Enlarged vestibular aqueduct syndrome (EVAS) is the most common radiographically identified cause of paediatric hearing loss (HL). For counselled families, avoiding contact sports or other potentially traumatic activities is not supported by meta-analysis of existing data. EVAS can present with 3 types of HL: conductive, sensorineural and mixed, with sensorineural HL being the most common. It is important to perform a CT-scan in the case of conductive or mixed types of HL found on the pure-tone audiogram, intact stapedial reflexes and an A-type tympanogram, in order to exclude EVAS and/or other third window pathologies of the inner ear. Since HL commonly progresses with time, it is important to carry out audiometric follow-up of patients with EVAS. Hearing rehabilitation can be provided by using hearing aids, bone conduction hearing devices and cochlear implants (CI). In the case of surgical rehabilitation (bone conduction hearing devices or CI), it is essential to consider possible anomalies of the inner ear such as Gusher syndrome.