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.
Parkin
Monique
M
John Hunter Children's Hospital, Newcastle, Australia.
Walker
Paul
P
D000293:Adolescent; D017668:Age of Onset; D001301:Audiometry, Pure-Tone; D001315:Australia; D002648:Child; D002675:Child, Preschool; D015331:Cohort Studies; D015897:Comorbidity; D005260:Female; D006319:Hearing Loss, Sensorineural / Q000175:diagnosis / Q000453:epidemiology*; D006801:Humans; D015994:Incidence; D007223:Infant; D010033:Otitis Media / Q000453:epidemiology / Q000473:pathology; D012008:Recurrence; D012189:Retrospective Studies; D014424:Turner Syndrome / Q000453:epidemiology*