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2.12.3 Ear Pain and Hearing Loss
2.12.3 Ear Pain and Hearing Loss
https://doi.org/10.1093/med/9780199665549.001.0001
Published: 2016 Online ISBN: 9780191810817 Print ISBN: 9780199665549
CHAPTER
Abstract
The pitfall for the non-ear specialist is to think that ear symptoms can only be due to ear disease—this
is far from the case. Due to its complex nerve supply, pain and paraesthesia often have an origin away
from the ear but symptoms are referred to the ear; consequently earache in the presence of a normal
eardrum is due to a malignancy until proven otherwise. In additional to referral to the ear of symptoms
from regional disease, systemic disease can also present with earache, a good example of which is
Wegener’s granulomatosis and the painless deafness associated with vitamin D de ciency. While
unrelenting pain with aural discharge can be a presenting symptom of life-threatening condition such
as osteitis of the skull base or a malignancy of the ear, unrelenting discharge without pain can also be a
sign of life-threatening disease such as cholesteatoma, tuberculosis, and tertiary syphilis. Assessment
of the patient with an ear problem should include a thorough history to include symptoms of systemic
and regional disease as well as auditory and neural symptoms. Examination should include the ear but
also the head and neck and upper respiratory mucosal surfaces as well as looking for signs of systemic
disease. Investigation should include assessment of the hearing, search for systemic conditions and
local imaging of the head and neck. Treatment of hearing loss is not just con ned to hearing aids but
advice should be given on prevention, and the use of environmental aids or surgery considered.
Keywords: otalgia, deafness, hearing aid, cochlear implant, bone anchored hearing aid, petrous temporal
bone, tumour, malignant otitis externa, infection
Subject: Surgery, Urology, Paediatric Surgery, Cardiothoracic Surgery, Peri-operative Care, Trauma and
Orthopaedic Surgery, Upper Gastrointestinal Surgery, Colorectal Surgery, Surgical Oncology, Neurosurgery,
Breast Surgery, Transplant Surgery, Vascular Surgery, Surgical Skills
Series: Oxford Textbooks in Surgery
Ear pain
In addition to redness and swelling, moving the external ear exacerbates the pain and as such is a useful way
In the early stages of a herpetic infection there may be little to see until vesicles appear.
Malignant otitis externa, on the other hand, usually presents in the immunosuppressed or the elderly.
Initially pain with pus in the external auditory canal is the cardinal feature, which after a number of weeks
progresses to the development of cranial nerve palsies, VII, IX, X, XI, and eventually XII. Bone destruction is
clear on computed tomography (CT) scanning and a biopsy is required to exclude cancer.
Chronic pain accompanied by signs of in ammation can be due to chronic suppurative otitis media, though
this condition is not characterized by severe pain, more a grumbling ache. Rarer in ammatory conditions
such as Wegener’s granulomatosis can present with severe relentless pain. Malignancy of the middle ear is
rare and usually associated with many years of discharge and infection.
Primary tumours of the bone itself are rare, and so tumours that present with pain are nearly always
secondary tumours usually of breast, prostate, and lung cancer. The more common benign tumours
associated with petrous bone (e.g. paragangliomas, neuromas, schwannomas, and meningiomas) do not
cause pain.
Osteitis arising from malignant otitis externa or middle ear suppuration is becoming increasingly common
in the UK as the population ages.
It is really important, however, not to miss tumours of the upper aerodigestive tract. Common examples
include carcinoma of the tongue, tonsil, oropharynx, hypopharynx, larynx, and oor of mouth.
Investigation of earache
After taking a thorough history, a full ear, nose, and throat examination should take place. If the ear is
normal, a bre optic examination of the upper aerodigestive tract is mandatory. The tongue should be
bimanually palpated and neck lumps searched for. The mouth should be carefully examined, paying
particular attention to the back and sides of the tongue and the retromolar trigone.
In cases that do not follow the pattern of the typical upper respiratory infection, additional tests should be
carried out: an erythrocyte sedimentation rate, immunoglobulin electrophoresis, and autoantibody assays
(including the antinuclearcytoplasmic antibody).
Finally, in cases of intractable pain in which no diagnosis has been found after blood tests and scanning,
removing the tonsil on the side of the ear pain and blind biopsies of the tongue and oropharynx should be
considered, especially in those at high risk for cancer such as smokers and alcohol abusers.
◆ wax
◆ exostoses
◆ foreign body
◆ rare disorders are: congenital absence or stenosis of the external auditory canal, and tumours.
Painless chronic discharge can be due to cholesteatoma, which is basically a ‘cyst’-like structure containing
dead skin within the middle ear cleft. They can occur just with a conductive hearing loss and no discharge
but the majority become infected. With time the disease destroys middle ear structures such as the ossicles
and eventually the inner ear. They can present with a facial palsy.
Otosclerosis is an inherited disorder that usually presents with a conductive deafness and an otherwise
normal ear on clinical examination. It is progressive, usually a ecting both ears, and eventually will a ect
the cochlea causing a sensory deafness.
Endocrine disorders rarely a ect the ear; however, rickets and osteomalacia may occur, usually presenting
in an Asian patient with a conductive loss and tinnitus.
In ammations such as vasculitis, Wegener’s granulomatosis, and sarcoidosis all occur but are rare.
Surprisingly rheumatoid arthritis does not appear to a ect the synovial joints of the ossicles to any extent.
Speci c infections such as tuberculosis and syphilis occur but are rare, as are tumours.
Infections
Sudden hearing loss due to herpetic infection is quite common. Bacterial meningitis is another major cause
of total loss of hearing. Other infections are rare but important to spot as they may be treatable (e.g.
penicillin for tertiary syphilis).
Inflammations
The same in ammations that a ect the middle ear can a ect the inner ear, such as sarcoidosis and
Wegner’s granulomatosis.
Endocrine
Pendred’s syndrome or deafness associated with an underactive thyroid in childhood can cause a severe
sensorineural deafness. Diabetes mellitus does not cause deafness directly but it may be part of inherited
syndromes such as DIDMOAD syndrome where it is joined by diabetes insipidus. It can cause hearing loss
through the mechanism of stroke.
Vascular causes
The primary vascular cause of sensory deafness is vasculitis. Thrombosis can cause sudden deafness and is
typically seen in young women on the oral contraceptive pill.
Iatrogenic
Apart from surgery, the primary cause of deafness inadvertently initiated by doctors is through ototoxic
drugs (usually aminoglycosides) but occasionally through diuretics and certain chemotherapy agents used
in oncology. A common group that are vulnerable to iatrogenic damage are premature babies.
Tumours
Most tumours that cause deafness are benign with vestibular schwannoma and meningioma being the most
common. Both are very slow growing and usually present with a VIII nerve isolated partial palsy.
Trauma
This is the same as for the middle ear, with the exception of barotrauma where the bends can cause sudden
sensory deafness but this is rare.
The corner stone of investigating hearing loss is the pure tone audiogram. Inner ear function can be tested
in isolation using a bone conductor. As humans have two ears it is possible for the non-test ear to be the one
that is hearing the test thresholds and not the ear that the audiologist thinks they are testing. To avoid this
problem, masking noise has to be played to the ear that is not being tested. The bone conduction gives the
sensorineural hearing loss. However, the pure tone audiogram cannot tell whether the deafness is due to a
cochlea problem (sensory) or a nerve of hearing problem (neural).
Sensory deafness is characterized by a reduction in the dynamic range of hearing. In a normal person the
threshold of hearing is between 0 and 10 dB and the threshold for pain is about 90 dB to 100 dB. The
di erence between threshold for hearing and threshold for discomfort is known as the dynamic range. In
the normal ear it is typically about 90 dB. In a sensory deafness it can be much reduced. The patient may
notice that a small change in the volume of the sound now produces a marked increase in loudness
compared to when they had normal hearing. This is seen in deafness of old age (presbycusis). An example of
which is a nurse asking an elderly person ‘Do you want a cup of tea dear,’ and the elderly person saying
‘Pardon, you’ll have to speak up.’ The nurse raises her voice slightly and the patient then says ‘You don’t
have to shout, I’m not deaf you know!’
In neural deafness there is abnormal facilitation of sound. A normal-hearing person should be able to hear a
tone at 5 dB above threshold for a minute. Those who require the test sound to be between 5 dB and 15 dB
above threshold to hear the tone for a minute are borderline, and those that require it to be greater than
20 dB above threshold to hear a sound for a minute probably have a neural deafness. There are a number of
tests that are based on the two phenomenon of reduced dynamic range and abnormal facilitation that can be
used to di erentiate between neural and sensory deafness (e.g. tone decay and loudness discomfort levels).
In practical terms though, when a patient presents with asymmetric hearing these days they get an MRI
scan in most Western countries.
MRI scans are useful at picking up soft tissue disease (e.g. a vestibular schwannoma) that may be causing a
hearing loss. CT scans are useful for detecting bone destruction (e.g. in osteitis) or bone disease (e.g.
otosclerosis).
If vasculitis is suspected then an erythrocyte sedimentation rate is a useful screening test before going on to
look at autoantibodies.
If a bone disease is suspected then calcium, phosphate, and alkaline phosphatase assays should be
Treating hearing loss is not just the provision of a hearing aid. Before prescribing a hearing aid advice
should be given.
It is important to provide advice in order to avoid further loss. This could be advice on hearing protection in
those that are exposed to noise. Infections can be reduced in those with otitis externa and a perforation of
the eardrum can be avoided by keeping water out of the ear. Hygiene advice may be appropriate for those
patients that have to wear hearing protection.
Careers advice is often essential, particularly in those of school and working age. If someone is 20 years old
and has a progressive condition such as otosclerosis then working in an industry with strict hearing criteria
may not be sustainable in the long term. Examples would include the armed forces, working on rail track, or
in the o shore oil industry.
Middle ear e usion, which is common in childhood, can be treated surgically. Current NHS guidelines
require the child to have a hearing loss in both ears present for at least 3 months. If this guideline is met
then ventilation tubes can be inserted into both ears. Tubes may be also inserted to prevent recurrent
earache. An exception to this guideline is children with trisomy 21 in which a hearing aid is recommended.
The e ects of chronic suppurative otitis media and trauma due to blast or penetrating trauma can also be
treated surgically. Myringoplasty or grafting holes in the eardrum has a success rate between 70% and 95%,
with those cases due to trauma having the better results. An ossiculoplasty can be o ered where there is
ossicular discontinuity. If not too badly damaged, the patient’s own ossicles can be used or alternatively an
arti cial ossicle can be used. These are usually made from hydroxylapatite or titanium. Provided the stapes
superstructure is still present, success rates of 70% are common; however, the success rate drops to less
than 30% when the stapes superstructure is absent.
The stapes is xed in otosclerosis, causing a conductive loss. This can be corrected by replacing the stapes
with a prosthesis. There are many di erent designs on the market but the Fisch 0.4 mm Te on/steel
prosthesis gives good results with a low rate of incus necrosis as a late complication.
In patients that cannot wear a conventional hearing aid and with reasonable cochlear function a bone
anchored hearing aid can be used. This clips onto a permanently positioned titanium ‘bolt’ that is screwed
into the skull.
While a vestibular schwannoma can be removed surgically, most patients opt for stereotactic radiosurgery
due to its high success rate and low morbidity. The treatment does not, however, prevent further hearing
loss but it does allow the patient to retain some hearing in the a ected ear at least for a while.
For those patients with no hearing in both ears, and who have failed a trial of conventional hearing aids, a
cochlear implant may be an option. As a general rule they are not suitable for those born profoundly deaf,
with no recollection of sound, and are over 5 years of age. Children born profoundly deaf do best when
implanted under the age of 2.
One group that require urgent referral to a cochlear implant team are those deafened by meningitis.
Meningitis cases often have pus in the cochlear duct. This can organize and ossify, making it impossible to
get an implant inserted into the cochlea.
One downside to cochlear implants is that MRI scanning is restricted. Before ordering an MRI scan in a
Monopolar diathermy is usually contraindicated in the presence of a cochlear implant; however, if it has to
be used advice can be obtained from your local implant team on where to place the diathermy pad to
minimize risk of damage to the implant. Bipolar diathermy can be used safely below the clavicles, but if it
has to be used in the head advice should be obtained from your implant service.
Many patients derive considerable bene t from learning lip reading skills.
The profoundly deaf child does not have to be brought up with aids or a cochlear implant; some families may
choose for their child to learn sign language. In the UK the dominant sign language is British Sign Language
(BSL). Most hospitals and local authorities have access to BSL interpreters for important meetings and
hospital appointments.
Environmental aids can also be very helpful to the deaf and hard of hearing. Examples are lights that ash
when the doorbell is pushed or the telephone rings. Other gadgets include vibrating alarm clocks and voice
®
apps for use with smart phones and electronic note pads such as the iPad . A useful website is
www.sarabec.com, where details on a wide range of devices for the hard of hearing and the deaf can be
found.
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