Peer-reviewed article
Bilateral sensorineural hearing loss in children: causes and long-term progression
Andra Lazar, Luca Verrecchia, Inger Uhlén, Anna Persson, Josephine Wincent et al. ·
Summary
Written by AI, not yet AICS's view · claude-sonnet-5-5This retrospective study looked at 194 children in Stockholm County, born 2010 to 2015, who had moderate to profound hearing loss in both ears caused by inner ear or nerve damage. It examined how often doctors looked for the cause of the hearing loss and how often they found one. It also looked at which causes were linked to hearing getting worse over time. Three quarters of the children had a cause investigated, and a cause was found in about half of those. Hearing got worse in 16% of the children, most often because of enlarged vestibular aqueduct syndrome or congenital cytomegalovirus infection.
Key points
- 146 of 194 children (75%) had their cause investigated, and a cause was found in 75 of them (51.4%).
- Of the causes found, 52 (69%) were genetic, for example GJB2, and 10 (13%) were congenital cytomegalovirus infection (cCMV). Inner ear malformations were also reported.
- Hearing loss progressed in 16% of the children.
- The most common identified causes of progression were enlarged vestibular aqueduct syndrome (EVA), followed by cCMV.
- Genetic testing gave the highest diagnostic yield, either as a first test or after imaging.
Limitations
This is a descriptive retrospective study from one referral centre in one Swedish county, so it cannot show how common these causes are elsewhere or in lower-resource settings. It does not test any intervention. The abstract also gives an inner ear malformation figure (33, 33%) that does not clearly match the other numbers.
Abstract
PURPOSE: Childhood hearing loss (CHL) in need of assisting listening devices require a constant surveillance for hearing loss (HL) progression and adjustment of interventions. Despite advances in aetiological investigations, there are still important gaps in understanding causal determinants and progression patterns of CHL. The aim of this study was to analyze the prevalence and yield of aetiological investigations, and to identify causes associated with HL progression in older children. METHODS: This descriptive retrospective study included data from 194 children with moderate-profound senorineural HL born between January 1st 2010 and December 31st 2015, retrieved from the regional registry at the tertiary referral audiological centre in Stockholm County. Medical information and pure tone averages were gathered through the regional public health record system. RESULTS: 146 children (75%) underwent causal investigation, with a positive finding in 75 (51,4%) of them. The discovered aetiologies were genetic in 52 (69%) such as GJB2, congenital cytomegalovirus infection (cCMV) in 10 (13%) and inner ear malformations in 33 (33%). HL progression was observed in 16% of the children and the most common identified cause was enlarged vestibular aqueduct syndrome (EVA), followed by cCMV. CONCLUSION: The highest diagnostic yield was observed when genetic testing was performed, either as a first-line investigation or following imaging. Few children reported a progression of HL in this study group in which EVA and cCMV were the most prevalent causes. Comprehensive or targeted genetics remains as an effective diagnostic method, in isolation when clinically strengthened or in combination with imaging and infectious investigations.