Systematic Literature Review and Early Benefit of Cochlear Implantation in Two Pediatric Auditory Neuropathy Cases.

Approximately 1 in 10 children with hearing loss is affected by auditory neuropathy spectrum disorder (ANSD). People who have ANSD usually have great difficulty understanding speech or communicating. However, it is possible for these patients to have audiograms that may indicate profound hearing loss up to normal hearing. This disorder is prognosed with positive, intact or present otoacoustic emissions (OAE) and/or cochlear microphonics (CM) as well as abnormal or absent auditory brainstem responses (ABR). Treatment methods include conventional hearing aids as well as cochlear implants. Cochlear implants (CI) usually promise better speech understanding for ANSD patients. We performed a systematic literature review aiming to show what improvements can effectively be achieved with cochlear implants in children with ANSD and compare this with our experience with two cases of ANSD implanted at our clinic. The retrospective review of two young CI patients diagnosed with ANSD during infancy demonstrated improvements over time in speech development communicated by their parents.

Multicentric study on surgical information and early safety and performance results with the Bonebridge BCI 602: an active transcutaneous bone conduction hearing implant.

AIM: This European multicentric study aimed to prove safety and performance of the Bonebridge BCI 602 in children and adults suffering from either conductive hearing loss (CHL), mixed hearing loss (MHL), or single-sided sensorineural deafness (SSD). METHODS: 33 patients (13 adults and 10 children with either CHL or MHL and 10 patients with SSD) in three study groups were included. Patients were their own controls (single-subject repeated measures), comparing the unaided or pre-operative to the 3-month post-operative outcomes. Performance was evaluated by sound field thresholds (SF), word recognition scores (WRS) and/or speech reception thresholds in quiet (SRT) and in noise (SNR). Safety was demonstrated with a device-specific surgical questionnaire, adverse event reporting and stable pure-tone measurements. RESULTS: The Bonebridge BCI 602 significantly improved SF thresholds (+ 25.5 dB CHL/MHL/SSD), speech intelligibility in WRS (+ 68.0% CHL/MHL) and SRT in quiet (- 16.5 dB C/MHL) and in noise (- 3.51 dB SNR SSD). Air conduction (AC) and bone conduction (BC) thresholds remained stable over time. All adverse events were resolved, with none unanticipated. Mean audio processor wearing times in hours [h] per day for the CHL/MHL group were ~ 13 h for adults, ~ 11 h for paediatrics and ~ 6 h for the SSD group. The average surgical length was 57 min for the CHL/MHL group and 42 min for the SSD group. The versatility of the BCI 602 (reduced drilling depth and ability to bend the transition for optimal placement) allows for treatment of normal, pre-operated and malformed anatomies. All audiological endpoints were reached. CONCLUSIONS: The Bonebridge BCI 602 significantly improved hearing thresholds and speech understanding. Since implant placement follows the patient's anatomy instead of the shape of the device and the duration of surgery is shorter than with its predecessor, implantation is easier with the BCI 602. Performance and safety were proven for adults and children as well as for the CHL/MHL and SSD indications 3 months post-operatively.

Modified Power Piston Versus Simultaneous Stapedotomy With Hearing Aids in Otosclerosis: A Follow-Up Study Exploring Speech Recognition, Quality of Life and Usage of Device.

OBJECTIVE: To compare audiologic outcomes, quality-of-life (QoL) and usage-of-device (UoD) between case-matched, otosclerotic patients with mixed hearing loss (MHL) which received (a) stapedotomy and postoperative amplification with hearing aids (SDT+HA) or (b) short-incudial process coupled active middle ear implant with simultaneous stapedotomy (mPP). STUDY DESIGN, SETTING, AND PATIENTS: Prospective, matched case-control, follow-up study conducted at two tertiary otologic referral centers. Eligible were all otosclerotic patients with MHL, which received mPP at either of the two institutions. A case-matched-cohort of SDT+HA-patients was generated from the hospitals database based on preoperative audiologic findings. MAIN OUTCOME MEASURES: For sound- and speech perception, primary outcome parameters were the mean postoperative, aided air-conduction pure tone average (mpa-AC-PTA) and word recognition score at 80 dB speech level (mpa-WRS), for QoL the mean Nijmegen-Cochlear-Implant-Questionnaire (NCIQ) total-score, and for UoD the mean score rated on a 10-point Likert-scale. RESULTS: A total of 28 patients were included; 14 received mPP; mpa-AC-PTA and mpa-WRS significantly improved from 47.1 dB-HL to 34.3 dB-HL (-12.8 dB-HL; p < 0.001) and from 75.0% to 93.2% (+18.2%; p = 0.002) compared to 46.5 dB-HL to 31.9 dB-HL (-14.8 dB-HL; p < 0.008) and 75.0% to 93.2% (+18.2%; p = 0.002) for SDT+HA. No significant difference between groups was observed (all p > 0.1). NCIQ total-score between groups did not significantly differ (70.4 vs. 69.9; p = 0.93). UoD for mPP was significantly higher (6.1 vs. 3.0; p < 0.001). CONCLUSIONS: If medical/technical problems prevent usage of HA in otosclerosis with MHL, mPP can be considered as effective treatment option with similar audiological outcome and QoL. A significantly higher UoD for mPP was observed.

Direct Cochlear Recordings in Humans Show a Theta Rhythmic Modulation of Auditory Nerve Activity by Selective Attention.

The architecture of the efferent auditory system enables prioritization of strongly overlapping spatiotemporal cochlear activation patterns elicited by relevant and irrelevant inputs. So far, attempts at finding such attentional modulations of cochlear activity delivered indirect insights in humans or required direct recordings in animals. The extent to which spiral ganglion cells forming the human auditory nerve are sensitive to selective attention remains largely unknown. We investigated this question by testing the effects of attending to either the auditory or visual modality in human cochlear implant (CI) users (3 female, 13 male). Auditory nerve activity was directly recorded with standard CIs during a silent (anticipatory) cue-target interval. When attending the upcoming auditory input, ongoing auditory nerve activity within the theta range (5-8 Hz) was enhanced. Crucially, using the broadband signal (4-25 Hz), a classifier was even able to decode the attended modality from single-trial data. Follow-up analysis showed that the effect was not driven by a narrow frequency in particular. Using direct cochlear recordings from deaf individuals, our findings suggest that cochlear spiral ganglion cells are sensitive to top-down attentional modulations. Given the putatively broad hair-cell degeneration of these individuals, the effects are likely mediated by alternative efferent pathways compared with previous studies using otoacoustic emissions. Successful classification of single-trial data could additionally have a significant impact on future closed-loop CI developments that incorporate real-time optimization of CI parameters based on the current mental state of the user.SIGNIFICANCE STATEMENT The efferent auditory system in principle allows top-down modulation of auditory nerve activity; however, evidence for this is lacking in humans. Using cochlear recordings in participants performing an audiovisual attention task, we show that ongoing auditory nerve activity in the silent cue-target period is directly modulated by selective attention. Specifically, ongoing auditory nerve activity is enhanced within the theta range when attending upcoming auditory input. Furthermore, over a broader frequency range, the attended modality can be decoded from single-trial data. Demonstrating this direct top-down influence on auditory nerve activity substantially extends previous works that focus on outer hair cell activity. Generally, our work could promote the use of standard cochlear implant electrodes to study cognitive neuroscientific questions.

Long-Term, Multicenter Results With the First Transcutaneous Bone Conduction Implant.

OBJECTIVE: Investigation of long-term safety and performance of an active, transcutaneous bone conduction implant in adults and children up to 36 months post-implantation. STUDY DESIGN: Prospective, single-subject repeated-measures design. SETTING: Otolaryngology departments of eight German and Austrian hospitals.∗†‡§||¶#∗∗†† Affiliations listed above that did not participate in the study.‡‡§§||||¶¶. PATIENTS: Fifty seven German-speaking patients (49 adults and eight children) suffering from conductive or mixed hearing loss, with an upper bone conduction threshold limit of 45 dB HL at frequencies between 500 and 3000 Hz. INTERVENTION: Implantation of the Bonebridge transcutaneous bone conduction hearing implant (tBCI). MAIN OUTCOME MEASURES: Patients' audiometric pure tone averages (PTA4) (0.5, 1, 2, 4 kHz) thresholds (air conduction, bone conduction, and sound field) and speech perception (word recognition scores [WRS] and speech reception thresholds [SRT50%]) were tested preoperatively and up to 36 months postoperatively. Patients were also monitored for adverse events and administered quality-of-life questionnaires. RESULTS: Speech perception (WRS: pre-op: 17.60%, initial activation [IA]: 74.23%, 3M: 83.65%, 12M: 83.46%, 24M: 84.23%, 36M: 84.42%; SRT50%: pre-op: 65.56 dB SPL, IA: 47.67 dB SPL, 3M: 42.61 dB SPL, 12M: 41.11 dB SPL, 24M: 41.74 dB SPL, 36M: 42.43 dB SPL) and sound field thresholds (pre-op: 57.66 dB HL, IA: 33.82 dB HL, 3M: 29.86 dB HL, 12M: 28.40 dB HL, 24M: 28.22 dB HL, 36M: 28.52 dB HL) improved significantly at all aided postoperative visits. Air and bone conduction thresholds showed no significant changes, confirming preservation of patients' residual unaided hearing. All adverse events were resolved by the end of the study. CONCLUSIONS: Safety and performance of the tBCI was demonstrated in children and adults 36 months postoperatively.

Modified-Power-Piston: Short-Incudial-Process-Vibroplasty and Simultaneous Stapedotomy in Otosclerosis.

OBJECTIVE: If mixed-hearing-loss (MHL) occurs in otosclerosis, hearing-aids (HA) in addition to conventional-stapedotomy (SDT) may be necessary. If otosclerosis progresses or technical or medical problems prevent use of HA, combining active-middle-ear-implants (AMEI) with SDT ("power-piston") may be considered. Previously, AMEI-coupling to the long-incudial-process was suggested. Here, a "modified-power-piston" surgery (mPP) coupling to the short-incudial-process was proposed, so no coupling over the positioned stapes-piston is required. We questioned whether mPP is as safe and effective as SDT. METHODS: Otosclerotic patients with MHL and limited satisfaction with previously worn HA receiving mPP were retrospectively reviewed at two Austrian tertiary otologic referral centers. Patients, receiving stapedotomy, were case-matched for preoperative pure-tone averages (PTA), bone-conduction (BC-PTA), air-conduction (AC-PTA), and air-bone gap (ABG-PTA). Postoperative changes in BC-PTA and in AC-PTA and ABG-PTA were defined as safety- and as efficacy outcome parameter. RESULTS: Of 160 patients, 14 received mPP and 14 stapedotomy. Preoperative findings were comparable (all p = 1.000). BC-PTA improved from 38.0 to 36.7 and from 37.1 to 36.9 dB-HL for mPP and SDT, respectively (Δ -1.3 versus -0.2 dB-HL; p = 0.077). AC-PTA improved from 66.8 to 47.1 and from 66.3 to 46.5 dB-HL for mPP and SDT, respectively (Δ -19.6 versus -19.7 dB-HL; p = 0.991). ABG-PTA improved from 28.8 to 10.4 and from 29.1 to 9.6 dB-HL for mPP and SDT, respectively (Δ -18.3 versus -19.5 dB-HL; p = 0.771). CONCLUSION: In otosclerosis with MHL and limited satisfaction with HA, mPP appeared as safe and effective as SDT and may be considered a treatment alternative in these patients.

Minimal Reporting Standards for Active Middle Ear Hearing Implants.

There is currently no standardized method for reporting audiological, surgical and subjective outcome measures in clinical trials with active middle ear implants (AMEIs). It is often difficult to compare studies due to data incompatibility and to perform meta-analyses across different centres is almost impossible. A committee of ENT and audiological experts from Germany, Austria and Switzerland decided to address this issue by developing new minimal standards for reporting the outcomes of AMEI clinical trials. The consensus presented here aims to provide a recommendation to enable better inter-study comparability.

Masking release with changing fundamental frequency: Electric acoustic stimulation resembles normal hearing subjects.

It has been shown that patients with electric acoustic stimulation (EAS) perform better in noisy environments than patients with a cochlear implant (CI). One reason for this could be the preserved access to acoustic low-frequency cues including the fundamental frequency (F0). Therefore, our primary aim was to investigate whether users of EAS experience a release from masking with increasing F0 difference between target talker and masking talker. The study comprised 29 patients and consisted of three groups of subjects: EAS users, CI users and normal-hearing listeners (NH). All CI and EAS users were implanted with a MED-EL cochlear implant and had at least 12 months of experience with the implant. Speech perception was assessed with the Oldenburg sentence test (OlSa) using one sentence from the test corpus as speech masker. The F0 in this masking sentence was shifted upwards by 4, 8, or 12 semitones. For each of these masker conditions the speech reception threshold (SRT) was assessed by adaptively varying the masker level while presenting the target sentences at a fixed level. A statistically significant improvement in speech perception was found for increasing difference in F0 between target sentence and masker sentence in EAS users (p = 0.038) and in NH listeners (p = 0.003). In CI users (classic CI or EAS users with electrical stimulation only) speech perception was independent from differences in F0 between target and masker. A release from masking with increasing difference in F0 between target and masking speech was only observed in listeners and configurations in which the low-frequency region was presented acoustically. Thus, the speech information contained in the low frequencies seems to be crucial for allowing listeners to separate multiple sources. By combining acoustic and electric information, EAS users even manage tasks as complicated as segregating the audio streams from multiple talkers. Preserving the natural code, like fine-structure cues in the low-frequency region, seems to be crucial to provide CI users with the best benefit.

Towards a Unified Testing Framework for Single-Sided Deafness Studies: A Consensus Paper.

BACKGROUND: While hearing aids for a contralateral routing of signals (CROS-HA) and bone conduction devices have been the traditional treatment for single-sided deafness (SSD) and asymmetric hearing loss (AHL), in recent years, cochlear implants (CIs) have increasingly become a viable treatment choice, particularly in countries where regulatory approval and reimbursement schemes are in place. Part of the reason for this shift is that the CI is the only device capable of restoring bilateral input to the auditory system and hence of possibly reinstating binaural hearing. Although several studies have independently shown that the CI is a safe and effective treatment for SSD and AHL, clinical outcome measures in those studies and across CI centers vary greatly. Only with a consistent use of defined and agreed-upon outcome measures across centers can high-level evidence be generated to assess the safety and efficacy of CIs and alternative treatments in recipients with SSD and AHL. METHODS: This paper presents a comparative study design and minimum outcome measures for the assessment of current treatment options in patients with SSD/AHL. The protocol was developed, discussed, and eventually agreed upon by expert panels that convened at the 2015 APSCI conference in Beijing, China, and at the CI 2016 conference in Toronto, Canada. RESULTS: A longitudinal study design comparing CROS-HA, BCD, and CI treatments is proposed. The recommended outcome measures include (1) speech in noise testing, using the same set of 3 spatial configurations to compare binaural benefits such as summation, squelch, and head shadow across devices; (2) localization testing, using stimuli that rove in both level and spectral content; (3) questionnaires to collect quality of life measures and the frequency of device use; and (4) questionnaires for assessing the impact of tinnitus before and after treatment, if applicable. CONCLUSION: A protocol for the assessment of treatment options and outcomes in recipients with SSD and AHL is presented. The proposed set of minimum outcome measures aims at harmonizing assessment methods across centers and thus at generating a growing body of high-level evidence for those treatment options.