While the majority of people with hearing loss develop it due to aging or loud noise exposure, there are other factors that can cause hearing loss, as well. Disease can play a role, too. Not just recognizable diseases such as diabetes, chronic kidney disease, thyroid disorders and heart disease, but also some rare diseases. Let’s go over a few of these and how they might affect hearing.
Rare Diseases Explained
To be classified as “rare,” a disease must affect fewer than 200,000 people worldwide. Scientists have identified around 7,000 diseases that are considered rare. It’s estimated that 80% of these 7,000 diseases are inherited (genetic), and that approximately 50% of them develop at birth or during childhood.
Rare Diseases that Affect Hearing
About 400 of these 7,000 diseases are known to be associated with hearing loss. They may affect hearing in different ways and to different degrees. We’re not going to cover all 400 here, of course, but here are some of them.
Usher’s Syndrome
This disorder affects both hearing and vision, causing partial or total loss in both in all patients. In fact, it is the leading cause of combined deaf-blindness. It is caused by mutations in specific genes that control how hearing and vision develop during fetal development.
Mondini Dysplasia
Babies born with Mondini dysplasia have a structural abnormality in the inner ear that causes their cochlea to be deformed; they’ll have one and a half “coils” instead of the normal two. Because the cochlea is the main organ in hearing, this significantly impacts hearing ability. Hearing loss for children born with Mondini dysplasia may be present at birth or develop over time.
Turner Syndrome
This syndrome only affects female children. It occurs when the baby is born with a missing or only partially formed X chromosome. This results in a variety of symptoms, such as heart defects, differences in sexual development, kidney problems, slowed growth, edema and bone fragility. Because of issues in body growth and structure, the delicate tissues and organs within the ear are more susceptible to infection, damage or other dysfunctions.
Auditory Neuropathy Spectrum Disorder
This disorder specifically occurs in the auditory system. With ANSD, signals from the inner ear are not transmitted to the brain properly, either because the signals are jumbled up or because they don’t reach the brain at all, leading to difficulty understanding speech. It is caused by damage along the auditory nerve, and it can run in families, but it can also appear without any family history.
Paget’s Disease
This disorder is characterized by weakened bone structure. People with Paget’s disease have a higher risk of deformities and a higher chance of fracture or break in the event of an injury of any kind. Hearing loss can occur due to head injury or trauma, neck injury or trauma, stretching of the auditory nerve or damage to the cochlea.
Treating Hearing Loss Caused by a Rare Disease
Treatment for hearing loss caused by a rare disease will depend greatly on what disease the patient has and how severe the hearing loss is. For some, hearing aids can help by amplifying sounds through the ear canal or inner ear that might otherwise struggle to collect sound information. For others, a cochlear implant might be more effective as it transmits sound directly to the auditory nerve, bypassing the ear entirely. And for others, surgery might be required to correct a structural issue. Still others might require both surgery and a hearing device.
To learn more about hearing loss, either in relation to rare diseases or otherwise, contact The Hearing & Speech Center.
