title: Cochlear implantation in patients with inner ear bone malformations with posterior labyrinth involvement: an exploratory study.
abstract: 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.
authors.affiliation: Cochlear Implant Unit, Department of Otolaryngology, San Cecilio University Hospital, Granada, Spain. jm.palomeque@hotmail.com.|Department of Otolaryngology, Costa del Sol Hospital, Autovía A-7, km 187, 29603, Marbella, Málaga, Spain. jm.palomeque@hotmail.com.
authors.forename: Juan Miguel
authors.initials: JM
authors.lastname: Palomeque Vera
authors.affiliation: Department of Internal Medicine, La Axarquía Hospital, Vélez-Málaga, Málaga, Spain.
authors.forename: María
authors.initials: M
authors.lastname: Platero Sánchez-Escribano
authors.affiliation: Department of Otolaryngology, La Inmaculada Hospital, Húercal-Overa, Almería, Spain.
authors.forename: Javier
authors.initials: J
authors.lastname: Gómez Hervás
authors.affiliation: Department of Preventive Medicine and Public Health, University Hospital Central de Asturias, Oviedo, Spain.
authors.forename: María
authors.initials: M
authors.lastname: Fernández Prada
authors.affiliation: Fundación Pública Andaluza para la Investigación Biosanitaria de Andalucía Oriental -Alejandro Otero-, San Cecilio University Hospital, Granada, Spain.
authors.forename: Amanda Rocío
authors.initials: AR
authors.lastname: González Ramírez
authors.affiliation: Cochlear Implant Unit, Department of Otolaryngology, San Cecilio University Hospital, Granada, Spain.
authors.forename: Manuel
authors.initials: M
authors.lastname: Sainz Quevedo
mesh_terms: D000293:Adolescent; D016022:Case-Control Studies; D002648:Child; D002675:Child, Preschool; D019929:Cochlear Implantation / Q000379:methods / Q000534:rehabilitation; D004558:Electric Stimulation / Q000379:methods; D005260:Female; D006319:Hearing Loss, Sensorineural / Q000150:complications / Q000175:diagnosis / Q000209:etiology / Q000601:surgery; D006320:Hearing Tests / Q000379:methods; D006801:Humans; D007804:Language Development; D008297:Male; D011184:Postoperative Period; D012665:Semicircular Canals / Q000002:abnormalities / Q000503:physiopathology; D013030:Spain; D016896:Treatment Outcome; D014723:Vestibular Aqueduct / Q000002:abnormalities* / Q000503:physiopathology
keywords: Cochlear implantation; Enlarged vestibular aqueduct syndrome; Hearing loss; Posterior labyrinth bone malformations