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Otological problems in children with Turner's syndrome
A E Stenberg1, O Nylén, M Windh
1Department of Otorhinolaryngology, Karolinska Hospital, Stockholm, Sweden.
Hearing Research
|November 21, 1998
Summary
Girls with Turner syndrome frequently experience ear infections and hearing loss. Early audiological evaluations are crucial for identifying progressive hearing impairment in young girls with this condition.
Area of Science:
- Otolaryngology
- Genetics
- Audiology
Background:
- Turner syndrome is associated with a high incidence of ear and hearing disorders in affected individuals.
- Recurrent acute otitis media and sensorineural hearing impairment are common in girls and women with Turner syndrome.
- Early identification and management of these conditions are vital for preserving hearing function.
Purpose of the Study:
- To investigate the prevalence of eardrum pathology and hearing impairment in young girls with Turner syndrome.
- To explore the relationship between karyotype and otological findings.
- To assess the potential for early detection of progressive hearing loss.
Main Methods:
- A study involving 56 girls aged 4-15 years with Turner syndrome.
- Assessment of eardrum pathology through otoscopy.
- Audiometric evaluations, including pure-tone audiometry and tympanometry.
- Karyotype analysis to correlate with clinical findings.
Main Results:
- A high prevalence of recurrent acute otitis media (61%) and eardrum pathology (57%) was observed.
- Conductive hearing loss was present in 43% of the girls, and sensorineural hearing impairment with a characteristic frequency dip was found in 58%.
- Auricular anomalies were noted in 23%, particularly in the 45, X karyotype group.
Conclusions:
- The study confirms a high prevalence of ear pathologies and hearing loss in girls with Turner syndrome.
- Progressive hearing loss, detectable as early as age 6, underscores the need for early and regular audiological monitoring.
- Regular otological and audiological examinations are essential for all girls with Turner syndrome to manage and potentially predict hearing impairment.