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Hearing Loss

SOURCE: www.NationalMSSociety.org

 

 

 

Hearing loss is an uncommon symptom of MS. About 6% of people who have MS complain of impaired hearing. In very rare cases, hearing loss has been reported as the first symptom of the disease. Deafness due to MS is exceedingly rare, and most acute episodes of hearing deficit caused by MS tend to improve.

 

Hearing loss may take place during an acute exacerbation. An exacerbation is a sudden worsening of an MS symptom or symptoms, or the appearance of new symptoms, which lasts at least 24 hours and is separated from a previous exacerbation by at least one month. An exacerbation is also known as an attack, a relapse, or a flare.

 

 

Linked to Other Symptoms That Suggest Brainstem Damage

 

Hearing loss is usually associated with other symptoms that suggest damage to the brainstem - the part of the nervous system that contains the nerves that help to control vision, hearing, balance, and equilibrium. Hearing deficits caused by MS are thought to be due to inflammation and/or scarring around the eighth cranial nerve (the auditory nerve) as it enters the brainstem, although plaques at other sites along the auditory pathways could also contribute to hearing problems. Plaques are abnormal areas that develop on nerves whose myelin-- the fatty sheath that surrounds and protects nerve fibers--has been destroyed. Plaques cause the nerve impulses to be slowed or halted, producing the symptoms of MS.

 

Because hearing deficits are so uncommon in MS, people with MS who do develop hearing loss should have their hearing thoroughly evaluated to rule out other causes.

 


 More from www.NLM.NIH.gov/MedlinePlus

 

Decreased hearing; Deafness; Loss of hearingDecreased hearing; Deafness; Loss of hearing

 

 

Definition

 

Hearing loss is the total or partial inability to hear sound in one or both ears.

 

 

Considerations

 

Minor decreases in hearing are normal after age 20. Hearing problems usually come on gradually, and rarely end in complete deafness.

There are many causes of hearing loss. They can be divided into two main categories:

 

Conductive hearing loss (CHL) occurs because of some mechanical problem in the external or middle ear. The three tiny bones of the ear (ossicles) may fail to conduct sound to the cochlea, or the eardrum may fail to vibrate in response to sound. Fluid in the middle ear can cause CHL

 

 

Sensorineural hearing loss (SNHL) results from a dysfunction of the inner ear. It most often occurs when the tiny hair cells (called cilia) that transmit sound through the ear are injured. This type of hearing loss is sometimes called "nerve damage," although this is not accurate.

 

CHL is often reversible -- SNHL is not. People who have both forms of hearing loss are said to have mixed hearing loss.

 

Screening for hearing loss is now recommended for all newborns. In children, hearing problems may cause speech to develop slowly.

 

Ear infections are the most common cause of temporary hearing loss in children. Fluid may linger in the ear following an ear infection. Although this fluid can go unnoticed, it can cause significant hearing problems in children.

 

Any fluid that stays longer than 8 - 12 weeks is cause for concern.

 

Preventing hearing loss is more effective than treating it after "the damage is done."

 

 

Causes

 

Genetic:

 

Osteogenesis imperfecta

Leopard syndrome (multiple lentigines)

Otosclerosis

Robinson type ectodermal dysplasia

Cockayne syndrome

Bjorn pili torti and deafness syndrome

Multiple synostosis syndrome

Hunter syndrome

Taybi oto-palato-digital syndrome

Hereditary nephritis

Mohr syndrome

Hurler syndrome

Waardenburg syndrome

Kartagener syndrome

Fronto-metaphyseal dysplasia syndrome

Morquio syndrome

 

 

Trisomy 13

 

 

Multiple lentigines syndrome

 

 

Treacher Collins syndrome

 

 

Stickler syndrome

 

 

Congenital:

 

Rubella syndrome

 

 

Congenital atresia of the external auditory canal

 

 

Congenital cytomegalovirus

 

 

Congenital perilymphatic fistula

 

 

Fetal methyl mercury effects

 

 

Fetal iodine deficiency effects

 

Infectious:

 

Meningitis

 

 

Mumps

 

 

Measles

 

 

Ear infection (otitis media)

 

 

Scarlet fever

 

Traumatic:

 

Traumatic perforation of the eardrum

 

 

Skull fracture (temporal bone)

 

 

Acoustic trauma such as from explosions, fireworks, gunfire, rock concerts, and earphones

 

 

Barotrauma (differences in pressure)

 

Toxic:

 

Aminoglycoside antibiotics

 

 

Ethacrynic acid - oral

 

 

Aspirin

 

 

Chloroquine

 

 

Quinidine

 

Age-related:

 

Age-related hearing loss (presbycusis)

 

Occupational:

 

Any occupation with exposure to loud noises on a continuous day-to-day basis can result in hearing loss due to nerve end damage. Increased attention to conditions in the work environment has markedly decreased the likelihood of work-related hearing loss. See occupational hearing loss

 

Other:

 

 

Temporary hearing loss can be caused by:

 

The build-up of wax in the ear canal

 

 

Foreign body lodged in the ear canal

 

 

Injury to the head

 

 

Allergy

 

 

Blocked Eustachian tubes

 

 

Scarred or perforated eardrum

 

 

Ear infections (chronic otitis externa, chronic otitis media, malignant otitis externa)

 

 

Reaction to medication such as aminoglycosides, chloroquine, quinidine

 

 

Home Care

 

Wax build-up can frequently be flushed out of the ear (gently) with ear syringes (available in drug stores) and warm water. Wax softeners (like Cerumenex) may be needed if the wax is hard and impacted.

Care should be taken when removing foreign bodies. Unless it is easy to get to, have your health care provider remove the object. Don't use sharp instruments to remove foreign bodies.

 

 

When to Contact a Medical Professional

 

Call your health care provider if:

 

Hearing problems are persistent and unexplained

 

 

Hearing problems adversely affect lifestyle

 

 

Hearing problems go along with other symptoms such as ear pain

 

 

What to Expect at Your Office Visit

 

The medical history will be obtained, and a physical examination performed.

 

Medical history questions documenting hearing loss in detail may include:

 

Is the hearing loss in both ears or one ear?

 

 

Is the hearing loss mild or severe?

 

 

Is all of the hearing lost (inability to hear any sound)?

 

 

Is there decreased hearing acuity (do words sound garbled)?

 

 

Is there decreased ability to understand speech?

 

 

Is there decreased ability to locate the source of a sound?

 

 

How long has the hearing loss been present?

 

 

Did it occur before age 30?

 

 

What other symptoms are also present?

 

 

Is there tinnitus (ringing or other sounds)?

 

 

Is there ear pain?

 

The physical examination will include a detailed examination of the ears.

 

Diagnostic tests that may be performed include:

 

Audiometry (an electronic hearing test )

 

 

Auditory response test

 

 

CT scan of the head (if a tumor or fracture is suspected)

 

 

X-ray of the head

 

 

Tympanometry

 

 

Caloric test

 

 

MRI of the ear (see MRI of the head)

 

A hearing aid or cochlear implant may be provided to improve hearing.

 

References

 

Rakel RE. Textbook of Family Practice. 6th ed. Philadelphia, PA: WB Saunders; 2005:443-444.