Hearing loss in Turner syndrome

Area of Science:

• Genetics
• Otolaryngology
• Pediatrics

Background:

• Turner syndrome is a genetic condition affecting females.
• Hearing loss and otitis media are common in Turner syndrome.
• The cause of hearing issues in Turner syndrome requires further investigation.

Purpose of the Study:

• To determine if sensorineural hearing loss (SNHL) in Turner syndrome is progressive.
• To investigate the correlation between the parental origin of the X chromosome and SNHL.
• To assess the relationship between SNHL and otitis media (OM) in Turner syndrome.

Main Methods:

• Retrospective study analyzing medical records and questionnaires from 24 individuals with Turner syndrome.
• Assessment of otologic status, including SNHL and history of OM.
• Molecular techniques to determine the parental origin of the X chromosome in 17 subjects.

Main Results:

• Two-thirds (16/24) of subjects had SNHL, with 7 experiencing progressive loss starting in late childhood or early adulthood.
• A majority (21/24) reported problematic OM.
• No correlation was found between the parental origin of the X chromosome and hearing status.

Conclusions:

• Sensorineural hearing loss in Turner syndrome can be progressive.
• SNHL and frequent OM are independent variables in Turner syndrome.
• Unpaired genes on the X chromosome may contribute to hearing loss and other phenotypic abnormalities.