Profound childhood hearing loss in a South Africa cohort: risk profile, diagnosis and age of intervention.

OBJECTIVE To describe profound childhood hearing loss in a South African population of pediatric cochlear implant recipients in terms of risk profile, and age of diagnosis and intervention. METHODS A retrospective review of patient files for 264 pediatric cochlear implant recipients from five cochlear implant programs was conducted. Data was captured from 264 eligible subjects, of which all were implanted between 1996 and 2013 and PCEHL was confirmed under the age of 5 years old. Data collected included demographical information, risk factors from case histories, diagnostic test procedures conducted, diagnosis (type, onset and degree of hearing loss) and documented ages of caregiver suspicion, initial diagnosis and intervention. RESULTS Risk factors for permanent childhood hearing loss were present in 51.1% of cases, with the most prevalent risks being NICU admittance (28.1%), family history of childhood hearing loss (19.6%) and prematurity (15.1%). An associated syndrome was diagnosed in 10% of children and 23.5% presented with at least one additional developmental condition. Hearing loss for most (77.6%) children was confirmed as congenital/early onset, while 20.3% presented with postnatal onset of hearing loss. ANSD was diagnosed in 5% of children, with admittance to NICU (80%) and hyperbilirubinemia (50%) being the most prevalent risk factors for these cases. Hearing loss was typically diagnosed late (15.3 months), resulting in delayed initial hearing aid fitting (18.8 months), enrollment in early intervention services (19.5 months) and eventual cochlear implantation (43.6 months). CONCLUSION Most prevalent risk factors in profound childhood hearing loss were admittance to NICU, family history and prematurity. Diagnosis and intervention was typically delayed predisposing this population to poorer outcomes.