40 The ear

GRANT J.E.M. BATES

Surgical anatomy of the ear

The external ear consists of the pinna and the ear canal. The pinna is made of yellow
elastic cartilage covered by tightly adherent skin. The external and middle ear develop
from the first two branchial arches. The external ear canal is 3 cm in length, the outer
two-thirds is cartilage and the inner third is bony. The skin on the lateral surface of the
tympanic membrane and the inner two-thirds of the ear canal is highly specialised. It
does not simply shed like the skin from the rest of the body. It migrates outwards from
the tympanic membrane and along the ear canal. As a result of this migration most
people’s ears are self-cleaning. Disorders of skin migration can result in ear disease
(e.g. cholesteatoma). The external canal is richly innervated and the skin is tightly
bound down to the perichondrium so that oedema in this region results in severe pain.
The lymphatics of the external ear drain to the retro auricular, parotid, retropharyngeal
and deep upper cervical lymph nodes.
The middle ear contains the ossicles. Laterally it is bounded by the tympanic
membrane, medially by the cochlea, anteriorly by the eustachian tube and posteriorly
it communicates with the mastoid air cells (Fig. 40.1). Entwined in this tiny space is
the facial nerve which pursues a tortuous course through the middle ear and exits the
skull base at the stylomastoid foramen. Knowledge of the anatomy of the middle ear
is important because infection can spread through it to the cranial cavity which lies
millimetres away.
The tympanic membrane has three layers: an inner mucosal layer, a dense fibrous
middle layer and the outer stratified squamous epithelium (skin). The upper portion
that lies above the lateral process of the malleus is called the pars flaccida. The lower
portion, making up the majority of the drum, is called the pars tensa (Fig. 40.2).
The tympanic membrane and ossicles act as a transformer system converting
vibrations in the air to vibrations within the fluid-filled inner ear (perilymph).The
evolution of the middle ear is interesting. Fish do not have one, whereas amphibians
(e.g. salamanders) have a single strut for an ossicle. At an air—water interface there is
a 30 decibels loss of sound energy. The mammalian middle ear overcomes virtually all
of this potential loss of sound energy.
The inner ear comprises the cochlea and vestibular labyrinth (saccule, utricle and
semicircular canals). These structures are embedded in dense bone called the otic
capsule. The cochlea is a minute spiral of two and three-quarter turns. Within this
spiral, perilymph and endolymph are partitioned by the thinnest of membranes
(Reissner’s membrane). The endolymph has a high concentration of potassium similar
to intracellular fluid, and the perilymph has a high sodium concentration similar to
extracellular fluid. Maintenance of the ionic gradients is an active process and is
essential for neuronal activity.
There are approximately 15 000 hair cells in the human cochlea. They are arranged in
rows of inner and outer hair cells. The inner hair cells act as mechanicoelectric
transducers, converting the acoustic signal into an electric impulse. The outer hair
cells contain contractile proteins andhave an efferent nerve supply from the brain.
They serve to tune the basilar membrane on which they are positioned.
Each inner hair cell responds to a particular frequency and when stimulated it
depolarises and passes an impulse to the cochlea nuclei in the brainstem.
The vestibular labyrinth consists of the semicircular canals, the utricle and saccule,
and their central connections. The three semicircular canals are arranged in the three
planes of space at right angles to each other. As in the auditory system, hair cells are
present. In the lateral canals the hair cells are embedded in a gelatinous cupula, and
shearing forces, caused by angular movements of the head, produce hair cell
movements and generate action potentials. In the utricle and saccule the hair cells are
embedded in an otoconial membrane which contains particles of calcium carbonate.
These respond to changes in linear acceleration and the pull of gravity.
Impulses are carried centrally by the vestibular nerve, and connections are made to the
spinal cord, cerebellum and external ocular muscles.
The sensory nerve supply of the ear is complex. The external ear is supplied by the
auriculotemporal branch of the trigeminal nerve (V), and this supplies most of the
anterior half of the pinna and the external auditory meatus. The greater auricular nerve
(C2,3), together with branches of the lesser occipital nerve (C2), supply the posterior
part of the pinna. The VIIth, IXth and Xth cranial nerves also supply small sensory
branches to the external ear; this explains why the vesicles of herpes zoster affecting
the VIIth nerve appear in the concha (see Fig. 40.29 later). The middle ear is supplied
by the glossopharyngeal nerve (IX).
This complicated and rich sensory innervation means that referred otalgia is common
and may originate from the normal area of distribution of any of the above nerves. A
classic example is the referred otalgia caused by a malignancy in the pyriform fossa of
the pharynx or a cancer of the larynx.
Anatomy of the ear
•Referred otalgia has many causes (e.g. cancer of the larynx)
•Middle ear is intimately related to the cranial cavity
•The Vllth nerve has a tortuous course through the ear

Conditions of the external ear

Congenital anomalies
Congenital anomalies can range from total absence of the ear through to mild
cosmetic deformities such as tiny accessory auricles or skin tags. External ear
anomalies can be isolated or may be associated with middle ear deformity. The
external and middle ear originate from the first and second branchial arches, whereas
the cochlea is of neuroectodermal origin. This means that an individual may have no
pinna or ear canal but a normal cochlea may well be present. In these circumstances,
sound can be transmitted from a hearing aid connected to an osteo integrated peg that
is screwed into the mastoid bone. (Tab 40.1)
Children who have a significant deformity of the pinna (microtia) can be helped with
osteointegrated implants to which a prosthetic ear is connected (Fig. 40.3). The ear
can be unclipped prior to playing violent sport (e.g. rugby) and this unsettles the
opposition. Preauricular sinuses are a common congenital abnormality and
occasionally need excising because of recurrent infections and discharge. The sinus
usually ends near the external canal but occasionally the track is very extensive and is
closely related to the facial nerve, which makes life exciting.
Prominent ears are a common deformity which usually results from the absence of the
antihelix curve. Various cartilage scoring methods are available to correct this
deformity.
Trauma
Trauma often affects the external ear. A haematoma of the pinna occurs when blood
collects between the penchondrium and the cartilage. The cartilage receives its blood
supply from the perichondrial layer and will die if the haematoma is not evacuated
(cauliflower ear). An extensive excision, under general anaesthetic, with a pressure
dressing and antibiotic cover is recommended (see Fig. 40.4).
Foreign bodies in the ear canal need to be treated with the greatest respect. If an
object is not simply removed at the first attempt, it is better to do it with the aid of a
microscope and general anaesthesia. An active 2-year-old with a bead in the ear can
be a formidable opponent (Fig. 40.5).
Inflammatory disorders
Inflammatory disorders of the external ear are extremely common. Otitis externa
frequently presents to general practice and to ear, nose and throat (ENT) surgeons.
There is generalised inflammation of the skin of the external auditory meatus. It can
occur as an acute episode or can run a more chronic course. The cause is often
multifactorial but includes general skin disorders, such as psoriasis and eczema, and
trauma. Common pathogens are pseudomonas and staphylococcus bacteria, and
amongst fungi, candida and aspergillus. Once the skin of the ear canal becomes soggy
and oedematous, skin migration stops and debris collects in the ear canal which acts
as a substrate for the pathogens. The hallmark of acute otitis externa is severe pain
(evidently on a par with childbirth). Unlike otitis media, movement of the pinna elicits
pain. The condition is often bilateral.
The initial treatment is with topical antibiotics and steroid ear drops, together with
analgesia. If this fails meticulous removal of the debris with the aid of an operating
microscope is required. Regular cleaning of the canal, together with topical steroids,
needs to be continued until normal skin migration resumes. If fungal infection is
present it can easily be recognised by the presence of hyphae and spores within the
canal (Fig. 40.6). Fungal infection causes irritation and itch, and the treatment is
meticulous removal of the fungus and any debris, as well as stopping any concurrent
antibiotics.
Systemic antibiotics are rarely required for otitis externa but should be used if
cellulitis of the pinna occurs (Fig. 40.7).
Necrotising otitis externa is a rare but very important condition. It presents as a
severe, persistent, unilateral otitis externa in an immunocompromised individual, for
example it is important to think of the diagnosis in an elderly diabetic. Osteomyelitis
of the skull base occurs and usually the infecting organism is Pseudomonas
aeruginosa. Several cranial nerves (VII, IX and X) may be destroyed by the
progressing infection. Intensive systemic antibiotic treatment is required and the
disease process is monitored by high-resolution imaging.
A furuncle of the external ear is an infection of a hair follicle and is due to a
staphylococcal infection. Moving the pinna causes extreme pain. Local treatment of
the ear canal (oto-wick and steroid drops) together with systemic antibiotic therapy is
required.
Neoplasms
Benign neoplasms
Benign neoplasms of the external ear are common if osteomas are included. These
arise from the bone of the ear canal in individuals who have done a lot of swimming
in cold water. No treatment is required unless they obstruct the migration of skin out
of the canal. Other benign tumours include papillomas and adenomas.
Malignant primary tumours
Malignant primary tumours of the external ear are either basal cell or squamous cell
carcinomas (Fig. 40.8). Both may present as ulcerating or crusting lesions which grow
slowly and may be ignored by elderly patients. Squamous cell carcinomas may
metastasise to the parotid and/or neck nodes and need radical surgical clearance. The
ear canal may be invaded by tumours from the parotid and postnasal space carcinoma
which ‘creep’ up the eustachian tube. All resectable malignant tumours of the ear are
treated primarily with surgery with or without the addition of radiation therapy.
The external ear
• Otitis externa responds to topical medication
• Unilateral otitis externa in a diabetic may be fatal
•Auricular haematoma needs a robust incision, drainage and pressure dressing
•Think osteo integration for congenital malformations

Conditions of the middle ear (Tab 40.2)

Congenital anomalies
Congenital anomalies of the middle ear may be isolated or may be associated with
other ear or general congenital deformities. There is a number of branchial arch
syndromes — for example Pierre Robin’s syndrome, craniofacial dysostosis, Down’s
syndrome and Treacher Collins’ syndrome. If there is an external ear abnormality, it
should raise suspicion of an underlying middle ear deformity. Middle ear deformity
can be assessed by high-resolution computerised tomography (CT) scanning and, if
the inner ear is normal, reconstructive surgery of the middle ear can be very
successful.
Trauma
Trauma to the middle ear can result in a perforated tympanic membrane (Fig. 40.9a).
Such perforations usually heal spontaneously (Fig. 40.9b). Trauma can result is
ossicular discontinuity and typically it is the incus that is displaced. Various
operations termed ‘tympanoplasties’ are available to reconstruct the damaged
ossicular chain and repair the tympanic membrane if necessary.
Inflammatory disorders
The most common inflammatory condition of the middle ear is acute suppurative
otitis media. It is extremely common in childhood and is characterised by purulent
fluid in the middle ear. Mastoiditis may be associated with otitis media because the
mastoid air cells connect freely with the middle ear space. The tympanic membrane is
hyperemic and bulges owing to pressure from the pus in the middle ear (Fig. 40.10).
The child suffers extreme pain until the tympanic membrane bursts. The most
common infecting organisms are Streptococcus pneumoniae and Haemophilus
influenzae. Appropriate systemic antibiotics should be given for 10 days.
The incidence of acute mastoiditis has diminished with the widespread use of
antibiotics for otitis media. Sometimes, however, a child will have had a number of
courses of antibiotics, none of which completely resolves the middle ear infection. In
such cases the pain and swelling behind the ear may not be quite so apparent as in Fig.
40.11. When mastoiditis is present, if the tympanic membrane can be seen, there is
always a sag in the posterior superior part of the drum. (Conversely, a normal
tympanic membrane excludes mastoiditis.) Treatment requires hospital admission and
intensive parenteral antibiotics. If this does not resolve the infection quickly a cortical
mastoidectomy is required, together with a myringotomy.
Mastoiditis
• Sequelae of acute otitis media
• May be masked by antibiotics
•Requires intensive antibiotics and/or drainage
Otitis media with effusion (glue ear) is very common with the majority of children
experiencing at least one episode of it during development. Many factors have been
implicated, although it is primarily thought to be due to poor eustachian tube function.
Oxygen is continually being absorbed by the middle ear mucosa and this results in a
negative middle ear pressure unless the eustachian tube opens to replenish the air.
This negative middle ear pressure initially results in transudation of fluid into the
middle ear space (Fig. 40.12). If the hypoxia continues, a mucoid exudate is produced
by the glands within the middle ear mucosa. This sticky exudate is referred to as ‘glue
ear’.
The following symptoms may be associated with glue ear:
•hearing impairment which often fluctuates;
•delayed speech;
•behavioral problems;
•recurrent ear infections — this occurs because the exudate is an ideal culture medium
for microorganisms;
•reading and learning difficulties at school.
If these symptoms are present for a short time only, it is likely that no long-term
sequelae will develop. However, if symptoms persist, particularly a long-term
bilateral conductive hearing loss, the child will miss out on educational opportunities
and may not fulfil his or her academic potential. The otoscopic findings of exudative
glue ear are of a dull drum that is immobile on pneumatic otoscopy (Fig. 40.13).
The tympanic membrane is retracted and radial blood vessels may be present (Fig.
40.14). Of children first presenting with bilateral glue ear, 50 per cent of the effusions
will resolve spontaneously within 6 weeks of onset. Initially, a ‘wait and watch’ policy
is therefore appropriate. If the bilateral glue ear persists with a significant hearing loss
then treatment is required.
There is no substantial evidence for medical treatment. Use of the Otovent device
(Fig. 40.15) may improve eustachian tube function and is worth trying while waiting
for resolution of the effusion. However, surgical intervention is the only effective way
of curing glue ear. Both ventilation tube (groin-mets) and adenoidectomy are
effective. The controversy is not whether surgery works but when to
intervene.insertion of ventilation tubes and/or an adenoidectomy require a general
anaesthetic. The ventilation tube is placed in the anterior inferior portion of the
tympanic membrane because there is no important clockwork behind this part of the
drum. The ventilation tubes stay in position for approximately 6—18 months and are
then extruded because of the migratory behaviour of the tympanic membrane. There is
no reason why children with ventilation tubes should not be allowed to swim (Fig.
40.16).
Otitis media with effusion (glue ear)
•Very common, peaks at 18 months and 5 years
• Majority of children need no treatment
•Prolonged hearing loss treated with ventilation tubes and/or adenoidectomy
A middle effusion in adults is relatively rare and when it occurs does not usually last
long. The condition is often associated with an upper respiratory tract infection. A per-
sistent unilateral effusion in an adult should always be viewed with suspicion. A
nasopharangeal carcinoma may cause the effusion by blocking the opening of the
eustachian tube in the postnasal space. This is the most common carcinoma in males
in southern China.
Chronic suppurative otitis media
Chronic suppurative otitis media (CSOM) is classified into two types: tubotympanic
disease, in which there is a perforation of the pars tensa; and atticoantral disease, in
which a retraction pocket develops from the pars flaccida.
CSOM of the tubotympanic type. CSOM of the tubotympanic type can result from
trauma or infection. When perforated the tympanic membrane usually repairs itself,
but occasionally the outer layer of the tympanic membrane fuses with inner mucosa
and a chronic perforation results (Fig. 40.17). With this type of disease the patient’s
main symptoms are of an intermittent or chronic mucoid discharge associated with a
mild conductive hearing loss. It is rare for this type of disease to be associated with
intracranial complications.
A diagnosis is made on otoscopy and the tuning forks usually suggest a conductive
hearing impairment. The first-line treatment is topical antibiotic and steroid drops,
and on occasion microsuction. If medical treatment fails, the patient may request an
operation to graft the tympanic membrane in order to give a dry ear. This operation is
termed a myringoplasty (type I tympanoplasty). The edges of the perforation are
freshened and a small piece of temporalis fascia is inserted under the tympanic
membrane to graft the drum. The raw epithelial edges then grow across the graft to
repair the tympanic membrane.
CSOM of the atticotympanic type. CSOM of the atticoantral type is important
because of the complications associated with it. Cholesteatoma is the alternative name
and means a cyst or sac of squamous epithelium that is present in the attic part of the
middle ear. The exact aetiology of cholesteatoma is not known, although poor
eustachian tube function is implicated (e.g. patients with cleft palates have relatively
poor eustachian tube function and have a higher incidence of cholesteatoma).
A retraction pocket develops in the pars flaccida and, if the squamous epithelium
cannot migrate out of this pocket, a cholesteatoma results. The expanding ball of skin
causes a low-grade osteomyelitis which results in the release of fatty acids from the
bone. This gives the discharge its characteristic faecal smell. Invariably the discharge
is accompanied by hearing loss and mild discomfort. The patient may simply put up
with the symptoms until a severe complication occurs.
The hearing loss that is caused by cholesteatoma may be conductive due to erosion of
the incus or sensori neural due to direct erosion of the cochlea or migrations of toxins
into the inner ear. Vestibular symptoms may occur because of erosion of the
semicircular canals or the migration of toxins into the vestibule. Pressure or erosion of
the facial nerve is relatively unusual.
The close proximity of the middle ear and mastoid to the middle and posterior cranial
fossae means that intracranial
sepsis can result from chronic ear disease. The infection spreads to the dura via
emissary veins which connect the middle ear mucosa to the dura or by direct
extension of the disease through the bone. Meningitis, extradural, subdural or
intracerebral abscess, or a combination of these may occur. The main causes of
intracranial sepsis in the UK are chronic ear disease and chronic sinus disease.
Diagnosis should be suspected on otoscopy (Fig. 40.18). Pus, crusts, granulations or a
whitish debris in the attic are hallmarks of the disease. Examination under the
microscope, audiometry and, sometimes, CT scanning are indicated.
The treatment is surgical and follows the principle of exposing the disease, excising
the disease and then exteriorising the affected area. Two commonly applied operations
for this disease are an atticotomy and a modified radical mastoidectomy.
An atticotomy or modified radical mastoidectomy is performed by making an incision
behind the ear (post auricular), or between the tragus and the pinna (end aural). The
attic part of the bony ear can is drilled away and the retraction pocket is followed back
into the mastoid until the end of the disease is found. An attempt is made to excise
totally the pocket and then the resulting cavity is usually lined with temporalis fascia.
A mastoid cavity heals with normal skin that does not migrate, and for this reason
patients with a mastoid cavity need to be seen in an out-patients’ clinic on a regular
basis. Any skin that collects in the mastoid cavity needs to be removed with the aid of
a sucker and a microscope (Fig. 40.19).
Chronic suppurative otitis media (CSOM)
•In tubotympanic CSOM there is a perforated tympanic membrane and frequently a
mucoid discharge
•Atticoantral CSOM:
— Is skin in an attic retraction? = cholesteatoma Presents with hearing loss and
smelly discharge
— Is a common cause of intracranial sepsis
Tuberculous otitis media is an important cause of suppuration in some countries. The
diagnosis should always be considered in any ear which fails to respond to standard
therapy. A swab for appropriate culture studies, coupled with chest radiography, will
usually confirm the diagnosis.
Otosclerosis is a condition in which new abnormal spongy bone is laid down in the
dense otic capsule. Of particular importance is the bone that is laid down around the
footplate of the stapes which impedes the mobility of the stapes and results in a
conductive hearing loss (Fig. 40.20). Toxins released from the new bone formation
may also cause a gradual sensorineural hearing loss. Otosclerosis is more common in
women, and in 50 per cent of patients there is a family history. The typical
presentation is of a conductive hearing loss in a young woman with the condition
being exacerbated by the hormonal flux of pregnancy. A similar type of stapes fixation
occurs in osteogenesis imperfecta and is known as Van der Hoeve’s syndrome.
Otosclerosis is often bilateral. A diagnosis should be suspected in any patient with a
conductive hearing loss and a normal tympanic membrane.
The treatment options are simple reassurance, a hearing aid or a stapedotomy
operation. In the stapedotomy operation, the stapes crura are removed and a small
hole is drilled in the fixed stapes footplate. A vein graft is then inserted over the hole
and a piston linking the incus to the vein graft is delicately placed in position (Fig.
40.21). In 90 per cent of cases the operation is highly successful, but rare complica-
tions include severe sensorineural hearing loss and balance disturbance.
Otosclerosis
•New bone formation in otic capsule
•Stapes fixation
•Options:
— Reassurance;
— Hearing aid;
— Stapedotomy
Neoplasms
Neoplasms of the middle ear are rare, the most common being a glomus tumour (Fig.
40.22). Glomus tumours arise from nonchromaffin paraganglionic tissue. The carotid
body tumour arising in the neck is an example of this type of tumour. In the temporal
bone three types of glomus tumour are recognised and classification depends on the
location:
glomus tympanicum (arising in the middle ear), glomus jugularae (arising next to the
jugular bulb) and glomus vagali (skull base).
Pulsatile tinnitus is a classic symptom of these tumours. Hearing loss occurs and may
be either conductive or sensorineural, and paralysis of the VIIth, IXth, Xth, XIth
and/or XIIth nerves may occur. The classic sign is a cherry-red mass lying behind the
tympanic membrane. An audible bruit may be heard with a stethoscope over the
temporal bone. The treatment of choice is preoperative einbolisation followed by
surgical excision. Radiotherapy is also effective.
Squamous cell carcinoma may also occur within the middle ear. It usually presents
with deep seated pain and a bloodstained discharge. The facial nerve may be
paralysed. Squamous carcinomas usually arise in a chronically discharging ear and
can certainly arise in a chronically infected mastoid cavity. Radical surgical excision
with or without radiotherapy provides the only chance of cure.

Conditions of the Inner ear(Tab 40.3)

Congenital
Congenital inner ear disorders may be associated with external or middle ear
abnormalities or exist on their own. The most common anomaly is dysplasia of the
membranous labyrinth, although dysplasia of the bony labyrinth and even total aplasia
of the ear may occur. Intrauterine infections, including rubella, toxoplasmosis and
cytomegalovirus, can cause inner ear damage. Perinatal hypoxia, jaundice and pre-
maturity are also risk factors for a hearing loss. After birth, meningitis may cause
profound sensorineural hearing loss.
If a child’s parents suspect a hearing impairment it is important to believe them,
especially when glue ear has been excluded. In children in whom there is a suspicion
of sensorineural hearing loss, brainstem evoked audiometry is used to establish
hearing thresholds (Fig. 40.23). If some hearing is present, the early fitment of
hearing aids can maximise the neural plasticity that is present in the developing brain.
If a child has a profound hearing loss, then early intervention with a cochlea implant
may he appropriate (Fig. 40.24). Most cases of profound sensorineural hearing loss
are due to loss of cochlear hair cells so that an implant inserted through the round
window can selectively stimulate the cochlear neurons which usually remain intact.
Degenerative
Presbyacusis, a degenerative disorder, is a term used to describe the hearing loss of
old age. It is characterised by a gradual loss of hearing in both ears, with or without
tinnitus. The hearing loss usually affects the higher frequencies and a classical
audiogram is shown in Fig. 40.25.
The consonants of speech lie within the high-frequency range which makes speech
discrimination difficult. Examination of an elderly person s cochlea shows loss of hair
cells, particularly at the basal turn of the cochlea. With ageing the dynamic range of
hearing is also reduced so that elderly people often find loud noises uncomfortable.
This phenomenon is known as ‘recruitment’.
Many patients with presbycusis are concerned that they may lose their hearing
completely and need reassurance. Hearing aid technology has improved dramatically
and most patients now benefit (Fig. 40.26). Care and attention to detail when fitting
the hearing aid are essential, together with monitoring the patient’s progress. If this
does not occur the hearing aid ends up in the bedroom drawer.
Tinnitus describes an abnormal noise that appears to come from the ear or within the
head. It may have an extrinsic cause, for example the pulsatile tinnitus of a glomus
jugularae tumour. Usually, however, the tinnitus is generated within the cochlea, and
most people will experience tinnitus at some time in their life.
Tinnitus frequently accompanies presbycusis, as well as any condition that damages
the inner ear structures. Most individuals adapt to the presence of tinnitus but in some
patients it proves intrusive. Reassurance and relaxation therapy are highly effective, as
is a hearing aid for patients who also have presbycusis. An ENT surgeon who was a
keen fisherman found that he could not hear his tinnitus when fishing next to a
waterfall. From this observation tinnitus maskers have been developed (Fig. 40.27). A
masker provides a similar noise to the tinnitus and ‘blanks it Out.
Trauma
Trauma to the inner ear can be caused by noise or direct injury. Hair cells within the
cochlea are damaged by sudden acoustic trauma (blast injury or gun fire) or by
prolonged exposure to excessive noise. The sensorineural hearing loss that results is
greatest at high frequencies (particularly 4000 Hz) and is often accompanied by
tinnitus (Fig. 40.28). The law in the UK requires that workers are protected from
noise, but in a disco an individual relies on common sense!
The otic capsule is the hardest bone in the body but if trauma to the head is severe
temporal bone fractures may occur. These tend to be either longitudinal (80 per cent)
or transverse (20 per cent). Transverse fractures usually involve the labyrinth and lead
to a sensorineural hearing loss which is permanent. Profound vertigo occurs initially
followed by gradual compensation. In about 50 per cent of cases there is an associated
facial nerve paralysis.When assessing a severely injured patient, it is important to
record the facial nerve function and, in particular, whether any facial weakness is
partial or total. Total facial paralysis immediately following a head trauma suggests a
major injury to the nerve and under certain circumstances exploration of the facial
nerve to decompress it or repair it may be appropriate. Longitudinal fractures usually
spare the labyrinth but frequently involve the external meatus and roof of the middle
ear.Important physical findings that may accompany a skull base fracture include a
haematoma over the mastoid bone (Battle’s sign), blood in the external ear or a
laceration along the roof of the external canal. Cerebrospinal fluid (CSF) otorrhoea or
CSF rhinorrhoea (if the tympanic membrane is intact) may occur. A conductive
hearing loss may be present because of fluid in the middle ear or disruption of the
ossicular chair. A high-resolution CT scan is required to assess skull base fractures.
Facial paralysis
•Think complete or partial
•Protect the eye
•Otoneurological examination to find cause
•Early treatment with steroids and/or antiviral therapy dependent on aetiology
Barotrauma is a rare cause of a sudden sensorineural hearing loss (SNHL) or acute
vestibular disturbance. Rapid changes in pressure across the labyrinthine membranes
may occur with diving or flying and may allow air to be forced into the cochlea. Any
individual with a sudden sensorineural hearing loss requires urgent hospital
admission, and in those with a history of barotrauma it may be appropriate to raise the
tympanic membrane and to search for a leak of pen-lymph in the region of the oval or
round window.
Drug ototoxicity is a form of trauma that may damage the inner ear. Some drugs
differentially affect the cochlea causing hearing loss and tinnitus while others pick out
the vestibular system causing vertigo. Aminoglycocides are well known to be
ototoxic, as is cisplatinum. Recognition of risk factors,
such as poor renal function in patients being treated with aminoglycocides, is most
important. Although many topical ear drops contain aminoglycocides, there is little
evidence that such topical treatment causes sensorineural hearing loss if used for short
periods.
Benign paroxysmal positional vertigo (BPPV) may follow head or neck trauma.
Vertigo is an illusion of movement and BPPV is characterised by intermittent attacks
of vertigo that occur when the head is moved in a certain position. Typically the
vertigo only lasts for a few seconds and is not associated with other otological
symptoms. Positional testing can evoke nystagmus and helps in the diagnosis of this
condition. The condition is usually self-limiting and special manoeuvres described by
Epley help the majority of patients (Fig. 40.29).
Vascular occlusion
A reduction in labyninthine blood flow with associated hypoxia is the most likely
cause for most cases of sudden onset of severe sensorineural hearing loss. All patients
with a sudden sensorineural hearing loss should be referred immediately for specialist
treatment. The treatment consists of bed rest, steroids and, in some centres, the
administration of Carbogen (an oxygen and carbon dioxide mixture). Five per cent of
acoustic neuromas present with a sudden sensorineural hearing loss and therefore
radiological investigation, preferably with magnetic resonance imaging (MRI), is
required to exclude this tumour.
Inflammatory disorders
Inflammation caused by a viral infection is thought to account for acute vestibular
failure (vestibular neuroritis). This condition is characterised by a sudden onset of
vertigo. The vertigo is so severe that the patient often takes to his or her bed for
between 2 and 5days. Central compensation then occurs, although recurring episodes
of vertigo for up to 18 months can occur. This is thought to be due to incomplete
compensation for the original vestibular damage.
The aetiology of Ménière’s disease is not known. The condition is characterised by a
triad of symptoms. Intermittent attacks of vertigo, a fluctuating sensorineural hearing
loss and tinnitus. The patient often has a sensation of pressure in the affected ear
before an attack. The hearing loss typically affects the lower frequencies and is
virtually the only type of sensorineural hearing loss that fluctuates. The time-course of
the vertigo characteristically lasts between 30 minutes and 6 hours. It is often
accompanied by nausea and vomiting. Although the cause of the condition is
unknown, the pathology is well documented. There is an excessive accumulation of
endolymphatic fluid (hydrops) and it is thought that the distension of the
endolymphatic compartment may rupture Reissner’s membrane which leads to mixing
of endolymph and perilymph. This is the basis for the cochlear—vestibular failure
which characterises the condition. The investigations include puretone audiometry,
electrocochleography and MRI scan if available. The latter is required to exclude an
acoustic neuroma which may mimic the symptoms of Ménière’s disease.
Viral infections that involve the facial nerve are possibly one of the commonest
causes of facial weakness (80 per cent). Bell’s palsy results from a viral infection of
the facial nerve. The nerve swells and is compressed in its labyninthine portion as it
passes from the internal auditory meatus towards the middle ear. If the patient
presents within the first 48 hours, treatment with high-dose steroids is appropriate.
Not all facial nerve palsies are due to viral infection and a thorough otoneurological
examination is required. The facial nerve can be damaged within the brainstem at the
cerebellopontine angle, within the internal auditory meatus, within the middle ear, at
the skull base and within the parotid. It is essential to consider these potential sites of
facial nerve damage in any patient with VIIth nerve paralysis.
Ramsey Hunt’s syndrome is caused by herpes zoster virus. It is characterised by a
facial palsy and is often associated with facial pain and the appearance of vesicles on
the ear drum, ear canal and pinna (Fig. 40.30). Vertigo and sensorineural hearing loss
(VIIIth nerve) accompany it. Treatment with aciclovir is effective if given early.
Metabolic causes
The metabolic causes of inner ear damage include diabetes mellitus and thyroid
disease, both of which may cause sensorineural hearing loss.
Neoplasms
Tumours of the inner ear are uncommon but can present with sensorineural hearing
loss, tinnitus and vertigo. Acoustic neuromas, which are actually Schwannomas of the
vestibular division of the VIIIth nerve, are the most common, followed by
meningiomas. Acoustic neuromas grow slowly and somewhat unpredictably and as
they expand can cause cranial nerve palsies, brainstem compression and raised
intracranial pressure. The early symptoms are a unilateral sensorineural hearing loss
or unilateral tinnitus, or both. It is important to diagnose these tumours early and
remove them when they are small. The morbidity and mortality from surgery is
directly related to tumour size. If the tumour is removed when it is small, there is an
extremely good chance of preserving facial nerve function.
The investigation of choice for detecting acoustic neuromas is MRI (Fig. 40.31). In
many centres patients with any unilateral otological symptoms are screened using
MRI. If MRI is not available, CT scanning is the next best diagnostic tool.
Occasionally, in an elderly patient, ‘a wait and see’ policy may be adopted. In such
patients repeat MRIs can be used to monitor the tumour. There are three main surgical
approaches for resecting acoustic neuromas. A temporal craniotomy and a direct
middle fossa approach is used for small tumours only. The translabyrinthine approach
is entirely through the ear and does not breach the dura. The neurosurgeon and ENT
surgeon work together. There is minimal disturbance to the patient, although the
hearing is completely destroyed. A suboccipital approach is performed by a
neurosurgeon doing a craniotomy with the ENT surgeon removing bone at the internal
auditory meatus. Traction on the cerebellum is required. This increases the morbidity.
In some cases, however, what remains of the hearing can be preserved using this
approach. The physiological monitoring of the facial nerve and auditory nerve during
surgery has improved acoustic nerve surgery results
The inner ear
•Presbycusis: bilateral high-frequency loss
• Unilateral tinnitus or sudden sensory neural hearing loss needs to be investigated
for possible acoustic neuroma
•Sudden sensory neural hearing loss needs immediate treatment

Further reading
Booth, J. (ed.) (1997) Scott-Brown’s Otolaryngology, 6th edn, Vol. 3, Butterworth-
Heinemann, Oxford.
Ludman, H. and Wright, A. (eds) (1998) Mawson’s Diseases of the Ear, 6th edn,
Arnold, London.
Van Hassell, A., Milford, C.A. and Bleach, N. (eds) (1997) Operative
Otolaryngology, Blackwell Scientific, Oxford.