Bilateral sensorineural hearing loss in children: causes and long-term progression

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.

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.

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.

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.