Histiocytosis X of the ear and temporal bone: review of 22 cases.

Histiocytosis X encompasses three syndromes characterized by the idiopathic proliferation of histiocytes: eosinophilic granuloma, Hand-Schuller-Christian syndrome, and Letterer-Siwe disease. At the Mayo Clinic between 1926 and 1978, 22 patients with histiocytosis X had involvement of the ear or temporal bone. These patients comprised 15% of all patients with histiocytosis X seen during that period. The ages at onset of the disease ranged from 2 months to 49 years. The most frequent otologic symptom was aural discharge (15 patients), followed in frequency by swelling in the temporal region (11 patients), vertigo (6 patients), and deafness (5 patients). Clinical findings included otitis media (13 patients), otitis externa with or without granulation tissue (10 patients), and osteolytic lesions of the temporal bone (9 patients). A high index of suspicion is required to recognize the otologic manifestations of histiocytosis X for two reasons: the systemic manifestations of the disease are often so dramatic that the ear findings are overlooked, and the otologic findings of histiocytosis X can mimic more common diseases, including simple otitis externa, aural polyps, acute mastoiditis, chronic otitis media, and metastatic lesions.     

Mechanisms of retraction pocket formation in the pediatric tympanic membrane.

The epidemiological nature of acquired cholesteatoma in children has shown that it occurs more often in the posterosuperior quadrant of the pars tensa and in the pars flaccida. This type of cholesteatoma is rarely seen before the age of 3 years, and serous otitis media is the most important risk factor for its occurrence. In an attempt to present a pathological rationale for these clinical findings, tympanic membranes from 11 temporal bones with purulent otitis media and 13 with serous otitis media were evaluated light microscopically and compared with 14 temporal bones without disease. Ages ranged from newborn to 3 years 6 months. The persistence of mesenchyme and greater inflammatory reaction observed in the pars flaccida and the posterosuperior quadrant of the pars tensa and changes in collagen and elastin observed in purulent otitis media and serous otitis media may represent a pathological rationale for the epidemiological nature of cholesteatoma in children.     

Otogenic intracranial infections in adults

OBJECTIVE: To assess the type and bacteriology of otologic diseases associated with bacterial meningitis in adults. METHOD: Retrospective review of 79 patients over an 18-year period. RESULTS: Acute otitis media was diagnosed in 32 patients, chronic otitis in 29 (16 with cholesteatoma), and cerebrospinal fluid leak in 18. Streptococcus pneumoniae was a common cause of meningitis-complicating acute otitis media (69%) or cerebrospinal fluid leak (50%), whereas other bacteria or negative cultures were found in the cerebrospinal fluid of patients with chronic otitis. Surgery was performed promptly in 26 patients; four patients died. CONCLUSIONS: Early diagnosis of otogenic bacterial meningitis is essential to allow appropriate antimicrobial treatment. Antimicrobials active on gram negative bacilli and anaerobes should be used in patients with chronic otitis. An emergency surgical procedure is required in patients whose neurologic or infectious status fails to improve under antimicrobial treatment.     

Otologic manifestations of the immotile-cilia syndrome

The immotile-cilia syndrome is caused by a hereditary, inborn, ultrastructural defect of the cilia, rendering them immotile or poorly motile and thereby abolishing mucociliary clearance. Five cases are presented and the otologic manifestations are described in some detail. The syndrome should be suspected in children who have a persistent secretory otitis media with recurring bouts of acute otitis media, and a perpetual cough with repeated episodes of bronchitis. A lobar atelectasis is a frequent finding. Half the cases also have situs inversus. The immotile-cilia syndrome is of special interest to the otologist, as it seems to throw some light on the pathogenesis of secretory otitis media.     

Lipoperoxidation in otorrhea of the middle ear as a marker of infection. Clinical application

If the generation of free oxygen radicals is a pathophysiological finding in middle-ear infections, as has been reported, demonstration of the oxidative consequences of free radicals in middle-ear exudates may be a quick, simple, objective and accessible test for quantifying the degree of mucosal injury. Therefore, samples of the discharge fluid from cases of acute otitis media (n = 17), cholesteatoma (n = 17), chronic discharge in the absence of cholesteatoma (n = 10), and secretory otitis media (n = 15) were tested using an enzymatic colorimetric assay for lipid peroxide levels due to superoxide and hydroxyl radicals during infection. Lipid peroxide levels in acute otitis media, cholesteatoma, and chronic discharge without cholesteatoma ranged from 575 to 650 nmol/mg of exudate, and was 67 nmol/mg in secretory otitis media, which were statistically significant findings. Infectious otitis media showed findings secondary to increased oxidative metabolism, but not secretory otitis media. Measurement of this oxidative metabolism could serve to monitor the evolution of episodes, as well as to detect relapse or reinfection, and to evaluate response to new therapeutic options for suppurative otitis, such as topical antioxidants.     

Mastoid Surgery in the Only Hearing Ear

Every otologic procedure carries the risk of damage to the inner ear. On the other hand, cholesteatoma and uncontrolled tubotympanic disease can lead to partial or total sensorineural loss, as well as intracranial complications. Our experience of the surgical management of eight patients with an only hearing ear is presented. There were no dead ears in this series and no significant worsening of inner ear function. The air conduction pure-tone average was worse in two patients without change in bone condition. Of the eight patients, six wear a hearing aid with success and two patients manage without an aid. The present small series confirms that patients at risk of anacusis from cholesteatoma or chronic suppurative otitis media can be offered, in experienced tertiary referral units, a safe method of stabilizing the ear and preserving useful hearing.     

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.     

Tympanoplasty, with or without mastoidectomy, is highly effective for treatment of chronic otitis media in children

CONCLUSION: The overall success rate of tympanoplasty, with or without mastoidectomy, in the treatment of chronic pediatric otitis media, was high and did not depend on patient age, the status of the contralateral ear, the inclusion or absence of surgical mastoidectomy, or the method of mastoidectomy (when this procedure was employed). Tympanoplasty may be expected to improve hearing in cases of chronic otitis media accompanied by perforation, but not in cases of cholesteatoma. OBJECTIVES: This study analyzed the clinical features of pediatric patents with chronic otitis media undergoing tympanoplasty, with or without mastoidectomy. Follow-up data were examined to determine the effectiveness of these procedures on the course of the patients' conditions. SUBJECTS AND METHODS: We retrospectively reviewed the medical records of 111 children (a total of 119 ears were treated from this group) aged 15 years or less, who underwent surgical treatment for pediatric chronic otitis media. The subjects were composed of children suffering from chronic otitis media with perforation (COMP) (63 ears), and patients presenting chronic otitis media with cholesteatoma (COMC) (56 ears). The mean follow-up period was 40 months. Preoperative and postoperative (at the final follow-up) audiometry and otologic examinations were performed. Data from postoperative otologic examinations and audiometric measurements were accompanied by examination of both the operative ear and the contralateral ear. Surgical success was defined as the presence of an intact tympanic membrane without perforation, retraction, or evidence of recurring cholesteatoma. RESULTS: The mean ages at the time of operation were 11.1+/-3.3 years for COMP patients and 9.7+/-3.0 years for COMC subjects. Surgical treatments for pediatric COMP and COMC patients included tympanoplasty only in 45 ears (38% of ears treated) and tympanoplasty with mastoidectomy in 74 ears (62%). Most of patients with COMC received tympanoplasty with mastoidectomy. No patient with COMP underwent canal wall-down mastoidectomy. Mean pre-operative air-bone gaps (ABGs) and post-operative ABGs were compared. Significant improvement in ABG was evident in the COMP group, but not in the COMC group. Surgical success rates at follow-up after 6 months and 12 months were 97% and 95%, respectively, in the COMP group. In the COMC patients, surgical success rates at follow-up after 6 months and 12 months were 98% and 93%. There were no significant relationships between surgical success rate and patient age, the status of the contralateral ear, or the extent of surgery.     

A new pathogenesis of mesotympanic (congenital) cholesteatoma

OBJECTIVES: To introduce a new, acquired pathogenetic theory of mesotympanic cholesteatoma behind an intact eardrum in children and to present some doubts on congenital pathogenesis. STUDY DESIGN: Literature review. METHODS: The incidence and origination of mesotympanic cholesteatoma in children were thoroughly analyzed in the world literature and correlated to the histopathological studies on human middle ear epithelia and to epidemiological studies on secretory otitis, tubal occlusion, and acute suppurative otitis media. RESULTS: The new, acquired theory is based on the fact that that the place of origin of the anterosuperior mesotympanic cholesteatoma is the area of the malleus handle and malleus neck, and of the posterosuperior cholesteatoma, the long process of the incus. During the common pathological conditions there is a great risk of retractions and adhesions of the eardrum to these ossicles. After subsequent loosening of the retracted eardrum some cells of the keratinized squamous epithelium may be left behind and become included into the tympanic cavity, eventually causing an inclusion cholesteatoma. Four basic mechanisms of inclusions are proposed and the presence of great dynamics in middle ear disease in children, with high incidence of tubal dysfunction, retractions, secretory otitis, and acute suppurative otitis, is documented, making the acquired pathogenesis probable. The place of origin does not fit with the congenital pathogenesis of epithelial formation localized on the lateral wall of the eustachian tube close to the annulus. The origination around the malleus and incus fits better with the proposed acquired pathogenesis. CONCLUSIONS: There are no definitive proofs for the acquired pathogenesis of the mesotympanic cholesteatoma, nor is there experimental research to prove or disprove it. Mesotympanic cholesteatoma, congenital cholesteatoma, acquired pathogenesis of mesotympanic cholesteatoma, cholesteatoma in children, cholesteatoma behind intact eardrum.     

Management of chronic otitis media in the only hearing ear

OBJECTIVE: Chronic ear surgery is difficult. The management of such a disease either with or without cholesteatoma and in an only hearing ear is particularly challenging. Consequences of disease or unintended outcomes of therapy can both result in patient lifestyle alterations of major proportion. This report offers a diagnostic and treatment plan for chronic otitis media with and without cholesteatoma in the only hearing ear. METHODS: More than 10,000 charts of patients with chronic otitis media were retrospectively reviewed. Twenty-seven charts of patients with chronic otitis media in an only hearing ear who underwent surgical treatment were identified. The patients were followed for an average time of 43 months. RESULTS: Overall, the chronic otitis media was well controlled, and there was no change in the average discrimination or hearing thresholds when comparing preoperative and postoperative results. CONCLUSIONS: Chronic otitis media with and without cholesteatoma in an only hearing ear can be treated successfully with hearing preservation. Canal wall down tympanomastoidectomy is performed in most cases. Manipulation of the ossicular chain is avoided, and cholesteatoma that lies over a potential fistula is exteriorized.     

The role of preoperative CT scan in patients with chronic otitis media

Chronic otitis media may be due to chronic mucosal disease or cholesteatoma. Differentiating the two is usually achieved by clinical examination. The computed tomography (CT) scan is the standard imaging technique for the temporal bone, but its exact role in the preoperative assessment of patients with chronic otitis media is controversial. In this retrospective study we compared preoperative CT results with operative findings in 50 patients who had scan between January 2003 and December 2007. We analyzed the clinical presentation and checked if CT scan confirmed or excluded the presence of cholesteatoma and if this was affected by previous surgery. We concluded that CT scan could not be relied on to differentiate cholesteatoma from chronic mucosal disease. It should be used selectively in the preoperative preparation only if complications of the disease suspected.     

Comparative study on adhesive otitis media and pars tensa cholesteatoma in children

Objective: Recurrent otitis media and persistent otitis media with effusion in early childhood may cause an atelectatic eardrum and adhesive otitis media, which sometimes progress to pars tensa cholesteatoma. When and how children with adhesive otitis media should be operated on remain controversial. Therefore, this study aimed to analyze the clinical characteristics of children with adhesive otitis media and pars tensa cholesteatoma, and to determine the risk factors of progression to cholesteatoma.Methods: Seventeen ears of 15 children with adhesive otitis media (adhesive group) and 14 ears of 13 children with pars tensa cholesteatoma (tensa cholesteatoma group) who underwent tympanoplasty were included in this study. We analyzed the following clinical characteristics of children in both groups: medical and life history, associated diseases, sites of the adhesion, and development and aeration of mastoid air cells as shown by temporal bone computed tomography.Results: Most of the children in both groups had a history of recurrent otitis media and/or persistent otitis media with effusion. They showed a male predominance and a frequent association of allergic rhinitis. The number of ears showing undeveloped mastoid air cells in the tensa cholesteatoma group was significantly larger than that in the adhesive otitis media group (P=0.0068). A lack of aeration of the middle ear, including the eustachian tube, was more frequently found in ears with pars tensa cholesteatoma than in ears with adhesive otitis media (P=0.0012). Using multivariate logistic regression, the presence of otorrhea (odds ratio [OR], 14.847; 95% confidence interval [CI], 0.834–264.184), total adhesion (OR, 28.550; 95% CI, 0.962–847.508), and undeveloped mastoid air cells (OR, 19.357; 95% CI, 1.022–366.589) were related to pars tensa cholesteatoma.Conclusion: Children with adhesive otitis media should be carefully followed up in the outpatient setting. Ears with poor mastoid development may develop pars tensa cholesteatoma. Additionally, ears with middle ear effusion, total adhesion, and the presence of otorrhea tend to be at risk of pars tensa cholesteatoma. Tympanoplasty or tympanostomy tube insertion should be considered for children with adhesive otitis media who have these risk factors to prevent progression to pars tensa cholesteatoma.     

Otopathology in cranial facial dysostosis

Thirty patients with Crouzon's disease were evaluated longitudinally for type, incidence, and progression of otopathologic changes. The spectrum of otopathologic manifestations included otitis media with effusion, tympanic membrane atelectasis, tympanic membrane perforation, cholesteatoma, and grade I microtia. The frequency of ear disease progressed from 37% during infancy to 62% in older patients. The degree and type of hearing loss were also analyzed. Radiographic cephalometric measurements of the nasopharynx and temporal bone illustrated growth disturbances of the nasopharyngeal dimensions that worsened with increasing age. Our findings suggest that patients with Crouzon's disease are at risk from birth for otitis media with effusion and its sequelae and that the consequent incidence and severity of otopathologic changes increase with age.     

Presence of osteoclasts in middle ear cholesteatoma: a study of undecalcified bone sections

Conclusions: Osteoclasts are unlikely to be involved in bone resorption in middle ear cholesteatoma.

Objective: The authors searched for osteoclasts in undecalcified bone sections in patients with middle ear cholesteatoma to determine whether and to what extent these cells are involved in this disease.

Methods: Twelve patients, eight men and four women, aged 30–87 years, who underwent tympanomastoidectomy were enrolled. Six patients had primary acquired middle ear cholesteatoma (cholesteatoma group) and the other six patients had other otologic diseases including otosclerosis, non-cholesteatomatous chronic otitis media, adhesive otitis media, perilymphatic fistula and ossicular malformation (control group). The scutum bone was collected during surgery, fixed with ethanol, stained with Villanueva bone stain, and embedded in methyl methacrylate. Five-micrometer-thick sections were prepared and examined under a polarizing microscope. Images were analyzed using a semiautomatic graphics system.

Results: No osteoclasts were seen in any of the samples in either group. To avoid the risk of under-estimating the presence of osteoclasts, the number of osteoclasts was considered to be <1 in each sample, and the osteoclast density was calculated. The osteoclast densities in both the cholesteatoma and control groups were significantly lower than the sex- and age-matched standard value of the normal iliac cortical bone (p?=?.028).     

角化细胞生长因子及受体在鼓膜穿孔邻近皮肤的研究

目的观察鼓膜穿孔部位邻近皮肤中角化细胞生长因子（keratinocyte growth factor,KGF）及其受体角化细胞生长因子受体（keratinocyte growth factor receptor,KGFR）的表达情况,分析其在慢性化脓性中耳炎不同转归中的作用。方法应用免疫组化SP染色方法和多媒体图像分析系统,观察20例继发性胆脂瘤型中耳炎鼓膜穿孔部位邻近皮肤、胆脂瘤上皮以及耳道深部正常皮肤的KGF和KGFR表达,并和20例非胆脂瘤型中耳炎鼓膜穿孔部位邻近皮肤作对比。结果胆脂瘤型中耳炎鼓膜穿孔邻近皮肤KGF和KGFR的阳性表达率分别为（33.135±6.364）%和（19.965±10.570）%,介于胆脂瘤上皮与耳道正常皮肤之间,明显高于非胆脂瘤型中耳炎患者相应部位（19.380±2.827）%和（13.145±7.935）%。结论KGF和KGFR在慢性化脓性中耳炎不同部位的表达依次上升,在胆脂瘤型中耳炎鼓膜穿孔邻近皮肤的表达明显高于非胆脂瘤型中耳炎,说明胆脂瘤型中耳炎该处皮肤增生更活跃。     

Chronic otitis media: surgical failures and management

Failure of primary tympanomastoid surgery to control chronic otitis is a difficult otologic problem. The facial nerve, middle fossa dura, and inner ear structures are at high risk because of distorted anatomy from the primary surgery and/or recurrent disease. In this article our techniques and experience with 42 revision procedures for recurrent chronic otitis with and without cholesteatoma are presented. Control of otorrhea and/or cholesteatoma was achieved in 93% of the cases; however, this might be less after 5 years of follow-up. The mean postoperative air-bone gap was 25 dB, and this is a 6 dB improvement from the preoperative level. Revision tympanomastoid surgery was very effective and safe in the management of this difficult problem. For cholesteatoma in particular, the canal wall down technique is our procedure of choice.     

Complications of chronic otitis media and cholesteatoma

The incidence of complications of chronic otitis media and cholesteatoma has decreased since the proliferation of antibiotics early in the twentieth century. However, these complications continue to occur, and can be lethal if they are not identified and treated properly. Therapy for the complications associated with chronic otitis media, unlike that of acute otitis media, usually involves surgical intervention. As medical (antibiotic) therapy continues to improve, and new imaging techniques are introduced, less invasive treatment modalities may be shown to be as effective as the classic, time-tested, surgical options.     

Chronic suppurative otitis media and cholesteatoma. Vanishing diseases among Western populations?

A population-based survey of patients who had undergone surgery for chronic ear disease over the past 30 years in northern Finland revealed that the number of new surgical cases of both chronic suppurative otitis media and cholesteatoma has declined sharply after a peak in 1971^1974 and is now almost non-existent in contrast to the number of patients being operated on for chronic dry perforations, which has remained more or less the same. Most of the patients with chronic suppurative otitis media and cholesteatoma had had chronic ear trouble as early as the 1940s or 1950s. Moreover, if an active chronic otitis media developed in these cases, it started at a younger age than in cases with an onset after the 1950s. These facts may indicate that the decrease seen in this population is in close connection with the introduction of antimicrobials in the treatment of acute otitis media in the mid-1950s in the area.     

Cartilage-perichondrial composite graft tympanoplasty in the treatment of posterior marginal and attic retraction pockets

The atelectatic retraction pocket (ARP) has been implicated in the development of chronic otitis media and cholesteatoma. The ARP's tendency for persistence or recurrence despite treatment is a significant otologic problem. The purpose of this study is to define and discuss this clinical entity and the use of cartilage-perichondrium tympanoplasty as a safe and predictable method of treatment, reducing destructive complications while maintaining ear function. A retrospective analysis of 85 operated ears is presented.