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A rare cause of unilateral hearing loss

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Q1: What is the differential diagnosis?

The cause of sarcoidosis is still obscure and it is a diagnosis of exclusion. The differential diagnosis of sarcoidosis of nasopharynx is shown below (box 1).

Box 1: Differential diagnosis of sarcoidosis of nasopharynx

This can be classified into specific, non-specific, and others

Specific

  • Tuberculosis

  • Leprosy

  • Lupus vulgaris

  • Syphilis

  • Rhinosporidiosis

  • Mucormycosis

  • Aspergillosis

  • Histoplasmosis

  • Blastomycosis

  • Sporotrichosis

  • Leishmaniasis

    Non-specific

  • Foreign body granuloma

  • Wegener's granulomata

    Others

  • AIDS

  • Hodgkin's lymphoma

  • Lethal midline granuloma (midfacial lymphoma)

  • T cell lymphoma, for example, Lennert's lymphoma

  • Chronic beryllosis

  • Crohn's disease

  • Drugs

Q2: What does the chest radiograph show?

The chest radiograph (see p 584) shows bilateral hilar shadows and there are also widespread nodular shadows throughout both lung fields with a normal heart size.

Q3: What is the cause of deafness in the left ear?

The cause of the deafness is left otitis media with effusion (glue ear). All adult patients with unilateral otitis media must have biopsy of the nasopharynx to exclude pathology especially malignant tumour. Sarcoidosis may involve the nasopharynx causing inflammatory reaction which may result in eustachian tube dysfunction and leads to otitis media with effusion. There may also be direct involvement of the middle ear mucosa by sarcoidosis. Tympanometry is the measurement of compliance and resistance or impedance of the middle ear mechanism. The result obtained can be classified broadly into three groups. Type A shows symmetrical graph with maximum compliance at 0 mm H2O as in normal middle ear mechanism, type B shows a flat trace indicating a marked decrease in compliance usually due to damping of middle ear mechanism and tympanic membrane by presence of fluid associated with normal ear canal volume or a perforation of tympanic membrane if there is a large canal volume. Type C shows that the maximum compliance is displaced into the negative region below −100 mm H2O. Type B tympanogram in the case presented suggests otitis media with effusion.

Q4: What are the otolaryngological manifestations of sarcoidosis?

The otolaryngological manifestations of sarcoidosis are rare and the sites most frequently affected are the mucosal surfaces of the upper aerodigestive tract including the nose, pharnx, larynx, lymph nodes, salivary glands, conjunctiva, and uveal tract. Other less common sites affected are nasopharynx, middle ear, and facial nerve. Nasal sarcoidosis occurs in 3%–20% of systemic cases. The presenting nasal symptoms include discharge which ranges from serosanguinous to mucopurulent, nasal obstruction, and epistaxis. There may be secondary sinusitis as a result of superadded infection involvement with the disease. Nasal skin and bone lesions are asymptomatic. There may be generalised swelling with discoloration of the overlying skin. Septal perforation may occur spontaneously or after septal surgery in unrecognised cases. Sarcoidosis should always be considered in recurrent alternating Bell's palsy which is a component of Melkerson-Rosenthal syndrome. Involvement of the uveal tract in the eye and the parotid glands is seen in Heerfordt's syndrome.

Discussion

Sarcoidosis is a systemic disease of unknown aetiology characterised by non-caseating epitheliod granuloma of various organs. The manifestations of sarcoidosis are protean, ranging from asymptomatic but abnormal findings on chest radiography in many patients to progressive multiorgan failure in a minority. The aetiology of sarcoidosis is unknown, atypical mycobacteria tuberculi has been suggested as a causative factor but attempts to confirm this by polymerase chain reaction (PCR) has been inconclusive. It occurs world wide and affects all races, both sexes, and all ages but it is commoner in ages from 20 to 40 years. It is commoner and more severe in black people than whites.1 Evidence of involvement of the nasopharynx is very rare and there have been only two reports in the literature within the past three decades.2 3

There is still no specific diagnostic test for sarcoidosis. Kveim-Siltzbach is positive in all mucosal cases and 75% of active sarcoidosis but this is no longer used because of the risk of transmission of viral hepatitis and AIDS. Transbronchial biopsy is the most useful and it is positive in 90% of pulmonary sarcoidosis. Serum angiotensin converting enzyme may be positive but it is not specific for the disease. It is useful however in assessing the activity of the disease and also as a guide to treatment with corticosteroids. There have been attempts by several groups to isolate DNA fromMycobacteria tuberculosis from samples of patients with sarcoidosis using sequence capture-PCR but most reports showed that M tuberculosis does not play a pathogenic part in sarcoidosis in most patients.4

Systemic corticosteroids remain the mainstay of treatment and the use of topical beclomethasone dipropionate has been proved to be helpful in patients whose symptoms are confined to the nasal mucosa.5Other drugs that have been used in the treatment of sarcoidosis are azathioprine, chlorambucil, and cyclophosphamide but the experience of their efficacy is anecdotal and limited.1 Laser surgery may be used for reduction of ganuloma in the larynx or the nose until possible spontaneous regression occurs.6 Sarcoidosis is usually self limiting with spontaneous resolution, although in a few patients there is progressive downhill course, culminating in irreversible fibrosis and severe impairment of organ function.7

Final diagnosis

Sarcoidosis of the nasopharynx.

References

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