We report a case of pediatric bilateral enlarged vestibular aqueduct (EVA) involving profound hearing loss and language delay. A boy born uneventfully had impaired reaction to sound, noted at 20 months. Auditory steady-state response and conditioned orientation response audiometry confirmed profound bilateral deafness. The child underwent speech therapy with hearing aids in both ears and was treated for secretory otitis media. Computed tomography (CT) showed bilateral vestibular aqueduct enlargement and incomplete cochlear partition. Magnetic resonance imaging (MRI) showed bilateral dilated endolymphatic sac. At 36 months, he underwent right-ear cochlear implant (Cochlear, Nucleus® freedomTM CI24RE). Mild perilymph and cerebrospinal fluid leakage occurred pulsatively following cochleostomy at the scala tympani. This was controlled by soft tissue packing. Intra-operative neural response telemetry (NRT) showed no evoked action potential at apical channels, but we successfully mapped cochlear implant for all channels and infant-toddler meaningful auditory integration scale (IT-MAIS) and meaningful use of speech scale (MUSS) improved.
