Significance of unilateral enlarged vestibular aqueduct.

Objectives/hypothesis: To describe the clinical phenotype of pediatric patients with unilateral enlarged vestibular aqueduct (EVA) and then to compare the findings to two clinically related phenotypes: bilateral EVA and unilateral hearing loss without EVA. In view of clinical observations and previously published data, we hypothesized that patients with unilateral EVA would have a much higher rate of contralateral hearing loss than patients with unilateral hearing loss without EVA.

Study Design: Retrospective cohort study.

Audiologic and temporal bone imaging findings in patients with sensorineural hearing loss and GJB2 mutations.

Objectives/hypothesis: Our objectives were to determine genotype-phenotype correlations in patients with sensorineural hearing loss (SNHL) who undergo testing for GJB2 mutations and to examine the relationship of temporal bone anomalies seen on computed tomography (CT) and GJB2 mutations.

Study Design: We conducted a retrospective review of all children diagnosed with SNHL and who underwent GJB2 testing from 1997 to 2006.

Results: Of 840 patients, 146 (17.

The large vestibular aqueduct: a new definition based on audiologic and computed tomography correlation.

Objective: The study goal was to determine the prevalence and clinical significance of a large vestibular aqueduct (LVA) in children with sensorineural hearing loss (SNHL).

Study Design And Setting: We conducted a retrospective review of a pediatric SNHL database. One hundred seven children with SNHL were selected and their radiographic and audiometric studies were evaluated.

The influence of mutations in the SLC26A4 gene on the temporal bone in a population with enlarged vestibular aqueduct.

Objective: To correlate genetic and audiometric findings with a detailed radiologic analysis of the temporal bone in patients with enlarged vestibular aqueduct (EVA) to ascertain the contribution of SLC26A4 gene mutations to this phenotype.

Design: A retrospective review of patients with EVA identified in a database of pediatric hearing-impaired patients.

Setting: A tertiary care pediatric referral center.

Partial absence of the posterior semicircular canal in Alagille syndrome: CT findings.

We report a case of bilateral partial absence of the posterior semicircular canals (with normal lateral semicircular canals) imaged with CT in a patient with Alagille syndrome. Similar histologic findings have been reported in the pathology literature. This association has been previously reported only for Waardenburg syndrome in the imaging literature.

Audiometric, clinical and educational outcomes in a pediatric symptomatic congenital cytomegalovirus (CMV) population with sensorineural hearing loss.

Objective: To correlate audiometric findings and outcomes with the clinical, radiological and educational findings in a symptomatic congenital cytomegalovirus (CMV) population with sensorineural hearing loss.

Methods: A retrospective review of data from 21 symptomatic congenital CMV patients identified in a pediatric hearing impaired database of 1500 patients. Clinical data, audiometric thresholds and outcomes, radiographic abnormalities, communication and educational achievements were used as outcome measures.

Improving patient care: the use of a digital teaching file to enhance clinicians' access to the intellectual capital of interdepartmental conferences.

Objective: We describe a simple method for creating teaching cases from clinical data, radiologic images, surgical images, and images from pathologic slides that are presented at tumor board conferences.

Conclusion: The resulting interdisciplinary case files are of educational value both during and after conference presentations and can be used by clinicians to gather appropriate historical, laboratory, imaging, surgical, and pathologic data on their patients. This system improves the efficiency and accuracy in gathering patient histories when care is transferred among clinics, the emergency department, and wards.

The cochlear cleft.

Background And Purpose: Recent advances in the display of medical images permit the routine study of temporal bone CT images at high magnification. We noted an unfamiliar structure, which we now call the "cochlear cleft," in the otic capsule. To our knowledge, this report represents the first description of this structure in the medical imaging literature.

Temporal bone abnormalities associated with hearing loss in Waardenburg syndrome.

Objectives/hypothesis: The objectives were to correlate audiometric thresholds with radiological findings and to determine the prevalence of inner ear radiological abnormalities in patients with hearing loss and Waardenburg syndrome.

Study Design: The study was a retrospective review of patients with Waardenburg syndrome identified in a pediatric hearing-impaired population and human genetics clinic.

Methods: Nine children with Waardenburg syndrome were identified.

Enlarged vestibular aqueduct syndrome in the pediatric population.

Objective: To correlate clinical and audiometric findings with the radiologic appearance in patients with enlarged vestibular aqueduct.DESIGN A retrospective review of data from enlarged vestibular aqueduct patients identified in a pediatric hearing-impaired database of 1,200 patients. SETTING A tertiary care pediatric referral center.