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Background and Aim: Turner syndrome is the most common sex chromosome abnormality in females, affecting an estimated 3% of all conceiving females. Otologic disease is a common problem in Turner syndrome patients that is due to a combination of small dysfunction Eustachian tube, palatal dysfunction and cochlear malformation.
Methods: This study assessed the otologic and audiologic characteristics of a group of Turner syndrome patients. We studied 40 Turner patients aged 10 to 20 years (mean age: 15.84 years, SD=2.67). Pure tone audiometry was carried out for all of them.
Results: Forty percent of the patients reported a history of middle ear disease. Analysis of audiometric data in 40 patients tested reveals normal hearing in 47.5%, pure sensorineural hearing loss in 32.5%, pure conductive hearing loss in 17.5% and mixed hearing loss in 2.5% of patients.
Conclusion: Careful follow up during early childhood of children with Turner syndrome is necessary to detect middle ear disease and prevent the probable sequel. However, long term periodic follow up is mandatory even after resolution of middle ear disease to detect sensorineural hearing loss.

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Background and Aim: Behavioral audiometry is the best method of hearing evaluation. Hearing thresholds are estimated with auditory evoked responses in patients who are not able to cooperate in behavioral audiometry. Because rehabilitative decisions are based on behavioral thresholds, it is essential to determine the amount of difference between behavioral and evoked response thresholds. The purpose of this study was to evaluate the difference between functional and auditory steady-state response (ASSR) gains.

Methods: Twenty six moderate to severe hearing impaired adolescents and adults (age range: 14 to 55 years) participated in this interventional-comparative study. Audiometery and ASSR testing was performed (without hearing aid and then with hearing aid). Finally, functional gain with ASSR gain, and behavioral thresholds with ASSR thresholds were compared using paired t-test and correlation statistical methods. All tests were performed in the sound field.

Results: The mean difference was 6.19 dB between unaided behavioral and ASSR thresholds, 4.33 dB between aided thresholds, and 2.43 dB between functional and ASSR gains. There was no significant difference between functional and ASSR gains (p>0.05).

Conclusion: The difference between functional and ASSR gains is less than the difference between behavioral and ASSR thresholds and it has less variability between different frequencies. So, it may be better to use ASSR gain instead of ASSR thresholds in hearing aid fitting in difficult-to-test population.