Long term outcome of cochlear implantation in five children with common cavity deformity

IntroductionA common cavity (CC) deformity is a deformed inner ear in which the cochlea and vestibule are confluent forming a common rudimentary cystic cavity. The outcome of cochlear implantation (CI) in this deformity is not expected as good as in other cochlear deformity. Categories of Auditory Performance (CAP) score, Speech Intelligibility Rating (SIR) scale, Meaningful Auditory-Integration Scale (MAIS) and Meaningful Use of Speech Scale (MUSS) are scales designed to assess benefit of functional device (hearing aid, Cochlear implant, ABI) in young children. The aim of the study was to observe and report the long-term audiological progress and speech development of children with CC deformity after CI by means of CAP, SIR, MAIS, MUSS scores and CI aided threshold.Methods and materialsThe retrospective and prospective study was carried out in five Indian children with CC deformity who underwent CI from 2004 to 2010. Demographic data of onset and/or duration of deafness, age at implantation, time with hearing aids before implantation, duration with CI and co-morbidity were retrieved from a local data base. Their outcome score in audition and speech development on the basis of their CAP and SIR score at the end of 1 year of habilitation were collected. All the patients were called to follow up to obtain latest CI aided audiogram, CAP, SIR, MAIS and MUSS scores.ResultsOne male and four female children were detected with common cavity deformity among 258 deaf children of age below 6 years operated during study period. The CAP and SIR Scores increased from average of 0.4 and 1 to 3.4 and 2.4, respectively, over one year of habilitation. The CAP score improved in subsequent follow up to average of 5.0 after more than 3 years of follow up but the SIR score hardly improved. We assessed the MAIS and MUSS at last visit after 3 years. The mean of MAIS and MUSS was 32.4 and 26.4, respectively.ConclusionThough the habilitation outcome was not up to the mark in children with CC deformity, CI provides excellent awareness of environment sound and development of few words.