Incidence of hearing loss with Turner's syndrome

Hearing problems and ear malformations are common in children with Turner's syndrome. An estimated 90% have some type of middle ear disorder or conductive hearing loss. There's a 9% incidence for sensorineural hearing loss at a young age, increasing to 25% by age 45.

Middle ear infections and conductive hearing loss

Children with Turner syndrome are at higher risk of middle ear effusion and/or otitis media (ear infections) that may result from an abnormal relationship between the eustachian tube and middle ear - a consequence of abnormal cranial base anatomy.

Heightened surveillance for middle ear effusion should occur until at least 7 to 8 years of age and longer for those with a history of ear infections.

As a result of frequent ear infections, conductive hearing loss is common.

Conductive hearing loss and otitis media should be managed aggressively because of the significant impact hearing loss can have on speech and language development.

There's a risk for the formation of cholesteatoma (type of skin cyst in the middle ear) when there's persistent otitis media with inflammation or infection.

Progessive sensorineural hearing loss  (SNHL) with a unique dip in the 1.5 to 2 KHz region and/or a high frequency hearing loss above 8 KHz may present as early as age 6.

If the hearing loss is severe enough to cause speech and language delay, delayed academic progress or social issues, amplification may be recommended.

If hearing loss is identified, we recommend annual audiological evaluations and follow-up.

We also recommend you take ear protection measures (use of earplugs and limiting iPod use time and volume settings) to limit additional hearing loss from noise exposure.

Definitions

Cholesteatoma - a type of skin cyst located in the middle ear. Symptoms include dizziness, drainage from the ear and hearing loss in one ear.

Conductive hearing loss - occurs when there's a problem conducting sound waves anywhere along the route through the outer ear, eardrum or middle ear.

Eustachian tube - runs between the inside of the ear and the throat. Its job is to make sure the pressure is the same on either side of the eardrum.

Otitis media - inflammation of the middle ear characterized by the accumulation of fluid in the middle ear; ear infection.

Sensorineural hearing loss - occurs when there's damage to the inner ear (cochlea), or to the nerve pathways from the inner ear to the brain.

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