This can also present with tonsillitis, although clinically the tonsils have a white exudate, there is often a petechial rash on the soft palate and an accompanying lymphadenopathy.

Quinsy

Quinsy is a collection of pus outside the capsule of the tonsil usually located adjacent to its superior pole. The patient often has trismus making examination difficult but the pus pushes the uvula across the midline to the opposite side. The area is usually hyperaemic and smooth but unilateral tonsil ulceration is more likely to be a malignancy. In either case urgent referral to an ENT specialist is essential.

Indications for a tonsillectomy are shown in Table 20.4. This is carried out under a general anaesthetic and current surgical techniques include diathermy dissection, laser excision and coblation (using an ultrasonic dissecting probe). There are strong advocates for each technique and much will depend on the individual surgeon’s preference. Some departments now carry out tonsillectomy as a day case procedure as most reactionary bleeding will occur within the first 8 hours postoperatively.

Viral infections of the throat are common and, although many practitioners are under pressure from the patient to give antibiotics, the vast majority are usually self-limiting, settling with bed rest, analgesia and encouraging fluid intake. Fungal infections, usually candidiasis, are uncommon and may indicate an immunocompromised patient or undiagnosed diabetes.

Tonsillitis

Tonsillitis, with a good history of pyrexia, dysphagia, lymphadenopathy and severe malaise is usually bacterial with β-haemolytic streptococcus the commonest organism.

Genital herpes is one of the most common STIs world-wide. Between 1997 and 2002 there has been a 17% increase in diagnoses of genital herpes in the UK. The peak incidence is in 16- to 24-year-olds of both sexes. Infection may be either primary or recurrent. Transmission occurs during close contact with a person who is shedding virus. Most genital herpes is due to type 2. Genital contact with oral lesions caused by HSV-1 can also produce genital infection.

Susceptible mucous membranes include the genital tract, rectum, mouth and oropharynx. The virus has the ability to establish latency in the dorsal root ganglia by ascending peripheral sensory nerves from the area of inoculation. It is this ability which allows for recurrent attacks.

Clinical features

Asymptomatic infection has been reported but is rare. Primary genital herpes is usually accompanied by systemic symptoms of varying severity including fever, myalgia and headache. Multiple painful shallow ulcers develop which may coalesce. Atypical lesions are common. Tender inguinal lymphadenopathy is usual. Over a period of 10-14 days the lesions develop crusts and dry. In women with vulval lesions the cervix is almost always involved. Rectal infection may lead to a florid proctitis. Neurological complications can include aseptic encephalitis and/or involvement of the sacral autonomic plexus leading to retention of urine.

Recurrent attacks occur in a significant proportion of people following the initial episode. Precipitating factors vary, as does the frequency of recurrence. A symptom prodrome is present in some people prior to the appearance of lesions. Systemic symptoms are rare in recurrent attacks.

The clinical manifestations in immunosuppressed patients (including those with HIV) may be more severe, asymptomatic shedding increased, and recurrences occur with greater frequency. Systemic spread has been documented.

Diagnosis

Although the history and examination can be highly suggestive of HSV infection, a firm diagnosis can be made only on the basis of isolation of virus from lesions. Swabs should be taken from the base of lesions and placed in viral transport medium. Virus is most easily isolated from new lesions. Type-specific immune responses can be found 8-12 weeks following primary infection and may form the basis for newer serological assays, although these are not yet available in routine clinical practice.

Management

Primary

Saltwater bathing or sitting in a warm bath is soothing and may allow the patient to pass urine with some degree of comfort. Aciclovir 200 mg five times daily, famciclovir 250 mg three times daily or valaciclovir 500 mg twice daily, all for 5 days, are useful if patients are seen whilst new lesions are still forming. If lesions are already crusting, antiviral therapy will do little to change the clinical course. Secondary bacterial infection occasionally occurs and should be treated. Rest, analgesia and antipyretics should be advised. In rare instances patients may need to be admitted to hospital and aciclovir given intravenously, particularly if HSV encephalitis is suspected.

Recurrence

Recurrent attacks tend to be less severe and can be managed with simple measures such as saltwater bathing. Psychological morbidity is associated with recurrent genital herpes and frequent recurrences impose strains on relationships; patients need considerable support. Long-term suppressive therapy is given in patients with frequent recurrences. An initial course of aciclovir 400 mg twice daily or valaciclovir 250 mg twice daily for 6-12 months significantly reduces the frequency of attacks, although there may still be some breakthrough. Therapy should be discontinued after 12 months and the frequency of recurrent attacks reassessed.

HSV in pregnancy

The potential risk of infection to the neonate needs to be considered in addition to the health of the mother. Infection occurs either transplacentally or via the birth canal. If HSV is acquired for the first time during pregnancy, transplacental infection of the fetus may, rarely, occur. Management of primary HSV in the first or second trimester will depend on the woman’s clinical condition and aciclovir can be prescribed in standard doses. Aciclovir therapy during the last 4 weeks of pregnancy may prevent recurrence at term.

Primary acquisition in the third trimester or at term with high levels of viral shedding usually leads to delivery by caesarean section.
For women with previous infection, concern focuses on the baby acquiring HSV from the birth canal. The risk is very low in recurrent attacks. For women with recurrent episodes, only those with genital lesions at the onset of labour are delivered by caesarean section. Sequential cultures during the last weeks of pregnancy to predict viral shedding at term are no longer indicated.

Prevention and control

Patients must be advised that they are infectious when lesions are present; sexual intercourse should be avoided during this time or during prodromal stages. Condoms may not be effective as lesions may occur outside the areas covered. Sexual partners should be examined and may need information on avoiding infection.

Chancroid or soft chancre is an acute STI caused by Haemophilus ducreyi. It is probably the commonest cause of genital ulceration world-wide, and is prevalent in parts of Africa and Asia. Epidemiological studies in Africa have shown an association between genital ulcer disease, frequently chancroid, and the acquisition of HIV infection. A new urgency to control chancroid has resulted from these observations.

Clinical features

The incubation period is 3-10 days. At the site of inoculation an erythematous papular lesion forms which then breaks down into an ulcer. The ulcer frequently has a necrotic base, a ragged edge, bleeds easily and is painful. Several ulcers may merge to form giant serpiginous lesions. Ulcers appear most commonly on the prepuce and frenulum in men and can erode through tissues. In women the most commonly affected site is the vaginal entrance and the perineum. The lesions in women sometimes go unnoticed.
At the same time, inguinal lymphadenopathy develops (usually unilateral) and can progress to form large buboes which suppurate.

Diagnosis and treatment

Chancroid must be differentiated from other genital ulcer diseases (see Table 2.48). Co-infection with syphilis and herpes simplex is common. Isolation of H. ducreyi in specialized culture media is definitive but difficult. Swabs should be taken from the ulcer and material aspirated from the local lymph nodes for culture. Polymerase chain reaction (PCR) techniques are available. Gram stains of clinical material may show characteristic coccobacilli.

Single-dose regimens include azithromycin 1 g orally or ceftriaxone 250 mg i.m. Other regimens include ciprofloxacin 500 mg twice daily for 3 days, erythromycin 500 mg four times daily for 7 days. Clinically significant plasmid-mediated antibiotic resistance in H. ducreyi is developing.

Patients should be followed up at 3-7 days, when if treatment is successful ulcers will be responding.

Sexual partners should be examined and treated epidemiologically, as asymptomatic carriage has been reported.

HIV-infected patients should be closely monitored, as healing may be slower. Multiple-dose regimens are needed in HIV patients since treatment failures have been reported with single-dose therapy.

Syphilis is a chronic systemic disease, which is acquired or congenital. In its early stages diagnosis and treatment are straightforward but untreated it can cause complex sequelae in many organs and eventually lead to death.

The causative organism, Treponema pallidum (TP), is a motile spirochaete that is acquired either by close sexual contact or can be transmitted transplacentally. The organism enters the new host through breaches in squamous or columnar epithelium. Primary infection of non-genital sites may occasionally occur but is rare.

Both acquired and congenital syphilis have early and late stages, each of which has classic clinical features (Table 2.49).

Primary

Between 10-90 days (mean 21 days) after exposure to the pathogen a papule develops at the site of inoculation. This ulcerates to become a painless, firm chancre. There is usually painless regional lymphadenopathy in association. The primary lesion may go unnoticed, especially if it is on the cervix or within the rectum. Healing occurs spontaneously within 2-3 weeks.

Table 2-49. Classification and clinical features of syphilis

	Clinical features
Acquired
Early stages
Primary	Hard chancre Painless, regional lymphadenopathy
Secondary	General: Fever, malaise, arthralgia, sore throat and generalized lymphadenopathy Skin: Red/brown maculopapular non-itchy, sometimes scaly rash; condylomata lata Mucous membranes: Mucous patches, ’snail-track’ ulcers in oropharynx and on genitalia
Late stages
Tertiary	Late benign: Gummas (bone and viscera) Cardiovascular: Aortitis and aortic regurgitation Neurosyphilis: Meningovascular involvement, general paralysis of the insane (GPI) and tabes dorsalis
Congenital	Stillbirth or failure to thrive
Early stages	‘Snuffles’ (nasal infection with discharge) Skin and mucous membrane lesions as in secondary syphilis
Late stages	‘Stigmata‘: Hutchinson’s teeth, ’sabre’ tibia and abnormalities of long bones Keratitis, uveitis, facial gummas and CNS disease

Secondary

Between 4-10 weeks after the appearance of the primary lesion constitutional symptoms with fever, sore throat, malaise and arthralgia appear. Any organ may be affected – leading, for example, to hepatitis, nephritis, arthritis and meningitis. In a minority of cases the primary chancre may still be present and should be sought.

Signs include:

• Generalized lymphadenopathy (50%)
• Generalized skin rashes involving the whole body including the palms and soles but excluding the face (75%) – the rash, which rarely itches, may take many different forms, ranging from pink macules, through coppery papules, to frank pustules
• Condylomata lata – warty, plaque-like lesions found in the perianal area and other moist body sites
• Superficial confluent ulceration of mucosal surfaces – found in the mouth and on the genitalia, described as ’snail track ulcers’
• Acute neurological signs in less than 10% of cases (e.g. aseptic meningitis).

Untreated early syphilis in pregnant women leads to fetal infection in at least 70% of cases and may result in stillbirth in up to 30%.

Latent

Without treatment, symptoms and signs abate over 3-12 weeks, but in up to 20% of individuals may recur during a period known as early latency, a 2-year period in the UK (1 year in USA). Late latency is based on reactive syphilis serology with no clinical manifestations for at least 2 years. This can continue for many years before the late stages of syphilis become apparent.

Tertiary

Late benign syphilis, so called because of its response to therapy rather than its clinical manifestations, generally involves the skin and the bones. The characteristic lesion, the gumma (granulomatous, sometimes ulcerating, lesions), can occur anywhere in the skin, frequently at sites of trauma. Gummas are commonly found in the skull, tibia, fibula and clavicle, although any bone may be involved. Visceral gummas occur mainly in the liver (hepar lobatum) and the testes.

Infectious diseases can affect any organ or system, and can cause a wide variety of symptoms and signs. Fever is often regarded as the cardinal feature of infection, but not all febrile illnesses are infections, and not all infectious diseases present with a fever. History-taking and examination should aim to identify the site(s) of infection, and also the likely causative organism(s).

History

A detailed history is taken with specific questions about epidemiological risk factors for infection. These are based on the sources of infection and routes of transmission discussed above.

• Travel history: some diseases are more prevalent in certain geographical locations, and many infections common in the tropics are seen rarely if at all in the UK.
• Food and water history: systemic as well as gastroenteric infections can be caught via this route.
• Occupational history.
• Animal contact: domestic, farm and wild animals can all be responsible for zoonotic infection.
• Sexual activity: as well as the traditional sexually transmitted diseases, HIV, hepatitis B and very occasionally hepatitis C can all be transmitted sexually. Some enteric infections are more common among male homosexuals.
• Intravenous drug use: as well as blood-borne viruses, drug injectors are susceptible to a variety of bacterial and fungal infections due to inoculation. Tattooing, body piercing and receipt of blood products (especially outside the UK) are also risk factors for blood-borne viruses.
• Leisure activities: certain pastimes may predispose to water-borne infections or zoonoses.

Clinical examination

A thorough examination covering all systems is required. Skin rashes and lymphadenopathy are common features of infectious diseases, and the ears, eyes, mouth and throat should also be inspected. Infections commonly associated with a rash are listed in Box 2.1. Rectal, vaginal and penile examination is required in sexually transmitted infections.

The fever pattern may occasionally be helpful, e.g. the tertian fever of falciparum malaria, but too much weight should not be placed on the pattern or degree.

The ear can be divided into three parts: outer, middle and inner.
The outer ear has a skin-lined tube 2.5 cm long leading down to the tympanic membrane (the ear drum). Its outer third is cartilaginous and contains hair, sebaceous and ceruminous glands, but the walls of the inner two-thirds are bony. The outer ear is self-cleaning as the skin is migratory and there are no indications to use cotton wool buds. Wax should only be seen in the outer third.

The middle ear is an air-containing cavity derived from the branchial clefts. It communicates with the mastoid air cells superiorly, and the Eustachian tube connects it to the nasopharynx medially. The Eustachian tube ventilates the middle ear and maintains equal air pressure across the tympanic membrane. It is normally closed but opens via the action of the palatal muscles to allow air entry when swallowing or yawning. A defect in this mechanism, such as with a cleft palate, will prevent air entering the middle ear cleft which may then fill with fluid. Lying within the middle ear cavity are the three ossicles (malleus, incus and stapes) that transmit sound from the tympanic membrane to the inner ear. On the medial wall of the cavity is the horizontal segment of the facial nerve, which may be damaged during surgery or by direct extension of infection in the middle ear.

The inner ear contains the cochlea for hearing and the vestibule and semicircular canals for balance. There is a semicircular canal arranged in each body plane and these are stimulated by rotatory movement. The facial, cochlear and vestibular nerves emerge from the inner ear and run through the internal acoustic meatus to the brainstem

The ossicles, in the middle ear, transmit sound waves from the tympanic membrane to the cochlea. They amplify the waves by about 18-fold to compensate for the loss of sound waves moving from the air-filled middle ear to the fluid-filled cochlea. Hair cells in the basilar membrane of the cochlea detect the vibrations and transduce these into nerve impulses which pass to the cochlear nucleus and then eventually to the superior olivary nuclei of both sides; thus lesions central to the cochlear nucleus do not cause unilateral hearing loss.
If the ossicles are diseased, sound can also reach the cochlea by vibration of the temporal bone (bone conduction).

EXAMINATION

The pinna and postauricular region should first be examined for scars or swellings. An auroscope is used to examine the external ear canal whilst the pinna is retracted backwards and upwards to straighten the canal. Look for wax, discharge or foreign bodies. The tympanic membrane should always be seen with a light reflex anteroinferiorly. Previous repeated infections may cause a thickened, whitish drum but fluid in the middle ear may show as dullness of the drum. Perforations are marginal or central.

COMMON DISORDERS

The discharging ear (otorrhoea)

Discharge from the ear is usually due to infection of the outer or middle ear.
Otitis externa is a diffuse inflammation of the skin of the ear canal. The organism may be bacterial, viral or fungal and the patient usually complains of severe pain. Gentle pulling of the pinna is tender and there may be lymphadenopathy of the preauricular nodes.
Examination may reveal debris in the canal which needs to be removed either by gentle mopping or preferably by suction viewed directly under a microscope. In severe cases the canal may be swollen and a view of the tympanic membrane impossible. Any foreign body seen should be removed with great care by trained personnel.

Treatment is with regular cleansing and topical antibiotics combined with corticosteroids; it resolves in 3-4 days.
Otitis media can also present with discharge from the middle ear through a perforation of the tympanic membrane. There are no mucous glands in the external ear canal, however, and if the discharge is serous then middle ear pathology is unlikely.
Treatment is with systemic antibiotics.

Cholesteatoma

Cholesteatoma is defined as keratinizing squamous epithelium within the middle ear cleft and can present with foul smelling otorrhoea. Examination may show a defect in the tympanic membrane full of white cheesy material. Mastoid surgery is required to remove this sac of squamous debris as it can erode local structures such as the facial nerve or even extend intracranially.
Hearing loss

Hearing loss:

- Type Defect Rinne Weber
- Conductive Outer or middle ear Negative Sound heard louder on the affected side
- Sensorineural Inner ear or more centrally Positive Sound heard louder in the normal ear

Deafness can be conductive or sensorineural and these can be differentiated by the Rinne and the Weber tests

Rinne test

Normally a tuning fork, 512 Hz, will be heard as louder if held next to the ear (air conduction) compared to being placed on the mastoid bone (Rinne positive). If the tuning fork is perceived louder when placed on the mastoid (bone conduction), then a defect in the conducting mechanism of the external or middle ear is present (Rinne negative).

Weber test

A tuning fork placed on the bridge of the nose of a patient with normal hearing (or with symmetrical hearing loss) should be perceived centrally.

Conductive hearing loss may be due to many causes but wax is the commonest.

Perforated tympanic membrane

This may arise from trauma or chronic middle ear disease where recurrent infection results in a permanent defect. Surgical repair is only indicated if the patient is symptomatic with hearing loss or recurrent discharge.

This is an acute inflammation of the middle ear, causing severe pain (otalgia) and conductive hearing loss. This occurs because fluid accumulation in the middle ear impairs sound conduction to the cochlea. It is often viral in origin, e.g. following a cold, and will settle within 72 hours without antibacterial treatment. In patients with systemic features or after 72 hours, a systemic antibiotic, e.g. amoxicillin, should be given. Topical therapy is of no value. Complications include infection of the mastoid bone.
Acute otitis media may spread to the mastoid area and if there is tenderness and swelling over the mastoid then an urgent ENT opinion should be obtained.

Secretory otitis media with effusion (also called serous otitis media or glue ear)

This is common in children because of Eustachian tube dysfunction. The effusion resolves naturally in the majority of cases but can persist giving hearing loss, and it predisposes to recurrent attacks of acute otitis media. A grommet is a tube that is inserted into the tympanic membrane and ventilates the middle ear cavity, i.e. it takes over the Eustachian tube’s function. Grommets are extruded from the tympanic membrane as it heals (lasting from 6 months to 2 years) but if the Eustachian tube is still not working, fluid will reaccumulate in the middle ear cavity with a return of symptoms. In most children the middle third of the face grows around the age of 7-14 years and Eustachian tube dysfunction is rare after this.

Otosclerosis

This is usually a hereditary disorder where new bony deposits occur within the derivatives of the otic capsule, specifically the stapes footplate and in the cochlea. Characteristically seen in the second and third decades, it is commoner in females and can become worse during pregnancy. The hearing loss may be mixed, and treatment includes a hearing aid or replacement of the fixed stapes with a prosthesis (stapedectomy).

This is the commonest cause of deafness. It is a degenerative disorder of the cochlea and is seen in old age. The onset is gradual and the higher frequencies are affected most (Fig. 20.3). Speech has two components: low frequencies (vowels) and high frequencies (consonants). When the consonants are lost, speech loses its intelligibility. Increasing the volume merely increases the low frequencies and the characteristic response of ‘Don’t shout. I’m not deaf!’ A high-frequency-specific hearing aid will do much to ease the frustrations of both the patients and their close contacts.

Noise trauma

Cochlear damage can occur, for example, when shooting without ear protectors or from industrial noise (see p. 1030) and characteristically has a loss at 4 kHz.

Acoustic neuroma

This is a slow-growing benign Schwannoma of the vestibular nerve which can present with progressive sensorineural hearing loss. Any patient with an asymmetric sensorineural hearing loss should be investigated, e.g. with an MRI scan.

Vertigo

Vertigo is usually rotatory when it arises from the ear. The presence of otalgia, otorrhoea, tinnitus or hearing loss suggests an otologic aetiology. Vestibular causes can be classified according to the duration of the vertigo:
seconds to minutes – benign paroxysmal positional vertigo (BPPV)
minutes to hours – Ménière’s disease
hours to days – labyrinthine or central pathology.

Benign paroxysmal positional vertigo (BPPV)

BPPV is thought to be due to loose otoliths in the semicircular canals, commonly the posterior canal. Positional vertigo is precipitated by head movements, usually to a particular position, and may occur when turning in bed or on sitting up. The onset is typically sudden and distressing. The vertigo lasts seconds or minutes and the phenomenon becomes less severe on repeated movements (fatigue). There is no serious underlying cause but it sometimes follows vestibular neuronitis (p. 1190), head injury or ear infection.

Diagnosis

This is made not only on the history but by precipitating an attack by carrying out a positional test in the outpatient clinic. This test, called the Hallpike manoeuvre, involves sitting the patient on a couch and then turning the patient’s head towards the affected ear. The patient’s head is supported by the examiner and the patient then lies down so that the head is just below the horizontal. Nystagmus (following a latent interval of a few seconds) is noted, as is any subjective sensation of vertigo. A positive Hallpike test confirms BPPV, which can be cured in over 90% of cases by the Epley manoeuvre. This involves gentle but specific manipulation and rotation of the patient’s head to shift the loose otoliths from the semicircular canals.

A differential diagnosis is a cerebellar mass but here, positional nystagmus (and vertigo) is immediately apparent (no latent interval) and does not fatigue.

Ménière’s disease

This condition is characterized by recurring episodic rotatory vertigo lasting 30 minutes to a few hours; attacks are recurrent over months or years. Classically it is associated with a low frequency sensorineural hearing loss, feeling of fullness in the affected ear, loss of balance, tinnitus and vomiting. There is a build-up of endolymphatic fluid in the inner ear, although its precise aetiology is still unclear.

Treatment involves the use of vestibular sedatives, e.g. cinnarizine in the acute phase, low-salt diet, betahistine, and avoidance of caffeine. If the disease cannot be controlled in this way, then a chemical labyrinthectomy, which involves perfusing the round window orifice with ototoxic drugs such as gentamicin is possible. Gentamicin destroys the vestibular epithelium; therefore the patient has severe vertigo for around 2 weeks until the body compensates for the lack of vestibular input on that side. The patient will happily trade occasional mild vertigo when the balance system is challenged to the unpredictable, severe and disabling attacks of vertigo of Ménière’s disease.

Labyrinthine or central causes of vertigo

These are managed with vestibular sedatives in the acute phase. Most patients will settle over a few days but continuous true vertigo with nystagmus suggests a central lesion. A patient with a deficit of vestibular function due to viral labyrinthitis or neuronitis should be able to come off the vestibular sedatives within 2 weeks, as long-term use can give parkinsonian side-effects, delay central compensation and thus prolong the vertigo. Vestibular rehabilitation by a physiotherapist or audiological scientist can speed up the compensation process, although most patients will be able to do this themselves with time.

Tinnitus

This is a sensation of a sound when there is no auditory stimulus. It can occur without hearing loss. Patients describe a hissing or ringing in their ears and this can cause much distress. It usually does not have a serious cause but vascular malformation, e.g. aneurysms, or vascular tumours may be associated.
Tinnitus associated with deafness is described above.

Treatment

This is difficult. A tinnitus masker (a mechanically produced continuous soft sound) can help. A vestibular sedative, betahistine, is occasionally used.