Introduction
Various factors, including an aging population and expanding eligibility criteria, may increase the demand for cochlear implants (CIs), potentially resulting in longer waiting times. In most Dutch CI centers, the time between referral and surgery exceeds 6 months. Clinical experience suggests that during the waiting period for cochlear implantation, hearing and communication difficulties increase. Simultaneously, there is an interest in outcomes more closely aligned with patient values and needs, which resulted in the SMILE (Societal Merit of Interventions on hearing Loss Evaluation) study. This paper presents results on observed changes in societal and participatory outcomes during waiting time in participants with a time to CI surgery exceeding 6 months.

Methods
SMILE is a prospective multi-center study including 232 individuals who were referred for unilateral CI. Continuous and nominal data from multiple questionnaires, sent immediately after referral and shortly before surgery, were analyzed by computing differences, Cohen’s D, and odds ratios.

Results
Of the total 232 participants, 102 had a time between inclusion and surgery exceeding 6 months. Of these, 89 had (partially) filled out surveys at both time points. Of all the domain scores 55% did not show differences between timepoints. All Cohen’s D estimates were relatively small, ranging from − 0.298 to 0.388 for those outcomes that showed noteworthy changes.

Conclusion
Waiting time from referral to surgery, even though exceeding 6 months, was observed to not seriously affect non-clinically-prioritized patients in an adverse way. Future investigations should identify subgroups on tolerable waiting times regarding short- and long-term outcomes.

Trial registration
Trial registration number at ClinicalTrials.gov: NCT05525221, 25-8-2022.

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.

Objective: Measuring listening effort using pupillometry is challenging in cochlear implant (CI) users. We assess three validated speech tests (Matrix, LIST, and DIN) to identify the optimal speech material for measuring peak-pupil-dilation (PPD) in CI users as a function of signal-to-noise ratio (SNR). Design: Speech tests were administered in quiet and two noisy conditions, namely at the speech recognition threshold (0 dB re SRT), i.e. the SNR where speech intelligibility (SI) was 50%, and at a more favourable SNR of +6 dB re SRT. PPDs and subjective ratings of effort were obtained. Study sample: Eighteen unilaterally implanted CI users. Results: LIST sentences revealed significantly different PPDs between +6 and 0 dB re SRT and DIN triplets between quiet and +6 dB re SRT. PPDs obtained with the Matrix test were independent of SNR and yielded large PPDs and high subjective ratings even in quiet. Conclusions: PPD is a sensitive measure for listening effort when processing LIST sentences near 0 dB re SRT and when processing DIN triplets at more favourable listening conditions around +6 dB re SRT. PPDs obtained with the Matrix test were insensitive to SNR, likely because it is demanding for CI users even in quiet.

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.

Objectives:
We investigated whether listening effort is dependent on task difficulty for cochlear implant (CI) users when using the Matrix speech-in-noise test. To this end, we measured peak pupil dilation (PPD) at a wide range of signal to noise ratios (SNR) by systematically changing the noise level at a constant speech level, and vice versa.

Design:
A group of mostly elderly CI users performed the Dutch/Flemish Matrix test in quiet and in multitalker babble at different SNRs. SNRs were set relative to the speech-recognition threshold (SRT), namely at SRT, and 5 and 10 dB above SRT (0 dB, +5 dB, and +10 dB re SRT). The latter 2 conditions were obtained by either varying speech level (at a fixed noise level of 60 dBA) or by varying noise level (with a fixed speech level). We compared these PPDs with those of a group of typical hearing (TH) listeners. In addition, listening effort was assessed with subjective ratings on a Likert scale.

Results:
PPD for the CI group did not significantly depend on SNR, whereas SNR significantly affected PPDs for TH listeners. Subjective effort ratings depended significantly on SNR for both groups. For CI users, PPDs were significantly larger, and effort was rated higher when speech was varied, and noise was fixed for CI users. By contrast, for TH listeners effort ratings were significantly higher and performance scores lower when noise was varied, and speech was fixed.

Conclusions:
The lack of a significant effect of varying SNR on PPD suggests that the Matrix test may not be a feasible speech test for measuring listening effort with pupillometric measures for CI users. A rating test appeared more promising in this population, corroborating earlier reports that subjective measures may reflect different dimensions of listening effort than pupil dilation. Establishing the SNR by varying speech or noise level can have subtle, but significant effects on measures of listening effort, and these effects can differ between TH listeners and CI users.

Introduction: Cochlear implantation is the standard treatment for severe to profound hearing loss. While cochlear implant (CI) users can communicate effectively in quiet environments, speech understanding in noise remains challenging. Bimodal hearing, combining a CI in one ear and a hearing aid (HA) in the other, has shown advantages over unilateral electrical hearing, especially for speech understanding in noisy conditions. Beamforming is a technique used to improve speech understanding in noise by detecting sound direction and enhancing frontal (speech) sounds while attenuating background noise. One specific beamformer, Stereozoom, combines signals from microphones in both ears to create a focused beam toward the front resulting in a binaural beamformer (BB), in order to improve speech intelligibility in noise for bilateral and bimodal CI users. Methods: A prospective crossover study involving 17 bimodal CI users was conducted, and participants were tested with various device configurations (CI, HA, CI + HA) with and without BB. Speech recognition testing with the Dutch/Flemish matrix test was performed in a sound-attenuated booth with diffuse noise to simulate realistic listening conditions. Results: The results showed a statistically significant benefit of bimodal hearing over the CI configuration and showed a statistical significant benefit of BB for the CI and CI + HA configuration. The benefit of BB in the HA configuration was not statistically significant probably due to the higher variance. The benefit of BB in the three configurations did not differ statistically significant. Conclusion: In conclusion, bimodal hearing offers advantages for speech understanding in noise for CI users. BB provides a benefit in various device configurations, leading to improved speech intelligibility when speech comes from the front in challenging listening environments.