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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.

Background: Hearing response following an osteodural decompression of the internal auditory canal (IAC) is controversial., Objective: To evaluate the course of auditory brainstem responses (ABRs) and the early hearing response during the first year following IAC decompression for small to medium-sized vestibular schwannomas occurring in neurofibromatosis type 2 (NF2)., Methods: Retrospective chart review of middle fossa craniotomy for IAC osteodural decompression in NF2-related vestibular schwannomas., Results: Twelve NF2 patients were operated on from 2011 to 2016 for IAC decompression. All had NF2 according to the Manchester criteria. All had a progressive change of their ABRs documented from the diagnosis of NF2 over a mean period of 6.25 [0.36;10.9] yr. Treatment was proposed to stop hearing progression based on the speech discrimination scores (SDSs; n = 4) or for hearing maintenance (n = 8). In patients with prior hearing progression, hearing responses were observed in 3 of the 4 patients during the first year. One patient kept on progressing. In the hearing maintenance group, the SDSs remained stable. SDSs improved from 85% [20-100] to 92.5% [60-100] on average (n = 12) and from 55% [20-80] to 77.5% [50-100] in the hearing progression group (n = 4). ABRs improved in 4 patients following decompression., Conclusion: IAC decompression allows early objective hearing responses in select patients. We suggest that the procedure should be offered to patients with hearing progression based on their SDSs and/or associated progressive increases in their wave III and V latencies on ABRs., (Copyright © 2019 by the Congress of Neurological Surgeons.)

Objective: To analyze the impact of patient selection and auditory monitoring on hearing results after middle fossa craniotomy approach for resection of a vestibular schwannoma (VS)., Study Design: Retrospective case review., Setting: Tertiary referral center., Patients: Patients undergoing a middle fossa craniotomy for resection of VS at a single institution between 1995 and 2006 were included in the study population. Patients presenting with Neurofibromatosis Type 2 or who underwent a combined approach (middle fossa and retrosigmoid) were excluded., Main Outcome Measures: Hearing preservation as measured by serial audiograms., Results: Seventy-seven patients were identified. Before excluding patients with cochlear fossa enhancement and the use of auditory monitoring, 47% of the patients maintained serviceable hearing (American Academy of Otolaryngology-Head and Neck Surgery Class A or B). By selecting tumors that did not involve the cochlear fossa and using auditory monitoring, serviceable postoperative hearing was preserved in 76% of the patients., Conclusion: Modification of our selection criteria for surgery and the use of auditory monitoring have improved our hearing results for patients undergoing a middle fossa approach for resection of VS from 47% to 76%.

Background: The objective of this study was to describe the long-term hearing outcomes of gamma knife treatment for unilateral progressing vestibular schwannomas (VS) presenting with good initial hearing using audiologic data. Methods: A retrospective review was performed between 2010 and 2020 to select patients with progressing unilateral VS and good hearing (AAO-HNS class A) treated with stereotactic gamma knife surgery (GKS). Their audiograms were analyzed along with treatment metrics and patient data. Results: Hearing outcomes with a median follow-up of 5 years post-treatment showed statistically significant loss of serviceable hearing: 34.1% of patients maintained good hearing (AAO-HNS class A), and 56.1% maintained serviceable hearing (AAO-HNS class A and B). Non-hearing outcomes are favorable with excellent tumor control and low facial nerve morbidity. Conclusions: Hearing declines over time in intracanalicular VS treated with GKS, with a significant loss of serviceable hearing after 5 years. The mean cochlear dose and the presence of cochlear aperture obliteration by the tumor are the main statistically significant factors involved in the hearing outcomes. [ABSTRACT FROM AUTHOR]

Purpose: To provide more data on the clinical presentation and natural evolution of facial nerve schwannomas and to provide guidance for therapeutic decision making. Methods: A retrospective case review of eighty patients diagnosed with a facial nerve schwannoma between 1990 and 2018 in ten tertiary referral centers in Europe was performed. Patients' demographics, symptomatology, audiometry, anatomical site (segments involved), size and whenever possible volume measurement were registered. Results: At presentation, transient or persistent facial palsy was the most common symptom, followed by hearing loss. The schwannoma involved more than one segment in the majority of the patients with the geniculate ganglion being most commonly involved. Initial treatment consisted of a wait and scan approach in 67.5%, surgery in 30% and radiation therapy in 2.5% of the patients. Tympanic segment schwannomas caused mainly conductive hearing loss and were more prone to develop facial palsy at follow-up. Internal auditory canal or cerebellopontine angle schwannomas presented with significantly more sensorineural hearing loss. Conclusions: Although modern imaging has improved diagnosis of this tumor, choosing the best treatment modality remains a real challenge. Based on the literature review and current findings, more insights into the clinical course and the management of facial nerve schwannomas are provided. [ABSTRACT FROM AUTHOR]

Objective: To perform a long‐term evaluation of the localisation capabilities in the horizontal plane of single‐sided deaf patients fitted with a BAHA device. Design: Single‐centre retrospective study. Participants: Twenty‐one adults with single‐sided deafness (SSD) with normal hearing in the contralateral ear (pure tone average <20 dB, SDS > 90%) rehabilitated with a Cochlear BAHA device from 2003 to 2012 on the deaf side over a median follow‐up of 8 years. Outcome measures: The task used in this paper is a sound localisation identification task with a set‐up of seven loudspeakers on a semi‐circular array at 30‐degree intervals performed at three periods: before BAHA, initially and at last follow‐up. Our main criterion of judgement was the root‐mean‐square (RMS) localisation error. In addition, the Bern Benefit in Single‐Sided Deafness Questionnaire (BBSS) was administered. Results: The mean RMS localisation error was initially estimated at 64° without any rehabilitation (for a chance level RMS estimated at 81°). Initially, with the BAHA device, the RMS localisation error dropped to 51°. At the last follow‐up evaluation, a significant decrease at 23° was noted. Concerning the Bern Questionnaire, 19% of the patients (n = 4) did not report any change (score of 0), 33% (n = 7) are satisfied (score of +1 or +2) and 48% (n = 10) are very satisfied with the BAHA device (score better than +3). Conclusion: Improvement of sound localisation in the horizontal plane for some SSD patients is likely related to altered processing of monaural spectral cues. The time needed to learn to use the azimuth‐dependent spectral cues takes time. Long‐term follow‐up should be considered for studies investigating sound localisation performance. [ABSTRACT FROM AUTHOR]

Abstract: Objective: The aim of this study was to report speech performance in quiet and in noise, sound localization with cochlear implanted children bilaterally. Their performances were compared also in unilateral conditions. In addition, speech and language evaluation was analyzed. Methods: Twenty-three children implanted with Neurelec Digisonic SP devices in 3 tertiary centres were tested on a battery of speech perception tests in quiet and in noise. Localization was assessed by lateralization tasks (90° and 30°). Progress in speech and language development and subjective assessment of benefit were assessed using several rating scales and questionnaires (categories of auditory perception, speech intelligibility rating, family participating rating scale). Results: Children scored better when tested in bilateral conditions rather than in unilateral conditions. In quiet, the mean scores for the poorer and better side were 52% and 73%, respectively. In the bilateral condition, the mean score increased to 83%. In noise, the mean scores were 39% and 57% respectively, which increased to a mean of 70% in the bilateral condition. Nine children (<9 years) completed the ±90° lateralization task. For both unilateral conditions performance was not significantly different from chance level. In the bilateral condition, the mean score was 86%. The ±30° lateralization score was completed by eight of the older children (>9 years). The scores in the unilateral conditions were closed to chance level, but significantly better in the bilateral condition (mean of 86%). Conclusions: Performances in bilateral conditions were significantly better than in unilateral conditions on speech perception in quiet and in noise. Localization was significantly better when tested in the bilateral condition for ±90° lateralization task for the younger children and the ±30° task for the older children. All these results supported the hypothesis than bilateral cochlear implantation is more beneficial than unilateral implantation in children. [Copyright &y& Elsevier]

The Mongolian gerbil ( Meriones unguiculatus ) is commonly used in hearing research because the hearing frequency spectrum of the gerbil is rather similar to that of the human being. However, a precise description of the surgical post-auricular route has not been reported. The aim of this technical note is to provide details on the procedure and the surgical anatomy of the post-auricular route in the Mongolian gerbil. Surgery was performed under general anesthesia on eight (2 males and 6 females) adult Mongolian gerbils. All steps of the post-auricular route were detailed. This surgery provided an access to the following structures: the semi-circular posterior and lateral canals, the external auditory meatus, the tympanic membrane, the round window, the stapes, the stapedial artery and the reliefs of the cochlea. No anatomic variation was noticed among the 8 animals. This post-auricular route in the Mongolian gerbil defines a brief and simple surgery, overall standardized as a consequence of the absence of common anatomic variation, with painless and uncomplicated post-operative stage. [ABSTRACT FROM AUTHOR]