Cochlear Implantation in Advanced Otosclerosis: Pitfalls and Successes

Purpose of Review This review will highlight recent outcome-based evidence guiding decision making for cochlear implantation in advanced otosclerosis, related complications, and technical surgical considerations in otosclerosis and the obstructed cochlea. Recent Findings Cochlear implantation in advanced otosclerosis results in consistent, excellent auditory outcomes with improvement in both objective speech recognition scores and subjective quality of life measures. Facial nerve stimulation may occur at higher rates in otosclerosis cochlear implant recipients. Cochlear implantation in the setting of luminal obstruction in osteosclerotic patients may be managed with altered surgical technique to achieve successful auditory improvements. Pre-operative imaging with high resolution CT or MRI may help anticipate intraoperative challenges and post-operative complications in cochlear implantation. Summary Cochlear implantation is an established, successful treatment for profound hearing loss in advanced otosclerosis. Surgeon knowledge of outcomes, complications, and potential surgical challenges is important to appropriately counsel patients regarding auditory rehabilitation options in advanced otosclerosis.

Electrophysiology and Auditory Training

Electrophysiology is an objective evaluation method that allows investigating the responses of the central auditory nervous system arising from the capture of neuronal responses through surface electrodes. In addition to the possibility of investigating and diagnosing different pathologies, electrophysiology proves to be an effective and effective instrument in monitoring auditory intervention programs. Auditory rehabilitation programs is based on the premise of neuroplasticity that derives from a capacity for neuronal change due to intense sound stimulation, specific and directed to the patient’s needs. Throughout this chapter, current studies that correlate electrophysiology with auditory training programs in different clinical populations will be presented, such as: hearing in typically developed individuals, hearing and school difficulties, hearing and CAPD, hearing and otitis media, hearing and hearing loss, and hearing and voice. Electrophysiological tests are important objective measures in predicting the gains to be expected from auditory training programs.

The Rise of Social Media and Digital Content in Auditory Rehabilitation: Quantifying the Reach and Effectiveness of New Distribution Channels

Purpose: The primary aim of this study was to investigate the effectiveness of actively distributing auditory rehabilitation materials through digital channels, with a focus on quantifying overall reach and qualifying user experience. The secondary aim was to assess users' experience and satisfaction with each platform. Method: A retrospective analysis of website traffic to two digital properties: (a) a blog where readers may comment on articles (with active distribution through social media) and (b) a static website with no comment function (with passive organic distribution) from March 2017 to October 2018 was performed. Two key metrics were analyzed across these pages: users and pageviews. Results: The blog received 48.4% more users and 97.0% more pageviews than the static website over the entire 20-month study period. Users were significantly more satisfied with the ease of finding the information and its quality via the blog. Conclusions: We conclude that social media–focused active distribution was indeed effective because it resulted in more total traffic, a greater number of users, more frequent access, and an overall high level of satisfaction with the quality of information and resources. This may have useful implications for improving the distribution of educational health care resources through digital channels.

Hearing Rehabilitation with Cochlear Implants after CyberKnife Radiosurgery of Vestibular Schwannoma: A Report Based on Four Clinical Cases

Severe sensorineural hearing loss can be a symptom of the benign tumor vestibular schwannoma (VS). The treatment of VS with non-invasive stereotactic radiosurgery (SRS) offers a high local tumor control rate and an innovative possibility of sequential hearing rehabilitation with cochlear implantation. This study evaluated the feasibility, complications, and auditory outcomes of such a therapeutic approach. Three males and one female (mean age 65.3 ± 9.4 years) scheduled for cochlear implantation and diagnosed with sporadic VS classified as T1 or T2 (according to Samii) were enrolled in this study. All patients had progressive hearing loss qualifying them for cochlear implantation. First, the tumor was treated using CyberKnife SRS. Next, sequential auditory rehabilitation with a cochlear implant (CI) was performed. Clinical outcomes and surgical feasibility were analyzed, and audiological results were evaluated using pure tone audiometry and speech recognition tests. All patients exhibited open-set speech understanding. The mean word recognition score (at 65 dB SPL, Freiburg Monosyllabic Test, FMT) improved after cochlear implantation in all four patients from 5.0 ± 10% (with hearing aid) preoperatively to 60.0 ± 22.7% six months postoperatively. Our results suggest that in patients with profound hearing loss caused by sporadic vestibular schwannoma, the tumor removal with SRS followed by cochlear implantation is an effective method of auditory rehabilitation.

Genetic and Phenotypic Variability in Chinese Patients With Branchio-Oto-Renal or Branchio-Oto Syndrome

Background: Branchio-oto-renal syndrome (BOR) and branchio-oto syndrome (BOS) are rare autosomal dominant disorders defined by varying combinations of branchial, otic, and renal anomalies. Here, we characterized the clinical features and genetic etiology of BOR/BOS in several Chinese families and then explored the genotypes and phenotypes of BOR/BOS-related genes, as well as the outcomes of auditory rehabilitation in different modalities.Materials and Methods: Probands and all affected family members underwent detailed clinical examinations. Their DNA was subjected to whole-exome sequencing to explore the underlying molecular etiology of BOR/BOS; candidate variants were validated using Sanger sequencing and interpreted in accordance with the American College of Medical Genetics guidelines. In addition, a literature review concerning EYA1 and SIX1 alterations was performed to explore the genotypes and phenotypes of BOR/BOS-related genes.Results: Genetic testing identified the novel deletion (c.1425delC, p(Asp476Thrfs*4); NM_000,503.6), a nonsense variant (c.889C > T, p(Arg297*)), and two splicing variants in the EYA1 gene (c.1050+1G > T and c.1140+1G > A); it also identified one novel missense variant in the SIX1 gene (c.316G > A, p(Val106Met); NM_005,982.4). All cases exhibited a degree of phenotypic variability between or within families. Middle ear surgeries for improving bone-conduction component hearing loss had unsuccessful outcomes; cochlear implantation (CI) contributed to hearing gains.Conclusion: This is the first report of BOR/BOS caused by the SIX1 variant in China. Our findings increase the numbers of known EYA1 and SIX1 variants. They also emphasize the usefulness of genetic testing in the diagnosis and prevention of BOR/BOS while demonstrating that CI for auditory rehabilitation is a feasible option in some BOR/BOS patients.

Keypoints to Successful Newborn Hearing Screening. Thirty Years of Experience and Innovations

Congenital deafness is a major pediatric problem, affecting about 1.5–3 per 1000 newborns. The early treatment through cochlear implantation and auditory rehabilitation has been a historic milestone. Early diagnosis of congenital deafness is an essential requirement to obtain the best results, which is achieved through neonatal screening, a diagnostic practice that we began systematically at the Hospital Clínico in Valencia (Spain) 30 years ago. Neonatal hearing screening is successful in most developed countries. Its implementation has been slow due to the multiple difficulties that its universal application entails since it involves several health professionals and must be carried out, in a short time interval after birth. In addition, it must have a good performance that prevents the overload of other services and that requires experience and continuous adjustments in search of proper protocols. The aim of this review is to shed some light on some key points of neonatal hearing screening, highlighting our experience in the solutions to common problems. We will discuss about techniques, protocols and neonatal or nutritional factors that can influence the screening results. To a summary of our work, an update on the subject is provided with the intention of sharing experiences and facilitating the start-up of the new units.

Auditory and speech performance after unilateral cochlear implantation for cochlear nerve canal stenosis

To explore the correlation between the width of the bony cochlear nerve canal (CNC) and long-term auditory rehabilitation after unilateral cochlear implantation (CI) in pediatric patients with congenital deafness and bilateral cochlear nerve canal stenosis (CNCS). A retrospective review was performed on 10 patients with bilateral CNCS and bilateral congenital profound hearing loss who each underwent unilateral cochlear implantation. The width of the CNC was determined on computed tomography (CT) imaging and following CI, auditory and speech performance following CI were graded using categories of auditory performance (CAP), speech intelligibility rating (SIR), and the meaningful auditory integration scale (MAIS) at 24 months following implantation. No correlation was noted between CAP score and CNCS at 24 months post CI ( P > .05). A positive correlation was noted between SIR score and CNC width (ρ = .81, P < .05). Similarly, a positive correlation was noted between MAIS and CNC width (ρ = .71, P < .05). The width of the CNC in patients with CNCS is positively correlated with some long-term auditory and speech outcomes after CI.

Keypoints to a Successful Newborn Hearing Screening. Thirty Years of Experience and Innovations

Congenital deafness is a major pediatric problem, affecting about 1.5-3 per 1000 newborns. The early treatment through cochlear implantation and auditory rehabilitation has been a historic milestone. Early diagnosis of congenital deafness is an essential requirement to obtain the best results, which is achieved through neonatal screening, a diagnostic practice that we began systematically at the Hospital Cl&iacute;nico in Valencia (Spain) 30 years ago.

Benefit of cochlear implantation in a patient with Myhre syndrome

Myhre syndrome is a rare disorder characterised by short stature, skeletal anomalies, facial dysmorphism and hearing loss (HL), resulting from heterozygous mutations of the SMAD4 gene. We describe the benefits of cochlear implant (CI) in a patient with sensorineural HL carrying a mutation (NM_005359.6: c.1498A>G; p.lle500Val) within the SMAD4 gene, detected by whole-exome sequencing. The CI was inserted through the round window despite otospongiotic abnormalities. Pure-tone audiometry improved up to 20 dBHL. Speech perception in noise (Simplified Noise Reduction - SNR +10) increased from 0% pre implantation with hearing aids to 50% post implantation. The postoperative setting of the electrical stimulation limits yielded an asymmetric map, with lower levels for central electrodes and higher levels for lateral ones. Action potential could not be evoked via medial electrodes, suggesting a cochlear nerve dysfunction. Outcomes related to quality of life and cognitive impairment improved. CI was shown to be an effective auditory rehabilitation strategy.