Cochlear Implant in a Patient with Arnold-Chiari Syndrome

Abstract

Introduction: Chiari malformation is a congenital disease characterized by herniation of posterior fossa contents below the foramen magnum level. Three types exist. Chiari type II malformation (Arnold-Chiari) consists of herniation of the tonsils, cerebellar vermis, IV ventricle and a lower portion of the bulb through the occipital foramen. It usually manifests as myelomeningocele in the thoracolumbar spine, hypoplasia of the posterior fossa, encephalic herniation into the cervical spinal canal and compression of the cranial nerves. Objectives: To verify the efficiency of Cochlear Implant (CI) Surgery in a patient with Arnold-Chiari Syndrome. Resumed report: A 6-year-old female, with meningomyelocele and congenital hydrocephalus with deafness at 3 years of age after a meningitis episode. She was evaluated as hearing category-0 and Language-2. Tone Pure Audiometry revealed bilateral profound sensorineural hearing loss. The Nuclear Magnetic Resonance showed a diffuse reduction of the white matter, sharp tapering and arching of the corpus callosum with marked dilation of the supratentorial ventricles. (Chiari type II). Ear-CT (Computed Tomography) scan confirms bilateral hypoplasia for lateral semicircular canal and right cochlea. Cochlear Implant Surgery was performed on the left ear with the Medel Cochlear Implant-SONATAti100. Currently the child is 12 years old, 6 years after surgery, performs speech therapy 3 times per week and improve his skills to hearing category-6 and Language-5. Conclusion: Despite the presence of Arnold-Chiari syndrome and even with neurological alterations presented with DVP (Peritoneal ventricular valve), CI surgery has good hearing results.