Lake Charles residents with hearing loss experience a wide range of side effects, including exhaustion. This often leaves them with no energy for social activities; because withdrawal and isolation can lead to anxiety, depression and dementia, getting a handle on the physical and mental exhaustion caused by a hearing impairment is important for your long-term health.
There are many possible causes of hearing loss in Lake Charles. Most patients develop a hearing impairment as a result of common factors such as aging and noise exposure. Though far less frequent, other factors – such as rare diseases – can be blamed.
Genetic Disorders and Hearing Loss
There are about 7,000 diseases that scientists have classified as rare. By definition, these are conditions that affect fewer than 200,000 people. This might seem like a large number, but considering the global population is greater than 7 billion, it’s really a drop in the bucket. Still, an estimated 30 million Americans have been diagnosed with a rare disease – and around 400 of those can cause hearing loss. Many patients are diagnosed with a rare disease at birth or early in life, thanks to hereditary factors and faulty genes. Fortunately, hearing screenings for newborns are now routine in the U.S., making early detection easier and improving their long-term outlook. Hearing loss affects approximately 2-3 out of every 1,000 newborns.
Not all rare diseases are present at birth. Sometimes, symptoms don’t appear until several months after birth. Krabbe disease, for instance – a serious neurological disorder – can show up as late as six months after birth. Symptoms include fever, muscle weakness, vision and hearing loss. Some diseases don’t manifest themselves until years later. Alport syndrome is a genetic condition characterized by progressive kidney disease. Children often experience hearing loss in in late childhood or adolescence. Alström syndrome is another genetic disorder in which sensorineural hearing loss develops in childhood and grows progressively worse.
The degree of hearing loss caused by rare diseases varies – even among individuals with the same condition. Hearing aids are often an effective solution, but some rare diseases cause significant anatomical damage to the ears requiring more extensive treatment such as cochlear implants. One example is Turner syndrome, a chromosomal disorder that affects females; it affects physical growth and leads to conductive hearing loss in about 80 percent of patients. Mondini dysplasia is an inner ear abnormality in which babies are born with one and a half coils in the cochlea instead of the usual two; this can occur in one or both ears and generally leads to profound hearing loss that may require treatment with cochlear implants.
Disorders Linked to Hearing Loss
Some of the rare diseases that can cause hearing loss include:
- Usher syndrome. This is the most common disorder that affects both hearing and vision. Patients experience partial or total hearing and vision loss.
- Waardenburg syndrome. This genetic disorder is actually a group of six diseases that causes hearing loss in 80 percent or more of patients. One of the key characteristics involves eye coloring; people may have pale blue eyes or differently colored eyes.
- Auditory neuropathy spectrum disorder. This inner ear disease occurs when the inner ear is able to detect sound but can’t transmit it to the brain properly. It can occur at any age and doesn’t necessarily have a genetic component.
- Vogt-Koyanagi-Harada disease. This inflammatory disorder affects multiple parts of the body and may cause hearing loss, tinnitus and vertigo. Fortunately, hearing loss may be mild.
- Cogan’s syndrome. This autoimmune disease causes inflammation of the eyes and ears. Patients may develop vision and hearing loss, as well as vertigo.
Your Lake Charles audiologist is happy to provide more information on hearing loss and its association with certain rare diseases. You can also visit the National Organization for Rare Disorders (NORD) website for additional information.