Hearing loss in Turner syndrome.

OBJECTIVE: Recent reports have suggested a significant incidence of sensorineural hearing loss (SNHL) in children with Turner syndrome. This study reviewed a cohort of Australian children to clarify the incidence and pattern of conductive and sensorineural hearing loss.

METHODS: Retrospective cohort study of 23 individuals with Turner syndrome was identified - mean age 10 years 4 months. A retrospective review was undertaken and otologic status assigned.

RESULTS: Middle ear disease affected 91% of patients. Two children (9%) demonstrated sensorineural hearing loss attributable to Turner syndrome. Average age of onset was 15 years - one demonstrated a classic U-shaped mid-frequency loss, the other a bilateral down-sloping sensorineural loss.

CONCLUSIONS: In this Turner syndrome cohort, 91% of subjects suffered middle ear disease, whilst the incidence of SNHL was 9%. It is recommended that individuals with Turner syndrome should be screened for onset and progression of hearing loss.