Our preliminary results show that there is adequate contribution of brainstem implants in the development of auditory-verbal skills. Additional handicaps slow the progress of the prelingually deaf children.

On the 18th of September 2009, a group of health care professionals and scientists involved in implantation of the auditory system attended a meeting convened by the Hacettepe Cochlear Implant Group. The aim of the meeting was to have a detailed discussion on the pressing and pertinent issues around auditory brainstem implantation (ABI) in children and in non-neurofibromatosis Type 2 (NF2) cases and to reach a consensus based on these discussions. surgery, experience of individual ABI centers, intraoperative issues, and rehabilitation with the final session devoted to discussion of the issues raised. The centers presented their experience of ABI in children who had hearing impairment because of congenital abnormalities or pathology where cochlear implantation (CI) was either contraindicated or the possibility of successful placement of the electrode was unlikely. Altogether, 61 children with various types of inner ear malformations, cochleovestibular nerve (CVN) anomalies, cochlear ossification, and bilateral cochlear fractures with cochlear nerve avulsion were presented by different groups. The following topics were discussed, and a consensus was obtained at the end of the meeting on the following issues. IN WHICH CHILDREN AND NON-NF2 PATIENTS IS THE ABI A VIABLE INTERVENTION?Two patient categories were identified: 1) Prelingual patients with inner ear malformations and cochlear nerve hypoplasia/aplasia. ABI provides auditory perception in most patients. The potential for speech and language acquisition in the longer term will depend on the age of implantation, the presence or absence of additional disabilities, and the other established factors seen in CI. It also was concluded that open set speech discrimination is possible in selected cases. In addition, prelingually deafened children because of meningitis with total ossification of both cochleas also should be included in this group. 2) Individuals deafened postlingually because of meningitis, temporal bone fractures with cochlear nerve avulsion, otosclerosis with gross cochlear destruction, or unmanageable facial nerve stimulation with CI. As with CI, the duration of severe or profound deafness is a prognostic indicator, and with short

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Frequency of psychiatric diagnoses made with DSM-IV were in parallel to those reported in the literature. Comorbidity of seizures and psychiatric disorders was noteworthy. The percentage of 'high-functioning' full mutation males supports the previous observations. Ear nose and throat and eye examination revealed remarkably lower prevalence of abnormal findings than reported. MVP was slightly less frequent compared with the single study in the literature. Age at the time of examination had an effect on the outcome of cardiac evaluation. These findings will guide us in future management of the group of patients followed in our institution. The protocol applied provides an applicable outline for multidisciplinary institutional settings dealing with individuals with FXS.

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