Cochlear Implantation in a Child With Arnold-Chiari Syndrome, Severe Inner Ear Malformations, and Cochlear Nerve Hypoplasia: A Case Report.
Piotr Henryk Skarżyński, Aleksandra Chodkiewicz, Natalia Czajka et al.
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Case in brief
A 1.5-year-old girl with Arnold-Chiari malformation, hydrocephalus, spina bifida, and profound bilateral sensorineural hearing loss had limited auditory development despite early hearing-aid use. Imaging showed severe bilateral inner-ear malformations, including cochlear hypoplasia, and multiple intracranial abnormalities. She underwent left cochlear implantation; a cerebrospinal fluid gusher during cochleostomy was controlled with autologous tissue and tissue adhesive. At 12 months, behavioral auditory responses had improved, and her parents reported greater responsiveness to environmental sounds.
What made this case unusual
The combination of severe bilateral inner-ear malformations and cochlear nerve hypoplasia in a child with Arnold-Chiari syndrome complicated implantation; a significant intraoperative CSF gusher was controlled successfully.
Diagnostic / clinical pearl
In complex congenital malformations, preoperative imaging and multidisciplinary assessment are important for cochlear implant planning and prognosis; intraoperative CSF leakage may require control with autologous tissue and tissue adhesive.
Why it matters
The reported auditory improvement shows that benefit may be possible in a selected child despite severe inner-ear and central nervous system abnormalities, although outcomes and prognostic factors remain uncertain.