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.

• 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]

• Hypertension

  In addition, it is often found to be co-occurring with other health conditions, such as tooth sepsis, long-term acid reflux and use of acid reflux medications, hypertension, gout, high triglycerides, IBS, and rheumatoid and osteoarthritis conditions [ [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]

  The clinical manifestations include bilateral otalgia and conductive hearing loss. The diagnosis is based on the patient history, physical examination, and appropriate studies. [symptoma.com]

• Normal Hearing

  Symptoms/signs Severe otalgia,conductive hearing loss, younger ages, occasionally bilateral,associated with lung & sinus disease, can present with a plugged feeling. Chronic otorrhoea with normal hearing, itchiness, or pain. [shekharkrishnadebnath.wordpress.com]

• 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]

• 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].

The clinical symptoms, pathologic processes, and treatment are outlined and compared. Case reports are presented to illustrate the features of these two diseases. [ncbi.nlm.nih.gov]

Treatment: The treatment of keratosis obturans includes the mechanical removal of the epidermal plug and periodic cleansing of the canal. Granulation tissue may be managed by removal, cauterization, or the use of topical steroid drops. [medigoo.com]

Treatment Removal of plug Local treatment of granulations Biopsy to exclude malignancy/necrotizing otitis externa. May need continued cleanings. Surgically remove cholesteatoma & abnormal bone, Graft with fascia Biopsy. [shekharkrishnadebnath.wordpress.com]

It can be confused for EAC cholesteatoma but they are completely different entities requiring different treatment. Keratosis obturans is seen in younger patients usually less than 40 years old and is bilateral in 50% of cases. [radiopaedia.org]

[…] usually show air foci e xternal auditory canal cholesteatoma : EAC soft tissue density with bony erosion external auditory canal carcinoma: irregular mass with or without bony erosion otitis externa : surrounding inflammatory fat stranding Treatment and prognosis [radiopaedia.org]

Outlook (Prognosis) Regular follow-ups and cleaning by the provider are important to avoid infections. In some people, lifetime cleaning may be needed. [baptistjax.com]

Etiology: Keratosis obturans is postulated to occur due to abnormal epithelial migration of ear canal skin. The movement of the surface epithelium appears to be reversed in these patients. [sites.google.com]

{THIS IS PRESENT IN external auditory canal cholesteatoma} commonly seen b/w 5 & 20 years etiology: due to defect in epithelial migration increased production of keratin debris both Clinical features : generally BILATERAL & is PEARLY WHITE MASS. [lessons4medicos.blogspot.com]

Etiology The exact etiology is not known. It appears to be result of abnormal shedding of the epithelium and failure of migration of the cell from the surface of the tympanic membrane, which leads to accumulation of a mass. [dict.eudic.net]

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. [symptoma.com]

Seite 53 - A longitudinal study of respiratory viruses and bacteria in the etiology of acute otitis media with effusion - Henderson FW, Collier AM, Sanyal MA et al. ‎ [books.google.de]

Definitions, differential diagnosis, and epidemiology. Am J Surg Pathol 1996; 20(1):103-111. ‎ Seite 91 - Everett LA, Glaser B, Beck JC, Idol JR. Buchs A, Heyman M, Adawi F, Hazani E, Nassir E. [books.google.de]

Definitions, differential diagnosis, and epidemiology. Am J Surg Pathol 1996; 20(1):103-111. ‏ الصفحة 91 - Everett LA, Glaser B, Beck JC, Idol JR. Buchs A, Heyman M, Adawi F, Hazani E, Nassir E. [books.google.com]

Epidemiology Otitis externa is a common presenting complaint in UK general practice, with studies suggesting that it accounts for around just over 1% of all GP consultations. [patient.info]

J F Guest, MJ Greener, AC Robinson and AF Smith: Impacted cerumen: composition, production, epidemiology and management Q J Med 2004; 97 : 477-488. 3. Carr MM, Smith RL. Ceruminolytic efficacy in adults versus children. [ispub.com]

Pathophysiology Keratosis obturans is thought to occur due to abnormal epithelial migration of ear canal skin. The movement of the surface epithelium appears to be reversed in these patients. [wikidoc.org]

The disease is sometimes associated with bronchiectasis and chronic sinusitis Pathophysiology There are two different forms of the pathophysiology of keratosis obliturans or obliterans. [dayfres.blogspot.com]

Tympanic membrane and Cerumen Pathophysiology Integrity of tympanic membrane was found to have a role to play in leading to impacted ear wax but none in the production of ear wax. [ispub.com]

Another pathophysiologic hypothesis suggests that accumulation of keratin debris induces changes of cellular proliferation in the EAC ( 10 ). [ajnr.org]

Increased awareness of these uncommon conditions is warranted to prompt appropriate investigation and prevent iatrogenic complications such as facial nerve injury. [ncbi.nlm.nih.gov]

"External auditory canal cholesteatoma and keratosis obturans: the role of imaging in preventing facial nerve injury". Ear Nose Throat J. 90 (12): E1–7. PMID 22180115. [wikidoc.org]

It also includes three new key action statements on primary prevention, contraindicated intervention, and referral and coordination of care. [medpagetoday.com]

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]

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