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.

LF pitch perception was found to be abnormal in the majority of adult CI recipients, confirming poor TFS processing of CIs. Similarly, the STARR findings reflected a common performance deterioration with the HI/DI tests, suggesting the cause probably being a lack of access to TFS information. Contralateral hearing aid users obtained a remarkable bimodal benefit for all tests. Such results highlighted the importance of TFS cues for challenging speech perception and the relevance to everyday listening conditions. HI/DI and STARR tests show promise for gaining insights into how TFS and speech perception are being limited and may guide the customization of CI program parameters and support the fine tuning of bimodal listening.

The HiRes and HiRes 120 switch-over groups showed quite similar results for speech perception in quiet. However, the HiRes 120 switch-over group achieved these results within a shorter time after conversion. Switch-on children showed improvement in all speech perception categories, reaching approximately 60% recognition and 30% comprehension in quiet. Results for A section signE discrimination scores in quiet and noise showed a clear improvement for both HiRes 120 switch-on and switch-over groups compared with the HiRes group.

Hearing threshold has been measured in a group of patients following cochlear implantation with a Clarion device for the last eight years. The patients had received either a pre-curved carrier or the Hi-Focus I plus Electrode Positioner System (EPS). The assessment was carried out within the first post-operative week as well as at a later stage, between six and 87 months, post-operatively. Residual hearing thresholds were still measurable early after surgery in 24.5 per cent of the patients, without differences between the two different Clarion models. However, the number of subjects with measurable hearing dropped to 16.3 per cent as observed when hearing was measured at a later stage, with an equal distribution between the two groups of patients. From this study it has been possible to observe that only a limited number of patients maintain residual hearing after Clarion cochlear implantation, and that this tends to decrease further over time. Nevertheless, the performance of these patients for speech tests did not appear to be affected by deterioration of the pure-tone auditory threshold.