Cochlear implantation in patients with inner ear bone malformations with posterior labyrinth involvement: an exploratory study

• Published in: European archives of oto-rhino-laryngology 2016-04, Vol.273 (4), p.893-898
• Main Authors: Palomeque Vera, Juan Miguel, Platero Sánchez-Escribano, María, Gómez Hervás, Javier, Fernández Prada, María, González Ramírez, Amanda Rocío, Sainz Quevedo, Manuel
• Format: Article
• Language: English
• Subjects: Adolescent; Case-Control Studies; Child; Child, Preschool; Cochlear Implantation - methods; Cochlear Implantation - rehabilitation; Electric Stimulation - methods; Female; Head and Neck Surgery; Hearing Loss, Sensorineural - complications; Hearing Loss, Sensorineural - diagnosis; Hearing Loss, Sensorineural - etiology; Hearing Loss, Sensorineural - surgery; Hearing Tests - methods; Humans; Language Development; Male; Medicine; Medicine & Public Health; Neurosurgery; Otology; Otorhinolaryngology; Postoperative Period; Semicircular Canals - abnormalities; Semicircular Canals - physiopathology; Spain; Treatment Outcome; Vestibular Aqueduct - abnormalities; Vestibular Aqueduct - physiopathology
• ISSN: 0937-4477; 1434-4726
• DOI: 10.1007/s00405-015-3652-3

Inner ear bone malformations are one cause of profound sensorineural hearing loss. This investigation focused on those affecting the posterior labyrinth, especially enlarged vestibular aqueduct syndrome, which is associated with fluctuating and progressive hearing loss. The objectives of this study were to analyze the behavior of the electrical stimulation, auditory functionality and linguistic development in patients with inner ear malformations involving the posterior labyrinth. The study included ten patients undergoing cochlear implantation (cases: five with enlarged vestibular aqueduct, two with vestibular aqueduct stenosis/aplasia, and three with semicircular canal disorders). Post-implantation, data were gathered on the electrical stimulation threshold and maximum comfort levels and on the number of functioning electrodes. Evaluation of Auditory Responses to Speech (EARS) subtests were used to assess auditory functionality and language acquisition at 6, 12, and 24 months post-implantation. Results were compared with findings in a control group of 28 cochlear implantation patients without these malformations. No significant differences were found between case and control groups in electrical stimulation parameters; auditory functionality subtest scores were lower in cases than controls, although the difference was only statistically significant for some subtests. In conclusion, cochlear implantation patients with posterior labyrinth bone malformations and profound hearing loss, including those with enlarged vestibular aqueduct syndrome, showed no significant difference in electrical stimulation threshold with controls. Although some auditory functionality test results were lower in cases than in controls, cochlear implantation appears to be beneficial for all patients with these malformations.