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.

Translabyrinthine approach to internal auditory meatus: A retrospective study.

BACKGROUND: Ear and the lateral skull base surgery is challenging and yet fascinating for a Neuro-otologist. A thorough knowledge of the complex anatomy is indispensable for the surgeon in order to provide the best possible care. METHODS: The aim of the study was to highlight the present day indications for translabyrinthine approach to IAM from a Neuro-otologist perspective. RESULTS: There were a total of 7 patients who underwent Translabyrinthine approach at our centre. In the present study we have reported cases of Vestibular Schwannoma, Facial nerve schwannoma, Cholesteatoma involving the IAM, Meniere's disease with refractory vertigo which were managed via translabyrinthine approach. We also encountered, probably the first reported case, tuberculoma of the IAM which was clinical suspected to be vestibular schwannoma. CONCLUSION: The article presents different clinical situations where this approach can be suitably utilized and has been dealt with via a retrospective study encountered at our centre.

Does cerebellar flocculus size affect subjective outcomes in pediatric auditory brainstem implantation.

OBJECTIVES: The objectives of study was to 1) Describe relevant surgical anatomy in defining and accessing the lateral recess for placement of electrode, 2) Propose a working classification for grades of Flocculus; 3) To determine if different grades of cerebellar flocculus effects placement of ABI electrode and subjective outcomes in implantees. METHODS: Our study was a prospective study, and comprised of cohort of 12 patients who underwent ABI surgery via retrosigmoid approach between 1 Jan 2012 to 31 Dec 2014. All children with congenital profound sensorineural hearing loss with either absent cochlea or cochlear nerve were included in the study. Relevant anatomy was noted. We also noted down the difficulty encountered during the placement of ABI electrode. Auditory perception and speech intelligibility was scored post operatively for 1 year. RESULTS: Cerebellar flocculus was divided into 4 grades depending on the morphology of cerebellar flocculus. It was noted that Grade 3 & 4 flocculus (Group B) had difficult ABI electrode placement in comparison to Grade 1 & 2 flocculus (Group A). The subjective outcomes of Group A was better than Group B. However the p value was not statistically significant. CONCLUSION: Cerebellar flocculus can be graded depending on morphology and size. Flocculus of higher grades can make the placement of ABI electrodes difficult and adversely effects the postoperative subjective outcomes.

Genetics: A New Frontier in Otology.

Molecular genetics is a rapidly expanding field with possibilities for novel diagnostic and treatment strategies for otological diseases. Gene therapy, if theory is proven practical, could eliminate disease at the molecular level, thus obviating the need for pharmacologic or surgical treatment. Recent years have seen great advances in our understanding of the molecular genetic basis of many otological disorders. Building on the success of the Human Genome Project, new technologies are in development to identify disease-causing mutations through genetic testing. A basic understanding of the genetic basis of Otological diseases is crucial to the practising Otologist and the time has come for genetic services to be incorporated into regular Otological clinics.

Cochlear Implantation in Isolated Large Vestibular Aqueduct Syndrome: Report of Three Cases and Literature Review

Introduction Large vestibular aqueduct syndrome (LVAS) is characterized by the enlargement of the vestibular aqueduct associated with sensorineural hearing loss. It is the most common radiographically detectable inner ear anomaly in congenital hearing loss. LVAS may occur as an isolated anomaly or in association with other inner ear malformations. Objective To report three cases of isolated LVAS with a focus on preoperative assessment, surgical issues, and short-term postoperative follow-up with preliminary auditory habilitation outcomes. Resumed Report One girl and two boys with LVAS were assessed and cochlear implantation was performed for each. Various ways of intraoperative management of cerebrospinal fluid gusher and postoperative care and outcomes are reported. Conclusion Cochlear implantation in the deaf children with LVAS is feasible and effective.(AU)

Cochlear Implantation in Isolated Large Vestibular Aqueduct Syndrome: Report of Three Cases and Literature Review.

Introduction Large vestibular aqueduct syndrome (LVAS) is characterized by the enlargement of the vestibular aqueduct associated with sensorineural hearing loss. It is the most common radiographically detectable inner ear anomaly in congenital hearing loss. LVAS may occur as an isolated anomaly or in association with other inner ear malformations. Objective To report three cases of isolated LVAS with a focus on preoperative assessment, surgical issues, and short-term postoperative follow-up with preliminary auditory habilitation outcomes. Resumed Report One girl and two boys with LVAS were assessed and cochlear implantation was performed for each. Various ways of intraoperative management of cerebrospinal fluid gusher and postoperative care and outcomes are reported. Conclusion Cochlear implantation in the deaf children with LVAS is feasible and effective.

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.

Long term outcome of cochlear implantation in five children with common cavity deformity.

INTRODUCTION: A common cavity (CC) deformity is a deformed inner ear in which the cochlea and vestibule are confluent forming a common rudimentary cystic cavity. The outcome of cochlear implantation (CI) in this deformity is not expected as good as in other cochlear deformity. Categories of Auditory Performance (CAP) score, Speech Intelligibility Rating (SIR) scale, Meaningful Auditory-Integration Scale (MAIS) and Meaningful Use of Speech Scale (MUSS) are scales designed to assess benefit of functional device (hearing aid, Cochlear implant, ABI) in young children. The aim of the study was to observe and report the long-term audiological progress and speech development of children with CC deformity after CI by means of CAP, SIR, MAIS, MUSS scores and CI aided threshold. METHODS AND MATERIALS: The retrospective and prospective study was carried out in five Indian children with CC deformity who underwent CI from 2004 to 2010. Demographic data of onset and/or duration of deafness, age at implantation, time with hearing aids before implantation, duration with CI and co-morbidity were retrieved from a local data base. Their outcome score in audition and speech development on the basis of their CAP and SIR score at the end of 1 year of habilitation were collected. All the patients were called to follow up to obtain latest CI aided audiogram, CAP, SIR, MAIS and MUSS scores. RESULTS: One male and four female children were detected with common cavity deformity among 258 deaf children of age below 6 years operated during study period. The CAP and SIR Scores increased from average of 0.4 and 1 to 3.4 and 2.4, respectively, over one year of habilitation. The CAP score improved in subsequent follow up to average of 5.0 after more than 3 years of follow up but the SIR score hardly improved. We assessed the MAIS and MUSS at last visit after 3 years. The mean of MAIS and MUSS was 32.4 and 26.4, respectively. CONCLUSION: Though the habilitation outcome was not up to the mark in children with CC deformity, CI provides excellent awareness of environment sound and development of few words.

Public health implications of obstructive sleep apnea burden.

OBJECTIVE: To assess the implications of obstructive sleep apnea (OSA) burden among Indian children. METHODS: MonteCarlo simulations were performed in order to estimate the number of OSA related obesity cases among Indian children (1-14 y of age) and the number of cases of stroke, coronary heart disease (CHD) and type 2 diabetes, considered as main adverse outcomes of OSA related childhood obesity, according to untreated and treated [adenotonsillectomy (AT) alone and AT associated to continuous positive airway pressure (CPAP)] pediatric OSA. Data used to perform MonteCarlo simulations were derived from a review about current literature exploring OSA related obesity. RESULTS: The analysis on the number of adverse outcomes according to treated and untreated obesity related to OSA showed that treatments reduce the number of obesity cases, resulting in a great reduction of the amount of stroke, CHD and type 2 diabetes cases. However, the cost for treating adverse outcome was higher in patients treated for obesity related to OSA compared to those not receiving any treatment. CONCLUSIONS: The reduction in the number of adverse outcomes due to treatment of obesity related OSA implicates the urgent need for public health policies in providing screening for OSA among children population: an early detection and a consequently prompt reaction to pediatric OSA could improve the burden of OSA related obesity.

Effect of intravenous administration of steroids in the management of sudden sensori-neural hearing loss: our experience.

The aim of this study was to investigate the efficacy and outcomes of intravenous high dose steroids in patients diagnosed with sudden sensori-neural hearing loss (SSNHL). The study also looked at the various co-morbidities influencing the outcomes of IV steroid therapy and also evaluated the improvement in associated symptoms like vertigo and tinnitus. This prospective study involved 30 patients treated during the 1 year period from January 2010 to 2011 in the Department of Otolaryngology, Madras ENT Research Foundation, Chennai. Male: female ratio was 1.3:1 and age range was 19-80 years. For all patients, pre treatment pure tone audiometry (PTA) was compared with post treatment PTA at 1 month. Treatment was given in the form of intravenous high dose methyl prednisolone. The patients were divided into two groups. Group 1 (20 pts) included SSNHL with no co-morbidity, group 2 (10 pts) included SSNHL with various co-morbidities. The mean hearing level improved from an average of 79.53 dB (HL) before treatment to 42.33 dB (HL) after treatment. In patients with predominantly low frequency HL (16 pts) PTA improved from 76.01 to 32.6 dB while in high frequency HL PTA improved from 83.55 to 53.43 dB. In our study of 30 patients, complete recovery occurred in 56.66% cases and marked improvement (>30 dB) in 16.66% patients. There was no improvement in 26.66% cases. Patients in group 2 had co-morbid factors like diabetes mellitus, dys-thyroidism and hypertension. A statistically significant improvement in the associated symptoms of tinnitus/vertigo, were also noted after IV steroid treatment. According to our results, emergency administration of high dose of Intra-venous corticosteroids to patients with SSNHL is highly recommended. Patients with high frequency preservation have better hearing improvement at the end of treatment. The critical time period for commencing IV treatment is less than 6 h from onset of hearing loss in order to restore normal hearing. High dose Intravenous steroids are a safe and effective treatment in sudden sensori-neural hearing loss.

Efficacy & outcomes of balloon sinuplasty in chronic rhinosinusitis: a prospective study.

Balloon Sinuplasty is a new technique which has revolutionized sinus surgery in recent times. Since its introduction in USA in 2004, it has become popular world-wide, due to its sophisticated technology, which uses balloon dilatational systems for dilating the sinus ostia through a minimally invasive approach and has provided satisfactory results in patients with chronic rhinosinusitis. Recent world literature supports the efficacy and outcomes of Balloon Sinuplasty system, with large multi-centric studies proving it to be a very effective tool in the management of various sinus pathologies. We performed this prospective clinical study to assess the efficacy & outcomes of Balloon Sinuplasty among 20 patients at our institution, who were followed up for 12 months after surgery. Patients were included as per inclusion criteria formulated for this study & were analyzed with respect to their pre-operative & post-operative symptomatology scores in comparison with their objective Diagnostic Nasal Endoscopy (DNE) & Computerized Tomography Scan of Paranasal Sinuses (CT-PNS) scoring systems. Significant improvements were recorded in patient's symptoms, from the first post-operative week until the end of the study period, and were objectively confirmed by the DNE & CT-PNS scores. The observations & results of our study highlight the efficacy of Balloon Sinuplasty technology in comparison to similar studies reported in recent world literature.

Papillary carcinoma in a supra hyoid thyroglossal duct cyst - an unusual presentation.

Carcinomatous transformation of ectopic thyroid elements within the thyroglossal tract is a rare entity. We report a case of primary papillary carcinoma of thyroid presenting within the thyroglossal duct cyst in a 23 year old gentleman. The patient presented to us, as a case of suprahyoid thyroglossal cyst with sub-lingual involvement and he underwent surgical excision of the entire thyroglossal tract by Sistrunk's approach. The post-operative histopathological diagnosis was of a papillary carcinoma within the thyroglossal duct cyst. Hence, the patient was evaluated for a malignant focus in the thyroid which proved negative. He was counseled regarding the possibility of developing Carcinoma in the thyroid gland and offered two options of further management viz., total thyroidectomy followed by life long thyroid suppression or watchful observation and follow up. As the patient belonged to the low risk group, he opted for the second option. He is presently kept under meticulous follow up and remains asymptomatic till date. We present the pre- and post-operative imaging, intra-operative findings, histo-pathological features and review the present world literature on this rare entity.

Balloon sinuplasty - the first Indian experience.

The surgical management of sinusitis was revolutionized worldwide with the advent of the rigid Hopkins rod nasal endoscopes three decades ago. The traditional Messerklinger technique, was thus propagated worldwide by Prof. Stammberger, from the University of Graz in Austria and has come into vogue as functional endoscopic sinus surgery (FESS). The principal aim behind this procedure was the maximal preservation of the nasal mucosal integrity, while providing optimal disease clearance. Today, the introduction of a new technological innovation called 'balloon sinuplasty', has taken the field of sinus surgery a step further. This new technology is very similar to the principles of balloon angioplasty and today, this system has added an efficient, non-invasive tool in the armamentarium of the innovative endoscopic rhinologist. This FDA approved technique, in recent times has provided excellent results in various centers across the western world. We share our first surgical experience with the introduction of this cutting-edge technology in India.

Totally implantable hearing aid surgical technique and the first Indian experience with Envoy esteem.

Hearing aids are the principal means of auditory rehabilitation for patients with moderate to severe sensorineural hearing loss. Although technical improvements and modifications have improved the fidelity of conventional aids, hearing aids still have many limitations. Implantable hearing aids offer patients with hearing loss several potential advantages over conventional hearing aids. This presentation will highlight our first experience, the indications, the procedure, the advantages and the current status of totally implantable hearing aids.

Clinical audit of outcomes in Cochlear Implantation an Indian experience.

This retrospective study is based on the assessment of outcomes of Cochlear Implantation taking into account various scoring systems like Category of Auditory Performance (CAP) and Speech Intelligibility Rating (SIR). 100 candidates who were implanted between June 1997-June 2005 at Madras ENT Research Foundation (MERF), a tertiary referral center for cochlear implantation were included in the study. The results were analyzed using the above scoring systems to assess the performance levels of each implantee and to arrive at a cumulative result on the outcome of the implantation. Practical issues of concern to Cochlear Implantation in the Indian subcontinent were also analyzed.

Cochlear implantation in multi-handicapped patients: the merf experience.

Hearing loss is the most common form of sensory impairment in humans. When hearing impairment is present from birth or is acquired in early childhood, it can be devastating for normal development. Early childhood deafness also has implications for cognitive, emotional and social development. In the majority of cases of early childhood hearing impairment, deafness is an isolated disorder. However, in a substantial number of cases, early childhood hearing impairment is not isolated, but forms part of a genetic syndrome with associated handicaps, or is acquired through pre-, perior postnatal causes, possibly with associated deficits. This means that more demands are made on the child's family, the teachers and medical professionals to help these children cope with their rehabilitation, needs and demands. The presence of an additional handicap can pose special problems with regard to surgery, anesthesia or rehabilitation. Cochlear implantation is effective and aids in the effective rehabilitation of other additional handicaps. However, careful patient selection is required and thorough parental counseling regarding expectations must be given. In our Cochlear Implant Clinic a large proportion of implantees are in the paediatric age group. Of these, a few have had multiple disabilities in addition to their deafness. This paper attempts to share our experience in this extremely challenging and specialized field.

Laryngeal thrush: Merf experience.

Fungal infection of the larynx is a relatively uncommon condition. The lesions max be confined to the vocal folds or may involve various other sites in the larynx. There is, invariably, a risk factor that predisposes to fungal infection viz. immune deficiency, inhaled or systemic steroids, antibiotic usage, etc. These lesions may mimic malignancy or a premalignant condition. There have been very few cases of laryngeal thrush reported in the literature. Awareness of this entity is essential because the management depends on an accurate diagnosis. These lesions invariably respond to a course of oral antifungal therapy and correction of risk factors. We report 3 cases of laryngeal thrush.

Auditory brainstem implantation: The first Indian experience.

Multichannel auditory brainstem implants (ABI) are currently indicated for patients with neurofibromatosis type II (NF2) involving both vestibulocochlear nerves. The ABI helps bypass the damaged cochlear nerves and restores a level of auditory sensation via the electrical stimulation of the cochlear nucleus. The implant is usually placed in the lateral recess of the fourth ventricle at the time of tumor resection to stimulate the cochlear nucleus. We report a case of ABI done on a 15-year-old girl with bilateral vestibular schwannomas.

Unusual presentation of a giant glomus tumor.

Giant glomus tumors pose a challenge to the Otologist by virtue of their location and vascularity. A vast majority of them present with tinnitus, conductive hearing loss and cranial nerve palsies. We report the case of a 16-year-old male patient who presented with sudden right-sided sensorineural hearing loss. This is an unusual presentation of a giant glomus tumor. We present the clinical features and management of this unusual case.