Epidemiology of Otosclerosis in a British Cohort.

ABSTRACT: OBJECTIVE:To analyse the epidemiology of otosclerosis in a British cohort collected between 2011 and 2017. DESIGN:Retrospective cohort study. SETTING:Five UK ENT Departments. PATIENTS:Patients with surgically confirmed otosclerosis. MAIN OUTCOME MEASURES:Questionnaire data documented family history of otosclerosis, age of onset, medical history, and information on associated risk factors for 657 patients. Pre and post-surgical pure-tone audiometry was collected for 154 of these patients. RESULTS:The age of onset, incidence of bilateral disease, tinnitus and vertigo, a higher prevalence of women (65%) than men (35%) are similar to those reported previously for otosclerosis cohorts. No association with measles infection was detected. Patients with a family history (40%) have an earlier age of onset and a higher incidence of bilateral disease and vertigo than non-familial subjects. Pedigree analysis is consistent with an autosomal dominant inheritance with reduced penetrance being apparent in 44/91 pedigrees studied. Women who associate their hearing loss with pregnancy have an earlier age of onset than those that do not (p?=?6?×?10). CONCLUSIONS:This study confirms that otosclerosis is an early adult onset disease that is more prevalent in women than men with a large minority of patients having a family history of otosclerosis. We report new evidence to support a relationship between pregnancy and otosclerosis progression in a proportion of women. In addition, this is the first study to identify differences in severity between familial and non-familial cases of otosclerosis, highlighting the possibility that more than one etiology may be involved.

SUBMITTER: Crompton M 

PROVIDER: S-EPMC6314447 | biostudies-literature | 2019 Jan

REPOSITORIES: biostudies-literature