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Cochlear implantation in children with congenital inner ear malformation
Ohta Y. , Doi K. , Morihana T. , Suwa K. , Kitahara T. , Imai T. , Nishimura H. , Kawashima T. , Osaki Y. ,
Kizawa K. , Masumura C. , Iwamoto Y. , Hanamoto M. , Inohara H.
Osaka University, Otorhinolaryngology & Head and Neck Surgery, Suita, Japan, 2Kinki University, Otorhinolaryngology, Sayama, Japan,
Osaka Medical Center and Research Institute for Maternal and Child Health, Izumi, Japan, 4Yao Municipal Hospital, Yao, Japan, 5Suita
Municipal Hospital, Suita, Japan, 6Osaka Police Hospital, Osaka, Japan
Introduction: Recently, cochlear implantations for children with severe sensorineural hearing loss have not
been rare in Japan. In the literature, 20% of children with sensorineural hearing loss are supposed to show
radiological anomalies in the temporal bone. Although the inner ear malformation is not a contraindication to
cochlear implantation, there remain some cases with complications or cases that need special consideration for
rehabilitation after surgery.
Material and method: There were 532 cases (adults:337, children:195) that received cochlear implantation at
Osaka University Hospital from January 1991 to May 2013. Among them, 17 cases had some inner ear
malformations in children. We retrospectively assessed the grade of malformation, postoperative hearing results
and surgical complications. We used the classification of inner ear malformation that was suggested by
Sennaroglu et al. in 2002. We adopted meaningful auditory integration scale (MAIS) to evaluate behavior with
sound, and used meaningful use of speech scale (MUSS) to evaluate utterance.
Results: The details of cochleovestibular malformation were as follows; common cavity deformity:2, hypoplastic
cochlea:3, incomplete partition typeâ… :1, incomplete partition typeⅡ:6, vestibular aplasia:1, narrowing of internal
auditory canal(IAC):2. 11 cases were accompanied with some syndromes. 11 cases had also mental retardation.
Three children had severe perilymph/CSF leak during the operation. Three children were re-implanted to
opposite side but two of them are not in use now. In one case of narrowing of IAC, facial spasm is evoked when
the processor is switched on. Two children were re-implanted in the same side because of device failure. We
evaluated hearing results of 12 children who are still using CI now. There was no significant difference due to the
grade of inner ear malformation. We found that hearing results were poor in the cases with mental retardation.
Discussion: In the case of narrowing of IAC, hearing results were poor in comparison with other types of
malformation. But there was no significant difference among other types of malformation. It seemed that the
hearing results depend on accompanied syndromes, especially mental retardation, compared to the grade of
malformation. Of course, it is important to be aware of surgical complications, such as CSF gusher, bleeding,
abnormal course of facial nerve, but cochlear implant is still useful for the children with inner ear malformation.
Therefore, cochlear implantation should be performed for these cases without hesitation.