2019 Volume 62 Issue 4 Pages 299-306
Alport syndrome is primarily characterized by nephritis, but is also accompanied by eye problems and gradual progressive bilateral sensorineural hearing loss. Unfortunately, few reports are available in the literature on the treatment and long-term progression of hearing loss in individual cases of Alport syndrome. We therefore examined the decrease in hearing levels, medical history, and the use of hearing aids in three male Alport syndrome patients who have been regularly monitored at our ENT department over an extended period. The monitoring periods for the three patients were 8 years (from the ages of 3 to 11 years), 13 years (from the ages of 4 to 17 years), and 7 years (from the ages of 11 to 18 years). The first two patients had developed hearing loss at a relatively young age, and their progressive hearing loss was observed relatively early. The third patient was not diagnosed as suffering from Alport syndrome until he was older, and by that time, he had already been diagnosed as having hearing loss. All three patients have continued to experience a gradual decrease in the hearing levels, but none has reached the point of needing a hearing aid(s).