Cochlear Implant Outcomes in Cochlea Nerve Aplasia and Hypoplasia

Otology & Neurotology : Official Publication of the American Otological Society, American Neurotology Society [and] European Academy of Otology and Neurotology
Catherine S BirmanElizabeth J Elliott

Abstract

To assess cochlear implant (CI) outcomes, and factors affecting outcomes, for children with aplasia/ hypoplasia of the cochlea nerve. We also developed a new grading system for the nerves of the internal auditory meatus (IAM) and cochlea nerve classification. Retrospective patient review. Tertiary referral hospital and cochlear implant program. Children 0 to 16 years inclusive with a CI who had absent/hypoplastic cochlea nerve on magnetic resonance imaging (MRI). Cochlear implant. MRI, trans-tympanic electrical auditory brainstem response, intraoperative electrical auditory brainstem response, Neural Response Telemetry, Categories of Auditory Perception score, Main mode of communication. Fifty CI recipients (26 males and 24 females) were identified, 21 had bilateral CIs, 27 had developmental delay. MRI showed cochlea nerve aplasia in 64 ears, hypoplasia in 25 ears, and a normal nerve in 11 ears. Main mode of communication was analyzed for 41 children: 21 (51%) used verbal language (15 speech alone, 5 speech plus some sign, 1 bilingual in speech and sign), and 20 (49%) used sign language (10 sign alone, 9 sign plus some speech, 1 tactile sign). Seventy-three percent of children used some verbal language. Cochlea nerve aplasia/ h...Continue Reading

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