Middle ear cholesteatomas (MEC) initially present with mild symptoms, which become more pronounced with increasing cyst size. An early stage MEC may manifest with foul-smelling otorrhea. Patients may feel an increased feeling of pressure in the affected ear, which can occasionally lead to painful sensations in or behind the ear. The pressure and exerted by the MEC can also be conducive to an inflammatory response including the formation of granulation tissue because of an infection (acute or chronic otitis media [1] [2]) and result in progressive hearing loss, in particular around the frequency of 2 kHz [3].

If left untreated, the growing MEC will likely perforate the tympanic membrane, damage the vicinal middle ear bone structure and also present with complications like chronic ear swelling, facial palsy [4], sometimes dizziness due to the development of a labyrinthine fistula, ossicular damage, meningitis, sigmoid sinus thrombosis or intracranial abscess [5] [6] [7].

Risk factors for MEC development are recurring middle ear infections, allergies, frequent exposure to cigarette smoke, eustachian tube malformations or cleft palate [8]. MECs do not have blood supply and administration of topical antibiotics will not ease otorrhea symptoms.

• Inflammation

  OBJECTIVE: To evaluate the expression of type 2 TNF-α receptor (TNF-R2) in fragments of cholesteatoma and correlate it to the degree of inflammation present. [ncbi.nlm.nih.gov]

• Weakness

  Tumor necrosis factor (TNF)-like weak inducer of apoptosis (TWEAK) was recently identified as a member of the TNF superfamily of structurally related cytokines. It regulates TNF-α and numerous cellular responses. [ncbi.nlm.nih.gov]

  Finally, we emphasize that there is evidence to indicate that a weakness in the innate antimicrobial defense system of the human external ear skin may make a decisive contribution in the development cholesteatomas. [degruyter.com]

  In extreme cases, symptoms such as facial weakness / paralysis, dizziness, complete deafness and neurological symptoms occur. [californiaearinstitute.com]

  However, as with any type of surgery, there's a small risk associated with having anaesthetic, and a very small chance of facial nerve damage resulting in weakness of the side of the face. [nhs.uk]

• Swelling

  Cite this paper Bruzzi, C., Negri, M., Casale, M., Frari, V., Salvinelli, F. and Galli, S. (2014) A Rare Middle Ear Cholesteatoma Complication behind Painless Swelling of the Temporozygomatic Region. [scirp.org]

  Mastoiditis has been considered as an extracranial complication of otitis media when patients develop tender post-auricular swelling. [annexpublishers.co]

  It is not unusual to have some diffuse swelling in the region of the incision and even the side of the face slightly anterior to the ear. Any severe swelling or “goose egg swelling” should be reported to your doctor. [owensearcenter.com]

  This can cause pus-filled swelling in your brain or meningitis. Both are very rare. Causes A cholesteatoma can happen for several reasons: You get an ear infection or injury. [webmd.com]

  If left untreated, the growing MEC will likely perforate the tympanic membrane, damage the vicinal middle ear bone structure and also present with complications like chronic ear swelling, facial palsy, sometimes dizziness due to the development of a labyrinthine [symptoma.com]

• Chronic Infection

  Cholesteatoma can lead to more serious problems including chronic ear infections, permanent hearing loss and dizziness. [californiaearinstitute.com]

  It may not work properly due to any of the following: chronic ear infections sinus infections colds allergies If your eustachian tube isn’t working correctly, a partial vacuum might occur in your middle ear. [healthline.com]

• Tinnitus

  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]. [explainmedicine.com]

  Learn More About Topic Tinnitus Over 50 million Americans have experienced tinnitus, or ringing in ears, which is the perception of sound without an external source being present. [entnet.org]

  […] and headacheC Vertigo. tinnitus hearing loss and headacheD Vertigo tinnitus and hearing loss 55) Motion sickness cannot be prevented by:A PromethazineB AstemizoleC HyoscineD Buclizine 56) Eustachian tube opens into middle ear cavity at which of the following [gradestack.com]

  Common symptoms include: hearing loss, discharge from the ear with a strong odor, bleeding from the ear, dizziness, vertigo, balance disruption, ear ache, headaches, and tinnitus. [acadianent.com]

• Otalgia

  The other patient, a 55-year-old man, was admitted to our hospital for a detailed examination because he had right otalgia and progressive headache. [ncbi.nlm.nih.gov]

  Vertiginous syndromes in diseases classified elsewhere H83 내이의 기타 질환 Other diseases of inner ear 귀의 기타 장애(H90-H95) Other disorders of ear H90 전음성 및 감각신경성 쳥력손실 Conductive and sensorineural hearing loss H91 기타 청력손실 Other hearing loss H92 귀통증 및 귀의 삼출액 Otalgia [rich-world.tistory.com]

  Otalgia Cholesteatoma itself is not often painful. But when it is associated with infection, it may cause pain.[2]. [explainmedicine.com]

  Case report A 40-year-old male, was admitted to ENT Department of the 20 Aout hospital, Ibn Rochd university hospital, with large mastoiditis of the right ear, otalgia, fever, severe headache and nausea. [annexpublishers.co]

• Hearing Impairment

  CONCLUSION: Acquired middle ear cholesteatoma is associated with significant hearing impairment, although profound hearing loss is rare. Adults have greater AC and BC thresholds than those in children but similar ABGs to children. [ncbi.nlm.nih.gov]

  Thus, the sound is no longer transmitted adequately to the inner ear, resulting in a hearing impairment. Treatment If one wants to achieve complete healing of a cholesteatoma, the only option is surgery. [hear.com]

  He also had intermittent otorrhea, hearing impairment from right ear with mild dizziness for the past two years. The patient denied any history of diabetes mellitus, hypertension, or pulmonary tuberculosis. [annexpublishers.co]

• Ear Discharge

  discharge, perforation or ear surgery Congenital cholesteatomas occur at three important sites: the middle ear, the Petrous apex, and the cerebropontine angle. [en.wikipedia.org]

  The first symptom is a discharge from one ear. It is usually slightly watery, sometimes with a green or yellow colour. The discharge might be slightly smelly. [patient.info]

  […] with ear infection with foul smelling discharge. [gradestack.com]

• Hearing Problem

  After a while you may get hearing problems in that ear. If the cholesteatoma is left untreated it can spread into the balance centres of the inner ear, causing dizziness. [patient.info]

  Resulting hearing problems are due to conductive hearing loss Conduct : to channel, lead or convey from one point to another. [successforkidswithhearingloss.com]

• Dizziness

  Abstract Middle ear cholesteatoma caused by repeated ear infections over time, destroys the delicate middle ear bones and causes permanent hearing loss or dizziness. It may grow to involve the facial nerve causing facial paralysis. [ncbi.nlm.nih.gov]

  Erosion of the cholesteatoma into the inner ear can be recognized by dizziness induced by pressure, provoked by the " Valsalva " test, or dizziness induced by sound provoked by the "Tullio" test. These are very specific findings. [dizziness-and-balance.com]

  Symptoms of a cholesteatoma include: Persistent ear infection in one ear Pain or numbness in the ear or around the ear Drainage from the ear Dizziness or vertigo Diagnosis Diagnosing cholesteatoma requires careful examination supplemented by advanced [hopkinsmedicine.org]

  Dizziness: Some people experience dizziness that resolves within a day of surgery. It is not likely for dizziness to be a persistent problem. Facial paralysis: The nerve that innervates the muscles of the face courses through the ear. [marshfieldclinic.org]

  ENT specialists treat conditions such as ear infection, hearing loss, dizziness, ringing in the ears (called tinnitus), ear, face, or neck pain, and more. [entnet.org]

• Meningism

  Neither meningitis nor recurrence was observed in any patient during the follow-up periods (mean 29.4 months, range 6-64 months). [ncbi.nlm.nih.gov]

  There were no signs of meningeal irritation and fundus examination was normal. [annexpublishers.co]

  Severe intracranial complications like meningitis have become less fatal due to earlier recognition and more efficient surgical interventions. [symptoma.com]

  PubMed Source: http://www.ncbi.nlm.nih.gov/pubmed/28494066 DOI: http://dx.doi.org/10.1001/jamaoto.2017.0130 Medical and Biotech [MESH] Definitions Cholesteatoma A non-neoplastic mass of keratin-producing squamous EPITHELIUM, frequently occurring in the MENINGES [bioportfolio.com]

• Vertigo

  Headache and vertigo Disease may rarely causes vertigo.[1].Some patients may also present with dizziness and headache.[6]. [explainmedicine.com]

  Benign Paroxysmal Positional Vertigo (BPPV) Benign paroxysmal positional vertigo, or BPPV, is the most common inner ear problem and cause of vertigo, or false sense of spinning. [entnet.org]

  […] ear discharge, tinnitus and headacheC Vertigo. tinnitus hearing loss and headacheD Vertigo tinnitus and hearing loss 55) Motion sickness cannot be prevented by:A PromethazineB AstemizoleC HyoscineD Buclizine 56) Eustachian tube opens into middle ear [gradestack.com]

  SIGNS AND SYMPTOMS: Early symptoms of cholesteatoma include hearing loss, mucopurulent (brown/yellow) drainage from the ear, ear bleeding, ear pain, tinnitus and vertigo. [dallasear.com]

• Nystagmus

  If facial paralysis or a nystagmus (eye twitch, in the event of infection of the inner ear, specifically the balance organ) occurs, surgery is imperative. [hear.com]

  […] diagnosed to have acute otitis mediaC Xylitol is commonly used sweetener inhibits the growth of pneumococcal growthD Ventilation tube insertion the incidence by 50%. 100) 30 year female came to OPD with c/o feeling of giddiness with movements of head with nystagmus [gradestack.com]

• Hyperactivity

  This is a rare type of mydriasis that occurs especially in patients with migraines, parasympathetic deficits or sympathetic hyperactivity in the iris. [areaoftalmologica.com]

  A study of that tracked pupil sizes among children with attention deficit hyperactivity disorder (ADHD) reported in 2017 that their pupils enlarged when they were concentrating on memory-related tasks. [diamondvision.com]

MEC diagnosis relies on an otoscopic examination of the patient's ear as a first attempt to rationalize presented symptoms. Laboratory tests or histological investigations are usually not necessary, but may be ordered to corroborate diagnosis. Ear drainage may, however, be analyzed for potential middle ear infections, which can present as a complication of MEC growth, particularly if the tympanic membrane has been damaged. Histological findings of extracted MEC tissue specimens will yield typical squamous epithelial cell results indistinguishable from keratomas. Audiometric tests may also be taken into consideration in more complicated cases with significant hearing loss, especially before imminent surgery. Air and bone conduction as well as speech reception measured should be determined. In urgent cases presenting with severe complications, audiometry should be omitted in favor of an immediate surgical intervention [9].

The method of choice for a reliable diagnosis are computed tomography (CT) and nuclear magnetic resonance (MRI) scans. CT investigations will accurately resolve potential expansion of the aditus and the mastoid antrum or ossicle erosion as well as generic bone erosion in the middle ear structure and its vicinity, while MRI procedures will be able to localize the the MEC and assess its extension into the middle ear cavity. CT procedures may be inappropriate to distinguish between MECs and granulation tissues [9] [10] [11] [12].

The only effective means to treat MECs is their physical removal in the context of a surgical procedure. In case of a diagnosed inflammatory response as a complication MEC formation, prescription of topical antimicrobial agents is strongly advised prior to surgery. High pre-operative middle ear hygiene standards may slow down MEC growth.

Surgeons may have to decide between a canal wall-up (closed) and a canal wall-down (open) mastoidectomy. Canal wall-down operations usually provide the best long-term healing chances for patients. Wall-up procedures provide a better cosmetic prognosis for the patient at the cost of an increased risk of future MEC regrowth. After surgical removal, it is strongly recommended to schedule a tympanomastoidectomy procedure as a control measure within twelve months after the first intervention [13] [14].

Congenital MEC is often associated with a malfunction of the eustachian tube. Whenever such a malfunction is diagnosed or suspected, a canal wall-down procedure may provide a better prognosis, although an intact-canal technique has also been shown to provide satisfying healing chances in congenital MEC patients [15].

All mastoid air cells should be removed independent of the chosen technique. In case of ossicle damage, ossicle removal is imperative [15] and requires an ossicle reconstruction procedure. The tympanic membrane also needs to be reconstructed. Cartilage grafts or a meatoplasty may be required to recover a well-ventilated and structurally intact middle ear cavity.

In case of diagnosed hearing loss, use of hearing aids may be advisable.

Post-operative healing perspectives for patients are usually good, provided MECs were removed in an early stage. Patients may suffer from symptoms like temporary dizziness or distorted taste sensations in the immediate aftermath of the procedure. If the removed MEC was large, irreversible but moderate hearing loss, balance problems and vertigo may persist in rare cases [16].

A single MEC removal procedure need not be sufficient. MEC recurrence probability has been estimated to be around five percent in canal wall-down procedures, whereas the canal wall-up technique offers a MEC regrowth chance of twenty up to forty percent [17].

Severe intracranial complications like meningitis have become less fatal due to earlier recognition and more efficient surgical interventions.

Repeated middle ear infections and an impaired middle ear ventilation due to a malformed eustachian tube are hallmark culprits for the formation of MECs. If the eustachian tube does not allow for proper air circulation in the middle ear, a negative pressure in the middle ear may lead to a permanently distorted tympanic membrane, which is at an increased risk to develop a cyst, which can turn into MECs.

Congenital MECs have been rationalized with an entrapment of squamous epithelial cells within the temporal bone during embryogenesis. Typical locations for congenital MECs are the anterior mesotympanum or the perieustachian tube area [18]. Congenital MECs can also present behind intact tympanic membranes [19] [20] [21].

Acquired MEC cases can develop as a consequence of a direct lesion of the tympanic membrane or of a retraction of the tympanic membrane into the middle ear cavity, which typically leads to ossicular damage [22].

MEC development is also a possible complication of tympanostopy tube insertion procedures [23] [24].

The mean annual incidence of MEC has been estimated to be 9.2 per 100 000 (range 3.7-13.9) in industrialized countries. MECs are very rare in patients which present with an intact tympanic membrane and without a history of otitis media, accounting for less than 1% of diagnosed cases. Patients presenting with cleft palates are at an increased risk, as are patients with recurrent otitis media episodes. Men who are younger than 50 seem to be a risk group. MEC diagnosis does not correlate with social status [22].

There is strong and accumulating evidence that the origin of the squamous epithelial cells extracted from histological examinations is the tympanic membrane, not the middle ear epithelium, as shown from different protein and cytokeratin profiles [6] [25] [26].

MEC formation is caused by a complex and not yet fully disclosed imbalance of cytokines, growth factors and bacterial toxins conducive to a recurrent scheme of cell proliferation, keratinocyte differentiation and keratinocyte apoptosis. Cytokines have been implicated to be involved in osteoclast activation and maturation, which can lead to the degradation of the extracellular bone matrix and result in the observation of severe MEC complications [27] [28].

Congenital MECs cannot be prevented. Early treatment is thus a major factor to avoid severe complications. Acquired MECs can be quite efficiently prevented by swift and successful treatments of middle ear infections.

Animal studies have revealed that administration of vitamin A and Cortisporin may be effective in stalling MEC growth [29].

Long-term monitoring of the patient is imperative to prevent MEC recurrence.

Middle ear cholesteatomas are non-cancerous and initially present with mild symptoms and painless otorrhea but can have lead to severe consequences ranging from permanent moderate hearing loss to meniningitis or intracranial abscess. MEC cannot be cured with antimicrobial agents. The only efficient treatment requires the surgical removal within a mastoidectomy procedure. This intervention provides excellent healing chances with a very little chance of recurrence. Patients should be advised to have their middle ear regularly examined for regrowth.

Middle ear cholesteatomas are cysts in the middle ear that must be removed as soon as possible to avoid serious complications like permanent hearing loss or brain-related diseases. Immediate consultation of a medical professional is advisable, if foul-smelling ear drainage occurs concomitant with an increased feeling of pressure in the ear. Doctors will first check for possible infections, may prescribe antibiotics, and schedule a surgical removal procedure. Regular control examinations are necessary to avoid cholesteatoma regrowth.

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