Keratosis Obturans Research Articles

Ear canal cholesteatoma: meta-analysis of clinical characteristics with update on classification, staging and treatment

We present an update on clinical evaluation, staging, classification and treatment of canal cholesteatoma, including a meta-analysis of clinical data of the last 30 years. Ear canal cholesteatoma is frequently associated secondarily to other canal pathologies. The cause for the rare idiopathic form of the disease remains enigmatic. Epidemiologic and experimental studies of its pathogenesis have increased; however, the main explanatory theory of a deficient migratory capacity of the canal epithelium affected has been falsified only recently. Therefore, the debate on the pathogenesis has gained additional impetus and more data is needed. Canal cholesteatoma is a rarity in otologic pathology, often leading to misdiagnosis as external otitis or otomycosis by physicians unfamiliar with the disease. It presents typically with otorrhea, focal erosion and keratin accumulation in the osseous ear canal and has to be distinguished from keratosis obturans, which leads to otalgia and bilateral conductive hearing loss by ceruminal plugs, with circumferential distention of the ear canal. Treatment by canaloplasty is curative and highly successful. Alternative conservative treatment is feasible, however, requiring long-term follow up, with often painful cleaning of the lesion.

Cochlear implantation in children with keratitis-ichthyosis-deafness (KID) syndrome: outcomes in three cases

Three children with keratitis-ichthyosis-deafness (KID) syndrome received cochlear implants at the Royal Victorian Eye and Ear Hospital. KID syndrome is a rare genodermatosis associated with mutation of the connexin-26 gene with characteristics affecting skin, hair, vision and hearing. Ichthyotic involvement of the ear canal epithelium and associated non-erosive keratosis obturans complicate hearing assessment and aid fitting. The tendency to eczematous dermatitis and otitis media is an additional problem with cochlear implantation. All cases have required additional medical management, however the outcomes show that the cochlear implant can be effective in these patients. Copyright © 2008 John Wiley & Sons, Ltd.

Cochlear implantation in children with keratitis-ichthyosis-deafness (KID) syndrome: Outcomes in three cases

Three children with keratitis-ichthyosis-deafness (KID) syndrome received cochlear implants at the Royal Victorian Eye and Ear Hospital. KID syndrome is a rare genodermatosis associated with mutation of the connexin-26 gene with characteristics affecting skin, hair, vision and hearing. Ichthyotic involvement of the ear canal epithelium and associated non-erosive keratosis obturans complicate hearing assessment and aid fitting. The tendency to eczematous dermatitis and otitis media is an additional problem with cochlear implantation. All cases have required additional medical management, however the outcomes show that the cochlear implant can be effective in these patients. Copyright © 2008 John Wiley & Sons, Ltd.

External Auditory Canal Cholesteatoma and Keratosis Obturans: the Role of Imaging in Preventing Facial Nerve Injury

We conducted a retrospective study to compare the clinical characteristics of external auditory canal cholesteatoma (EACC) with those of a similar entity, keratosis obturans (KO). We also sought to identify those aspects of each disease that may lead to complications. We identified 6 patients in each group. Imaging studies were reviewed for evidence of bony erosion and the proximity of disease to vital structures. All 6 patients in the EACC group had their diagnosis confirmed by computed tomography (CT), which demonstrated widening of the bony external auditory canal; 4 of these patients had critical erosion of bone adjacent to the facial nerve. Of the 6 patients with KO, only 2 had undergone CT, and neither exhibited any significant bony erosion or expansion; 1 of them developed osteomyelitis of the temporal bone and adjacent temporomandibular joint. Another patient manifested KO as part of a dermatophytid reaction. The essential component of treatment in all cases of EACC was microscopic debridement of the ear canal. We conclude that EACC may produce significant erosion of bone with exposure of vital structures, including the facial nerve. Because of the clinical similarity of EACC to KO, misdiagnosis is possible. Temporal bone imaging should be obtained prior to attempts at debridement of suspected EACC. Increased awareness of these uncommon conditions is warranted to prompt appropriate investigation and prevent iatrogenic complications such as facial nerve injury.

Rogowacenie obturacyjne przewodu słuchowego zewnętrznego

Keratosis obturans is a rare condition of the bony part of external ear canal cased by hyperkeratosis of epiderm and disorders of migration process. Hyper-cumulation of desquamated epiderm in the external ear canal leads to hearing loss, earache and inflammation of ear canal skin. We present 2 cases of keratosis obturans as examples of epiderm migration disorders and difficulties in diagnosis and treatment of this disease. Keratosis obturans was diagnosed in both cases in histopathological and immunohistochemical examinations. Histopathological and molecular structure of keratosis obturans was discussed. In presented cases surgical, anti-inflammatory and keratinolytic treatment was applyied.

Epidermal hyperplasia of the tympanic membrane and deep external ear canal: novel histo‐pathological features, embryological and clinical correlates

Objectives. To study the histology and developmental biology of epidermis of tympanic membrane and deep ear canal and to correlate the histo‐pathological features with clinical conditions.Method. Histo‐pathological and electron microscopic examination was performed of deep meatal skin, keratosis, keratosis obturans, external canal cholesteatoma, retraction pockets, acquired cholesteatoma and tympanic membrane perforations.Results. In some mature ears, a marked change in the normal flat thin epidermis to one resembling that of the fetus is seen in the presence of local inflammation. This hyperplastic epidermis is present in varying lengths on the tympanic membrane and deep ear canal and displayed as hyperplastic bands growing down into sub‐epidermal tissue and sometimes penetrated the collagen layer of the tympanic membrane to enter the middle ear. Keratosis was frequent on the surfaces of the tympanic membrane and deep ear canal, which may also be found in the invading bands, where they produced small keratin cysts.Conclusions. Severe chronic otitis media, with inflammatory exudates, glandular metaplasia or tympanosclerosis, may be associated with hyperplasia. This type of epidermal hyperplasia appeared to be pathogenic and was observed in several structurally distinct entities such as keratosis, keratosis obturans, retraction pockets, and cholesteatoma of the deep external canal. Invading epidermis in the middle ear, in some cases, is indistinguishable from acquired cholesteatoma. This reverse differentiation highlights a primordial property of auditory epithelium, which was not previously recognised. Furthermore, hyperplastic epidermis appears to be pathogenic and may be the cause of some specific conditions or their complications.References 1 Johnson A., Hawke M., Berger G. (1984) Hodge memorial Award‐surface wrinkles, cell ridges, and desquamation in the external auditory canal. J Otolaryngol.13, 345–3542 Johnson A., Hawke M. (1985) Cell shape in the migratory epidermis of the external auditory canal. J Otolaryngol.14, 273–281

Complications of keratosis obturans

Three patients with extensive keratosis obturans were treated during a 12-month period. One presented with an idiopathic sensorineural hearing loss and was found to have keratosis obturans in the contralateral, asymptomatic ear. The disease process had resulted in a horizontal semicircular canal fistula in what was now, effectively, the only hearing ear. The second patient had an extensive dehiscence of the tegmen tympani. The third presented with a facial palsy. An automastoidectomy cavity was present, with circumferential skeletonization of the descending facial nerve over a length of 1.5 cm and dehiscence of the temporomandibular joint and jugular bulb. All three patients were successfully treated by surgical formalization of their automastoidectomy cavities. They appeared to represent cases of keratosis obturans rather than external auditory canal cholesteatoma, on the basis of previously published reports.These complications and patterns of bone erosion have not previously been described in keratosis obturans. The third patient is believed to have the most extensive case of keratosis obturans yet described.

Neglected keratosis obturans causing facial nerve palsy

Keratosis obturans is characterized by the accumulation of desquamated keratinous material in the bony portion of the external auditory canal. Classically, it is reported to present with severe otalgia, conductive hearing loss and global widening of the external auditory canal. Extensive erosion of the bony meatus, with exposure of the facial nerve, has been previously reported, but no case of facial nerve palsy has as yet been published. We report the first published case, to our knowledge, of a unilateral facial nerve palsy secondary to neglected keratosis obturans.

Keratosis obturans and external ear canal cholesteatoma: how and why we should distinguish between these conditions

Keratosis obturans and external ear canal cholesteatomas have been considered as separate entities for the last 20 years, after being regarded as variations of the same disease for at least 87 years. While both disorders are distinct, they do have some overlapping characteristics which may make it difficult to reach a definite diagnosis. This review explores the diagnostic dilemmas which may arise, and discusses the classification, aetiology, pathogenesis and management of these conditions. We concur that external ear canal cholesteatoma and keratosis obturans are different conditions and conclude that the presence of osteonecrosis and focal overlying epithelial loss are the most reliable features favouring the diagnosis of external ear canal cholesteatoma over keratosis obturans. Furthermore, whilst keratosis obturans can be managed successfully by regular aural toilet, external ear canal cholesteatoma may require surgical intervention depending on the extent of the disease.

Atypical keratosis obturans

Keratosis obturans is a rare condition characterized by the accumulation of desquamated keratin material in the bony portion of the external auditory canal. Classically, it is reported to present with severe otalgia, conductive deafness and global widening of the canal. 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. This presenting symptom and resorption pattern are atypical of keratosis obturans and have not been documented previously.

Yellow Nail Syndrome in Association with Protein-Losing Enteropathy in a Diabetic Patient

The yellow nail syndrome is a rare disorder which is characterized by the triad of y ellow nails, lymphedema and pleural effusion. The etiology of the syndrome is unknown, but impaired lymphatic drainage may play a role in its pathogenesis. The yellow nail syndrome has been reported in association with various conditions such as thyroid disease, hypogamma-globulinemia, nephrotic syndrome, rheumatoid arthritis, obstructive sleep apnea, keratosis obturans in the external ear, carcinoma of the gall bladder, xanthogranulomatous pyelonephritis, onychomycosis and proteinlosing enteropathy (PLE) in the literature. 1-3 A case of a diabetic woman with chronic recurrent pleural effusion, lymphedema, yellow discolored nails, chronic sinusitis, bronchiectasis and PLE is presented here. Case Report

Otological manifestations of primary ciliary dyskinesia.

Primary ciliary dyskinesia is a hereditary defect in the ultrastructure of cilia, leading to poor ciliary motility. The sinonasal and the bronchial manifestations of the disease are well documented; whereas its otological aspects have received less attention. In this report, we describe the clinical profile of 16 patients with primary ciliary dyskinesia laying particular emphasis on the otological manifestations. All children (11 patients) had bilateral otitis media with effusion. Of the five adults, three had tympanosclerosis; one had bilateral cholesteatoma; and one patient had bilateral keratosis obturans in combination with tympanosclerosis. Hearing improvement and a dry ear was achieved in all the children treated by tympanostomy tube insertion. The study suggests that otitis media is a prominent feature of this disorder. Most subjects suffer from protracted bilateral otitis media with effusion throughout childhood.

지속적인 점이액의 투여로 치유된 외이도 폐쇄성 각화증 1례

Keratosis obturans is characterized by dense keratin plug in the ear canal and its presenting symptoms are otalgia, and hearing loss. The treatment of keratosis obturans consists of mechanical removal of the epidermal plug, removal of any granulation, and periodic cleansing of any recurrence. Recently, the authors experienced a case of keratosis obturans treated by constant application of otic solution without surgery.

Epithelial migration in keratosis obturans

Keratosis obturans is a rare condition in which a cholesteoma-like mass is found filling the deep external auditory meatus. It has been suggested that a fault in auditory epithelium migration is responsible for the accumulation of squamous debris, but no studies have been carried out to confirm this. We present two cases of keratosis obturans in which delayed and abnormal pathways of migration of epithelium of the tympanic membrane were discovered, indicating that a fault in auditory epithelium may indeed be the basis of this condition.

Optimum management of the discharging ear.

Discharge from the ear can be the result of many disease processes. The ear may discharge blood, pus, cerebrospinal fluid (CSF) or wax. Keratosis obturans, stenosis of the external meatus and benign tumours of the external meatus all lead to wax build-up, which may cause recurrent attacks of otitis externa. Malignant tumours, such as basal cell carcinoma, squamous cell carcinoma and tumours of ceruminous gland origin may also present with discharge. Tumours should be excluded by submitting all material removed from the external canal for histological examination. Single or multiple abscesses (known as furuncles) may occur in the hair follicles in the skin of the external acoustic meatus (EAM). Compulsive scratching, hearing aids and foreign bodies placed in the ear predispose to otitis externa, which is also often associated with infection by Pseudomonas aeruginosa, Staphylococcus aureus and faecal organisms. Management may be with aluminium acetate 14%, topical antibiotic/steroid drops, a gauze wick soaked with icthammol 10% in glycerin or polymyxin B sulphate--neomycin sulphate--hydrocortisone acetate cream placed into the EAM and replaced every 24 to 48 hours, or systemic antibiotics according to severity. Malignant (necrotising) otitis externa causes progressive destruction of the temporal bone, and cranial nerve palsies (usually facial first). Treatment is limited debridement of infected bone, accompanied by intravenous aminoglycosides, and local antibiotic treatment and aural cleanout or oral ciprofloxacin. Middle ear conditions causing discharge include acute otitis media, infected grommets, traumatic perforations and chronic suppurative otitis media, as well as tumours of the ear canal skin and middle ear, radiation-induced otitis externa and osteoradionecrosis of the temporal bone, tuberculosis, Langerhans cell histiocytosis, spontaneous or post-traumatic CSF leaks, Wegeners granulomatosis and immune deficiency states. Topical application of aminoglycoside antibiotics to the middle ear of laboratory animals such as rats, guinea pigs and chinchillas causes sensorineural hearing loss, an effect rarely seen clinically in humans. If the external acoustic meatus and tympanic membrane are obscured by discharge cotton buds, microsuction equipment or syringing are used to remove it. It is often useful to initiate treatment (usually with topical drops, wicks or an oral antibiotic) with a provisional diagnosis. A full examination and adequate visualisation of the tympanic membrane must eventually be performed, if necessary under anaesthesia, or else serious progressive conditions may be neglected. The most useful initial investigation is a swab sent for bacteriological assessment; other investigations are usually indicated by clinical findings and the provisional diagnosis.

Ear Involvement in the Yellow Nail Syndrome

Recognized features of the yellow nail syndrome include yellow nails, lymphedema, and pleural effusions. We report a patient with the additional feature of keratosis obturans, which may be a manifestation of this syndrome in the external ear.

外耳道真珠腫の一症例

There are two types of external meatal cholesteatoma; keratosis obturans and external auditory canal cholesteatoma(EACC). They differ Pathologically and have different aspects. EACC was found in a 26-year-old female. Surgical removal of the cholesteatoma followed by meatal plasty using a pedicle temporal muscle cured the lesion. The pathogenesis and treatment of this entity are discussed.

Soft-tissue abnormalities of the external auditory canal: subject review of CT findings.

We review the normal anatomy and discuss characteristic findings of soft-tissue abnormalities of the external auditory canal (EAC). The indications for computed tomography (CT) of the temporal bone have been significantly expanded with the inclusion of soft-tissue abnormalities of the external ear and the auditory canal. Soft-tissue abnormalities of the EAC that can be evaluated with CT include atresia, edema, hemorrhage, fracture, posttraumatic or infection-caused keloid, malignant external otitis, hemangioma, lymphangioma, papilloma, keratosis obturans, acquired cholesteatoma, adenoma, ceruminoma, fibroma, mixed tumor, sarcoma, and basal cell, squamous cell, or adenocystic carcinoma. CT scans of 25 patients who had soft-tissue abnormalities of the EAC were reviewed. The clinical data were correlated with the radiographic findings. We conclude that CT is the best overall radiographic modality for evaluating the extent and character of soft-tissue abnormalities of the EAC. Significant clinical information that is helpful in patient management decisions is added by this technique.

External Auditory Canal Cholesteatoma

So-called external auditory canal cholesteatoma is not a rare disease, but the etiology is not clear. Keratosis obturans and external auditory canal cholesteatoma have been considered to be the same disease. However, recently Piepergerdes reported that keratosis obturans and external auditory canal cholesteatoma are two different clinical and pathological processes, the former being characterized by a dense plug of keratin debris within the deep meatus and inflammation of the subepithelial tissue. Recovery is usually complete with conservative treatment. On the other hand, external auditory canal cholesteatmoa is characterized by a localized erosion of the deep canal wall with localized periosteitis and sequestration; it usually requires operative treatment.We describe here two cases of so-called external auditory canal cholesteatoma which we considered to be keratosis obturans and discuss the differences between external auditory canal cholesteatoma and keratosis obturans reported in the literature.Clinical differentiation between external auditory canal cholesteatoma and keratosis obturans is important since the treatment is different.

The Pathologic Features of Keratosis Obturans and Cholesteatoma of the External Auditory Canal

The presence of a keratin plug occluding the deep external auditory canal was first noted and documented in the 19th century. It has subsequently been proposed that two different diseases can be responsible for the presence of this type of obstruction within the deep meatus: keratosis obturans and external auditory canal cholesteatoma. Keratosis obturans is characterized by a dense plug of keratin debris located primarily within the deep meatus. There is an associated hyperplasia of the underlying epithelium and evidence of chronic inflammation within the subepithelial tissue. There is no evidence of erosion or necrosis of the underlying bone. In external auditory canal cholesteatoma the significant finding is extensive erosion of the bony external auditory canal by a wide-mouthed sac, lined with stratified squamous keratinizing epithelium, that arises lateral to the tympanic membrane and is located in the inferior portion of the bony external canal. There is frequently evidence of sequestration of the underlying bone.