Rehabilitation for unilateral deafness - Narrative review comparing a novel bone conduction solution with existing options.

Patients with single sided deafness (SSD) struggle with sound localization and speech in noise. Existing treatment options include contralateral routing of signal (CROS) systems, percutaneous bone conduction hearing devices (BCHDs), passive transcutaneous BCHDs, active BCHDs, and cochlear implants. Implanted devices provide benefits in speech in noise compared to CROS devices. Percutaneous BCHDs transmit sound efficiently but have aesthetic drawbacks and skin complications. Scalp attenuation impacts passive transcutaneous BCHD performance. Active BCHDs overcome these issues and provide benefits for speech in noise. Cochlear implantation is the only existing option that restores binaural input but introduces electrical rather than acoustic stimuli to the deaf ear. Active BCHDs have been designed to maintain efficient sound transmission and avoid chronic skin irritation and cosmetic concerns that may occur with percutaneous BCHDs. Cochlear implantation may be a superior option for recently deafened SSD patients, though this requires further study. The duration of deafness, patient age and comorbidities, and a shared decision-making model among patients, surgeons, and audiologists should be considered in device selection. The aim of this manuscript is to review available devices, discuss surgical considerations for implantable devices, review available published results for speech in noise and sound quality with each device, and provide an overview to guide shared decision making for patients and providers. This review consolidates available literature and reviews experience with a newer active transcutaneous active BCHD available for use in the SSD population.

Unilateral Cochlear Implants for Severe, Profound, or Moderate Sloping to Profound Bilateral Sensorineural Hearing Loss: A Systematic Review and Consensus Statements.

Importance: Cochlear implants are a treatment option for individuals with severe, profound, or moderate sloping to profound bilateral sensorineural hearing loss (SNHL) who receive little or no benefit from hearing aids; however, cochlear implantation in adults is still not routine. Objective: To develop consensus statements regarding the use of unilateral cochlear implants in adults with severe, profound, or moderate sloping to profound bilateral SNHL. Design, Setting, and Participants: This study was a modified Delphi consensus process that was informed by a systematic review of the literature and clinical expertise. Searches were conducted in the following databases: (1) MEDLINE In-Process & Other Non-Indexed Citations and Ovid MEDLINE, (2) Embase, and (3) the Cochrane Library. Consensus statements on cochlear implantation were developed using the evidence identified. This consensus process was relevant for the use of unilateral cochlear implantation in adults with severe, profound, or moderate sloping to profound bilateral SNHL. The literature searches were conducted on July 18, 2018, and the 3-step Delphi consensus method took place over the subsequent 9-month period up to March 30, 2019. Main Outcomes and Measures: A Delphi consensus panel of 30 international specialists voted on consensus statements about cochlear implantation, informed by an SR of the literature and clinical expertise. This vote resulted in 20 evidence-based consensus statements that are in line with clinical experience. A modified 3-step Delphi consensus method was used to vote on and refine the consensus statements. This method consisted of 2 rounds of email questionnaires and a face-to-face meeting of panel members at the final round. All consensus statements were reviewed, discussed, and finalized at the face-to-face meeting. Results: In total, 6492 articles were identified in the searches of the electronic databases. After removal of duplicate articles, 74 articles fulfilled all of the inclusion criteria and were used to create the 20 evidence-based consensus statements. These 20 consensus statements on the use of unilateral cochlear implantation in adults with SNHL were relevant to the following 7 key areas of interest: level of awareness of cochlear implantation (1 consensus statement); best practice clinical pathway from diagnosis to surgery (3 consensus statements); best practice guidelines for surgery (2 consensus statements); clinical effectiveness of cochlear implantation (4 consensus statements); factors associated with postimplantation outcomes (4 consensus statements); association between hearing loss and depression, cognition, and dementia (5 consensus statements); and cost implications of cochlear implantation (1 consensus statement). Conclusions and Relevance: These consensus statements represent the first step toward the development of international guidelines on best practices for cochlear implantation in adults with SNHL. Further research to develop consensus statements for unilateral cochlear implantation in children, bilateral cochlear implantation, combined electric-acoustic stimulation, unilateral cochlear implantation for single-sided deafness, and asymmetrical hearing loss in children and adults may be beneficial for optimizing hearing and quality of life for these patients.

Outcomes of unilateral idiopathic sudden sensorineural hearing loss: Two decades of experience.

OBJECTIVES: (a) Determine the demographic and medical risk factors for patients who presented with unilateral idiopathic sudden sensorineural hearing loss (ISSNHL); (b) identify treatments that patients underwent; (c) evaluate the adequacy of follow-up and compliance with long-term hearing rehabilitation. METHODS: Retrospective review of patients who presented with unilateral ISSNHL between January 1998 and December 2017 at a tertiary care academic medical center. RESULTS: Two hundred-four patients met inclusion criteria. Of these, 129 (63.2%) did not undergo treatment at an outside hospital prior to our evaluation. In this subgroup, the average pretreatment pure tone average (PTA) was 61.9 ± 2.5 dB (dB). The most common treatment was oral steroids and was recommended in 76 patients (59.9%). Patients also underwent intratympanic (IT) steroid injections (7.2%) or oral steroids followed by salvage IT injections (19.4%). Mean follow-up duration was 17.9 (±29.2) months, and posttreatment PTA (45.6 ± 2.6 dB) was significantly better than baseline (P < .001). In this cohort, hearing amplification was infrequently recommended. Less than 20% of patients reported active hearing amplification use at their most recent visit. At follow-up, 90 patients (69.8%) reported subjective improvement in hearing after treatment. Only 55 patients (42.6%) showed improvement in PTA compared to their pretreatment audiograms. CONCLUSION: Many patients with ISSNHL experienced audiometric improvement after treatments, but most had persistent hearing loss. The duration of follow-up was short. Most patients did not use long-term hearing amplification. Future studies are needed to identify factors that contribute to reduced follow-up and low compliance with hearing amplification use in ISSNHL. LEVEL OF EVIDENCE: 2c.

Speech and language outcomes of cochlear implantation in children with isolated auditory neuropathy versus cochlear hearing loss.

OBJECTIVE: Children with auditory neuropathy (AN) have variable hearing on pure tone testing, and the presence of speech and language delays often play a major role in the decision to offer cochlear implantation (CI) in this population. Despite this fact, the speech and language outcomes in this group after CI are not well described. This study compares speech and language outcomes after CI in a subset of the pediatric AN population that does not have a confounding cognitive disorder with those of their peers with cochlear hearing loss (CoHL). STUDY DESIGN: Retrospective chart review. SETTING: Tertiary referral center. PATIENTS: Seventeen pediatric patients with AN who received a CI and a group of children with CoHL who received a CI were the subjects of this study. The 2 groups demonstrated similar ages at implant. Children with cognitive delays were excluded from each group. INTERVENTION: Cochlear implantation. MAIN OUTCOME MEASURES: All subjects were evaluated preoperatively and postoperatively with standardized age appropriate speech and language measures, including the Expressive Vocabulary Test (EVT), Peabody Picture Vocabulary Test (PPVT), and Preschool Language Scale (PLS). RESULTS: There was no significant difference between the groups on age of activation of the CI. Children with a diagnosis of AN had a significantly lower unaided pure tone average preoperatively as compared with children with cochlear hearing loss; however, there was no significant difference between the groups on either their preimplantation or postimplantation speech and language scores. CONCLUSION: Children with a diagnosis of AN without associated cognitive or developmental disorders have speech and language outcomes comparable to other children who received a CI.

Outcomes of cochlear implantation in children with isolated auditory neuropathy versus cochlear hearing loss.

OBJECTIVE: Auditory neuropathy (AN) is a heterogeneous clinical entity for which the optimal method of auditory rehabilitation has been a matter of some debate. Such patients often do not receive sufficient benefit from hearing aids. Previous studies have shown that select AN patients may benefit from cochlear implantation (CI), but reported outcomes are variable and likely are a reflection of the heterogeneous patient population included under the umbrella diagnosis of AN. This study compares CI outcomes in a subset of the pediatric AN population who do not have a confounding cognitive disorder with their cochlear hearing loss peers. Additionally, it examines the impact of a confounding cognitive or developmental disorder on CI outcomes within the AN population. STUDY DESIGN: Retrospective chart review. SETTING: Tertiary referral center. PATIENTS: Twenty-six pediatric patients with AN who received a CI were the subjects of this study. Seventeen of these children had a diagnosis of AN alone, and their CI outcomes were compared with those of a similar group of children with cochlear hearing loss. The remaining 9 children had a diagnosis of AN in association with a confounding cognitive or developmental disorder, and their CI outcomes were compared with those of children with isolated AN. INTERVENTION: Cochlear implantation. MAIN OUTCOME MEASURES: All subjects were evaluated preoperatively and postoperatively with age-appropriate speech perception testing. RESULTS: Children with a diagnosis of AN alone performed comparably to their peers with cochlear hearing loss. The presence of a confounding cognitive or developmental disorder within the AN population was correlated with significantly poorer CI outcomes as compared with those of children with isolated AN. CONCLUSION: Children with a diagnosis of AN without associated cognitive or developmental disorders perform at a level comparable to other children requiring a CI. Children with a diagnosis of AN associated with other developmental anomalies derive some benefit from CI but are significantly more likely to continue to rely on nonoral/aural methods of communication after implantation.

Role of electrically evoked auditory brainstem response in cochlear implantation of children with inner ear malformations.

OBJECTIVE: To evaluate the usefulness of promontory electric auditory brainstem response (EABR) testing in children with inner ear malformations before cochlear implantation indicated by postoperative speech performance. STUDY DESIGN: Retrospective analysis. SETTING: Tertiary academic cochlear implant center. PATIENTS: Forty-three children with congenital inner ear malformations, of which 39 received a cochlear implant. Age at implant ranged from 12 months to 13 years. Patients were categorized into 3 groups based on the type of malformations. Groups 1 and 2 involved malformations of the cochlea and/or vestibular organ. Group 3 consisted of children with narrow internal auditory canal. INTERVENTIONS: Preoperative EABR testing with threshold, Wave V amplitude, and latency determination. MAIN OUTCOME MEASURES: Postoperative speech perception performance was measured using Glendonald Auditory Speech Perception Tests for words and sentences, Northwestern University-Children's Perception of Speech test, and minimal pairs test. Patients were further categorized into a speech perception category based on these test results. RESULTS: Mean values for EABR threshold, Wave V amplitude, and latency for Group 1 (11 patients) were 485 microA, 0.21 microV, and 4.51 milliseconds, respectively. Mean values for Group 2 (20 patients) were 556 microA, 0.26 microV, and 4.45 milliseconds, respectively. Mean values for Group 3 (8 patients) were 500 microA, 0.11 microV, and 4.65 milliseconds, respectively. Open-set sentence recognition was possible in 73% in Group 1, 30% in Group 2, and 38% in Group 3 by 36 months after implant activation. Patients with lower preoperative EABR threshold (<600 microA) had better postoperative speech performance (p < 0.05). Larger Wave V amplitude and shorter latency were associated with better speech performance. CONCLUSION: Preoperative EABR is useful in determining cochlear implant candidacy in children with inner ear malformations. Results of this study indicate that the EABR accurately predicts outcome when cochlear implant efficacy is uncertain.

Comparison of continuous interleaved sampling and simultaneous analog stimulation speech processing strategies in newly implanted adults with a Clarion 1.2 cochlear implant.

OBJECTIVE: This study consisted of a within-subjects comparison of speech recognition and patient preference when subjects used two different cochlear implant speech processing strategies with a Clarion 1.2 (enhanced bipolar) device: Simultaneous Analog Stimulation (SAS), and Continuous Interleaved Sampling (CIS). These two strategies used two different electrode configurations: the SAS strategy used bipolar stimulation, whereas the CIS strategy used monopolar stimulation. STUDY DESIGN: This was a multicenter study that used a within-subjects balanced crossover design. Experience with the two strategies was replicated in each subject using an ABAB design. Order of strategy use was balanced across all subjects. SETTING: The study was carried out at several cochlear implant centers affiliated with tertiary medical centers. PATIENTS: Subjects consisted of 25 postlingually deafened adults who received a Clarion cochlear implant. INTERVENTIONS: Total involvement by each subject was 14 weeks. Speech perception testing and sound quality assessments were performed after use with each strategy. MAIN OUTCOME MEASURES: Primary outcome measures include speech perception data and patient responses to questionnaires regarding speech and sound quality. RESULTS: Analyses revealed that performance did not differ significantly by the strategy encountered first as relative to the strategy encountered second and that the order in which a strategy was used did not appear to affect subjects' eventual preference for a particular strategy. Although speech recognition scores tended to be higher for CIS for most of the test measures at most of the test intervals, the analysis of variance to evaluate differences in strategy did not reveal a significant effect of strategy. Further analysis of scores obtained at the replication interval, however, revealed that scores obtained with CIS were significantly higher than scores obtained with SAS on the Hearing in Noise Test sentences in quiet and noise. In addition, significantly more patients indicated a final preference for the CIS strategy than for the SAS strategy. Importantly, both the analysis evaluating order and the analysis evaluating strategy revealed significant effects of evaluation period, indicating that time/experience with the implant had a significant effect on scores for each strategy, regardless of the order in which it was used (first or second). CONCLUSIONS: This study demonstrates that important learning occurs during the first several weeks of cochlear implant use, making it difficult to adequately compare performance with different speech processing strategies. However, the finding that patients often prefer the strategy they understand speech the best with supports the clinical practice of letting adult patients select their preferred strategy without formally evaluating speech perception with each available strategy.