What is the major cause of hearing loss in Alport Syndrome?

In Alport Syndrome, the most significant cause of hearing loss is progressive sensorineural hearing loss. Alport Syndrome is a genetic condition primarily caused by mutations affecting collagen, which is crucial for maintaining the structure and function of various tissues, including the auditory system. As the disease progresses, individuals typically experience a gradual deterioration of the hair cells in the inner ear, which leads to sensorineural hearing loss.

This type of hearing loss is characterized by a decrease in sound perception that is related to the damage to the sensory nerves or hair cells within the cochlea, rather than issues with the outer or middle ear that would cause conductive hearing loss. Individuals with Alport Syndrome often begin to notice hearing difficulties in their teenage years or early adulthood, which is why it is considered to be progressive.

Progressive conductive hearing loss is not the primary issue in this syndrome, as that would imply problems in the outer or middle ear, which are not typical pathways for the hearing loss seen in Alport Syndrome. Static conductive hearing loss would suggest a constant inability to conduct sound, which is not representative of the gradual decline observed. Variable hearing loss with age may be true in a general sense but does not specifically capture the hallmark feature of Alport Syndrome's trajectory, which is