Cerebellopontine angle epidermoid with ipsilateral external ear atresia: an embryological association or a coincidence?

The cerebellopontine angle (CPA) is the most common site for intracranial epidermoids. They may have varied presentations viz facial pain, hemifacial spasms, headache and uncommonly features of raised intracranial pressure. Their association with external ear atresia has not been reported in literature to date. We present a very rare case of CPA epidermoid with ipsilateral external ear atresia.

SMS classification of inner ear malformations: our experience with implantation in type II anomalies.

PURPOSE: To study and analyse the radiological and surgical findings of 24 cochlear implantees with SMS type II cochleovestibular malformation and to compare their outcomes in terms of hearing and speech gains pre- and postoperatively. METHODS: Retrospective analysis of cochlear implanted candidates over a period of 8 year 6 months from 3 institutions was undertaken and 24 patients suffering from prelingual profound congenital sensori-neural hearing loss along with the presence of SMS type II cochleovestibular malformation were studied. Preoperative radiology, surgical difficulties and complication, and postoperative hearing and speech outcomes up to a period of 2 years, using IT-MAIS and SIR scores were noted. Statistical comparison pre- and postimplantation was done using Wilcoxon signed rank test and a p value of < 0.05 was considered statistically significant. RESULTS: In 24 cases, we encountered four cases of SMS type IIa malformation with a smaller modiolus, while the rest 20 were type SMS type IIb with partially defective modiolus. Intraoperatively, CSF leak was observed in 11 patients. Complete electrode insertion and good electrical response (NRT) was detected in all. No facial nerve anomaly was encountered. IT-MAIS and SIR scores increased from a preoperative mean of 5.6 and 1.16-34.56 and 3.88 after 2 years postsurgery, respectively and this difference was found to be statistically significant. CONCLUSION: Our experience with SMS Type II malformations show promising and motivating results with less chances of complications. Proper evaluation and surgical planning preoperatively can lead to an uneventful surgery with good outcomes. A simple and uniform classification system of these anomalies is a must for appropriate prognostication and right decision making.

Application of the new SMS system of cochleovestibular anomalies: our experience with nine cases of type III anomaly.

OBJECTIVE: To present and analyse the radiological findings, surgical findings, pre-implantation and post-implantation speech and hearing outcomes of nine children with SMS Type III cochleovestibular malformation. STUDY DESIGN: Retrospective case series of nine children with pre-lingual profound sensorineural hearing loss who underwent cochlear implantation (Jan 2012 to July 2019). These children had been classified as Type III malformation according to the SMS Classification of cochleovestibular anomalies. Facial nerve anomalies, CSF leaks and any other significant surgical finding were noted. Meaningful Auditory Integration Scale (MAIS) was used to report the child's pre-operative and two-year post-implantation auditory and speech abilities. Any significant improvement was assessed using the Wilcoxon signed rank test. P value < 0.05 was considered significant. RESULTS: Out of nine patients, five patients sustained CSF gushers, while three patients had mild CSF leak, which were plugged adequately. No facial nerve anomalies were encountered. Post-op course was uneventful for all nine patients. MAIS scores at a two-year follow-up showed significant statistical improvement (P < 0.05) when compared to pre-operative scores. CONCLUSION: Cochlear implant is the treatment of choice for children with type III cochleovestibular malformation. There are significant auditory and speech improvements expected. However, the surgeon should bear in mind the risk of CSF leak and subsequent meningitis.

Soft tissue reactions following cochlear implantation.

INTRODUCTION: Cochlear implantation is a boon to children with hearing loss. Rarely, it can be associated with complications. Soft tissue reaction can be a particularly distressing complication. MATERIALS AND METHODS: The study was conducted on all patients presenting with soft tissue reaction post-cochlear implant in a tertiary care referral institute from March 2011 to June 2018. We graded the severity of soft tissue reaction as per the severity and then managed these patients accordingly. RESULTS: Thirty-five patients were included in this study. Grade 1 had 12 patients, grade 2 had 9, grade 3 had 4 and grade 4 had 9 patients. Grade 1, 2 and 3 reactions were managed conservatively, while grade 4 required surgery. The incidence of explantations increased with the grade severity. CONCLUSION: Soft tissue reaction post-cochlear implant is a rare, but distressing complication. Grading and analyzing them can help us manage them in a better way.

Measuring cochlear duct length in Asian population: worth giving a thought!

INTRODUCTION: The anatomy of the cochlea forms the basis for a successful cochlear implantation. Cochlear duct length (CDL) is defined as the length of the scala media as measured from the middle of the round window to helicotrema. Preoperative measurement of CDL is particularly important when precise intracochlear electrode array placement is desired. It can be done both histologically and radiologically. Preoperative high-resolution computed tomography (HRCT) scan which forms an integral part of cochlear implant workup is a useful tool to calculate CDL using 3D reconstructions. METHOD: This study was done in SMS Medical College and Hospital, Jaipur, India, which is a tertiary care hospital and referral centre for cochlear implants. HRCT temporal bones of all children less than 6 years of age, with congenital bilateral severe-to-profound SNHL who were being worked up for cochlear implant were studied and analysed. 124 patients (56 females and 68 males) with hearing loss were evaluated for cochlear implantation. HRCT temporal bone of these patients was analysed and a variable A was measured which is defined as the linear measurement from the round window to the farthest point on the opposite wall of the cochlea on a reformatted CT scan slice. RESULTS: Mean of distance A for right ear of these patients was 8.10 mm (range 7.7-9.2 mm). Mean for the same in left ear of these patients was 8.14 mm (range 7.7-9.0 mm), giving an overall average of 8.12 mm. Using the formula, CDL = 4.16A-3.98, we calculated the length of cochlear duct. Mean cochlear duct length was 29.8 mm with a range from 28 to 34.3 mm. CONCLUSION: To the best of our knowledge, this is the first large sample study of cochlear length in population of this part of the world. A smaller cochlear length in this part of the world as compared to the Caucasian cochlear duct is a significant finding in understanding of the cochlear anatomy and physiology. It would also have great implications on the insertion depth in cochlear implantation.

Removal of an Unusual Neglected Foreign Body in Infratemporal Region Using Navigation.

A 19-year-old male presented with complaint of a bluish mass in the hard palate since 3 months. The patient had a history of trauma 8 years back in the left zygomatic area with a pen. It was lodged in the wound and removed at that time. Computed tomography scan was revealed a linear heterogenous dense structure extending from left infratemporal fossa to oral cavity, traversing through left maxillary sinus, with bone defect seen in lateral and medial wall of maxilla, and in the hard palate, most likely a neglected foreign body. The foreign body was removed by navigation-assisted endoscopic surgery and the palatal perforation repaired using local rotation flap. There were no intraoperative or postoperative complications. Navigation-guided removal of foreign body in proximity to vital structures, in the infratemporal region, is a valuable option with minimal morbidity.

Cochlear Implantation in a Case of Relapsing Polychondritis With Profound Hearing Loss and Labyrinthine Ossification.

Relapsing polychondritis is an autoimmune disorder causing inflammation of cartilaginous structures, sensory epithelium, and cardiovascular system. Hearing loss is a rare and dreadful complication of this pathology. We report a case of relapsing polychondritis in a 38-year-old female who developed gradually progressive bilateral profound hearing loss. She did not have any improvement with medical management. Cochlear implantation was performed to rehabilitate her hearing. As the scala tympani was obliterated, a scala vestibuli insertion was performed. A complete insertion was possible with a compressed electrode, and she had good evoked compound action potential scores. Her categories of auditory performance scores were 6 at the end of one year. Patients with relapsing polychondritis can progress to profound hearing loss in rare cases and should be carefully followed up to identify early labyrinthine ossification. A scala vestibuli insertion can be performed with good outcomes in cases with ossification involving scala tympani. The surgeon should be ready for a middle-turn cochleostomy or a drill-out procedure in patients with advanced ossification.

Round Window Visibility in Cochlear Implantation : Pre-operative Prediction Using Various Radiological Parameters.

Purpose: The round window approach has become the most preferred route for electrode insertion in cochlear implant surgery; however, it is not possible at times due to difficult round window membrane (RWM) visibility. Our study aims to investigate the relationship between preoperative radiological parameters and the surgical visibility of the RWM in Cochlear implant patients. Methodology: A prospective cross-sectional study of 31 patients, age < 6 years, with bilateral severe to profound sensorineural hearing loss was conducted at a tertiary care hospital. The preoperative HRCT temporal bone scan was studied, and the parameters evaluated were facial nerve location, facial recess width, and RWM visibility prediction. All patients were operated on via the posterior tympanotomy. The surgical RWM visibility was done after optimal drilling of the posterior tympanotomy recess. The relationship between the radiological parameters and surgical visibility of RWM was evaluated. Results: The difference in the facial nerve location as per the type of RWM was found to be significant (p value < 0.05). However, the facial recess width was not significantly associated with RWM visibility. The radiological prediction of RWM visibility by tracing the prediction line over RWM was significantly associated with intraoperative RWM visibility. Conclusion: The goal to look for preoperative scans is to predict the ease or difficulty of RWM visibility during surgery. The difficult visualization of the RWM, can result in dire intraoperative consequences. A comprehensive understanding of preoperative radiological parameters, coupled with meticulous surgical planning, is crucial to address these challenges effectively by focusing on enhancing RWM visualization.

Minimally Invasive Parathyroidectomy as the Surgical Management of Single Parathyroid Adenomas: A Tertiary Care Experience.

The aim of our study is to assess the feasibility, safety and adequacy of focused parathyroidectomy for single parathyroid adenoma accurately localized preoperatively by ultrasound neck and Tc99m sestamibi scan. The study was conducted in the department of Otorhinolaryngology of SMS Medical College and Hospital, Jaipur on 116 patients of primary hyperparathyroidism who underwent minimally invasive parathyroidectomy (MIP) for single gland adenoma localized by USG neck and tc99m sestamibi scans. S. Calcium and S. PTH followed up on day 1, 6th week and 6th month. Outcomes were determined on the basis of cure rate, duration of hospitalization, complication rates and disease persistence/recurrence rate. 82 (70.68%) females and 34 (29.31%) males belonging to the age group of 21-67 years (43.02 ± 14) formed the basis of our study. Skeletal (73.27%) and renal (62.93%) were the most common presentations. Accuracy of USG and sestamibi accurately was 93.10% and 96.55% respectively with combined accuracy of 100%. Preoperative serum calcium levels of 10.7-16.03 mg/dl (13.09 ± 2.11) and serum PTH 127-2196 pg/ml (846.57 ± 776.55) were observed. Cure rate of MIP was 99.13%. No permanent complications seen. MIP can be used as the surgical procedure of choice for PHPT caused by single gland adenoma aided by USG and sestamibi scan. The cure rates are equivalent to the bilateral neck exploration (> 95%) with minimal morbidity and complications. Hence, it is a more feasible, safe and effective treatment option.

Primary Hyperparathyroidism in a 21 Year Old Patient of Turner Syndrome : A Rare Case Report.

Turner syndrome is the most common chromosomal anomaly in females. The typical features include short stature, amenorrhoea, short webbed neck, shielded chest and many comorbidities like osteoporosis, cardiac anomalies, diabetes and hypothyroidism. Primary hyperparathyroidism caused by parathyroid adenoma is rarely reported in patients of turner syndrome. The exact cause is not known at present. We report a case of a 21 years old patient of Turner syndrome who had symptoms of renal stones and hypercalcemia. USG neck and sestamibi scans revealed left inferior parathyroid adenoma. Surgical excision of the involved gland was done which led to normalization of S. calcium and PTH levels. Although hyperparathyroidism is extremely rare in patients of Turner syndrome, any symptoms of renal stones, pathological fractures and hypercalcemia should raise the suspicion of parathyroid adenoma. Surgical management should be planned as early as possible.

Effect of Rhinoplasty on Various Angles of Nose on Ideal Position and Alignment in Indian Population.

Study of anthropometric measurements of the nose makes possible a qualitative and quantitative analysis of surgical results. Analysis of pre- and post-operative changes in nasal tip projection, rotation and nasofrontal angle may also emphasize the fact of over correction or under correction of deformed nose to surgeon. Aim of our study to compare preoperative and postoperative nasal tip projection, rotation and nasofrontal angle after rhinoplasty. The present study was a 1.5 years prospective interventional study conducted at SMS Medical College, Jaipur, India from January 2021 to July 2022. Total 51 patients with external nasal deformity were included in our study. Pre-operative, immediate post-operative and 6 months follow-up nasofrontal, projection and nasolabial angle were recorded, compared and analyzed objectively. In our study significant improvements were observed in the nasolabial angle from 92.9 ± 16.4 to 101.6 ± 10.4 and 110.3 ± 9.8 and nasal tip projection from 0.74 ± 0.17 to 0.58 ± 0.11 and 0.52 ± 0.09 immediate post-op and at 6-months follow up respectively. Mean Nasofrontal angle at pre-op, intra-op and post-op 6 months was 132.5 ± 11.9, 135.4 ± 9.6 and 134.8 ± 9.4 and this difference was not found to be statistically significant. We would like to conclude that Indian noses which are predominantly small and shorter, our surgical procedure aimed at making them more prominent aesthetically on the face, so in the present study results were satisfactory in term of anthropometric parameter.

A Descriptive Observational Study of GJB2 and GJB6 Mutations in Familial Autosomal Recessive Non-syndromic Hearing Impairment.

Mutations in the genes, GJB2 and GJB6 play an important role in autosomal recessive, non-syndromic hearing loss. This study is aimed to detect the association of mutations in GJB2 and GJB6 genes in familial autosomal recessive non-syndromic hearing impairment cases. We included 26 families with at least two affected individuals having congenital bilateral, non-syndromic sensorineural hearing loss. Blood samples were drawn, DNA was extracted, and sent for multiplex PCR and Sanger sequencing. Of the 26 families analyzed, GJB2 mutations were detected in 9(34.6%) and GJB6 mutations were not detected in any of the families. GJB2 mutations are a major cause of congenital, non-syndromic hearing loss in this study population. This study also suggests that GJB6 mutations do not contribute to autosomal recessive non-syndromic hearing loss in the Indian population.

A Study of Round Window and its Adjacent Anatomy to Guide the Cochlear Implant Electrode Insertion.

Introduction: Residual hearing preservation has gained attention now which has brought round window membrane into the light, as a port for cochlear implantation. Atraumatic insertion of electrodes can be achieved by study of anatomical variations of round window and its forms which can guide the surgeon. Objective: This study was undertaken to examine the anatomical variations of round window and its adjacent structures and their impact on surgical approach during cochlear implantation. Methods: A series of 40 adult human temporal bones underwent high-resolution CT scanning and were further dissected for microscopic study of the round window. Results: The antero posterior dimensions of RW ranged from 1.22 to 2.51 mm on radiology and on dissection 1.76 mm +/- 0.3 mm. Shape of round window in 72.5% of bones was oval, and in 27.5% bones it was round shaped. As per Saint Thomas hospital classification for Round window visualization we found 82.5% bones had type I RW visualization and 17.5% had type IIa RW visualization. Area of crista fenestra on dissection was ranging from 0.41 to 0.69 mm2. Conclusion: Residual hearing preservation has become a new motto for surgeons. Therefore thorough anatomic knowledge of round window is must for careful insertion, as round window is closely related to the sensitive inner ear structures.

Post Covid-19 Sinonasal Candidiasis: A Crisis Within the Pandemic.

The aim of this study was to find out the association of sinonasal candidiasis and Covid-19 infection. A prospective observational study was conducted at a tertiary care centre from April to September 2021, involving all patients with invasive candidiasis of the paranasal sinuses having a history of Covid-19 infection. A total of 18 patients of covid associated sinonasal candidiasis among the 475 cases of fungal rhinosinusitis were studied. All patients had involvement of nose and sinuses and 2 patients had orbital involvement with no loss of vision, while 3 had intracranial extensions and 1 had pulmonary involvement. Mandible was involved in 1 patient alone, while the maxilla and palate were involved in 5 patients. 15 patients were hypertensive, 12 diabetics and 1 had aplastic anaemia. Cultures showed that 8 patients had C. parapsilosis, 5 had C. albicans, 3 had C. tropicalis and 2 had mixed fungal infections. All patients underwent surgical debridement and antifungal administration. They were followed up for a minimum of 3 months. There was only one mortality (with aplastic anaemia), rest 17 were disease free at the time of writing this article. This is perhaps the first case series of post covid sinonasal candidiasis in the world. Invasive sinonasal candidiasis is a newer sequela of COVID-19 infection. Uncontrolled diabetes and over-zealous use of steroids at the time of Covid-19 are few of the known risk factors. Early surgical intervention and anti-fungal treatment should be sought for management.

COVID Associated Invasive Aspergillosis.

To study the possible association between invasive fungal sinusitis (aspergillosis) and coronavirus disease. An observational study was conducted at a tertiary care centre over 6 months, involving all patients with aspergillosis of the paranasal sinuses suffering from or having a history of COVID-19 infection. 92 patients presented with aspergillosis, all had an association with COVID-19 disease. Maxillary sinus (100%) was the most common sinus affected. Intraorbital extension was seen in 34 cases, while intracranial extension was seen in 5 cases. Diabetes mellitus was present in 75 of 92 cases. All had a history of steroid use during their coronavirus treatment. New manifestations of COVID-19 are appearing over time. The association between coronavirus and aspergillosis of the paranasal sinuses must be given serious consideration. Uncontrolled diabetes and overzealous use of steroids are two main factors aggravating the illness, and both of these must be properly checked.

Comparing accuracy of cochlear measurements on magnetic resonance imaging and computed tomography: A step towards radiation-free cochlear implantation.

Objective: Computed Tomography (CT) and Magnetic Resonance Imaging (MRI) are commonly employed in pre-operative evaluation for cochlear implant surgery. However, with a decrease in the age of implantation, even minor radiation exposure can cause detrimental effects on children over their lifetime. The current study compares different cochlear measurements from CT and MRI scans and evaluates the feasibility of using only an MRI scan for radiological evaluation before cochlear implantation. Methods: A longitudinal observational study was conducted on 94 ears/47 children, employing CT and MRI scans. The CT and MRI scan measurements include, A value, B value, Cochlear duct length (CDL), two-turn cochlear length, alpha and beta angles to look for cochlear orientation. Cochlear nerve diameter was measured using MRI. The values were compared. Results: The mean difference between measurements from CT and MRI scans for A value, B value, CDL, and two-turn cochlear length values was 0.567 ± 0.413 mm, 0.406 ± 0.368 mm, 2.365 ± 1.675 mm, and 2.063 ± 1.477 mm respectively without any significant difference. The alpha and beta angle measures were comparable, with no statistically significant difference. Conclusion: The study suggests that MRI scans can be the only radiological investigation needed with no radiation risk and reduces the cost of cochlear implant program in the paediatric population. There is no significant difference between the measurements obtained from CT and MRI scans. However, observed discrepancies in cochlear measurements across different populations require regionally or race-specific standardized values to ensure accurate diagnosis and precision in cochlear implant surgery. This aspect must be addressed to ensure positive outcomes for patients.

The Effect of Age at Cochlear Implantation on Speech and Auditory Performances in Prelingually Deaf Children.

To understand the effect of age at implantation on speech and auditory performances of 74 prelingually deaf Indian children after using cochlear implants for 3, 6 and 12 months. We also evaluate the causes of late implantation in this population. Seventy four children who underwent cochlear implantation from December 2013 to December 2015 in the Department of Otorhinolaryngology and Head Neck Cancer in SMS Medical College, Jaipur were participated in this study. To compare the efficacy of cochlear implant, candidates are classified into 2 groups according to the age at the time of implantation: 1-4 years and 4.1-7 years. The sample size is 37 in both age groups. Their auditory performance and speech intelligibility were rated using the Revised Categories of Auditory Perception scales, Speech Intelligibility Rating scales and Meaningful Auditory Integration Scale. The evaluations were made before implantation and 3, 6 and 12 months after implantation. The scores when compared in both the groups revealed that the results were comparable and significant after 12 months of follow up while the scores were not significant after 3 and 6 months. The results were statistically significant when baseline is compared with different postoperative stages. The children implanted before the age of 4 years had significantly better auditory and linguistic performances. At least 12 months of audio-verbal rehabilitation and speech and language therapy are required to compare the effects of cochlear implant in any set of children. Our study shows that hearing impaired children who receive cochlear implantation below 4 years of age acquires better auditory ability for developing language skills.

Outcomes of Acoustic and Linguistic Performances Following Cochlear Implantation in Large Vestibular Aqueduct Syndrome (LVAS).

AIM: This study aims to collate the outcomes in acoustic and linguistic performances after cochlear implantation in LVAS. METHODS: In a hospital-based prospective interventional study, seven prelingual children with bilateral profound sensorineural hearing loss (SNHL) with LVAS were recruited. They underwent unilateral cochlear implantation between December 2013 and December 2015 in the Department of Otorhinolaryngology and Head Neck Surgery at a tertiary care center. Outcomes of auditory and speech performances were assessed in the form of revised categories of auditory perception (CAP), infant toddler meaningful auditory integration scale (IT-MAIS) and speech intelligibility rating (SIR) scores preoperatively, at one and two years follow up. RESULTS: The mean age of implantation was 4 years. The median revised CAP, IT-MAIS, and SIR scores after one and two years of follow-up were 6, 27, 3, and 11, 36, 5 respectively. The mean follow-up duration was 21.3 months. CONCLUSION: Pre op median revised CAP, IT-MAIS, and SIR scores of 0, 1, 1 showed improvement to 11, 36, 5 at two years follow up which was statistically significant. Hence, we conclude that good functional outcomes post-operatively advocate the significance of cochlear implantation in LVAS.

CSF Otorhinorrhea Due to Inner Ear Malformation-A Case Report.

Inner ear malformations are an important cause of sensorineural hearing loss ranging from severe to profound and they also contribute to Cerebrospinal fluid leakage and recurrent meningitis. The most severe is the complete labyrinthine aplasia (Michel Deformity), then there is cochlear aplasia, cochlear hypoplasia, incomplete partition of the cochlea, enlarged vestibular acqueduct and cochlear aperture abnormality.