INTRODUCTION:Cochlear Nerve Deficiency, particularly Hypoplastic Auditory Nerve (HAN), creates uncertainty regarding Cochlear Implant (CI) outcomes. This study evaluated the utility of Transtympanic Electrically Evoked Auditory Brainstem Response (TT-EABR) in managing HAN cases. METHODS:Twenty-two children with MRI-confirmed HAN were assessed using TT-EABR to guide ear selection for CI vs Auditory Brainstem Implantation (ABI) candidacy. Consequently, 13 children received CIs (HAN -CI group). Their auditory and speech outcomes -measured via CAP, SIR, and MAIS/IT-MAIS at baseline and 12 months post-implantation (with Auditory-Verbal Therapy) -were compared against an age-matched control group of 13 children with normal auditory nerves. RESULTS:The HAN -CI group demonstrated significant improvements from baseline to 12 months in CAP (p=0.001), SIR (p=0.005), and MAIS/IT-MAIS (p=0.001) scores. Remarkably, no statistically significant differences were found between the HAN and control groups for CAP (p=0.93), SIR (p=0.70), and MAIS/IT-MAIS (p=0.97). Favorable outcomes correlated with Kumar-Dutt Type A and B TT-EABR responses, with Type A yielding the best results. DISCUSSION & CONCLUSION:While historical data associates HAN with poor outcomes, this study highlights that precise preoperative TT-EABR assessment and ear selection can lead to outcomes comparable to non-anomalous cases. TT-EABR is a pivotal prognostic tool, crucial for ear selection and managing parental expectations. Preoperative TT-EABR is essential for tailored clinical management and successful CI intervention in selected HAN cases.

IgG4-related disease (IgG4-RD) is a fibroinflammatory condition that often involves the head and neck region. It mimics malignancy, granulomatous diseases and chronic inflammation. This study aimed to characterise the clinical spectrum, diagnostic challenges, histopathological features, treatment strategies, and outcomes of IgG4-RD presenting in the head and neck. It is a retrospective multicentre case series across eight tertiary referral centres across India. Eighteen cases of IgG4-RD involving the head and neck region were retrospectively identified over a five-year period from January 2019 to December 2024. Cases were classified according to the 2020 Revised Comprehensive Diagnostic Criteria for IgG4-RD. Clinical, radiological, histopathological, and treatment data were extracted and analysed descriptively. The cohort comprised nine males and nine females with a mean age of 36.4 years (range 8–68 years). Nine distinct anatomical subsites were represented, with the sinonasal tract and nasopharynx most frequently involved (8 cases, 44.4

INTRODUCTION:Labyrinthitis ossificans is a known complication of bacterial meningitis which results in severe to profound hearing loss. Historically, labyrinthitis ossificans was considered a contraindication for cochlear implantation. However, with advances in technology, cochlear implantation is an accepted treatment for hearing loss in labyrinthitis ossificans. MATERIALS AND METHODS:17 cases with varying degrees of ossification in the cochlea were evaluated and treated at our tertiary referral hospital and cochlear implantation center out of a total of 832 profoundly deaf patients evaluated from January 2007 to June 2023. The etiology, radiology, intra-operative findings and treatment protocols followed in these patients are discussed. RESULTS:15 of the 17 patients could successfully be implanted with a cochlear implant, while two were referred to another center for Auditory Brainstem Implantation. The degree/grade of ossification decided the available cochlear lumen for implantation and hence the surgical approach and choice of the electrode. CONCLUSION:A protocol for the order of choice of electrode and site of insertion in various cases of labyrinthitis ossificans depending on the degree of ossification is proposed. In the order of preference, it would be thus: 1. Cochlear implantation with the active electrode in the scala tympani of the basal turn; 2. Electrode in the scala vestibuli of the basal turn; 3. Anterograde insertion of the implant from the second turn; 4. Retrograde insertion of the implant from the second turn; 5. Insertion of a split array electrode and finally 6. Auditory brainstem implantation.

Objectives: To compare COVID-19-associated pulmonary mucormycosis (CAPM) with COVID-19associated rhino-orbital mucormycosis (CAROM), ascertain factors associated with CAPM among patients with COVID-19, and identify factors associated with 12-week mortality in CAPM. Methods: We performed a retrospective multicentre cohort study. All study participants had COVID-19. We enrolled CAPM, CAROM, and COVID-19 subjects without mucormycosis (controls; age-matched). We collected information on demography, predisposing factors, and details of COVID-19 illness. Univariable analysis was used to compare CAPM and CAROM. We used multivariable logistic regression to evaluate factors associated with CAPM (with hypoxemia during COVID-19 as the primary exposure) and at 12-week mortality. Results: We included 1724 cases (CAPM [n = 122], CAROM [n = 1602]) and 3911 controls. Male sex, renal transplantation, multimorbidity, neutrophil-lymphocyte ratio, intensive care admission, and cumulative glucocorticoid dose for COVID-19 were significantly higher in CAPM than in CAROM. On multivariable analysis, COVID-19-related hypoxemia (aOR, 2.384; 95% CI, 1.209-4.70 0), male sex, rural residence, diabetes mellitus, serum C-reactive protein, glucocorticoid, and zinc use during COVID-19 were independently associated with CAPM. CAPM reported a higher 12-week mortality than CAROM (56 of the 107 [52.3%] vs. 413 of the 1356 [30.5%]; p = 0.0001). Hypoxemia during COVID-19 (aOR [95% CI], 3.70 [1.34 -10.25]) and Aspergillus co-infection (aOR [95% CI], 5.40 [1.23-23.64]) were independently associated with mortality in CAPM, whereas surgery was associated with better survival. Discussion: CAPM is a distinct entity with a higher mortality than CAROM. Hypoxemia during COVID-19 illness is associated with CAPM. COVID-19 hypoxemia and Aspergillus co-infection were associated with higher mortality in CAPM. Valliappan Muthu, Clin Microbiol Infect 2024;30:368 (c) 2023 European Society of Clinical Microbiology and Infectious Diseases. Published by Elsevier Ltd. All rights reserved.

Dear Editor, A large number of mucormycosis cases were reported in India following the second wave of COVID-19.[1] However, data on the imaging appearance of COVID-19-associated pulmonary mucormycosis (CAPM) are limited.[2] A few published case reports on CAPM and a systematic literature review suggested cavity and consolidation as the most common finding.[3-6] The primary objective of the current study was to describe the computed tomography (CT) thorax findings of CAPM at diagnosis. The secondary objectives were to compare the CT findings of (1) CAPM in patients with diabetes mellitus (DM) and those with no known traditional risk factors, and (2) survivors and non-survivors with CAPM. The MuCovi-2 study was a multicentre retrospective study conducted across 25 centres in India between 1 January 2021 and 30 June 2021. Data on COVID-19-associated mucormycosis (microbiologically or pathologically confirmed) cases and age-matched controls were collected in the original study.[7] COVID-19 was diagnosed by detecting SARS-CoV2 infection in nasopharyngeal or throat swabs by rapid antigen testing or reverse transcriptase polymerase chain reaction. Only subjects diagnosed with proven and probable CAPM were included in the present study, as defined previously.[2] The diagnosis and management of CAPM patients were per the prevalent guidelines.[2,8] The clinical presentation, risk factors, and outcome of the entire cohort of CAPM patients have been published recently.[9] For the current study, we excluded CAPM patients with inadequate CT data. We noted (as yes/no) the radiological findings on CT (cavity, consolidation, reversed halo sign (RHS), bird's nest sign, pneumothorax, mycotic aneurysm, pleural effusion, nodules, and others) at diagnosis of CAPM. Data are presented as numbers (percentage) or mean with standard deviation (SD). We used the Mann–Whitney U and Fisher's exact (or Chi-square) tests to analyse the difference between continuous and categorical variables. We assumed statistical significance as a P value <0.05. We included 84 subjects with CAPM [Table 1]. The participants' mean (SD) age was 53.8 (12.5) years. DM was the most common predisposing factor in 58/84 (69%) subjects. Glucocorticoid treatment for COVID-19 was prescribed in 82.3% (65/79) subjects, and nearly 20% of these subjects received inappropriate glucocorticoid therapy (i.e., in the absence of COVID-19-related hypoxemia). Cavity was the most common finding (56/84; 77.8%). RHS or bird's nest sign were observed in 14/84 (16.7%) subjects. Pleural effusion or >10 lung nodules were reported in 19/84 (22.6%) and 2/84 (2.4%) subjects, respectively. Tracheobronchial mucormycosis was noted in four subjects, one of whom had an isolated tracheal lesion with no other parenchymal or pleural abnormality. Of the 84 subjects, 43 (51.2%) and 41 (48.8%) had proven and probable CAPM, respectively. Bilateral disease was significantly more common in those with probable CAPM (20/41 [48.8%] versus 9/43 [20.9%] among proven cases), and pleural effusion was more frequent in proven CAPM (15/43 [34.9%] versus 4/41 [9.8%] in probable CAPM). None of the other findings were significantly different between proven or probable CAPM. The CT findings were similar in patients with COVID-19 as the only risk factor for CAPM versus those with DM, except for pleural effusion, which was significantly less common in DM [Table 2]. Survival at 6 weeks and 12 weeks was 43/79 (54.4%) and 37/73 (50.7%). We found no significant differences in CT findings among survivors versus non-survivors at 6-weeks [Table 3].Table 1: Baseline characteristics and imaging findings of CAPM (n=84) at diagnosisTable 2: Comparison of CT findings in CAPM patients with and without diabetes mellitusTable 3: Comparison of CT findings between survivors versus non-survivors at 6 weeks with CAPMTimely diagnosis improves survival in pulmonary mucormycosis (PM), a serious illness with a high mortality.[10,11] A CT scan provides one of the earliest clues for diagnosing PM [Figure 1].[12] However, most data on the CT findings of PM are from patients with risk factors such as haematological malignancies and organ transplant recipients.[13,14] Imaging appearances of CAPM are poorly studied. We found cavity (66.7%) the most common finding in CAPM, like in DM.[15,16] However, consolidation was less frequently reported in our study (13%) than in the previous studies (48%).[17] Interestingly, in the current study, consolidation was infrequent (12%), even in diabetic subjects with CAPM. The lesser frequency of consolidation and predominance of the cavities in our study could be related to the duration of illness before the CT scan.[15] The earliest imaging findings of PM include nodules, consolidation, and masses; cavitation occurs later when the lesions undergo central necrosis.[18] Neutropenic patients are evaluated early with chest CT and thus manifest nodules or masses. Contrarily, PM in DM or COVID-19 presents later and cavities are common. The current study participants (CAPM with and without DM) were possibly evaluated late during the illness due to severe COVID-19. Also, consolidation due to PM could have been attributed to COVID-19 or hospital-acquired pneumonia. Although insensitive, other notable findings in our study, like RHS and mycotic aneurysm, may suggest PM in those with predisposing factors (including COVID-19 and uncontrolled DM).Figure 1: Representative images showing a few typical signs of COVID-19-associated pulmonary mucormycosis: (a) consolidation in the right upper lobe along with diffuse ground glass opacities (GGOs; COVID-19 related) and pneumomediastinum, (b) reversed halo sign on the right side with patchy consolidation in bilateral lungs and a few GGOs, (c) cavity with air-fluid level in the right lower lobe and bilateral GGOs and (d) pulmonary artery aneurysm in a lesion in the right lower lobe; pneumothorax is also noted on the right sideOur study has a few limitations. The CT findings were retrospectively retrieved from patients' records, and the imaging was not centrally reviewed. Despite a multicentre study, the number of PM patients was relatively small. Further, the CT protocol, the use of contrast, and the differences in the reporting could have influenced our study results. The chest CT findings may vary depending on the duration of PM, and we do not have this information. We do not have a comparator group (e.g., invasive pulmonary aspergillosis); therefore, the specificity of the imaging findings is unknown. We included only individuals with a confirmed diagnosis of CAPM, and we might have missed cases where microbiological or pathological confirmation was unavailable. Isolated tracheobronchial mucormycosis (also reported in COVID-19) without any parenchymal abnormalities could have been missed in our series.[19] In summary, we found similar imaging findings in CAPM as previously reported in PM complicating DM. Cavity was the most common finding in CAPM patients in India. Prospective studies, including serial imaging performed on a larger number of patients with varying risk factors, are required. Financial support and sponsorship Nil. Conflicts of interest There are no conflicts of interest.

Background: Data on mixed mould infection with COVID-19-associated pulmonary aspergillosis (CAPA) and COVID-19-associated pulmonary mucormycosis (CAPM) are sparse. Objectives: To ascertain the prevalence of co-existent CAPA in CAPM (mixed mould infection) and whether mixed mould infection is associated with early mortality (<= 7 days of diagnosis). Methods: We retrospectively analysed the data collected from 25 centres across India on COVID-19-associated mucormycosis. We included only CAPM and excluded subjects with disseminated or rhino-orbital mucormycosis. We defined co-existent CAPA if a respiratory specimen showed septate hyphae on smear, histopathology or culture grew Aspergillus spp. We also compare the demography, predisposing factors, severity of COVID-19, and management of CAPM patients with and without CAPA. Using a case-control design, we assess whether mixed mould infection (primary exposure) were associated with early mortality in CAPM. Results: We included 105 patients with CAPM. The prevalence of mixed mould infection was 20% (21/105). Patients with mixed mould infection experienced early mortality (9/21 [42.9%] vs. 15/84 [17.9%]; p = 0.02) and poorer survival at 6 weeks (7/21 [33.3] vs. 46/77 [59.7%]; p = 0.03) than CAPM alone. On imaging, consolidation was more commonly encountered with mixed mould infections than CAPM. Co-existent CAPA (odds ratio [95% confidence interval], 19.1 [2.62-139.1]) was independently associated with early mortality in CAPM after adjusting for hypoxemia during COVID-19 and other factors. Conclusion: Coinfection of CAPA and CAPM was not uncommon in our CAPM patients and portends a worse prognosis. Prospective studies from different countries are required to know the impact of mixed mould infection.

Studies from developed countries show the prevalence of permanent childhood hearing loss to be 1 to 2 per thousand children. The estimated number of Ear, Nose, and Throat (ENT) specialists and otologists in India were 7000 and 2000. There is a great need for trained CI surgeons to care for that burden. Currently, only a handful of centres in the country provide CI training. This study aims to put together essential and desirable requisites for a clinical fellowship in CI surgery for ENT surgeons. A questionnaire was prepared and validated by 25 senior CI surgeons in India. Then the sixteen-question questionnaire was prepared and administered to 100 practising CI Surgeons (Group A) and 100 probable CI Fellowship Candidates (Group B). Group B involved surgeons currently pursuing their ENT post-graduation or have completed their postgraduate training and are inclined towards otology and CI surgery in the future. The responses ranged from 1 (Strongly Disagree) to 5 (Strongly Agree) on a Likert Scale. The responses from both groups were analyzed, and statistical analysis was performed using SPSS(Statistical Package for the Social Sciences) software. The results were analysed and tabulated from both groups. The weighted mean response and mean opinion to all the questions were calculated for both groups. Based on the response, "Essential" and "Desirable" criteria are given.

Cochlear Implantation (CI) is a well-accepted treatment for severe-to-profound sensorineural hearing loss, refractory to conventional hearing amplification. Pre-operative Computed Tomography (CT) and Magnetic Resonance Imaging (MRI) play pivotal roles in patient selection to rule out findings that preclude surgery or identify conditions that may impact the surgical procedure. A prospective study was carried out in a tertiary care center over three years, from January 2020 to January 2023. One hundred and ninety (380 ears) patients’ High-Resolution Computed Tomography (HRCT) studies of the temporal bone and MRI scans of the auditory pathways were analyzed. A reporting format was followed which was devised by a team of senior implant surgeons and senior neuro-radiologists. Our study aims to provide a comprehensive radiologic protocol for CI candidacy including normative data for the essential morphometrics in the Indian setting.

Bacterial meningitis is the most common cause of post-natal acquired hearing loss in children. Although cochlear implantation helps in improving the hearing in these patients, the fibrosis and ossification of the cochlear lumen that occurs as a result of bacterial meningitis, limits the chances of successful implantation. In developing countries like India, the reduced awareness, limited resources, and financial constraints warrant judicial use of radiological and audiological tests to increase the rate of successful cochlear implantation. The present paper is a review of the literature and a proposed protocol for follow-up of post-meningitis patients to help clinicians diagnose and hence, intervene early when profound hearing loss occurs. Every patient who has had an episode of bacterial meningitis must be followed up for atleast 2 years for possible hearing loss with frequent audiological and radiological evaluation, as required. Cochlear implantation must be done as early as possible when profound hearing loss is detected.

Objectives To comprehensively analyse the disease presentation and mortality of COVID-associated rhino-orbito-cerebral mucormycosis. Methods A retrospective analysis of the demographics, clinical and radiographic findings was performed. A binary logistic regression analysis was performed to examine the survival of patients with mucormycosis from hypothesised predictors. Results A total of 202 patients were included in this study. Statistical significance was demonstrated in the predilection to the male gender, recent history of SARS-COV-2, history of use of corticosteroid and hyperglycemia in this cohort of CAM. The mortality rate was 18.31%. Advanced age, raised HbA1c and intra-orbital extension were found to be predictors adversely affecting survival. Conclusion Early diagnosis, aggressive surgical therapy, early and appropriate medical therapy can help improve outcomes. Level of evidence Level 4.

We performed a case-control study across 25 hospitals in India for the period of January-June 2021 to evaluate the reasons for an COVID-19-associated mucormycosis (CAM) outbreak. We investigated whether COVID-19 treat-ment practices (glucocorticoids, zinc, tocilizumab, and oth-ers) were associated with CAM. We included 1,733 cases of CAM and 3,911 age-matched COVID-19 controls. We found cumulative glucocorticoid dose (odds ratio [OR] 1.006, 95% CI 1.004-1.007) and zinc supplementation (OR 2.76, 95% CI 2.24-3.40), along with elevated C-reactive protein (OR 1.004, 95% CI 1.002-1.006), host factors (re-nal transplantation [OR 7.58, 95% CI 3.31-17.40], diabetes mellitus [OR 6.72, 95% CI 5.45-8.28], diabetic ketoacidosis during COVID-19 [OR 4.41, 95% CI 2.03-9.60]), and rural residence (OR 2.88, 95% CI 2.12-3.79), significantly as-sociated with CAM. Mortality rate at 12 weeks was 32.2% (473/1,471). We emphasize the judicious use of COVID-19 therapies and optimal glycemic control to prevent CAM.

Schwannomas are benign neoplasms arising from myelinating Schwann cells. Nerve sheath tumours arising from the sympathetic chain are very sporadic, with very few cases reported that presented with Horner’s syndrome. The authors describe its radiological and histological features and surgical management in this case report. A sixty-eight-year-old man presented with throat discomfort and hoarseness of voice for three months. He had a palpable mass on the left side in the submandibular region on neck examination. The patient had left recurrent laryngeal nerve palsy and Horner’s syndrome on general examination. MRI with contrast was performed to see the extent of the tumour. Exploratory cervicotomy was performed; the tumour was seen originating from the lower cervical sympathetic chain posterior to the carotid sheath. Histopathological examination confirmed the diagnosis of cervical sympathetic schwannoma. Cervical sympathetic schwannoma is a benign, slow-growing neoplasm with varied presentation. Presentation with Horner’s syndrome is a rare but reported presentation. Radiological imaging plays a vital role in the identification and surgical management. For symptomatic tumours, exploratory cervicotomy is the preferred approach for its wide exposure. Histopathological examination is confirmatory for the diagnosis.

Benign Paroxysmal Positional Vertigo (BPPV), the most common vestibular disorder characterized by recurrent, brief episodes of vertigo, is attributed to the presence of otoconia in the semicircular canals. Two mechanisms contribute to its cause-canalolithiasis (otoconia freely mobile in the semicircular canal) and cupulolithiasis (otoconia adherent to the cupula). Posterior semicircular canal is the most common canal involved. Although the occurrence of BPPV in lateral and superior semicircular canal is rare, with the advancement in diagnostic techniques, their incidence is being reported in the past few years. Various diagnostic tests and therapeutic maneuvers have been described in the management of BPPV. The present report is a comprehensive review of the tests and maneuvers for BPPV written as a guide intended to help the clinicians in the accurate diagnosis and application of a canal-specific treatment maneuver for BPPV. A simplified algorithmic approach (“The Bangalore BPPV Algorithm”) for the management of BPPV is described.

Auditory Brainstem Implants have been recommended as the gold standard in hearing rehabilitation of Neurofibromatosis Type 2 patients who lose hearing completely in both the ears and the cochlear nerves are not stimulable. Some patients have undergone cochlear implantation, in whom the cochlear nerve was spared during surgery or have undergone stereotactic radiotherapy preserving the function of the cochlear nerve. Here we report a case in whom we chose cochlear implantation prior to any definitive treatment for the tumour itself during the ‘wait and watch’ period. The reasons in favour of this approach have been discussed in this article. Post switch-on the implant is benefitting the patient satisfactorily and she is on regular follow up for monitoring the bilateral tumours.

BACKGROUND:The occurrence of retained ear mould impression material is rare and can lead to complications. The current case report describes one such complication, where the silicone impression material used to take the impression of the ear canal flowed into the middle ear through the pre-existing tympanic membrane perforation. Five days later, the patient presented with worsened hearing and blood-tinged discharge from the ear. Ear microscopy revealed a greenish foreign body in the middle ear.CASE REPORT:The foreign body was removed by tympanotomy and the perforation repaired using a temporalis fascia graft. A hearing aid was prescribed after ensuring that the perforation had healed.CONCLUSION:It is essential that the audiologist perform a basic otological examination before prescribing a hearing aid and preparing an ear mould. A clinical approach algorithm for audiologists, for prior to taking an impression, is suggested.

To develop an accurate protocol for measuring the Cochlear Duct Length (CDL) by using Multi Detector Computerized Tomography (MDCT) imaging of the temporal bones and thereby make the appropriate choice of electrode for cochlear implantation. 79 MED-EL® Cochlear implantees were divided into three cohorts in chronological order of their implantation. CDL was calculated from MDCT images and correlated with the CDL calculated using the existing Jolly’s formula. Results of the CDL measured by unfurling the cochlea correlated well with the existing formula. In addition to CDL measurement, measuring diameter of each turn, especially the apical turn, helped in choosing the appropriate electrode for complete cochlear coverage. Having dedicated radiographers and neuro-radiologists can avoid inter-observer variations in CDL measurements. Measuring the CDL and the diameter of each turn helps in choosing an appropriate electrode thus minimizing intra-operative difficulties and achieving complete safe insertion.

OBJECTIVE:To evaluate the utility of pre-operative transtympanic electrically evoked auditory brainstem responses and post-operative neural response telemetry in auditory neuropathy spectrum disorder patients.METHODS:Four auditory neuropathy spectrum disorder patients who had undergone cochlear implantation and used it for more than one year were studied. All four patients underwent pre-operative transtympanic electrically evoked auditory brainstem response testing, intra-operative and post-operative (at 3, 6 and 12 months after switch-on) neural response telemetry, and out-patient cochlear implant electrically evoked auditory brainstem response testing (at 12 months).RESULTS:Patients with better waveforms on transtympanic electrically evoked auditory brainstem response testing showed superior performance after one year of implant use. Neural response telemetry and electrically evoked auditory brainstem response measures improved in all patients.CONCLUSION:Inferences related to cochlear implantation outcomes can be based on the waveform of transtympanic electrically evoked auditory brainstem responses. Robust transtympanic electrically evoked auditory brainstem responses suggest better performance. Improvements in electrically evoked auditory brainstem responses and neural response telemetry over time indicate that electrical stimulation is favourable in auditory neuropathy spectrum disorder patients. These measures provide an objective way to monitor changes and progress in auditory pathways following cochlear implantation.

Submandibular sialadenitis is a common ailment in otorhinolaryngological and oral surgical practice. Some of the common causes of sialadenitis include sialolithiasis, inspissated mucous plugs, strictures and anatomical ductal variations. Very rarely do foreign bodies cause obstructive sialadenitis. Fish bone as a foreign body is routinely seen, with the most common locations being oropharynx, hypopharynx, oseophagus and tongue. We report an intriguing case of a 40 year old male with sialadenitis of the right submandibular gland due to an intra-ductal fish bone.

Profound hearing loss requiring cochlear implantation and arachnoid cyst requiring placement of Cysto-Peritoneal Shunt (CPS) are two commonly seen entities. However, there are very few published cases of patients requiring both of them. The present report describes the importance of multidisciplinary surgical planning in one such patient.