Remote Check as a tele-health instrument for cochlear implant recipients: Analysis of impact and feasibility of application.

PURPOSE: The number of cochlear implant (CI) surgeries is growing over time, with the risk of overloading CI centers in the post-surgical management. Telemedicine is a possible solution to address this phenomenon. Remote Check (RC) is an application that is specific for CI recipients monitoring. The aim of this study is to evaluate the feasibility of application, potential economic impact and patients' acceptance of RC. METHODS: The study is retrospective investigation, data on clinical, audiological features (from remote and on-site evaluation), and satisfaction surveys of 66 consecutive patients were collected; costs required for RC and for on-site evaluations were recorded as well. RESULTS: One hundred and ninety RC sessions were completed by the patients (2.88 sessions per patient). RC and on-site audiometry significantly correlated except for the 500 Hz frequency. Estimated costs for the Italian National Health System for RC review and on-site evaluations were 1.32€ and 3.49€ per minute, respectively. High satisfaction for RC was reached in 91 % of patients. CONCLUSION: RC revealed to be a reliable, cost-effective and well accepted tool in CI monitoring. This study preliminarily supports the hypothesis that RC could be a valid instrument to reduce CI management overload in the outpatient clinic.

Hearing loss in beta-thalassaemia: An Italian multicentre case-control study.

BACKGROUND: Despite numerous studies, the true scenario of hearing loss in beta-thalassaemia remains rather nebulous. MATERIALS AND METHODS: Pure tone audiometry, chelation therapy, demographics and laboratory data of 376 patients (mean age 38.5 ± 16.6 years, 204 females, 66 non-transfusion-dependent) and 139 healthy controls (mean age 37.6 ± 17.7 years, 81 females) were collected. RESULTS: Patient and control groups did not differ for age (p = 0.59) or sex (p = 0.44). Hypoacusis rate was higher in patients (26.6% vs. 7.2%; p < 0.00001), correlated with male sex (32.6% in males vs. 21.8% in females; p = 0.01) and it was sensorineural in 79/100. Hypoacusis rate correlated with increasing age (p = 0.0006) but not with phenotype (13/66 non-transfusion-dependent vs. 87/310 transfusion-dependent patients; p = 0.16). Sensorineural-notch prevalence rate did not differ between patients (11.4%) and controls (12.2%); it correlated with age (p = 0.01) but not with patients' sex or phenotype. Among adult patients without chelation therapy, the sensorineural hypoacusis rate was non-significantly lower compared to chelation-treated patients while it was significantly higher compared to controls (p = 0.003). CONCLUSIONS: Sensorineural hypoacusis rate is high in beta-thalassaemia (about 21%) and it increases with age and in males while disease severity or chelation treatment seems to be less relevant. The meaning of sensorineural-notch in beta-thalassaemia appears questionable.

Autosomal Recessive Non-Syndromic Deafness: Is AAV Gene Therapy a Real Chance?

The etiology of sensorineural hearing loss is heavily influenced by genetic mutations, with approximately 80% of cases attributed to genetic causes and only 20% to environmental factors. Over 100 non-syndromic deafness genes have been identified in humans thus far. In non-syndromic sensorineural hearing impairment, around 75-85% of cases follow an autosomal recessive inheritance pattern. In recent years, groundbreaking advancements in molecular gene therapy for inner-ear disorders have shown promising results. Experimental studies have demonstrated improvements in hearing following a single local injection of adeno-associated virus-derived vectors carrying an additional normal gene or using ribozymes to modify the genome. These pioneering approaches have opened new possibilities for potential therapeutic interventions. Following the PRISMA criteria, we summarized the AAV gene therapy experiments showing hearing improvement in the preclinical phases of development in different animal models of DFNB deafness and the AAV gene therapy programs currently in clinical phases targeting autosomal recessive non syndromic hearing loss. A total of 17 preclinical studies and 3 clinical studies were found and listed. Despite the hurdles, there have been significant breakthroughs in the path of HL gene therapy, holding great potential for providing patients with novel and effective treatment.

Intraoperative Measurement of Insertion Speed in Cochlear Implant Surgery: A Preliminary Experience with Cochlear SmartNav.

OBJECTIVES: The objectives were to present the real-time estimated values of cochlear implant (CI) electrode insertion speed (IS) during intraoperative sessions using the Cochlear Nucleus SmartNav System to assess whether this measure affected CI outcomes and to determine whether real-time feedback assists expert surgeons in achieving slow insertion. METHODS: The IS was measured in 52 consecutive patients (65 implanted ears) using the CI632 electrode. The IS values were analyzed in relation to procedure repetition over time, NRT ratio, and CI audiological outcomes. RESULTS: The average IS was 0.64 mm/s (SD = 0.24); minimum and maximum values were 0.23 and 1.24 mm/s, respectively. The IS significantly decreased with each array insertion by the operator (p = 0.006), and the mean decreased by 24% between the first and last third of procedures; however, this reduction fell within the error range of SmartNav for IS (+/-0.48 mm/s). No correlation was found between IS and the NRT ratio (p = 0.51), pure-tone audiometry (PTA) at CI activation (p = 0.506), and PTA (p = 0.94) or word recognition score (p = 0.231) at last evaluation. CONCLUSIONS: The estimated IS reported by SmartNav did not result in a clinically significant reduction in insertion speed or an improvement in CI hearing outcomes. Real-time feedback of IS could potentially be used for training, but its effectiveness requires confirmation through additional studies and more accurate tools. Implementation of IS assessment in clinical practice will enable comparisons between measurement techniques and between manual and robot-assisted insertions. This will help define the optimal IS range to achieve better cochlear implant (CI) outcomes.

Intratympanic Gels for Inner Ear Disorders: A Scoping Review of Clinical Trials.

OBJECTIVE: Intratympanic injections are a safe, well tolerated procedure routinely performed by ENT's specialists. Intratympanic injections of gels have the potential to deliver therapeutics into the cochlea through the round window membrane prolonging the release of drugs in the inner ear compartment. Aim of the present review is to summarize clinical trials testing pharmacological treatments for inner ear pathologies through intratympanic gel formulations. DATA SOURCES: Online databases (Google scholar and PubMed) and registers (Clinicaltrials.gov and Euclinicaltrial) were used to identify clinical trials performed between 1990 and 2022. REVIEW METHODS: PRISMA criteria have been followed. Clinical trials testing gel formulations administered through local intratympanic injections and targeting inner ear disorders were included. All the reports were identified by the authors working in pairs sequentially selecting only studies respecting the inclusion criteria. RESULTS: A total of 45 clinical studies have been noticed; the gels for intratympanic injection are in the form of poloxamers or hyaluronic acid combinations; the trials found target different kind of inner ear disorders: acquired-stable SNHL, tinnitus, acute sudden SNHL, Meniere disease, cisplatin induced ototoxicity and hearing preservation in patients undergoing cochlear implant surgery. CONCLUSION: Few studies listed do not provide the specific kind of gel formulation used but only report the intratympanic delivery vehicle as "gel" or "thermogel". Multiple clinical studies have been targeting several forms of inner ear disorders by injecting different compounds through poloxamer and hyaluronic acid formulations. Larger and more advanced clinical stages are necessary to confirm the efficacy of these chemical compounds.

Bilateral simultaneous cochlear implants in children: Best timing of surgery and long-term auditory outcomes.

PURPOSE: Evaluate the hearing outcomes of bilateral deaf children implanted simultaneously and define the most appropriate timing for surgery. MATERIALS AND METHODS: Audiological CI results were retrieved in both the short-term and long-term period and compared by stratifying the patients into different subcohorts according to their age at surgery. Additional data collected were age at implant activation, etiology and timing of onset of deafness, presence of psychomotor delay. RESULTS: fifty-six bilaterally implanted children were included. The short-term outcomes differed significantly when comparing groups of different ages at implantation: younger patients achieved better aided pure tone audiometry results. Considering long-term follow-up, a significant correlation was identified between an early age at implantation and the hearing outcome at ages 2 to 5 years. Perceptive levels were better at 4 years of age in the younger group. No significant differences were found between children implanted at before 12 months and between 12 and 16 months of age. CONCLUSIONS: The results of the analyzed follow-up data support the hypothesis that children implanted at before 24 months are expected to have better hearing performances. Nevertheless, these results are referred to a widely heterogeneous group of patients and the duration of auditory deprivation should be considered.

Cochlear implantation after head and neck radiotherapy: A multicentric study and systematic review.

OBJECTIVE: The objective of this study is to assess whether cochlear implantation is feasible in patients treated with radiotherapy of the temporal bone (for diseased other than vestibular schwannoma), in terms of surgical management and auditory outcome. METHODS: A systematic review of the literature was performed, screening PubMed, Embase, and Scopus databases, according to PRISMA criteria, retrieving 12 articles. Moreover, 9 cases coming from a multicentric study involving Padova and Pisa University Hospitals, were added, for a total of 62 cases of irradiated patients receiving cochlear implantation. RESULTS: In our multicentric study we included a total of 9 patients, 6 males, and 3 females (mean age at first cochlear implantation was 53.89 years ± 21.07), while from the literature we considered 53 cases (mean age at first cochlear implantation 48.78 years ± 12.41). We considered for both groups a set of preoperative imaging abnormalities, intraoperative complications and possible postoperative complications. In our cohort, the preoperative pure tone average (PTA) in the implanted ear was 105.6 dB, and the postoperative one was 34.6 dB. In the literature the preoperative PTA was 111.5 dB (when reported), and the postoperative one 52.8 dB. CONCLUSION: Both our experience and the literature suggest that cochlear implantation in an irradiated ear is feasible with a good auditory outcome. Surgical difficulties and fitting challenges can be easily managed by professionals aware of these findings, making these patients good candidates to this rehabilitative option.

Age-related changes in auditory perception. Hearing loss in the elderly: aging ear or aging brain?

Hearing loss in the elderly is a very common disease: it is estimated to affect up to a third of the population aged 65 years or more, and 50% of people over 75 years old. There is a growing amount of data concerning the association between hearing loss and cognitive decline. Various possible mechanisms at the basis of this association have been proposed, such as the "common cause hypothesis," the "cascade hypothesis," and the "cognitive load hypothesis."Critically reviewing the data is essential to highlight the features of the relationship between hearing loss and cognitive decline. Most of the hearing tests available should take into account that dementia or even just mild cognitive impairment (MCI) may lead to poor performance during examination. On the other hand, clinicians should also remember that tests used to assess cognitive function require an adequate hearing capacity.In this article we propose to analyze current diagnostic tests, treatment options, auditory processing, and rehabilitation strategies for hearing loss in the elderly in order to facilitate the management of this handicap in this fragile population.

Hearing Aid in Vestibular-Schwannoma-Related Hearing Loss: A Review.

(1) Background: Several types of hearing aids are available for the rehabilitation of vestibular-schwannoma (VS)-related hearing loss. There is a lack of recently published papers regarding this theme. The aim of the present work is to organize current knowledge. (2) Methods: A review of the literature regarding the topics "vestibular schwannoma", "hearing loss", and "hearing aid" was performed. Nineteen studies were thus considered. (3) Results: Conventional hearing aids, contralateral routing of signal (CROS) aids, bone anchored hearing aids (BAHA), and others are available options for hearing rehabilitation in VS patients. The speech discrimination score (SDS) is considered the best measure to assess candidacy for rehabilitation with hearing aids. The best hearing rehabilitative conditions in VS patients when using conventional hearing aid devices are a mild-moderate hearing loss degree with good word recognition (more than 50% SDS). CROS-Aid and BAHA are reported to be beneficial. CROS-Aid expands on the area of receiving hearing. BAHA aids use direct bone-conduction stimulation. Unfortunately, there are no available studies focused specifically on VS patients that compare CROS and BAHA technologies. (4) Conclusions: Hearing aids, CROS, and BAHA are viable options for rehabilitating hearing impairment in VS, but require an accurate case-by-case audiological evaluation for rehabilitating hearing impairment in VS. Further studies are needed to prove if what is currently known about similar hearing illnesses can be confirmed, particularly in the case of VS.

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.

Genetic Evaluation of Prelingual Hearing Impairment: Recommendations of an European Network for Genetic Hearing Impairment.

The cause of childhood hearing impairment (excluding infectious pathology of the middle ear) can be extrinsic (embryofoetopathy, meningitis, trauma, drug ototoxicity, noise trauma, etc [...].

Vestibular anomalies and dysfunctions in children with inner ear malformations: A narrative review.

About 20% of children with congenital hearing loss present malformations of the inner ear. In the past few years much has been understood about the morphology and function of the anterior part of the labyrinth, since hearing loss may have a dramatic effect on the overall development of a child. Nowadays, for most of them, a chance for hearing rehabilitation is available, making hearing loss a treatable condition. The anomalies range from the lack of development of the whole inner ear to specific anomalies of isolated structures. Despite the frequent concomitant involvement of the posterior part of the labyrinth, this part of the inner ear is frequently neglected while discussing its morphology and dysfunction. Even though vestibular and balance function/dysfunction may have a significant impact on the global development of children, very little is known about these specific disorders in patients with inner ear malformations. The aim of this review is to summarize the available literature about vestibular anomalies and dysfunctions in children with inner ear malformations, discussing what is currently known about the topic.

Early cochlear implantation in prelingual profound hearing loss in Italy, analyzed by means of a social media survey.

Objective: To assess newborn hearing screening (NHS) impact on timing of cochlear implant (CI) surgery of patients with prelingual bilateral profound hearing impairment (BPHI), in order to evaluate whether the NHS ultimately serves the needs of the target population in Italy. Methods: An online questionnaire was created to survey subjects affected by prelingual BPHL born between 1990 and 2018. Questions focused on age at BPHI diagnosis, first and second CI surgery (if performed), and the region in which the surgery was performed. The survey was distributed to potential participants via social media communities used by hearing impaired people or their family members for sharing advice and offering support. Responses were analyzed using descriptive statistics. Results: Among the 318 respondents who completed the questionnaire, 276 (87%) reported having chosen CI surgery, 2/3 of them bilaterally. In the vast majority (97%) of cases the CI is used on a daily basis. Most of the people residing in the center (65%) and southern Italy (71%) had to move from their region of residence to perform the surgery. Late CI surgery was associated with failure to perform NHS (p = 0.007), birth before 2011 (p = 0.009), definitive diagnosis of BPHI after 6 months of life (p = 0.002), and progressive hearing impairment (p < 0.001). Conclusion: The worldwide scientific approval of the NHS as the current best opportunity for early diagnosis and CI treatment for prelingual BPHI is confirmed by what patients and families reported via the online questionnaire used for this study. In recent years, early bilateral cochlear implantation has become increasingly available in Italy, but late diagnosis, progressive hearing loss, failure to perform the NHS and lack of follow-up are still open questions. A large proportion of families had to move from the region of residence to have their child undergo CI surgery, revealing inequalities in terms of geographical disparities. Social media has proved to be a valuable, fast and inexpensive tool for gathering information on the effectiveness of health prevention programs, involving a large sample of individuals in a short amount of time.

A Peculiar Case of Ossicular Chain Fixation and Enlarged Vestibular Aqueduct.

We present the case of a patient treated as affected by conductive hearing loss due to recurrent otitis, then as a juvenile otosclerosis, who was finally diagnosed as affected by bilateral ossicular chain fixation and enlarged vestibular aqueduct by means of cone-beam CT.

Improving Auditory Perception in Pediatric Single-Sided Deafness: Use of Cochlear Implants' Direct Connection for Remote Speech Perception Rehabilitation.

PURPOSE: Unilateral cochlear implants (CIs) can restore binaural hearing in single-sided deafness (SSD). Direct stimulation of the CI can be obtained via the Bluetooth connection of a device to the CI, thus excluding the typical-hearing ear. This study aims to investigate the use of CI connectivity features as a rehabilitative strategy in a small cohort of children affected by SSD. METHOD: A newly developed training protocol was presented to children with SSD who have less than 2 years of experience with CI. We developed a user-friendly and short-term (six sessions within 20 days) training protocol for speech perception that patients themselves can manage independently at home using CI connectivity systems. Children were telematically tested before and after the training by trained speech and language therapists. RESULTS: Four patients met the inclusion criteria. They all showed better results in the final online assessment, when compared with the initial one. CONCLUSIONS: To our knowledge, this is the first clinical focus article presenting an auditory training protocol for SSD patients with CI using the Bluetooth connectivity systems for teletherapy. The results, although preliminary, support the efficacy of this rehabilitative strategy. Further research is needed to support our findings.

Application of Patient Reported Outcome Measures in Cochlear Implant Patients: Implications for the Design of Specific Rehabilitation Programs.

INTRODUCTION: Cochlear implants (CI) have been developed to enable satisfying verbal communication, while music perception has remained in the background in both the research and technological development, thus making CI users dissatisfied by the experience of listening to music. Indications for clinicians to test and train music abilities are at a preliminary stage compared to the existing and well-established hearing and speech rehabilitation programs. The main aim of the present study was to test the utility of the application of two different patient reporting outcome (PRO) measures in a group of CI users. A secondary objective was to identify items capable of driving the indication and design specific music rehabilitation programs for CI patients. MATERIALS AND METHODS: A consecutive series of 73 CI patients referred to the Audiology Unit, University of Padova, was enrolled from November 2021 to May 2022 and evaluated with the audiological battery test and PRO measures: Musica e Qualità della Vita (MUSQUAV) and Nijmegen Cochlear Implant Questionnaire (NCIQ) Italian version. RESULTS: The reliability analysis showed good consistency between the different PRO measures (Cronbach's alpha = 0.873). After accounting for the epidemiological and clinical variables, the PRO measures showed a correlation with audiological outcomes in only one case (rho = -0.304; adj. p = 0.039) for NCIQ-T with the CI-pure tone average. A willingness for musical rehabilitation was present in 63% of patients (Rehab Factor, mean value of 0.791 ± 0.675). CONCLUSIONS: We support the role of the application of MUSQUAV and NCIQ to improve the clinical and audiological evaluation of CI patients. Moreover, we proposed a derivative item, called the rehab factor, which could be used in clinical practice and future studies to clarify the indication and priority of specific music rehabilitation programs.

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.