Introduction <p>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.</p> Purpose <p>To determine the number of patients diagnosed with SNHL (&lt; 5&#xa0;years) who initially passed their NHS<b>,</b> and characterize this group in terms of patient demographics, NHS results, hearing loss (HL) characteristics and etiology of, to identify opportunities for earlier detection.</p> Methods <p>A retrospective descriptive study was conducted on patients (&lt; 5&#xa0;years) seen between 2014 and 2024, passed their NHS and had SNHL with an unknown cause, for which they underwent etiological diagnostics.</p> Results <p>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%).</p> Conclusions <p>We estimate that in the Netherlands approximately 50 patients per year present with SNHL (&lt; 5&#xa0;years) after&#xa0;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.</p>

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Sensorineural hearing loss in early childhood after passing newborn hearing screening

  • I. E. C. Smetsers,
  • N. Uilenburg,
  • R. J. E. Pennings,
  • C. P. Lanting,
  • F. L. J. Cals

摘要

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.