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.

• Swelling

  She presented with severe ear pain, ear canal swelling, ear discharge since last 7 days. She also gives history frequent cold - cough, allergy, ear itching & post nasal discharge since last 5 years. [om.vlipa.lv]

  An exostosis of the external auditory canal (EAC) is a benign growth of periosteal bone, which forms a smooth, sessile, hemispherical swelling in the deep part of the meatus, adjacent to the tympanic membrane. [taylorfrancis.com]

  Overview of Sinusitis Sinusitis is the inflammation or swelling of the sinuses that surround your nose, usually caused by a viral or bacterial infection. It is a common condition that often clears up without medical treatment. [ascentent.com]

  Drops will make it swell and can aggravate it before removal. Otitis media is painful but, if the drum bursts, the pain ceases and a discharge follows. [patient.info]

  Common symptoms include itch, ear discharge, temporary dulled hearing, oedema (swelling) and pain. Your ear may feel blocked or full. One or both ears can be affected. [earcare.co.nz]

• Sepsis

  Sepsis of the jaw appears to immunologically interact with sepsis of keratosis in the ears. Other causes that can inspire keratosis formation are: • A serious bout of acute pneumonia, esp. fungal type, or other serious infection. [omicsonline.org]

  If swimmer's ear develops into necrotising otitis externa, haematogenous extension leading to sepsis may result. This rare complication can be life-threatening. Referral [ 3 ] Admit urgently if malignant otitis is suspected. [patient.info]

• Chills

  General symptoms such as fever, chills, and malaise are usually present in either process. [neupsykey.com]

• Pneumonia

  Penicillin-resistant Streptococcus pneumoniae in acute otitis media: risk factors, susceptibility patterns, and antimicrobial management. Pediatr Infect Dis J 1995; 14:751-759. ‎ Página 124 - ... combination of the two. [books.google.es]

  Other causes that can inspire keratosis formation are: • A serious bout of acute pneumonia, esp. fungal type, or other serious infection. [omicsonline.org]

  The primary cause is thought to be viral or possibly due to Mycoplasma pneumonia infection. Treatment is symptomatic; the blebs can be opened in an attempt to relieve pain. [helpmehear.ca]

  Use of the heptavalent pneumococcal vaccination (Prevnar) has become routine for preventing invasive infection in infants (meningitis and pneumonia). [neupsykey.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]

• 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]

  Clinically, a picket fence fever pattern is typical and some patients present with signs of intracranial hypertension (see p. 235). [neupsykey.com]

• Keratosis

  We discuss here a case of keratosis obturans in a 28 year old male patient who came with complaints of left ear pain. [worldwidejournals.com]

  Keratosis obturans and external ear canal cholesteatoma: How and why we should distinguish between these conditions. Clin Otolaryngol Allied Sci. 2004; 29: 577-581 Persaud R. Chatrath P. Cheesman A. Atypical keratosis obturans. [jaad.org]

  Abstract Aim: To find out the extent of simple disease like keratosis obturans and possible complications it can give rise to. Background: Keratosis obturans was first properly described and named by Wreden of St. [aijoc.com]

  Chaytor, “Wax keratosis in children’s ears,” BMJ, vol. 2, no. 5097, pp. 673–675, 1958. View at: Google Scholar A. W. Morrison, “Keratosis obturans,” The Journal of Laryngology & Otology, vol. 70, no. 5, pp. 317–321, 1956. View at: Google Scholar M. [hindawi.com]

  Additionally, keratosis obturans is associated with bronchiectasis and sinusitis. Complications There are a few incidences in the literature describing facial nerve palsy in patients with keratosis obturans. [symptoma.com]

• Hyperkeratosis

  Acanthosis nigricans Confluent and reticulated papillomatosis Callus Ichthyosis acquisita Arsenical keratosis Chronic scar keratosis Hyperkeratosis lenticularis perstans Hydrocarbon keratosis Hyperkeratosis of the nipple and areola Inverted follicular [en.wikipedia.org]

  […] inflamed epithelium May cause severe erosion of petrous bone ( Br J Radiol 1975;48:170 ) Treatment Removal of keratin plug Surgery not required Microscopic (histologic) description Tightly packed keratin squames in lamellar pattern Diffuse acanthosis and hyperkeratosis [pathologyoutlines.com]

  […] voorbeelden: English-Dutch dictionary > adenoidal См. также в других словарях: keratosis pharyngea — a condition in which white horny masses project out from the tonsils and from the orifices of lymph follicles in the wall of the pharynx … Medical dictionary hyperkeratosis [translate.academic.ru]

• Furunculosis

  The pathogenesis of KO remains obscure, although it has been associated with eczema, seborrhoeic dermatitis, or furunculosis. [hindawi.com]

  Multiple authors postulated that KO etiopathogenesis could be related to bronchiectasis, seborrheic dermatitis, eczema, and furunculosis [6-8] with subepithelial chronic inflammation [9]. [clinmedjournals.org]

• Otalgia

  Patients with this condition typically present with severe otalgia, conductive hearing loss, and global widening of the external auditory canal. We present a case of keratosis obturans that was misdiagnosed as impacted wax. [hindawi.com]

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

  A 61-year-old male had intermittent otorrhea and otalgia for several months in the left ear. The diagnosis of otitis externa was made by an otolaryngologist, and topical medications were applied. [jstage.jst.go.jp]

• Ear Discharge

  Case description: A 56-year-old female presented with right ear discharge since 15 days and giddiness with vomiting since 5 days with past history of right ear discharge since 5 years. [aijoc.com]

  This video show endoscopic Removal of Ear Wax / Keratosis obturans by micro-suction clearance under local anesthesia spray in girl. She presented with severe ear pain, ear canal swelling, ear discharge since last 7 days. [om.vlipa.lv]

  Symptoms Severe pain Ear discharge Tinnitus (ringing sound in ear) along with discharge from ear. [dict.eudic.net]

  discharge, and she consulted ENT clinics without benefits. [austinpublishinggroup.com]

• Vertigo

  It is relieved following removal of debris.[1] Vertigo Experienced by some patients. Extension of bony erosion with subsequent invasion of the adjacent structures of the temporal bone may leads to vertigo as well as facial nerve palsy.[1],[3]. [explainmedicine.com]

  In severe cases VII, VIII, IX nerve may become involved with facial palsy, vertigo, and retrocochlear deafness. Has to be differentiated from myringitis bullosa by viral serology. [docraza.tripod.com]

  […] suggested well-designed randomized clinical trials to assess the various methods of cerumen removal, as well as prospective clinical studies to evaluate the impact of cerumen removal on the resolution of symptoms, such as itching, hearing loss, pain, and vertigo [medpagetoday.com]

  Patients will have vertigo, hearing loss, and disequilibrium. The diagnosis of labyrinthitis can be made clinically in the setting of vertigo and SNHL. Treatment consists of supportive measures, antibiotics, and systemic steroids. [neupsykey.com]

  Depending on the pathway of the fracture line, symptoms may include conductive or sensorineural hearing loss, facial paralysis, tinnitus, or vertigo. [helpmehear.ca]

• Giddiness

  Case description: A 56-year-old female presented with right ear discharge since 15 days and giddiness with vomiting since 5 days with past history of right ear discharge since 5 years. [aijoc.com]

  Motion of the ear canal provided by ordinary chewing movements together with the process of epithelial proliferation and lateral migration propel the cerumen outward in a self-cleansing manner. complaints: hearing loss or blocked sensation tinnitus & giddiness [lessons4medicos.blogspot.com]

• Nystagmus

  Patient had horizontal nystagmus, gaze evoked with head shake test positive. Fistula sign was negative. High-resolution computed tomography (HRCT) temporal bone showed erosion of lateral semicircular canal and no ossicles. [aijoc.com]

  In such cases, a positive fistula test (air insufflation into the EAC, with resulting vertigo and nystagmus) further supports this diagnosis. [neupsykey.com]

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].

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]

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]

(Image Source: radiopaedia.org) Treatment Debridement of the mass is the primary treatment of keratosis obturans. It is done with microscope or endoscope guidance. [ent360.in]

It is possible that these new capillaries are formed as a result of inflammation of the surrounding bony canal.[1] Treatment[edit] Canaloplasty, where the ear canal is widened using grafts, was first proposed as the treatment for keratois obturans. [en.wikipedia.org]

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]

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

CT well-defined soft tissue mass within the bony EAC which may diffusely enlarge the canal with no bony erosion, as opposed to EAC cholesteatoma tympanic canal is usually spared but may be slightly thickened Treatment and prognosis Usually treated by [radiopaedia.org]

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. [drtbalu.co]

{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]

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]

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]

Embryology and epidemiology of microtia. Facial Plast Surg. 2009;25(3):145-148. Castilla EE, Orioli IM. Prevalence rates of microtia in South America. Int J Epidemiol. 1986;15(3):364-368. Bhat SM, Vuppala R. [pacificaudiologygroup.com]

Epidemiology Otosclerosis can be divided into histologic and clinical subtypes. [neupsykey.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]

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]

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]

Pathophysiology (Fig. 4.13B) The origin of SCC of the temporal bone is not well established. [neupsykey.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]

Comparative Study External auditory canal cholesteatoma and keratosis obturans: the role of imaging in preventing facial nerve injury Edward D McCoul et al. Ear Nose Throat J. 2011 Dec. [pubmed.ncbi.nlm.nih.gov]

Patients need to attend ENT clinics for regular micro-suction of their ears to minimise the discomfort, prevent infection and help them to hear better with or without hearing aids. [gavinpublishers.com]

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]

After complete removal of the mass, canal bone can get exposed, it is essential to prevent infection by applying topical ear drops, ointments or oral antibiotics. [ent360.in]

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

1. Dalton SR, Ferringer T, Mowad CM. Obstruction of the external auditory canal by a keratin cast: Keratosis obturans or cholesteatoma? J Am Acad Dermatol. 2011;65(3):e88-89.
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.
4. Tran LP, Grundfast KM, Selesnick SH. Benign lesions of the external auditory canal, Otolaryngol Clin North Am. 1996;29(5):807-825.
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.
6. Holt JJ. Ear canal cholesteatoma. Laryngoscope 1992;102(6):608–613.
7. Piepergedes JC, Behnke EE. Keratosis obturans and external auditory canal cholesteatoma. Laryngoscope. 1980;90(3):383-391.
8. Glynn F, Keogh IJ, Burns H. Neglected keratosis obturans causing facial nerve palsy. J Laryngol Otol. 2006;120(9):784-785.
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.