Hearing loss in adult women with Turner’s syndrome and other congenital hypogonadisms

Abstract

Objectives: To define the patterns and causes of hearing decline associated to Turner’s syndrome (TS).

Methods: An observational study with three cohorts was designed: 31 TS patients, 15 women with other congenital hypogonadims (OCH) and 41 healthy age-matched women taking contraception. Microotoscopy, standard pure-tone audiometry brain auditory evoked potentials (BAEP) were performed to study hearing function.

Results: Up to 87% of TS subjects suffered from some degree of hearing loss (HL) in the audiograms, compared with 20% OCH and 27% controls. Sensorineural hearing loss (SNHL) was the most frequent type of hypoacusia found in TS group. BAEP study demonstrated that 61% of TS women showed HL compared to 20% in OCH patients. No significant differences in latencies, amplitudes, and interpeaks of waves I, III and V were found between TS and OCH, nor when compared to reference population. Worse results were observed among the oldest TS patients, those with pure monosomy or isochromosome, and those with a history of recurrent otitis.

Conclusions: More than a half of TS females presented HL. SNHL is the most frequent pattern among middle-aged women with TS. Old age, karyotype and recurrent otitis are predisposition factors to produce HL, while oestrogens play a minor role.

• Brain auditory evoked potentials
• congenital hypogonadisms
• hearing loss
• pure-tone audiometry
• Turner’s syndrome

Acknowledgements

The authors would like to thank Dr. Irene Halperin, Dr. Margarita Montaner and Ester Crespo for their help in the recruitment of patients, and Dr. Lluís Aragonès for his support in the statistical analysis.