The aim of the present study is to construct a reference model with the indication for the attitude, the requirements and the resources needed in order to be able to deal with deafness in the presence of disabilities or associated problems. The study group consisted of 13 adults and 18 children affected by profound deafness, with associated problems and disabilities, who were implanted with Clarion® and Med-El® devices. Selection criteria for candidacy to cochlear implantation and counselling, hospitalization, fitting and speech therapy/rehabilitation are described. Findings were assessed evaluating: (i) use of acoustic feedback, on the ground of Erber's model; (ii) self-sufficiency: assessed by a questionnaire; and (iii) social and family relationships: qualitative judgment based on direct observation, analysis of drawings and structured interviews with family teachers and therapists. The whole group showed benefit from cochlear implantation, with particular satisfaction for post-lingual deaf-blind adults, as well as for subjects with associated psychopathologies and mental retardation. In conclusion, cochlear implants can improve life quality in profoundly deaf subjects with associated disabilities, increasing both listening and communication skills as well as self-sufficiency while family and social relationships tend to remain stable.

The aim of the present study is to assess long-term outcomes of CI in prelingual deafened adolescents and adults, describing, where present, differences in performance, self perceived benefit and highlighting specific characteristics. Twenty-three patients were enrolled: 10 adolescents, 13 young adults. Each patient underwent speech perception/language development, psychological evaluation and structured interviews on self perception concerning CI. 70 % adolescents and 100 % adults used their cochlear implant for most of the day; two adolescents were partial users and one was a non-user. Adolescents' average word recognition and comprehension scores improved respectively from 7 to 29.8 % (p = 0.01) and 3 to 26 % (p = 0.1). Adults' average scores improved significantly from 1.5 to 41.9 % (p = 0.01) and from 18.5 to 52.7% (p = 0.001), respectively. None of the subjects showed a linguistic age adequate to the chronological one: average linguistic age was 7.6 years for adolescents and 19.3 for adults. Structured interviews showed improvement in self-esteem. Adults and most adolescents were fully or moderately satisfied with their implant. Cochlear implantation can be considered a valid option for the rehabilitation of highly motivated and well-selected pre-lingual deafened adolescents and adults. Although there is a substantial variability in both groups of patients and language skills are only marginally influenced by CI, there is still a significant improvement in speech perception. CI was described by both groups as having had a positive impact on their lives; nevertheless adolescents were the ones with a tendency to under-use CI, even those with better hearing outcomes.

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Objective: To evaluate, with a long-term follow-up, the speech perception and language development in children with cytomegalovirus (CMV)-related deafness after cochlear implantation. Study Design: A retrospective study on CMV-related profound deafness and cochlear implantation was performed from 1995 to 2010. Six children with an average follow-up of 10 years were included in this research. Medical history, imaging, cognitive delay, speech perception and production data were reviewed. Results: Two of the 6 patients developed a functional language with the use of phrases and word sequences based on morphological and syntactic rules; the others demonstrated the development of a preverbal or transitional language with the use of single words only. Conclusion: Patients with CMV-related deafness benefit from cochlear implantation; however, the expectations of the parents must be evaluated in a series of counseling efforts prior to the surgery.