Mucormycosis of the Middle Ear and Neck Post COVID 19.

Mucormycosis is an opportunistic infection caused by fungi of order Mucorales and affect immunosuppressed patients. The cases of mucormycosis have surged during the COVID pandemic, especially in cases requiring steroids and mechanical ventilation. Here we present a case of a 40-year-old diabetic post COVID female patient. She presented with right earache, right facial paralysis (grade four) of two weeks duration and right sided neck swelling for ten days. Ultrasonography and computed tomography revealed multiple abscesses in the right side of the neck. Histopathological examination of tissue from the neck and middle ear confirmed the diagnosis of mucormycosis. This is a rare case of concurrent neck and middle ear mucormycosis in a post COVID patient. Keywords: Coronavirus; COVID 19; middle ear; mucormycosis; neck.

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.

Anatomical Variations, Surgical Difficulties, and Complications Associated with Cochlear Implantation in Different Age Groups of the Pediatric Population of Nepal: A Tertiary Level Hospital-Based Study.

Pediatric cases account for the major proportion of the population for whom cochlear implantation is indicated. This study aims to review the anatomical variations, surgical difficulties, and complications associated with cochlear implantation surgery in different age groups of the pediatric population of Nepal.This study was conducted at Tribhuvan University Teaching Hospital, Nepal. A prospectively set data of cases who underwent cochlear implantation between January 2015 and March 2020 were analyzed for details of surgical procedure, surgical difficulties, and intraoperative and postoperative complications. The anatomical variations encountered during surgery were classified as: developmental anomalies, round window niche variations and acquired abnormalities resulting from inflammation. Intraoperative surgical difficulties were defined based on the operating surgeon's perspective. Complications following cochlear implantation were classified as surgical and nonsurgical or device-related. We used SPSS version 25 for the analysis of our data. Chi-square test and Fisher's exact test were used to analyze the statistical association.The most commonly encountered difficulty was the requirement of an extended posterior tympanotomy approach due to poor visualization of round window niche. There was a statistically significant association of difficult insertion of electrodes with round window niche visibility. The common complications encountered were intraoperative facial nerve exposure, bleeding, electrode-related problems, cerebrospinal fluid gusher, and device failure.Cochlear implantation with an experienced surgeon in pediatric population is a relatively safe procedure. There is no association of the difficulties and complications related to surgery with the different age groups.

External Auditory Canal Cholesteatoma: Clinical and Radiological Features

Abstract Introduction External auditory canal cholesteatoma (EACC) is often misdiagnosed. Objectives To outline the clinical presentation of EACC, and to describe its radiological findings on high-resolution computed tomography (HRCT) of the temporal bone. Methods The clinical records of all patients diagnosed with EACC from April 2017 to March 2020 in a tertiary care center were retrospectively reviewed. The clinical presentation, the findings on the HRCT of the temporal bone, and the treatment provided were analyzed. Results A total of 9 patients, 7 males and 2 females, with a mean age of 30 years, were diagnosed with primary EACC. Six patients presented with otorrhoea, three, with otalgia, three. with hearing loss, and one with facial palsy. Some patients had multiple symptoms. The most common findings on otomicroscopy were destruction of the posterior and inferior canal walls, with cholesteatoma and intact tympanic membrane (six patients). Two patients had aural polyp, and one had a narrow ear canal due to sagging of the posterior canal wall. On HRCT, all nine patients showed soft-tissue density in the external auditory canal with erosion of the canal wall. The disease extended to the mastoid in eight cases, and to the cavity of the middle ear in one. There were three cases of dehiscence of the facial canal. Dehiscence of the dural and sinus plates was observed in two cases each. Eight patients underwent mastoidectomy, and one underwent debridement with canalplasty. Conclusion Review of the clinical and radiological findings is essential to reduce the rate of misdiagnosis.

External Auditory Canal Cholesteatoma: Clinical and Radiological Features.

Introduction External auditory canal cholesteatoma (EACC) is often misdiagnosed. Objectives To outline the clinical presentation of EACC, and to describe its radiological findings on high-resolution computed tomography (HRCT) of the temporal bone. Methods The clinical records of all patients diagnosed with EACC from April 2017 to March 2020 in a tertiary care center were retrospectively reviewed. The clinical presentation, the findings on the HRCT of the temporal bone, and the treatment provided were analyzed. Results A total of 9 patients, 7 males and 2 females, with a mean age of 30 years, were diagnosed with primary EACC. Six patients presented with otorrhoea, three, with otalgia, three. with hearing loss, and one with facial palsy. Some patients had multiple symptoms. The most common findings on otomicroscopy were destruction of the posterior and inferior canal walls, with cholesteatoma and intact tympanic membrane (six patients). Two patients had aural polyp, and one had a narrow ear canal due to sagging of the posterior canal wall. On HRCT, all nine patients showed soft-tissue density in the external auditory canal with erosion of the canal wall. The disease extended to the mastoid in eight cases, and to the cavity of the middle ear in one. There were three cases of dehiscence of the facial canal. Dehiscence of the dural and sinus plates was observed in two cases each. Eight patients underwent mastoidectomy, and one underwent debridement with canalplasty. Conclusion Review of the clinical and radiological findings is essential to reduce the rate of misdiagnosis.

Natural expulsion of a sharp iron nail: Right main bronchus to the alimentary tract: A case report.

A young girl inhaled a pointed iron nail into the right main bronchus. However, both rigid bronchoscopy and oesophagoscopy failed to locate the nail. Surprisingly, it was detected in the abdomen by C-arm and was expelled uneventfully while defecating two days later.

A clinical profile of revision cochlear implant surgery: MERF experience.

Objective: To study the prevalence of revision cochlear implant study in a tertiary care referral center. To assess the various indications and surgical outcomes of revision cochlear implant surgery. Methods: A retrospective chart review of revision cochlear implant surgery done from June 1997 to December 2019. All the surgeries that were done in children of 12 years and below were included. The revision surgeries were either with or without explantation and reimplantation. The causes included were device failures, electrode extrusion/malposition, magnet migration, persistent foreign body reaction, facial twitching, wound infection, and cholesteatoma. Results: A total of 1636 pediatric cochlear implantation surgery were performed during the study period of 22 years. There were 94 (5.7%) revision surgeries done for various indications during this period. Out of them, 67 patients (71.3%) had device failure thus being the commonest indication for revision surgery, followed by infection in 12.8% of the total patients. Among the total revision, 81 (86.2%) patients had explantation and reimplantation of the new device. Conclusion: Indications of revision cochlear implant surgery are manifold that can be either device-related or patient-related. Revision surgery needs a highly skilled and experienced team of surgeons, audiologists, and habilitationists for achieving optimal results.

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.

Subtotal Petrosectomy in Cochlear Implant Surgery: Our Experience.

AIMS: Subtotal petrosectomy (SP) with cochlear implant (CI) is required in certain specific situations in the management of patients who are candidates for cochlear implants. To study and review the indications, surgical issues, and complications of this procedure. MATERIALS AND METHODS: Retrospective review of all patients who underwent subtotal petrosectomy with cochlear implant during the period January 2010-December 2016 at a tertiary care and referral centre. RESULTS: 19 patients underwent 20 subtotal petrosectomy with cochlear implant during this period. One patient had simultaneous bilateral implantation. The indications were previous mastoid cavity in 7 patients, 5 patients had chronic otitis media, inner ear malformations in 3,ossified cochlea in 3 and unfavourable anatomy in 2 patients. Fat or musculoperiosteal flaps were used to obliterate the cavity. Ninety percent of patients underwent single stage surgery and ten percent underwent two stage procedure.Complications were seen in three patients (15%). CONCLUSION: SP helps in isolating the cavity from external environment after removal of disease, improves the exposure and access, reduces risk of infection and cerebrospinal fluid (CSF) leaks and facilitates CI. Meticulous surgical technique will reduce the complications and long term follow up is needed to detect entrapped cholesteatoma. Subtotal petrosectomy with blind sac closure of external ear canal is required in certain specific situations. It is a safe and effective surgery with acceptable rate of complications.

Congenital Cholesteatoma in a Case of Congenital Aural Atresia: A Case Report.

Congenital external canal atresia is one of the congenital ear anomalies that can occur in patients. Similarly, congenital cholesteatoma is also another congenital disease that is often diagnosed in early adulthood. Both the above-mentioned diseases can occur independently but the presence of both these entities is a rare occurrence and needs a high degree of suspicion aided by a computed tomography scan to make the diagnosis. We are presenting a case of a sixteen-year-old patient who presented with unilateral ear anomaly, earache, facial palsy, and postaural swelling and was diagnosed as a right sided congenital aural atresia with congenital cholesteatoma. He was surgically managed with right-sided modified radical mastoidectomy with canaloplasty and closure of mastoid fistula under general anesthesia.

Complications of Acute and Chronic Otitis Media in a Tertiary Referral Center in Nepal.

OBJECTIVE: The aim of this study was to report the incidence of complications in otitis media and to determine the frequencies of various extracranial and intracranial complications at a tertiary care hospital. METHODS: We retrospectively reviewed the clinical records of patients of all ages and genders who were treated in a tertiary care hospital in Nepal from May 2015 to May 2020 for complications of acute and chronic otitis media. The complications were broadly classified as extracranial and intracranial. The details of patient profiles, histories, examination findings, investigations and treatments were reviewed from the charts. RESULTS: The mean age of 164 patients with complications of otitis media was 47.44±18.58 years. There were 79 (48.2%) male and 85 (51.8%) female patients. The overall incidence of otitis media complications was 0.78%. The incidences of the complications from acute otitis media, chronic otitis media without cholesteatoma, and chronic otitis media with cholesteatoma were 0.5%, 0.06% and 5.6%, respectively. Extracranial complications, intracranial complications and combined extracranial and intracranial complications were seen in 80%, 11% and 9% of the patients, respectively. The most common extracranial and intracranial complications were subperiosteal abscesses and brain abscesses, respectively. There was one mortality due to complication. CONCLUSION: The incidences of complications and mortality from otitis media have declined with the availability of suitable antibiotics, improved imaging, and multidisciplinary management. Antibiotic resistance and masking of signs and symptoms, however, could pose challenges in the future.

Outcome of Graft Uptake and Hearing Results between 'U' Flap Technique and Conventional Tympanomeatal Flap Technique for Anterior and Subtotal Tympanic Membrane Perforation.

BACKGROUND: Myringoplasty done for anterior and subtotal perforation needs expertise and practice than conventional underlay myringoplasty. The objective of this study was to compare the graft uptake and postoperative hearing results between 'U' flap and conventional tympanomeatal flap technique in anterior and subtotal tympanic membrane perforation. METHODS: Sixty three patients of age fifteen years and above with chronic otitis media mucosal type with any sized anterior and subtotal perforation of tympanic membrane were randomly allocated for myringoplasty by lottery method. There were thirty one cases in 'U' flap group and thirty two cases in conventional tympanomeatal flap group. Graft uptake and hearing results were assessed after six weeks and results were compared within and between the groups. RESULTS: Graft uptake rate was 90.3%(28/31) in'U' flap group and it was 87.5%(28/32) in conventional tympanomeatal flap group with no statistically significant difference (p= 0.772) between the two groups. The mean pre and post-operative air bone gap in 'U' flap group were 23.63dB±7.77dB; 13.26dB±5.50dB and that in the conventional tympanomeatal flap group were 20.88±9.88dB, 9.42dB±6.70dB respectively. There was no statistically significant difference in hearing results between the two groups (p= 0.504). CONCLUSIONS: The graft uptake rate and hearing results of 'U' flap group were comparable and showed no statistically significant difference to those of conventional tympanomeatal flap group.

A Rare Case Report and Literature Review of External Auditory Canal Cholesteatoma with Circumferential Destruction of Canal Wall Exposing Facial Nerve.

External auditory canal cholesteatoma (EACC) is a rare condition with an estimated incidence of 1.2 per 1000 new otological patients. It is often mistaken with keratosis obturans. We discuss an extensive primary EACC with an aural polyp in a male which was managed by modified radical mastoidectomy.