High-frequency sensorineural hearing loss in children.
Determine the prevalence of high-frequency sensorineural hearing loss (HFSNHL) in our hearing loss population and a diagnostic algorithm for these patients.



Retrospective case series.



We identified patients diagnosed with sensorineural hearing loss (SNHL) at our pediatric tertiary care institution from 1981 to 2010. Based on audiometric profiles, these patients were subdivided into those with a flat SNHL configuration and those with HFSNHL. Imaging and genetic testing data and data regarding age at diagnosis, laterality, and risk factors were obtained for both groups. Comparisons were then made between the two groups.



Of 2,867 patients included in the study, 7.6% had HFSNHL. Age at diagnosis was significantly higher in HFSNHL patients (8.3 years vs. 6.1 years; P < .0001). These patients also had a significantly higher proportion of unilateral versus bilateral loss (49.1% vs. 26.1%; P < .0001); unilateral losses were also less severe. Genetic testing showed no significant difference between groups in the proportion of patients tested or in those who tested positive. Similarly, imaging data revealed no difference in the proportion of patients tested in the two groups; however, overall diagnostic yield was significantly higher in flat SNHL patients (29.5% vs.17.3; P = .02).



The positive predictive value of simple genetic testing is similar to that of imaging studies. However, given cost differences between genetic testing and imaging, it is prudent to perform genetic testing as the initial diagnostic test. Determination of whether high-throughput, multigene diagnostic platforms offer an added benefit in the evaluation of children requires further study.



4. Laryngoscope, 126:1236-1240, 2016.
Department of Otolaryngology-Head and Neck Surgery, University of Washington School of Medicine and Seattle Children's Hospital, Seattle, Washington, U.S.A.
Kaalan
K
Johnson
Division of Biostatistics and Epidemiology, Cincinnati Children's Hospital Medical Center, Cincinnati, Ohio, U.S.A.
Meredith
M
Tabangin
Division of Biostatistics and Epidemiology, Cincinnati Children's Hospital Medical Center, Cincinnati, Ohio, U.S.A.|Ear and Hearing Center, Division of Pediatric Otolaryngology-Head and Neck Surgery, Cincinnati Children's Hospital Medical Center, Cincinnati, Ohio, U.S.A.
Jareen
J
Meinzen-Derr
Ear and Hearing Center, Division of Pediatric Otolaryngology-Head and Neck Surgery, Cincinnati Children's Hospital Medical Center, Cincinnati, Ohio, U.S.A.
Aliza P
AP
Cohen
Ear and Hearing Center, Division of Pediatric Otolaryngology-Head and Neck Surgery, Cincinnati Children's Hospital Medical Center, Cincinnati, Ohio, U.S.A.|Department of Otolaryngology-Head and Neck Surgery, University of Cincinnati College of Medicine, Cincinnati, Ohio, U.S.A.
John H
JH
Greinwald
D017668:Age of Onset; D000465:Algorithms; D001299:Audiometry; D002648:Child; D002675:Child, Preschool; D005260:Female; D005820:Genetic Testing / Q000191:economics; D006319:Hearing Loss, Sensorineural / Q000175:diagnosis* / Q000000981:diagnostic imaging; D006801:Humans; D008297:Male; D011237:Predictive Value of Tests; D012189:Retrospective Studies; D012307:Risk Factors; D013701:Temporal Bone / Q000000981:diagnostic imaging; D014057:Tomography, X-Ray Computed / Q000191:economics
High frequency; sensorineural hearing loss