Introduction. Congenital cholesteatoma of the middle ear is un uncommon and
   yet not well-defined disease. Only few cases of cholesteatoma in the fossa
   ovalis with unusual clinical presentation have been reported in medical
   literature. Case report. We reported a 16-year-old girl with congenital
   cholesteatoma in the fossa ovalis with minimal clinical presentation. A small
   mass was found occluding the fossa ovalis and mimicking otosclerotic process
   within tympanic cavity. The operation started as stapedotomy, and when the
   process was confirmed it converted to mastoidectomy via the retroauricular
   approach. Conclusion. The diagnosis of congenital cholesteatoma in children
   should always be considered, even if the clinical symptoms imitate other ear
   disorders, in our case otosclerosis. [Projekat Ministarstva nauke Republike
   Srbije, br. 179055: Cochlear implantation impact on education of deaf and
   hearing-impaired

Arsović Nenad

Babić Borivoj

Bukurov Bojana

Dimitrijević Milovan

Folić Miljan

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Vojnosanit Pregl 2014; 71(5): 503–505. VOJNOSANITETSKI PREGLED Strana 503Correspondence to: Bojana Bukurov, Clinic for Otorhinolaringology and Maxilofacial Surgery, Clinical Center of Serbia, Pasterova 2, 11000 Belgrade, Serbia. Phone: +381 11 366 22 05, E-mail: panovicbojana@yahoo.comCASE REPORT UDC: 616.284-02/-08DOI: 10.2298/VSP1405503BCongenital cholesteatoma of the middle ear – uncommon clinicalpresentationNeobiþna kliniþka prezentacija kongenitalnog holesteatomasrednjeg uvaBojana Bukurov, Borivoj Babiü, Milovan Dimitrijeviü, Miljan Foliü,Nenad ArsoviüClinic for Otorhinolaryngology and Maxillofacial Surgery, Clinical Center of Serbia,Faculty of Medicine, University of Belgrade, Belgrade, SerbiaAbstractIntroduction. Congenital cholesteatoma of the middle earis un uncommon and yet not well-defined disease. Onlyfew cases of cholesteatoma in the fossa ovalis with unusualclinical presentation have been reported in medical litera-ture. Case report. We reported a 16-year-old girl withcongenital cholesteatoma in the fossa ovalis with minimalclinical presentation. A small mass was found occludingthe fossa ovalis and mimicking otosclerotic process withintympanic cavity. The operation started as stapedotomy,and when the process was confirmed it converted tomastoidectomy  via the retroauricular approach. Conclu-sion. The diagnosis of congenital cholesteatoma in chil-dren should always be considered, even if the clinicalsymptoms imitate other ear disorders, in our case otoscle-rosis.Key words:cholesteatoma; congenital abnormalities; ear, middle;diagnosis; otorhinolaryngologic surgical procedures.ApstraktUvod.  Kongenitalni holesteatom lokalizovan u kavumutimpani retko je oboljenje, još uvek nerazjašnjene etiologije.Do sada je objavljeno samo nekoliko radova u medicinskojliteraturi o kongenitalnom holestatomu u ovalnom prozorusa minimalnom kliniÿkom prezentacijom. Prikaz bolesni-ka.  Prikazana je 16-godišnja devojÿica sa kongenitalnimholesteatomom lokalizovanim u ovalnom prozoru sa mini-malnim kliniÿkim simptomima. PronaĀen je mali holestea-tom koji je u popunosti ispunjavao fosu ovalis i imitirao otos-klerotiÿni proces u kavumu timpani. Operacija je zapoÿetakao stapedotomija, a kada je proces konstatovan, nastavljenaje kao mastoidektomija kroz retroaurikularni pristup. Zaklju-ÿak. Trebalo bi uvek razmotriti dijagnozu kongenitalnog ho-lesteatoma kod dece, ÿak i kada simptomi imitiraju neko dru-go oboljenje srednjeg uva, u našem sluÿaju otosklerozu.Kljuÿne reÿi:holesteatom; anomalije; uvo, srednje; dijagnoza;hirurgija, otorinolaringološka, procedure.IntroductionCongenital cholesteatoma is relatively uncommon con-dition. It is defined as the whitish mass behind an intact ear-drum, in a patient with no history of ear trauma or previoussurgery and with no retraction pocket, perforation or granu-lation tissue that can be detected on the surface of an ear-drum 1. The term “congenital” has been used rather conven-tionally because the pathogenesis of congenital choleste-atoma remains unclear. Various hypotheses suggest anythingfrom development during the fetal period to a condition ac-quired during infancy 2. Almost any recently published paperemphasizes the importance of early diagnosis and interven-tion, as late diagnosis may be associated with extensivenessof the disease.In spite of reports suggesting the age at the time of sur-gery being an important factor that affects treatment out-come, and widely accepted fact that congenital choleste-atoma increases over the time, there is no firm evidence inthe literature confirming such relationship.Usual age at the time of diagnosis is 4–5 years, and70% of patients are asymptomatic at that point 3.We reported a patient with congenital cholesteatoma inthe fossa ovalis with minimal clinical symptoms, presentingas an otosclerotic process within tympanic cavity.Case reportA 16-year-old female patient was referred to the Clinicfor Otolaryngology due to the right-sided hearing loss thatStrana 504 VOJNOSANITETSKI PREGLED Volumen 71, Broj 5Bukurov B, et al. Vojnosanit Pregl 2014; 71(5): 503–505.was diagnosed two years before. The patient had no previoushistory of otitis media, middle ear trauma, or any previousear surgery.Otoscopic examination showed intact tympanic mem-branes on both sides, although the right membrane had tym-panosclerotic plaque in its anterior-inferior quadrant. Therewere no symptoms related to the vestibular system, nose orthroat. Audiometric evaluation revealed mild conductivehearing loss of about 40 dB on the right side with a dip ofbone conduction at 4 kHz (Figure 1). Stapedius reflex wasabsent on the same side. The results of general physical ex-amination and routine laboratory and blood chemistry testswere within normal range.Computed tomography (CT) of the temporal bone wasperformed and the findings in the middle ear appeared nor-mal, with normally aerated and pneumatized mastoid cells.Based on these findings, explorative surgery was per-formed on the right ear with the presumptive diagnosis ofotosclerosis. Open type cholesteatoma was found obliteratingoval window (Figure 2). The long process of incus and headof stapes was missing. Due to the findings, operation wascontinued as mastoidectomy and posterior timpanotomy inorder to completely remove the process. After removal ofcholesteatoma, mobile stapes footplate was covered withtemporalis fascia and TTP™ (Tuebingen Titanium Prosthe-sis, AERIAL) was implanted between the malleus and stapesfootplate.Four years after the surgery, there were no signs of re-lapse and liminar tonal audiometry of the right ear showedclosure of air bone gap within the 10 dB.DiscussionGenerally, the initial otologic symptom in a patient withopen type of cholesteatoma is hearing loss, and in some cases,a white mass can be seen through tympanic membrane 3. Manyof these patients are unaware of their hearing loss, especiallybecause it is manifested in childhood. Most of congenitalcholesteatoma in the middle ear are detected early in life, butour patient was 16-years-old when first referred to ourClinic, and had no previous history of any ear, nose or throatcondition.The primary localization of open type cholesteatoma, assuggested by many authors, is around the oval window, thesame as in the presented patient. From there, it may spread toepitympanic recess, mastoid antrum, or to mesotympa-num 4, 5.Congenital cholesteatoma commonly develops in theisthmus of the tympanic cavity and the most frequent os-sicular malformations are involvements of a long process ofthe incus and suprastructures of stapes in more than 60% ofpatients 3, 6, 7 corresponding to the junction of the first andsecond branchial arches. As congenital cholesteatoma tendsto spread into the posterior-superior part of tympanic cavity,the fact that the suprastructure of stapes was affected in thepresented patient suggested congenital origin of choleste-atoma.Mastoid pneumatization in our patient was normal andthese findings are in accordance with Iino et al. 8 and otherauthors 9, 10 who reported better pneumatization in patientswith congenital, compared to acquired cholesteatoma.The initial symptom, intact tympanic membrane, audi-ological findings, and normal CT scans suggested an otoscle-rotic process in the middle ear, the diagnosis being supportedby the age of the patient at the time of detection of hearingloss. However, these findings were proved to be misleadingin the presented patient. There are few other cases in the lit-Frequency (Hz) Frequency (Hz)Fig. 1 – Preoperative liminar tonal audiogram.Fig. 2 – Intraoperative findings showing a cholesteatoma inthe fossa ovalis.Volumen 71, Broj 5 VOJNOSANITETSKI PREGLED Strana 505Bukurov B, et al. Vojnosanit Pregl 2014; 71(5): 503–505.erature describing congenital cholesteatomas that have beensilent for many years or have been accompanied with mini-mal clinical presentation 4, 11.ConclusionThe diagnosis of congenital cholesteatoma should al-ways be considered, even if the clinical symptoms mimicother ear disorders, in our case otosclerosis. In some cases,such as this one, even CT findings are not able to detectcholesteatoma. Therefore, clinicians should always keep highlevel of cautiousness when dealing with young patients whopresent with hearing loss as the only symptom in order todeliver early treatment and improve final outcome.AcknowledgementsThe study was supported within project from Ministryof Education, Science and Technological Development of theRepublic of Serbia “Cochlear implantation impact on educa-tion of deaf and hearing-impaired” (No. 179055).REFERENCES1. Levine JL, Wright CG, Pawlowski KS, Meyerhoff WL. Postnatalpersistence of epidermoid rests in the human middle ear. La-ryngoscope 1998; 108(1 Pt 1): 70î3.2. Persaud R, Hajioff D, Trinidale A, Khemani S, Battacharya MN, Pa-padimitriou N, et al. Evidence-based review of etiopathogenictheories of congenital and acquired cholesteatoma. J LaryngolOtol 2007; 121(11): 1013î9.3. Lim HW, Yoon TH, Kang WS. Congenital cholesteatoma: clini-cal features and growth patterns. Am J Otolaryngol 2012;33(5): 538î42.4. Kojima H, Tanaka Y, Shiwa M, Sukurai Y, Moriyama H. Con-genital cholesteatoma clinical features and surgical resulats.Am J Otolaryngol 2006; 27(5): 299î305.5. McGill TJ, Merchant S, Healy GB, Friedman EM. Congenital cho-lesteatoma of the middle ear in children: a clinical and histo-pathological report. Laryngoscope 1991; 101(6 Pt 1): 606î13.6. Wang R, Zubick HH, Vernick DM, Strome M. Bilateral congeni-tal middle ear cholesteatomas. Laryngoscope 1984; 94(11 Pt 1):1461î3.7. Levenson MJ, Michaels L, Parisier SC, Juarbe C. Congenital cho-lesteatomas in children: an embryologic correlation. Laryngo-scope 1988; 98(9): 949î55.8. Iino Y, Imamura Y, Hiraishi M, Yabe T, Suzuki J. Mastoid pneu-matization in children with congenital cholesteatoma: an as-pect of the formation of open-type and closed-type choleste-atoma. Laryngoscope 1998; 108(7): 1071î6.9. Warren FM, Bennett ML, Wiggins RH 3rd, Saltzman KL, BlevinsKS, Shelton C, et al. Congenital cholesteatoma of the mastoidtemporal bone. Laryngoscope 2007; 117(8): 1389î94.10. Mevio E, Gorini E, Sbrocca M, Artesi L, Lenzi A, Lecce S,  et al.Congenital cholesteatoma of mastoid origin. Otolaryngol HeadNeck Surg 2002; 127(4): 346î8.11. Lee JH, Hong SJ, Park CH, Jung SH. Congenital cholesteatomaof mastoid origin. J Laryngol Otol 2007; 121(11): e20.Received on April 29, 2012.Revised on February 13, 2013.Accepted on February 27, 2013.

Vojnosanitetski pregled

Congenital cholesteatoma of the middle ear - uncommon clinical presentation

Introduction. Congenital cholesteatoma of the middle ear is un uncommon and
   yet not well-defined disease. Only few cases of cholesteatoma in the fossa
   ovalis with unusual clinical presentation have been reported in medical
   literature. Case report. We reported a 16-year-old girl with congenital
   cholesteatoma in the fossa ovalis with minimal clinical presentation. A small
   mass was found occluding the fossa ovalis and mimicking otosclerotic process
   within tympanic cavity. The operation started as stapedotomy, and when the
   process was confirmed it converted to mastoidectomy via the retroauricular
   approach. Conclusion. The diagnosis of congenital cholesteatoma in children
   should always be considered, even if the clinical symptoms imitate other ear
   disorders, in our case otosclerosis.</jats:p

Congenital cholesteatoma of the middle ear - uncommon clinical presentation

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