Keratosis obturans is a disease of the external auditory meatus in which keratinaceous debris accumulate and form a plug in the ear canal, thereby causing occlusion and widening of the bony component [1] [2]. This rare disorder was previously considered as a variation of cholesteatoma of the external auditory canal (CEAC) but is now regarded as a distinct condition [1]. While the etiology is not fully understood, keratosis obturans is classified as either inflammatory, which is associated with a recent acute infection or silent, which does not have a predisposing event [3]. The estimated incidence is 4 to 5 cases per 1000 patients with ear complaints [3].

This disorder occurs predominantly in younger individuals [1] [4] who manifest with acute bilateral ear pain and conductive hearing loss [1] [5]. Furthermore, discharge is not a frequent symptom in these patients [4] [6]. Moreover, the cases are overall healthy [5]. Additionally, keratosis obturans is associated with bronchiectasis and sinusitis [1] [7].

Complications

There are a few incidences in the literature describing facial nerve palsy in patients with keratosis obturans [5] [8] [9]. This neurologic sequela may develop secondary to inflammation [5] or bone erosion [8] [9], of which the latter is not a feature of keratosis obturans [1].

Physical exam

Otoscopy typically reveals an occluding mass in the ear canal [10]. Additionally, tuning fork [5] and pure tone audiometry (PTA) [10] are performed to test and confirm conductive hearing loss.

The victim should be evaluated for neurological deficits such as facial nerve palsy. Additionally, a full head, neck, and oropharynx exam should be completed.

• Pathologist

  Edited by four leading surgical pathologists - Noel Weidner, MD, Richard J. Cote, MD, Saul Suster, MD and Lawrence M. [books.google.com]

• Respiratory Distress

  When acidosis conditions set in—as a result of chronic dehydration, environmental toxicities, untreated sepsis of the jaw or teeth, upper respiratory distress, intestinal infections, or from infected implanted prostheses—the EAC keratin layer often begins [omicsonline.org]

• Metallic Taste

  A case of keratosis obturans is described in which the principal symptom was a metallic taste and the main finding was extensive erosion of the hypotympanum with exposure of the facial nerve and the annulus of the tympanic membrane. [ncbi.nlm.nih.gov]

  Patients with Keratosis obliterans comes usually with pain and hearing loss and can also be accompanied by symptoms such as metallic taste. Patients can come without interruption ears but only with impaired only metallic taste. [dayfres.blogspot.com]

• Yellow Nails

  Ear Involvement in the Yellow Nail Syndrome. Chest, Vol. 98, Issue. 6, p. 1534. CrossRef Google Scholar Gadre, Arun K. and Gadre, K. C. 1989. Cholesteatoma of the external auditory canal in hemifacial hypertrophy (hyperplasia). [cambridge.org]

• Otalgia

  Classically, it is reported to present with severe otalgia, conductive deafness and global widening of the canal. [ncbi.nlm.nih.gov]

  Classically, it is reported to present with severe otalgia, conductive deafness and global widening of the canal. The frequency of keratosis obturans has been estimated as 4-5 in 1000 new otological cases. [austinpublishinggroup.com]

  In our patient, his main complaint was severe otalgia and otorrhea which was followed by unilateral facial weakness and hearing loss. [bjorl.elsevier.es]

  Risk Factors Sinusitis Bronchiectasis Diagnosis History and Symptoms Partial hearing loss Otalgia Otorrhea Associated sinusitis or bronchitis may be present. [wikidoc.org]

• Normal Hearing

  Chronic otorrhoea with normal hearing, itchiness, or pain. Older populations, usually unilateral. Pathology Keratin plug occluding canal, TM thicken& standing out in relief in a widened ear canal. Ear canal grossly widen or ballooned. [shekharkrishnadebnath.wordpress.com]

• Neck Mass

  Enhance your understanding with multiple-choice questions accompanying each case, emphasizing cranial nerves, skull base lesions, sinonasal, orbital, salivary gland, aerodigestive system mucosal lesions and deep space neck masses. [books.google.de]

• Tampon Use

  The most important thing is to make- the ear canal is shaped like a funnel so the ear canal dry spontaneously can be more Assured. keratosis appointed Zalf ear tampon use betadine. [dayfres.blogspot.com]

• Hot Flushes

  Keratosis obturans (medical condition where extra skin cells are built up in the ear canal) has been reported by people with hot flush, pain, nutritional support, hiatal hernia, thyroid diseases. [ehealthme.com]

• Neglect

  This case demonstrates dramatically the extensive erosion of bone that can occur in a patient with neglected keratosis obturans. [ncbi.nlm.nih.gov]

  Regular follow up is necessary to prevent recurrences or EACC development in long standing or neglected cases. References 1. Edward D. McCoul, Mathew B Hanson. [indianjotol.org]

  Burns Neglected keratosis obturans causing facial nerve palsy [8] A.N. Al-Juboori Keratosis obturans: a rare cause of facial nerve palsy Austin J Otolaryngol, 2 (2015), pp. 1039 [9] M. Hawke,L. [bjorl.elsevier.es]

• Altered Mental Status

  General assessment to avoid missed on the assessment of fever, altered mental status and other assessments that may provide clues towards complications. [dayfres.blogspot.com]

• Unilateral Facial Weakness

  In our patient, his main complaint was severe otalgia and otorrhea which was followed by unilateral facial weakness and hearing loss. [bjorl.elsevier.es]

The clinical presentation should raise suspicion for keratosis obturans and CEAC, in which a workup consisting of the patient history, physical exam, and appropriate tests will help determine the diagnosis.

Imaging

Key imaging modalities include high-resolution computed tomography (HRCT) scan of the temporal bone, which will display the soft-tissue accumulation residing in the external ear canal(s) [5] [11] and dilation of the walls of the canal [2] [11]. These findings are typically bilateral in people with keratosis obturans but unilateral in CEAC [12]. Moreover, imaging of the external auditory canal with axial CT reveals no necrosis or bone erosion [1]. Very importantly, keratosis obturans does not involve osseous erosion of the canal as seen with CEAC [2] [12].

Pathology

Keratosis obturans is associated with an enlarged ear canal, epithelial hyperplasia, and subepithelial inflammation [13]. Moreover, histopathological analysis of the debris reveals lamellar keratinous elements [1].

Other

In addition to performing a complete physical exam including a focus on the head and neck region, testing with the tuning fork and PTA are important to evaluate hearing loss [5] [10].

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2. Persaud RA, Hajioff D, Thevasagayam MS, Wareing MJ, Wright A. Keratosis obturans and external auditory canal cholesteatoma: how and why we should distinguish between these conditions Clin Otolaryngol Allied Sci. 2004;29(6):577-581.
3. Tristram HJ. Keratosis obturans and primary auditory canal cholesteatoma. Scott-Brown's Otorhinolaryngology: Head and Neck Surgery. 7th ed. London, England: Hodder Arnold; 2008: 3342-3344.
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5. Saniasiaya J, Nik Othman NA, Mohamad Pakarul Razy NH. Keratosis obturans complicated with facial nerve palsy: a diagnostic dilemma. Braz J Otorhinolaryngol. 2016; pii: S1808-8694(16)30092-1.
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9. Al-Juboori AN. Keratosis obturans: a rare cause of facial nerve palsy. Austin J Otolaryngol. 2015; 2:1039.
10. Corbridge RJ, Michaels L, Wright T. Epithelial migration in keratosis obturans. Am J Otolaryngol. 1996;17(6):411-414.
11. Brea B, Roldán Fidalgo A. Imaging Diagnosis of Benign Lesions of the External Auditory Canal. Acta Otorrinolaringol Esp. 2013;64(1):6-11.
12. Dubach P, Mantokoudis G, Caversaccio M. Ear canal cholesteatoma: meta-analysis of clinical characteristics with update on classification, staging and treatment. Curr Opin Otolaryngol Head Neck Surg. 2010;18(5):369-376.
13. Naiberg J, Berger G, Hawke M. The pathologic features of keratosis obturans and cholesteatoma of the external auditory canal. Arch Otolaryngol. 1984;110(10):690–693.