prelingual non-syndromic genetic hearing loss

Prelingual non-syndromic genetic hearing loss is a rare condition that affects the inner and/or middle ear or the hearing nerve. It causes severe to profound hearing loss that is usually present in both ears before a child begins to speak. The hearing loss is typically sensorineural with an average impairment of 60 dB or more for common speech frequencies, and people with this condition do not have external ear abnormalities or other medical problems.

People with this condition typically experience significant, bilateral hearing loss from a very early age. The hearing loss is largely sensorineural and is severe enough to impact the development of oral language. There are no obvious abnormalities of the outer ear and the condition is usually nonprogressive.

This condition is genetic and highly heterogeneous, meaning that it can result from various anomalies in the structures of the inner ear, middle ear, or the hearing nerve. It is known to be present before the onset of speech and is not linked with other medical issues or external ear abnormalities.

Information about treatment is currently limited for this condition.

The condition is usually nonprogressive, meaning that the hearing loss does not worsen over time. However, the significant hearing loss can affect the development of oral language, and support such as speech therapy or hearing aids may be important for managing the condition.

Information about research is currently limited for this condition.