Surgical removal of the tonsils, which is now performed only if a child suffers frequent, recurrent attacks of severe tonsillitis. The operation is also carried out to treat quinsy (an abscess around the tonsil).
Abnormal prion proteins accumulate in the brain and the spinal cord, damaging neurones (see NEURON(E)) and producing small cavities. Diagnosis can be made by tonsil (see TONSILS) biopsy, although work is under way to develop a diagnostic blood test. Abnormal prion proteins are unusually resistant to inactivation by chemicals, heat, X-RAYS or ULTRAVIOLET RAYS (UVR). They are resistant to cellular degradation and can convert normal prion proteins into abnormal forms. Human prion diseases, along with scrapie in sheep and BSE in cattle, belong to a group of disorders known as transmissible spongiform encephalopathies. Abnormal prion proteins can transfer from one animal species to another, and variant CJD has occurred as a result of consumption of meat from cattle infected with BSE.
From 1995 to 1999, a scienti?c study of tonsils and appendixes removed at operation suggested that the prevalence of prion carriage may be as high as 120 per million. It is not known what percentage of these might go on to develop disease.
One precaution is that, since 2003, all surgical instruments used in brain biopsies have had to be quarantined and disposable instruments are now used in tonsillectomy.
Measures have also been introduced to reduce the risk of transmission of CJD from transfusion of blood products.
In the past, CJD has also been acquired from intramuscular injections of human cadaveric pituitary-derived growth hormone and corneal transplantation.
The most common form of CJD remains the sporadic variety, although the eventual incidence of variant CJD may not be known for many years.... creutzfeldt-jakob disease (cjd)
Examination of the ear includes inspection of the external ear. An auriscope is used to examine the external ear canal and the ear drum. If a more detailed inspection is required, a microscope may be used to improve illumination and magni?cation.
Tuning-fork or Rinne tests are performed to identify the presence of DEAFNESS. The examiner tests whether the vibrating fork is audible at the meatus, and then the foot of the fork is placed on the mastoid bone of the ear to discover at which of the two sites the patient can hear the vibrations for the longest time. This can help to di?erentiate between conductive and nerve deafness.
Hearing tests are carried out to determine the level of hearing. An audiometer is used to deliver a series of short tones of varying frequency to the ear, either through a pair of headphones or via a sound transducer applied directly to the skull. The intensity of the sound is gradually reduced until it is no longer heard and this represents the threshold of hearing, at that frequency, through air and bone respectively. It may be necessary to play a masking noise into the opposite ear to prevent that ear from hearing the tones, enabling each ear to be tested independently.
General symptoms The following are some of the chief symptoms of ear disease: DEAFNESS (see DEAFNESS). EARACHE is most commonly due to acute in?ammation of the middle ear. Perceived pain in this region may be referred from other areas, such as the earache commonly experienced after tonsillectomy (removal of the TONSILS) or that caused by carious teeth (see TEETH, DISORDERS OF). The treatment will depend on the underlying cause. TINNITUS or ringing in the ear often accompanies deafness, but is sometimes the only symptom of ear disease. Even normal people sometimes experience tinnitus, particularly if put in soundproofed surroundings. It may be described as hissing, buzzing, the sound of the sea, or of bells. The intensity of the tinnitis usually ?uctuates, sometimes disappearing altogether. It may occur in almost any form of ear disease, but is particularly troublesome in nerve deafness due to ageing and in noise-induced deafness. The symptom seems to originate in the brain’s subcortical regions, high in the central nervous system. It may be a symptom of general diseases such as ANAEMIA, high blood pressure and arterial disease, in which cases it is often synchronous with the pulse, and may also be caused by drugs such as QUININE, salicylates (SALICYLIC ACID and its salts, for example, ASPIRIN) and certain ANTIBIOTICS. Treatment of any underlying ear disorder or systemic disease, including DEPRESSION, may reduce or even cure the tinnitis, but unfortunately in many cases the noises persist. Management involves psychological techniques and initially an explanation of the mechanism and reassurance that tinnitus does not signify brain disease, or an impending STROKE, may help the person. Tinnitus maskers – which look like hearing aids – have long been used with a suitably pitched sound helping to ‘mask’ the condition.
Diseases of the external ear
WAX (cerumen) is produced by specialised glands in the outer part of the ear canal only. Impacted wax within the ear canal can cause deafness, tinnitis and sometimes disturbance of balance. Wax can sometimes be softened with olive oil, 5-per-cent bicarbonate of soda or commercially prepared drops, and it will gradually liquefy and ‘remove itself’. If this is ineffective, syringing by a doctor or nurse will usually remove the wax but sometimes it is necessary for a specialist (otologist) to remove it manually with instruments. Syringing should not be done if perforation of the tympanic membrane (eardrum) is suspected. FOREIGN BODIES such as peas, beads or buttons may be found in the external ear canal, especially in children who have usually introduced them themselves. Live insects may also be trapped in the external canal causing intense irritation and noise, and in such cases spirit drops are ?rst instilled into the ear to kill the insect. Except in foreign bodies of vegetable origin, where swelling and pain may occur, syringing may be used to remove some foreign bodies, but often removal by a specialist using suitable instrumentation and an operating microscope is required. In children, a general anaesthetic may be needed. ACUTE OTITIS EXTERNA may be a di?use in?ammation or a boil (furuncle) occurring in the outer ear canal. The pinna is usually tender on movement (unlike acute otitis media – see below) and a discharge may be present. Initially treatment should be local, using magnesium sulphate paste or glycerine and 10-per-cent ichthaminol. Topical antibiotic drops can be used and sometimes antibiotics by mouth are necessary, especially if infection is acute. Clotrimazole drops are a useful antifungal treatment. Analgesics and locally applied warmth should relieve the pain.
CHRONIC OTITIS EXTERNA producing pain and discharge, can be caused by eczema, seborrhoeic DERMATITIS or PSORIASIS. Hair lotions and cosmetic preparations may trigger local allergic reactions in the external ear, and the chronic disorder may be the result of swimming or use of dirty towels. Careful cleaning of the ear by an ENT (Ear, Nose & Throat) surgeon and topical antibiotic or antifungal agents – along with removal of any precipitating cause – are the usual treatments. TUMOURS of the ear can arise in the skin of the auricle, often as a result of exposure to sunlight, and can be benign or malignant. Within the ear canal itself, the commonest tumours are benign outgrowths from the surrounding bone, said to occur in swimmers as a result of repeated exposure to cold water. Polyps may result from chronic infection of the ear canal and drum, particularly in the presence of a perforation. These polyps are soft and may be large enough to ?ll the ear canal, but may shrink considerably after treatment of the associated infection.
Diseases of the middle ear
OTITIS MEDIA or infection of the middle ear, usually occurs as a result of infection spreading up the Eustachian tubes from the nose, throat or sinuses. It may follow a cold, tonsillitis or sinusitis, and may also be caused by swimming and diving where water and infected secretions are forced up the Eustachian tube into the middle ear. Primarily it is a disease of children, with as many as 1.5 million cases occurring in Britain every year. Pain may be intense and throbbing or sharp in character. The condition is accompanied by deafness, fever and often TINNITUS.
In infants, crying may be the only sign that something is wrong – though this is usually accompanied by some localising manifestation such as rubbing or pulling at the ear. Examination of the ear usually reveals redness, and sometimes bulging, of the ear drum. In the early stages there is no discharge, but in the later stages there may be a discharge from perforation of the ear drum as a result of the pressure created in the middle ear by the accumulated pus. This is usually accompanied by an immediate reduction in pain.
Treatment consists of the immediate administration of an antibiotic, usually one of the penicillins (e.g. amoxicillin). In the majority of cases no further treatment is required, but if this does not quickly bring relief then it may be necessary to perform a myringotomy, or incision of the ear drum, to drain pus from the middle ear. When otitis media is treated immediately with su?cient dosage of the appropriate antibiotic, the chances of any permanent damage to the ear or to hearing are reduced to a negligible degree, as is the risk of any complications such as mastoiditis (discussed later in this section). CHRONIC OTITIS MEDIA WITH EFFUSION or glue ear, is the most common in?ammatory condition of the middle ear in children, to the extent that one in four children in the UK entering school has had an episode of ‘glue ear’. It is characterised by a persistent sticky ?uid in the middle ear (hence the name); this causes a conductive-type deafness. It may be associated with enlarged adenoids (see NOSE, DISORDERS OF) which impair the function of the Eustachian tube. If the hearing impairment is persistent and causes problems, drainage of the ?uid, along with antibiotic treatment, may be needed – possibly in conjunction with removal of the adenoids. The insertion of grommets (ventilation tubes) was for a time standard treatment, but while hearing is often restored, there may be no long-term gain and even a risk of damage to the tympanic membrane, so the operation is less popular than it was a decade or so ago. MASTOIDITIS is a serious complication of in?ammation of the middle ear, the incidence of which has been dramatically reduced by the introduction of antibiotics. In?ammation in this cavity usually arises by direct spread of acute or chronic in?ammation from the middle ear. The signs of this condition include swelling and tenderness of the skin behind the ear, redness and swelling inside the ear, pain in the side of the head, high fever, and a discharge from the ear. The management of this condition in the ?rst instance is with antibiotics, usually given intravenously; however, if the condition fails to improve, surgical treatment is necessary. This involves draining any pus from the middle ear and mastoid, and removing diseased lining and bone from the mastoid.
Diseases of the inner ear
MENIÈRE’S DISEASE is a common idiopathic disorder of ENDOLYMPH control in the semicircular canals (see EAR), characterised by the triad of episodic VERTIGO with deafness and tinnitus. The cause is unknown and usually one ear only is affected at ?rst, but eventually the opposite ear is affected in approximately 50 per cent of cases. The onset of dizziness is often sudden and lasts for up to 24 hours. The hearing loss is temporary in the early stages, but with each attack there may be a progressive nerve deafness. Nausea and vomiting often occur. Treatment during the attacks includes rest and drugs to control sickness. Vasodilator drugs such as betahistine hydrochloride may be helpful. Surgical treatment is sometimes required if crippling attacks of dizziness persist despite these measures. OTOSCLEROSIS A disorder of the middle ear that results in progressive deafness. Often running in families, otosclerosis affects about one person in 200; it customarily occurs early in adult life. An overgrowth of bone ?xes the stapes (the innermost bone of the middle ear) and stops sound vibrations from being transmitted to the inner ear. The result is conductive deafness. The disorder usually affects both ears. Those affected tend to talk quietly and deafness increases over a 10–15 year period. Tinnitus often occurs, and occasionally vertigo.
Abnormal hearing tests point to the diagnosis; the deafness may be partially overcome with a hearing aid but surgery is eventually needed. This involves replacing the stapes bone with a synthetic substitute (stapedectomy). (See also OTIC BAROTRAUMA.)... ear, diseases of
An adenoidectomy is usually performed on a child with abnormally large adenoids that are causing recurrent infections of the middle ear or air sinuses.
The operation may be performed together with tonsillectomy.... adenoidectomy
People with sleep apnoea may not be aware of any problem during the night, but they may be sleepy during the day, with poor memory and concentration. Severe sleep apnoea is potentially serious and may lead to hypertension, heart failure, myocardial infarction, or stroke.
Obstructive sleep apnoea is the most common type and may affect anyone, but more often middle-aged men, especially those who are overweight. The most common cause is over-relaxation of the muscles of the soft palate in the pharynx, which obstructs the passage of air. Obstruction may also be caused by enlarged tonsils or adenoids. The obstruction causes snoring. If complete blockage occurs, breathing stops. This triggers the brain to restart breathing, and the person may gasp and wake briefly.
In central sleep apnoea, breathing stops because the chest and diaphragm muscles temporarily cease to work, probably due to a disturbance in the brain’s control of breathing. Causes include paralysis of the diaphragm and disorders of the brainstem. Snoring is not a main feature.People who are overweight may find losing weight helps.
Alcohol and sleeping drugs aggravate sleep apnoea.
In one treatment, air from a compressor is forced into the airway via a mask worn over the nose.
Night-time artificial ventilation may be needed.
Tonsillectomy, adenoidectomy, or surgery to shorten or stiffen the soft palate may be performed.... sleep apnoea
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