ORIGINAL ARTICLE Idiopathic Inflammatory Medial Meatal Fibrotizing Otitis

Erkki Hopsu, MD, PhD; Anne Pitka¨ranta, MD, PhD

Background: Idiopathic inflammatory medial meatal fi- over the lateral cartilage of the canals remained rig- brotizing otitis (IMFO) is rare. Only a few cases with un- orously and constantly uninflamed through the active, known cause have been reported. relentless progression of the disease over several years, resulting in the formation of a fibrous plug of the Objective: To report 3 cases of IMFO as a specific di- medial meatal canal. The middle and mastoid air agnostic entity. cells were not affected during the active inflammatory phase. Patients and Methods: Two adults and 1 child with bilateral IMFO were observed for several years at the De- Conclusion: IMFO has its own specific pathophysi- partment of of Helsinki Univer- ologic characteristics, and perhaps also etiopathologic sity Hospital, Helsinki, Finland. characteristics, which are still unknown.

Results: Only the osseous part of the external ear ca- nals was affected by IMFO. The skin and skin organs Arch Otolaryngol Head Neck Surg. 2002;128:1313-1316

CQUIRED ATRESIA of the ex- medial meatal atresia cases in which the ternal is rela- disease presents as a defined entity. tively uncommon and may be postinflammatory, trau- matic, postoperative, or METHODS 1,2 neoplastic.AReflecting this heterogeneous Two of the patients were referred to the Otorhi- etiology, numerous synonyms have been nolaryngology Department of the Helsinki Uni- used to describe the postinflammatory cat- versity Hospital because of continuous external egory, including atresia meatus acusticus ex- ear canal and granulation. The third pa- ternus,3 chronic stenosing ,4 ac- tient, when referred to the clinic, had already de- quired external auditory atresia,5 veloped a medial canal fibrous atresia. Com- postinflammatory acquired atresia,2,6 postin- mon for all of these patients was an infectious, flammatory medial meatal fibrosis,7,8 and chronic, granulative but painless process in both medial meatal fibrosis.9 medial ear canals. Clinically, no patient had any 7 ongoing or mastoid symptoms. Com- Katzke and Pohl described a series puted tomographic scans demonstrated variabil- of 6 patients with postinflammatory me- ity in the medial meatal fibrotizing otitis: from dial meatal fibrosis and suggested that this somewhat shadowed ear canals and thickened progressive disease was a discrete clinico- tympanic membranes to medial meatal atresia pathologic entity in which the granula- (Figure 1). Individual times for the progres- tion process produced the final fibrous sion of the into the atresia phase plug. However, Slattery and Saadat8 found, and the preoperative and postoperative hearing in their review of 24 cases of postinflam- status are shown in the Table. matory medial meatal fibrosis, a condi- Bacterial cultures grown out during acute tion that they subclassified as an idio- infected phases have varied: Escherichia coli, Serratia marcescens, Streptococcus β-hemolyti- pathic postinflammatory medial canal cus group G, but mostly Staphylococcus au- From the Department of fibrosis: 4 patients with no readily iden- reus or the bacterial cultures have remained Otorhinolaryngology, Helsinki tifiable cause for the development of the negative (Table). Fungal cultures have been University Hospital, Helsinki, atresia. Following these reports, we de- negative (Table). In more distinct inflam- Finland. scribe an additional 3 bilateral idiopathic mation periods, no particular bacterial over-

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©2002 American Medical Association. All rights reserved. Downloaded From: https://jamanetwork.com/ on 09/26/2021 growth in culture specimens has occurred in any of the nal skin granulated (Figure 1). The tube was extracted patients. and protruding granulation tissue mechanically re- Histologic specimens from the resected fibrous plug taken moved. Local treatment with topical antibiotic-steroid during surgery and the meatal tissue specimens tested during drops was started. Over the next several visits, the ful- different phases of the ongoing inflammation have shown no minant meatal canal granulative infection resolved. Af- specific pathologic conditions. They have shown dense fi- brotic tissue or chronic lymphocytic inflammation cytologi- ter tube extraction, the tympanic membrane perfora- cally, and proliferative small vessels, some fibroblasts, scarce tion healed in a few weeks, but the medial meatal skin collagen fibers, parakeratosis, and scanty calcification. Histo- did not heal entirely (Figure 2). pathologic findings from all 3 patients showed no tuberculous The medial meatal inflammation and/or infection . In the florid inflammatory stage, histologic speci- then reactivated. The patient underwent several medi- mens taken from the meatal tissue lining the bone have shown cal interventions with varying combinations of treat- no stratified epidermal lining or dermis. Except for ear canal ments including topical applications of antibiotics and symptoms, all 3 patients were healthy and had no immuno- steroids in eardrops, ear packing, thorough and regular logic deficiencies. ear irrigation, a few sessions of surgical abrasion of granu- lation tissue, and oral antibiotic and steroid treatments RESULTS in combination with local treatment or without local treat- ment. The medial ear canal skin in both ears never com- CASE 1 pletely healed. A 5-year-old girl had a persistent aural discharge, meatal Her right ear canal was medially sealed with fibrous infection, and ear canal granulation bilaterally. Other- tissue by age 6 years (Figure 3). The infection and/or in- wise, her general health was good. She had previously had flammation stabilized in the right ear, and the skin epithe- tympanostomy tubes inserted because of secretory otitis me- lial lining over the atresia has not shown symptoms since dia and acute recurrent middle ear without tym- the final fibrosing. The meatal skin lateral to the bony ca- panic membrane perforations. A year thereafter, when the nal has been healthy in both ears. The boundary between middle ear symptoms had clinically resolved, the meatal the healthy and inflammatory skin has been distinct infection and/or inflammation began. throughout her medical history in both ear canals. On her first clinic visit, she had no discharge through At age 9 years, her left ear canal active process contin- the tympanostomy tube still in position on the left side, ues. In noninfectious stages she seemed to gain some ben- and both middle ears were clinically uninfected. Both tym- efit from a potent local steroid treatment (betamethasone). panic membranes were thickened, and the medial ear ca- To prevent total fibrosing, treatment with oral prednisone and clarithromycin was started, but 4 months of this treat- ment has provided no permanent solution. In pediatric consultations, the patient’s general health has been consistently excellent, and no immunologic de- ficiencies have been found. Test results for C-reactive pro- tein have been negative; hemoglobin levels and com- plete blood cell counts, normal; erythrocyte sedimentation ␣ rates, less than 10 mm/h; and 1-antitrypsin values, nor- mal. Immunoglobulin levels, including IgG subclasses, have been within normal limits.

CASE 2

A 55-year-old otherwise healthy man had continuous ex- ternal ear canal infection and/or inflammation for 2 years. Figure 1. Temporal bone computed tomographic scan of patient 1 shows the Clinical examination revealed purulent external ear ca- middle ears to have aerated tympanums and no signs of ongoing acute or chronic middle ear or mastoid cavity disease. Medial ear canals are nal infection with granulation tissue in both ear canals. shadowed, and the tympanic membranes are thickened. Infection and granulation affected only the medial me-

Clinical Follow-up of Progression of Disease Into the Atresia Phase, Hearing Status, and Microbiological Findings From Ear Canals

Hearing, dB (Right/Left) Sex/ Time to Atresia, y Age, y (Right/Left) Preoperative Postoperative Bacterial Culture Fungal Culture F/5 1.5/3* 30/5-30 Not operated on 13 ϫ Negative, 6 ϫ Staphylococcus 5 ϫ Negative, 1 negative from aureus/epidermidis,1ϫ Haemophilus tissue culture influenzae,† 1 ϫ Streptococcus pneumoniae † M/55 3.5*/3.5* 10-15/10-15 Not operated on 3 ϫ S aureus,1ϫ Escherichia coli,2ϫ 4 ϫ Negative Streptococcus ␤-hemolyticus group G M/44 11/12 45/45 25/10 1 ϫ Negative, 4 ϫ S aureus,2ϫ E coli 1 Culture negative, 1 tissue negative

*Continuing follow-up. †Haemophilus influenzae and S pneumoniae have been found in the year 1997.

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©2002 American Medical Association. All rights reserved. Downloaded From: https://jamanetwork.com/ on 09/26/2021 Figure 2. Left ear of patient 1 after tube extraction and healing of the Figure 4. Inflammation status of the left ear of patient 2: no purulent tympanic membrane perforation. Slight granular web formation is visible at infection or granulation formations; the skin is defective; histologic biopsy the boundary of the cartilage and bony ear canal. The bony ear canal skin is specimen shows no epithelia. defective, and the tympanic membrane is thick.

Figure 5. Inflammation status of the left ear of patient 2 is almost stable Figure 3. Mature fibrous plug formation in the right ear canal of patient 1. under continuous local steroid treatment, but the active disease still The situation is stable and no infection or inflammation is present. proceeds slowly. This status has continued with a white, fibrinlike membrane Conductive hearing level is 30 dB. for 11⁄2 years, fluctuating slightly, but without any granulation.

eczematous and defective with a white fibrinous cover atal canal and tympanic membrane. The granulation tis- (Figure 5). sue was mechanically removed during the next several visits, and infection was treated with topical antibiotic CASE 3 and steroid eardrops. He never noticed any middle ear symptoms. A temporal bone computed tomographic scan A 44-year-old man presented with a history of continu- revealed entirely healthy middle ears and mastoid cavi- ous external otitis symptoms over the last 10 years, for ties (data not shown). which he regularly used topical antiseptic and antibiotic- The active infection and prominent inflammation corticosteroid drops. At age 40 years, stricturous atresia resolved, but the epidermal layer of the medial meatal was noted in the middle of the ear canals, and medial to skin was in very loose contact with the scanty inflam- that, eczematous inflammation was found. The ear ca- matory derma, and the epidermal layer desquamated in nals then experienced stenosis with fibromatous medial large pieces under very gentle touch. The boundary be- canal atresia. tween the healthy lateral canal skin and the affected me- After the stenosis progression of this inflammation dial canal skin has been very distinct at every stage of the was noticed, the right ear canal developed a total medial disease: the cartilaginous area of the ear canal skin has atresia within 1 year; on the left, progression to the ste- remained normal even in the purulent discharge peri- nosed quiescent stage took 2 years. After the formation ods. Under thorough, active, local and periodic oral pred- of the fibromatous plug, the inflammation disappeared nisone and antibiotic treatments (ciprofloxacin, clar- completely. ithromycin), the recurrent infections have eased, but the basic inflammatory stage persists and progresses slowly COMMENT but relentlessly (Figure 4). At this stage, the best re- sponse has been to a potent local steroid treatment Postinflammatory meatal atresia is most often caused (betamethasone). During local steroid treatment, no by chronic or by recurrent external otitis.1 granulation has been seen, but the skin is permanently Slattery and Saadat8 conclude that the small sample size

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©2002 American Medical Association. All rights reserved. Downloaded From: https://jamanetwork.com/ on 09/26/2021 in their own and previous reports precludes generaliza- no benefit, and when used in combination with cortico- tion in regard to the bilateral nature of the idiopathic me- steroids, they give no auxiliary benefit. dial canal atresia disease. The specific cause of the con- The medial meatal skin of the ear canal has certain dition is unclear.7,10 It has been proposed that loss of the peculiar characteristics that distinguish it from skin any- squamous epithelium on the lateral surface of the tym- where else on the body: the dermis here is attached di- panic membrane results in exposure of its fibrous layer, rectly to the bone with no subcutis; the skin is very thin and in the presence of ongoing inflammation, healing oc- and has no skin organs; it has a lateral migratory keratin curs through granulation formation.2,11 The stenosing pro- desquamation capacity; and this area is the only locus cess tends to continue up to the junction between the (excluding the natural orifices of the body) where the en- bony and cartilaginous canal.2 The active, relentlessly pro- dodermal and ectodermal epithelia are so close after birth gressive stages are followed by a quiescent, mature fi- that under pathologic conditions they may come into di- brotic phase in which conductive is the main rect contact and interfere with each other. (and often only) symptom. A histologic specimen taken from the affected skin area In most published series, etiologies show variabil- in the nongranular and/or noninfectious stage shows no ity even in a single patient. This poses a challenge to ex- epithelial lining, which is very interesting. The inflamma- act diagnosis and treatment, although the final status is tion and/or disease stabilizes by itself when the skin over fairly distinct and consistent regardless of possibly dif- the bony canal has been replaced by the fibrous plug. fering pathophysiologic etiologies. The literature also in- The chronic progression and the response, although cludes a few cases in which chronic otitis externa is con- not permanent, only to corticosteroid treatment raise the sidered the primary and only etiopathologic factor in the suspicion of an autoimmune disease or a dermolytic skin fibrotizing process.3-9 defect prone to secondary purulent infections and granu- The middle ear status of our patients is normal or lation formation. As Stoney et al11 speculate with regard to healed, with no major chronic purulent middle ear symp- granular myringitis, “The initiator is almost certainly some toms (currently or previously). Clinically and radiologi- form of local trauma or microbial infection, which de- cally, the inflammation and infection have been found to stroys the epithelium, exposing the fibrous layer. The in- be restricted to the medial meatal canal skin over the bony flammation is then confined to the epithelia and underly- part of the canals and tympanic membranes in all 3 pa- ing fibrous layers.” In our patients, the process seems to tients. In children, acquired meatal fibrosis is even more be a continuous inflammatory condition and not primar- rare than among adults. Keohane et al9 described a child ily the result of repeated fulminant infectious episodes, al- who underwent surgery (tympanomastoidectomy) for sus- though an infection seems always to have been a trigger- pected chronic middle ear disease, but the middle ear and ing factor. Also, the inflammation predisposes the tissue mastoid were found to be normal at surgery in both ears. to recurrent infections, recurrent granulation formation, The child in the present study developed medial meatal ear and scarring. Based on these cases, we suggest that idio- canal atresia without ongoing middle ear disease, as veri- pathic inflammatory fibrotizing chronic otitis of the me- fied clinically and by computed tomography. dial ear canal is its own clinical and/or pathologic entity. Our 3 patients share several features. The disease is a bilateral, local fibrotizing inflammation of the medial Accepted for publication May 20, 2002. ear canals with poor response to conservative and sur- Corresponding author and reprints: Erkki Hopsu, MD, gical interventions. The symptoms are “quiet” through- PhD, Department of Otorhinolaryngology, Helsinki Uni- out the history: even in acute phases with purulent in- versity Hospital, Haartmaninkatu 4E, Helsinki, 00029 HUS, fectious discharge and granulation, there is usually no Finland (e-mail: [email protected]). . The only complaint besides the discharge in these phases is that hearing is abnormal. 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