Hearing loss

Structure of the ear

The ear - normal hearing

The ear is roughly divided into three parts. The outer (external) ear includes the part you can see (called the pinna) and the narrow tube-like structure (the ear canal), which your health professional can look down with a torch.

At the end of the canal is the eardrum. This separates the external ear from the middle ear.

Patient picks for Hearing problems

Ear, nose and throat

Can deaf people have therapy?

The NHS has spent the best part of 15 years promoting 'talking therapies' as part of its Improving Access to Psychological Therapies (IAPT) programme. But the Deaf community - statistically at high risk from social isolation - has been almost entirely locked out.

by Ellie Broughton

Ear, nose and throat

Ménière's disease

Ménière's disease typically causes attacks of dizziness with a spinning sensation (vertigo), hearing loss and noises in the ear (tinnitus). The attacks can vary in severity and in how often they occur. You may develop permanent hearing loss and/or permanent tinnitus in some cases. There is no cure but treatment can help to ease and prevent symptoms. If you are a driver, you must stop driving if Ménière's disease is diagnosed. The Driver and Vehicle Licensing Agency (DVLA) will permit driving again if there is good control of symptoms.

by Dr Laurence Knott

The middle ear is an air-filled compartment. Inside it are the three smallest bones in the body, called the malleus, incus and stapes which are all connected.

The air space of the middle ear connects to the back of the nose by the Eustachian tube.

Inner ear diagram

The inner ear is made up of two fluid-filled components, the cochlea (hearing) and the vestibular (balance) system. Both are lined with special sensory cells which transform sound waves into electrical signals. The cochlea is attached to a nerve that leads to the brain. Both the cochlea and the vestibular system are connected to a nerve which carries electrical signals to the brain.

How do we hear?

Sound waves are created when air vibrates. To hear, the ear must change sound into electrical signals which the brain can interpret. The outer part of the ear (the pinna) funnels sound waves into the ear canal.

When sound waves reach the eardrum they make it vibrate. Vibrations of the eardrum make the tiny bones in the middle ear move too. The last of these bones (the stapes) passes on the vibrations to the fluid-filled chamber called the cochlea.

When the vibrations reach the cochlea, the fluid inside it moves. As the fluid moves it vibrates the hairs on the cells that line the cochlea. Each cell is stimulated by a particular note (or frequency) of sound.

The vibration of the hair cells is turned into an electrical signal which is sent along the hearing (auditory) nerve to the brain. Special areas in the brain receive these signals and translate them into what we know as sound.

What causes hearing loss?

Damage to any part of the ear can cause hearing loss.

Conductive hearing loss

If there is a problem in the ear canal or the middle ear, this causes what is known as a conductive hearing loss. In conductive hearing loss, the movement of sound (conduction) is blocked or does not pass into the inner ear.

This often arises due to the presence of wax or fluid in the middle ear, although it may also be caused by a burst (ruptured) eardrum or by thickening of the bones in the middle ear known as otosclerosis (see below).

Sensorineural hearing loss

If the inner ear organ of hearing, the cochlea, or the hearing nerve is not working properly this causes a sensorineural hearing loss.

Usually this means that hair cells in the cochlea are not working properly or there is a problem with the hearing nerve so that some or all sounds are not being sent to the brain.

It does not usually affect the entire range of sound frequencies, at least not at first. Sensorineural hearing losses are usually permanent.

They can be mild, moderate, severe or profound and affect one or both ears. It is also possible for sensorineural and conductive hearing losses to occur together in a mixed hearing loss.

Causes of conductive hearing loss: the eardrum and ear canal

Blockage of the ear canal

The most common cause of blockage in the ear canal is wax. Removal of ear wax is advised if it is contributing to hearing loss or other symptoms. Usually softening treatments, such as olive oil ear drops, are advised first to see if using these cause the wax to come out of the canal without further treatment. However, if it remains, wax removal can be performed by an appropriately trained healthcare professional using water or suction. See the separate leaflet called Earwax for more details.

Something in the ear canal

Objects that shouldn't be there (foreign bodies) are most often found in the ears of children. Peas, beads or small pieces of a toy are the most common foreign bodies to block the ear and affect hearing.

It is usually best to have the object removed. This is done either by syringing it out with warm water or with a special extracting device. Smaller objects can also be removed using suction.

Otitis externa

Sometimes the skin of the ear canal can become inflamed. This may be caused by infection, allergy or other causes. Common symptoms include itch, ear discharge and dulled hearing. It is treated with ear drops. See the separate leaflet called Ear Infection (Otitis Externa) for more details.

Perforated eardrum

A torn (perforated) eardrum is not usually serious and often heals on its own without any complications. It may cause hearing loss or repeated ear infections, in which case a small procedure to repair it is an option. See the separate leaflet called Perforated Eardrum for more details.

Scarred eardrum

Scarring of the drum is usually due to repeated perforation, either by infection or due to previous grommet surgery. A grommet is a small tube which is inserted into the eardrum to ventilate the middle ear where there has been accumulation of fluid. See the separate leaflet called Otitis Media with Effusion (Glue Ear) for more details. .

Cholesteatoma

Cholesteatoma is an uncommon condition where a growth develops in the ear. It can be present at birth but usually it occurs as the growth of an abnormal collection of skin cells inside the ear.

The most common symptoms are hearing loss and a smelly discharge from the ear. See the separate leaflet called Cholesteatoma for more details.

The need for an eardrum

Part of the job of the eardrum is to boost (amplify) sound. Without the eardrum the sound will still reach the middle ear; however, it will not be as loud. Its other job is to seal off the middle ear and prevent it from damage by water and soap.

Causes of conductive hearing loss: the middle ear

The middle ear consists of an air space and the three small hearing bones (ossicles). Conduction of sound through the middle ear depends upon both of these.

A small tube, the Eustachian tube, connects the middle ear to the back of the nose and throat. The tube allows air in and out. This allows the air pressure inside the ear to equalise with that outside and replaces air that gets absorbed by the cells lining the middle ear.

The middle ear system can therefore be affected by problems affecting the Eustachian tubes and the middle ear space itself and by problems affecting the hearing bones.

Conditions affecting the air space

Eustachian tube dysfunction
When your Eustachian tube isn't working properly this can dull your hearing. It is usually a temporary problem that lasts a week or so and most commonly happens during or after a cold. The middle ear can fill with fluid. This is called a middle ear effusion. See the separate leaflet called Eustachian Tube Dysfunction for more details.

Glue ear
Glue ear arises when glue-like liquid from a middle ear effusion does not clear without treatment. An operation to clear the fluid and put in a tiny tube (grommet) to allow air to get into the middle ear may be advised if glue ear persists. Temporary use of hearing aids may be an alternative option to grommet insertion. Glue ear is more common in children due to the size and position of the inner ear structures, particularly the eustachian tube. If glue ear occurs only on one side, particularly in adults, further investigation by an ear specialist may be needed to ensure there is no blockage at the back of the nose causing the glue ear. See the separate leaflet called Glue Ear for more details.

Ear infection
Ear infections are very common, particularly in children. The main symptoms are earache and feeling unwell but it can cause temporary hearing loss. See the separate leaflet called Ear Infection (Otitis Media) for more details.

Conditions affecting the hearing bones

Otosclerosis
Otosclerosis is a cause of hearing loss in young people. It mainly affects the third of the three bones in the middle ear (the stapes). It causes gradual hearing loss. Treatments include hearing aids and surgery. See the separate leaflet called Otosclerosis for more details

Causes of sensorineural hearing loss: the cochlea

Hearing loss affecting older people (presbyacusis)

The most common cause of hearing loss is age-related. Most people over the age of 60 develop hearing loss to some degree.

The exact cause is not known but it is thought to be due to the cells in the cochlea becoming damaged over time. A hearing aid may be needed to amplify sound.

Loud noise damages the cochlea and can result in permanent hearing loss and ringing in the ears (tinnitus). The risk is based on how loud the noise is and how long you have been exposed to it.

Those who work with loud equipment - people who shoot, use pneumatic drills or operate heavy machinery - should always wear their protective ear-wear in order to prevent long-term damage. If you work with loud machinery or other equipment that is noisy, it is a legal requirement for your employer to provide hearing protection.

The cumulative effect of prolonged exposure to loud noise speeds up the process of hearing loss. Loud noise from music gigs is thought to be the reason why hearing loss is increasingly affecting young people.

If you have ever experienced ringing in your ears or dull hearing after listening to music, it was too loud. Many musicians also now wear ear filters to protect their hearing whilst playing.

Other causes of cochlear damage

The cochlea can be damaged by a severe head injury. Such trauma can also disrupt the tiny ear bones (the ossicles). Both mechanisms can cause hearing loss..

Some infections may damage the hearing nerves and/or the cochlea. These include measles, mumps, bacterial and viral meningitis and tuberculosis. The zoster virus, which causes shingles, can affect the hearing nerves. In all of these cases the resulting hearing loss can be permanent.

The cochlea is also vulnerable to damage by poisonous substances (toxins). This includes some medicines which can damage the hearing. Certain antibiotics are known to carry a risk of this but they may still be used where nothing else will work, particularly if a person's life is at risk. The small risk of hearing nerve damage is outweighed by the need for the medicine. One example is gentamicin, an antibiotic which is used in severe infections by particular germs (bacteria), especially in babies. A baby who is treated with gentamicin may require a hearing nerve test once they have recovered from the infection.

Ménière's disease
Ménière's disease causes attacks of vertigo, tinnitus and hearing loss. See the separate leaflet called Ménière's Disease for more details.

Causes of sensorineural hearing loss: the auditory nerve and the brain

Conditions affecting the hearing (auditory) nerve

Acoustic neuroma
An acoustic neuroma is a rare non-cancerous growth on the hearing nerve inside the skull. If it causes hearing loss, this usually affects just one ear. See the separate leaflet called Acoustic Neuroma for more details.

Conditions affecting the brain
Ultimately, sound is heard and interpreted by the brain; conditions that affect the hearing centre in the brain and the associated hearing nerve pathways can also cause hearing loss.

Examples are brain injury through trauma, stroke, brain infection (encephalitis) and multiple sclerosis. In some cases there might be partial or even complete recovery over time, although in other cases the loss is permanent.

Congenital hearing loss

Congenital hearing loss is hearing loss present at birth. Most cases are inherited but about 1 in 4 cases are due to events that happen to the baby in the womb.

This includes infections (such as rubella or cytomegalovirus), being premature or not getting enough oxygen at birth. Microtia, which can also cause hearing loss, is a condition in which the ear is underdeveloped or doesn't develop at all.

There are many inherited (genetic) syndromes which may cause hearing loss. These include Down's syndrome, Treacher Collins' syndrome and Waardenburg's syndrome. They may have their effects through the mechanisms described in this leaflet, although in some cases they are due to abnormal development of the hearing apparatus in the womb.

Some inherited hearing loss in children is not present at birth but develops in the early years. The first sign can be poor speech development. In the UK all newborn babies have a hearing test to make sure the hearing (auditory) nerve is working properly.

Most babies are checked again at about 8 months, although a formal assessment of hearing is not carried out at this stage. Families are encouraged to talk to health visitors or doctors if their child's speech seems delayed or if parents are concerned their child seems less responsive to sound; most cases of hearing loss are detected fairly quickly.

In many cases of hearing loss treatment to improve hearing is possible. This might involve hearing aids, cochlear implants or tiny tubes called grommets. In cases of profound deafness, where hearing cannot be improved, children and their families are taught how to use sign language from a very early age in order to help communication.

Hearing loss in one ear

The most common causes of hearing loss, such as age-related hearing loss, tend to affect both ears. However, this is often unequal, with the hearing loss of the ears differing from one another. Some people will have hearing aids fitted to just one ear, whereas others will choose to aid both ears.

Loss of hearing in just one ear is sometimes called single-sided deafness (SSD). If hearing loss is seen only on one side, or there is a significant difference in the hearing on the two sides, a scan (MRI) of the inner ear structures may be recommended, to look for an underlying structural cause for this difference. However, in the majority of cases, this scan is normal and there is no underlying cause found.

Being deaf in one ear presents particular challenges:

• It affects directional hearing - that is, the ability to work out which direction sound is coming from. This is also called spatial hearing. If you have normal hearing, you use the time difference in a sound arriving at each ear and the difference in loudness between two ears, to work out where a sound is coming from and how far away it is. If you have significant hearing loss in just one ear this makes it much harder to localise a sound. For example, hearing someone call you outside or hearing whether a car is coming when you are about to cross the road.

• When a sound is coming from your affected side, your head gets in the way of the sound getting to your good ear - the head shadow effect. This is particularly noticeable with higher-pitched sounds such as the c, f, p, s, t, ch and sh sounds, making it harder to discriminate between some words. This is why people with single-sided hearing loss have problems hearing speech when there is background noise, even when the hearing is normal in their other ear.

If you are affected by one-sided hearing loss, here are some coping strategies that may help:

• Don't be afraid to tell people that you are deaf in one ear or that you hear much better in one ear than in the other.

• Always make sure people are seated or walk on your good side.

• If there is background noise coming from only one direction, position yourself so that the noise next to your bad ear.

• If your affected ear is on the passenger side of your car, seating someone behind you rather than next to you may help.

• If you are going to a meeting, arrive early enough to choose a seat so that most people are on your good side. (This is easier at a rectangular rather than a round table - for example, in the corner with your back to a wall.)

• Remind your friends, family, teachers and co-workers not to expect you to answer if they call your name in a crowded place or across a road; otherwise they may think you are brusque or aloof and ignoring them.

• Keep your mobile phone in your pocket, as locating it when it rings can be very hard.

• Use a mono-splitter to listen to stereo music.

• Enjoy being able to sleep in a noisy environment by sleeping on your 'good' side!

Bone-anchored hearing aid (BAHA) and contralateral routing of signal (CROS) hearing aids are specifically designed for people with one-sided hearing loss. They pipe the sounds from your 'bad ear' side to your good or better side.

Protecting your hearing

The best way to protect your hearing is to avoid exposure to very loud noise completely and to limit the amount of time you are exposed to loud noise.

Discussing your hearing loss with your doctor

If you think you are losing hearing then discuss this with your GP. You may notice difficulty distinguishing what people are saying, or that everything seems quieter. Others may comment that you have the TV turned up very loud. You have difficulty hearing specifically in the presence of background noise. Perhaps you have been exposed to loud noise in the past.

Your doctor may ask you the following questions:

• Has your hearing worsened suddenly or gradually? Sudden deafness is uncommon but can be serious and you may need to see a hearing specialist urgently.

• Are both ears affected? Clearly ear infections, glue ear and earwax may be one-sided or two-sided. However, if you have severe hearing loss and wax is present in the ear canals then the wax needs to be cleared. After this the ear needs to be reassessed in case the wax was not the only reason for hearing reduction.

• Do you have associated sinus problems/congestion? If so glue ear is very likely. This may be present following a cold, when mucus remains in the middle ear space for several weeks. It may also be associated with allergic conditions such as hay fever.

• Have you had persistent exposure to loud noise in the past?

• Do you have particular difficulties with hearing conversation?

• Is there a family history of hearing loss? Some causes of deafness run in families, particularly otosclerosis.

Your doctor may perform some tests on your hearing in the surgery to decide whether your hearing loss is conductive, sensorineural, or mixed. These tests are undertaken using a small metal fork-like structure called a tuning fork. Your ears will be examined for wax and for obvious problems affecting the eardrum. They may then refer you for formal hearing tests. See the separate leaflet called Hearing Tests for more details.

Summary

There are many causes of hearing loss: the most common in younger people is otosclerosis, whereas the most common in older people is hearing loss of older people (presbyacusis). Many people have mixed hearing loss: you may have hearing loss of older people and earwax (cerumen) for instance, or otosclerosis and earwax.

If you experience sudden loss of hearing in one or both ears, you should seek urgent medical attention via your GP (or the out of hours services if the GP practice is closed) as soon as possible, ideally within 24 hours. Depending on the findings at the assessment, a course of steroid tablets may be recommended to try to improve the hearing. Further assessment and investigations via an ear service in the hospital may also be needed. However, most conditions causing hearing loss are treatable and very few need urgent management.

Dr Mary Lowth is an author or the original author of this leaflet.