Cochlear Implantation for Rudimentary Otocysts: Two Case Reports

OTOLOGY & NEUROTOLOGY(2023)

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Abstract
ObjectiveRudimentary otocyst (RO) is characterized by an otic capsule without an internal auditory canal, which is considered a contraindication to cochlear implantation (CI). In this study, we were the first to report two patients with ROs who underwent CI.PatientTwo patients (18 months old and 2 years old) presenting with bilateral congenital hearing loss were diagnosed with ROs.InterventionCI was performed. The transmastoid slotted labyrinthotomy approach was used with customized MED-EL electrode arrays.Main Outcome MeasuresCategorical auditory performance, infant-toddler meaningful auditory integration of sound, the speech intelligibility rating, and meaningful use of speech scale.ResultsBoth children could understand common phrases and had intelligible, connected speech 2 years after CI.ConclusionWith proper indication, surgical approach and postoperative training, a child with an RO may benefit from CI.
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Key words
Cochlear implants,Inner ear malformation,Language development,Rudimentary otocyst,Sensorineural hearing loss
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