Rapid Hearing Loss a Symptom of Fatal Creutzfeldt-Jakob Disease

It is believed that hearing loss is a common problem that occurs with aging. But researchers at the University of Henry Ford Hospital state that rapid hearing loss in both the ears is a symptom of a rare and fatal disease called Creutzfeldt-Jakob Disease. This symptom requires the clinicians to test for the disorder.

Creutzfeldt-Jakob Disease (CJD) is a degenerative neurological disorder and is incurable. One in million every year worldwide gets affected with this disease. In the U.S., there are about 200 cases reported every year. It appears in later life at the age of 60 and the victim dies within one year.

Researchers at the Henry Ford Hospital came up with the explanation after analyzing a 67-year-old patient. The patient progressively noticed a hearing loss in both the ears for two months and was later found to be a victim of CJD.

Symptoms of CJD include change in personality, impaired memory, impaired vision, insomnia and involuntary muscle jerks called myoclonus. Once infected with CJD, the brain develops holes, causing the tissue to look like a sponge.

The lead author Ahmad Riad Ramadan, M.D., neurologist at Henry Ford, states that when the patient sought treatment, he didn't have any significant medical history and just reported a rapid hearing loss in both the ears. He also reported the common side effect of hearing loss i.e., tinnitus - a constant ringing in the ears.

"This was followed by the kind of cognitive decline that is typical of CJD," Ramadan said in a press statement. "During the patient's hospital stay, he also showed signs of ataxia - a lack of coordination - and myoclonus - a spastic muscle twitch."

During the test, they discovered the presence of a telltale protein and other conditions that led to the diagnosis of CJD.

The "sensorineural hearing loss", that is also known as the "nerve deafness", is the most common cause of permanent impairment.

The researchers will present the report March 19 at the annual scientific meeting of the American Academy of Neurology in San Diego.