Ear Disorders

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Acquired diseases of the pinna are frequently seen in community practice.
Among the most commonly seen pinnal lesions are keloids; a firm, dermal nodule develops in the dermis, usually following trauma.
The most common site is in the earlobe, following ear-piercing, particularly in girls of Afro-Caribbean origin. 

Trauma to the pinna is usually the result of a violent blow, sustained during criminal assault or its socially acceptable equivalent, boxing .
The most common lesion is a tense, tender haematoma, incomplete resolution of which leads to physical distortion of the pinna (cauliflower ear).

Inflammatory skin disease particularly affects the back of the pinna and the groove behind the ear. Atopic eczema is the most common disease, particularly in children with the atopic tendency.
Chondrodermatitis nodularis helicis presents as a small intractable and often tender ulcer, usually on the helix. It is most common in the elderly and there may be degeneration of the pinnal cartilage beneath the ulcerated area.

Tumours and pre-tumorous conditions mainly occur in the elderly. They are usually squamous cell carcinomas or basal cell carcinomas (both of which tend to be single lesions), or solar or actinic keratoses (which may be multiple).

Ear infections

There are two patterns of inflammation of the external auditory meatus (otitis externa). Inflammation may be localized, due to a boil (furuncle) in the ear canal, or diffuse, usually due to either bacterial or fungal infection. A common fungus is Aspergillus niger, black threads of which can be identified in the inflammatory exudate and may even be visible in the ear canal.Allergic otitis externa is usually a response to topical ear drops. 

Viral warts, basal cell carcinomas and squamous cell carcinomas can occur in the external auditory meatus, but all are rare.

The external auditory meatus is inflamed and covered by a scaling exudate, which spreads onto the pinna. Some of the pinnal changes are due to superimposed allergic otitis, a response to antibiotic eardrops given for the original infection.

Perforation of the tympanic membrane usually results from middle ear infection, but may occasionally follow trauma

The tympanic membrane (eardrum) is a three-layered structure. There is a central sheet of fibrocollagenous support tissue containing numerous elastic fibres, covered on the external surface by stratified squamous epithelium continuous with that of the external auditory meatus, and on the inner surface by low cuboidal epithelium continuous with that lining the middle ear.

Infection (usually acute otitis media) is the most important cause of perforation, particularly in children. Less commonly, trauma, e.g. sustained during attempted removal of foreign bodies, may result in perforation. Most central perforations of the drum heal spontaneously. 
After perforation most eardrums heal by fibrosis

Although small central perforations heal spontaneously by fibrosis within a few days, larger perforations sometimes fail to heal and may require surgical closure using a fascial graft. A healed perforation is sometimes visible as a white scar or thinned area on the eardrum.

The most important complications of a central perforation are: predisposition to recurrent middle ear infection (otitis media), with impaired hearing; and failure to heal.

Chronic suppurative otitis media usually follows permanent perforation of the eardrum

Recurrent chronic inflammation in the middle ear is an important cause of chronic earache, deafness, and persistent discharge from the external auditory meatus. It usually occurs in people with a persistent, non-healing perforation of the eardrum. Chronic suppurative otitis media (CSOM) is usually subdivided into two broad groups:

Tubotympanic disease, in which the perforation is in the pars tensa of the eardrum, and the discharge is typically copious and mucopurulent. The mucosal lining of the middle ear becomes chronically inflamed with a heavy infiltrate of lymphocytes and plasma cells, leading to thickening and the formation of inflammatory granulation tissue, often in the form of chronic inflammatory 
granulation polyps.

Atticoantral disease, in which the perforation is located in the eardrum at the attic region, and is typically associated with the development of cholesteatoma. Atticoantral disease is also associated with a higher risk of major complications, e.g. brain abscess and other intracranial infection.

OME (glue ear) in children is commonly associated with upper respiratory tract infections

In otitis media with effusion (OME), mucoid fluid accumulates in the middle-ear cavity because it is unable to drain through the child's narrow Eustachian tube. This is possibly associated with lower tube blockage due to reactive hyperplasia of the adenoid lymphoid tissue.

The fluid is sterile, and is often thick and tenacious, resembling greyish brown liquid glue, hence the common term 'glue ear'. It is associated with conductive deafness with intermittent earache. Because of the stasis of the fluid within the middle ear, there is a predisposition to acute suppurative otitis media due to secondary bacterial infection.

Tympanosclerosis is a hyaline degeneration of the eardrum sub-mucosa. Appearing either as a crescentic white area or as chalky-looking patches, it may occur in the tympanic membrane in association with OME. It is most commonly seen after insertion of a grommet. 
The most important primary disease of the small bones of the middle ear is otosclerosis

In otosclerosis the normal bone of the auditory ossicles is replaced and thickened by newly deposited woven bone. The disease, which is usually bilateral and eventually produces deafness, may be hereditary. There is an adult female preponderance. The disease usually starts at the otic capsule between the cochlea and vestibule, and may spread to involve the footplate and limbs of the stapes. It is the involvement of the stapes and of the cochlea that leads to deafness.

Cholesteatoma is an important middle ear disease

Cholesteatoma is a form of epidermoid cyst. Most commonly located in the epitympanic recess (attic) and mastoid antrum, it often extends into the mastoid process. Its precise pathogenesis is disputed, but it is frequently associated with an atticoantral perforation of the eardrum. It is a cystic structure lined by squamous epithelium, which constantly produces keratin. This leads to expansion of the lesion, damaging the small structures in the cavity. The area may become colonized by Gram-negative saprophytic bacteria, which probably stimulate continuing keratin formation. The enlarging keratinous mass, lined by stratified squamous epithelium, can eventually erode bone, and may destroy the labyrinth, mastoid air cells, and facial nerve. It may even erode through the skull forming the base of the middle cranial fossa. Although non-neoplastic, cholesteatomas have the same effects as a slow-growing benign tumour.

Common causes of conductive deafness

Although it is usually initially unilateral, up to 50% of patients may eventually develop disease in the other ear, sometimes many years later. Its cause is unknown, but the most important abnormality is marked distension of the cochlear duct by excess fluid, such that the vestibular membrane of Reissner, which separates two fluids of different composition, bulges into the scala vestibuli. This membrane may rupture, allowing the two fluids to mix. Histological study of the disease is hampered by the difficulty of obtaining untraumatized cochlea at post mortem examination.

Many diseases of the ear are associated with temporary or permanent hearing loss 

Hearing loss can be classified as conductive (usually due to some abnormality in external or middle ear), sensorineural (usually due to some abnormality in the inner ear, auditory nerve or brain), or mixed (i.e. with features of both conductive and sensorineural hearing loss).

Conductive hearing loss occurs when sound waves cannot be transmitted to the inner ear

Conductive hearing loss is the most common type of temporary hearing loss encountered in family practice. In most cases it is due to occlusion of the external auditory canal by wax, and the hearing improves when the wax is carefully removed.

Sensorineural deafness is due to damage to the inner ear, or to the nerve tracts transmitting messages to the brain

The most common type of permanent hearing loss is presbycusis, a pattern of sensorineural hearing impairment in the elderly. There is decrease in hair cells (associated with atrophy of the epithelial tissue in the basal turn of the cochlea), atrophy of the stria vascularis, and neuronal loss in the spiral ganglia, all of which lead to a progressive sensorineural hearing loss. This type of deafness is characterized by loss of high tones combined with distortion.
Schwannoma of the vestibulocochlear nerve is an important cause of unilateral hearing loss. As with paraganglioma, it is more common in women than in men, usually presenting between the ages of 30 and 50 years.

Tumours of the ear are not common, and mainly occur in the external ear

Both basal cell carcinoma and squamous cell carcinoma can originate in the epithelium of the external auditory meatus, and may spread to involve the middle ear.

The most important primary tumour presenting in the middle ear is a paraganglioma. Derived from the glomus jugulare, it is a neuroendocrine tumour. There is a female preponderance and most patients are between 40 and 60 years old. The tumours are slow-growing and may present late, causing damage by destruction of the ossicles and perforation of the eardrum.

In children, rhabdomyosarcoma is an important tumour of the ear.
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