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Background: Sessile bacteria within biofilms are highly resistant to eradication by antimicrobial agents. Previously, we have shown that the most common organisms cultured from experimentally induced cholesteatomas are biofilm formers. Additionally, the keratin "matrix" of a cholesteatoma is an ideal environment for the support of biofilm formation., Objective: To determine if microbial biofilms occur within the keratin matrix of infected cholesteatomas., Design: We evaluated the histomorphologic characteristics of 24 human and 22 experimental cholesteatomas for evidence of biofilm formation using light and transmission electron microscopy., Subjects: Human tissues were collected during surgical eradication of existing cholesteatomas. Twenty-two gerbil cholesteatomas were either spontaneously occurring or induced by external auditory canal ligation and harvested several months later., Results: Gram-positive and gram-negative bacteria were seen within acellular deposits among the keratin accumulations in 21 of 22 gerbil and 16 of 24 human cholesteatomas. Regions of accumulated bacteria possessed the ultrastructural appearance of typical amorphous polysaccharide biofilm matrix., Conclusions: There is strong anatomic evidence for the presence of bacterial biofilms in experimental and human cholesteatomas. The existence of bacterial biofilms within cholesteatomas may explain the clinical characteristics of infected cholesteatomas, that is, persistence and recurrence of infection, with surgical eradication being the only effective treatment.

Objectives: To assess whether a classification system for congenital cholesteatoma (CC) can be derived from analysis of a large clinical sample of cases and to assess whether such a classification system is a reliable guide for surgical intervention, reexploration, and hearing outcome., Design: A retrospective review of clinical and surgical records of 119 patients with CC., Setting: Four tertiary care children's hospitals., Patients: One hundred nineteen children with CC (age range, 2-14 years)., Results: Congenital cholesteatomas in the anterior mesotympanum were treated successfully with exploratory tympanotomy. Congenital cholesteatomas involving the posterior superior quadrant and the attic usually had concurrent involvement of the incus and stapes and often required a canal wall up tympanomastoidectomy and a second look for its control. Congenital cholesteatoma involving the mastoid usually involved all of the ossicles, was inconsistently controlled with canal wall up tympanomastoidectomy, and had a poor prognosis for restoration of conductive hearing loss. The mean +/- SD age of children with CC was 5.6 +/- 2.8 years, while that of children with acquired cholesteatoma was 9.7 +/- 3.3 years., Conclusions: The sequence of spread of CC, involving 3 sites, suggests a natural classification system. The CC usually originates in the anterior superior quadrant, but does not consistently remain there, and may variably occupy the middle ear and mastoid and result in ossicular destruction and conductive hearing loss. The location of CC and the involvement of the ossicles is an accurate predictor of the type of surgery necessary for its control and for the success of hearing restoration.

Objectives: To describe the natural history of congenital cholesteatoma (CC) and to determine whether such a description provides clues about the origins and end points of these lesions., Design: A retrospective qualitative analysis of intraoperative illustrations of 34 consecutive patients with 35 CCs (1 bilateral)., Setting: Two tertiary care children's hospitals., Patients: Thirty-four children with CC, mean age, 5.6 years (range, 2-13 years)., Results: Congenital cholesteatoma originates generally, but not universally, in the anterior superior quadrant. The progression of growth is toward the posterior superior quadrant and attic and then into the mastoid. Contact with the ossicular chain generally results in loss of ossicular continuity and in conductive hearing loss., Conclusions: Congenital cholesteatoma appears to have a predictable trajectory of growth, starting as a small pearl in the middle ear, eventually growing to involve the ossicles and mastoid, and causing varying degrees of destruction and functional impairment. The clinical picture of a young child with otorrhea, conductive hearing loss, tympanic membrane perforation in a nontraditional location, and a mastoid filled with cholesteatoma may represent the end point in the natural history of CC, despite the fact that this type of lesion is outside the accepted definition of CC.

Objective: To analyze immunocompetent cells as well as 2 factors involved in inflammation and also thought to be involved in bone remodeling-interleukin 6 (IL-6) and inducible nitric oxide synthase in the human middle ear, including the tympanic membrane., Design: Biopsy specimens were obtained from the human middle ear and tympanic membrane during surgery. Using an immunohistochemical technique, the expression of macrophages, T cells, B cells, IL-6, and inducible nitric oxide synthase were analyzed., Materials: Nine biopsy specimens from tympanic membranes in children having a transtympanic ventilation tube inserted as a treatment for secretory otitis media and 11 biopsy specimens from tympanosclerotic plaques from patients with chronic otitis media and tympanosclerosis., Results: More positively stained specimens showing macrophages, B cells, and IL-6 were seen in the biopsy specimens from children with secretory otitis media compared with the biopsy specimens from patients with chronic otitis media and tympanosclerosis. The biopsy specimens from patients with chronic otitis media and tympanosclerosis more often showed positive stainings for inducible nitric oxide synthase than the biopsy specimens from children with secretory otitis media. The presence of IL-6 and inducible nitric oxide synthase was shown by staining to be mostly in the surface cells, while macrophages and B cells were stained deeper in the tissues, in connective tissue, or around sclerotic lesions., Conclusions: The 2 patient groups differed in antigen presentation so that macrophages, B cells, and IL-6 were labeled more frequently in patients with secretory otitis media, that is, an early phase of the disease. Inducible nitric oxide synthase was seen more frequently in the patients with already established tympanosclerosis in a later phase of the disease.

Objective: To assess whether a correlation exists between the degree of pars flaccida (PF) retraction and the degree of mastoid pneumatization., Design: The degree of PF retraction was defined by means of an operating microscope and a pneumatic otoscope. Degree of mastoid pneumatization was assessed planimetrically, using mastoid x-rays., Setting: Private otologic clinic., Participants: A total of 595 ears, with intact pars tensa, of 332 adult patients., Results: The degree of PF retraction was found to be inversely correlated to the level of mastoid pneumatization. Poorly pneumatized mastoids were associated with PF retractions. The poorer the pneumatization, the deeper the retraction. Well-pneumatized mastoids were associated with normal position of the PF., Conclusions: This study lends further support to the possibility that the mastoid pneumatic system functions as a middle ear pressure buffer. This possibility gives further explanation as to why ears with poorly pneumatized mastoids tend to develop tympanic membrane retractions and perforations, incus necrosis, or retraction pocket cholesteatoma, while ears with a large pneumatic system are rarely at such risk.

Objective: To understand the interaction between cholesteatoma epithelium and subepithelial connective tissue as a paracrine regulation by keratinocyte growth factor., Design: Preparation of a specific DNA probe from human fetal fibroblast and detection of localization of keratinocyte growth factor messenger RNA in subepithelial granulation tissue of middle-ear cholesteatoma by a nonradioactive in situ hybridization method., Participants: The cholesteatoma specimens were excised from 12 patients during surgery. Normal skin specimens collected from the external ear canal of six patients were used as controls., Results: Signals specific for keratinocyte growth factor messenger RNA were not expressed in the normal skin of the external ear canal, but were observed in fibroblasts of subepithelial connective tissue of cholesteatoma specimens. Signals were observed only in specimens collected from patients whose subepithelial connective tissue was thick and proliferated and whose inflammation was strong., Conclusions: A paracrine regulation mechanism involving keratinocyte growth factor may exist for proliferation of epithelium of cholesteatoma. The subepithelial connective tissue of cholesteatoma may play an important role in the proliferation and development of the cholesteatoma, especially under inflammatory conditions.

We describe three cases of a fibroinflammatory pseudotumor (tumefactive fibroinflammatory lesion) of the middle and inner ear. The patients presented with total deafness in the affected ear and no response to caloric stimulation. The computed tomographic pattern showed destruction of inner ear structures and a typical widening of parts of the labyrinth. Magnetic resonance imaging performed in all three patients showed an extension greater than expected based on computed tomographic images of both areas of destruction, as well as areas of radiologic normality. An enhancing mass was seen in the inner ear with a characteristic extension into both the internal auditory canal and the middle ear. A transotic approach or subtotal petrosectomy was used to remove the tumor in all three cases. Although histologically benign, these tumors are locally destructive and, as such, behave like a neoplastic lesion. They are composed of fibrovascular tissue admixed with chronic inflammatory cells. To our knowledge, this is the first report on pseudotumors of the middle ear, inner ear, and internal auditory canal. Inflammatory pseudotumor used to be a somewhat confusing term for a recognized entity of unknown origin. It is likely that infection is an important contributing factor in the development of these lesions. Although surgical removal seems to be the treatment of choice, no clear judgment of its prognosis can be made owing to the rarity of this tumor.

Adenomas, although the most common benign tumors in the middle ear, are of low incidence. They may arise in a previously normal adult ear, causing a conductive deafness and later an aural discharge. Histologic definition may be difficult in these tumors, which are assumed to arise from middle ear epithelium. Fairly extensive surgery may be required for their eradication. Two cases are described.

Objective: To determine the behavior of epidermal cells after transplantation in the middle ear., Design: In a rat model, full-thickness meatal skin grafts were transplanted into the middle ear and studied morphologically and immunohistochemically with the use of antibodies directed against different cytokeratin (Ck) polypeptides, which are markers of different types of epithelial cell differentiation., Results: The grafts had either transformed into epithelial cysts or had become integrated into the middle ear epithelium. The epithelium of the integrated grafts showed gradual transition into the epithelium of the middle ear. A clear distinction between epidermal cells and middle ear epithelium could be made only on the basis of their Ck profiles. The Ck profiles of the grafts revealed a decrease in the expression of epidermal Cks, while nonepidermal Cks became expressed. These changes can be ascribed to replacement of the dermal mesenchyma by mesenchyma from the middle ear. In two ears with superimposed infection, the graft epithelium showed expansive growth., Conclusions: Meatal epidermis is well tolerated in the middle ear, but superimposed infection can induce expansive growth. These findings favor the concept that the progressive growth of cholesteatoma is related to the presence of inflammatory processes.

Velocardiofacial syndrome is the most common syndrome associated with clefting of the secondary palate. The endoscopically diagnosed occult submucous cleft palate is the most common palate anomaly associated with this syndrome. Patients with velocardiofacial syndrome present a special challenge to the otolaryngologist, who must be familiar with the diagnostic and therapeutic aspects of this syndrome. We report the findings in 21 patients with velocardiofacial syndrome. Only 11 (52%) had the typical manifestations, and others had only partial phenotype. Adenoidectomy must be avoided in these patients, since undiagnosed occult and overt submucous cleft palate are the most common cause of velopharyngeal insufficiency after adenoidectomy. Three patients had aberrant carotid arteries. This anomaly must be excluded by nasoscopy and computed tomographic scanning before pharyngeal flap surgery is considered in these patients. Criteria for exclusion of high-risk patients from adenoidectomy are presented and discussed.

Using light microscopy, we studied the morphologic events of cholesteatoma progression 8 months after ligation of the ear canal in 14 Mongolian gerbils. Although we observed a wide range of progression, four stages of morphologic development could be clearly distinguished among the 28 specimens: stage I, an orthokeratotic plug in the ear canal without tympanic membrane involvement; stage II, partial retraction of the tympanic membrane; stage III, the interface between cholesteatoma encapsulated within the tympanic membrane and mucosal membranes covering the promontorial wall of the middle-ear cavity was characterized by a buildup of granulation tissue with prominent macrophage infiltration; and stage IV, bone destruction. Chronic inflammation was observed in some but not all stage II to IV cases. Our results indicate that hyperkeratosis is a primary event in cholesteatoma development, that the development of the granulation tissue with activated macrophages may be responsible for the bone destruction, and that acute or chronic inflammation is not a prerequisite for the development of cholesteatoma or the bone resorptive process.

Eosinophilic granuloma is a localized form of histiocytosis X, or Langerhans' cell histiocytosis, a benign lesion of proliferating Langerhans' cells. It is the least severe of the histiocytosis syndromes, and is characterized by lytic lesions of one or more bones. Temporal bone lesions usually occur in association with multifocal disease; however, isolated lesions may occur in either the mastoid bone alone, or in the entire temporal bone, without disease elsewhere in the body. We present the first case (to our knowledge) of eosinophilic granuloma, or unifocal Langerhans' cell histiocytosis, limited to the petrous apex. The patient, an 8-year-old girl, presented with facial nerve paralysis. Because of delay in definitive diagnosis, the disease extended beyond its original boundaries and resulted in complete destruction of the temporal bone. We will also review Langerhans' cell histiocytosis, with attention to its involvement of the temporal bone.

The aggressive nature of childhood cholesteatoma has generated much controversy regarding the optimal management of this challenging disorder. To identify potential predictors of residual-recurrent disease, we studied 232 children (244 ears) treated with 427 surgical procedures between 1973 and 1990. Cause of the primary cholesteatoma was congenital in 43 patients (18%), acquired in 83 (36%), and unknown in 106 (46%). Of 90 patients with residual-recurrent disease, 21 (23%) instances were detected during a second-look surgical exploration. The 3- and 5-year residual-recurrence rates were 48% and 57%, respectively. Development of residual-recurrent disease was associated with ossicular erosion (Cox regression) but not with status of the canal wall (up vs down) during tympanomastoidectomy. This study suggests a need for intense and prolonged postoperative follow-up of children with cholesteatoma, especially those with ossicular erosion.

We treated a patient with pseudocyst of the auricle and reviewed the 113 cases previously published in the world literature. Pseudocyst of the auricle is an asymptomatic, noninflammatory cystic swelling that involves the anthelix of the ear, results from an accumulation of fluid within an unlined intracartilaginous cavity, and occurs predominantly in men (93% of patients). Characteristically, only one ear is involved (87% of patients), and the lesion is usually located within the scaphoid or triangular fossa of the anthelix. Previous trauma to the involved ear is uncommon. The diagnosis may be suggested by the clinical features, and analysis of the aspirated cystic fluid and/or histologic examination of a lesional biopsy specimen will confirm the diagnosis. Therapeutic intervention that maintains the architecture of the patient's external ear should be used in the treatment of this benign condition.

The human temporal bones of five drowning victims, the largest such series, to our knowledge, were evaluated to determine what histopathologic changes occurred. Thickening of the periosteal epithelium, especially on the surgical dome of the otic capsule, was evident in all cases. There was also hemorrhage in the middle ear cavity in four of the cases. In the fifth case, a cholesteatoma and ruptured tympanic membrane were observed, but there was no evidence of hemorrhage. It is proposed that an intact tympanic membrane is needed to create sufficient negative pressure in the middle ear cavity to cause rupture of the blood vessels and hemorrhage. Such bleeding is indicative of drowning when the tympanic membrane is intact.

Multifocal eosinophilic granuloma (Hand-Schüller-Christian disease) confined to soft tissues in the head and neck region is described in a man, 32 years of age. The lesions were detected at varying time intervals within a period of 14 months. They were located in the gingivae, the submaxillary salivary glands, the thyroid gland, and in the dermal lining of the external auditory meatus. Clinical, histologic, ultrastructural, and immunohistochemical findings are presented and discussed. Some of these findings go along with the tenet that the typical proliferating histiocyte in eosinophilic granuloma is a pathologic Langerhans' cell, or a close kindred to it.

A mother and daughter both presented at age 5 years with the triad of right-sided congenital cholesteatoma, right preauricular pits, and bilateral sensorineural hearing loss. Twenty-six years apart, both were treated with middle ear exploration and removal of a cholesteatoma that filled the sinus tympani, facial recess, and middle ear. The sensorineural hearing losses were nonprogressive, and the preauricular pits were asymptomatic. These two cases may represent a unique variant of branchio-oto dysplasia. The mechanism of formation of these anomalies and the possible modes of inheritance are conjectural. This triad, however, supports genetic predisposition rather than aberrant epithelial rests during morphogenesis as a possible cause in congenital cholesteatoma.

Children with secretory otitis who, during the period from 1970 through 1975, had been treated with adenoidectomy and insertion of ventilating tubes were reexamined three to eight years after treatment and, again, ten to 16 years after treatment. The changes of the tympanic membranes were analyzed with regard to the occurrence of attic retractions and changes of the pars tensa of the membranae tympani. At the first follow-up examination, varying degrees of attic retractions were found in 37% of ears and, at the second follow-up examination, in 27% of ears. This general improvement was mainly due to normalization of the slight retractions, whereas the frequency of severe retractions increased. Pathology of the pars tensa of the membranae tympani was found in 57% of ears at the first examination and comprised the following: tympanosclerosis (19%), tympanosclerosis and atrophy (10%), atrophy (22%), atrophy and pexi (3%), and adhesive otitis (3%). At the second follow-up examination, pathology of the pars tensa of the membranae tympani occurred in 58.05% of ears and was distributed as follows: tympanosclerosis (26%), atrophy and tympanosclerosis (11%), atrophy (14%), atrophy and pexi (5%), adhesive otitis (2%), and sinus cholesteatoma (0.5%).