Background: Hearing problems and ear malfunctions are frequent in Turner syndrome (TS) and correlate with the karyotype. As a result of the frequent otitis media, conductive hearing loss is common in girls with TS. Sensorineural hearing loss is also common and may occur as early as 6 years of age.
Objective and hypotheses: This study reviewed a cohort of children to clarify the incidence and pattern of conductive and sensorineural hearing loss in girls with TS.
Method: Retrospective cohort study of 27 girls with TS was identified by karyotype 14 monosomy, four mosaisms and nine structure anomalies mean age 11 years 4 months. A retrospective review was undertaken and otologic status assigned.
Results: Middle ear disease affected 30% of patients. 34% of this children demonstrated hearing loss attributable to TS. Sensorineural hearing loss was found in 31% of them and conductive hearing loss in 44% of cases. Cholesteatoma was found in one girl aged 12 years and was cure by surgery at 7 years.
Conclusion: The only possible intervention to reduce hearing loss in girls and women with TS remains the assiduous treatment of ear problems in childhood. All Turner individuals should be screened second yearly for onset and progression of hearing loss,whether conductive or sensorineural. Once hearing loss is recognized, patients should be fully investigated to exclude other causes, especially in cases of unilateral SNHL.
20 - 22 Sep 2014
European Society for Paediatric Endocrinology