History

Fact Explanation
Introduction to the disease The meaning of cholesteatoma is “skin in the wrong place”.It usually consists of squamous epithelium which is trapped within the middle ear and mastoid.[1]. It is not cancerous but it can erode surrounding tissues and cause destruction of the ear.[2].Cholesteatoma can be either congenital or acquired.[7].
Otorrhea Painless otorrhea is a typical presentation of cholesteatoma. It can be either persistent or frequently recurrent even with aggressive antibiotic treatment.[1]. Associated infection may leads to foul smelling discharge which sometimes may contain blood.[2].
Otalgia Cholesteatoma itself is not often painful. But when it is associated with infection, it may cause pain.[2].
Tinnitus and fullness of the ear Patients are usually asymptomatic but can present with ear fullness and tinnitus.[6].Tinnitus is ringing or humming of one or both ears without any stimulus. It is a manifestation of tinnitus.[3],[6].
Hearing loss It can leads to gradual loss of hearing.The expanding sac may causes destruction of the ear drum and ossicles which may ultimately lead in to conductive type hearing loss.[2],[1].
Headache and vertigo Disease may rarely causes vertigo.[1].Some patients may also present with dizziness and headache.[6].
Meningitis and brain abscess Those are intracranial complications of cholesteatoma due to local extension of the disease and invasion into surrounding tissues including internal structures of brain.[4].
Sigmoid sinus thrombosis and hydrocephalus, Those two are also intracranial complications of cholesteatoma.[4].
Disturbance of balance Disruption of balance in cholesteatoma can result due to erosion of the balance organs, such as labyrinth and semicircular canal [1],[4].
Tympano ossicular chain disruption and mastoid abscesses Tympano ossicular chain disruption, labyrinthine fistula as well as mastoid abscesses are intratemporal complication of cholesteatoma due to extension of disease in to surrounding structures.[4].
History of past infection/fluid problems in the ear Patients who had cholesteatoma frequently have had some previous problems with middle ear fluid and/or infections. However most patients with history of ear fluid/infections do not develop cholesteatoma.[2].
History of improper function of the Eustachian tube Some studies suggest that improper function of the Eustachian tube may leads to formation of cholesteatoma. What happen in these situation is if the Eustachian tube does not open often enough to equalize the pressures in the middle ear. So that negative pressure will develop behind the ear drum lead in to retraction of ear drum by forming a pocket. Gradually it becomes trapped in the ear forming cholesteatoma.[2].
History of otitis media Cholesteatoma is found to be frequently associated with middle ear infections like chronic otitis media.[1],[5].
History of trauma and surgery Cholesteatoma can be result from metaplasia of the middle ear mucosa or implantation following trauma or even after surgery.[7].
History of congenital cholesteatoma Congenital cholesteatoma of middle ear may manifests in young ages as a whitish mass in the middle ear with intact tympanic membrane. It is a relatively rare disease and account for about 2% of middle ear cholesteatoma. Several etiological hypotheses including defect of the tympanic ring, , epithelial metaplasia and implantation theory as well as epidermoid formation are used to explain the development of this disease.[6].
References
  1. TROJANOWSKA A, DROP A, TROJANOWSKI P, ROSIńSKA-BOGUSIEWICZ K, KLATKA J, BOBEK-BILLEWICZ B. External and middle ear diseases: radiological diagnosis based on clinical signs and symptoms Insights Imaging [online] , 3(1):33-48 [viewed 14 October 2014] Available from: doi:10.1007/s13244-011-0126-z
  2. HOLT JJ. Cholesteatoma and Otosclerosis: Two slowly progressive causes of hearing loss treatable through corrective surgery Clin Med Res [online] 2003 Apr, 1(2):151-154 [viewed 14 October 2014] Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC1069039
  3. POLAT CAHIT, BAYKARA MURAT, ERGEN BURHAN. Evaluation of Internal Auditory Canal Structures in Tinnitus of Unknown Origin. Clin Exp Otorhinolaryngol [online] 2014 December [viewed 14 October 2014] Available from: doi:10.3342/ceo.2014.7.3.160
  4. BRUZZI CATERINA, NEGRI MAURIZIO, CASALE MANUELE, FRARI VALERIA, SALVINELLI FABRIZIO, GALLI STEFANO. A Rare Middle Ear Cholesteatoma Complication behind Painless Swelling of the Temporozygomatic Region. NM [online] 2014 December, 05(04):196-199 [viewed 14 October 2014] Available from: doi:10.4236/nm.2014.54022
  5. RICCIARDIELLO F, CAVALIERE M, MESOLELLA M, IENGO M. Notes on the microbiology of cholesteatoma: clinical findings and treatment Acta Otorhinolaryngol Ital [online] 2009 Aug, 29(4):197-202 [viewed 14 October 2014] Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2816367
  6. PARK KH, PARK SN, CHANG KH, JUNG MK, YEO SW. Congenital Middle Ear Cholesteatoma in Children; Retrospective Review of 35 Cases J Korean Med Sci [online] 2009 Feb, 24(1):126-131 [viewed 14 October 2014] Available from: doi:10.3346/jkms.2009.24.1.126
  7. PALUDETTI G, CONTI G, DI NARDO W, DE CORSO E, ROLESI R, PICCIOTTI PM, FETONI AR. Infant hearing loss: from diagnosis to therapy Official Report of XXI Conference of Italian Society of Pediatric Otorhinolaryngology Acta Otorhinolaryngol Ital [online] 2012 Dec, 32(6):347-370 [viewed 16 October 2014] Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3552543

Examination

Fact Explanation
Ear discharge Cholesteatoma has characteristic pearly grey sheen. Cholesteatomatous materials are yellowish in color.[1].Initial inspection may reveals ear canal full of discharge. A foul smelling discharge which contain blood is detected in case of associated infection.[2].
Swelling behind the ear Some may present with pain and swelling behind the ear which may detect on examination, indicative of ongoing infection.[2].
Erosion of bones and surrounding structures of ear The classic acquired cholesteatoma can cause erosion of scutum and ossicular destruction. It can erode mastoid , tegmen tympani as well as lateral semicircular canal.[3].
Facial nerve palsy Cholesteatomas which arising from posterior tympanic membrane, are likely to cause facial nerve exposure.They can erode bony cover of facial nerve canal as well as destruction of the stapedial suprastructure further lead in to facial nerve palsy.[3].
Hearing loss Examination of cranial nerves may reveals conductive hearing loss that may due to destruction of the ear drum and ossicles. Usually it is a gradual loss of hearing.[2],[3].
References
  1. MICHAELS L. Pathology of cholesteatoma: a review. J R Soc Med [online] 1979 May, 72(5):366-369 [viewed 14 October 2014] Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC1436860
  2. HOLT JJ. Cholesteatoma and Otosclerosis: Two slowly progressive causes of hearing loss treatable through corrective surgery Clin Med Res [online] 2003 Apr, 1(2):151-154 [viewed 14 October 2014] Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC1069039
  3. TROJANOWSKA A, DROP A, TROJANOWSKI P, ROSIńSKA-BOGUSIEWICZ K, KLATKA J, BOBEK-BILLEWICZ B. External and middle ear diseases: radiological diagnosis based on clinical signs and symptoms Insights Imaging [online] , 3(1):33-48 [viewed 14 October 2014] Available from: doi:10.1007/s13244-011-0126-z

Differential Diagnoses

Fact Explanation
Otosclerosis It is a condition affecting the stapes bone.A callus of bone accumulates on the stapes creating a partial fixation is seen in otosclerosis which leads to limitation of movement of the stapes bone. This may leads to hearing loss. It is a common symptom and commonly found in their teens or early adulthood. Associated tinnitus or a sound in the affected ear may experienced by the most of affected individuals. Around 50% of patients have positive family history of otosclerosis. Unlike cholesteatoma, this may not detect by office examination and it will reveal normal ear canal and eardrum.[1].
Acute otitis media It results as a sequelae of viral upper respiratory tract infection. It causes disruption of the mucosal barrier so that bacteria which comes from the nose and nasopharynx may spread to the middle ear. The common clinical features include ear pain, erythema of the tympanic membrane, fever as well as cold symptoms.[5].
Chronic suppurative otitis media It causes either recurrent or persistent discharge called otorrhoea, through a perforation in the tympanic membrane. It is a well known cause of hearing impairment,disability, as well as poor scholastic performance.Fatal intracranial infections and acute mastoiditis may result in serious conditions. Disease may leads to mucosal polyps,thickening of the middle ear mucosa as well as cholesteatoma.[2].
Middle ear schwannoma It is a tumour arising within middle ear cavity. It originating from the facial nerve, chorda tympani nerve (7th branch), 9th branch or either 10th branch. But the most common nerve of origin is facial nerve. It may present either with conductive hearing loss or with facial nerve dysfunction.Fleshy-white mass behind intact tympanic membrane can be seen in otoscopic examination.[3].
Tympanosclerosis It causes deposition of hyalinised collagen in the tympanic cavity. It is called myringosclerosis when it occurs in tympanic membrane. [3]. It causes hearing impairment and on examination it can be recognized easily due to the presence of white plaques in tymphanic membrane and middle ear.[4].
Traumatic tympanic membrane perforation, It causes by direct or indirect source.Trauma to the tympanic membrane and the middle ear can be resulted from overpressure such as slap, fight, assault , RTA ( Road traffic accident). It can also resulted from thermal burns, blunt or penetrating injuries such as instrumentations and barotraumas. This may leads to conductive type hearing loss.[6],[5].
References
  1. HOLT JJ. Cholesteatoma and Otosclerosis: Two slowly progressive causes of hearing loss treatable through corrective surgery Clin Med Res [online] 2003 Apr, 1(2):151-154 [viewed 15 October 2014] Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC1069039
  2. ACUIN J. Chronic suppurative otitis media Clin Evid (Online) [online] :0507 [viewed 16 October 2014] Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2943814
  3. TROJANOWSKA A, DROP A, TROJANOWSKI P, ROSIńSKA-BOGUSIEWICZ K, KLATKA J, BOBEK-BILLEWICZ B. External and middle ear diseases: radiological diagnosis based on clinical signs and symptoms Insights Imaging [online] , 3(1):33-48 [viewed 16 October 2014] Available from: doi:10.1007/s13244-011-0126-z
  4. GIBB AG. President's address. Tympanosclerosis. Proc R Soc Med [online] 1976 Mar, 69(3):155-162 [viewed 16 October 2014] Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC1864179
  5. PALUDETTI G, CONTI G, DI NARDO W, DE CORSO E, ROLESI R, PICCIOTTI PM, FETONI AR. Infant hearing loss: from diagnosis to therapy Official Report of XXI Conference of Italian Society of Pediatric Otorhinolaryngology Acta Otorhinolaryngol Ital [online] 2012 Dec, 32(6):347-370 [viewed 16 October 2014] Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3552543
  6. AFOLABI OA, AREMU SK, ALABI BS, SEGUN-BUSARI S. Traumatic Tympanic Membrane perforation: An aetiological profile BMC Res Notes [online] :232 [viewed 16 October 2014] Available from: doi:10.1186/1756-0500-2-232

Investigations - for Diagnosis

Fact Explanation
CT scan Diagnosis is mainly clinical with the help of imaging, such as CT and MRI. The choice of CT and MRI depend on the clinical situation.Pars flaccida cholesteatoma will show soft tissue mass in Prussak’s space, medial to attic wall as well as lateral to head of malleus and body of incus. Ossicle erosion also can be recognized.Pars tensa cholesteatoma will show erosive mass of soft tissue density in posterior tympanum. It may also involves sinus tympani, facial recess as well as mastoid. By looking at a CT we can get an idea about Ossicle erosion and displacement, Bony cover of tegmen tympani and lateral semicircular canal, bony walls, as well as extension of cholesteatoma to other adjacent structures. CT not only helpful in diagnosis of the disease, but also to exclude several differential diagnosis mentioned under DD.[1].
MRI Cholesteatoma will show bright, hyper-intense signal on B-1,000 images, on diffusion-weighted MRI images,lack of enhancement on post-gadolinium T1-weighted sequence as well as hypo-intense signal on ADC map. Unlike hyperintensity seen in inflammation, cholesteatoma will show moderate intensity on T2-weighted images. The MRI models such as delayed post-gadolinium T1-weighted sequences and diffusion-weighted MRI or even a combination of both techniques can be used. However non–EPI is found to be superior to EPI. MRI also used to exclude similar conditions to cholesteatoma which is mentioned under DD.[1].
Audiometry Performed for hearing assessment since patients are complained of hearing loss.[2],[3].
FBC Performed as a basic investigation to detect ongoing infection.[2].
ESR Also can performed as a baseline investigation in suspected cases of associated infection.[2].
References
  1. TROJANOWSKA A, DROP A, TROJANOWSKI P, ROSIńSKA-BOGUSIEWICZ K, KLATKA J, BOBEK-BILLEWICZ B. External and middle ear diseases: radiological diagnosis based on clinical signs and symptoms Insights Imaging [online] , 3(1):33-48 [viewed 16 October 2014] Available from: doi:10.1007/s13244-011-0126-z
  2. HOLT JJ. Cholesteatoma and Otosclerosis: Two slowly progressive causes of hearing loss treatable through corrective surgery Clin Med Res [online] 2003 Apr, 1(2):151-154 [viewed 16 October 2014] Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC1069039
  3. PARK KH, PARK SN, CHANG KH, JUNG MK, YEO SW. Congenital Middle Ear Cholesteatoma in Children; Retrospective Review of 35 Cases J Korean Med Sci [online] 2009 Feb, 24(1):126-131 [viewed 16 October 2014] Available from: doi:10.3346/jkms.2009.24.1.126

Investigations - Fitness for Management

Fact Explanation
Full blood count (FBC) Carried out to detect Hb and platelet levels. It is performed to assess the fitness for surgery.[1].
Fasting blood sugar (FBS) It is also performed to assess the fitness before surgery since it is done under GA to assess the blood glucose level.[1].
ESR Performed as a routine investigation to find out ongoing inflammation.[1],[2].
CT and MRI CT is the primary and sole imaging technique prior to first-stage surgery.Ossicular and epitympanic erosion can be diagnose by this as well as it is useful in evaluating facial nerve canal and lateral semicircular canal integrity prior to surgery. MRI also play a role in diagnosing extension of disease prior to surgery.[2].
References
  1. HOLT JJ. Cholesteatoma and Otosclerosis: Two slowly progressive causes of hearing loss treatable through corrective surgery Clin Med Res [online] 2003 Apr, 1(2):151-154 [viewed 16 October 2014] Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC1069039
  2. TROJANOWSKA A, DROP A, TROJANOWSKI P, ROSIńSKA-BOGUSIEWICZ K, KLATKA J, BOBEK-BILLEWICZ B. External and middle ear diseases: radiological diagnosis based on clinical signs and symptoms Insights Imaging [online] , 3(1):33-48 [viewed 16 October 2014] Available from: doi:10.1007/s13244-011-0126-z

Investigations - Screening/Staging

Fact Explanation
CT scan It is useful in staging congenital middle ear cholesteatoma. In stage 1 is affect single quadrant and there is no ossicular involvement or mastoid extension. Level 2 involves multiple quadrants without ossicular involvement or mastoid extension. Level 3 causes ossicular involvement with ossicular erosion but there is no mastoid extension. Level 4 causes mastoid extension.[1].
References
  1. PARK KH, PARK SN, CHANG KH, JUNG MK, YEO SW. Congenital Middle Ear Cholesteatoma in Children; Retrospective Review of 35 Cases J Korean Med Sci [online] 2009 Feb, 24(1):126-131 [viewed 16 October 2014] Available from: doi:10.3346/jkms.2009.24.1.126

Management - General Measures

Fact Explanation
Patient education Patient / family should be educate regarding the disease, and what consequences resulted in if left untreated. Then doctor should explain the available surgical methods to them. After that should inform the possible surgical complications and take written consent for the surgery. Those possible complications are hearing loss which can be complete and permanent,Dizziness which may resolves within a day of surgery,partial or complete facial nerve paralysis, Tinnitus and taste abnormalities. And also possible complications of anesthesia since this is performed under GA, bleeding, infection and other more remote operative problems should be informed to the patient.[1].
Ciprofloxacin and levofloxacin Even though treatments are solely surgical, antibiotics such as ciprofloxacin and levofloxacin seem to be effective because they are active against Pseudomonas, Staphylococcus and also against anaerobic bacteria which can be found in associated ear infections.[2].
References
  1. HOLT JJ. Cholesteatoma and Otosclerosis: Two slowly progressive causes of hearing loss treatable through corrective surgery Clin Med Res [online] 2003 Apr, 1(2):151-154 [viewed 16 October 2014] Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC1069039
  2. RICCIARDIELLO F, CAVALIERE M, MESOLELLA M, IENGO M. Notes on the microbiology of cholesteatoma: clinical findings and treatment Acta Otorhinolaryngol Ital [online] 2009 Aug, 29(4):197-202 [viewed 16 October 2014] Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2816367

Management - Specific Treatments

Fact Explanation
Tympanoplasty Treatment are solely surgical. [3].Type of surgery depend on the stage of disease,extent of the cholesteatoma and the size of the mastoid.The primary aim of surgery is to remove the disease and where the secondary target is to restore or maintain hearing.[1].stage 1 and 2 can be treat with tympanoplasty.[2]. The surgery involves the tympanum, which is the area of the ear behind the ear drum where the ossicles are located.[1].The wall of external auditory canal is preserved in canal wall up tympanoplasty, and the wall of external auditory canal is removed and large mastoid cavity is left in canal wall down procedure.[3].
Mastoidectomy It is a surgery performed in the mastoid.[1]. Type 2 may require extended tympanoplasty, canal wall up mastoidectomy and also ossiculoplasty. And where as in type 3 may require canal wall down mastoidectomy. Due to high possibility of developing cavity problems,canal wall down mastoidectomy needs continuous ear treatment throughout the life.[2].
Tympanomastoidectomy Another available surgical method that involves both the tympanum and mastoid.[1].
Ossiculoplasty Ossiculoplasty or so called ossicular reconstruction is repair or reconstruction of the bones of hearing.There are several techniques and prostheses that can be used for the reconstruction.[1]. Useful in type 2 and type 3 cases.[2]. There are two basic surgical approaches to the ear. One that performed through the ear canal is called Transcanal where the surgery that is performed by making an incision behind the ear and moving the ear forward to allow exposure of the mastoid and middle ear is called Postauricular.[1].
References
  1. HOLT JJ. Cholesteatoma and Otosclerosis: Two slowly progressive causes of hearing loss treatable through corrective surgery Clin Med Res [online] 2003 Apr, 1(2):151-154 [viewed 16 October 2014] Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC1069039
  2. PARK KH, PARK SN, CHANG KH, JUNG MK, YEO SW. Congenital Middle Ear Cholesteatoma in Children; Retrospective Review of 35 Cases J Korean Med Sci [online] 2009 Feb, 24(1):126-131 [viewed 16 October 2014] Available from: doi:10.3346/jkms.2009.24.1.126
  3. TROJANOWSKA A, DROP A, TROJANOWSKI P, ROSIńSKA-BOGUSIEWICZ K, KLATKA J, BOBEK-BILLEWICZ B. External and middle ear diseases: radiological diagnosis based on clinical signs and symptoms Insights Imaging [online] , 3(1):33-48 [viewed 16 October 2014] Available from: doi:10.1007/s13244-011-0126-z