Re: Otology versus Otolaryngology

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I will post this informative article written by my surgeon. If you have any questions feel free to ask as some of it is technical.Cholesteatoma: recognition and management American Family Physician, June, 1991 by X. McKennanCholesteatomas are one of the most common serious problems of the middle ear and mastoid in both children and adults. A cholesteatoma is a collection of viable and desquamated squamous epithelium in the middle ear or mastoid air spaces, which are normally lined only by mucous membrane. It often develops into an expansile mass filled with desquamative skin debris. As desquamation

occurs, the cholesteatoma slowly enlarges. The lining of the cholesteatoma has the capacity to dissolve bone, resulting in destruction of the ossicles, mastoid air cells, semicircular canals, wall of the ear canal, cochlea and even the protective bony barriers between the ear and the middle and posterior cranial fossae. All of these middle ear structures may be damaged if cholesteatoma is not recognized and treated early. Timely surgical excision is necessary to avoid permanent conductive or sensorineural hearing loss, erosion into the semicircular canals, compression and paralysis of the facial nerve, and intracranial complications, such as brain abscess and meningitis. Etiology There are two types of cholesteatoma: congenital and acquired. Congenital cholesteatoma is due to an error in embryogenesis. Squamous epithelium, normally present only in the lining of the ear canal,

becomes trapped behind the tympanic membrane, which leads to the development of a progressively enlarging epithelial mass. There is no known preventive treatment. Congenital cholesteatoma is most commonly detected when children are two to eight years of age. This form of cholesteatoma is rare. Acquired cholesteatoma is secondary to inadequate eustachian tube function., Poor eustachian tube function results in inadequate middle ear ventilation, delayed clearance of infection and prolonged effusion, setting the stage for recurrent otitis media and progressive retraction of the tympanic membrane. Severe tympanic membrane retraction or perforation permits the ingrowth of squamous epithelium into the middle ear, forming a cholesteatoma. The vast majority of cholesteatomas are of the acquired type. Many cases can be prevented with proper treatment of eustachian tube dysfunction and secondary middle ear infection. Symptoms Symptoms of cholesteatoma

develop insidiously over weeks, months or years, and patients may neglect or minimize their symptoms. The symptoms are nonspecific, occurring with many other common ear problems. Ear pain or a sense of irritation deep within the ear may occur with cholesteatoma, but pain is common with many other ear problems and is not a useful signal, unless it becomes severe or persistent. Another possible symptom is hearing loss, which also may be the result of many other conditions, including cerumen excess, middle ear effusion and otitis media. Hearing loss is considered a warning symptom of cholesteatoma only when it is longstanding, moderately severe or unilateral. Vertigo and dizziness, potentially serious symptoms, are also nonspecific. The most telltale symptom of cholesteatoma is foul-smelling otorrhea. The patient frequently reports that there is an odor in the ear. The physician should never ignore this symptom. Signs Some cholesteatomas

are obvious, while others are difficult to detect without careful examination by means of binocular otomicroscopy. The physical signs of cholesteatoma depend on whether it is congenital or acquired. Congenital cholesteatomas most commonly appear as a pearly spherical mass behind the tympanic membrane. In the early stages, there is no associated infection or hearing loss. As growth of the mass progresses, the middle ear and mastoid are gradually destroyed. The tympanic membrane may bulge outward from the pressure of the cholesteatoma, giving the appearance of tympanosclerosis (a much more common calcific scarring of the tympanic membrane) or mucoid otitis media. Acquired cholesteatomas develop either from progressive retraction of the tympanic membrane (retraction cholesteatoma) or from ingrowth of squamous epithelium through a perforation (perforation related cholesteatoma). Retraction cholesteatomas develop most often in the pars flaccida or the

posterosuperior aspect of the pars tensa. Those that originate in the pars flaccida portion of the tympanic membrane are called attic cholesteatomas. (The term "attic" refers to the uppermost section of the middle ear.) Often, retraction cholesteatomas are obscured by crust formation or by keratin debris. Since this type of cholesteatoma originates from the nonvibratory portion of the tympanic membrane (pars flaccida), hearing loss from ossicular erosion is often a late and permanent complication. Attic cholesteatomas may be overlooked unless a careful examination of the pars flaccida is performed. Perforation-related cholesteatomas result from inward migration of squamous epithelium through a perforation. They may appear as a pearly mass behind the tympanic membrane or as skin (squamous epithelium) within the middle ear that has replaced the normal mucous membrane. Perforation-related cholesteatomas are often associated with conductive hearing impairment from loss of

the vibrating surface area of the tympanic membrane and ossicular erosion. Cholesteatomas of this type tend to be detected earlier than retraction cholesteatomas because the symptoms are more significant and the pars tensa is more easily visualized than the pars flaccida. Diagnostic Tests Diagnosis of cholesteatoma depends on a careful otoscopic examination. While no tests are available to confirm the diagnosis, several are helpful in evaluating the extent of the cholesteatoma and in documenting the amount of damage preoperatively. Once cholesteatoma is diagnosed, audiograms should be performed periodically. Testing can be started in children over 18 months of age; the older the child, the more reliable and extensive the information about hearing. Conductive hearing loss can usually be differentiated from sensorineural hearing loss in children as young as two to five years of age. Culture and sensitivity studies of exudate from the

middle ear or ear canal usually reveal a variety of gram-positive and gram-negative organisms. The most common pathogen is Pseudomonas aeruginosa. Appropriate oral and topical antibiotic therapy should be instituted to reduce or eliminate secondary infection. Radiographic imaging of the temporal bone with either mastoid films or computed tomography provides additional information but is not routinely necessary. Surgical Treatment If perforation is present, the ear canal and middle ear must be meticulously cleansed to reduce the amount of suppuration and to clarify the anatomy before surgery. Long-term medical management should include treatment of any other condition that may exacerbate eustachian tube dysfunction, such as allergies, chronic sinusitis and adenotonsillar enlargement or infection. Cholesteatomas must be surgically excised since they progressively enlarge and destroy the middle and inner ear. Removal of the

cholesteatoma from the middle ear with reconstruction of the tympanic membrane (tympanoplasty) is the most commonly performed procedure. Mastoidectomy is frequently necessary for adequate removal of the cholesteatoma and any associated infection. Technologic advances, such as microdrills, the argon laser and artificial ossicles (plastic, calcium crystal or metallic replacements), have significantly improved the success of surgery. For most of the twentieth century, a radical or modified radical mastoidectomy was the procedure used to eradicate cholesteatoma. This procedure was nearly always successful, but often resulted in severe conductive hearing loss and necessitated yearly cleaning of the mastoid bowl. As a result, intact canal wall mastoidectomy has become popular during the past 15 years. With this technique, excision of the cholesteatoma is accomplished with preservation of the ear canal wall; tympanoplasty and ossicular chain reconstruction are then

performed. If the intact canal wall technique is used, a second operation is usually necessary to explore the middle ear and mastoid cavity, making sure that part of the cholesteatoma is not buried beneath the reconstructed tympanic membrane or behind the wall of the ear canal. Usually, the second operation is performed six to 18 months after the first operation. Simultaneously, allographic ossicles can be inserted to reduce the conductive hearing loss. Prognosis The long-term outcome depends on the extent of disease at the time of surgery and the success of treating any conditions that impair ventilation of the middle ear or mastoid spaces. Many patients have normal ear function decades after surgery. For others, it is difficult to prevent recurrent cholesteatoma formation, infection and hearing loss. In these more difficult cases, multiple operations are sometimes necessary.

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