Treatment of Partial Deafness: The Polish School in World Science

Introduction of the method of treatment of partial deafness with cochlear implants has given many patients the previously inaccessible chance to (re)enter the world of sounds. This method of treatment had been developed and introduced to the world’s medicine by H. Skarżyński in 2002, after the extensive research since 1997. This task involved creation of a multispecialty team of experts, development of a special surgical method, and concept of the therapy reflecting on the world largest number of hearing improving surgeries being performed for 13 years averaging 15,000 otosurgical procedures yearly. This chapter introduces the treatment of partial deafness, considered as the Polish School in world science and medicine

Comprehensive approach to the National Network of Teleaudiology in World Hearing Center in Kajetany, Poland

To improve the quality of patient care, the Institute of Physiology and Pathology of Hearing (IPPH) developed and implemented into the clinical practice in 2009 the National Network of Teleaudiology (NNT). NNT is a specialized network allowing the use of the internet and modern IT tools to provide medical care, rehabilitation and technical support for patients visiting a one of the affiliated policlinics of the Institute. This was the basis for the development and implementation of the implant fitting procedure, using modern internet and telediagnostic technologies - telefiting. This procedure, implemented in several centers throughout the country and aboard, solves the growing need for constant specialist care, check-ups and periodic reviews of speech processors. Clinical engineers, through teleconferencing links, can carry out the whole process of assessing and fitting the cochlear implant assisted by auxiliary staff of the cooperating center. Material of this study consisted of 316 cochlear implant users scheduled for telefitting in one of 8 centers selected for this study. Each patient underwent a teleconsultation procedure. After telefitting the patient was presented with a questionnaire consisting of questions relating to the quality and time effectiveness of telefitting and the preparation process, assessment of the ease of contact with the audiologist, the sense of security and calm during the session, and an overall assessment of its usefulness as an alternative to different kinds of standard visits. Significantly smaller distances that have to be overcome by patients to cooperating centers have significantly reduced the travel time. The benefits of the telefitting program include the results of surveys, the satisfaction of which was reported by majority of them

Hearing Screening around the World

Newborn hearing screening programs for congenital disorders and chronic diseases are expanding worldwide, and children are identified at the earliest possible stage. However, the practice is limited or absent in much of the developing world, such as Africa. Recent epidemiological studies show significant increase of hearing impairments in school-age children (around 20 in 100). Hearing disorders disturb the child’s perception of sound, as well as the development of speech which in consequence negatively affects the child relations in society. The early detection of hearing impairments in children enables the effective implementation of medical and rehabilitation procedures or preventive treatment. According to the guidelines of the European Scientific Consensus on Hearing, the detection and treatment of hearing disorders in early school-age children are of the highest importance. That idea was one of the priorities during Polish Presidency of the Council of the European Union (the second half of 2011). The Institute team, in collaboration with numerous national centers, has laid the groundwork for screening programs and developed methods, procedures, and devices for carrying them out. In addition, the Institute was the coordinator and producer of many programs. Based on this, two screening models have been created—newborn and school-age children

The Stimulation of Polymodal Sensory Perception by Skarżyński (SPPS-S): comparison of stationary and remote therapy results

Hearing is a sense, which has a significant impact on a child’s development. Disorders connected with hearing can have impact in a various form and affect each area of life. Hearing disorders may concern peripheral auditory system as well as its parts responsible for central processing. It is estimated that central auditory processing disorders in its isolated form concern 2-3% of the population of school-age children, however, the problem co-occurring with other disorders may affect even several dozen percents of children. According to the available recommendations, there are three main therapeutic approaches in the treatment of patients with auditory processing disorders: transforming the school environment, teaching the child strategies how to compensate his or her difficulties or using hearing trainings focused on a specific deficit. The main aim of the study is to present the results of SPPS-S therapy dedicated to patients with central auditory processing disorders who have completed the remote version of the method in comparison with patients performing therapy in a rehabilitation center. The Stimulation of Polymodal Sensory Perception by Skarżyński (original name in polish SPPS-S) is a treatment applicable for many different groups of disorders showing comorbidity with central auditory processing disorders. Solutions present in SPPS-S offer multifaceted therapy activating different perceptual modalities (hearing, vision and touch) at the same time, as well as their integration and coordination. Patient may receive the SPPS-S therapy either in the rehabilitation center or at home. The material used to assess the effectiveness of SPPS-S-based therapy included the results of 100 patients who received remote SPPS-S therapy compared to the results of 100 patients who performed therapy at a specialized center. Statistical analysis of the results obtained, which showed that the therapy used resulted in a statistically significant improvement in all auditory functions studied. Results confirm the high effectiveness of The Stimulation of Polymodal Sensory Perception by Skarżyński, both in stationary and remotely implemented form. The quality of telerehabilitation interventions was maintained at the same level as in therapeutic work at the therapeutic center, which was confirmed by the results of patients. Remote SPPS-S therapy as an effective telerehabilitation method has become an effective form of supporting patients in their own homes

Ototoxicity prevention during the SARS-CoV-2 (COVID-19) emergency

Aim of this paper is to remind the risk of ototoxicity when using chloroquine and hydroxychloroquine, in particular as prophylactic agents against SARS-CoV-2, during the pandemic. Healthy subjects taking chloroquine and hydroxychloroquine as prophylactic agents against SARS-CoV-2, during the pandemic, should be screened periodically, at least by Otoacoustic Emissions (OAEs) in order to detect early manifestations of possible cochlear ototoxic damages

Genetic Polymorphisms in Sudden Sensorineural Hearing Loss: An Update

Objective: To investigate the association between genetic polymorphisms and sudden sensorineural hearing loss (SSNHL). Most of the SSNHL cases still remain idiopathic, and several etiopathogenetic hypotheses, including a genetic predisposition, have been proposed. Methods: A literature review was conducted using different databases: Medline/PubMed, EMBASE, and CINAHL, according to the Preferred Reporting Items for Systematic Reviews and Meta-Analyses guidelines. All databases have been searched from May 2016 to April 2020. Results: Genetic susceptibility could represent a key element in the pathogenesis of SSNHL. A number of genetic polymorphisms related to (1) inner ear microvascular disease and endothelial dysfunction and (2) to inner ear oxidative stress and inflammation have been addressed in the current literature. Conclusions: The potential identification of a genetic profile related to SSNHL could provide a more accurate prognostic evidence of idiopathic SSNHL (ISSNHL), offering to the patients not only early-prevention strategies but eventually information on various inheritance modalities. © The Author(s) 2020

Don't forget ototoxicity during the SARS-CoV-2 (Covid-19) pandemic!

Aim of this communication is to remind clinical professionals to be aware of ototoxic side effects of several specific drugs proposed for the treatment of the new virus SARS-CoV-2 (Covid-19). In particular, chloroquine and hydroxychloroquine, azithromycin, as well as antiviral drugs such as remdesivir, favipiravir and lopinavir can all present potential ototoxic side effects. The data in the literature do not offer specific information on their potential synergetic effects nor on their interactions

SARS-CoV-2 (COVID-19) and audio-vestibular disorders

To describe the audio-vestibular disorders related to the newly SARS-CoV-2 infection, including the possible ototoxicity side-effects related to the use of drugs included in the SARS-CoV-2 treatment protocols. A systematic review was performed according to the PRISMA protocol. The Medline and Embase databases were searched from March 1, 2020 to April 9, 2021. Initially the search yielded 400 manuscripts, which were reduced to 15, upon the application of inclusion criteria. Sensorineural hearing loss (SNHL) is the most frequent audio-vestibular symptom described, occurring alone or in association with tinnitus and vertigo. The etiopathogenesis of the inner ear disorders related to COVID-19 infection is still poorly understood. The number of reports of COVID-19 infections associated to audio-vestibular disorders is increasing; even if the quality of the studies available is often insufficient, audio-vestibular disorders should be considered as possible manifestations to be included among the symptoms of this infection

QTc prolongation in patients with hearing loss: Electrocardiographic and genetic study

Background: The aim of the study was to determine, whether electrocardiogram (ECG) screening could reduce the risk of sudden cardiac death in patients with hearing loss through the early diagnosis of Jervell and Lange-Nielsen syndrome and the introduction of the therapy. Methods: One thousand and eighty patients with hearing loss (aged 21.8 ± 19.9 years) underwent ECG. Additionally, all subjects were asked to complete a 3-question survey. Those who met, at least, one of the high-risk criteria underwent further cardiac assessment and genetic testing. Results: QTc assessment was possible in 1,027 patients. Mean QTc measured 422.8 ± 23.7 ms in 313 women, 414.9 ± 27.7 ms in 273 men and 421.1 ± 21.5 ms in 441 children (individuals younger than 14 years). Abnormal QTc was found in 13 (4.1%) women, 20 (7.3%) men, and 72 (16.3%) children. In the studied group, no recessive mutation of KNCQ1 or KCNE1 was found. In 6 patients, other mutations were found: in KCNQ1 (n = 1), in KCNH2 (n = 3) and in SCN5A (n = 1), which were pathogenic for long-QT-syndromes (LQTS), and 2 mutations of unknown clinical significance in SCN5A. Overall, out of these 6 patients LQTS was diagnosed in 3 asymptomatic patients, but with abnormal QTc and in 2 patients with normal QTc, but who were previously treated for epilepsy. Conclusions: Jervell and Lange-Nielsen syndrome is a very rare condition even in a population with hearing loss. In this population, the prevalence of prolonged QT interval is increased over the general population. Further investigations are necessary.

Cognitive Improvement After Cochlear Implantation in Older Adults With Severe or Profound Hearing Impairment: A Prospective, Longitudinal, Controlled, Multicenter Study

Objective: To compare the cognitive evolution of older adults with severe or profound hearing impairment after cochlear implantation with that of a matched group of older adults with severe hearing impairment who do not receive a cochlear implant (CI).Design: In this prospective, longitudinal, controlled, and multicenter study, 24 older CI users were included in the intervention group and 24 adults without a CI in the control group. The control group matched the intervention group in terms of gender, age, formal education, cognitive functioning, and residual hearing. Assessments were made at baseline and 14 months later. Primary outcome measurements included the change in the total score on the Repeatable Battery for the Assessment of Neuropsychological Status for Hearing impaired individuals score and on its subdomain score to assess cognitive evolution in both groups. Secondary outcome measurements included self-reported changes in sound quality (Hearing Implant Sound Quality Index), self-perceived hearing disability (Speech, Spatial, and Qualities of Hearing Scale), states of anxiety and depression (Hospital Anxiety and Depression Scale), and level of negative affectivity and social inhibition (Type D questionnaire).Results: Improvements of the overall cognitive functioning (p = 0.05) and the subdomain “Attention” (p = 0.02) were observed after cochlear implantation in the intervention group; their scores were compared to the corresponding scores in the control group. Significant positive effects of cochlear implantation on sound quality and self-perceived hearing outcomes were found in the intervention group. Notably, 20% fewer traits of Type D personalities were measured in the intervention group after cochlear implantation. In the control group, traits of Type D personalities increased by 13%.Conclusion: Intervention with a CI improved cognitive functioning (domain Attention in particular) in older adults with severe hearing impairment compared to that of the matched controls with hearing impairment without a CI. However, older CI users did not, in terms of cognition, bridge the performance gap with adults with normal hearing after 1 year of CI use. The fact that experienced, older CI users still present subnormal cognitive functioning may highlight the need for additional cognitive rehabilitation in the long term after implantation