Audiological practice and COVID-19: recommendations that audiological centers can use to maintain the safety and quality of service-expert opinion.

PURPOSE: Audiology is an essential service for some patient groups and some interventions. This article sets forth experience-based recommendations for how audiological centers can continue to safely and effectively function during COVID-19. METHODS: The recommendations are the result of panel discussion and are based on the clinical experience of the panelists/authors. RESULTS: The recommendations cover which patient groups and which interventions should be treated when and whether this can be performed in the clinic or remotely; how to maintain the safety of workplace via optimizing patient flow within the clinic and the sanitation of rooms and equipment; and overcoming communication challenges that COVID-19 intensifies. CONCLUSION: For essential audiological services to continue under COVID-19, safety measures must be implemented and maintained, and treatment and communication strategies must be adapted to offset communication difficulties due to personal protective equipment (PPE) and social distancing and to bolster patient confidence. In short, it is vital that staff feel safe, that patients either feel the clinic is safe enough to visit or that remote treatment may be an option, and that clinics and patients have a broad agreement on the urgency of any needed service. We hope that these recommendations help clinics effectively accomplish these goals.

Surgical anatomy of lateral recess in paediatric auditory brainstem implant patients and its clinical correlates including grades of flocculus.

INTRODUCTION: Auditory brainstem implant (ABI), a standard technique in treatment of profound sensorineural hearing loss in patients with neurofibromatosis 2, is now being increasingly employed in children with congenital bilateral sensorineural hearing loss, as in Michele's deformity. A detailed knowledge of the relevant surgical anatomy of the lateral recess and its anatomical landmarks including the flocculus, the choroid plexus and the root entry zones of facial-vestibulocochlear and glossopharyngeal-vagus nerve complexes and their anatomical variants is mandatory, as it is the conduit for electrode array placement. The placement of electrode may be eased or impeded by these variations. MATERIALS AND METHODS: Thirty-two children with congenital bilateral hearing loss underwent surgery through retromastoid suboccipital approach for placement of auditory brainstem implant. The preoperative anatomy was reviewed in detail during procedure and again later in the operative videos. RESULTS: The flocculus was classified into four grades based on its anatomy and relations. Among these, grade II (11 children) was the commonest while grade IV (five children) was least common. Choroid plexus was variable in size across grades of flocculus. Difficulty in defining the anatomy was significantly more (p value = 0.003) in the group with higher grade flocculus (grade III and IV) than in lower grade flocculus (grade I and II). CONCLUSION: The flocculus in these patients is classifiable into one of the four grades and the surgical nuances such as difficulty in defining the anatomy for placement of ABI are dependent on the characteristics exhibited by the floccular anatomy and relations.

Translabyrinthine approach to internal auditory meatus: A retrospective study.

BACKGROUND: Ear and the lateral skull base surgery is challenging and yet fascinating for a Neuro-otologist. A thorough knowledge of the complex anatomy is indispensable for the surgeon in order to provide the best possible care. METHODS: The aim of the study was to highlight the present day indications for translabyrinthine approach to IAM from a Neuro-otologist perspective. RESULTS: There were a total of 7 patients who underwent Translabyrinthine approach at our centre. In the present study we have reported cases of Vestibular Schwannoma, Facial nerve schwannoma, Cholesteatoma involving the IAM, Meniere's disease with refractory vertigo which were managed via translabyrinthine approach. We also encountered, probably the first reported case, tuberculoma of the IAM which was clinical suspected to be vestibular schwannoma. CONCLUSION: The article presents different clinical situations where this approach can be suitably utilized and has been dealt with via a retrospective study encountered at our centre.

Spectrum of DNA variants for non-syndromic deafness in a large cohort from multiple continents.

Hearing loss is the most common sensory deficit in humans with causative variants in over 140 genes. With few exceptions, however, the population-specific distribution for many of the identified variants/genes is unclear. Until recently, the extensive genetic and clinical heterogeneity of deafness precluded comprehensive genetic analysis. Here, using a custom capture panel (MiamiOtoGenes), we undertook a targeted sequencing of 180 genes in a multi-ethnic cohort of 342 GJB2 mutation-negative deaf probands from South Africa, Nigeria, Tunisia, Turkey, Iran, India, Guatemala, and the United States (South Florida). We detected causative DNA variants in 25 % of multiplex and 7 % of simplex families. The detection rate varied between 0 and 57 % based on ethnicity, with Guatemala and Iran at the lower and higher end of the spectrum, respectively. We detected causative variants within 27 genes without predominant recurring pathogenic variants. The most commonly implicated genes include MYO15A, SLC26A4, USH2A, MYO7A, MYO6, and TRIOBP. Overall, our study highlights the importance of family history and generation of databases for multiple ethnically discrete populations to improve our ability to detect and accurately interpret genetic variants for pathogenicity.

Towards a Unified Testing Framework for Single-Sided Deafness Studies: A Consensus Paper.

BACKGROUND: While hearing aids for a contralateral routing of signals (CROS-HA) and bone conduction devices have been the traditional treatment for single-sided deafness (SSD) and asymmetric hearing loss (AHL), in recent years, cochlear implants (CIs) have increasingly become a viable treatment choice, particularly in countries where regulatory approval and reimbursement schemes are in place. Part of the reason for this shift is that the CI is the only device capable of restoring bilateral input to the auditory system and hence of possibly reinstating binaural hearing. Although several studies have independently shown that the CI is a safe and effective treatment for SSD and AHL, clinical outcome measures in those studies and across CI centers vary greatly. Only with a consistent use of defined and agreed-upon outcome measures across centers can high-level evidence be generated to assess the safety and efficacy of CIs and alternative treatments in recipients with SSD and AHL. METHODS: This paper presents a comparative study design and minimum outcome measures for the assessment of current treatment options in patients with SSD/AHL. The protocol was developed, discussed, and eventually agreed upon by expert panels that convened at the 2015 APSCI conference in Beijing, China, and at the CI 2016 conference in Toronto, Canada. RESULTS: A longitudinal study design comparing CROS-HA, BCD, and CI treatments is proposed. The recommended outcome measures include (1) speech in noise testing, using the same set of 3 spatial configurations to compare binaural benefits such as summation, squelch, and head shadow across devices; (2) localization testing, using stimuli that rove in both level and spectral content; (3) questionnaires to collect quality of life measures and the frequency of device use; and (4) questionnaires for assessing the impact of tinnitus before and after treatment, if applicable. CONCLUSION: A protocol for the assessment of treatment options and outcomes in recipients with SSD and AHL is presented. The proposed set of minimum outcome measures aims at harmonizing assessment methods across centers and thus at generating a growing body of high-level evidence for those treatment options.

Development of Automated Tool for Electrode Array Insertion and its Study on Intracochlear Pressure.

Cochlear implantation is the most successful approach for people with profound sensorineural hearing loss. Manual insertion of the electrode array may result in damaging the soft tissue structures and basilar membrane. An automated electrode array insertion device is reported to be less traumatic in cochlear implant surgery. OBJECTIVES: The present work develops a simple, reliable, and compact device for automatically inserting the electrode array during cochlear implantation and test the device to observe intracochlear pressure during simulated electrode insertion. METHODS: The device actuates the electrode array by a roller mechanism. For testing the automated device, a straight cochlea having the dimension of the scala tympani and a model electrode is developed using a 3D printer. A pressure sensor is utilized to observe the pressure change at different insertional conditions. RESULTS: The electrode is inserted into a prototype cochlea at different speeds without any pause, and it is noticed that the pressure is increased with the depth of insertion of the electrode irrespective of the speed of electrode insertion. The rate of pressure change is observed to be increased exponentially with the speed of insertion. CONCLUSION: At an insertion speed of 0.15 mm/s, the peak pressure is observed to be 133 Pa, which can be further evaluated in anatomical models for clinical scenarios. LEVEL OF EVIDENCE: N/A Laryngoscope, 134:1388-1395, 2024.

Age dependent vestibulo-ocular reflex gain in video head impulse test among normal Indians.

Determination of the normative data of Vestibulo-Ocular Reflex gain using VHIT of all three semicircular canals (anterior, posterior, horizontal) on both sides in different age groups is of interest. This is an observational study comprised of 10 healthy individuals in each decade from less than 10 years to 80 years of both sexes making a total of 80. The study was done using the equipment SYNAPSYS VHIT ULMER with Software EVOLUTION 3.0. Mean VOR gain of each decade for all the semicircular canals is calculated and they are compared using ANOVA (Analysis of variance). In our study, percentage of patients with overt saccades is nil. Hence, in our study, the occurrence of covert saccades was insignificant as compared to the above studies. Age dependent VOR gain in normal individuals did not have any significance in our study of 80 patients performed by video head impulse test. VOR gain in our study is not affected by age. Normative data for different age groups obtained compared among different age groups showing no significant difference in Mean VOR gain. The normative values of VOR gain can be compared to patients with dizziness thus helping in determining any vestibular loss.

Noise Pollution and Associated Hearing Loss in a Metropolitan City-a Preliminary Report.

To measure the amount of noise produced in busy parts of a metropolitan and also to assess the audiological status of the civilians exposed to such noise. Cross-sectional study for one year between June 2017 and May 2018 was conducted. Noise was measured in four busy parts of an urban city with a digital sound level meter. People involved in various occupations in the busy parts for more than one year within the age range of 15-45 years were included. Maximum noise level recorded was 106.4 dBA in Koyembedu. Average noise was around 70-85 dBA in Chennai. Totally 100 people were subjected to audiological assessment (69 Males; 31 Females). Among them 93% had hearing loss. Hearing loss was almost equal both in sexes. Sensory hearing loss was the major type (83%). All areas were almost equally affected with maximum (100%) being affected in Annanagar and Koyembedu. The right ear was more affected than the left. All age groups were affected among which the working age group (36-45) years was most affected. The unskilled occupation group was most affected (100%). There was a positive relation between noise levels and hearing loss. Duration of exposure did not have positive correlation with hearing loss. Noise pollution and its induced hearing loss was more prevalent and increased in all four areas. As hearing loss due to noise pollution is predominant as observed in the study, awareness about noise pollution and its effects among the community is a necessity.

Complication profile in a cochlear implantation- surgical audit in a large study population of low socio-economic status in a developing country.

OBJECTIVES: To audit surgical complications and their management in cochlear implant (CI) recipients in a tertiary care referral otorhinolaryngology center in South India. MATERIALS AND METHODS: Hospital data on 1,250 CI surgeries performed from June 2013 to December 2020 was reviewed. This is an analytical study with data collected from medical records. The demographic details, complications, management protocols and relevant literature were reviewed. Patients were divided into the following five age groups: 0-3 years, 3-6 years, 6-13 years, 13-18 years and above 18 years. Complications were divided into major and minor and complication occurrence was divided into peri-operative, early post-operative, and late post-operative, and the results were analyzed. RESULTS: The overall major complication rate was 9.04% (including 6.0% due to device failure). If the device failure rate was excluded, the major complication rate was 3.04%. The minor complication rate was 6%. DISCUSSION: CI is the gold standard in the management of patients with severe to profound hearing loss with minimal benefit from conventional hearing aids. Experienced tertiary care CI referral and teaching centers manage complicated implantation cases. Such centers typically audit their surgical complications, providing important reference data for young implant surgeons and newer centers. CONCLUSION: Although not bereft of complications, the list of complications and its prevalence is sufficiently low to warrant the advocacy of CI worldwide, including developing countries with low socio-economic status.

Effectiveness and Safety of Prochlorperazine in Indian Patients with Acute Vertigo: Results from a Large, Prospective, Post-marketing Observational Study.

Aim: To assess the efficacy and safety of prochlorperazine in Indian patients with acute vertigo. Methods: In this prospective, multicenter, open-label, post-marketing observational study, patients with acute peripheral vertigo of different etiologies received 5 mg prochlorperazine thrice a day for 5 days. The primary endpoints were percentage of patients with improvement in (1) vertigo symptoms and (2) clinical response as per scale for vestibular vertigo severity level and clinical response evaluation (SVVSLCRE) from baseline to end of treatment (Day 6). The key secondary endpoints were (1) improvement in nystagmus grading, and (2) safety and tolerability Efficacy of prochlorperazine by route of administration of first prochlorperazine dose (oral or intramuscular) was also assessed. Results: Of 1716 enrolled patients (mean [standard deviation, SD]) age (42.0 [12.95] years; 53.6% men), 57.4% were diagnosed with Meniere's disease, followed by vestibular neuritis (17.4%), labyrinthitis (16.7%), or ear surgery (8.5%). In the overall population, 91.1% of patients showed improvement in clinical response per SVVSLCRE grading at Day 6 (p < 0.0001 vs. non-responders). Nystagmus grading was improved in 99.7% (of patients. No adverse drug reactions events were reported. Tolerability of prochlorperazine was rated as good, very good, and excellent by 43.6%, 32.9% and 20.7% of patients, respectively. Among patients with postoperative vertigo, 80.1% showed improvement in clinical response. In the intramuscular and oral subsets, 85.5% and 92.1% of patients showed improved clinical response, respectively. Conclusion: Prochlorperazine showed improvement in severity of symptoms and clinical response in all subsets of vertigo patients, with a good safety and tolerability profile. Trial Registration Number: CTRI/2022/01/039287. Date of Registration: 10 January 2022.

A Case of Langerhan's Cell Histiocytosis of Temporal Bone.

We present a case of isolated Langerhans' cell histiocytosis in a six year old child who presented with right otorrhoea, retro-aural swelling and hearing loss. The preoperative provisional diagnosis was cholesteatoma, per operative differential diagnosis was rhabdomyosarcoma and final histological and immunohistochemistry diagnosis was Langerhans' cell histiocytosis.

Cochlear Implantation in Biotinidase Enzyme Deficiency.

Metabolic syndromes associated with hearing loss are rare and are characterized by specific enzyme pathway deficiencies involving lysosomal storage, peroxisomes, fatty acid enzymes, organic acids and amino acids. The deficiency of biotinidase, an enzyme involved in the metabolism of biotin, is one such rare cause of congenital hearing loss estimated at 1:60,000 newborns. The parents of a 5-year-old girl presented to the clinic with complaints that she was hard of hearing with no speech development. At age 2 she had been diagnosed with organic aciduria and hydronephrourethrosis and was operated for renal calculi. Clinical examination showed periorificial scaly skin lesions and eczematous otitis externa. An audiological evaluation showed bilateral profound SNHL. Imaging and routine investigations were unremarkable, except for a mild low anion gap metabolic acidosis. General anaesthesia involved avoidance of neuromuscular agents due to the risk of inducing hypotonia. Surgery consisted of cortical mastoidectomy followed by the facial recess approach. A standard electrode array was inserted via the round window technique and complete atraumatic insertion was achieved. Intraoperative electrode impedance and NRT tracings were good. Hearing loss in biotinidase deficiency may be expected to be of progressive nature and regular evaluation of hearing and speech is required. Cochlear implantation is currently the best available solution for severe to profound hearing loss in this disorder although the enzymatic pathology affects the entire auditory pathway. Biotin supplementation is required lifelong for its management.

Worldwide Variation in Cochlear Implant Candidacy.

BACKGROUND: The aim of this study was to find out how candidacy criteria have evolved differently across the globe. METHODS: Candidacy criteria and outcome measurements applied in 19 HEARRING clinics were analyzed. RESULTS: Candidacy criteria vary between clinics. Overall, both bilateral implantation and cochlear implantation in patients with single-sided deafness are becoming more frequent. CONCLUSION: Standardized outcome measurement instruments need to be applied to provide access to the hearing world to all patients with hearing loss who would benefit from cochlear implantation.

Suitable Electrode Choice for Robotic-Assisted Cochlear Implant Surgery: A Systematic Literature Review of Manual Electrode Insertion Adverse Events.

Background and Objective: The cochlear implant (CI) electrode insertion process is a key step in CI surgery. One of the aims of advances in robotic-assisted CI surgery (RACIS) is to realize better cochlear structure preservation and to precisely control insertion. The aim of this literature review is to gain insight into electrode selection for RACIS by acquiring a thorough knowledge of electrode insertion and related complications from classic CI surgery involving a manual electrode insertion process. Methods: A systematic electronic search of the literature was carried out using PubMed, Scopus, Cochrane, and Web of Science to find relevant literature on electrode tip fold over (ETFO), electrode scalar deviation (ESD), and electrode migration (EM) from both pre-shaped and straight electrode types. Results: A total of 82 studies that include 8,603 ears implanted with a CI, i.e., pre-shaped (4,869) and straight electrodes (3,734), were evaluated. The rate of ETFO (25 studies, 2,335 ears), ESD (39 studies, 3,073 ears), and EM (18 studies, 3,195 ears) was determined. An incidence rate (±95% CI) of 5.38% (4.4-6.6%) of ETFO, 28.6% (26.6-30.6%) of ESD, and 0.53% (0.2-1.1%) of EM is associated with pre-shaped electrodes, whereas with straight electrodes it was 0.51% (0.1-1.3%), 11% (9.2-13.0%), and 3.2% (2.5-3.95%), respectively. The differences between the pre-shaped and straight electrode types are highly significant (p < 0.001). Laboratory experiments show evidence that robotic insertions of electrodes are less traumatic than manual insertions. The influence of round window (RW) vs. cochleostomy (Coch) was not assessed. Conclusion: Considering the current electrode designs available and the reported incidence of insertion complications, the use of straight electrodes in RACIS and conventional CI surgery (and manual insertion) appears to be less traumatic to intracochlear structures compared with pre-shaped electrodes. However, EM of straight electrodes should be anticipated. RACIS has the potential to reduce these complications.

A Retrospective Clinical Audit of Outcomes of Cochlear Implantation in Children with Multiple Disabilities in Comparison with Normal Implantees: A South Indian Experience.

AIMS: Cochlear implantation (CI) is established as a standard remedy for children with congenital bilateral profound hearing loss to attain hearing perception and thereby develop speech and language. A subgroup includes children with multiple disabilities in whom the implant helps to improve their quality of life and also of their families via enhanced communication skills. Cochlear implants today form an integral part of their multi-handicap rehabilitation process. MATERIAL AND METHODS: A retrospective cohort study was carried out on children with and without multiple handicaps who have received cochlear implantation at the cochlear implant clinic of MERF, Chennai, India over the past decade. Category of Auditory Performance (CAP) scores, Speech Intelligibility Rating (SIR) scores, and also Meaningful Auditory-Integration Scale (MAIS) and Meaningful Use of Speech Scale (MUSS) scores were compared at set time frequencies of 6 months and 12 months post-implantation between the two groups of implanted children. RESULTS: All the four CAP, SIR, MAIS and MUSS scores showed improvement over time with auditory and speech therapy in both groups of children as reflected by the improvement in their quality of life. The normative group of implantees showed better improvement compared to the group of children with multiple disabilities. CONCLUSION: Intensive habilitation is essential especially for children with multiple disabilities who have received cochlear implantation in which their special needs are addressed individually and optimised for the best outcome. The study shows that restoration of the special sense of hearing helps as a remedy to alleviate their other multi-handicaps to a notable extent.

Posterior semi-circular canal electrode misplacement in Goldenhar's syndrome.

Literature documents the incidence of electrode misplacement within the range of 0.2% to 5.8% with the superior SCC as the most common site, followed by the vestibule. In this report, we present the finding of electrode misplacement in the posterior SCC in a child with Goldenhar syndrome which was subsequently corrected. This child with bilateral congenital profound SNHL presented for unilateral cochlear implant surgery. Intraoperatively, the lateral SCC bulge, stapes, oval window, round window niche and pyramid were noted absent, leading to a surgical decision in favour of a subtotal petrosectomy. Using the aberrant facial nerve and jugular bulb as critical landmarks, a cochleostomy was performed in the posteroinferior aspect of the promontory bulge. Although electrode insertion was smooth and complete, NRT was absent in the presence of normal electrode impedance. A post-operative HRCT scan showed the electrodes in the posterior SCC. Repositioning of the electrodes was carried out by creating a new cochleostomy anteroinferior to the previous one. Complete correct electrode insertion was accomplished via this cochleostomy complimented by robust NRT tracings. Cochlear implant electrode insertion should be supported by correct identification of surgical landmarks. Intraoperative impedance testing and NRT help confirm device integrity and correct placement, the absence of which may raise the suspicion of malposition. In the presence of such a suspicion, the post-operative HRCT scan is a useful retrospective guide to corrective action, in a low-cost setting lacking intra-operative imaging facilities like X-ray, Fluoroscopy, Flat Panel CT & CBCT. Abbreviations, BERA: Brainstem Evoked Response Audiometry; CBCT: Cone Beam Computed Tomography; EAC: External Auditory Canal; ITD: Insertion Test Device; LSCS: Lower Segment Caesarean Section; MDT: Multi-Disciplinary Team; NICU: Neonatal Intensive Care Unit; NRT: Neural Response Telemetry; OAE: Oto-Acoustic Emissions; PIH: Pregnancy Induced Hypertension; SCC: Semi-Circular Canal; SNHL: Sensori-Neural Hearing Loss.

Systematic review of cochlear implantation in adults with asymmetrical hearing loss.

BACKGROUND: Rapid advances in cochlear implantation has witnessed an expanding spectrum for candidacy worldwide. This includes a subgroup of adults with asymmetrical hearing loss who have a wide range in their hearing capacity between the two ears. As per guidelines they are not included in mainstream candidacy for CI across the world. Evidence is now emerging to support the benefits of CI in AHL. METHODS: This review analyzed literature regarding the outcomes of CI in AHL. Primary outcome measure was to assess audiological benefits and secondary outcome measure was to assess hearing related quality of life. 15 relevant articles, published worldwide between 2009 and 2019 were chosen. CASP checklist for systematic reviews was used to ascertain the quality of literature. The strength of recommendations from each study was analyzed and classified as strong, moderate, weak or none based on GRADE guidelines. RESULTS: Heterogeneity in samples was obvious and samples varied largely between the studies. The levels of evidence ranged from systematic review to expert opinion, but overall they reflected positively on both audiological and QOL benefits. CONCLUSION: CI provides important auditory and QOL benefits in AHL, but there is no high level evidence as yet to strongly support CI for AHL. A long term multi-centric study is necessary to influence a change in practice for a growing population of AHL.Trial registration: ClinicalTrials.gov identifier: NCT03052920.

Audiological and Surgical Outcomes of Pediatric Cochlear Implantation in Mondini's Dysplasia: Our Experience.

OBJECTIVES: Aim of present study is to compare audiological and surgical outcomes in prelingual deaf children with Mondini's dysplasia (MD) and those with normal inner ear anatomy. MATERIALS AND METHODS: Retrospective data was collected from Jan 2008 to Dec 2016. Children with bony IEM other than MD, syndromic association, multiple disabilities, those lost to follow up, and perilingual or postlingual deafness were excluded from study. Audiological outcomes for auditory perception (CAP score) and speech intelligibility (SIR score) was noted for a follow up period of 1 year. RESULTS: Mean age at implantation was 2.8 years (Range of 2 to 6 years). 2 patients had intraoperative CSF ooze which was controlled intraoperatively by conservative measures. Post operative facial nerve function was normal in all patients. None of the patient in either group had any complications at one year of follow up period. There was statistically significant improvement in CAP - SIR score in Group A at 6 - 12 months compared to pretreatment. There was no statistically significant difference between the 2 groups in terms of CAP - SIR score at 6 - 12 months. CONCLUSION: The study stresses the fact that cochlear implantation can be safely performed in children with MD although there is a risk of intraoperative CSF leak which can be controlled intraoperatively. Cochlear implantation in children with MD has good surgical, auditory and speech outcomes at par with children with normal bony inner ear anatomy.