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 ear 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 ears 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 Otorhinolaryngology 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 ear canal is rela- disease presents as a defined entity. tively uncommon and may be postinflammatory, trau- matic, postoperative, or METHODS 1,2 Aneoplastic. Reflecting 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 infection and granulation. The third pa- ternus,3 chronic stenosing otitis externa,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 middle ear 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 inflammation 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- (REPRINTED) ARCH OTOLARYNGOL HEAD NECK SURG/ VOL 128, NOV 2002 WWW.ARCHOTO.COM 1313 ©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 granulomas. 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 infections 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. (REPRINTED) ARCH OTOLARYNGOL HEAD NECK SURG/ VOL 128, NOV 2002 WWW.ARCHOTO.COM 1314 ©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