Sudden sensorineural hearing loss is a condition in which the nerves in the inner ear suddenly stop working. While earwax or cold may be to blame, sudden deafness is not. Although this condition can be scary, there are treatments for sudden sensorineural hearing loss that can help prevent permanent hearing loss. In some cases, there may be partial or complete recovery. If this is the case, you should consult a doctor to discuss your options.
Idiopathic sudden sensorineural hearing loss
Idiopathic sudden sensorineural hearing (SSNHL) is a common condition in audiology. It is characterized by a sudden, unexplained loss of hearing of 30 decibels (dB) or more over three contiguous audiometric frequencies over a 72-hour period. While the cause of SSNHL is unknown in many cases, treatment for patients with this condition is often directed at the known etiology. Unfortunately, this condition often leads to poor hearing outcomes. In the United States, steroid therapy is the mainstay treatment for idiopathic SSNHL.
The onset of ISSNHL usually occurs suddenly and occurs more quickly in the morning. In addition to sudden hearing loss, patients may experience dizziness, balance problems, tinnitus, and/or ringing in the ears. Often, patients delay seeking medical treatment because they believe the hearing problem is a sign of allergy or sinusitis. However, if these symptoms persist, the condition could be a sign of an underlying health problem.
Although idiopathic SSNHL is rare, the condition is often associated with pre-existing disease. In some cases, a previous illness in the affected ear may have contributed to the symptoms. A pre-existing disease may also provide clues to the diagnosis. For example, if a patient has a family history of multiple sclerosis, they may be at increased risk of developing this condition.
The ISSNHL study population was age-matched and sex-matched. The researchers searched the MEE audiometry database to identify patients with SNHL. They matched patients with similar audiometric results and prioritized those with the best matching audiometric data. Moreover, they analyzed the patient's treatment options and the recovery of hearing. This study has shown that a variety of therapeutic regimens are available for treating ISSNHL.
Genetic causes
Recent studies have identified several genetic causes of sudden sensorineural hearing loss (SSNHL). A Chinese study investigated the gene variants in 19 genes related to deafness and their association with the onset of the disorder. Researchers found that the incidence of sudden sensorineural hearing loss (SSNHL) is significantly higher in patients with familial history of deafness than in individuals with normal hearing. A subsequent study found that genetic mutations of 307 nuclear genes were responsible for more than half of SSNHL cases.
Other causes of sudden sensorineural hearing loss include exposure to loud noises and certain medications. The condition affects five to 20 per 100,000 people. Exposure to loud noises damages stereocilia, tiny hairs in the inner ear that convert sound waves into neural signals. These signals travel to the brain. When 30 to 50 percent of these specialized cells are damaged, the condition can lead to severe hearing loss. Thankfully, most cases of SNHL are not life-threatening. However, they can interfere with communication, which is why it is important to learn more about the causes of sudden sensorineural hearing loss.
One genetic cause of sudden sensorineural hearing loss is a mutation in the GJB2 gene, which controls the formation of cells. Mutations in this gene affect the structure of gap junctions, which are crucial for the survival and function of cells. Some mutations are familial, while others do not. Hearing loss in multiple family members is often familial. The inherited mutation is responsible for hearing loss in both sexes.
Immune disorders
A number of autoimmune diseases are associated with sudden sensorineural hearing loss. These include systemic lupus erythematosus, polyarteritis nodosa, and Wegener granulomatosis. Other autoimmune diseases that have been associated with sudden sensorineural hearing loss include celiac disease, primary antiphospholipid syndrome, Behcet's disease, and Sweet syndrome. There is also a connection between HLA-B27 positivity and vertigo.
A recent study has identified a gene involved in autoimmune hearing loss as HSP70. It is used in the diagnosis and treatment of sensorineural autoimmune hearing loss. The study was published in Cell Stress & Chaperones. Further research is necessary to determine whether this gene causes this condition. For now, doctors are treating the condition with corticosteroids and immunosuppressive drugs.
There are several reasons why an autoimmune disease could cause sudden sensorineural hearing loss, including an allergic reaction. In some cases, the underlying disease is so complex that it affects multiple parts of the body, including the inner ear. In these cases, the immune system attacks blood vessels and inner ear tissue, leading to hearing loss. The body's immune response is triggered by antigens, which it recognizes as foreign substances. The immune system then creates antibodies that protect it from the attack. The disease is typically only diagnosed after other underlying factors have been ruled out. Currently, the prevalence of AIED is estimated at 15 out of every 100,000 people, although it accounts for less than 1% of all cases of SNHL.
A doctor may also recommend testing for AIED. In addition to the blood tests, FDG PET scans may be useful in this case. Although FDG PET scans are very expensive, they do not rule out the possibility of an autoimmune disease. Rather, doctors will seek other evidence of autoimmune involvement. Positive blood tests do not necessarily indicate an autoimmune disease, but they can document inflammation. If you suspect that your immune system is playing a role, a blood test should be performed before any immunosuppressive drugs are taken.
Viral infections
While some types of inner ear diseases are clearly a result of viral infection, others are not. Viral infections have an unlikely, though not unproven, etiology. Viral infections in the inner ear may be caused by immune system disturbances or by direct invasion of the organ of the Corti. In these cases, the symptoms of sudden sensorineural hearing loss are often similar to those of a cold.
Although the exact causes of sudden sensorineural hearing loss are not known, scientists believe that viral infections are responsible for a significant percentage of cases. One viral infection that may cause SSNHL is COVID-19, which affects the cochlea and the nerve pathways between the ear and brain. While there is no direct evidence of a causal link, COVID-19 has been linked to cases of SSNHL in recent years.
There are several theories underlying the etiology of SSNHL, but the etiology is not known. The most common theories suggest that it may be caused by a viral infection, vascular occlusion, or immune-mediated mechanisms. A recent bibliographic review analyzed different aspects of etiology and disease mechanisms. However, the cause of SSNHL is still not completely understood, and the prognosis is unpredictable. Moreover, many patients initially receive a different diagnosis than the one they were suffering from. Consequently, the true nature of the disease may not be discovered until after the fact.
As far as treatment goes, this condition is best managed by a doctor. A hearing healthcare professional may conduct pure tone audiometry to detect the infection. If the virus is causing your sudden loss of hearing, it's best to seek immediate medical evaluation. Treatment can help to prevent permanent hearing damage and prevent it from becoming chronic. The NCBI has a thorough review of viral infections. There are several vaccines available that can help prevent hearing loss.
Eustachian tube problems
In cases of sudden sensorineural hearing loss, eustachian tube dysfunction (ETD) can lead to fluid accumulation in the middle ear. The fluid can be accumulated due to upper respiratory tract infections, pollution, or smoking. Chronic nasal issues also contribute to the condition. Some treatments for ETD include taking medication to reduce inflammation, or in severe cases, surgery. This page highlights a few of the most common procedures for treating ETD.
When a Eustachian tube is obstructed, it causes a negative pressure in the middle ear. The eardrum retracts as a result of this negative pressure. This pressure can cause a ringing or a hollow feeling in the ear. If left untreated, eustachian tube obstruction may lead to conductive hearing loss. The good news is that there are many different treatments available to address eustachian tube problems.
Some patients experience both chronic and acute eustachian tube disease. Serous otitis media, also known as ear infections, can occur for years without causing permanent damage to the middle ear. In addition to increasing susceptibility to recurrent bacterial infections, fluid in the middle ear interferes with the transmission of sound. The condition also causes conductive hearing loss.
A woman with hypertension presented to an urgent care clinic with right-sided hearing loss and a full right ear. She also reported suffering from an upper respiratory infection. Her physical examination revealed a normal appearance and mobility of the tympanic membrane, but a tuning-fork examination was not done. The physician diagnosed eustachian tube dysfunction and prescribed oral decongestants to alleviate her symptoms.
