Jacob R. Brodsky

13‐cis Retinoic Acid (Accutane) Suppresses Hippocampal Cell Survival in Mice

Abstract: Use of the acne drug Accutane (13‐cis retinoic acid, [13‐cis RA]) has been associated with severe depression. This association has been considered controversial because no causative link has been found between 13‐cis RA and this disorder. A recent hypothesis has suggested that atrophy of the hippocampus can result in depression. We now show, in a mouse model, that endogenous RA generated by synthetic enzymes in the meninges acts on hippocampal granule neurons, and chronic (3‐week) exposure to a clinical dose of 13‐cis RA may result in hippocampal cell loss. In humans this may be conjectured to be the mechanism by which Accutane contributes to depression.

Otologic outcomes after blast injury: the Boston Marathon experience

Objective Otologic trauma was the most common physical injury sustained after the April 15, 2013, Boston Marathon bombings. The goal of this study is to describe the resultant otologic morbidity and to report on early outcomes. Study Design Multi-institutional prospective cohort study. Methods Children and adults seen for otologic complaints related to the Boston Marathon bombings comprised the study population. Participants completed symptom assessments, quality-of-life questionnaires, and audiograms at initial and 6-month visits. Otologic evaluation and treatment, including tympanoplasty results, were reviewed. Results More than 100 patients from eight medical campuses have been evaluated for blast-related otologic injuries; 94 have enrolled. Only 7% had any otologic symptoms before the blasts. Ninety percent of hospitalized patients sustained tympanic membrane perforation. Proximity to blast (RR = 2.7, p < 0.01) and significant nonotologic injury (RR = 2.7, p < 0.01) were positive predictors of perforation. Spontaneous healing occurred in 38% of patients, and tympanoplasty success was 86%. After oral steroid therapy in eight patients, improvement in hearing at 2 and 4 kHz was seen, although changes did not reach statistical significance. Hearing loss, tinnitus, hyperacusis, and difficulty hearing in noise remain persistent and, in some cases, progressive complaints for patients. Otologic-specific quality of life was impaired in this population. Conclusion Blast-related otologic injuries constitute a major source of ongoing morbidity after the Boston Marathon bombings. Continued follow-up and care of this patient population are warranted.

Video head impulse testing (VHIT) in the pediatric population

OBJECTIVES VHIT is the first vestibular test to evaluate all six semicircular canals. This test has advantages over the rotary chair and caloric tests in evaluating children since it does not require fear-inducing darkness or provocation of dizziness. The goal of this study was to review our initial experience with VHIT in children and adolescents at a pediatric vestibular program. METHODS Results using the ICS Impulse VHIT device in 33 patients <20 years of age were retrospectively reviewed. Rotary chair testing was used to designate 26 subjects into groups with normal (n=20) and abnormal (n=6) lateral semicircular canal (LSC) function for comparison. RESULTS Ages ranged from 3 to 19 years (mean 13±4.3) with no statistically significant difference in mean lateral canal gains between age groups (3-10, 11-14, 15-19 years, respectively) by one-way ANOVA, p=0.111. LSC VHIT gain of <0.7 demonstrated sensitivity of 66.7%, specificity of 100%, positive predictive value of 100%, negative predictive value of 90.9% for detecting abnormal LSC function. Corrective saccades demonstrated 100% sensitivity and 100% specificity for detecting abnormal LSC function. VHIT gain <0.7 in an anterior (n=7) or posterior (n=9) canal was only found in subjects with a clinical history and abnormal findings on other tests indicative of a diagnosis involving the affected canal (e.g. benign paroxysmal positioning vertigo, vestibular neuritis, etc.). CONCLUSION VHIT is an effective test for evaluating semicircular canal function in children and offers major potential advantages over rotary chair and caloric testing.

Objective vestibular testing of children with dizziness and balance complaints following sports-related concussions.

Objective To conduct objective assessment of children with balance and vestibular complaints following sports-related concussions and identify the underlying deficits by analyzing laboratory test outcomes. Study Designs Case series with chart review. Setting Pediatric tertiary care facility. Subjects and Methods Medical records were reviewed of 42 pediatric patients with balance and/or vestibular complaints following sports-related concussions who underwent comprehensive laboratory testing on their balance and vestibular function. Patients’ characteristics were summarized and results analyzed. Results More than 90% of the children with protracted dizziness or imbalance following sports-related concussion had at least 1 abnormal finding from the comprehensive balance and vestibular evaluation. The most frequent deficit was found in dynamic visual acuity test, followed by Sensory Organization Test and rotational test. Patient’s balance problem associated with concussion seemed to be primarily instigated by vestibular dysfunction. Furthermore, semicircular canal dysfunction was involved more often than dysfunction of otolith organs. Yet, <10% of the children experienced a hearing loss following sports-related concussion. Conclusions Vestibular impairment is common among children with protracted dizziness or imbalance following sports-related concussion. Our study demonstrated that proper and thorough evaluation is imperative to identify these underlying deficits and laboratory tests were helpful in the diagnosis and recommendation of following rehabilitations.

CERVICAL LIPOBLASTOMA: CASE REPORT, REVIEW OF LITERATURE, AND GENETIC ANALYSIS

Lipoblastoma is a rare, benign tumor of infants and children, usually occurring in the extremities and trunk, with only a few cases reported in the neck.

Teratoma of the neonatal head and neck: A 41-year experience

OBJECTIVE To review our institutions experience with the presentation, evaluation, and management of teratoma of the head and neck in the neonatal population. DESIGN Retrospective case series (November 1970 through September 2011). SETTING Tertiary care childrens hospital. PATIENTS 14 patients (12 boys and 2 girls). INTERVENTION Detailed review of presentation, diagnostic approaches, surgical management, and outcomes. MAIN OUTCOME MEASURES Anatomic sites, use of pre and post-natal imaging, use of EXIT (ex utero intrapartum treatment) procedure, presenting symptoms, surgical approaches, additional therapeutic modalities, and outcomes are reviewed. RESULTS Seven patients were diagnosed prenatally, while the remaining 7 patients were diagnosed at birth or shortly thereafter. The tumor emanated from the neck in 9 patients, the nasopharynx/oropharynx in 3 patients, the external nose in 1 patient and the face in 1 patient. Nine patients had associated upper airway obstruction. Four underwent an EXIT procedure, with 3 requiring intubation and 1 requiring tracheostomy. All patients underwent surgical resection. One patient demonstrated recurrence at follow-up. CONCLUSIONS Teratoma of the head and neck, though rare, is an important part of the differential diagnosis of neck masses in children, particularly in the perinatal period. The ability to make this diagnosis prenatally with high-resolution fetal ultrasound and MRI (magnetic resonance imaging) permits planning for airway and tumor management prior to delivery. An EXIT procedure should be considered when airway compromise by tumor compression is suspected. Early surgical excision is the treatment of choice and recurrence is rare when a complete resection is achieved.

Vestibular neuritis in children and adolescents: Clinical features and recovery §

OBJECTIVE Describe the clinical presentation and recovery of vestibular neuritis in children and adolescents. STUDY DESIGN Retrospective case series. SETTING Pediatric tertiary care center. SUBJECTS AND METHODS Eleven patients diagnosed with vestibular neuritis were identified from a database of 301 patients evaluated at our pediatric vestibular clinic from January 2012 through January 2015. Medical records were reviewed to determine clinical presentation, vestibular testing results, treatment, and recovery. Incomplete recovery was defined as residual dizziness or imbalance at most recent follow-up >30 days from symptom onset. RESULTS Patients were 5-19 years old (mean 13.1±5.34) and included 6 boys and 5 girls. All presented with a sudden rotational vertigo, imbalance, and nausea for an average of 10 days without other associated symptoms. Testing included rotary chair (8 of 9 abnormal), caloric (2 of 2 abnormal), video head impulse (5 of 8 abnormal), subjective visual vertical (4 of 8 abnormal), and cervical vestibular evoked myogenic potential (0 of 6 abnormal) tests. All patients with incomplete recovery (n=4; 36%) were ≥15 years old at symptom onset. All patients with incomplete recovery that underwent vestibular rehabilitation (n=2) initiated it ≥90 days from symptom onset, while 3 out of 4 patients with complete recovery that underwent vestibular rehabilitation initiated it ≤14 days from symptom onset. Two patients received oral steroids, neither of whom had incomplete recovery. CONCLUSION Vestibular neuritis should be considered in pediatric patients with vertigo and may result in longstanding symptoms, particularly in adolescents. The treatment of pediatric vestibular neuritis with rehabilitation and steroids deserves further study.

Subjective visual vertical testing in children and adolescents

Subjective visual vertical (SVV) is a vestibular test commonly used in adults that has not been well studied in children. In this test, the patient aligns a projected line with the perceived true vertical. Deviation of >2° is usually associated with utricular dysfunction and may also be seen with central vestibular lesions. The goal of this study was to determine the efficacy of SVV in children.

Peripheral vestibular loss detected in pediatric patients using a smartphone-based test of the subjective visual vertical

INTRODUCTION Detection of peripheral vestibular loss (PVL) in children with dizziness is an important and challenging task. The static subjective visual vertical (SVV) test can effectively detect PVL, but requires specialized equipment. OBJECTIVE The goal of this study was to determine the efficacy of a smartphone-based SVV test at detecting PVL in pediatric patients. METHODS Thirty-nine patients between 7 and 18 years old (mean=14.0±2.70) underwent conventional SVV (conv-SVV) and smartphone-based SVV (ip-SVV) testing. Subjects included 6 with PVL (based on clinical history and other vestibular tests), 6 with benign paroxysmal positioning vertigo (BPPV), 11 with central causes of vertigo (CV), 8 with non-vestibular dizziness (NVD), and 8 controls. RESULTS Mean ip-SVV score in the PVL group (2.77±1.45) was significantly higher than in each of the other groups (BPPV=0.89±0.55; CV=1.08±0.68; NVD=1.45±1.19; Control=1.08±0.73; one-way analysis of variance, p=0.008), and remained significant after adjusting for age and gender by multiple linear regression analysis. Receiver operating characteristic analysis predicted an optimal ip-SVV cut-off score of >2.13° with a sensitivity of 66.7%, specificity of 97.0%, positive predictive value (PPV) of 80%, and negative predictive value of 94.1% for detecting PVL. Sensitivity and PPV improved to 75% and 100%, respectively, when subjects tested >1 month after symptom onset (n=24) were excluded. CONCLUSION Smartphone-based SVV testing is a simple and useful office-based method for detecting PVL in children with dizziness.

Impairment of habituation of the auditory brain stem response in migrainous vertigo.

Objectives: We evaluated the auditory brain stem response (ABR) in migrainous vertigo (MV). Methods: Four subjects who met clinical criteria for definite MV and 4 subjects with non-vertiginous migraine (NVM) underwent ABR testing while asymptomatic and within 16 hours of a symptomatic episode. Four control subjects were also tested. A set of 4 consecutive 750-click series was administered at 50-, 60-, and 70-dB intensities. We compared the groups in terms of habituation of the amplitude of wave IV-V (habituation of IV-V) from the first through fourth series for each set. Results: The habituation of IV-V amplitude to 50-dB stimuli was significantly less (p = 0.047) in the symptomatic MV group (5.08% ± 22.32%) than in the symptomatic NVM group (–21.44% ± 13.50%) or the control group (–26.06% ± 9.76%). The habituation of IV-V amplitude to 70-dB stimuli in the MV group was significantly less (p = 0.031) during symptomatic testing (–3.43% ± 8.89%) than during asymptomatic testing (–21.23% ± 6.41%). Conclusions: The habituation of IV-V amplitude is reduced during MV attacks. This finding suggests impaired brain stem inhibition at the level of the inferior colliculus, which shares serotonergic connections with the dorsal raphe nucleus, an area hyperactive in migraine.