Cochlear implantation in children with Mondini dysplasia: our experience

Objective This study details the intra-operative complications, and compares auditory scales post-implantation of either profoundly deaf young children with radiologically normal inner ears (group A) or children with Mondini dysplasia (group B). Methods A retrospective survey was carried out of 338 patients with severe to profound sensorineural hearing loss who underwent cochlear implant surgery from February 2015 to May 2017. Patients were divided into 2 groups of 27 patients each. Both groups were followed up to three years post-implantation. Results Cerebrospinal fluid ooze developed in 12 patients, and 2 patients had a cerebrospinal fluid ‘gusher’, one of which had to be explored within 24 hours. After implant use for one year, both groups had similar speech perception scores. Conclusion The cerebrospinal fluid gusher in Mondini dysplasia should be anticipated and adequately managed intra-operatively. This study highlights the tailoring of a post-implantation rehabilitation programme according to individual needs.

TIGA KASUS IMPLAN KOKLEA PADA DISPLASIA MONDINI

Mondini dysplasia is a kind of cochlear malformation caused by a failure growth of cochlea in the seventh week of pregnancy, results in cochlear turn which only reaches 1,5 turn until less than 2,5 turn. There is sensorineural hearing loss in Mondini dysplasia caused by anatomical malformation. The diagnosis of Mondini dysplasia is made by accurate anamnesis, audiology assessments and imaging results. This case study explains the importance of the holistic process of diagnosis and treatment of Mondini's dysplasia. Purpose: To explain that Mondini dysplasia should be diagnosed immediately and given the cochlear implant. Cases: There had been reported three cases of cochlear malformation of Mondini dysplasi type. They were first diagnosed with sensorineural hearing loss and speech delay in the age of 2-4 years. After had been done sequence of audiology assessments and imaging CT-scan and MRI simultaneously there were obtained that those three patients had Mondini dysplasia. Case Management: The use of hearing aid in those three patients had no effectiveness that brought them to the decision of cochlear implantation. Cochlear implant became such a great challenge even through for experienced physicians considering the scarcity of the case and the risk for complications like pelymph gusher and incorrect insertion of electrode. Cochlear implant surgeries had been done to those three patients with carefu consideration and the selection for appropriate type of electrode. On the first case there had been applied right ear cochlear implant, on the second case was bilateral, and on the third case was in the left ear. Complications of the surgeries were bleeding and perilymph gusher but they had been solved properly. Electrode insertion in all three cases had been placed correctly. Conclusion: Found three cases of Mondini dysplasia with symptoms of bilateral hearing loss, speech development, bilateral Mondini dysplasia, techniques and complications of cochlear implants. These three cases differ in the shape, width and size of the cochlear anatomy if they are studied more specifically. Two cases of unilateral cochlear implant placement and one bilateral case.

THE VALUE OF NAVIGATION SYSTEM IN COCHLEAR IMPLANTATION IN PATIENTS WITH ABNORMALITIES OF THE INTERNAL AND MIDDLE EAR

The use of navigation system during the coclear implantation (CI) in patients with congenital and acquired malformation of the inner ear is necessary because of surgical difficulties. The purpose of our work is to evaluate the effectiveness of the navigation system in congenital and acquired anomalies of the middle and inner ear. Materials and methods The authors followed-up 27 children with bilateral congenital malformation of the inner ear and 58 patients with acquired cochlea obliteration (12 adults and 46 children with the acquired obliteration were included in the group). 55 patients have the acquired obliteration of the inner ear, 3 patients – the total obliteration of the inner ear. Audiological, CT and MRI were performed. All our patients the CI was performed. Conclusion.The use of navigation system during CI in patients with a combination of malformation of the inner and middle ear to increase the efficiency of surgical treatment. The duration of anesthesia and surgery in children with congenital malformation increases when navigation is applied All patients after meningitis or with the fracture of the temporal bone should be performed MRI The patients with an extension of the internal auditory canal, anomaly of Mondini dysplasia of the cochlear, the partial obliteration of the cochlea have good rehabilitation results after CI. The patients with the total obliteration of the inner ear have unsatisfactory results of rehabilitation.