Otologic disease in turner syndrome.
OBJECTIVE
This study involved the assessment of the otologic and audiologic characteristics of a group of patients with Turner syndrome.


METHODS
Prospective study consisting of a questionnaire (77 of 123 responders) and an otologic and audiologic evaluation in patients with Turner syndrome (41 participants).


METHODS
Tertiary academic medical setting.


METHODS
Children, adolescents, and adults with Turner syndrome (median age, 24 yr).


METHODS
Otomicroscopy, audiometry, immittance measurements, and diagnostic imaging.


RESULTS
Otologic disease is an important characteristic in Turner syndrome. Sixty-six percent of the patients studied via the questionnaire reported a history of chronic or recurrent middle ear disease. Analysis of audiometric data in 40 patients tested reveals an equal amount of normal ears (38.8%) and pure sensorineural ears (38.8%), each constituting approximately one-third of the patient population. Pure conductive losses represent only one-fifth (21.3%) of auditory abnormality encountered.


CONCLUSIONS
Careful follow-up during early childhood of children with Turner syndrome is necessary to detect middle ear disease and prevent sequelae. However, long-term periodic review is mandatory even after resolution of middle ear disease to detect sensorineural hearing loss.
Dhooge
Ingeborg J M
IJ
Department of Otorhinolaryngology, Ghent University Hospital, Ghent, Belgium. ingeborg.dhooge@ugent.be
De Vel
E
E
Verhoye
C
C
Lemmerling
M
M
Vinck
B
B
D000293:Adolescent; D000328:Adult; D001301:Audiometry, Pure-Tone; D001309:Auditory Threshold; D001844:Bone Conduction / Q000235:genetics; D002648:Child; D002675:Child, Preschool; D004427:Ear Diseases / Q000175:diagnosis* / Q000235:genetics; D004431:Ear, External / Q000002:abnormalities; D004432:Ear, Middle; D005260:Female; D005500:Follow-Up Studies; D006314:Hearing Loss, Conductive / Q000175:diagnosis* / Q000235:genetics; D006319:Hearing Loss, Sensorineural / Q000175:diagnosis* / Q000235:genetics; D006801:Humans; D007621:Karyotyping; D008297:Male; D008875:Middle Aged; D009030:Mosaicism; D010033:Otitis Media / Q000175:diagnosis / Q000235:genetics; D017084:Otoacoustic Emissions, Spontaneous / Q000235:genetics; D011446:Prospective Studies; D013063:Speech Discrimination Tests; D014424:Turner Syndrome / Q000175:diagnosis* / Q000235:genetics