syndrome and Miesher’s cheilitis granulomatosa. Melkersson-Rosenthal syndrome is primarily a triad of fissured tongue, facial or labial swelling, and facial palsy but there may be other lesions." Some patients with this syndrome have allergic symptoms,13 slightly raised serum immunoglobulin concentrations, and a raised erythrocyte sedimentation rate.13 The suggestion14
Crohn’s disease). Nevertheless, only very few patients with Melkersson-Rosenthal syndrome have manifested sarcoidosis or Crohn’s disease. 18 Miescher’s granulomatous cheilitis is characterised by isolated labial swelling, and is probably an oligosymptomatic form of Melkersson-Rosenthal syndrome.13 A very few patients have had or have progressed to Crohn’s disease, but again most do not. Thus it is clear that orofacial lesions with noncaseating granulomas on biopsy specimens may arise in the absence of any evidence of intestinal Crohn’s disease. The aetiology of these conditions is unclear, but some appear to be idiosyncratic responses to defmed food constituents or additives or to other exogenous factors. Management should include biopsy of the lesion and exclusion of intestinal Crohn’s disease by haematological and biochemical investigations; if there are abdominal symptoms, bowel radiography and possibly biopsy are indicated. Histological features of oral biopsy specimens in Crohn’s disease and in orofacial granulomatosis are essentially identical. There is oedema of the superficial corium with lymphangiectasia, and both diffuse and focal aggregates of small lymphocytes. In longstanding lesions there may be irregular fibrosis. Non-caseating epithelioid granulomas with or without multinucleated giant cells may be present but are often poorly formed and patchily distributed. Aggregates of mononuclear cells or frank granulomas can sometimes be seen bulging into or blocking lymphatic vessels. Occasionally there is ulceration of the overlying epithelium. Sarcoid granulomas are typically well-formed and discrete or confluent and are usually readily distinguishable from those of orofacial granulomatosis. For gingival lesions the distinction is much harder because of the dense inflammatory overlay. It may therefore be necessary to exclude sarcoidosis by chest radiography, serum angiotensin-converting enzyme measurements, and possibly a Kveim test. The outlook for patients with orofacial granulomatosis is variable and treatment is often unrewarding. Occasionally the condition remits spontaneously, especially in younger individuals.6,19 Some patients have only mild features and require no treatment, although tooth-associated infections should be eliminated.B Facial swelling, oral ulceration, or other lesions will necessitate therapy; a few of these abnormalities resolve with elimination diets but others may require topical, systemic, or intralesional corticosteroids.6,8,13 Danazol may be helpfupo and clofazimine has been effective, at least in Miescher’s granulomatous cheilitis.21 Hydroxychloroquine benefited one patient with Miescher’s cheilitis.22 Salazosulphapyridine, co-
that the condition is a variant of sarcoidosis has not been confirmed,ls although occasional patients have a positive Kveim reaction16 or raised serum angiotensin-converting enzyme concentrations17 (the Kveim reaction is occasionally positive in classic
trimoxazole, metronidazole, azathioprine, or cyclosporin have had little beneficial effect in the few patients in whom they have been tried.6,8,13 Surgical reduction of the facial swelling is probably not warranted or reliably effective.13
5. Ward JR, Williams HJ, Egger MJ, et al. Comparison of auranofin, gold sodium thiomalate, and placebo in the treatment of rheumatoid arthritis: a controlled clinical trial. Arthritis Rheum 1983; 26: 1303-15. 6. Williams HJ, Ward JR, Dahl SL, et al. A controlled trial comparing sulfasalazine, gold sodium thiomalate, and placebo in rheumatoid arthritis. Arthritis Rheum 1988; 31: 703-13. 7. Kushner I. Does aggressive therapy of rheumatoid arthritis affect outcome? J Rheumatol 1989; 16: 104. 8. Epstein WV. Parenteral gold therapy for rheumatoid arthritis: a treatment whose time has gone. J Rheumatol 1989; 16: 1291-94. 9. Editorial. Gold therapy in 1975. Br Med J 1975; ii: 156-57. 10. Epstein WV, Henke CJ, Yelin EH, Katz PP. Effect of parenterally administered gold therapy on the course of adult rheumatoid arthritis. Ann Intern Med 1991; 114: 437-44. 11. Corkill MM, Kirkham BW, Chikanza IC, Gibson T, Panayi GS. Intramuscular depot methylprednisolone induction of chrysotherapy in rheumatoid arthritis: a 24-week randomized controlled trial. Br J Rheumatol 1990; 29: 274-79. 12. Lehtinen K, Isomaki H. Intramuscular gold therapy is associated with long survival in patients with rheumatoid arthritis. J Rheumatol 1991; 18: 524-29.
Oral lesions are common in patients with proven Crohn’s disease of the intestine and include aphthae,l,2 diffuse swellings of the cheeks or lips,2 chronic inflammatory hyperplasia with fissuring and a cobblestone appearance of the mucosa,3mucosal tags,22 vertical fissures in the lips,4 and hyperplastic gingivitis.S These lesions may antedate bowel symptoms, often by several years,and may be the only obvious site of disease.Some patients with oral lesions-between 10% and 48% in various serieshave symptomless intestinal disease.8 Wiesenfeld et al8 have introduced the term orofacial granulomatosis for lesions that resemble those of Crohn’s disease clinically and histologically in patients who do
have accompanying gastrointestinal abnormalities, on the grounds that non-caseating granulomas are often seen in biopsy specimens. A familial pattern has been recognised in some cases of orofacial granulomatosis9 but not all.8 Between 12% and 60% of patients are atopic, and a few patients seem to have specific intolerances to foods or not
additives-eg, cinnamaldehyde, carvone, peperitone, cocoa, carmosine, sun yellow, or monosodium glutamate"—or to materials such as cobalt. 12 The distinction between orofacial granulomatosis with other conditions and non-caseating granulomas--eg, oral Crohn’s disease-may not be easy. Orofacial granulomatosis may be part of a spectrum of disorders that includes MelkerssonRosenthal
1. Croft CB, Wilkinson AR. Ulceration of the mouth, pharynx, and larynx in Crohn’s disease of the intestine. Br J Surg 1972; 59: 249-52. 2. Basu MK, Asquith P, Thompson RA, Cooke WT. Oral manifestations of Crohn’s disease. Gut 1975; 16: 249-54. 3. Issa MA. Crohn’s disease of the mouth. Br Dent J 1971; 130: 247-48. 4. Schiller KFR, Golding PL, Peebles RA, Whitehead J. Crohn’s disease of the mouth and lips. Gut 1971; 12: 864-65. 5. Frankel DH, Mostofi RS, Loriniz AL. Oral Crohn’s disease: report of two cases in brothers with metallic dysgeusia and a review of the literature. J Am Acad Dermatol 1985; 112: 260-68. 6. Williams AJK, Wray D, Ferguson A. The clinical entity of orofacial Crohn’s disease. Q J Med 1991; 79: 451-58. 7. Scully C, Cochran KM, Russell RI, et al. Crohn’s disease of the mouth: an indicator of intestinal involvement. Gut 1982; 23: 198-201. 8. Wiesenfeld D, Ferguson MM, Mitchell DN, et al. Orofacial granulomatosis—a clinical and pathological analysis. Q J Med 1985; 54: 101-13. 9. Levenson MJ, Ingerman M, Grimes C, Anand V. Melkersson-Rosenthal syndrome. Arch Otolaryngol 1984; 110: 540-2. 10. James J, Patton DW, Lewis CJ, et al. Orofacial granulomatosis and clinical atopy. J Oral Med 1986; 41: 29-30. 11. Sweatman MC, Tasker R, Warner JO, et al. Oro-facial granulomatosis response to elemental diet and provocation by food additives. Clin Allergy 1986; 16: 331-38. 12. Pryce DW, King CM. Orofacial granulomatosis associated with delayed hypersensitivity to cobalt. Clin Exp Dermatol 1990; 15: 384-96. 13. Worsaae N, Christensen KC, Schiodt M, et al. Melkersson-Rosenthal syndrome and cheilitis granulomatosa. Oral Surg 1982; 54: 404-13. 14. Hering H, Scheid P. Kritische Bemerkungen zum MelkerssonRosenthal-syndrom als Teilbild des Morbus Besnier-BoeckSchaumann. Arch Dermatol Syph (Berlin) 1954; 197: 344-82. 15. Lindelof B, Eklund A, Liden S. Kveim test reactivity in MelkerssonRosenthal syndrome (cheilitis granulomatosa). Acta Derm Venerol 1985; 65: 443-45. 16. Shehade SA, Foulds IS. Granulomatous cheilitis and a positive Kveim test. Br J Dermatol 1986; 115: 619-22. 17. Orlando MR, Atkins JS. Melkersson-Rosenthal syndrome. Arch Otolaryngol Head Neck Surg 1990; 116: 728-29. 18. Greene RM, Rogers RS. Melkersson-Rosenthal syndrome: a review of 36 patients. J Am Acad Dermatol 1989; 21: 1263-70. 19. Field EA, Tyldesley WR. Oral Crohn’s disease revisited-a 10 year review. Br J Oral Maxillofac Surg 1989; 27: 114-23. 20. Madanes AZ, Farber M. Danazol. Ann Intern Med 1982; 96: 625-30. 21. Podmore P, Burrows D. Clofazimine—an effective treatment for Melkersson-Rosenthal syndrome or Miescher’s cheilitis. Clin Exp Dermatol 1986; 11: 173-78. 22. Allen CM, Camisa C, Hamzeh S, Stephens L. Cheilitis granulomatosa: report of six cases and review of the literature. J Am Acad Dermatol
1990; 23: 444-50.
The UK National Study of Hearing has shown that, after age, the most common aetiological factor associated with permanent hearing loss in men is noise exposure;’ the same is true of tinnitus.2 In the USA, about 28 million people have impaired hearing and about half of these cases are at least partly attributable to damage from exposure to loud sounds.3 Hazardous noise exposure is by far the most common preventable cause of hearing loss, and has therefore attracted much research,4industrial health activity ,** standardisation,6 and legislation.Last year the National Institutes of Health in Bethesda held a consensus conference on the subject and subsequently issued a consensus statement.3 Noise-related hearing impairment can occur at any age; even young infants may be affected. The disability is often accompanied by tinnitus, which can be very troublesome. Loud short-lived sounds—eg, from an explosion or gunfire--can lead to immediate, severe, and permanent loss of hearing; this condition is known as acoustic trauma. Chronic exposure to less
intense, hazardous sounds—eg,
work or during leisuretime activities-results in a gradual diminution of hearing sensitivity of which the victim is initially unaware. The latter disorder is noise-induced hearing loss (NIHL). In NIHL, each exposure to noise damages a few hair cells. As exposure continues, more and more hair cells are damaged and some are destroyed. The cells are not replaced after destruction. For reasons that are unclear, there are considerable differences in susceptibility to noise damage between individuals, between ears, and at different times within the same individual. Hearing loss is measured with pure-tone audiometry, by which auditory thresholds are determined at various frequencies. The first audiometric sign of NIHL in each ear is usually loss of sensitivity at higher frequencies, maximal at 3, 4, or 6 kHz but with less loss at 8 kHz, hence the characteristic notch configuration. This notch may be eliminated with further hearing loss from noise or ageing, so the presence or absence of NIHL cannot be firmly established from the audiogram alone. Both ears are usually affected, but often not to the same at
As the consensus statement points out, although the energy in speech is largely contained within the low frequency range, much of the information required to understand speech resides within the higher frequencies, and is therefore inaudible to patients with NIHL. Other associated dysfunctions—eg, of frequency and temporal resolution-cause particular hearing difficulties with background noise, competing voices, or room reverberation. Permanent hearing loss is common with sound levels above 85 dB(A) and exposure of 8 h/day for many years.Thus, the often cited 90 dB(A) level does not imply safety, but is a compromise limiting the potential hazard. To predict the likelihood of NIHL one requires knowledge of typical durations and the number of exposures. The trade-off between noise level and exposure duration has been defmed
intemationally6,7 by the total-energy concept; a doubling of noise exposure duration is regarded as equivalent to a 3 dB increase in noise level. Nevertheless, some American regulations use 5 dB per doubling of duration, whereas a more recent analysis of worldwide epidemiological data8 casts doubt on any simple rule. With noise levels above 140 dB peak, as in acoustic trauma, far more damage is liable to result than is predicted from the energy concept. As a rule of thumb, hazardous noise should be suspected if the listeners find communication difficult while exposed to the sound, or if they experience ringing in the ears and/or sounds seem muffled after they leave the sound exposure area. The acoustic energy of the sound reaching the ear, not its source, is the most important factor-at work, at school, at home, or during leisure activities.9,10 With regard to prevention, the NIH consensus report draws four conclusions. Personal hearing protection should be used whenever hazardous