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.

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.

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.

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.

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.

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.

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.

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.

Giant Parathyroid Adenoma: Are They Different?

Primary hyperparathyroidism is a common endocrinology anomaly which involves head and neck region. The diagnosis and treatment of parathyroid adenoma however still remains a challenge. Giant parathyroid adenomas are rare and involve more surgical expertise. Retrospective study was carried in Departments of ENT and Endocrinology, SMS Medical College and Hospital, Jaipur. Records of patients operated between 2010 and 2019 were evaluated and analysed for size and site of parathyroid adenomas, biochemical parameters, intra-operative findings and postoperative course. Tumours with size > 3 cm were termed as giant parathyroid adenomas and this is a rarity in literature. Records of 79 patients were found to be complete. Out of these 9 had giant parathyroid adenomas: 4 left inferior, 2 right inferior, 1 ectopic location (superior mediastinum) and 1 each in left and right superior glands. The size, symptomatology and biochemical parameters are discussed in light of available literature. Giant parathyroid adenomas are a rare entity and their behaviour are same as of non giant parathyroid adenomas. In the view of literature published on pub Med, this is found to be largest case series on parathyroid Adenoma considering size more than 3 cm.

Anterior and Central Skull Base Fibrous Dysplasia: A 12 Years' Experience.

Fibrous dysplasia, specially of anterior and central skull base region, is a rare disorder. This article discusses about our experience in this pathology. A tertiary care institute based retrospective type study was conducted over a period of 12 years. Demographics, radiology, intraoperative details, pathology and follow up were taken into consideration and the data was analysed. Sixteen patients with complaints of proptosis, diplopia, nasal obstruction and/or facial deformity, underwent endoscopic sinus surgery. Subtotal resection was done in 5 patients. Ethmoid bone involvement was seen in 12 patients. Post operatively, diplopia persisted in one patient and one patient had epistaxis. All patients were followed up for 2-10 years with no other complications reported. Anterior and central skull base involvement is rare in fibrous dysplasia. However, it can be removed effectively by endoscopic approach. Overall safety of patient has more concern rather than complete removal of disease.

Efficacy of Topical High Volume Budesonide Nasal Irrigation in Post FESS Patients of Chronic Rhinosinusitis With or Without Nasal Polyposis.

Chronic rhinosinusitis (CRS) is a chronic inflammatory process of nasal mucosa and paranasal sinuses, lasting more than 12 weeks, without complete resolution of symptoms. CRS is treated medically, followed by Endoscopic sinus surgery (ESS) if necessary, and supplemented by post-operative topical treatment with highly variable clinical outcomes. However, till date there is no consensus on the composition and duration of maximal medical treatment. Despite proven role of topical steroids, the mode of delivery, dose and duration of topical intranasal corticosteroids still remains debatable. Studies found that high volume sinonasal irrigation (> 50 ml) using budesonide is most efficient method but still there is not sufficient data to prove this and results are variable with multiple modifiable factors therefore, this study has been conducted. (1) To determine the difference in mean decrease in Lund-Kennedy endoscopic scores and SNOT-22 scores among post ESS patients with high volume budesonide nasal irrigation nasal cavity and control nasal cavity of chronic rhinosinusitis patients. (2) To determine safety by measuring serum cortisol levels and intra ocular pressure. This is hospital based interventional, randomised, double blind, control trial study. A total of 66 patients of CRS with previous failed medical therapy were included. Same patients nasal cavities were divided into control and case nasal cavities, to avoid demographic bias. All subjects had a baseline SNOT-22 scores (Sino Nasal Outcome Test scores), Lund Kennedy endoscopy score, NCCT PNS score. All patients were undergone ESS procedure. After nasal pack removal, nasal cavities were randomly assigned 1:1 to receive normal saline irrigation (control group) or 1 mg of budesonide irrigation (case group) for transnasal irrigation twice daily for 12 weeks. A total of 66 patients with 132 nasal cavities were included in the study. Out of which 16 were female and 50 were male with mean age 33 year and mean duration of symptoms was 38.19 months. Mean duration of follow up was for 3 months. Mean decrement in SNOT-22 score in control nasal cavity from 52.54(16.309) to 30.06 (18.16) and in endoscopic score from 6.53 (1.33) to 3.93 (1.6) which is statically significant (p value < 0.05) in both scores. Mean decrement in SNOT-22 score in case nasal cavity from 53.73 (15.75) to 21.15 (13.52) and in endoscopic score from 6.74 (1.8) to 2.77 (1.4) which is statically significant (p value < 0.05) in both scores. Decrement in SNOT-22 scores and endoscopy scores in case nasal cavity in comparision to control nasal cavity were compared by student 't' test and found to be statically significant (p value equals to 0.0001). In subjective outcomes 57.57% shows total improvement in control nasal cavities while 72.73% case nasal cavities shows total improvement. Our study shows high volume budesonide irrigation is safe and superior over normal saline irrigation and results were statistically comparable. Still further studies with larger sample size and longer duration of irrigation needed.Based on available evidence, high volume budesonide irrigation is statically safe and superior over normal saline irrigation.

Lateral Skull Base Lesions: Our Experience of 15 Years.

To analyse and report various aspects of lateral skull base surgery by describing the incidence and demographic variables, diagnostic and management challenges, surgical choices along with complications and their management and the long term morbidity and survival outcomes in our experience of 15 years. Retrospective review of complete records of all cases operated for lateral skull base tumors at a tertiary care teaching institution in India between timespan of 15 years from 2003 to 2018 was undertaken. 53 cases were selected and analysed. Those having follow up of less than 6 months were excluded. Outcomes were assessed in terms of incidence of types of tumors, benign or malignant, age and sex variation of the patients, staging status at presentation, status of facial and other lower cranial nerves pre-operatively, surgical techniques with complications if any, recurrences and survival. Of 35 benign tumors, tympanojugular paragangliomas were most common. Average age for these was 53 years with 19 females and 14 males. All were non-functional. Tinnitus and hearing loss were most common presentations. Class B2 and C1 tumors were most commonly encountered and the ITF A approach was most commonly used. Hearing loss and Facial palsy were commonest complication post-operatively. There were 2 recurrences. 18 malignancies were observed. Most were stage IV at presentation. Otorrhea and otalgia were most common presenting symptoms followed by hearing loss. 5 year survival was 55%. Successful treatment of lateral skull base lesions requires a multimodality therapy with team approach. Surgical resection is the primary management choice with variable approaches. ICA status related to the tumor is the most important consideration. Malignancies require more aggressive treatment for obtaining clear margins along with pre/post-op chemoradiation. Good results with acceptable complications can be obtained even with advanced tumors.

Correlation of Preoperative High-resolution Computed Tomography Temporal Bone Findings with Intra-operative Findings in Various Ear Pathologies.

High resolution computed tomography (HRCT) is a tool which provide fine details of temporal bone and its associated pathologies which are of extreme use in making diagnosis, to evaluate extension of disease and most important to plan surgical approach. Aim of the present study was to correlate HRCT findings with operative findings in different ear pathologies. This observational, prospective study enrolled 70 patients of different ear pathologies required surgical intervention. They were subjected to HRCT temporal bone and its findings were correlated with surgical findings. Mean age of the study population was 20.3 ± 12.04 years with M: F = 1.12: 1. HRCT showed specificity and sensitivity of 100% and 92.31% respectively in detecting ossicular erosion. It was 100% sensitive and 98.51% specific in detecting LSCC erosion, 85.71% sensitive and 96.83% specific in detecting facial canal dehiscence, 100% sensitive and 98.11% specific in detecting scutum erosion, 75% sensitive and 96.97% specific to detect tegmen erosion, 100% sensitive and 97.01% specific in detecting sinus plate erosion, 100% sensitive and 95.38% specific in detecting high jugular bulb, sensitivity and specificity both are 100% in detecting labyrinthitis ossificans and 100% sensitive in detecting otosclerotic foci. HRCT findings showed a good association with operative findings in terms of sensitivity and specificity. Thus, HRCT is a acceptable tool to make diagnosis and to plan surgical approach.

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.

Efficacy of endoscopic repair for CSF otorrhoea in children with recurrent meningitis due to incomplete partition type 1.

OBJECTIVE: To review the efficacy of endoscopic management with or without cochlear implantation in a pediatric population presenting with recurrent meningitis due to inner ear incomplete partition type 1 (IP-1). MATERIALS AND METHODS: A multicentric report of 6 patients with a history of recurrent meningitis due to IP-1 was done. Radiological evaluation was performed to confirm the diagnosis. Transcanal endoscopic repair of the defect was performed in 3 cases, 2 cases underwent endoscope-assisted repair followed by cochlear implantation in the contralateral ear later, and 1 patient underwent endoscopic repair along with simultaneous cochlear implantation. RESULTS: Radiology confirmed the diagnosis and identified the site of the leak. A leak was detected in all cases from the stapes footplate. Two patients with unilateral deformity had a CSF-filled cyst protruding through a defect in the stapes footplate, and the rest of the four had a high flow CSF leak from the footplate. Five patients who underwent repair have had no further episodes of meningitis. One patient who received simultaneous implantation and repair developed postoperative meningitis managed successfully with antibiotics. CONCLUSION: Hearing loss due to inner ear deformity can be easily missed as a cause for recurrent meningitis, especially if unilateral. A high index of suspicion, audiological screening and radiology are essential to clinch the diagnosis. In such cases, a pure endoscopic or endoscopic assisted transcanal approach to repair CSF otorrhoea is an effective alternative to more radical procedures like subtotal petrosectomy with obliteration. In patients with bilateral profound hearing loss, simultaneous cochlear implantation and repair of the defect can be performed successfully.

Cochlear Implantation in Goldenhar Syndrome.

Goldenhar syndrome is a rare genetic condition characterized by hemifacial microsomia, mandibular hypoplasia, auricular malformations, and epibulbar dermoids. The syndrome has both sporadic and familial occurrence. Incidence of congenital hearing loss in these patients is 1:1000 in children with a male to female ratio of 3:2. In our case study we report a case of Goldenhar Syndrome who underwent cochlear implantation. The patient had right side microtia, right hemifacial microsomia and right side torticollis, pterygium in her right eye, right hypoplastic thumb and unilateral right side kidney. Radiologically, there was narrow duplicated internal auditory canal on right side with absent right cochlear nerve with normal anatomy on left side and the left side showed malformed facial nerve at tympani segment and second genu. Therefore, the patient was planned for left side cochlear implantation. Intraoperatively, there were malformed ossicles with anomalous facial nerve covering whole of oval window and partially the round window. Thus, a separate cochleostomy was done. Impedance was < 5 Hertz in all electrodes and electrically evoked action potential (ECAP) thresholds were obtained on all electrodes.

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.