Four Rare Diseases Associated with Hearing Loss

Many people associate hearing loss with only a handful of common causes, including aging, exposure to loud noises, and trauma. However, there are also many congenital and acquired diseases that can lead to varying levels of hearing loss throughout a person's life. Here's a look at four rare diseases commonly associated with hearing loss.

Usher Syndrome

Of all the rare diseases that may lead to hearing loss, Usher syndrome is the most common. This disease involves mutations in the genes that allow the inner ears and retinas to function, which often cause sufferers to experience both hearing and vision loss, as well as potentially severe balance problems. The severity of hearing loss associated with Usher syndrome depends on several factors, including the age of onset and the speed at which degeneration occurs.

Waardenburg Syndrome

Made up of a group of six genetic disorders, Waardenburg syndrome causes hearing loss or deafness in about 80% of patients. Certain physical characteristics often indicate Waardenburg syndrome, including pale blue eyes, differently colored eyes, or eyes containing two or more colors. Patients may also develop patches of white hair or experience gray hair early in life.

Auditory Neuropathy Spectrum Disorder

Auditory neuropathy spectrum disorder can occur in people randomly and appear at any age. The disease prevents auditory signals received in the inner ear from properly reaching the brain. Patients with auditory neuropathy spectrum disorder may experience alternating periods of functional hearing and hearing loss.

Vogt-Koyanagi-Harada Disease

An autoimmune disorder, Vogt-Koyanagi-Harada disease causes inflammation of melanin cells, which give human skin, eyes, and hair their color. When cells in the ear are affected, hearing loss, tinnitus, and vertigo can result. Luckily, hearing loss caused by Vogt-Koyanagi-Harada disease can often be successfully treated with hearing aids.

While these diseases are rare, any signs of hearing loss should always be evaluated and, if necessary, treated. Serving patients throughout the Columbus area, Absolute Hearing Solutions offers hearing aid evaluations, advanced testing, and a wide range of hearing aid models and brands. To learn more or to schedule an appointment, contact us today by calling 614.452.4280.