Neurofibromatosis type 2 (NF2) confronts patients and their relatives with the dual issue of a progressive disease and disability. Deafness creates a brutal rupture in the patients' course of life, and other disabilities often follow in addition, that further deteriorates their quality of life. Hearing rehabilitation, via a cochlear implant and auditory brainstem implant, attempts to reduce the feeling of isolation and suffering due to communication impairment. A NF2-specific quality of life questionnaire not only helps to evaluate the impact of the disease but it is also useful therapy proposals (treatment, auditory implants). This may contribute to improve the quality of care for patients and their families. Within the multidisciplinary NF2 team, the psychologist offers constant listening of patients, and their suffering at each stage of the disease, that takes into account the life context in which the disease occurs, thus playing a major role in the patient care offered.
Keywords: Auditory brainstem implant; Deafness; NF2; Neurofibromatosis type 2; Psychological; Vestibular schwannoma.
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