Hearing Rehabilitation Research Articles

Hearing rehabilitation in postmeningitis deafness in the context of the time factor

Postmeningitis deafness appears in 0-11% of the meningitis cases. Cochlear ossification can develop in these patients, which may make the hearing rehabilitation impossible with cochlear implantation. Due to ossification, it is critical to refer patients to the implant centre without any delay. The aim of this study was to examine the time factor between the appearance of deafness and the first examination in a cochlear implant centre, the possibilities and effectivity of hearing rehabilitation. In our tertial referral centre, postmeningitis deafened patients were examined between 2014 and 2022 retrospectively. Hearing results, imaging, possibilities of rehabilitation, complications of cochlear implantations and the hearing results were investigated. 8 patients (3 children, 5 adults) were investigated. The time between the start of deafness and the first appearance varied between 3 weeks to 9 years. Bilateral profound hearing loss was measured in all patients. In 6 cases, cochlear ossification was observed (4 patients bilateral). Cochlear implantation was conducted in 5 patients (4 bilateral, 1 unilateral). In 3 cases, implantation was impossible due to severe ossification. Hearing results showed good hearing levels with poor speech perception in all cases. The rehabilitation of severe hearing loss caused by meningitis can present many challenges to clinicians. A critical point in the care is the urgent referral of patients to a cochlear implantation centre as soon as possible after the life-threatening condition has passed. The implementation of further diagnostic and the earliest possible implantation is the responsibility of the implantation centre itself. It is recommended to develop a new protocol with the involvement of allied professions to clear patient pathways for an effective treatment strategy. Orv Hetil. 2023; 164(19): 729-738.

Hearing Rehabilitation in Vestibular Schwannoma.

The most common complaint among patients with vestibular schwannoma (VS) is hearing loss. This significantly affects the quality of life before, during, and after treatment for patients with VS. Untreated hearing loss in VS patients may even lead to depression and feelings of social isolation. A variety of devices are available for hearing rehabilitation for patients with vestibular schwannoma. These include contralateral routing of hearing signals (CROSs), bone-anchored hearing devices, auditory brainstem implants (ABI), and cochlear implants. In the United States, ABI is approved for patients 12 years of age and older with neurofibromatosis type 2. In the past few years, cochlear implantation has been offered simultaneously or sequentially with tumor resection or irradiation, or even to patients whose VS have been monitored with serial imaging. However, determining the functional integrity of the auditory nerve in patients with vestibular schwannoma is a challenge. This review article consists of (1) the pathophysiology of vestibular schwannoma (VS), (2) hearing loss in VS, (3) treatment of VS and associated hearing loss, (4) options for auditory rehabilitation in patients with VS with their individual benefits and limitations, and (5) challenges in hearing rehabilitation in this cohort of patients to determine auditory nerve functionality. (6) Future directions.

Early management of presbycusis: recommendations from the French Society of Otorhinolaryngology and Head and Neck Surgery, the French Society of Audiology, and the French Society of Geriatrics and Gerontology.

Presbycusis is the physiological decrease in hearing due to advancing age and begins well before the sixth decade. These recommendations recall the principles of early diagnosis of presbycusis and the means of optimal rehabilitation as soon as the first symptoms appear. The recommendations are based on a systematic analysis of the literature carried out by a multidisciplinary group of ENT physicians, audiologists, geriatricians and hearing specialists from all over France. They are classified as grade A, B, C or professional agreement according to a decreasing level of scientific evidence. The diagnosis of presbycusis is more difficult at the beginning of its evolution but a certain number of tools are available for its early diagnosis and its face-to-face or remote management. In the case of a clinical profile suggestive of presbycusis in a young subject, especially if there are several family cases, it is recommended to propose a genetic investigation. Free-field speech audiometry in noise is recommended to measure intelligibility in a realistic environment. Questionnaires in addition to audiometric tests would allow the best assessment of the patient's disability. Hearing rehabilitation with a hearing aid or cochlear implant may slow or prevent cognitive decline. Combined auditory and cognitive rehabilitation should be offered regardless of the time since the hearing was fitting. It is recommended to integrate programs accessible via smartphones, tablets or the Internet, that include different training domains to complement face-to-face sessions.

(Zu-)Hören mit alterndem Gehirn – eine kognitive Herausforderung

Hearing impairment has been recently identified as a major modifiable risk factor for cognitive decline in later life and has been becoming of increasing scientific interest. Sensory and cognitive decline are connected by complex bottom-up and top-down processes, a sharp distinction between sensation, perception, and cognition is impossible. This review provides a comprehensive overview on the effects of healthy and pathological aging on auditory as well as cognitive functioning on speech perception and comprehension, as well as specific auditory deficits in the 2 most common neurodegenerative diseases in old age: Alzheimer disease and Parkinson syndrome. Hypotheses linking hearing loss to cognitive decline are discussed, and current knowledge on the effect of hearing rehabilitation on cognitive functioning is presented. This article provides an overview of the complex relationship between hearing and cognition in old age.

Survey of Auditory Training Awareness for Hearing Professionals and Elderly Individuals Using Hearing Aids

Purpose: The purpose of this study was to identify the demand for auditory training provision and survey beneficiary experience.Methods: A total of 161 participants (80 hearing professionals and 81 elderly hearing aid users) were surveyed online or in-person. Both groups completed 26 questions related to their experience of providing or receiving auditory training, awareness of need, and preference.Results: The hearing professional group was strongly aware of the need to provide auditory training to improve communication problems in elderly individuals using hearing aids, and the absence of a systematic auditory training program was the biggest factor in not providing auditory training. The elderly hearing aid user group also recognized the need for auditory training to improve communication problems, but they responded that the hassle of visiting a clinic for training and lack of training time were factors that made participation in auditory training difficult.Conclusion: Hearing professionals should expand the scope of aural rehabilitation services to provide effective auditory training programs that fit the concept of rehabilitation, rather than simply providing hearing aid fitting management services to improve communication skills. Therefore, hearing professionals should conduct an intervention program after hearing aid fitting for elderly hearing aid users so that hearing rehabilitation can be comprehensively performed according to auditory and cognitive perceptual changes. In addition, efforts are required to solve limitations that make it difficult for elderly individuals who use hearing aids to participate in auditory training, such as the development of a web-based auditory training program.

Conception and implementation of acertification system for quality control of cochlear implant treatment in Germany. German version

Astandardized and structured process is indispensable for optimal hearing rehabilitation with cochlear implants (CI). The Executive Committee of the German Society of Otorhinolaryngology, Head and Neck Surgery (DGHNO-KHC), initiated the conception of acertification system and aWhitepaper based on the Association of the Scientific Medical Societies in Germany (AWMF) clinical practice guideline (CPG) describing the current medical standards of CI care in Germany. The goal was to independently confirm implementation of this CPG and make this information publicly available. With the support of an independent certification organization, successful implementation of the CI-CPG by ahospital would then be verified and confirmed by awarding the "Cochlear implant-provision institution" (Cochlea-Implantat-versorgende Einrichtung, CIVE) quality certificate. Astructure for implementation of acertification system was developed based on the CI-CPG. The following steps were required: 1)conception of aquality control system for certification of hospitals working in accordance with the CI-CPG; 2)development of required structures for an independent review of quality-relevant structure, process, and result parameters; 3)development of astandard procedure for independent certification of hospitals; 4)development of acertificate and alogo to demonstrate successful certification; 5)practical implementation of the certification. Following design of the certification system and the required organizational structure, the certification system was successfully launched in 2021. Applications for the quality certificate could be formally submitted from September 2021. Atotal of 51off-site evaluations were performed by December 2022. In the first 16months from introduction, 47hospitals were successfully certified as CIVE. In this period, 20experts were trained as auditors, who have since then carried out 18on-site audits in hospitals. In summary, the conceptual design, structure, and practical implementation of acertification system for quality control in CI care was successfully implemented in Germany.

Postnatal genetic umbilical cord analysis for earliest possible detection of inherited hearing impairment

IntroductionThe most common sensorineural disorder in humans is hearing impairment and approximately 60% of prelingual hearing disorders are genetic. Especially parents with a congenital deaf child want to know as early as possible whether their second born child has the same genetic defect or not. The aim of this study is to demonstrate that postnatal genetic umbilical cord analysis is both the earliest detection possibility and sufficient.MethodsWe included first born children with severe hearing impairment that underwent cochlear implantation. All included patients were analyzed genetically and exhibited mutations of either DFNB1 loci or SLC26A4 gene. Additionally, the umbilical cord of the sibling underwent genetic analysis to detect hereditary genetic mutations as early as possible.Results49 newborn children out of 22 families were included in this study. Genetic analysis revealed clinical relevant mutations in all first born children and in four siblings via umbilical cord analysis. All patients who have been diagnosed with a relevant genetic mutation that caused severe hearing impairment underwent hearing rehabilitation via cochlear implant surgery.ConclusionThis study demonstrates the sufficient and early as possible detection of known genetically hearing disorders via umbilical cord analysis. In case of a known familial genetic hearing disorder, it is advisable to analyze newborn siblings for the corresponding genetic defect as soon as possible, to be able to plan and initiate clinical care for the patient as early as possible. It is also extremely important for the parents to obtain clear information about the auditory status of the newborn.

Management of Congenital Aural Atresia and Microtia

Microtia is commonly associated with aural atresia, which will cause cosmetic problems as well as hearing impairment. The management of microtia and aural atresia should improve the aesthetic of the external ear and enhance functional hearing. The reconstruction of the auricle can be performed with autologous rib cartilage or a porous polyethylene framework. Surgeons ideally perform the autologous costal cartilage method at 9 years old or higher considering the development of costal cartilage. For porous polyethylene microtia repair, surgical reconstruction can be performed after 5 years of age. Atresiaplasty can be performed in patients who have normal inner ear function and a well-developed middle ear. The Jahrsdoerfer grading scale is used for assessment of middle ear anatomy and scoring 7 or higher expects good results. The other options for hearing rehabilitation include bone conduction hearing devices or middle ear implants. The surgery for bone conduction devices or middle ear implants can be proposed after 5 years of age. Atresiaplasty is recommended in combination with or after autologous rib graft microtia repair and before porous polyethylene microtia repair. The implantation of the hearing devices should not interfere with auricular reconstruction. It can be performed after autologous rib graft microtia repair or combined with ear elevation.

Comparison of speech understanding taking into account the exact electrode position (SRA/MRA/CA)

Cochlear implantation has been a routine hearing rehabilitation procedure for years. Nevertheless, not all parameters that influence speech understanding after implantation are known. We test the hypothesis whether there is a connection between speech understanding and the position of different electrode types in relation to the modiolus in the cochlea with identical speech processors. For this purpose, in this retrospective study, we compare the hearing results with different electrode types ("Straight Research Array" [SRA], "Modiolar Research Array" [MRA] and "Contour Advance" [CA]) from the manufacturer Cochlear in matched pair groups.After creating three groups using "matched pairs" (n=52 patients per group), the cochlear parameters (length of the outer wall, angle of insertion, insertion depth, cochlear coverage and total length of the electrode in the cochlea, wrapping factor) were measured in the routinely performed manner pre- and post-operative high-resolution CT or DVT. The Freiburg monosyllabic understanding was used as a target variable one year after implantation.In the Freiburg monosyllabic test one year postoperatively, patients with MRA had a monosyllabic understanding of 51.2%, patients with SRA 49.5% and patients with CA 58.0%. It could be shown that with increasing cochlear coverage with MRA and CA, the speech understanding of the patients decreases and with SRA it increases. In addition, it could be shown that the monosyllabic understanding increases with increasing "wrapping factor".The results show that the position of the electrode to the modiolus is not the only factor explaining differences in outcome after cochlear implantation.

Value Propositions of Public Adult Hearing Rehabilitation in Denmark.

Objective: To obtain and evaluate detailed descriptions of potential value propositions as seen by adults undergoing hearing rehabilitation with hearing aids. Design: Semi-structured interviews with patients and audiologists, a literature search, and the inclusion of domain knowledge from experts and scientists were used to derive value propositions. A two-alternative forced-choice paradigm and probabilistic choice models were used to investigate hearing aid users' preferences for the value propositions through an online platform. Study sample: Twelve hearing aid users (mean age 70, range 59-70) and eleven clinicians were interviewed. A total of 173 experienced hearing aid users evaluated the value propositions. Results: Twenty-nine value propositions as described by patients, clinicians, and hearing care experts where identified, from which twenty-one value propositions were evaluated. Results of the pair-wise evaluation method show that the value propositions judged to be the most important for the hearing aid users were: "13. To solve the hearing problem you have", "09. Thorough diagnosis of the hearing", and "16. The hearing aid solution is adapted to individual needs", which are related to finding the correct hearing solution and to be considered in the process. The value propositions judged to be least important were: "04 Next of kin and others involved in the process", "26. To be in the same room as the practitioner", and "29. The practitioner's human characteristics", related to the involvement of others in the process and the proximity and personal manner of the practitioners.

Hearing outcomes in children with single sided deafness: Our experience at a tertiary paediatric otorhinolaryngology unit.

Hearing rehabilitation options for single sided deafness (SSD) include contralateral routing of sound (CROS) aids and bone conduction devices (BCDs). This study aimed to review the management of children with SSD at our tertiary paediatric otolaryngology unit over the last 15 years. A retrospective cohort study was performed. Primary hearing outcomes were measured using the Children's Home Inventory for Listening Difficulties (CHILD) questionnaire score and secondary hearing outcomes were measured using hearing thresholds for speech in noise. Outcomes were measured pre and post bone conduction device (BCD) trial. 49 patients with SSD were identified. 20 children had trial of a BCD. 16 patients had pre- and post- BCD trial CHILD scores available for analysis. There was a statistically significant improvement in CHILD scores and speech in noise testing at +5dB and +0dB following amplification with a BCD. The mean use of BCD was 1.3h per day. We have described the management of children with SSD in our unit. This study demonstrated a statistically significant benefit of BCD use on hearing outcomes. However, device compliance is low suggesting hearing advice choice in the population is complex and further research is warranted.

Cochlear Implantation: Systematic Approach to Preoperative Radiologic Evaluation

Sensorineural hearing loss results from abnormalities that affect the hair cells of the membranous labyrinth, inner ear malformations, and conditions affecting the auditory pathway from the cochlear nerve to the processing centers of the brain. Cochlear implantation is increasingly being performed for hearing rehabilitation owing to expanding indications and a growing number of children and adults with sensorineural hearing loss. An adequate understanding of the temporal bone anatomy and diseases that affect the inner ear is paramount for alerting the operating surgeon about variants and imaging findings that can influence the surgical technique, affect the choice of cochlear implant and electrode type, and help avoid inadvertent complications. In this article, imaging protocols for sensorineural hearing loss and the normal inner ear anatomy are reviewed, with a brief description of cochlear implant devices and surgical techniques. In addition, congenital inner ear malformations and acquired causes of sensorineural hearing loss are discussed, with a focus on imaging findings that may affect surgical planning and outcomes. The anatomic factors and variations that are associated with surgical challenges and may predispose patients to periprocedural complications also are highlighted. © RSNA, 2023 Quiz questions for this article are available through the Online Learning Center. Online supplemental material and the slide presentation from the RSNA Annual Meeting are available for this article.

888 Artificial Neural Network Analysis of Evoked Auditory Brainstem Responses for Prediction of Active Auditory Brainstem Implant (ABI) Electrodes in Patients With Neurofibromatosis Type II

INTRODUCTION: Auditory brainstem implantation (ABI) is used to provide auditory sensations through electric stimulation of the cochlear nucleus and is a key strategy for hearing rehabilitation in patients with neurofibromatosis type II (NF2). The exact position of the 3 by 7 electrode array with respect to the cochlear nucleus is not known, due to complex anatomy following tumor removal, procedures during surgery, potential distortions to the anatomy, and post-operative array migration, and other factors. METHODS: A retrospective study of a prospectively maintained database of ABI users with evoked brainstem responses was carried out at an academic institution. Multi-class classification was conducted using the multilayer perceptron (MLP) feed forward neural network model (FF-NN) to classify and predict the post-operative active electrodes. RESULTS: 1,177 electrodes were stimulated intraoperatively among 85 patients [females, n = 48 (56.6%)]. ABI was implanted to the right and left side in 44 patients (51.7%) and 43 patients (48.3%) respectively. The mean follow-up of ABI was 29.6 months (range, 18-33.4 months). Notably, the heat map showed that electrodes at the center and the medial end (E9-16) of the array showed higher likelihoods of being active, irrespective of right or left side. The network was trained for 500 epochs with a learning rate of 0.3 and achieved a mean classification of about 92.3%. The open accessible software for predicting the monopolar electrode stimulation postoperatively based on bipolar intraoperative stimulation can be found here at: https://hifla.org/abi CONCLUSIONS: An ideal MLP FF-NN structure was achieved and hence may result in understanding more precisely about the ABI stimulation and bringing consistency among the neurosurgeons and audiologists worldwide.

A Review of Virtual Reality for Individuals with Hearing Impairments

Virtual Reality (VR) technologies have the potential to be applied in a clinical context to improve training and rehabilitation for individuals with hearing impairment. The introduction of such technologies in clinical audiology is in its infancy and requires devices that can be taken out of laboratory settings as well as a solid collaboration between researchers and clinicians. In this paper, we discuss the state of the art of VR in audiology with applications to measurement and monitoring of hearing loss, rehabilitation, and training, as well as the development of assistive technologies. We review papers that utilize VR delivered through a head-mounted display (HMD) and used individuals with hearing impairment as test subjects, or presented solutions targeted at individuals with hearing impairments, discussing their goals and results, and analyzing how VR can be a useful tool in hearing research. The review shows the potential of VR in testing and training individuals with hearing impairment, as well as the need for more research and applications in this domain.

Validation and prospects for the use of the Russian version of the Spatial Hearing Questionnaire (SHQ)

Aim to validate the Russian version of the Spatial Hearing Questionnaire (SHQ) used for clarifying the spatial hearing impairment, translated from English and adapted.
 Material and methods. After cultural and linguistic adaptation, the reliability of the Russian version of SHQ was validated using the "test - retest" method in 35 people (mean age 51,215,6 years) in two groups: group I - people with normal hearing (17 people), group II - persons with chronic sensorineural hearing loss levels I-IV (18 people).
 Results. The reliability was assessed by -Cronbach and was equal to 0.981, the total correlation values 0.68 - 0.92. There was a statistically significant difference (p0.05) between mean SHQ scores in the two groups.
 Conclusion. Based on the results of our study, the Russian version of the SHQ can be used in practice of an otorhinolaryngologist as an additional diagnostic tool for spatial hearing disorders and during the hearing rehabilitation.

Developing and Validating a Contextualized Science Literacy Assessment for Adults: The Case of Parents of Hard of Hearing Children

<p style="text-align:justify">The diversity of definitions of science literacy has resulted in a diversity of measurement tools. However, adult science literacy is mainly assessed on short standardized and non-contextualized questions, thus making the study of adult science literacy more qualitative than quantitative. Here we describe the rationale, development, and validation of a questionnaire that associates the use of science in the specific science-related setting of parents of hard of hearing children with general and topic-specific science knowledge. The questionnaire went through four developmental steps: (1) gathering input from hearing rehabilitation experts and parents, (2) testing the close-ended questionnaire (n=10), (3) open-ended questionnaire (n=24), (4) online close-ended questionnaire (n=91). These all assessed general science knowledge, contextual science knowledge in the field of hearing and parents' advocacy knowledge and attitudes. These steps and the resulting assessment tool can thus inform the further development of measures of adult science literacy in context. The findings suggest that although general science knowledge enables the application of science to everyday science-related problems it only explained a small proportion of the variance in contextual science knowledge. Thus, the results strongly point to the importance of measuring adults' science literacy in a context that is relevant to the responders. The findings also underscored the disappointing outcomes of secondary science education, in that formal scientific background predicted general science knowledge but did not account for contextual science knowledge at all. This should elicit concern as to the ability of students to use science knowledge in future personally important science related contexts.</p>

Bonebridge Implantation in Treacher-Collins Syndrome With Conductive Hearing Loss-Case Report.

The Bonebridge (BB) was the first active transcutaneous implantation system for bone conduction. The main indications are conductive or mixed hearing loss and single-sided deafness. Treacher-Collins syndrome (TCS) is a rare genetic disease that affects craniofacial development. The disorder results in deformations of facial structure including ear malformations, especially microtia and ear canal atresia. These patients suffer from conductive hearing loss. CT scans often show unfavorable temporal bone anatomy making placement of an implant difficult. For implantable hearing rehabilitation, patients may decide for conduction implants, such as a BAHA, a Ponto, a Vibrant Soundbridge, or a Bonebridge. In this case report, we present 2 patients with TCS implanted with the Bonebridge system, their audiological results, and quality of life.

Early management of presbycusis: recommendations from the French Society of Otorhinolaryngology and Head and Neck Surgery, the French Society of Audiology, and the French Society of Geriatrics and Gerontology

Presbycusis is the physiological decrease in hearing due to advancing age and begins well before the sixth decade. These recommendations recall the principles of early diagnosis of presbycusis and the means of optimal rehabilitation as soon as the first symptoms appear. The recommendations are based on a systematic analysis of the literature carried out by a multidisciplinary group of doctors and audioprosthetists from all over France. They are graded A, B, C or expert opinion according to decreasing level of scientific evidence. The diagnosis of presbycusis is more difficult at the beginning of its evolution but a certain number of tools are available for its early diagnosis and its management in face-to-face or even distance learning. In case of a clinical profile suggestive of presbycusis in a young subject, especially if there are several family cases, it is recommended to propose a genetic investigation. It is recommended to perform free-field speech audiometry in noise to measure intelligibility in an environment as close as possible to reality. Questionnaires can be used in addition to audiometry to best assess the patient's disability. It is recommended that hearing rehabilitation with a hearing aid or cochlear implant may slow or prevent cognitive decline. Combined auditory and cognitive rehabilitation should be offered regardless of the time elapsed since the fitting. It is recommended to integrate programs accessible via smartphones, tablets or the Internet, integrating different training domains in addition to face-to-face sessions.

A Novel Representation of Audiological and Subjective Findings for Acoustical, Bone Conduction and Direct Drive Hearing Solutions.

The benefit of hearing rehabilitation is often measured using audiological tests or subjective questionnaires/interviews. It is important to consider both aspects in order to evaluate the overall benefits. Currently, there is no standardized method for reporting combined audiological and patient reported subjective outcome measures in clinical practice. Therefore, this study focuses on showing the patient's audiological, as well as subjective outcomes in one graph using data from an existing study. The present paper illustrated a graph presenting data on four quadrants with audiological and subjective findings. These quadrants represented speech comprehension in quiet (unaided vs. aided) as WRS% at 65 dB SPL, speech recognition in noise (unaided vs. aided) as SRT dB SNR, sound field threshold (unaided vs. aided) as PTA4 in dB HL, wearing time and patient satisfaction questionnaire results. As an example, the HEARRING graph in this paper represented audiological and subjective datasets on a single patient level or a cohort of patients for an active bone conduction hearing implant solution. The graph offered the option to follow the user's performance in time. The HEARRING graph allowed representation of a combination of audiological measures with patient reported outcomes in one single graph, indicating the overall benefit of the intervention. In addition, the correlation and consistency between some results (e.g., aided threshold and aided WRS) can be better visualized. Those users who lacked performance benefits on one or more parameters and called for further insight could be visually identified.

Health-related quality of life of the persons with prelingual hearing loss in early adulthood

The paper presents research on health-related quality of life (HRQoL) in young adults diagnosed with various degrees of prelingual hearing loss. HRQoL was measured using the Assessment of Quality of Life (AQoL-8d) questionnaire that allows assessment of the overall health-related quality of life and the dimensions such as independence, senses, pain, mental health, happiness, coping, (social) relations, and self-esteem. The study included 27 persons with hearing loss. Most of them were persons with moderate hearing loss using conventional hearing aids. The control group also included persons with normal hearing matched in age and sex. We found that the overall HRQoL in the studied group with prelingual hearing loss was significantly lower than in the hearing group. It was also lower in the dimensions of independence, pain, and senses (physical sphere) and relations (psychosocial sphere). In psychosocial sphere in the dimensions such as mental health, coping, and self-esteem, the hearing-impaired group obtained lower scores than the hearing group at the level of statistical tendency. Moreover, higher education was related to the higher HRQoL in the physical sphere in general, including in the dimension of independence, but only in the group with hearing loss.Our results indicate that people with prelingual hearing loss require psychological support especially in interpersonal relationships self-esteem, and mental health, as well as hearing and speech rehabilitation to improve their auditory communication with the environment. Further research is required for the greater intensity and frequency of pain experienced in the study group of people with prelingual hearing loss compared to the hearing group.