An unusual case of necrotizing otitis externa By A. C. JOHN and N. B. HOPKIN (London) Introduction
are presented of a non-diabetic patient who survived bilateral necrotizing otitis externa with multiple cranial nerve palsies. DETAILS
Case report S.A. This 80-year-old male was referred to the Royal National Throat, Nose and Ear Hospital with a long history of severe pain in both ears. A diagnosis of otitis externa had been made. He had been previously fit and had swum most days till his illness. Examination showed bilateral gross oedema of the meatal walls. The condition did not respond to intensive daily out-patient treatment and systemic antibiotic therapy, and the patient was admitted on 10.9.76 for examination of the ears under general anaesthesia. Granulations bathed in pus were evident in both canals at the junction of the bony and cartilaginous meati. This pus showed a pure growth of pseudomonas pyocyanea on culture. The severe pain continued and the patient developed tenderness and oedema at the angle of the mandible on the left. At this time a diagnosis of necrotizing otitis externa was made in spite of three negative random blood sugar results and a negative full glucose tolerance test on two occasions (Table I). The patient proved to be allergic to carbenicillin and treatment with systemic and local gentamicin was started. On 6 October wide surgical debridement of both ears was carried out and much obviously diseased bone removed from the anterior walls of both bony canals. The tympanic membranes appeared normal. A rapidly rising blood urea and the sudden onset of marked unsteadiness necessitated discontinuation of the systemic gentamicin. A few days after this a profound bilateral sensorineural deafness became apparent. Pain continued unabated and on 3 November the ears were explored by an endaural approach. Further bone was removed along with much necrotic cartilage. On the right side there was a large abscess cavity at the junction of the bony and cartilaginous canals anteriorly. Regular post-operative packing with Eusol and paraffin gauze was started. By 15 November the pain in the ears had gone, the patient's general condition had improved dramatically and he was discharged. Three days after discharge the patient presented with acute dysphagia and hoarseness and on examination he was found to have a left vocal cord palsy, a left palatal palsy, left pharyngeal weakness and paralysis of the left side of the tongue. A left 'jugular foramen syndrome' was diagnosed. He was readmitted and a nasogastric tube introduced. X-rays of the left jugular foramen showed bone erosion in this region. On the 2 December the left ear was explored (A.D.C.). Cortical mastoidectomy showed oedematous mucosa in a cellular mastoid but there was no obvious 259
A. C. JOHN AND N. B. HOPKIN
bone erosion, granulation tissue or evidence of infection in the middle-ear cleft. The inferior aspect of the temporal bone was then explored by an infra-temporal approach, with total decompression of the facial nerve in its vertical course. Large amounts of necrotic bone were removed from the temporal bone inferior to the middle ear and in the region of the styloid process. Post-operatively a neurapraxia of the facial nerve was noted. With daily dressings of the large wound with phenoxytol there was a rapid improvement in the condition of the left ear and the pain subsided. On 13 January, 1977, a gastrostomy was performed and the right ear explored since obvious infection with severe local pain persisted in this ear. The lower half of the right pinna had become necrotic and was excised (Fig. 1). Findings on the right side were similar to those on the left. There was a considerable amount of necrotic bone in the region of the jugular foramen. To cover the large raw area created by the infratemporal debridement a posteriorly based skin flap was rotated to cover this area (Fig. 2). At this time a left corneal abrasion was noticed. A swab from the surface of the eye showed a heavy growth of pseudomonas pyocyanea and in spite of local therapy hypopyon developed. The patient was seen by Mr. John Wright of
FIG. 1 Photograph to show necrosis of the lower part of the right pinna which was subsequently excised.
FIG. 2 Photograph to show the posteriorly based skin flap rotated to fill the infra-temporal defect.
Moorfield's who recommended subconjunctival injections of Soframycin, 1 per cent atropine drops and hourly Soframycin drops. Vision was reduced to light perception only. By 31 January the left ear was clean and dry. The right ear was still producing some pus from which pseudomonas pyocyanea could be grown and the patient was started on metronidazole orally. Daily packing of the right ear with phenoxytol soaked gauze was continued, By 3 March both ears were clean and pain-free and the patient was discharged seven months after admission and after eight surgical procedures. At discharge the patient could swallow without difficulty and his hoarseness had gone. His left facial palsy had recovered. However, he is now totally deaf in both ears probably as a result of the continuing effect of geniamicin toxicity. Pathological, radiological and audiological tests Repeated swabs of the discharge from both ears from 11 September, 1976 to 28 January, 1977, showed heavy growths of pseudomonas pyocyanea with in vitro sensitivity to gentamicin, carbenicillin and colomycin among others. No* other organism was grown.
A. C. JOHN AND N. B. HOPKIN
On four occasions during his stay in hospital the patient needed transfusion because of hypochromic anaemia. Presumably this was caused by the depressant effect of chronic infection on the bone marrow. Serum immunoglobulin levels were normal. Electrophoresis showed a small diffuse increase in the gammaglobulins. At initial presentation there was a bilateral conductive deafness of about 40 dB. This was probably due to mucosal oedema and granulations in the middle ear and Eustachian tube obstruction. In the case discussed by Morgenstein and Seung (1971) in which the temporal bone was examined post mortem, the middle ear was filled with exuberant fibrous tissue of various degrees of maturity. There was also an intense inflammatory response especially near the Eustachian tube. After gentamicin therapy a profound bilateral sensori-neural deafness of about 80 dB loss developed and subsequently hearing loss became complete. Apart from the effects of progressive surgical bone removal routine mastoid X-rays showed only generally diminished translucency. Bone and cartilage removed at the various operations showed only intense chronic inflammatory changes along with some new bone formation (Fig. 3). Repeated random blood sugars, mostly post prandial, were within normal limits. Urinalysis was always negative. Full glucose tolerance tests were per-
FIG. 3 Detail from histological slide of tissue taken at operation from the region of the right styloid process 1o show necrotic bone (top left), new bone formation (top right), immature fibrous tissue (bottom right) and acute inflammatory tissue (bottom left).
TABLE I (a) Glucose Tolerance Test results of 24 Fasting blood glucose level Half hour glucose level One hour glucose level One and a half hour glucose level Two hour glucose level Two and a half hour glucose level Three hour glucose level All urines sugar free (b) Glucose Tolerance Test results of 29 Fasting blood glucose level Half hour glucose level One hour glucose level One and a half hour glucose level Two hour glucose level Two and a half hour glucose level All urines sugar free
September, 1976 4-4 millimol per litre 8-5 „ 10-1 91 5-8 3-2 3-2
October, 1976 5-0 millimol per litre 8-2 8-6 8-6 7-3 7-0
formed (Table I). In view of the importance of establishing the metabolic state of this patient, these test results were later assessed by a diabetologist nominated by the British Diabetic Association. This physician stated that, taking into account the advanced age of the patient, neither test result could be interpreted to uphold a diagnosis of diabetes in this case. Discussion Malignant or necrotizing otitis externa is now a well recognized entity. Fiftyfour cases have appeared in the literature since its description by Meltzer and Kelemen (1959). Chandler (1972) has described a series of 38 cases, many referred from other parts of the United States. The case described above ran along a familiar path with severe pain, an elderly victim, pseudomonas pyocyanca infection, multiple cranial nerve palsies, prolonged hospitalization and the necessity for radical excision of the disease. Wilson et al. (1971) state: 'The catalyst of the disease appears to be diabetes mellitus. Diffuse pseudomonas otitis externa is a common infection that responds to local treatment without the use of systemic antibiotics. However, it is wellknown that any infection in a diabetic can be a serious problem.' Of the fifty-four cases referred to in the literature, fifty-two seem to have been unequivocally diabetic, Shanon et of. (1972) refer to a patient with unilateral necrotizing otitis externa who died during a craniotomy and who did not appear to have diabetes. It would appear that necrotizing otitis externa, especially bilateral and of such severity as to cause cranial nerve palsies, is of very rare occurrence in the nondiabetic. As the above case illustrates the diagnosis should not be ruled out on this account. Management in this case was complicated by the patient's allergy to carbenicillin and the severe side effects caused by gentamicin which ultimately made the drug unusable. The speed with which the persisting intractable discharge in the right ear cleared and the improvement in the patient's general condition after metronidazole was started could lead one to advocate use of this drug earlier in the course of this disease.
A. C. JOHN AND N. B. HOPKIN
Likewise there appeared to be little response to locally applied antibiotic ear drops including gentamicin in spite of the apparent in vitro sensitivity of the organism to them. Once extensive excision had created a large open wound it seemed better to rely on repeated packing with antiseptics, and phenoxytol was found to be especially useful. Acknowledgements
We are grateful to Professor D. F. N. Harrison and Mr. A. D. Cheesman of the Professorial Unit, Royal National Throat, Nose and Ear Hospital, London WC1, for permission to report this case. The illustrations were produced by the Photographic Department of the Royal National Throat, Nose and Ear Hospital with the help of Dr. M. Gregory. REFERENCES CHANDLER, J. R. (1972) Annals of Otology, Minotogy and Laryngology, 81, 648. MELTZER, P. E., and KELEMEN, G. (1959) The Laryngoscope, 69, 1300. MORGENSTEIN, K. M., and SEUNG, H. I. (1971) The Laryngoscope, 81, 200. SHANON, E,, BIALYSTOCK, G., SCHUJMAN, E., and LOEWENTHAL, M. (1972) Ada Otolaryngo-
logica, 73, 374. WILSON, D. E., PULEC, J. L., and LINTHICUM, F. H. (1971) Archives ofOtolaryngology, 93, 419.
The Roya] National Throat, Nose and Ear Hospital, Gray's Inn Road, London, WC1.