Characteristics and progression of hearing loss in children with turner's syndrome

Objectives/hypothesis: Evaluate patterns of hearing impairment in children with Turner's syndrome (TS) and determine factors influencing severity and progression.

Study design: Retrospective database review.

Methods: Demographic, audiological, and medical data for children with TS were extracted from the Audiological and Genetic Database to analyze patterns of hearing loss with comorbidities, demographics, and interventions.

Results: Two hundred seventy-two children with TS were identified; 213 had audiological data. Of these children, 72.3% (N = 154) had hearing loss in which 84.4% (N = 130) was bilateral. In individual ears, conductive hearing loss was most common (28.7%, n = 73), followed by mixed (22.0%, n = 93) and sensorineural (7.9%, n = 20). Otitis media (odds ratio [OR] = 2.7, 95% confidence interval [CI]: 1.2-6.5), eustachian tube dysfunction (OR = 9.5, 95% CI: 3.2-35.2), and aortic valve anomalies were also associated with higher rates of hearing loss (OR = 3.6, 95% CI: 1.3-11.5). Of ears with quantifiable severity, 16.3% (n = 40) had moderate or worse hearing loss. Aortic coarctation (36.3 vs. 21.3 dB, P < .001), seizures (40.6 vs. 21.3 dB, P = .006), facial anomalies (32.5 vs. 21.3 dB, P = .029), and hypertension (36.3 vs. 21.3 dB, P = .015) portended more severe loss.

Conclusions: Children with TS have high rates of hearing loss. High rates of sensorineural loss were unexpected. Nonotologic comorbidities, including seizures, hypertension, and anomalies of the kidney and aorta were associated with greater prevalence, severity, and progression of loss. Children with TS, particularly with the above comorbidities, are at risk for poor hearing outcomes, and should be offered audiological intervention to efficiently direct patient care.

Level of evidence: NA Laryngoscope, 130:1540-1546, 2020.