Effect of corticosteroid-antibiotic agents on granulation tissue in a murine model.

OBJECTIVE: To compare the effects of 3 commonly used ototopical corticosteroid-antibiotic agents, currently available for use in the treatment of inflammatory conditions of the external and middle ear, on granulation tissue in an established murine model of wound healing. SUBJECTS: Twelve C57/BL6J mice. DESIGN: Eight-millimeter wounds, created bilaterally on the dorsum of the mice, were treated with combinations of 0.3% ciprofloxacin and 0.1% dexamethasone (CiproDex), 0.3% tobramycin and 0.1% dexamethasone (TobraDex), 0.2% ciprofloxacin hydrochloride and 1% hydrocortisone (Cipro HC), or phosphate-buffered saline (n = 6 each) for 3 days (days 4-6) and then harvested on day 7. Wound sections were stained with hematoxylin-eosin, Gomori trichrome, and CD31. Extracellular matrix deposition was graded from 1-4, and neovascularization was assessed by counting the number of endothelial-lined vessel lumens per high-power field (HPF). RESULTS: The mean +/- SEM grade of extracellular matrix deposition was lower in CiproDex- (1.7 +/- 0.2) and TobraDex- (2.0 +/- 0.2) but not Cipro HC-(2.9 +/- 0.3) treated wounds compared with control wounds (2.9 +/- 0.2) (P<.01). The mean +/- SEM number of vessel lumens per HPF was lower in CiproDex- (0.9 +/- 0.2 lumens/HPF), TobraDex- (1.5 +/- 0.3 lumens/HPF) and Cipro HC-(0.9 +/- 0.3 lumens/HPF) treated wounds compared with controls (3.3 +/- 0.5 lumens/HPF) (P<.01). CONCLUSIONS: All 3 ototopical corticosteroid-antibiotic agents studied were equally effective at reducing neovascularization, although dexamethasone-based products were more effective at reducing extracellular matrix deposition. The results of this study suggest that ototopical agents containing dexamethasone may be more effective for the treatment of granulation tissue resulting from external and middle ear inflammatory conditions.

A staging system for congenital cholesteatoma.

OBJECTIVE: To develop a staging system for congenital cholesteatoma in predicting the likelihood of residual disease. DESIGN: Retrospective analysis of data from a case series, to identify predictors of residual disease. SETTING: Tertiary care pediatric hospital. PARTICIPANTS: Children undergoing surgical removal of congenital cholesteatoma. There were 156 patients, with 160 cholesteatomas; 4 children had bilateral disease. INTERVENTIONS: Each case was scored as to quadrants of the middle ear involved, ossicular involvement, and mastoid extension. MAIN OUTCOME MEASURE: Surgically confirmed residual disease at any time after the initial procedure. RESULTS: Four stages were defined as follows: stage I, disease confined to a single quadrant; stage II, cholesteatoma in multiple quadrants, but without ossicular involvement or mastoid extension; stage III, ossicular involvement without mastoid extension; and stage IV, mastoid disease. There was a strong association between stage and residual disease, ranging from a 13% risk in stage I to 67% in stage IV. CONCLUSIONS: This simple staging system may be particularly useful in standardizing the reporting of congenital cholesteatoma and in adjusting for severity in evaluating outcomes. It also provides information that is useful in counseling parents.

Congenital cholesteatoma.

PURPOSE OF REVIEW: Congenital cholesteatomas have historically been considered a rare disorder. However, a review of the literature reveals an incidence ranging from 4 to 24%, and these values are probably underestimated. This article summarizes the general diagnostic criteria of congenital cholesteatomas, their usual presenting symptoms and findings, perioperative studies, a proposed staging system to assist with predicting postoperative outcomes, general surgical procedures, and recidivism rates. RECENT FINDINGS: Congenital cholesteatomas have been defined as evidencing no prior history of otorrhea, tympanic perforation, or previous otologic procedures; a normal pars flaccida and tensa; and a pearly white mass medial to the tympanic membrane. Their cause remains controversial; however, the epithelial rest theory is most commonly accepted. The incidence of this disorder seems to be on the rise; however, this may be a result of increased awareness and reporting. The most common sites of presentation on physical examination are the anterior-superior and posterior-superior quadrants of the tympanic membrane. Conductive hearing loss is the most common presenting symptom. There is a lack of uniformity of reporting and classifying congenital cholesteatomas. Staging systems have recently been proposed. One in particular is reported and correlates with outcomes of treatment. Treatment of congenital cholesteatomas is still surgical. A comparison is made between canal wall up and canal wall down tympanomastoidectomy for the treatment of nonlocalized cholesteatoma pearls. Postoperative hearing results are associated with the status of the ossicular chain perioperatively. SUMMARY: Heightened awareness and early diagnosis of congenital cholesteatomas is imperative. Early treatment decreases the extent of the disease and reduces the risk of recidivism and complications. Management of this disease is surgical. Preoperative computed tomography should be considered to assist in preoperative planning and prediction of postoperative issues. Consideration of a standard staging system and classification is important for consistent reporting and comparison.

Congenital cholesteatoma: 20 years' experience at The Children's Hospital of Philadelphia.

OBJECTIVE: We report our experience with congenital cholesteatoma over a span of 20 years with an emphasis on presenting characteristics and predictors of outcome. METHODS: We conducted a retrospective review from 1981 through 2000. RESULTS: One hundred seventy-two congenital cases were identified in 167 patients. Five patients had bilateral disease. The majority (72%) were found in boys, with an average age of 5.0 years. Hearing loss was slight to moderate. When confined to 1 quadrant, cholesteatoma was anterosuperior in 82% of cases; 47% had cholesteatoma in 2 or more quadrants. Ossicular chain involvement was found in 43% of all cases, and mastoid extension was evident in 23%. The rate of recurrent disease was directly related to the extent and number of quadrants involved. CONCLUSION: To our knowledge, this is the largest series of congenital cholesteatomas to be reported. This review confirms the male predominance and predilection for the anterosuperior quadrant. The extent of cholesteatoma and its relation to residual disease should be used as a guide for planning a second-look procedure.

Effects of Clarion electrode design on mapping levels in children.

The design of the placement of the Clarion cochlear implant's intracochlear electrode array has undergone 2 revisions since its introduction, each to improve modiolar proximity. Stimulation with modiolar proximity may reduce current requirements for threshold levels and most comfortable levels of stimulation. This study analyzed the effects of electrode design on programming levels for deaf children implanted with the 3 cochlear implant designs and followed at The Children's Hospital of Philadelphia. Psychophysical data were reported if measurements were taken approximately 3 months after initial activation, and programming parameters included nonsequential monopolar stimulation of 75-micros-per-phase biphasic pulses presented at 813 Hz per electrode. The threshold level and most comfortable programming level were measured by standard clinical techniques appropriate for children. The results indicate that the 2 electrode placement revisions have each significantly reduced threshold levels and most comfortable stimulation levels. These results are discussed in the context of device aesthetics, safety, and function.

Outcomes of clinical examination and genetic testing of 500 individuals with hearing loss evaluated through a genetics of hearing loss clinic.

Hearing loss (HL) occurs in approximately 2 out of every 1,000 births and is genetic in origin in approximately 50% of cases. This high incidence coupled with the increasing number of genes implicated in HL and the trend toward universal newborn screening led to the establishment of the Genetics of Hearing Loss Clinic at The Children's Hospital of Philadelphia to manage the diagnosis, genetic screening, and counseling of families with an affected child. To date 500 individuals have been evaluated from 1999 to 2004. To determine the cause of their HL and screen for syndromic forms of HL, individuals were offered a panel of tests. Depending on the type and severity of the HL, recommendations included GJB2 mutation analysis, renal and thyroid function studies, a CT scan of the temporal bones, an ophthalmology evaluation, an EKG, and, at times, additional genetic tests. Of the 500 patients evaluated 70 (14%) had a syndromic etiology for their HL. Twenty-eight different syndromic etiologies were identified. Enlarged vestibular aqueducts (EVAs) and/or Mondini malformations were seen in 18% of individuals with HL who had a CT or MRI of the temporal bones. Genetic testing of the GJB2 gene was completed for 310 of the 377 patients with bilateral sensorineural HL (82.2%). Nineteen different variants were identified in the GJB2 gene. Through GJB2 mutational analysis, clinical examination, and laboratory testing, a definitive etiologic diagnosis was established in 110/500 (22%) of patients.

Screening and prevention of hearing loss in children.

Congenital hearing loss is the most common neurosensory handicap in neonates. Recently, technology has become available that has allowed states to implement universal screening programs at a relatively low cost and with minimal expertise needed for hospital personnel to operate the screening machines. In successful programs, the age of diagnosis has been reduced from 2(1/2) years to 3 to 6 months. Children diagnosed with hearing loss before speech develops have been shown to have better speech and language outcomes than those who are diagnosed later. Strategies for screening and early intervention are discussed, as well as the causes, prevention, and treatment of more common forms of childhood hearing loss.