Title: SHRUTHITHARANGAM COCHLEAR IMPLANATAION PROJECT-  AN OUTCOME MEASURE

Name & Designation: Principal Investigators

1. Ms. Jeena Mary Joy, Sr.Audiologist, Dept. of ASLP, NISH
2. Dr.SujaKunnath, HOD, Dept of Neurodevelopmental Sciences, NISH

Name &Designation: Co-Investigators

1. Ms.Sita, Audiologist, Dept. of ASLP, NISH
2. Mr.Sameer Pootheri, Asst.Professor, Govt.Medical College, Calicut
3. Mr.Sasidharan Nair, Clinical Specialist-Cochlear Implants, Dr.Manoj’s ENT Clinic, Calicut
4. Ms.Manju Thomas, Audiologist, Ephaphtha Speech and Hearing Center, Ernakulam 

Sruthitharangam Cochlear Implantation Project (SCIP) by the Govt. of Kerala is a program by which children in lower socio-economic status aged 1 to 3 years are provided with Cochlear Implants free of cost and the subsequent rehabilitation through auditory verbal therapy is provided at approved centers under trained AV therapists. The project was started in 2012 and is the first of its kind in India which is laid out with the objective of early intervention and speech and language development for children with profound deafness.

A longitudinal Outcome Measurement of the children who undergo post-surgical Auditory Verbal therapy under SCIP has been initiated so as to monitor the effectiveness of the program. The first objective of the project is to assess the major predictors of the outcome on a uniform platform. It was very essential to create a medium for reporting of the outcome measures and monitor the therapy plans from all the centers across the state of Kerala on a uniform scale. This steered to the development of web-based software “Outcome Measure Management System” which acts as an interface for the documentation of assessments and daily therapy plans. The second objective of the project is to assess the major predictors of outcomes such as auditory perceptual abilities, speech perception and production skills, language skills and hearing related quality of life measures using standardized test measures and self-developed questionnaires every three months for a span of two years. The outcome is measured from the children undergoing Auditory Verbal Therapy in 11 empanelled centers under the project located across the state which are divided into three zones – North, Central and South based on thegeographical location of the state. The predictors of outcome will be correlated with the progress obtained from the documentation of the daily lesson plans. This is achieved by a User Interface in the software which helps the Auditory Verbal Therapist to document the daily progress in the domains of audition, reception, expression, communication and cognition.

This project argues the importance of documentation of the periodic outcome measures as well as the daily progress of the children using cochlear implants in a systematic manner to study the effectiveness of a government initiated project. Such evidence based research data on outcome is very essential to identify critical elements of improvement in the existing technology as well as the assessment and rehabilitation procedures.

Title: Understanding the DFNB mutation map in Autosomal recessive Non Syndrome Deafness from Kerala

Name and Designation of Principal :
Dr.M.Padmaja, ENT Consultant (Hon.)
Investigator (PI) NISH, Thiruvananthapuram

Name and designation of Co-investigators:
a) Dr. MoinakBannerjee,
Human Molecular Genetics lab,
Rajiv Gandhi Centre for Bio-Technology
Thiruvananthapuram

b) Pravena Davis
Senior Audiologist, Grade I
NISH, Thiruvananthapuram

Though there are several causes for hearing loss, more than 50% is accounted to be of genetic reasons, which are largely due to mutation or deletion in the candidate genes. Mutations in the same gene can result in a variety of clinical phenotypes with different modes of inheritance. In the present study we propose to develop a mutation map specific for Autosomal recessive Non Syndrome Deafness based on DFNB genes. The DFNB1 gene in Indian families account for approximately 40% of the molecular causes of deafness in India and also in Kerala. There is a need to define the magnitude of the problem in India, in view of our large population and social custom of consanguineous marriages among many communities. There is a need for awareness and accurate genetic counseling to high-risk families, for which it is necessary to establish genetic testing of 'at risk' couples for the common deafness mutations prevalent in the ethnic population. This information further enables in counseling the families regarding their possible risk of recurrence in their progeny, and also counseling them against assortative mating to reduce enhancement in recurrence of Non Syndromic Hearing Impairment (NSHI).