Common ear and hearing Issues in girls and women with Turner Syndrome

Hearing

Ear Formation and Hearing Issues

Common ear formation and hearing issues are found in girls and women with Turner Syndrome. Early screening and interventions will impact learning and social development. Access to secondary hearing aids can be a financial burden as replacement hearing aids are generally not covered by insurance.  Below you will find some resources that you can pursue for financial assistance.

Hearing
Conductive and sensorineural hearing loss are common in girls with TS. The outer, middle, and inner ear are all affected, and hearing problems and ear malformations correlate with the karyotype.

How We Hear 101
The ear is divided into 3 parts that lead up to the brain.
The External Ear: sound travels down the ear canal and strikes the ear drum causing it to vibrate.
The Middle Ear: Vibrations from the ear drum cause the ossicles to vibrate which then creates movement of the fluid in the inner ear.
The Inner Ear: the movement of the fluid in the inner ear cause changes in tiny hair cells. Movement of hair cells sends electrical signals from the inner ear to the auditory nerve which leads to the brain.The brain then interprets these electrical signals as sounds.

Degree and Types of Hearing Loss
Hearing loss is described in terms of degree of severity:

Normal:
0-15dB for children
0-25dB for adults

Conductive Hearing Loss:
Problems within the outer and/or middle ear
Bone conduction hearing is within the normal range
Air conduction hearing can range from mild to moderate-severe
Ear infections and otitis media (fluid in the middle ear) are common in childhood because of the position of the Eustachian Tube early in life.

Children with TS are at even greater risk for middle ear problems and are thought to be pre-disposed to middle ear problems due to ear malformations and abnormalities. Some persistent middle ear problems can lead to sequelae such as TM perforations, drainage and the development of cholesteatomas.

Sensorineural Hearing Loss:

There are problems within the inner ear. Bone conduction and air conduction are approximately equal and can range from mild to profound. Studies shows there can be either a progressive SNHL with a midrange bilateral symmetric dip in the 1.5-2.0kHz region or a high frequency downward sloping SNHL. Most common onset for a patient with TS is during the 2nd and 3rd decades of life but may be present as early as 6 years old. This hearing loss may begin in childhood and worsen over time, more than 90% of women with TS in their forties have some degree of HL and more than 25% of them need hearing aids.

Mixed Hearing Loss:
Problems within the outer and/or middle ear as well as inner ear.
Hearing loss by both bone conduction and air conduction.
Hearing loss via air conduction is greater than hearing loss via bone conduction.

Tympanometry
Assessment of the function of the ear drum (tympanic membrane)
Classification:
Type A – WNL
Type B – Flat (fluid, cerumen, TM perf)
Type C – Retracted/negative pressure
Type Ad – Hypermobile
Type As – Stiffness

Outer Ear
More than 80% of patients with TS have ear malformations, including low set ears, elongated ears, cup-shaped ears and thick ear lobes.

Middle Ear 
Otitis media is extremely common in girls with TS and may progress to mastoiditis and/or cholesteatoma formation. It occurs particularly between 1 and 6 yr of age, with a maximum incidence (>60%) at 3 yr of age. The cause is still unknown, but growth retardation of the temporal bone may be important. Aggressive treatment of otitis media is appropriate, and insertion of ventilation tubes should be considered. Careful follow-up is important. Patients with chronic middle ear problems should be operated on without delay to prevent sequelae. Short girls with extensive otitis media problems should be referred to an endocrinologist if TS has not previously been diagnosed.

Inner Ear
The majority (50–90%) of women with TS have sensorineural hearing loss, manifest by a sensorineural dip in the 1.5–2 kHz region, sensorineural high frequency loss, or all of these. The sensorineural dip can occur as early as 6 yr of age and occasionally leads to hearing impairment during childhood. The condition is progressive, however, and commonly leads to hearing problems in later life, which may have serious social consequences.

Management
Obtain a baseline audiologic evaluation at the time of diagnosis of Turner’s Syndrome and follow-up annually, preferably before heading back to school in the Fall. Heightened surveillance for middle ear problems, especially in childhood. Follow-up with ORL specialist for medical management and audiologist to monitor hearing. If a hearing loss is identified – hearing aids may be recommend dependent on the type and severity of the hearing loss. If patient is of school age, an FM system may also be appropriate in academic settings. Academic/Education Support for Speech and Language Intervention.

Tips for Healthy Ears and Hearing for All

  • Refraining from cotton swab use on yourself or child.
  • Hearing conservation to prevent noise induced hearing loss or additional hearing loss or additional hearing loss from noise exposure.
  • Ear plugs should be utilized in high noise environments.
  • Reducing the volume and limiting time listening to personal audio devices.

 

View the webinar recording- learn how to access support under the ADA, and more!

White Papers:

To read more about Otolaryngology and hearing, scroll to page(s) G34-G35 in the Clinical Guidelines found here: https://turnersyndromefoundation.org/wp-content/uploads/2017/07/Clinical-Practice-Guideslines-International-G1-2017.full_.pdf

The National Association of the Deaf, http://www.NAD.org,  is a national organization with resources you may find helpful. They provide Advocacy Letters that can help you advocate for your rights.

The Americans with Disabilities Act (ADA) became law in 1990. The ADA is a civil rights law that prohibits discrimination against individuals with disabilities in all areas of public life, including jobs, schools, transportation, and all public and private places that are open to the general public. The purpose of the law is to make sure that people with disabilities have the same rights and opportunities as everyone else. The ADA is divided into five titles (or sections) that relate to different areas of public life. ADA National Network www.adata.org

Relay Conference Captioning (RCC) is a free service for anyone who is deaf or hard of hearing to engage in meetings (in-person or remote), group conversations in a videoconference and multi-party conference call.

Computer-aided transcription service (CART) enables people who are deaf, hard of hearing, or who have auditory processing difficulties, to participate in meetings and training events by viewing realtime text of the lecture/training/meeting. However, sometimes it is not practical or possible to have a CART writer available on-site. It may be possible to access CART services remotely. – JAN

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References

  • American Speech-Language-Hearing Association | ASHA. (2011, October 10).
  • American Speech-Language-Hearing Association| ASHA.
  • Retrieved July 21, 2014, from Newborn Hearing Screening. (2013, March 29).  
  • Retrieved July 7, 2014, from EHDI Legistlation: Overview. (2014, January 10).
  • Retrieved July 2, 2014, from Anthony, T. (2011, February 2010). Spotlight on Turner Syndrome.
  • Retrieved July 1, 2014, from Serra, A , Cocuzza, S., Caruso, E., Mancuso, M., & Martia, I L, Audiological range in Turner Syndrome.
  • International Journal of Pediatric Otorhinolaryngology, 67, 841-845 Marimoto, et al.
  • Hearing Loss in Turner Syndrome. The Journal of Pediatrics. 691-701 Parkin, M., & Walker, P.
  • Hearing Loss in Turner Syndrome. International Journal of Pediatric Otorhinolaryngology, 73, 243-247.
  • Olveira, C.S., Ribeiro, F.M., lago, R., & Alves C. Audiological Abnormalities in Patients with Turner Syndrome. American Journal of Audiology, 22, 226-232
  • Incidence of Hearing Loss with Turners Syndrome. (2014, February 2). . Retrieved July 1, 2014, from Jung, J.H. (1989). Genetic syndromes in communication disorders. Boston: College-Hill Press.

 

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