This is the first study to investigate long-term follow-up of CI patients, in all aspects of QoL combined with speech perception performance, in comparison with postlingually deaf adults without CI.

Objectives: The objectives of this descriptive, retrospective study were to evaluate quality of life, hearing, and vision in patients with Usher syndrome type I with and without cochlear implant. Methods: Quality of life (QoL) of 14 patients with Usher type I (USH1) with a cochlear implant (CI) (seven adults, seven children) was compared with those of 14 patients with USH1 without a CI (12 adults, two children) by means of three questionnaires: NCIQ, SF12, and the Usher Lifestyle Survey. Additional information on hearing level was obtained by the equivalent hearing loss (EHL) principle and on the visual deterioration by the functional vision score (FVS). Results: A significant benefit of CI was seen in the hearing‐specific questionnaire NCIQ. This difference could not be detected in the generic SF12 survey. The Usher Lifestyle Survey indicated that patients with USH1 with a CI tend to be able to live an independent life more easily than the profoundly deaf unimplanted patients with USH1. EHL and FVS scores varied in both groups. Conclusions: Overall QoL can be enhanced by CI in patients with USH1, although effects are mostly seen in hearing‐related QoL items.

Cochlear implantation in patients with USH1 improves the audiologic performance when patients are implanted at an earlier age and is beneficial according to the G(C)BI when performed within the first 2 decades of life.

Patients with Usher syndrome face a special set of challenges in order to maintain their independence when their sight and hearing worsen. Three different types of Usher (I, II and III) are distinguished by differences in onset, progression and severity of hearing loss, and by the presence or absence of balance problems. In this study 93 Usher patients from seven European countries filled out a questionnaire on maintaining independence (60 patients type I, 25 patients type II, four patients type III and four patients type unknown). Results of Usher type I and II patients are presented. Following the Nordic definition of maintaining independence in deaf-blindness, three domains are investigated: access to information, communication and mobility. Research variables in this study are: age and type of Usher, considered hearing loss- and the number of retinitis pigmentosa-related sight problems. Usher type I patients tend to need more help than Usher type II patients and the amount of help that they need grows when patients get older or when considered hearing loss worsens. No patterns in results were seen for the number of retinitis pigmentosa related sight problems.

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