Management of acquired cholesteatoma in the pediatric population.

PURPOSE OF REVIEW: This review focuses on the most recent literature pertaining to pediatric acquired cholesteatoma and aims to integrate findings into a comprehensive management approach. RECENT FINDINGS: Pediatric acquired cholesteatoma has been shown to differ from the adult variety secondary to anatomy and physiologic factors. Whereas the goals of therapeutic management are ultimately similar in adult and pediatric patients, special considerations must be taken into account when deciding on a treatment plan for a child. Although avoidance of an unstable mastoid cavity is an important consideration in this population, successful management has been reported with canal wall-up, canal wall-down, and hybrid techniques. Second-look procedures are also important when concern of recurrence exists. Newer innovations include endoscopic ear surgery and diffusion-weighted imaging. SUMMARY: The principal goal of pediatric cholesteatoma management is eradication of disease. An individualized approach is paramount in yielding superior results in these patients. Special consideration should be given to anatomical and social factors.

Hearing aid tolerance after revision and obliteration of canal wall down mastoidectomy cavities.

OBJECTIVE: To review the tolerance of hearing aid use after revision and obliteration of a previously unstable canal wall down mastoidectomy cavity. STUDY DESIGN: Retrospective case series. SETTING: Academic tertiary referral center. PATIENTS: Adults and children who underwent the described surgical procedure followed by attempted hearing aid use. INTERVENTION(S): Surgical revision and obliteration of a chronically unstable canal wall down mastoidectomy cavity and subsequent attempted use of a conventional, ear-level hearing aid. MAIN OUTCOME MEASURE(S): Stability of mastoid cavity after starting conventional hearing aid usage. RESULTS: From a review of 87 consecutive mastoid obliteration procedures performed on previously unstable open cavities, 20 ears in 19 subjects were identified for study inclusion. The indication for hearing aid use was mixed hearing loss in the majority of subjects (85%). Among included ears, 7 (35%) had at least one documented temporary period of hearing aid nonuse because of otorrhea; however, permanent discontinuation of hearing aid use in favor of bone-anchored hearing implant placement only occurred in 3 ears (15%). The mean follow-up from the start of hearing aid use was 49 months. CONCLUSION: Although failures do exist, attempted use of a hearing aid after revision of an unstable canal wall down mastoidectomy cavity seems feasible and generally well tolerated. However, the exact likelihood of achieving this result is yet uncertain, and hearing performance was not assessed in this study.

Risk analysis of unilateral severe-to-profound sensorineural hearing loss in children.

OBJECTIVE: To explore the etiology of pediatric unilateral severe-to-profound sensorineural hearing loss (USPSNHL) and the risk of hearing loss in the contralateral ear. METHODS: Pediatric patients with USPSNHL referred to a tertiary children's hospital for unilateral bone-anchored hearing aid implantation were identified for study. Clinical charts, audiograms, and radiographic imaging of the temporal bones were reviewed. RESULTS: Eighty-four children with USPSNHL were reviewed (40 male, 44 female). The etiology of USPSNHL could be identified in 35 patients (41.7%), with perinatal events (16.2%) and family history of hearing loss (10.0%) being the most common identifiable risk factors. Forty percent of children with available newborn hearing screens passed in both ears but later developed USPSNHL. Progressive unilateral hearing loss leading to USPSNHL was found in 21% percent of patients. No patient experienced worsening hearing in the contralateral ear (mean follow-up=31.9 months). Of the 49 temporal bone scans available for review, 40.8% demonstrated aberrant inner ear anatomy, including semicircular canal dysplasia (10.2%), cochlear aperture stenosis (10.2%), hypoplastic cochlea (8.1%), enlarged vestibular aqueduct (14.3%), incomplete partition I/II (6.1%), and anomalous internal auditory canal (2.0%). CONCLUSION: Pediatric USPSNHL can be attributed to a variety of sources with a cause identifiable in approximately 40% of patients. Temporal bone CT scan acquisition appears to be relatively high yield in this patient population, but only rarely do results have clinical implications. Early follow-up suggests that this population of children with USPSNHL does not appear to have a significantly increased risk for hearing loss in the contralateral ear.

Outcomes of cartilage tympanoplasty in the pediatric population.

OBJECTIVE: To justify the successful use of a patient selection algorithm based on age for primary cartilage tympanoplasty. STUDY DESIGN: Case series with chart review. SETTING: Tertiary care pediatric hospital. SUBJECTS AND METHODS: We performed a retrospective chart review of patients between ages 4 and 13 years who underwent cartilage tympanoplasty for tympanic membrane perforations from August 2005 to November 2011. Demographics, complication data, and auditory outcomes were collected. RESULTS: Patients were subdivided into 3 age groups. Group 1 consisted of patients younger than 7 years (n = 43); group 2, ages 7 to 10 years (n = 40); and group 3, ages 10 to 13 years (n = 36). Mean follow-up was 595 days (range, 48-1742). Complication rates respective to the 3 groups were as follows: remnant perforation (6.97%, 5.00%, 2.78%), revision tympanoplasty (2.33%, 2.50%, 0%), and need for tympanostomy tubes (4.65%, 2.50%, 0%). Logistic regression models were used to evaluate complication rates between groups. No significant differences were found (remnant perforation, P = .710; repeat tympanoplasty, P = .998; tympanostomy tubes, P = .875). No significance was found among audiological outcomes between the 3 groups. CONCLUSION: These data suggest cartilage tympanoplasty can be performed effectively in young children when appropriate conditions exist.

Hemangiomas and vascular malformations: current theory and management.

Vascular anomalies are a heterogeneous group of congenital blood vessel disorders more typically referred to as birthmarks. Subcategorized into vascular tumors and malformations, each anomaly is characterized by specific morphology, pathophysiology, clinical behavior, and management approach. Hemangiomas are the most common vascular tumor. Lymphatic, capillary, venous, and arteriovenous malformations make up the majority of vascular malformations. This paper reviews current theory and practice in the etiology, diagnosis, and treatment of these more common vascular anomalies.

Using 5-aminolevulinic acid and pulsed dye laser for photodynamic treatment of oral leukoplakia.

OBJECTIVE: To determine the safety and efficacy of photodynamic therapy in the treatment of oral leukoplakia with 5-aminolevulinic acid and pulsed dye laser. DESIGN: Nonrandomized, single-arm, single-site phase 1/2 pilot study. SETTING: Academic referral center. PATIENTS: A total of 23 patients, aged 37 to 79 years, having a confirmed diagnosis of leukoplakia with or without dysplasia measuring at least 10 mm in diameter. INTERVENTIONS: Application of 5-aminolevulinic acid to lesions followed by activation with high-power 585-nm pulsed dye laser. MAIN OUTCOME MEASURES: Maximum tolerated dose of laser, postprocedure complications, objective response to treatment, and immunohistochemical changes in treated tissue. RESULTS: No significant adverse events occurred; minor local adverse effects were observed during and following photodynamic therapy in the safety phase of the study. The maximum tolerated dose was 8 J/cm(2). Of 17 patients, 7 (41%) had more than 75% regression (significant response) and 9 (53%) had more than 25% regression (partial response), for an overall response rate of 94% at 90 days. This response rate was far higher than the null-hypothesis 20% rate (P < 10(-10)) and the alternative-hypothesis 50% rate (P = .0001) for which the study was powered. When compared with baseline levels immunohistochemically, p53 expression was increased in 8 of 11 available samples (73%) and Ki-67 expression was decreased in 7 of 12 available samples (58%). CONCLUSIONS: Photodynamic therapy with 5-aminolevulinic acid and pulsed dye laser could be used to achieve regression of oral leukoplakia. The treatment is safe and well tolerated. An application time of 1.5 hours and laser radiant exposure of 8 J/cm(2) with 1.5-ms pulse time were found to be the optimal settings in this study. The high-power laser used in this study allows completion of laser therapy within 1 to 3 minutes. Further studies are necessary to determine the optimal laser radiant exposure and drug application to maximize the response rate.