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

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

Role of Immediate Pre-Operative Tympanometry in Cochlear Implantation: MERF Protocol and Experience.

(1) To study the association between an immediate pre-operative tympanometric profile in patients undergoing cochlear implantation with their intraoperative findings. (2) To analyse the intraoperative middle ear findings that require a staged cochlear implantation in patients presenting with a B-type tympanogram. (3) To study the complications in this group of patients during the 1-year follow-up. This retrospective non-interventional cohort study is done over a period of 6 years. Bilaterally profound deaf children, less than 6 years of age, and no history of otitis media with effusion were included in the study. Children who met the inclusion criteria were divided into 4 groups based on their tympanometric profiles that are A, As, B, and C type tympanogram and, their intraoperative findings were categorized as normal, mild oedema, minimal granulation with mild oedema, moderate to extensive granulation with or without oedematous mucosa and glue. Then finally, depending on the intraoperative middle ear and mastoid finding, a single-stage surgery or a two stage surgery was decided upon. A total of 1025 patients were implanted during the study period, 975 patients met our inclusion criteria. In our series, we found a statistically significant difference (p < 0.0001) between the tympanograms and their respective intra-operative middle ear findings. A statistically significant difference was seen (p < 0.0001) between patients who underwent a single-stage cochlear implant and those who underwent a two-staged surgery, regarding their intraoperative middle ear findings. No statistical significance was seen in the occurrence of complications between the groups undergoing a single stage and a two-staged surgery (p > 0.5). This study showcases the importance of immediate pre-operative tympanometry in cochlear implant surgeries. Two-stage surgery is a decision taken on the operating table, depending on the extent of pathology and visibility of the round window niche.

Primary Secretory Paraganglioma of Facial Nerve Canal: A Case Report.

Paragangliomas (PGLs) of Head and Neck region account for 0.6% of Head and Neck Tumours. These may originate in paraganglionic tissues in the area of carotid bifurcation, vagus nerve, tympanic plexus and very rarely along vertical Facial nerve canal (FNC). We intend to describe a rare case of primary paraganglioma of FNC associated with hypoxia of submarine environment, its characterization and multidisciplinary approach towards its management.

Satellite habilitation centres following cochlear implantation - Are they the way ahead in improving outcomes in developing countries?

INTRODUCTION: Cochlear implantation is a safe surgery for restoration of hearing in profoundly deaf children. Following cochlear implantation, children undergo rehabilitation (or 'habilitation' for those without previous hearing). The device is programmed after the surgery, so that the user can hear sounds through it and through rehabilitation training, the heard sounds are made to understand. OBJECTIVE: Our study was aimed at analysing the role of satellite habilitation centres following cochlear implantation by analysing the outcomes following habilitation and comparing it with the outcomes of the main centre and correlating it with the percentage of attendance of classes. Our study also aims to compare the attendance of implant patients from outside the geographical area of the main centre before and after starting the satellite centre. MATERIALS AND METHODS: 1004 profoundly deaf children (6 years and below) who had undergone cochlear implantation and completed 12months of habilitation in our institution from July 2013 to December 2019 were retrospectively analysed. The outcomes of all the centres were assessed by comparing the baseline CAP with CAP scores at 12 months and baseline SIR with SIR scores at 12 months. The outcomes of the main centre and satellite centres were also compared. The outcomes were correlated with percentage of attendance of classes. OBSERVATION: The overall attendance in all the centres was between 75 and 80%. Both main and satellite centres showed statistically significant good outcomes and this correlates with percentage of attendance. CONCLUSION: Satellite centres for habilitation across the state has greatly helped to improve the attendance of these patients and outcomes. Reduced drop-out rates and improved speech language outcomes can be achieved by starting satellite centres for habilitation post cochlear implantation in developing countries like India.

Comparison of Clinician Versus Parental Perspectives of Outcomes in Cochlear Implantees: A South Indian Experience.

Outcomes of cochlear implantation (CI) are generically assessed using standard validated measures like CAP, SIR, MAIS and MUSS scales. Although this reflects the improvement in auditory verbal skills among the implantees with habilitation over one year, the overall perception of their skill development may vary between the parents of these children and the clinicians who provide the habilitation. This study aimed to compare the CAP and SIR scores sequentially over habilitation and further analyzes the correlation between clinician assessment (with CAP/SIR scores) and parental perspective (with MAIS/MUSS scores), at the end of one year of habilitation. 388 children aged 1-6 years who underwent unilateral CI were included in the study. Their baseline CAP and SIR scores were recorded post implantation. All children received 1 year of intensive auditory verbal therapy and their 12 month CAP, SIR, MAIS and MUSS scores were then recorded. The baseline CAP/SIR scores were compared with 12 month CAP/SIR scores and then their 12 month CAP/SIR scores were correlated with 12 month MAIS/MUSS scores respectively. There was significant difference between baseline and the 12 month CAP/SIR scores (p < 0.001). There was strong positive correlation between CAP and SIR scores after 12 months of habilitation (r = 0.7), while there was moderate positive correlation between CAP and MAIS scores (r = 0.59) and between SIR and MUSS scores (r = 0.49) respectively. Though the parents note significant improvement in child's communication abilities, the parental perspective of final outcomes does not always match with the clinician's assessments at the end of habilitation, as highlighted by the moderate correlations. A more precise method of holistic assessment is lacking currently and stands warranted.

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

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

Quality of life assessment in paediatric cochlear implant recipients in South India: Parental assessment and correlation with rehabilitation scores using a bilingual quality of life assessment questionnaire.

Objectives: To assess the quality of life (QoL) of prelingually deaf paediatric population after Cochlear implantation (CI), using a bilingual questionnaire and to correlate with rehabilitation scores; to validate the questionnaire and recommend its use in future studies.Methods: This was a questionnaire based cross-sectional study. Parents of 151 paediatric prelingual CI recipients filled the questionnaire using a 5-point rating scale, with a score of 1-5. These scores were correlated with the CAP and SIR scores.Results and discussion: There was a positive correlation of QoL score with rehabilitation outcomes (r = 0.4638 and P ≤ 0.00001 for CAP; r = 0.3563 and P ≤ 0.00001 for SIR scores). The maximum scores (≥4.0) were given by parents for integration into educational system (4.86), response to environmental sounds (4.12) and overall parental satisfaction about child's performance (4.09). Minimum scores were given for ability to speak in sentences (2.9) and response to verbal sounds at a distance (3.23).Conclusion: The QoL of these children as assessed by the institutional questionnaire have correlated with the rehabilitation outcomes. These QoL results have a positive impact in counselling for early CI as well as for rehabilitation.

A Study of Outcome of Pediatric Cochlear Implantation in Patients with Cochleovestibular Nerve Deficiency.

OBJECTIVES: A cochleovestibular nerve deficiency (CVND) could compromise stimulation of nerve by electrical pulses delivered from a cochlear implant, thereby hindering activity along auditory pathway. The evaluation of children with congenital hearing loss with a high-resolution magnetic resonance imaging is presently the investigative modality of choice to diagnose CVND. The aim of this study was to determine the outcomes in pediatric cochlear implant recipients with a diagnosis of CVND. The objectives included (1) to study the prevalence of CVND among children with prelingual congenital severe to profound hearing loss; (2) to assess post cochlear implantation (CI) outcomes in children with CVND using categories of auditory performance (CAP), speech intelligibility rating (SIR), and cortical auditory evoked potentials (CAEPs); and (3) to propose a management protocol for these children. MATERIALS AND METHODS: All CI procedures performed during the study period in children 5 years or younger were included in study. All patients who were older than 5 years or had syndromic associations, multiple disabilities, second side or revision CI were excluded from the study. Children with unilateral cochleovestibular nerve aplasia and all other cases of CVND (type IIa and IIb) were advised to undergo CI on side with more radiologically robust nerve and/or cochlea anatomy. Children with bilateral CVND were included in group A, and age-matched cochlear implant candidates with normal cochleovestibular nerve anatomy were included in group B for statistical comparison of outcomes. RESULTS: In group A, post CI CAP and SIR, CAEP amplitude and latency at 12 months showed statistically significant difference (p<0.05) compared with preoperative values. However, mean score of CAEP latency and amplitude and SIR score was worse for group A compared with group B at 12 months, which was statistically significant (p<0.05). CONCLUSION: This study supports the fact that CI is a viable option to be offered in children with CVND (type IIa and IIb) for the development of auditory perception and speech.

A Retrospective Study of Cochlear Re-Implantations - Experience from a Large Centre in India.

OBJECTIVE: Cochlear re-implantation (CRI) is becoming increasingly common throughout the world. However, studies regarding CRI incidence and etiology are lacking from developing countries like ours. The aim of this study was to present the Indian experience with CRI based on our experience. Objectives were to determine the incidence and the indications of CRI and the cumulative survival rate (CSR) of cochlear implantation (CI). METHODS: Our study was a retrospective one, conducted at a tertiary care centre in southern India. 1,500 consecutive cochlear implanted ears from 1997 to 2016 were studied. All patients who underwent CRI during this period were included in the study. RESULTS: There were a total of 53 ears (31 male and 22 female ears) who underwent CRI. This gives an incidence of 3.53%. The most common indication of CRI was device failure in 39 ears contributing to 73.6% of the total CRI. The overall CSR of CI in pediatric population was 96.5% over a 20-year period. CONCLUSION: The CRI incidence and etiology at our centre appears to reflect the findings of the literature. Cochlear implant centres across the world should report the CSR of devices used at their respective centres so that it can be made an important criterion in choice of implant.

Outcomes of Cochlear Implantation in Auditory Neuropathy Spectrum Disorder and the Role of Cortical Auditory Evoked Potentials in Benefit Evaluation.

OBJECTIVE: To compare the outcomes of cochlear implantation (CI) in children with auditory neuropathy spectrum disorder (ANSD) and age-matched controls with profound sensorineural hearing loss, using categories of auditory performance (CAP), speech intelligibility rate (SIR), meaningful auditory integration scale (MAIS), and meaningful use of speech scale (MUSS), and to determine the role of Cortical Auditory Evoked Potentials (CAEP) in benefit evaluation after CI. METHODS: Ten patients (8 males and two females) with ANSD who underwent CI were included in the study. Auditory and speech scores were compared between baseline and after 12 months of habilitation in children with ANSD. Post CI speech scores in children with ANSD were compared with the control group (age-matched children with profound sensorineural hearing loss) at 12 months of habilitation. P1 latency of CAEP has a good correlation with auditory and speech scores in children with ANSD in the study group. RESULTS: Significant benefits were seen in children with ANSD who underwent CI compared to the baseline CAP and SIR scores and one year after habilitation. There is no statistically significant difference in outcomes between the two groups with CI (ANSD and profound sensorineural hearing loss) (p-value: CAP=1.00, SIR=0.84, MAIS=0.33, MUSS=0.08). Speech perception in noise test (SPIN) scores in children with ANSD were 63% and 80% with 0 dB signal noise ratio (SNR) and +10dB SNR, respectively. P1 wave of CAEP has a good correlation with the subjective outcomes. CONCLUSION: CI in children with ANSD has showed benefits comparable to children with profound sensorineural hearing loss. CAEP is a useful tool in objectively assessing cortical maturity in children with ANSD following CI.

Syndromic deafness-prevalence, distribution and hearing management protocol in Indian scenario.

BACKGROUND: The estimated prevalence of Sensory Neural Hearing Loss (SNHL) in patients less than 18 years of age is 6 per 1000. Roughly 50% of cases of congenital SNHL can be linked to a genetic cause, with approximately 30% being syndromic and the remaining 70% being non-syndromic. The term "syndromic" implies the presence of other distinctive clinical features in addition to hearing loss. The aim of our study was to find the distribution of various Syndromic associations in patients with profound deafness, presented at Madras ENT Research foundation, Chennai and to formulate a management protocol for these patients and to discuss in detail about the clinical features of commonly encountered syndromic deafness. MATERIALS AND METHODS: Our retrospective study was aimed at describing the various Syndromic associations seen in patients with congenital profound deafness. Information was collected from the medical records. At our centre all patients undergo a comprehensive evaluation. The distribution, etiological factors and management protocol for various syndromes are here presented. RESULTS: Out of 700 patients with congenital profound deafness all patients with Syndromic associations (n = 35) were studied. 5% of profoundly deaf candidates were found to be syndromic. Most common syndrome in our series was found to be congenital rubella syndrome followed by Jervell and Lange-Nielsen syndrome. CONCLUSION: Congenital deafness is an associated feature of many syndromes. Detailed history taking with comprehensive evaluation is mandatory to rule out the associated syndromes. Diagnosis must be confirm by a genetic study. Multidisciplinary approach is essential for appropriate diagnosis and management.

Comparison of treatment modalities in syndromic children with obstructive sleep apnea--a randomized cohort study.

INTRODUCTION: Obstructive Sleep Apnea (OSA) is a common medical problem in adults that is becoming increasingly recognized in children. It occurs in the pediatric age group, from newborns to teens. More recently, many specialists have estimated OSA prevalence to be between 5 and 6%. However, in syndromic children, the prevalence of OSA can be from 50 to 100%, having a significant effect on their Quality-of-Life. As they are a challenging population for management, it is essential to evaluate them thoroughly before planning appropriate intervention. OBJECTIVE: To compare the efficacy of Adenotonsillectomy (T&A) and Continuous Positive Airway Pressure (CPAP) in syndromic children [Down syndrome (DS) and Mucopolysaccharidoses (MPS)] with Obstructive Sleep Apnea (OSA). MATERIALS AND METHODS: In a prospective, randomized, cohort comparative study, 124 syndromic children (DS and MPS) aged between 6 and 12 years were recruited from a private MPS support group and the Down Syndrome Society, Chennai. A standard assessment was performed on all children who entered the study including a full overnight Polysomnogram (PSG), Epworth Sleepiness Scale-Children (ESS-C) and Quality-of-Life (QOL) tool OSA-18. The children with positive PSG who consented for the study (n = 80) were randomly distributed to two groups, T&A group & CPAP group. The children were followed up with repeat PSG, clinical evaluation, ESS-C and Quality-of-Life (QOL) tool OSA-18 for a period of 1 year. OBSERVATION AND RESULTS: Follow-up was available for 73 syndromic children. Both the groups, T&A group and CPAP group, showed statistically significant (p < 0.05) improvement in Apnea-Hypoapnea Index (AHI), ESS-C, QOL from the intervention. In our study, T&A showed equal outcome compared to CPAP. The contrasting feature between the two groups was that CPAP use gave immediate sustained improvement while T&A gave gradual progressive improvement of symptoms over a period of 1 year. CONCLUSION: On average, T&A gives equal outcomes as CPAP and it can be suggested as a first-line treatment in this group of syndromic children.

Randomised comparative study of adenotonsillectomy by conventional and coblation method for children with obstructive sleep apnoea.

INTRODUCTION: Adenotonsillectomy is one of the most common surgical procedures throughout the world for children in otolaryngology. One of the current indications for adenotonsillectomy is adenotonsillar hypertrophy causing Obstructive Sleep Apnoea (OSA). The choice of surgical tools and technique affects the outcome and morbidity due to adenotonsillectomy. AIM OF THE STUDY: To assess the efficacy and safety of coblation adenotonsillectomy as compared to dissection method. To evaluate the morbidity and to study complications associated with each procedure. MATERIALS AND METHODS: This prospective and comparative study of dissection and coblation method of adenotonsillectomy was conducted in our institute, Madras ENT Research Foundation, Chennai over a period of 6 months. 50 cases of children with OSA age group between 5 and 12 years were randomly selected for each group and studied. Duration of surgical procedure, blood loss, post operative pain, post operative reactionary and secondary bleeding was noted and compared. OBSERVATION AND RESULTS: Operative time was more in dissection method compared to coblation technique. Blunt dissection tonsillectomy was associated with greater blood loss than coblation tonsillectomy. Post operative pain was more in dissection method and it was less in coblation technique. Post operative bleeding in both the techniques were found to be minimal. CONCLUSION: We conclude that the use of coblation for adenotonsillectomy may have several advantages over standard methods for the treatment of children with Obstructive Sleep Apnoea. It is highly efficacious, practical and safe with less morbidity and less complications.