Cochlear implantation in pediatric patients with Cockayne Syndrome.

Cockayne Syndrome (CS) is a rare, autosomal recessive disorder characterized by a spectrum of phenotypic abnormalities, including progressive sensorineural hearing loss (SNHL) that involves both peripheral and central components. To date, a single series of CS patients undergoing cochlear implant (CI) placement has been reported; this study reports on additional previously unreported pediatric CI recipients. Subjective benefits were noted early after activation in both patients, and speech perception scores improved over time as well, varying from 42 to 70% (versus 0-12% previously).

Utility of Stepping, Walking, and Head Impulses for Screening Patients for Vestibular Impairments.

Objective: To determine if some common screening tests predict scores on detailed, objective diagnostic tests of the vestibular system.

Study Design: Sixty patients with vestibular disorders were compared with 60 asymptomatic controls.

Setting: Vestibular diagnostic laboratory, tertiary care center.

Cochlear implant electrode misplacement: incidence, evaluation, and management.

Objectives/hypothesis: To review the presentation and management of improper electrode array placement, and to help guide clinical decision-making.

Study Design: Retrospective case series.

Methods: Pediatric and adult cochlear implant patients managed from January 2001 to present whose electrode arrays were not placed properly within the cochlea or extended beyond the cochlea into the internal auditory canal or adjacent structures.

Auditory performance after cochlear implantation in late septuagenarians and octogenarians.

Objective: Previous studies have demonstrated consistent benefit in older adults undergoing cochlear implantation as compared with younger control groups, with age category thresholds between 60 and 70 years. The objective of this study is to report auditory performance in implant recipients older than 75 years, a cohort for which few data have been reported.

Study Design: Retrospective chart review.

Advanced pediatric mastoiditis with and without intracranial complications.

Objectives/hypothesis: Recently, several groups have noticed an increase in cases of advanced pediatric mastoiditis and intracranial complications. The objective of this study was to review the bacteriology of advanced mastoiditis in pediatric patients, with the hypothesis that a difference in bacteriology might explain the development of an intracranial complication.

Study Design: Retrospective chart review.

Facial Nerve Grading System 2.0.

Objective: To present an updated version of the original Facial Nerve Grading Scale (FNGS), commonly referred to as the House-Brackmann scale.

Study Design: Controlled trial of grading systems using a series of 21 videos of individuals with varying degrees of facial paralysis.

Results: The intraobserver and interobserver agreement was high among the original and revised scales.

Pathologic prion protein is specifically recognized in situ by a novel PrP conformational antibody.

Prion diseases are characterized by the accumulation in the brain of abnormal conformers (PrP(Sc)) of the cellular prion protein (PrP(C)). PrP(Sc) immunohistochemistry, currently based on antibodies non-distinguishing between PrP(C) and PrP(Sc), requires pre-treatments of histological sections to eliminate PrP(C) and to denature PrP(Sc). We employed the PrP(Sc)-specific antibody 89-112 PrP motif-grafted IgG on mildly fixed, untreated brain sections from several cases of human prion diseases.

Coronal computed tomography prevalence of superior semicircular canal dehiscence.

Objectives: The relatively new clinical entity superior canal dehiscence syndrome (SCDS) is diagnosed by clinical symptoms and signs. Coronal computed tomography (CT) has been used to confirm the diagnosis. A consecutive series of temporal bone CT scans was reviewed to define the prevalence of a dehiscent-appearing superior semicircular canal.

Inflammatory pseudotumor of the temporal bone.

Objective: To characterize the clinical presentation, imaging characteristics, intraoperative findings, and key histopathologic features of inflammatory pseudotumors of the temporal bone. Findings from an index case are presented, and the literature is reviewed for comparison.

Study Design: Retrospective case review.