Objective: To assess the impact of cochlear implantation (CI) and speech perception outcomes on the quality of life (QoL) of adult CI users and their communication partners (CP) one-year post-implantation. Design: This research is part of a prospective multicenter study in The Netherlands, called SMILE (Societal Merit of Intervention for hearing Loss Evaluation). Study sample: Eighty adult CI users completed speech perception testing and the Nijmegen Cochear Implant Questionnaire (NCIQ). Fifty-four CPs completed the Hearing Impairment Impact–Significant Other Profile (HII-SOP). Results: CI resulted in a large improvement in the QoL of both users and CPs. Despite this, some users still reported difficulties in the NCIQ sub-domains Social Interactions, Activity Limitations and Self-esteem. Similarly, some CPs still reported moderate and severe third-party disability on the HII-SOP. Additionally, the correlation between speech perception results and self-reported QoL was weak to negligible for both groups. Conclusions: Speech perception in quiet is not an accurate measure of real-life success with a CI. A subset of CI users and CPs still experience substantial QoL challenges regardless of speech perception performance. To improve counselling and intervention, QoL measures should be a standard part of the CI care pathway for CI users and their CPs.

Here, we describe two congenitally deaf male siblings with the same compound heterozygotic, likely pathogenic mutations in the FGF3 gene, associated with the labyrinthine aplasia, microtia and microdontia (LAMM) syndrome. Both children had bilateral cochleovestibular aplasia, precluding cochlear implantation. The elder brother received an auditory brainstem implant (ABI) with very limited auditory responses. During the ABI-surgery of the younger subject, it was discovered that excellent auditory responses could be obtained when the electrode array was placed considerably more caudally and more medially than standard. It was observed that the foramen of Luschka, the entrance to the lateral recess of the fourth ventricle was located more caudally. In view of this observation the good auditory development of the latter child, it was decided to give the older child a contralateral ABI. Again, it turned out that the anatomy of the brainstem was abnormal with a more caudal location of the foramen of Luschka and the cochlear nucleus, and this child is showing good progress with his auditory development. It is concluded that one should be aware of the anatomical differences at the level of the brainstem when placing an ABI in children with this genetic disorder (and most likely also in the LAMM syndrome). This also underpins the need of a multidisciplinary approach with closely collaborating team members and good family guidance when diagnosing and treating children with rare deafness.