Implantable hearing devices in clinical practice. Systematic review and consensus statements.

Objective: Implantable hearing devices represent a modern and innovative solution for hearing restoration. Over the years, these high-tech devices have increasingly evolved but their use in clinical practice is not universally agreed in the scientific literature. Congresses, meetings, conferences, and consensus statements to achieve international agreement have been made. This work follows this line and aims to answer unsolved questions regarding examinations, selection criteria and surgery for implantable hearing devices. Materials and methods: A Consensus Working Group was established by the Italian Society of Otorhinolaryngology. A method group performed a systematic review for each single question to identify the current best evidence on the topic and to guide a multidisciplinary panel in developing the statements. Results: Twenty-nine consensus statements were approved by the Italian Society of Otorhinolaryngology. These were associated with 4 key area subtopics regarding pre-operative tests, otological, audiological and surgical indications. Conclusions: This consensus can be considered a further step forward to establish realistic guidelines on the debated topic of implantable hearing devices.

Benefits of bone conduction hearing aid in children with unilateral aural atresia.

Objective: To assess the hearing benefit with a unilateral bone conduction hearing aid in a cohort of children with unilateral aural atresia. Methods: Cross-sectional case series pilot study involving 7 children (median age: 10 years, range 6-11). All patients underwent pure-tone, speech, aided sound field and aided speech audiometry and Simplified Italian Matrix Test (SIMT) with and without bone conduction hearing aid (Baha 5® CochlearTM). Cognitive abilities were assessed in 5 patients. Results: The mean air conduction pure-tone average (PTA) of the atretic ear was 63.2 ± 6.9 dB, while the bone conduction PTA was 12.6 ± 4.7 dB. Speech discrimination score of the atretic ear was 88.6 ± 3.8 dB, while with the hearing aid it was 52.8 ± 1.9 dB. In the contralateral ear, there was no significant air-bone gap, and PTAs for air and bone conduction thresholds were within normal range (PTA ≤ 25 dB). The mean aided air conduction hearing threshold was 26.2 ± 7.97. Mean speech recognition threshold without the hearing aid was -5.1 ± 1.9 dB, and -6.0 ± 1.7 dB with the hearing aid tested with the SIMT. The mean score of the cognitive test was 46.8 ± 42.8. Conclusions: These preliminary findings should encourage clinicians in proposing a unilateral bone conduction hearing aid in children with unilateral atresia.

Long-Term Impedance Trend in Cochlear Implant Users with Genetically Determined Congenital Profound Hearing Loss.

BACKGROUND: Impedance is a basic parameter registered at any cochlear implant (CI) fitting section. It is useful in monitoring electrode functioning and the status of the surrounding anatomical structures. PURPOSE: The main aim of this study is to evaluate the 5-year impedance-value trend in patients affected by congenital genetically determined profound hearing loss implanted with Cochlear Nucleus devices. RESEARCH DESIGN: Observational, retrospective, monocentric study. STUDY SAMPLE: Twenty-seven consecutive patients (9 females: 12.0 ± 7.6 years old; range: 4.2-40.4) with genetic diagnosis of GJB2 mutation causing congenital profound hearing loss who underwent cochlear implantation from 2010 to 2020 with good auditory benefit. INTERVENTION: Impedance values of the CIs were obtained from the CIs' programming software that registers those parameters for each follow-up section of each patient. DATA COLLECTION AND ANALYSIS: Impedance values were measured over time (activation, 6, 12, 24, and 60 months after cochlear implantation), for each of the 22 electrodes, in common ground, monopolar 1, monopolar 2, and monopolar 1 + 2 stimulation modes. RESULTS: A significant variation was found between CI activation and 6-month follow-up. This difference was found for each of the 22 electrodes. Electrodes 1 to 4 showed higher impedance values compared with all other electrodes in each time interval. Repeated-measures analysis of variance ruled out significant variations in impedance values from 6-month to 5-year follow-up. CONCLUSIONS: Impedance values were extremely stable after activation, at least for the first 5 years. In these cases, even minimal impedance variations should be carefully evaluated for their possible implications on hearing performance.

Epidemiologic, Imaging, and Clinical Issues in Bezold's Abscess: A Systematic Review.

Bezold's abscess is a deep neck abscess related to otomastoiditis. Due to the insidious clinical presentation, diagnosis can be extremely challenging, leading to delays in treatment and possible life-threatening complications. The literature currently provides a fragmented picture, presenting only single or small number of cases. The present study aims at examining our experience and the literature findings (based on PRISMA criteria) of 97 patients with Bezold's abscess, summarizing their epidemiology, pathogenesis, clinical presentation, imaging findings, and treatments. Bezold's abscess is found at any age, with overt male prevalence among adults. The clinical presentation, as well as the causative pathogens, are strikingly heterogeneous. Otomastoiditis and cholesteatoma are major risk factors. A clinical history of otitis is commonly reported (43%). CT and MRI are the main diagnostic tools, proving the erosion of the mastoid tip in 53% of patients and the presence of a concomitant cholesteatoma in 40%. Intracranial vascular (24%) or infectious (9%) complications have also been reported. Diagnosis might be easily achieved when imaging (CT) is properly applied. MRI has a limited diagnostic role, but it might be crucial whenever intracranial complications or the coexistence of cholesteatoma are suspected, helping to develop proper treatment (prompt antibiotic therapy and surgery).

Cochlear implant in vestibular schwannomas: long-term outcomes and critical analysis of indications.

PURPOSE: To describe our institutional experience in cochlear implantation after vestibular schwannoma (VS) resection, and compare the audiological outcomes between sporadic and neurofibromatosis type 2 (NF2) VS sub-cohorts of patients, and in relation to preoperative contralateral hearing. METHODS: Seventeen patients (8 sporadic and 9 NF2-associated VSs) who had undergone VS resection and cochlear implant (CI) were analyzed retrospectively. Audiological outcomes at 24 months were correlated with preoperative clinical variables. The results according to VS type (sporadic vs. NF2-associated) and contralateral hearing (impaired vs. normal) were compared. RESULTS: Fourteen CIs were actively used by the patients (77.8%). Twenty-four months after CI activation, the median postoperative PTA (pure tone average) was 45.6 dB nHL and a measurable WRS (Word Recognition Score) was achieved by 44.4% of patients (median WRS = 40%). The median postoperative PTA in the implanted ear resulted better in the group with an impaired contralateral hearing (36.3 dB nHL vs. 78.8 dB nHL, p = 0.019). Good preoperative contralateral hearing status (A-B classes of AAO-HNS) was a negative prognostic factor for CI performance on open-set discrimination (OR = 28.0, 95% CI 2.07-379.25, p = 0.012). CONCLUSIONS: CI is a viable rehabilitative option for patients with sporadic or NF2-associated VS. A good contralateral hearing adversely affects CI outcome and should be taken into consideration for patients' selection and rehabilitation programs.

Pediatric Hearing Loss Management in the COVID-19 Era: Possible Consequences and Resources for the Next Future.

Pediatric hearing loss early diagnosis and treatment have been limited by the current restrictions due to the coronavirus disease 2019 pandemic. The difficulty in accessing the multiple facilities required for the rehabilitative process is influencing the timing of each step of the process. Auditory hearing screening programs, etiological characterization, surgical timing, and speech therapies have all been limited in the past year. The current conditions have forced us to adopt different strategies to overcome the necessary social distancing prescriptions. Although their efficacy should be proved over time, some of these resources will be probably useful even in a nonpandemic future.

Bulging of the Oval Window in Common Cavity Deformity: A Possible Predictor of Meningitis.

OBJECTIVE: To investigate the prevalence-rate of oval window bulging in the common cavity and its association with bacterial meningitis. PATIENTS: CT and clinical files of 29 children with preliminary diagnosis of common cavity deformity were collected from 13 Italian centers. INTERVENTION: A retrospective case review study was conducted with a centralized evaluation of the temporal bone CT imaging was performed at Azienda Ospedale - Università Padova, Padova, Italy. MAIN OUTCOME MEASURE: Diagnosis of common cavity was reviewed; in addition, a fluid protrusion into the middle-ear cavity through the oval window at CT imaging was considered as oval window bulging. Its association with the history of bacterial meningitis was investigated. RESULTS: Common cavity deformity was confirmed in 14/29 children (mean-age 11.4 ±â€Š3.8; age-range 5-20; nine females) referred with this diagnosis. In 7/14 patients, the common cavity deformity was bilateral (i.e., 21 common cavities). Oval window bulging was found in 3/19 common cavities (concomitant middle-ear effusive otitis hampered the evaluation in two cases), while the internal acoustic meatus fundus was defective in 10/21 cases. History of bacterial meningitis was found in three children (21%) and two of them had oval window bulging at CT. In the case unrelated to oval window bulging, meningitis occurred late at the age of 12 during acute otitis contralateral to common cavity deformity (ipsilaterally to incomplete partition type 1). CONCLUSION: Patients harboring common cavity deformity have a high risk of meningitis in their first years of life. Oval window bulging seems to be associated with a higher risk of meningitis. This information might be important for appropriate surgical planning.

Genetics of Inner Ear Malformations: A Review.

Inner ear malformations are present in 20% of patients with sensorineural hearing loss. Although the first descriptions date to the 18th century, in recent years the knowledge about these conditions has experienced terrific improvement. Currently, most of these conditions have a rehabilitative option. Much less is known about the etiology of these anomalies. In particular, the evolution of genetics has provided new data about the possible relationship between inner ear malformations and genetic anomalies. In addition, in syndromic condition, the well-known presence of sensorineural hearing loss can now be attributed to the presence of an inner ear anomaly. In some cases, the presence of these abnormalities should be considered as a characteristic feature of the syndrome. The present paper aims to summarize the available knowledge about the possible relationships between inner ear malformations and genetic mutations.

How great is the negative impact of masking and social distancing and how can we enhance communication skills in the elderly people?

During COVID-19 pandemic, protective measures such as social distancing and face masks posed a challenge in daily communication, in this context the elderly are one of the most at risk categories as widely exposed to hearing loss. This article focuses on how the COVID-19 pandemic affected verbal communication, especially on those people that even in normal conditions present an increased difficulty in speech perception. Special attention has been paid to hearing aids and cochlear implant users, these devices indeed can be affected by a speech intelligibility reduction and could be uncomfortable if used together with face masks. Possible alternatives and solutions will be proposed to reduce the negative impacts of face coverings on communication, to enhance speech intelligibility and to manage wearability of hearing rehabilitation devices.

A Modern Case Sheds Light on a Classical Enigma: Beethoven's Deafness.

Two hundred and fifty years have passed since the birth of Ludwig van Beethoven, and the enigma about his hearing loss and overall health status seems to be not completely solved. However, the admission to the hospital of a 64-year-old woman in 2018 with symptoms extremely similar to those experienced by the great composer may add further evidence to a theory previously underestimated. The health issues of the modern patient were found to be due to chronic lead intoxication. The lead was released during daily cooking using a ceramic-coated frying pan with worn surface that poisoned her breakfast most probably for years. Abdominal pain, asthenia, and hearing loss affecting the high frequencies with a many impact on speech intelligibility tormented the patient, as they had Beethoven. An extensive review of the music and medical literature was performed, as well as re-examination of manuscripts, correspondence, and autopsy reports of the famous composer; and great similarities have been found. The soundness of the most-cited classical theories about Beethoven's hearing loss will be discussed. After close scrutiny of the theories, our analysis points toward a progressive sensorineural hearing loss due to lead intoxication as the most probable cause of not only Beethoven's hypoacusis but his overall health status as well. Laryngoscope, 131:179-185, 2021.

Auditory Outcome after Cochlear Implantation in Children with DFNB7/11 Caused by Pathogenic Variants in TMC1 Gene.

INTRODUCTION: Non-syndromic hereditary hearing loss is characterized by extreme genetic heterogeneity. So far, more than 100 pathogenic or likely pathogenic variants in TMC1 gene have been reported in patients with autosomal recessive hearing loss (HL) DFNB7/11. The prevailing auditory phenotype of individuals with DFNB7/11 is congenital, profound, bilateral HL, but the functional outcome after cochlear implantation (CI) described in the literature is variable. The objective of this work is to evaluate the auditory outcome after CI in pediatric patients with DFNB7/11, born to non-consanguineous parents. METHODS: A retrospective analysis of genetic and audiological data of DFNB7/11 patients followed up in a single Italian otolaryngology clinic was performed. Cases with biallelic pathogenic variants in TMC1 were selected from the cohort of children with non-syndromic hearing loss who had undergone CI and had been molecularly characterized by multigene panel testing. All patients underwent extensive audiological assessment, and the auditory outcome after CI was evaluated. RESULTS: DFNB7/11 was diagnosed in a total of 3 patients from 2 non-consanguineous families; a novel disease-causing variant in TMC1 was detected [c.962G>A p.(Trp321*)]. All the affected children showed the typical DFNB7/11 phenotype characterized by prelingual, severe-to-profound HL. The patients showed an excellent functional outcome after CI; speech perception, nonverbal cognition, and speech performance were comparable to those of patients with DFNB1 deafness. DISCUSSION/CONCLUSION: Our results do not support the variable auditory outcome reported in the literature, which may be affected by several social and environmental factors and by the genetic background.

Cochlear Implants in Alström Syndrome.

OBJECTIVES: Too little is known about hearing loss rehabilitation in patients with Alström syndrome (AS). Benefits of hearing aids (HA) have not been fully documented and only one case treated with a Cochlear Implant (CI) has been described in the proceedings of a conference. Furthermore, comorbidities and risk of complications following surgical intervention may contraindicate Cochlear Implant procedures in these patients.The present case report concerns the first AS patient with CI in the literature. METHODS: After reporting a concise description of the audiological profile of patients with AS described in the literature, the case of a 22-year-old woman with genetically confirmed Alström syndrome who underwent a sequential bilateral CI (Bi-CI) rehabilitation is reported. Audiological results before and after cochlear implantation are described. RESULTS: The patient showed an excellent functional outcome with CIs, which enabled her to achieve communicative, social and academic results comparable with her peers, and no complications occurred. CONCLUSIONS: AS is not necessarily an absolute contraindication to CI. For many AS patients, a good cognitive function and adequate life expectancy represent a clear indication to prompt and adequate hearing rehabilitation with CIs. The description of this type of clinical cases could in the future also generate indications for a tailored audiological treatment of patients with very specific needs, such as patients with Alström Syndrome.

Cochlear implantation in adults with auditory deprivation: What do we know about it?

INTRODUCTION: In the ENT community, auditory deprivation is frequently considered as a negative prognostic factor for a good hearing outcome of cochlear implantation (CI), even if a growing literature suggests that this is not completely true. The purpose of this study is to evaluate the results of CI in patients with hearing deprivation, to compare them to results from non-deprived patients and then estimate how time of deprivation impacts on CI outcome and how a bilateral deprivation can affect the outcome compared to a unilateral deprivation. METHODS: Seventy-eight adults with severe to profound post-verbal hearing loss, with and without auditory deprivation history, received CI; audiological results obtained at 3-6-12-24 months follow up post CI were analyzed. RESULTS: No differences were founded between patients with unilateral deprivation and patients with no deprivation. Patients with bilateral deprivation seem to have a worse hearing outcome compared to that of those patients with unilateral deprivation or no deprivation at all. Long time deprivation (>15 years) seems to have a negative influence on the hearing outcome but results with CI remain excellent. CONCLUSIONS: Auditory deprivation should not be considered a contraindication to CI. The duration of auditory deprivation in the implanted ear seems to be a negative prognostic factor only for ears deprived from more of 15 years.

Cochlear implant outcomes in the elderly: a uni- and multivariate analyses of prognostic factors.

PURPOSE: To assess preoperative features that could predict the audiological outcome after cochlear implantation in the elderly, in terms of pure tone audiometry, speech audiometry, and speech perception performance. METHODS: All available records of patients with cochlear implants aged 65 or more at the time of their implantation at our Institution were reviewed (50 patients, mean age 70.76 ± 4.03 years), recording preoperative clinical features. Pure tone audiometry, speech audiometry, and speech perception performance 1 year after cochlear implant activation and fitting were used as outcome measures. RESULTS: No statistically significant association emerged between clinical features and pure tone audiometry. On univariate analysis, progressive sensorineural hearing loss of unknown origin was associated with a better outcome in terms of speech audiometry and speech perception performance (p = 0.035 and p = 0.033, respectively). On multivariate analysis, progressive sensorineural hearing loss retained its independent prognostic significance in terms of speech perception performance (p = 0.042). The discriminatory power of a two-variable panel (age and etiology of hearing loss) featured an AUC (ROC) of 0.738 (an acceptable discriminatory power according to the Hosmer-Lemeshow scale). CONCLUSIONS: A progressive sensorineural hearing loss of unknown origin was associated with a better outcome in terms of speech perception in the elderly in our case study. Further features that can predict audiological outcome achievable with cochlear implants in the elderly are desirable to perform adequate counselling and rehabilitation programs.

Cochlear-Vestibular Impairment due to West Nile Virus Infection.

OBJECTIVES: West Nile virus (WNV) has been spreading over the last 20 years. Human infection is asymptomatic in most cases. When the disease becomes clinically manifest, it may involve a range of issues, from a mild infection with flu-like symptoms to a neuroinvasive disease. Albeit rarely, WNV-associated sensorineural hearing loss (SNHL) has also been reported. Here we describe two new cases of SNHL and balance impairment caused by WNV infection. METHODS: The patients were investigated with repeated audiometric tests and, for the first time, videonystagmography was also used. RESULTS: Unlike findings in the few other published cases, an improvement in audiometric thresholds and vestibular function was documented in both of our patients. CONCLUSIONS: In the light of our findings, a prospective study would be warranted on a large series of patients with WNV infection in order: (i) to better define the epidemiology of the related cochlear-vestibular involvement; and (ii) to elucidate the virus-related changes to peripheral and central auditory and vestibular functions.

Cogan's syndrome: new therapeutic approaches in the biological era.

INTRODUCTION: Cogan's syndrome (CS) is a rare autoimmune disease, characterized by ocular and vestibulo-auditory involvement. Treatment of CS could be challenging, and the only evidence-based data comes from case reports or series. AREAS COVERED: There have recently been several reports of new treatment strategy involving the use of biological disease-modifying anti-rheumatic drugs such as TNFα inhibitors, anti-CD20 or anti-IL6 receptor antibodies, in cases resistant to first- or second-line drugs. EXPERT COMMENTARY: Corticosteroids are the cornerstone of CS therapy at disease onset and during acute phases of the disease. Conventional immunosuppressive therapy, such as methotrexate, could be used in relapsing patients or as a glucocorticoid sparing agent, but efficacy is often modest. The anti-TNFα monoclonal antibody Infliximab appears to be the most frequently used, leading to an improvement in hearing loss in 89% of the cases and allow corticosteroid tapering in 86% of the patients. The appropriate timing of Infliximab treatment has yet to be thoroughly investigated, but it seems to be more effective when started at an early stage of the disease. Efficacy of others anti-TNFα agents is controversial. Rituximab and Tocilizumab are a safe option, but results on hearing loss have still to be confirmed on larger patients' cohorts.