Cochlear Implant in Patients with Intralabyrinthine Schwannoma without Tumor Removal

(1) Background: Schwannomas of the vestibulocochlear nerve are benign, slow-growing tumors, arising from the Schwann cells. When they originate from neural elements within the vestibule or cochlea, they are defined as intralabyrinthine schwannomas (ILSs). Cochlear implant (CI) has been reported as a feasible solution for hearing restoration in these patients. (2) Methods: Two patients with single-sided deafness (SSD) due to sudden sensorineural hearing loss and ipsilateral tinnitus were the cases. MRI detected an ILS. CI was positioned using a standard round window approach without tumor removal. (3) Results: The hearing threshold was 35 dB in one case and 30 dB in the other 6 mo after activation. Speech audiometry with bisillables in quiet was 21% and 27% at 65 dB, and the tinnitus was completely resolved or reduced. In the localization test, a 25.9° error azimuth was obtained with CI on, compared to 43.2° without CI. The data log reported a daily use of 11 h and 14 h. In order to not decrease the CI’s performance, we decided not to perform tumor exeresis, but only CI surgery to restore functional binaural hearing. (4) Conclusions: These are the sixth and seventh cases in the literature of CI in patients with ILS without any tumor treatment and the first with SSD. Cochlear implant without tumor removal can be a feasible option for restoring binaural hearing without worsening the CI’s performance.

Long-term data of the new transcutaneous partially implantable bone conduction hearing system Osia®

Purpose The new active transcutaneous partially implantable osseointegrated system Cochlear™ Osia® System is indicated in case of conductive or mixed hearing loss (CHL/MHL) with a maximum average bone conduction hearing loss of 55 dB, or in single-sided deafness (SSD). The implant directly stimulates the bone via a piezoelectric transducer and is directed by an external sound processor. We conducted a monocentric retrospective longitudinal within-subject clinical study at our tertiary academic referral center. The aim was to investigate long-term data (2017–2021) on audiological outcomes and hearing-related quality of life for the Osia system. Methods Between 2017 and 2020, 22 adults (18: CHL/MHL; 3: SSD) were implanted with the Osia100 implant; seven received bilateral implants. As of 10/2020, the sound processor was upgraded to Osia 2. Results Mean Osia system use by 04/2021 was 30.9 ± 8.6 months (range 17–40 months). Unaided bone conduction thresholds were unchanged postoperatively. One patient had to be explanted because of prolonged wound infection. Aided hearing thresholds were significantly lower compared to the unaided thresholds preoperatively, along with a marked increase in speech recognition in quiet. Speech processor upgrade resulted in a stable benefit. Patients with CHL/MHL and SSD showed a similar improvement in self-rated hearing performance revealed by SSQ, APHAB, and HUI questionnaires. Conclusion The Osia system is a safe, effective and sustainable option for treatment of conductive and mixed hearing loss or single-sided deafness.

Cochlear Implants in Single-Sided Deafness. Comparison Between Children and Adult Populations With Post-lingually Acquired Severe to Profound Hearing Loss

Objective: To determine audiological and clinical results of cochlear implantation (CI) comparing two populations with single-sided deafness (SSD): post-lingually deaf children between 6 and 12 years of age, and post-lingually deaf adults, in order to evaluate the effect of CI in different age groups.Design: Retrospective case review.Setting: Tertiary clinic.Patients and Method: Twenty-three children and twenty-one adult patients that were candidates for CI with single-side deafness were included. In all cases we evaluate: Speech perception thresholds; disyllabic words test (65 dB SPL) were performed in the modalities S0–SCI–SNH and Auditory Lateralization Test. The Speech, Spatial, and Qualities of Hearing Scale (SSQ) questionnaire was also used. All results were obtained after 12 months of CI activation.Results: In children, the most common etiology was idiopathic sensory-neural hearing loss. They showed positive results in the Auditory Lateralization Test. In the Speech Test, word recognition in noise improved from 2% preoperatively to 61.1% at a mean follow-up of 1 year (S0 condition) in children [test with signal in CI side 60% and signal normal hearing side (plugged) 31%]. The processor was used for >12 h in all cases. With respect to the SSQ questionnaire, parents were more satisfied within the postoperative period than within the preoperative period. For adults, the most common etiology was idiopathic sudden sensorineural hearing loss (SNHL). Positive results in the Auditory Lateralization Test were found. With respect to the Speech Test in quiet conditions: Word recognition in noise improved from 5.7% preoperatively to 71.8% at a mean follow-up of 1 year [test with signal in CI side 68% and signal normal hearing side (plugged) 41%]. The processor was used for >12 h. In the SSQ questionnaire, the post-operative results showed a beneficial effect of the CI. No adverse events were reported during the study period. No differences were found between children and adults in all tests in this study.Conclusions: Cochlear implantation in post-lingually deaf adults and children with SSD can achieve a speech perception outcome comparable with CI in conventional candidates. Improvements in spatial hearing were also observed. Careful patient selection and counseling regarding potential benefits are important to optimize outcomes.

Sound Localization in Single-Sided Deaf Participants Provided With a Cochlear Implant

Spatial hearing is crucial in real life but deteriorates in participants with severe sensorineural hearing loss or single-sided deafness. This ability can potentially be improved with a unilateral cochlear implant (CI). The present study investigated measures of sound localization in participants with single-sided deafness provided with a CI. Sound localization was measured separately at eight loudspeaker positions (4°, 30°, 60°, and 90°) on the CI side and on the normal-hearing side. Low- and high-frequency noise bursts were used in the tests to investigate possible differences in the processing of interaural time and level differences. Data were compared to normal-hearing adults aged between 20 and 83. In addition, the benefit of the CI in speech understanding in noise was compared to the localization ability. Fifteen out of 18 participants were able to localize signals on the CI side and on the normal-hearing side, although performance was highly variable across participants. Three participants always pointed to the normal-hearing side, irrespective of the location of the signal. The comparison with control data showed that participants had particular difficulties localizing sounds at frontal locations and on the CI side. In contrast to most previous results, participants were able to localize low-frequency signals, although they localized high-frequency signals more accurately. Speech understanding in noise was better with the CI compared to testing without CI, but only at a position where the CI also improved sound localization. Our data suggest that a CI can, to a large extent, restore localization in participants with single-sided deafness. Difficulties may remain at frontal locations and on the CI side. However, speech understanding in noise improves when wearing the CI. The treatment with a CI in these participants might provide real-world benefits, such as improved orientation in traffic and speech understanding in difficult listening situations.

Postoperative Benefit of Bone Anchored Hearing Systems: Behavioral Performance and Self-Reported Outcomes

Introduction Bone anchored hearing solutions are a well-known option for patients with a conductive, mixed conductive-sensorineural hearing loss and those with single-sided deafness. Objective The aim of the present study was to evaluate the Ponto bone-anchored hearing system in terms of behavioral performance and self-reported outcomes, by comparing unaided and aided performance (softband and abutment), as well as aided performance with the sound processor on softband (preoperatively) versus abutment (postoperatively). Methods Fourteen adult bone-anchored candidates, with either a bilateral (n = 12) or unilateral (n = 2) conductive or mixed hearing loss, participated in the present study. Sound-field hearing thresholds were evaluated unaided and aided (softband and abutment). A speech-in-noise test was also performed unaided and aided for two spatial configurations (S0N90; implanted side; S0N90; nonimplanted side). The Glasgow Health Status Inventory and the Speech, Spatial and Quality of sound questionnaires were administered pre- and postsurgery to compare quality of life and perceived unaided and aided performance. Skin reaction (Holgers scores) was evaluated at 15 days, 6 weeks, and 10 weeks after surgery. Results Significant improvements postoperatively relative to unaided were obtained for sound-field thresholds at all tested frequencies. Additionally, sound-field thresholds were significantly improved with the sound processor on abutment relative to the softband at frequencies > 1 kHz. Improved performance postoperatively relative to unaided was also obtained in the speech-in-noise test and in self-reported outcomes. Conclusions Improvements in behavioral performance and self-reported outcomes were obtained with the sound processor mounted on abutment.

Short-term outcomes of cochlear implantation for single-sided deafness compared to bone conduction devices and contralateral routing of sound hearing aids—Results of a Randomised controlled trial (CINGLE-trial)

Single-sided deafness (SSD) leads to difficulties with speech perception in noise, sound localisation, and sometimes tinnitus. Current treatments (Contralateral Routing of Sound hearing aids (CROS) and Bone Conduction Devices (BCD)) do not sufficiently overcome these problems. Cochlear implants (CIs) may help. Our aim was to evaluate these treatments in a Randomised Controlled Trial (RCT). Adult SSD patients were randomised using a web-based randomisation tool into one of three groups: CI; trial period of ‘first BCD, then CROS’; trial period of ‘first CROS, then BCD’. After these trial periods, patients opted for BCD, CROS, or No treatment. The primary outcome was speech perception in noise (directed from the front (S0N0)). Secondary outcomes were speech perception in noise with speech directed to the poor ear and noise to the better ear (SpeNbe) and vice versa (SbeNpe), sound localisation, tinnitus burden, and disease-specific quality of life (QoL). We described results at baseline (unaided situation) and 3 and 6 months after device activation. 120 patients were randomised. Seven patients did not receive the allocated intervention. The number of patients per group after allocation was: CI (n = 28), BCD (n = 25), CROS (n = 34), and No treatment (n = 26). In S0N0, the CI group performed significantly better when compared to baseline, and when compared to the other groups. In SpeNbe, there was an advantage for all treatment groups compared to baseline. However, in SbeNpe, BCD and CROS groups performed worse compared to baseline, whereas the CI group improved. Only in the CI group sound localisation improved and tinnitus burden decreased. In general, all treatment groups improved on disease-specific QoL compared to baseline. This RCT demonstrates that cochlear implantation for SSD leads to improved speech perception in noise, sound localisation, tinnitus burden, and QoL after 3 and 6 months of follow-up. For most outcome measures, CI outperformed BCD and CROS. Trial registration: Netherlands Trial Register (www.trialregister.nl): NTR4580, CINGLE-trial.