Sensorineural hearing loss in early childhood after passing newborn hearing screening
摘要
The newborn hearing screening (NHS) program in the Netherlands consist of two rounds of otoacoustic emissions (OAE) and a third round of automated auditory brainstem responses (AABR). The program identifies about 200 newborns with sensorineural hearing loss (SNHL) annually, enabling early intervention. Nevertheless, a subgroup of patients passes the NHS but present with SNHL in early childhood.
PurposeTo determine the number of patients diagnosed with SNHL (< 5 years) who initially passed their NHS, and characterize this group in terms of patient demographics, NHS results, hearing loss (HL) characteristics and etiology of, to identify opportunities for earlier detection.
MethodsA retrospective descriptive study was conducted on patients (< 5 years) seen between 2014 and 2024, passed their NHS and had SNHL with an unknown cause, for which they underwent etiological diagnostics.
Results119 patients with 205 ears were included. Three-fourth of patients had an abnormal speech and language development (SLD). A relatively large number of ears passed in the third AABR round (36%). HL characteristics were highly variable. An etiological diagnosis was found in 74 patients (62%), most commonly a genetic cause (40%) or a cochleovestibular malformation (18%).
ConclusionsWe estimate that in the Netherlands approximately 50 patients per year present with SNHL (< 5 years) after passing their NHS, of which the majority manifest abnormal SLD. Highly variable HL characteristics and etiology make it difficult to identify them. Nevertheless, a possibility for earlier detection is a second screening for specific subgroups. No evidence of false negatives during NHS was found.