By Dr. Maddie Swannack and Dr. Sachin Sudhakaran
Next Lesson - The Nose
Core
This article follows on from our Anatomy of the Ear article.
Conditions of the External Ear
Foreign bodies within the ear canal are a common ear-related complaint in children. Children may not volunteer a history, so diagnosis can be delayed. A foreign body should be considered in any child with ear symptoms. Foreign bodies in the ears must be removed, but this should be done carefully to avoid pushing the object further in or causing more damage. Refer the child to a specialist if the object is difficult or dangerous to remove. Examples include a child who cannot be kept safely still, an object that is penetrating (piercing) or earlier attempts at removal that have failed. A button battery (a small, flat, round battery) in the ear canal is a surgical emergency and needs urgent removal.
Wax in the ears is normal. It only becomes pathological (a problem) if the wax has become so impacted that it causes symptoms or stops the ear from being examined. Hearing loss is the most common symptom. Impacted wax can also cause a feeling of fullness in the ear, ear discomfort or pain, tinnitus (ringing or other sounds in the ear), itching and dizziness. It can be softened with self-administered olive oil drops (or sodium bicarbonate 5% ear drops), used 3 to 4 times a day for 3 to 5 days. This helps the wax to fall out naturally. Ear drops should not be used if a perforated ear drum, active dermatitis or an active infection of the ear canal is suspected.
A pinna haematoma is the accumulation of blood between the cartilage of the pinna (the visible part of the outer ear) and its overlying perichondrium (the membrane that covers the cartilage). This is usually secondary to blunt trauma.
If left untreated the haematoma can cause increased pressure depriving the cartilage of its blood supply, eventually leading to necrosis. Prompt drainage is required, followed by a pressure dressing or splint (a bolster) to stop the blood collecting again. Without drainage and a pressure dressing, fibrosis (scar tissue) and asymmetrical cartilage development can lead to a ‘cauliflower deformity’. The ear should be rechecked after 24 to 48 hours.
Image - Haematoma of the pinna
Creative commons source by James Heilman, MD [CC BY-SA 4.0 (https://creativecommons.org/licenses/by-sa/4.0)]
Image - Cauliflower deformity caused by an untreated pinna haematoma
Creative commons source by MartialArtsNomad.com [CC BY-SA 4.0 (https://creativecommons.org/licenses/by-sa/4.0)]
Otitis externa is inflammation of the structure of the outer ear, which is typically the result of infection. Acute otitis externa is typically caused by bacteria (commonly Pseudomonas aeruginosa or Staphylococcus aureus). Persistent inflammation may be caused by fungal infection, for example with Aspergillus species or Candida albicans.
Risk factors include foreign bodies, swimming and placing cotton wool buds in the ear. Cotton wool buds and foreign bodies can damage the skin of the ear canal, and water from swimming reduces the protection that earwax gives against infection.
Patients may present with a painful ear canal with visible erythema (redness), oedema and serous exudate (clear, watery fluid leaking from the tissue). The pain is often worse when the pinna is pulled or the tragus (the small flap in front of the ear canal) is pressed. In severe cases there may be a conductive hearing loss (sound cannot pass through the outer or middle ear) due to stenosis (narrowing) of the ear canal.
The condition may be mild and self-limiting (it gets better on its own) in some cases, but many patients benefit from topical treatment (treatment applied to the ear). Management includes appropriate analgesia (pain relief), avoiding precipitating factors (things that trigger the problem), and topical ear drops such as acidifying or antimicrobial preparations. Patients with severe pain, spreading infection, poorly controlled diabetes or immunosuppression (a weakened immune system) require prompt medical review. Necrotising otitis externa (previously called malignant otitis externa) is a potentially life-threatening infection. It can spread from the ear canal to the temporal bone (the bone of the skull around the ear). If it is suspected, arrange emergency hospital admission or urgent referral to an ear, nose and throat (ENT) specialist.
Image - Otitis externa
Creative commons source by Klaus D. Peter, Wiehl, Germany [CC BY-SA 4.0 (https://creativecommons.org/licenses/by-sa/4.0)]
Ramsay Hunt syndrome (also known as herpes zoster oticus) is a disorder caused by the reactivation of the varicella zoster virus (the virus that causes chickenpox and shingles). The reactivation occurs in the geniculate ganglion (a cluster of nerve cell bodies) of the facial nerve. It is therefore ‘shingles of the facial nerve’.
Ramsay Hunt syndrome typically presents with unilateral paralysis of the muscles of facial expression. There is usually a painful vesicular (blistering) rash in or around one ear. Other features can include loss of hearing in that ear, a change in taste, and dry eyes and mouth.
Treatment is more effective if started early and includes antiviral and steroid medication. Prognosis is generally worse than in Bell palsy (sudden one-sided facial weakness with no identified cause). This is why early recognition matters.
Image - Typical facial nerve palsy and rash seen in Ramsay Hunt syndrome
Creative commons source by Worme M, Chada R, Lavallee L [CC BY-SA 4.0 (https://creativecommons.org/licenses/by-sa/4.0)]
Acute otitis media (AOM) is an infection of the middle ear. As discussed in our Anatomy of the Ear article, it is a common condition in children due to the flatter Eustachian tube (the tube linking the middle ear to the back of the nose). Enlarged adenoids (lymphoid tissue at the back of the nose) can also make infection more likely.
Common causative organisms include bacteria such as Haemophilus influenzae, Streptococcus pneumoniae, Moraxella catarrhalis and Streptococcus pyogenes, and viruses such as respiratory syncytial virus (RSV) and rhinovirus.
Patients usually complain of otalgia (pain in the ear). In children this may show as tugging at the ear, and there may also be fever and malaise (feeling generally unwell). On otoscopy (looking into the ear with an otoscope), the tympanic membrane (TM, the ear drum) may appear to be bulging and erythematous (red). There may also be air bubbles or a visible fluid level behind it. There may also be discharge from the ear if the ear drum perforates from the pressure inside the middle ear.
Treatment of acute otitis media is mainly symptomatic (aimed at relieving symptoms), with analgesics (painkillers) and antipyretics (fever-reducing drugs), for example regular paracetamol or ibuprofen.
Most children with acute otitis media get better within about 3 days without antibiotics. For most children, NICE advises considering either no antibiotic prescription or a back-up antibiotic prescription. A back-up prescription is an antibiotic to use only if symptoms do not start to improve within 3 days or if they worsen rapidly or significantly. Children who may be more likely to benefit from antibiotics are those of any age with otorrhoea (discharge from the ear), and those under 2 years of age with bilateral infection (infection in both ears). For these children, consider no prescription, a back-up prescription or an immediate antibiotic prescription. Offer an immediate antibiotic prescription to children who are systemically very unwell (unwell throughout the body), have signs of a more serious illness, or are at high risk of complications. Refer children to hospital if they have a severe systemic infection or acute complications such as mastoiditis (infection of the bone behind the ear), meningitis or facial nerve paralysis (Source). Always check your local guidelines.
Complications of AOM include the following. Facial nerve palsy, mastoiditis and meningitis are rare but serious.
- Perforation of the tympanic membrane +/- hearing loss
- Recurrence of infection
- Facial nerve palsy
- Mastoiditis
- Meningitis
Image - Otitis media. Note the erythematous (red) tympanic membrane, which is bulging outward, shown by the loss of the cone of light (the triangle of reflected light seen on a healthy ear drum). There is no fluid level visible, but that does not rule out infection. The yellow on the left is ear wax and is not pathological in this case.
Creative commons source by Michael Hawke MD [CC BY-SA 4.0 (https://creativecommons.org/licenses/by-sa/4.0)]
Perforation of the Tympanic Membrane
If the TM is damaged there is a risk that it may perforate. This can lead to otalgia and discharge from the ear, with possible sudden conductive hearing loss. Most cases of perforation heal on their own, usually within about 2 months. It is still recommended that suspected perforations be examined, and the ear should be kept dry until the ear drum has healed. Careful inspection is required to identify the location and extent of the perforation and if there are any signs of infection. In rare cases attic perforations (at the top) can lead to cholesteatoma.
If the hole does not heal (a chronic perforation is one that does not close within 3 months of acute otitis media), there may be a long-lasting effect on hearing. A chronic perforation can also predispose to chronic suppurative otitis media, a long-term inflammation of the middle ear with recurrent discharge. Chronic suppurative otitis media should be managed by an ear, nose and throat (ENT) specialist.
Perforations can be repaired surgically. This is usually considered once there is no infection present and when there is clinically significant hearing impairment or other ongoing problems such as recurrent infection.
Image - Perforated tympanic membrane, the perforation is visible just under the umbo (the centre of the ear drum, where the tip of the malleus attaches)
Creative commons source by Michael Hawke MD [CC BY-SA 4.0 (https://creativecommons.org/licenses/by-sa/4.0)]
Otitis media with effusion (OME), also known as glue ear, is a condition characterised by a collection of fluid in the middle ear without active infection.
The precise cause of the condition is unclear but is thought to occur when the Eustachian tube becomes blocked usually after an episode of AOM. As discussed in our Anatomy of the Ear article, the mucosa of the middle ear is constantly absorbing air. Usually this is not an issue as the Eustachian tube allows air to enter the middle ear from the nasopharynx (the part of the throat behind the nose). However, in OME the Eustachian tube becomes blocked and as a result the air in the middle ear cannot be replaced leading to a negative pressure within the middle ear. As a result, fluid is drawn into the middle ear and collects causing symptoms.
The condition is much more common in children due to the anatomy of the Eustachian tube. Many children get better on their own, but OME can impair hearing. This can cause delay in the development of language skills in children, so a child with suspected OME should be referred for formal assessment, including a hearing test. Signs in children can include these delays, or the child turning the television volume up very high.
On examination, the tympanic membrane is likely to be concave or retracted (pulled inwards, due to negative pressure), with fluid (and sometimes air bubbles) visible behind the surface. There is often a feeling of ‘fullness’ in the ear.
Risk factors for otitis media with effusion include increased colonisation (growth of bacteria) of the adenoids (lymphoid tissue at the back of the nose) or nasopharynx. Other risk factors include attending day care (where children are exposed to more infections), parents who smoke and Eustachian tube dysfunction.
Many cases resolve spontaneously over time, so after formal assessment most children are monitored first. Children with Down syndrome or a cleft palate are less likely to improve on their own and may be referred to a specialist in hospital. Antibiotics, antihistamines and decongestants (drugs that reduce nasal blockage) are not recommended for OME. If a child has OME with hearing loss in both ears, their hearing is reassessed after 3 months. Hearing aids can be considered for OME-related hearing loss, and grommets can also be considered. Grommets are inserted by an operation called a tympanostomy. This is the surgical cutting of the tympanic membrane (ear drum) to place a grommet (small plastic tube) through it. Grommets let air get in and out of the middle ear, which helps to equalise the pressure, and they also let fluid drain away. Grommets stay in the ear for around 6-12 months allowing time for the condition to resolve and the Eustachian tube to develop.
Image - Otitis media with an effusion. Notice the presence of straw coloured fluid through the tympanic membrane and the retracted appearance of the membrane
Creative commons source by Michael Hawke MD [CC BY-SA 4.0 (https://creativecommons.org/licenses/by-sa/4.0)]
Mastoiditis is a serious condition caused by the spread of infection from the middle ear cavity into the air cells (small air-filled spaces) of the mastoid bone (the bone behind the ear). The middle ear cavity is connected to the mastoid air cells via the mastoid antrum (an air-filled space that links the middle ear to the air cells). This provides a path for the spread of infection. From the mastoid bone, the infection can spread further and cause brain abscesses, meningitis or permanent hearing loss. The patient therefore needs prompt admission to hospital and treatment with IV (intravenous) antibiotics.
Mastoiditis presents classically with the pinna of the affected ear being pushed forward by a swelling over the mastoid process (posterior to the ear). There may also be symptoms similar to AOM along with erythema, tenderness, and pain over the mastoid process.
Image - Presentation of mastoiditis with a swollen and erythematous mastoid process pushing the pinna forwards
Creative commons source by B. Welleschik [CC BY-SA 4.0 (https://creativecommons.org/licenses/by-sa/4.0)]
A cholesteatoma is a rare condition caused by a collection of trapped epithelial cells. If left untreated the trapped cells can proliferate (multiply) and erode into surrounding structures, leading to bony destruction. For example, the mastoid bone, the petrous bone (the dense part of the skull base that holds the inner ear) and the ossicles (the three tiny bones of the middle ear) can be eroded. This can lead to hearing loss and infection. Cholesteatomas usually present with a persistent or recurrent, pus-containing and offensive-smelling discharge from one ear, and gradual hearing loss. Ear pain, tinnitus, vertigo or facial weakness can also occur, and facial weakness suggests advanced disease.
A cholesteatoma is thought to form when the pars flaccida (the floppy upper part of the ear drum) is pulled into the middle ear by negative pressure. This pressure is associated with Eustachian tube dysfunction (see above). This traps epithelial cells, which can then proliferate.
Untreated, cholesteatomas can lead to facial nerve palsy, meningitis and cerebral abscess. Any patient with suspicion of cholesteatoma should therefore be referred promptly to an ear, nose and throat (ENT) specialist. Emergency hospital admission or urgent specialist advice is needed if there is facial nerve palsy, neurological symptoms or signs, or the patient is systemically unwell (unwell throughout the body).
Image - Cholesteatoma following an attic perforation in the tympanic membrane
Creative commons source by Michael Hawke MD [CC BY-SA 4.0 (https://creativecommons.org/licenses/by-sa/4.0)]
Otosclerosis is a condition where abnormal bone growth affects the ossicles, most commonly fixing the stapes (the smallest ossicle, next to the inner ear) in place. As a result, vibrations aren’t transmitted to the cochlea (the hearing organ of the inner ear) as effectively, causing a conductive hearing loss. This can be associated with tinnitus (ringing in the ears) or vertigo (a spinning sensation), depending on the spread of the bony growth.
It is an important differential diagnosis in any young person presenting with conductive hearing loss.
When considering conditions of the inner ear, it is important to understand two key symptoms.
- Vertigo: the illusion of movement when no movement is present, for example feeling like the room is spinning, or feeling like the patient is on a ship.
- Tinnitus: the hearing of a sound that is not present, for example dripping or ringing.
Benign Paroxysmal Positional Vertigo (BPPV)
BPPV is a disorder characterised by repeated episodes of positional vertigo, meaning the symptoms occur following movement of the head.
It is caused by crystals of calcium carbonate (otoconia) that become dislodged from the utricle (a chamber of the inner ear that senses head position) and move into the semi-circular canals (fluid-filled loops in the inner ear that sense head movement). This causes the fluid to continue to shift after the movement of the head has stopped.
It presents with dizziness on head movement that resolves when the crystals settle (normally takes less than one minute). The dizziness can be accompanied by nystagmus (involuntary movement of the eyes), nausea and vomiting. BPPV does not affect hearing.
It is diagnosed using the Dix-Hallpike Test. The manoeuvre is conducted by first asking the patient to sit on the couch and asking the patient to keep their eyes open throughout the procedure. First, turn the patient’s head 45 degrees to one side. Then rapidly lie them down supine (over about 2 seconds, supporting the head and neck). The head should hang over the edge of the couch, extended by 20 to 30 degrees. Keep the head in this position for at least 30 seconds and observe the patient’s eyes. The test is positive if the manoeuvre provokes vertigo and torsional (rotating) upbeating nystagmus (the fast phase of the eye movement is upwards), usually after a short delay of 5 to 20 seconds. Then repeat with the head turned 45 degrees to the other side.
BPPV affecting the posterior semi-circular canal (the most common type) is treated using the Epley Manoeuvre. This is a series of movements of the head that help the crystals to move out of the semi-circular canals. The crystals then no longer make the fluid move when the head moves. It is carried out on the affected side, which is the side that gave the positive Dix-Hallpike test. Drug treatment is not usually helpful.
The Epley Manoeuvre begins with the Dix-Hallpike Manoeuvre, with the head turned 45 degrees towards the affected side. The patient ends up lying supine with their head turned to that side and hanging about 30 degrees over the edge of the couch. Maintain each head position for at least 30 seconds. If vertigo continues, wait until it has subsided. Then, keeping the neck extended, rotate the head 90 degrees to the other side. Next, ask the patient to roll onto the same side that they are now facing. They should look obliquely down towards the floor, with the nose about 45 degrees below the horizontal. Finally, help the patient to sit up sideways with the head still turned and tilted towards the side they are facing. Then bring the head to the central position and tilt the chin down by about 45 degrees.
Meniere’s disease is a condition of excess fluid in the ducts of the inner ear. The exact cause is unknown, but possible associated factors include viral infection, head trauma and autoimmune conditions.
It presents with a fluctuating sensorineural hearing loss (hearing loss caused by damage to the inner ear or the hearing nerve), as well as tinnitus and vertigo. The hearing loss helps to differentiate it from BPPV, as there is no hearing loss in BPPV.
There is no specific diagnostic test for Meniere’s disease, so the diagnosis is made from the typical clinical features. Diagnosis is important because the condition can contribute to falls, can affect the ability to work or drive, and may have psychological effects (it can contribute to anxiety and depression).
Labyrinthitis and Vestibular Neuronitis
These are conditions where inflammation affects the inner ear (labyrinthitis) or cranial nerve VIII, the vestibulocochlear nerve (vestibular neuronitis). They are usually caused by a viral infection, although a bacterial cause is possible. In labyrinthitis, the labyrinth (the fluid-filled organ of hearing and balance in the inner ear) is inflamed and this typically causes hearing loss, and sometimes tinnitus, as well as vertigo. In vestibular neuronitis, the vestibular part of the nerve (the part that carries balance signals) is affected, so vertigo occurs without hearing loss or tinnitus. Clinically, they are similar in cause and management.
They present with acute and persistent vertigo that starts spontaneously, without a trigger, and is present even when the head is still (although head movement makes it worse). Hearing loss and tinnitus can occur in labyrinthitis but not in vestibular neuronitis.
Both conditions are generally self-limiting (they get better on their own), but anti-nausea drugs may be needed. These should be taken for no more than about 3 days, as longer use may delay recovery. Vertigo can also have a central (brain) cause such as a stroke, so patients with atypical symptoms (for example additional neurological symptoms) need further assessment.
An acoustic neuroma (also called a vestibular schwannoma) is a benign tumour of the vestibulocochlear nerve (the nerve for hearing and balance). It is rare, but it should be considered in anyone with gradual unilateral sensorineural hearing loss that has no outer or middle ear cause. Routine referral to an ear, nose and throat (ENT) service should be considered.
Presbycusis is age-related, progressive, bilateral sensorineural hearing loss, mainly affecting high-frequency (high-pitched) sounds.
Persistent exposure to loud noise can cause hearing loss. It can be occupational in origin, for example in people who work with machinery such as power tools without adequate ear protection. The hearing loss may be temporary or permanent, depending on the intensity and duration of the exposure.
Edited by: Dr. Ben Appleby
Reviewed by: Dr. Thomas Burnell and Dr. Marcus Judge
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