THE SICHL MISSION

The goal of SICHL is to devise treatments that repair the damaged inner ear and restore lost hearing, quiet tinnitus, and improve balance.

The Genetics of Hearing Loss

Hearing loss is the most common congenital sensory impairment, affecting up to 1 in 500 newborns, and 1 in 300 children by the age of 4. Approximately 1 in 1000 newborns have genetically inherited hearing loss. Later on in life, exposure to chronic infection, noise and chemicals can cause further hearing loss. Age-related hearing impairment (ARHI) is the most common sensory deficit of the elderly, since it eventually affects all individuals.

We are discovering that almost all of these causes of hearing loss, whether from noise exposure, chemicals, or aging, have significant genetic components. A few hundred genes are thought to be involved in hearing loss, but to date only half of them have been identified. At SICHL, our research is dedicated to characterizing this missing knowledge by discovering these genes. We are actively studying all forms of hearing loss:

• non-syndromic (hearing loss as the only symptom)
• syndromic (hearing loss associated with another impairment such as blindness)
• age-related
• chemical-induced, and
• noise-induced hearing loss.

We are using advanced genomic technologies to facilitate the discovery of new deafness genes. The first direct benefit of the discovery of these genes for patients will be the availability of comprehensive genetic diagnostic testing. This test will improve patient care through:

• Facilitating rapid diagnosis
• Providing recurrence risk information and genetic counseling
• Identifying the most appropriate preventative measures for persons susceptible to age-related and noise-induced hearing loss
• Contributing to development of therapeutic approaches