Sathiya Murali

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.

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.

Cochlear implantation in branchio-oto-renal syndrome — A surgical challenge

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.

Surgical management of trismus due to Oral Submucous Fibrosis - Lysis of fibrotic bands with the KTP-532 Laser.

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.

KTP-532 laser in the management of rhinosporidiosis.

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.

Fish Karyome version 2.1: a chromosome database of fishes and other aquatic organisms

A voluminous information is available on karyological studies of fishes; however, limited efforts were made for compilation and curation of the available karyological data in a digital form. ‘Fish Karyome’ database was the preliminary attempt to compile and digitize the available karyological information on finfishes belonging to the Indian subcontinent. But the database had limitations since it covered data only on Indian finfishes with limited search options. Perceiving the feedbacks from the users and its utility in fish cytogenetic studies, the Fish Karyome database was upgraded by applying Linux, Apache, MySQL and PHP (pre hypertext processor) (LAMP) technologies. In the present version, the scope of the system was increased by compiling and curating the available chromosomal information over the globe on fishes and other aquatic organisms, such as echinoderms, molluscs and arthropods, especially of aquaculture importance. Thus, Fish Karyome version 2.1 presently covers 866 chromosomal records for 726 species supported with 253 published articles and the information is being updated regularly. The database provides information on chromosome number and morphology, sex chromosomes, chromosome banding, molecular cytogenetic markers, etc. supported by fish and karyotype images through interactive tools. It also enables the users to browse and view chromosomal information based on habitat, family, conservation status and chromosome number. The system also displays chromosome number in model organisms, protocol for chromosome preparation and allied techniques and glossary of cytogenetic terms. A data submission facility has also been provided through data submission panel. The database can serve as a unique and useful resource for cytogenetic characterization, sex determination, chromosomal mapping, cytotaxonomy, karyo-evolution and systematics of fishes. Database URL: http://mail.nbfgr.res.in/Fish_Karyome

Endolymphatic Sac Tumour: A Neoplastic Cause for Meniere’s Syndrome

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.

PRIMARY NASAL TUBERCULOSIS - A CASE REPORT

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.

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.

Magnet migration in a cochlear implantee — a serendipitous diagnosis

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.