Sensorineural hearing loss in early childhood after passing newborn hearing screening.

INTRODUCTION

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.

PURPOSE: To 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.

METHODS

A 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.

RESULTS

119 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%).

CONCLUSIONS

We 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.