When is hearing loss due to DFNA9?

DFNA9 is a genetic disorder that causes progressive hearing and balance loss. The disease occurs almost exclusively in Flemish and Dutch people.

DFNA9 is an autosomal dominant form of hearing loss. This means that a person only needs to inherit one copy of the mutated gene from one parent to develop the condition. DFNA9 is specifically associated with mutations in the COCH gene, which encodes the cochlin protein. This protein plays a crucial role in the functioning of the inner ear, which is involved in both hearing and balance.

DFNA9 usually begins with hearing loss in the high frequencies and can eventually lead to severe to complete hearing loss. This hearing loss is progressive, meaning it gets worse over time. Since the inner ear is also involved in balance, many patients with DFNA9 experience progressive balance disorders. This can lead to dizziness, unsteady walking and an increased risk of falls.

Hearing aids can help to compensate for hearing loss. In cases of severe hearing loss, cochlear implants may be an option. Physiotherapy and balance exercises can help improve balance function and reduce the risk of falls.

DFNA9 is a serious genetic disorder that causes progressive hearing and balance loss. Although there is currently no cure, various treatments and supportive therapies can help manage symptoms and improve patients' quality of life.

Source: https://www.ncbi.nlm.nih.gov/pmc/articles/PMC2840995/

Author: Prof. Dr. Dirk Devroey - Latest update: 2022-02-22 - Copyright: Clinifacts 2024