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.     

Screening for coronary heart disease and diabetes risk in a dental setting

Objectives

Diabetes mellitus (DM) and heart disease, among the most prevalent chronic conditions worldwide, are increasing among younger adults who are unaware of their risk status. Previous studies in the United States have shown the efficacy of screening for risk of heart disease and diabetes in a dental setting. A screening strategy was applied to facilitate early identification of individuals at increased disease risk in a single Indian dental institute.

Methods

158 patients >30 years old, with no reported heart disease or diabetes, and unaware of any increased disease risk were enrolled. Blood pressure, total cholesterol, high-density lipoprotein levels and body mass index were collected. The Framingham Risk Score (FRS) was calculated as an indication of global risk of developing a coronary heart disease (CHD) event within 10 years; hemoglobin A1c level was used to determine DM risk.

Results

Eleven percent had increased risk of heart disease (FRS >10 %) and 32 % had abnormal A1c levels (>5.7 %). At least one risk factor was present in 61 and 39 % presented with two or more risk factors. Hypertension and obesity were the most common risk factors.

Conclusions

The use of a dental setting in a developing country could serve as a resource for early identification of patients at increased risk of developing CHD and DM, yet unaware of their increased risk. The dental setting can also serve as an entry point into the medical care system by identifying asymptomatic patients at increased risk of disease and referring these individuals to a primary care provider.     

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.     

Alveolar soft part sarcoma of tongue base — A rare presentation of a rare tumor

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.     

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.     

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.     

Kidney-bone, bone-kidney, and cell-cell communications in renal osteodystrophy

The relationship between bone and the kidney in renal osteodystrophy is a complex interplay of kidney to bone connections, bone to kidney connections, and cell to cell connections. In addition, such interactions have a profound effect on the vasculature. In this review, we discuss the role of the bone morphogenetic proteins (BMPs) in the skeleton, kidney, and vasculature. In addition, we propose that deficiencies of these BMPs seen in chronic kidney disease (CKD) result in decreased bone remodeling and a compensatory secondary hyperparathyroidism (high turnover state). Treatment of the hyperparathyroidism blocks this compensatory arm and thus decreased bone remodeling occurs (low turnover). We review animal models of CKD in which treatment with BMP-7 resulted in normalization of both high and low turnover states. Finally, we discuss vascular calcification as it relates to bone metabolism. We discuss the roles of BMP-7 and 2 other bone regulatory proteins, osteoprotegerin (OPG) and alpha2-HS glycoprotein (AHSG, human fetuin), in the human vasculature and their implications for vascular calcification.