A recent article on healthyhearing.com drew some interesting parallels between hearing loss and rare health disorders. By one estimate, as many as 30 million Americans currently live with a disease that is considered rare (there are about 7,000 of these diseases). Many of them are autoimmune or linked to genetic causes.
What’s really interesting is that over 400 of these diseases are associated with hearing loss as well.
Some of these disorders with a hearing loss component include:
- Mondini dysplasia – babies with this disorder have one and a half coils in the cochlea instead of the normal two and as a result have profound hearing loss. This could be the case in one ear or both.
- KID syndrome, Donnai-Barrow syndrome, and Wildervanck syndrome – also feature hearing loss in infants.
- Krabbe disease – babies can hear fine right out of the womb, but usually develop hearing loss in the first six months.
- Alport syndrome – takes longer to affect the hearing, as long as late childhood or early adolescence.
- Usher syndrome – can lead to three different types of hearing loss, depending on the onset and severity of symptoms. It is also accompanied by vision loss and balance issues.
- Auditory neuropathy spectrum disorder – involves a failure to transmit signals from the inner ear to the brain, resulting in, among other things, mild to severe hearing loss.
- Waardenburg syndrome – features hearing loss in 80+% of patients.
- Vogt-Koyanagi-Harada disease – causes inflammation of melanocytes which are present in the inner ear, leading to mild to severe hearing loss.
- Cogan’s syndrome – another autoimmune disorder that can affect the structure of the inner ear and lead to hearing loss.
Occasionally, it will be the discovery of hearing loss that tips parents off to the presence of the other disorder. But more frequently, hearing loss may go unnoticed while parents are dealing with a more obvious and rare disorder.
It’s not a given that these disorders will affect hearing from birth. So, a baby could pass its hearing screening at birth, only to have its hearing deteriorate later on. Parents of children with a rare disorder should do regular hearing tests because even when dealing with a serious disorders, something as simple as correcting one’s hearing could have a dramatic impact on the patient’s quality of life.
If you have a friend or loved one who suffers from one of these disorders and you suspect they might be experiencing hearing loss, get in touch with us to schedule a hearing test!
