Cortical Auditory Evoked Potential Testing in Children With Auditory Neuropathy Spectrum Disorder.

Purpose: In the present report, we reviewed the role of cortical auditory evoked potentials (CAEPs) as an objective measure during the evaluation and management process in children with auditory neuropathy spectrum disorder (ANSD).

Method: We reviewed the results of CAEP recordings in 66 patients with ANSD aged between 2 months and 12 years and assessed the relationship between their characteristics (prevalence, morphology, latencies, and amplitudes) and various clinical features, including the mode of medical management.

Results: Overall, the CAEPs were present in 85.

Asymmetric Ocular Vestibular Evoked Myogenic Potentials in Pediatric Vestibular Migraine.

Objective: Although ocular vestibular evoked myogenic potentials (oVEMP) abnormalities have been associated with vestibular migraine (VM) in adults, no studies have evaluated this in pediatric patients with VM. oVEMP asymmetry with normal cervical VEMP (cVEMP) findings may be a reliable VM biomarker in adults. We characterize VEMP results among pediatric patients with VM and benign recurrent vertigo of childhood (BRVC), a migraine precursor, and compare these results with VEMP findings from pediatric patients with nonmigrainous vestibular diagnoses.

A 15-Year Review of 260 Children With Auditory Neuropathy Spectrum Disorder: I. Demographic and Diagnostic Characteristics.

Objectives: The aim of the study was to review the demographic and clinical characteristics of all pediatric patients diagnosed with auditory neuropathy spectrum disorder (ANSD) by a pediatric health care system from 2005 to 2020 and examine whether or not our diagnostic capabilities in an ANSD population have evolved as our institutional experience has grown and knowledge in the field has expanded.

Design: This was a retrospective study reviewing the demographic data, medical history, imaging studies, audiological and speech-language data, type of audiological intervention and mode of communication in 260 pediatric patients diagnosed with ANSD over a 15-year period.

Results: The study revealed that male and female children were equally affected with all levels of hearing detection being represented and that about 40% of affected children were premature and most were admitted to the neonatal intensive care unit.

Congenital soft tissue stenosis of the external auditory canal with canal cholesteatoma: Case report and literature review.

Congenital external auditory canal stenosis (EACS) is a spectrum of abnormalities affecting the external and middle ear. We report a 6 year-old patient with EACS affecting the lateral fibrocartilaginous canal that was successfully repaired. This patient highlights a variant of EACS characterized by lateral soft tissue narrowing with normal osseous development.

Cochlear Nerve Deficiency and Brain Abnormalities in Pediatric Patients.

Hypothesis: To investigate the intracranial abnormalities present in children with cochlear nerve deficiency (CND), including abnormalities of other cranial nerves, and to describe their auditory abilities.

Background: The prevalence of CND has increased with the development of high resolution magnetic resonance imaging (MRI). There are varying degrees of CND from true aplasia to hypoplasia.

A pearl in the ear: Intracranial complications of pediatric cholesteatomas.

A nine-year-old male had a cholesteatoma of the mastoid and middle ear found incidentally after myringotomy tube placement. Associated asymptomatic sigmoid plate dehiscence with sinus invasion or thrombosis and ossicular chain destruction complicated his case. He had canal wall down tympanomastoidectomy and was followed for 4.

The role of gastric pepsin in the inflammatory cascade of pediatric otitis media.

Importance: Otitis media is characterized as an ongoing inflammation with accumulation of an effusion in the middle ear cleft. The molecular mechanisms underlying the pathogenesis, particularly the inflammatory response, remain largely unknown. We hypothesize that aspiration of gastric contents into the nasopharynx may be responsible for the initiation of the inflammatory process or aggravate a preexisting condition.

A homozygous SLITRK6 nonsense mutation is associated with progressive auditory neuropathy in humans.

Objectives/hypothesis: SLITRK family proteins control neurite outgrowth and regulate synaptic development. In mice, Slitrk6 plays a role in the survival and innervation of sensory neurons in the inner ear, vestibular apparatus, and retina, and also influences axial eye length. We provide the first detailed description of the auditory phenotype in humans with recessive SLITRK6 deficiency.

Clinical characteristics of pediatric granular myringitis.

Objective: To determine the clinical characteristics of children with granular myringitis.

Study Design: Case series with chart review of children with granular myringitis.

Setting: Tertiary care children's hospitals in Delaware and Florida.

Clinical characteristics of children with cochlear nerve dysplasias.

Objectives/hypothesis: To describe the clinical and audiometric characteristics of children with cochlear nerve dysplasia (CND).

Study Design: Retrospective chart review of clinical database of children with inner ear anomalies treated at a tertiary care children's hospital.

Methods: Institutional review board-approved retrospective review from June 30, 2006, to July 1, 2011; 18 children were identified with magnetic resonance imaging (MRI) evidence of CND defined as a cochlear nerve 50% smaller than the adjacent facial nerve.

Auditory nerve function following cochleitis.

We present a case of temporary cochlear nerve dysfunction due to endocochlear inflammation with subsequent recovery. Retrospective case review at a pediatric tertiary care hospital. A nine-year-old male presented seven years post-cochlear implantation with an electrode array extruded into the external auditory canal.

Atypical presentation of geniculate ganglion venous malformation in a child: conductive hearing loss without facial weakness.

We describe an unusual presentation of geniculate ganglion venous malformation, a rare facial nerve lesion, emphasizing the importance of the differential diagnosis, imaging characteristics, and controversies in management. A child presented with moderate right-sided conductive hearing loss and a House-Brackmann grade I facial nerve function bilaterally. Computed tomography and magnetic resonance imaging showed a mass demonstrating features consistent with a geniculate ganglion venous malformation.

Complications of otitis media.

Otitis media is the most common reason for antibiotic prescription in the United States. Whether due to disease virulence or growing antimicrobial resistance, complications of otitis media seem to be seen more frequently. These complications may be difficult to identify and treat.

Comprehensive vestibular and balance testing in the dizzy pediatric population.

Objective: To describe the spectrum of balance disease in a large population of children presenting to a tertiary care vestibular and balance laboratory.

Study Design: Case series with chart review.

Setting: Tertiary care pediatric hospital.

Prevalence of vestibular and balance disorders in children.

Objective: Determine the prevalence of vestibular and balance disorders in children, rate of complaints of imbalance, and odds ratio of related diagnoses.

Patients And Methods: Retrospective review of pediatric health system during a 4-year period for International Classification of Diseases, 9th Revision, codes related to balance disorders. Identified records were searched for chief complaints related to balance and for codes of related otologic and neuro-otologic diagnoses.

Central auditory processing deficiency with anatomic deficit in left superior temporal lobe.

Objectives/hypothesis: Describe the clinical presentation and treatment of a patient with central auditory processing deficiency associated with an anatomic deficit in the left superior temporal lobe.

Study Design: Case report.

Methods: We report a case and the treatment of an 8-year-old boy with abnormal speech development and auditory processing disorder who was found to have a large cystic lesion of his left superior temporal lobe.

Inflammatory pseudotumor of the inner ear: are computed tomography changes pathognomonic?

This case study presents a report of inflammatory pseudotumor of the inner ear in a child, discusses radiographic findings and clinical management, and reviews the current literature on this rare disease. A 2.5-year-old presented with otalgia, transient vertigo, and fluctuating facial palsy partially responsive to myringotomy with tube.

Ophthalmologic disorders in children with syndromic and nonsyndromic hearing loss.

Objective: To determine the rate of ophthalmologic anomalies among patients with syndromic and nonsyndromic, congenital sensorineural hearing loss (SNHL) to assess the need for comprehensive ophthalmologic evaluation in these children.

Design: Retrospective medical chart review of children with SNHL who underwent comprehensive evaluation by pediatric ophthalmologists and geneticists.

Setting: Tertiary care pediatric hospital.

Gastric pepsin in middle ear fluid of children with otitis media: clinical implications.

Gastroesophageal reflux and extraesophageal reflux have been postulated to be involved in the pathogenesis of otitis media. This is supported by recent studies revealing the presence of gastric pepsin in the middle ear space of children with otitis media but not in control patients without otitis media. Reflux's role in otitis media appears to be most pronounced in younger children and those with purulent effusions.

The role of extraesophageal reflux in otitis media in infants and children.

Objectives/hypothesis: Gastroesophageal reflux disease (GERD) is common in children, and extraesophageal reflux disease (EORD) has been implicated in the pathophysiology of otitis media (OM). We sought to 1) determine the incidence of pepsin/pepsinogen presence in the middle ear cleft of a large sample of pediatric patients undergoing myringotomy with tube placement for OM; 2) compare this with a control population of pediatric patients undergoing middle ear surgery (cochlear implantation) with no documented history of OM; 3) analyze potential risk factors for OM in children with EORD demonstrated by the presence of pepsin in the middle ear cleft; and 4) determine if pepsin positivity at the time of myringotomy with tube placement predisposes to posttympanostomy tube otorrhea.

Study Group: prospective samples of 509 pediatric patients (n = 893 ear samples) undergoing myringotomy with tube placement for recurrent acute OM and/or otitis media with effusion in a tertiary care pediatric hospital with longitudinal follow-up of posttympanostomy tube otorrhea.