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Braz J Otorhinolaryngol. 2016;xxx(xx):xxx---xxx

www.bjorl.org

Brazilian Journal of

OTORHINOLARYNGOLOGY

CASE REPORT

Keratosis obturans complicated with facial nerve palsy:a diagnostic dilemma�

Queratose obliterante complicada com paralisia do nervo facial:um dilema diagnóstico

Jeyasakthy Saniasiayaa,∗, Nik Adilah Nik Othmanb, Nur Hidayati Mohamad Pakarul Razyc

a Department of Otorhinolaryngology-Head & Neck Surgery, School of Medical Sciences, Universiti Sains Malaysia Health Campus,Kelantan, Malaysiab Audiology Program, School of Health Sciences, Health Campus Universiti Sains Malaysia, Kelantan, Malaysiac Department of Pathology, School of Health Sciences, Health Campus Universiti Sains Malaysia, Kelantan, Malaysia

Received 18 December 2015; accepted 20 April 2016

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Introduction

Keratosis obturans is estimated to occur among 4 to 5patients among 1000 new otological cases.1 Typical clini-cal manifestations includes severe otalgia and hearing lossattributed by the accumulation of desquamated epidermalplug in ear canal. The epidermal plug formation may occurdue to faulty migration or excessive production of epithelialcells, as proposed by Paparella and Shumrick.2 Ballooning orwidening of bony external auditory canal can also specifykeratosis obturans.3 For many years, keratosis obturans andexternal canal cholesteatoma were used interchangeablyup until 1980 when Piepergedes and Behnke distinguishedthem as a separate entity.4 To our knowledge this is the firstreported case of an unusual presentation of keratosis obtu-rans causing unilateral facial palsy with no evidence of bonyerosion.

� Please cite this article as: Saniasiaya J, Nik Othman NA,Mohamad Pakarul Razy NH. Keratosis obturans complicated with

facial nerve palsy: a diagnostic dilemma. Braz J Otorhinolaryngol.2016. http://dx.doi.org/10.1016/j.bjorl.2016.04.012

∗ Corresponding author.E-mail: shakthy 18@yahoo.com (J. Saniasiaya).

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http://dx.doi.org/10.1016/j.bjorl.2016.04.0121808-8694/© 2016 Associacao Brasileira de Otorrinolaringologia e Cirurgiaaccess article under the CC BY license (http://creativecommons.org/lic

ase report

previously healthy 22 year-old Malay gentleman presentedo our clinic with a two-month history of worsening left otal-ia with bloody otorrhea. There was also left sided reducedearing followed by asymmetry on the left side of face forhe past 1 week. Patient claimed that there was no recentr any recurrent upper respiratory tract infection prior tohis.

Further history from patient revealed that he had similaromplaints a year ago involving the right ear with no facialsymmetry. Patient was diagnosed with right aural polyp andolypectomy was done under local anaesthesia in anotherovernment hospital. However, patient defaulted his follow-p as the problem resolved completely.

Upon examination, patient was comfortable, not sep-ic looking and afebrile. Facial nerve examination revealedouse---Brackmann Grade III lower motor neuron palsy ashere was loss of left nasolabial fold and drooping ofeft angle of lip. Otoscopic examination revealed a poly-oidal mass occluding the entire left ear canal covered

ith bloody, foul smelling otorrhea (Fig. 1). Right ear canalxamination was normal with an intact tympanic mem-rane. Oropharynx examination was unremarkable and neckodes were not palpable. Nasoendoscopy revealed mild

Cervico-Facial. Published by Elsevier Editora Ltda. This is an openenses/by/4.0/).

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Figure 1 Polypoidal mass occluding left ear canal.

denoid enlargement with no signs of active infection.ll other cranial nerves were intact and no other neu-ological deficit was evident. Systemic examination wasormal. Tuning fork test revealed left conductive hearingoss. Full blood count and electrolytes were within normalange. Preliminary diagnosis of left aural polyp with facialerve palsy grade III was made with a differential diagno-is of external canal cholesteatoma. Patient was startedith intravenous ciprofloxacin with tapering dose of pred-isolone. Subsequent day, facial nerve palsy was noted tomprove demonstrating facial nerve palsy House---Brackmannrade II.

A high-resolution computer tomography (HRCT) scan ofemporal was obtained, which revealed non-enhancing soft-issue mass occupying left external auditory canal, leftrussak’s space and middle ear with erosion of left scutum.he left inner ear was intact with normal right ear struc-

ures. There was no evidence of facial canal dehiscence orrosion; however signs of inflammation over the tympanicegment of facial canal were noted (Fig. 2).

igure 2 Inflammation over tympanic segment of facialerve.

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Figure 3 Cyst wall containing lamellated keratin flakes.

Patient underwent microscope-guided examinationnder anaesthesia which revealed polypoidal mass occupy-ng entire lateral third of ear canal with whitish keratin-likeebris noted medial to the mass. Polypectomy and aural toi-eting was commenced. As tympanic membrane appearedulging, myringotomy and grommet insertion was per-ormed. Histopathologic examination revealed cyst wallovered with stratified squamous epithelium containingamellated keratin flakes suggestive of keratosis obturansFig. 3) Post-operatively patient was well. He was dischargedome following day as the facial asymmetry improved withnly slight weakness noted upon close inspection witho other accompanying complications. Oral ciprofloxacin,apering dose of prednisolone and ofloxacin ear drops wasrescribed for 1 week duration upon discharge. He wasiven a one week appointment which he defaulted.

iscussion

eratosis obturans can be of two types: Inflammatory typer silent type.1 It has been proposed that the inflammatoryype occurs secondary to an acute infection, for instanceiral infection whereby the inflammatory process may tem-orarily alter the epithelial migration. The inflammatoryype may be cured after removal of the keratin. As for theilent type, it is due to an abnormal separation of the ker-tin causing the disease to progress continuously even afterhe first removal thus, requiring continuous aural toileting.ur patient may be categorised into the inflammatory typef keratosis obturans.

Keratosis obturans usually affects younger age group,ccurs bilaterally and manifests as severe otalgia, con-uctive hearing loss and widened ear canal.5 Otorrhea isonsidered a rare presentation.5 Seventy seven percent ofhildren and twenty present of adults have an associatedinusitis and bronchiectasis which is due to reflex sympa-hetic autonomic activation leading to excessive cerumenecretion thus epidermal plug formation.6

Computer tomography typically demonstrates soft tissue

lug in bilateral external ear canal with evidence of balloon-ng of the osseous part. In our patient, his main complaintas severe otalgia and otorrhea which was followed by uni-

ateral facial weakness and hearing loss. Although rare, few

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ARTICLEKeratosis obturans complicated with facial nerve palsy: a di

cases of keratosis obturans, presenting with facial weak-ness have been reported.7,8 Facial nerve palsy followingkeratosis obturans are caused by bony erosion,7,8 which maybe due to the pressure effect exerted by keratin mass inthe external canal.9 In our patient, albeit no evidence ofbony erosion in the HRCT temporal, pressure exerted byacute inflammation may have caused the facial nerve palsywhich was supported by complete resolution after com-mencement of antibiotics and surgical removal of the earmass.

Clinical differential diagnosis for mass in aural canalwith facial nerve palsy includes external auditory canalcholesteatoma, neoplasms of external canal and malignantotitis externa. It is important to distinguish the diagnosis, asmanagement of each of the differential diagnosis is notablydifferent. Hence, thorough and detailed history, physicalexamination, radiological examination and most importantlyhistopathological examination is crucial prior to a diagno-sis. Histopathological examination of the biopsied or excisedmass is the main modality of diagnosis, more so when thereis an atypical or rare presentation.

As for management, keratosis obturans usually requiresfrequent aural toileting to remove the keratin plug andtopical medication. This may be done under general anaes-thesia especially among non-cooperative patients. Split skingraft and canalplasty method have also been reported forrefractory keratosis obturans.10 As for cases of keratosisobturans complicated with facial nerve palsy, steroids maybe prescribed along with antibiotics if there is precipitatinginfection.

Conclusion

Albeit commonly regarded benign, keratosis obturans

may result in devastating complications including facialnerve palsy as in our patient. Hence, high clinical sus-picion and prompt management among clinicians are ofdire importance as absence of typical clinical features

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sually lands both the attending physician and patient inilemma.

onflicts of interest

he authors declare no conflicts of interest.

cknowledgement

e would like to sincerely thank Dr. Ani Darwina, Registrarf Radiology Department for reporting this HRCT temporal.

eferences

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2. Paparella M, Shumrick D. Otolaryngology. Philadelphia: WBSaunders; 1973. p. 2.

3. Persaud RAP, Hajioff D, Thevasagayam MS, Wareing MJ,Wright A. Keratosis obturans and external auditory canalcholesteatoma: how and why we should distinguish betweenthese conditions. Clin Otol. 2004;24:577---8.

4. Piepergedes JC, Behnke EE. Keratosis obturans and externalauditory canal cholesteatoma. Laryngoscope. 1980;90:383---90.

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6. Morrison AW. Keratosis obturans. J Laryngol Otol.1956;70:317---21.

7. Glynn F, Keogh IJ, Burns H. Neglected keratosis obturans causingfacial nerve palsy. J Laryngol Otol. 2006;120:784---5.

8. Al-Juboori AN. Keratosis obturans: a rare cause of facial nervepalsy. Austin J Otolaryngol. 2015;2:1039.

9. Hawke M, Shanker L. Automastoidectomy caused by keratosis

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0. Paparella MM, Goycoolea MV. Canalplasty for chronic intractableexternal otitis and keratosis obturans. Otolaryngol Head NeckSurg. 1981;89:440---3.