Ears

 

Infants: Sometimes girls with TS are born with prominent or mis-shapen ears.  Depending on the severity of the malformation of the ears, a parent may elect to use a device (not researched specific to TS) or surgery to correct for function or cosmetic reasons.

 

Hearing  

 

Because of the high rate of ear and hearing problems and the effect they can have on a person’s quality of life (including their speech), everyone with TS should have their hearing and middle ear function checked regularly throughout life. It also is important that girls and women with TS protect their hearing by avoiding loud noises and using ear protection in loud environments. Sensorineural hearing loss is common in TS. This type of hearing loss is related to the function of the inner ear, where the vibrations caused by sound waves are converted to nerve impulses that travel to the brain. In the study mentioned below, 58% of the girls also had some sensorineural loss. This hearing loss may begin in childhood and appears to worsen over time, so that more than 90% of women with TS in their forties have some degree of hearing loss, with more than 25% of them needing hearing aids.

 

• Otitis media (ear infection) is extremely common in girls with TS particularly in infancy and early childhood. Aggressive treatment of infections is appropriate.

• Ear infections and hearing loss are common. 

• In one study of girls with TS aged 4 to 15 years, 57% had some eardrum problem and 43% had hearing loss.Changes in the structure of the middle ear and eustachian tube (tube connecting the middle ear with the back of the throat) probably account for poor drainage and ventilation in the middle ear and easier movement of germs from the throat into the ear - all of which play a role in causing frequent middle ear infections (otitis media) and effusions (fluid in the middle ear), especially in early childhood.

• Middle ear effusions cause conductive hearing loss because sound does not move normally through an ear filled with fluid. Middle ear infections should be treated promptly with antibiotics.