Premium
Characteristics and progression of hearing loss in children with turner's syndrome
Author(s) -
Hamberis Alexandra O.,
Mehta Charmee H.,
Dornhoffer James R.,
Meyer Ted Albert
Publication year - 2020
Publication title -
the laryngoscope
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.181
H-Index - 148
eISSN - 1531-4995
pISSN - 0023-852X
DOI - 10.1002/lary.28264
Subject(s) - medicine , hearing loss , sensorineural hearing loss , confidence interval , turner syndrome , odds ratio , conductive hearing loss , audiology , otitis , pediatrics , cardiology , surgery
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