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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.

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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.

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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.

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Patients with intractable vertigo often present a diagnostic dilemma to the treating physician. A wide spectrum of diseases, ranging from those of the labyrinth onto the central nervous system, may present predominantly with vertigo. In some cases, it requires the clinical acumen of an experienced neuro-otologist, to decipher these vertiginous symptoms and arrive at a definitive diagnosis. Meniere's syndrome is one such phenomenon, where the endolymphatic hydrops may be attributable to varied aetiology. We report a case of sporadic (non-syndromic) Endolymphatic Sac Tumor which presented to us, mimicking a classical Meniere's syndrome. We discuss its clinical presentation, diagnostic modalities, operative technique and histo-pathological features. The case is reported along with a review of the world literature on this tumor, highlighting the diagnostic and management protocols advocated for this rare entity. Endolymphatic sac tumor is a rare entity which may masquerade as a classical case of Meniere's syndrome, wherein the triad of symptoms (as in endolymphatic hydrops) may not be alleviated by the usual treatment protocols. Tracing the aetiology of these symptoms, remains the most crucial factor in treating such patients.

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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.

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The successful outcome of a cochlear implant habilitation program depends upon a multitude of vital factors, including the avid cooperation of the cochlear implantee, committed participation of the parents/family members and total dedication of the habilitation team of implant audiologists and auditory verbal therapists. In a rare situation, where the implantee's performance is suboptimal or poor inspite of successful implantation, anxious moments engulf the parents and the implant team, with the morbid fear of a device failure lingering at the back of their minds. We report such an incident in a 13-year-old cochlear implantee, who was an excellent cochlear implant performer for 8 years, following which she had rapid deterioration of her auditory verbal skills within the next few weeks. The hidden etiology, was the inconspicuous migration of the internal magnet of the Receiver-Stimulator Coil placed in the mastoid temporal bone of her skull, due to unexplained reasons. We share our experience with the diagnosis and management of this condition and review the existing world literature on this rarely reported entity.

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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.

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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.

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During the past two decades, Tuberculosis - both pulmonary and extrapulmonary have re-emerged as a major health problem worldwide. Nasal tuberculosis may be primary, or secondary to pulmonary tuberculosis or facial lupus. However all of them are rare entities. Nasal tuberculosis should be considered in the differential diagnosis of chronic nasal granulomas. We report a case of primary nasal tuberculosis in an adult female who presented with a polypoidal lesion in the nasal cavity. The diagnosis was based upon smear study, histopathology, culture & polymerase chain reaction. The patient successfully responded to antituberculous therapy and is presently disease free. Given the resurgence of tuberculosis in recent times, it is important that otolaryngologists remain aware of this rare clinical entity.

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Branchio-oto-renal syndrome (Melnick-Fraser Syndrome) is a rare Autosomal Dominant disorder characterized by the syndromic association of branchial cysts or fistulae along with external, middle & inner malformations and renal anomalies. Incomplete penetrance and variable expressivity are common with the phenotypic variation ranging from mild to severe forms & consisting of various eye, ear, oral and craniofacial abnormalities. Mutations in the EYA1 gene on chromosomal site 8q13.3 are identified as the primary cause of BOR syndrome. We present a 3year old child with BOR syndrome, who came to us with bilateral low set, malformed ears & profound cochlear hearing loss along with bilateral branchial fistulae & unilateral renal agenesis. This child underwent successful cochlear implantation recently. The clinical presentation, pre-operative investigations, intra-operative findings & post-op habilitation status are presented with special highlights on the unique facial nerve course along with middle and inner ear anomalies which posed a surgical challenge during cochlear implantation.

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Alveolar soft part sarcoma is a rare, aggressive malignancy of uncertain histologic origin and enigmatic clinical behaviour. It has a characteristic histopathological picture, with a propensity for vascular invasion and distant metastasis. We report a case of alveolar soft part sarcoma involving the tongue base in an adolescent female. She underwent laser assisted excision of the tongue base tumour followed by post-operative radiotherapy. The clinical presentation, histopathological picture, immunohistochemical & cytogenetic studies, radio-imaging, management protocols and prognosis of this tumor have been discussed.

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Oral Submucous Fibrosis is an insidious, chronic disease affecting the oral cavity, sometimes the pharynx and rarely the tongue. 15 patients with Oral Submucous Fibrosis presenting with severe trismus were treated with lysis of the fibrotic bands with a KTP-532 Laser and adjunctive treatment with excellent results over a 12 month follow-up period.

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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.

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Rhinosporidiosis, a difficult granulomatous disease of the nose is notorious for its high rate of recurrence and vascularity. Potassium Titanyl Phosphate (KTP) laserization of the mass seems to have provided an optimal solution in the management of this disease. We present our experience with the use of KTP-532 laser for this challenging disease.

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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.