Therapeutic challenges and clinical characteristics of single-sided deafness in children

Introduction: The clinical implications of single-sided deafness (SSD) in children has historically been underappreciated by patients and providers alike, despite a large body of literature on the wide-ranging neurocognitive, language, scholastic, and functional impairments that occur. Conventional amplification options are marked by variable results and frequent loss of follow-up. Methods: Retrospective case series for pediatric SSD from 2008 to 2018. Results: 88 children with congenital SSD were identified. Seventeen (N = 17/88, 23.9%) passed their newborn hearing screen. Median age at first otolaryngology evaluation was 0.65 years (range 0.1–16.9 years). Most common etiologies included cochlear nerve deficiency (N = 39, CND, 44.3%), unknown (N = 30, 35.2%), inner ear malformation (N = 7, 8.0%), and congenital cytomegalovirus (N = 6, 6.8%). 32.5% of patients elected for continued observation only, followed by bone conduction hearing aid (27.7%), contralateral routing of sound aid (20.5%), conventional hearing aid (13.3%), or cochlear implant (6%). Lack of follow-up at ≥1 year was common (39.8%). Of those with device use data (N = 39), 84.7% reported either discontinued or <6 h of daily use. Conclusions: Despite early diagnosis and evaluation, the pediatric SSD cohort is characterized by high rates of loss of follow-up and amplification discontinuation. Cochlear nerve deficiency is commonly seen in congenital SSD. Early specialist referral is critical for habilitation evaluation. Patients and caregivers should be educated on the significant implications of unilateral hearing loss.