Hearing Loss Related Diseases

A human’s ear has three main parts: inner, middle, and outer ear. All three components of the ear are vital to hearing. Sound waves first travel through your outer ear before they reach your middle ear, where vibrations occur. The vibrations that are caused in the middle ear are transmitted through three tiny bones — ossicles. These vibrations then travel to your inner ear, which is a snail shaped organ and creates the nerve impulses that are sent to your brain. Your brain is then able to decipher these impulses as noises. When damage occurs to these hairs or nerve cells in your inner ear such as tearing, the electrical signals that are sent do not work as efficiently and result in hearing loss. 

Diagram of the inner ear
Types of hearing loss diseases

  • Meniere’s Disease
  • Middle ear Infections
  • Otosclerosis
  • Autoimmune inner ear disease
  • Acoustic neuroma
The remainder of this post will discuss in greater depth each of these hearing loss diseases, who they affect, what the causes are, and what symptoms a person may experience. 

Meniere’s Disease (primary idiopathic endolymphatic hydrop)

Meniere's Disease Statistics

What is Meniere’s?

Meniere’s Disease is an inner ear disease which can affect people of any age, but people in their 40’s and 50’s are much more likely to experience the effects of Meniere’s. This condition is considered to be chronic (progressive) and there is currently no cure.

Potential Causes or Triggers

There is no exact cause for Meniere's. It is believed that it is caused when the ear fills with a fluid known as endolymph, causing pressure.
  • Head Injury
  • Infection to the inner or middle ear
  • Alcohol consumption/ smoking
  • Side effects of certain medications
  • Family history of the disease
  • Migraines
  • Respiratory infections
  • Abnormal immune response (autoimmune disease)


Symptoms vary from person to person, and some will experience many attacks over a period of several days, while others will only experience an attack every once in a while. To diagnose the disease, one must experience tinnitus, hearing loss, and vertigo more than once. Symptoms typically occur in this order.
  • Feeling of pressure in the ear
  • Sudden dizzy spells
  • Tinnitus
  • Muffled hearing or hearing loss
  • Stages of Meniere’s
Early stage: During this time, a person will experience sudden and often out-of-the-blue episodes of vertigo that last anywhere from 20 minutes to an entire day. A person's ear may feel blocked or full, and they may have some hearing loss, which typically goes away after the episode fades. It is also common to feel the effects of tinnitus.
Middle stage: Symptoms of vertigo tend to become less severe during this stage, while hearing loss and tinnitus will increase in severity. Many people will also experience long-term remission (the disease goes away) that can last several months.
Late stage: During the late stages of Meniere's disease, patients will not suffer from vertigo as often, and some people will be relieved from it forever. However, tinnitus and hearing loss will likely get progressively worse, and people will likely experience unsteady balance regularly. Most people at this stage feel unstable in dark conditions, for example.

Middle ear infections (acute otitis media)

Why adults need ear tubes
In short, middle ear infections almost always develop during or after upper respiratory infections, such as the common cold. The infection causes inflammation and swelling in the back of the throat, including the anatomical part known as the Eustachian tube. This tube connects the throat to the middle ear, and when it’s swollen, it can no longer equalize the pressure in the middle ear space. This pressure builds up in the tiny middle ear space and normal secretions can’t drain away as they normally would. 

Who gets middle ear infections?

Middle ear infections can happen to anyone with a cold, however, younger children are more prone to middle ear infections for two reasons.
First, their immune systems are less developed, so it is harder to fight off respiratory infections.
Second, their Eustachian tubes are more horizontal, making it harder for fluid to drain.
Another risk factor for middle ear infection is a chronic infection of the adenoids or tonsils. These glands are close to the Eustachian tube, so viral or bacterial invaders can easily spread to the middle ear. 


young children (babies/toddlers)
  • Fever
  • Frequent scratching or pulling at the ears (indicating pain or discomfort)
  • Slowed response to voices and other sounds (indicating trouble hearing)
  • Irritability
  • Secretions from the ear
older children, adolescents, and adults
  • A constant earache
  • A feeling of pressure in the ears
  • Difficulty understanding speech
  • Feelings of dizziness or imbalance
  • Vomiting or general nausea
  • Treatment
Antibiotics work to destroy the infection in your inner ear. As a result, over time, inflammation will get better, and the Eustachian tubes can properly ventilate the middle ear.
Until the backed-up fluids have cleared, a person is prone to recurrent infections. For recurrent middle ear infections, a minor surgical procedure (ear tube surgery) to insert a tube into the eardrums may be performed. The tube, also called a pressure equalization tube, aerates the middle ear space through the ear canal to allow any trapped fluids to dissipate. 


Diagram of the ear

What is Otosclerosis?

In the ear, sound waves are converted first to mechanical energy, then to electrical energy. As part of that effort, three small bones within your middle ear vibrate. For people with otosclerosis, the smallest bone in the body, the stapes, gets affixed to the surrounding structure, making it unable to move. Meaning that sound enters the ear, but the bone growth in the middle ear prevents it from making its way to the inner ear. Otosclerosis is a form of conductive hearing loss and is relatively uncommon in hearing loss patients.

Risk Factors

Some known risk factors include:
Genetics: If there is a family history of otosclerosis, you have an increased risk of developing otosclerosis.
Pregnancy: there is evidence that pregnancy accelerates the hearing loss component of otosclerosis in some patients.
Race, gender, age: White women are particularly at risk for otosclerosis. The condition tends to start in your 20s and 30s is the most common cause of middle ear hearing loss in young adults.  


Otosclerosis can affect one or both ears. If both ears are affected it is referred to as bilateral otosclerosis, but in both cases the symptoms are the same. 
  • Gradual hearing loss
  • Tinnitus
  • Dizziness (sensation of vertigo)
  • Diagnosis of otosclerosis is typically very straight forward with the use of an audiogram. 
  • Treatment
Surgery: Stapedectomy 
The non-moving stapes bone are removed and replaced with a prosthesis made out of titanium
The majority of the time, this surgery is successful at restoring hearing. Rare cases can lead to complications resulting in profound hearing loss or other side effects.
Hearing Aids
Getting hearing aids will treat the primary symptom—hearing loss—but does not treat the underlying conditions of otosclerosis

Autoimmune inner ear disease (AIED)

Diagram of Cochlear Implant

What is AIED?

Autoimmune inner ear disease is a rare disease that happens when your body's immune system mistakenly attacks your inner ear.
Less than 1% of the 28 million Americans who have hearing loss have it because of AIED. It is slightly more common in middle-aged women.


If you have AIED, you'll have hearing loss that starts in one ear and then spreads to the other. This may take weeks, or it could happen over a few months.
  • Dizziness or problems with balance
  • Fullness (pressure) in your ear 
  • Tinnitus
  • Vertigo 
  • Causes
If your immune cells mistake cells in your inner ear for a virus or bacteria, they attack them. This is called an autoimmune reaction. Your immune cells may harm other organs as well. Just under 30% of people who have AIED have another autoimmune disease that affects their whole body, such as rheumatoid arthritis, lupus, scleroderma, ulcerative colitis, or Sjoegren's syndrome (dry eye syndrome).


  • Steroids have shown to help with the inflammation in your ear that is caused by AIED
  • Medications such as azathioprine (Imuran), cyclophosphamide (Cytoxan), and methotrexate are used as treatment to calm down the reaction of one’s immune system and stop your immune cells from attacking your inner ear. 
Cochlear Implant
This is a small device that affects the nerves in your inner ear that send signals to your brain. There, the brain turns them into sound. Part of the cochlear implant sits behind your ear. The other part is put under your skin during surgery.

Acoustic Neuroma (vestibular schwannoma)

Acoustic Neroma and Cochlear nerve diagram of ear

What is Acoustic Neuroma?

Acoustic neuroma is a noncancerous and usually slow-growing tumor that develops on the main (vestibular) nerve leading from your inner ear to your brain. Branches of this nerve directly influence your balance and hearing. 
Acoustic neuroma usually arises from the Schwann cells covering this nerve and grows slowly or not at all. Rarely, it may grow rapidly and become large enough to press against the brain and interfere with vital functions.


Signs and symptoms of acoustic neuroma are often subtle and may take many years to develop. They usually arise from the tumor's effects on the hearing and balance nerves. As the tumor grows, it may be more likely to cause more noticeable or severe signs and symptoms. Some symptoms may become permanent.
  • Gradual hearing loss on one side or more pronounced on one side
  • Ringing (tinnitus) in the affected ear
  • Unsteadiness, loss of balance
  • Dizziness (vertigo)
  • Facial numbness, weakness or loss of muscle movement


The cause of acoustic neuromas appears to be a malfunctioning gene on chromosome 22. Normally, this gene produces a tumor suppressor protein that helps control the growth of Schwann cells covering the nerves.
In most cases of acoustic neuroma, there is no identifiable cause. This faulty gene is also inherited in neurofibromatosis type 2 (NF2), a rare disorder that usually involves the growth of tumors on balance nerves on both sides of your head (bilateral vestibular schwannomas).

Risk Factors

The only confirmed risk factor for acoustic neuroma is having a parent with the rare genetic disorder NF2, which is known as an autosomal dominant disorder (dominant gene), meaning the mutation can be passed on by just one parent. Each child of an affected parent has a 50-50 chance of inheriting it.
Treatments for acoustic neuroma include regular monitoring, radiation and surgical removal.


Loss of hearing can be caused by several different types of diseases with no clear explanation of why. Whether hearing loss is caused by genetics, age, tumors, autoimmune responses, etc. there are always precautions we can take to ensure we are not damaging our hearing permanently. Consider these tips to help prevent hearing loss.
  • Avoid too much noise
  • Wear hearing protection
  • earplugs 
  • earmuffs
  • Do not smoke
  • Remove earwax properly
  • Check medications for hearing risks
  • Have your hearing tested
Although frustrating, there are many ways to treat and combat the loss of hearing you may be experiencing. If you are experiencing multiple symptoms discussed in this post, on a regular basis, consider talking to your doctor about different options to help improve your hearing. 
We hope you found the information discussed in this article helpful and are now more aware of how to protect your ears from permanent damage and identify symptoms of hearing loss diseases. Be sure to check out our high quality and affordable hearing products at Audien Hearing!


Kathleen Williams

Senior Editor, Audien Hearing


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