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The high frequency of vergence and accommodation deficits coexisting in patients with a vestibular diagnosis merits a detailed visual function examination.Deficits in vergence and saccades have been reported in patients with vestibular symptomatology. We retrospectively evaluated visual function deficits in adolescents with vestibular diagnoses and concussion.The following inclusion criteria were used: vestibular and optometric evaluations between 2014 and 2020, 6 to 22 years old, and 20/25 best-corrected vision or better. Clinical criteria assigned vestibular diagnoses and concussion diagnoses. Vestibular diagnoses included vestibular migraine, benign paroxysmal positional vertigo, and persistent postural perceptual dizziness. Visual function deficits were compared with a pediatric control group (30). Nonparametric statistics assessed differences in group distribution.A total of 153 patients were included: 18 had vestibular diagnoses only, 62 had vestibular diagnoses related to concussion, and 73 had concussion only. Vergence deficits were more frequent in patients with vestibular diagnoses and concussion (42%) and concussion only (34%) compared with controls (3%; all P = .02). Accommodation deficits were more frequent in patients with vestibular diagnoses only (67%), vestibular diagnoses and concussion (71%), and concussion (58%) compared with controls (13%; all P = .002). Patients with vestibular migraine and concussion (21) had more vergence deficits (62%) and accommodation insufficiency (52%) than concussion-only patients (47%, P = .02; 29%, P = .04). Patients with benign paroxysmal positional vertigo and concussion (20) had lower positive fusional vergence and failed near vergence facility (35%) more than concussion-only patients (16%; P = .03).Visual function deficits were observed at a high frequency in patients with a vestibular diagnosis with or without a concussion and particularly in vestibular migraine or benign paroxysmal positional vertigo. Visual function assessments may be important for patients with vestibular diagnoses.

This review summarizes the most current information on cause, evaluation and treatment of dizziness in children.There has been an increased understanding of the multifactorial cause of dizziness in the paediatric population. Quantitative vestibular testing is increasingly used and valuable as a diagnostic adjunct. Vestibular rehabilitation, migraine hygiene, psychological therapies, pharmaceuticals and/or surgery can be used as well tolerated and effective treatments for vertigo in children and adolescents when tailored to cause.Paediatric vertigo can be effectively evaluated through careful history taking and physical examination along with adjunctive tests, such as vestibular testing and audiometry, when appropriate. Options for treatment of vestibular disorders in children and adolescents have greatly expanded in recent years allowing for the effective management of nearly all cases of paediatric vertigo, though a multimodal and/or multidisciplinary approach is often needed.

This study aimed to determine the clinical characteristics of benign paroxysmal positional vertigo (BPPV) in young children.Retrospective case review.All children10 years old that have been diagnosed with BPPV at our pediatric vestibular program between December 2012-July 2021 were selected. Clinical features were identified by medical record review, including demographics, comorbidities, canal involvement, response to treatment, and incidence of recurrence.A total of 34 children were diagnosed with BPPV with a mean age of 7.9 years old (SD ± 1/7; range 5-9) at the time of diagnosis and a male:female ratio of 1:1. Involved semicircular canals included posterior in 82% (n = 28), horizontal in 41% (n = 14), and superior in 24% (n = 8) of patients, respectively. Comorbid diagnoses included migraine (n = 14), concussion (n = 10), acute vestibular syndrome (n = 4), and persistent postural perceptual dizziness (n = 6). Recurrence with initially confirmed resolution occurred in 10 patients (29%) with a mean of 2.5 recurrences per patient (SD: 2.2; range 1-8). A family history of vertigo or migraine was identified in 11 and 17 patients, respectively.BPPV is a cause of vertigo in children that may be overlooked. A relatively high proportion of patients demonstrated horizontal or superior canal involvement, recurrence, and additional comorbid causes of dizziness. Thus, providers evaluating young children with dizziness should perform diagnostic maneuvers to evaluate BPPV of all semicircular canals and continue to monitor children after successful treatment for recurrence.4, Case Series Laryngoscope, 2022.

Objective Persistent postural-perceptual dizziness (PPPD) is a recently defined diagnostic syndrome characterized by chronic symptoms of dizziness, unsteadiness, and/or non-spinning vertigo. Although PPPD has been studied in adults, reports in the pediatric population are few. The goal of this study was to describe the presentation and treatment of PPPD in a group of pediatric patients. Study design Retrospective chart review. Setting Tertiary referral center. Patients ≤21 years old, who met Barany Society consensus criteria for a diagnosis of PPPD and were followed for ≥6 months or until symptom resolution. Main outcome measuress Patient demographics, comorbidities, symptom chronicity, and response to treatment(s). Results Of the 53 patients identified, 44 (83.0%) were women. Mean age at the time of initial evaluation was 14.6 years old. Common diagnoses in addition to PPPD included benign paroxysmal positional vertigo (64.2%), vestibular migraine (56.6%), and anxiety (28.3%). A high proportion of patients (43.4%) reported initially missing school or work due to their symptoms. Eighteen patients (34.0%) reported symptom resolution ranging from 2 to 48 months after diagnosis (median 9 mo). Of these patients, 15 of 18 attended physical therapy (PT), 11 of 18 attended cognitive behavioral therapy (CBT) and/or biofeedback therapy, and 10 of 18 took selective serotonin reuptake inhibitor (SSRI) medications, and 7 of 18 (40%) did a combination of all three therapies. Conclusion PPPD can impact patients at a young age, and prolonged symptoms present a significant burden to children and adolescents, many of whom are unable to attend school. Treatments such as PT, CBT, and SSRI medication may be effective.

Background Otoscopy examination can be challenging. Traditional teaching uses still image illustrations. Newer attempts use video samples to simulate the otoscopy exam which is a dynamic process. Aims/Objective: To assess whether recorded otoscopy videos from a smartphone adaptable otoscope can be used to develop a video-based otoscopy quiz which may be used for instructing and familiarizing participants to normal anatomy and pathologic ear conditions. To use this quiz to assess current pediatric residents’ competency of common otoscopy diagnosis Method and Materials This study was conducted in 2018. Video samples of ear pathology were collected at the Albany Medical Center using a smartphone adaptable otoscope- Cellscope. The videos were used to create a video otoscopy quiz (VOQ) without clinical vignettes. 45 pediatric residents from 3 academic institutions were evaluated with the quiz. Results The weighted mean for the VOQ was 66.90% (95%CI 58.89%-68.42%). The breakdown by questions are: myringosclerosis 72.88%, retraction pocket 80.65%, cholesteatoma 42.22%, hemotympanum 75.04%, tympanic membrane perforation 79.62%, cerumen impaction 95.46%, otitis externa 52.54%, otitis media with effusion 63.30%, acute otitis media 75.55%, normal ear 36.39%. Conclusion We found that videos of otoscopy exams can be obtained with a smartphone adaptable otoscope and validated to develop a video-based quiz, which may be used to supplement otoscopic instruction. Following our testing process, we found pediatric residents are relatively well equipped to identify ear pathology on VOQ.

Background: Dizziness after concussion is primarily attributed to effects on the brain, but traumatic inner ear disorders can also contribute. Benign paroxysmal positional vertigo (BPPV) is a common vestibular disorder that can result from minor head trauma and can be easily diagnosed and rapidly treated in an office setting. The role of BPPV in pediatric postconcussive dizziness has not been well-studied. Purpose: To evaluate the prevalence and clinical features of BPPV in a group of pediatric patients with concussion and prolonged dizziness after concussion. Study Design: Case-control study. Level of Evidence: Level 3. Methods: Retrospective review of 102 patients seen within the past 3 years in a pediatric multidisciplinary concussion clinic for evaluation of postconcussive dizziness. Results: BPPV was diagnosed in 29.4% (30/102) of patients with postconcussion syndrome and dizziness. All patients with BPPV were treated with repositioning maneuvers, except for 5 patients who had spontaneous resolution of symptoms. Patients were evaluated at an average of 18.8 weeks (SD, 16.4 weeks) after the injury. BPPV was diagnosed at similar rates regardless of gender or age group (children vs adolescents). The mean Post-Concussion Symptom Scale (PCSS) score did not differ significantly between patients with (58.3 [SD, 22.5]) or without BPPV (55.8 [SD, 29.4]; P = 0.39). The PCSS “balance problems or dizziness” subscore also did not differ between patients with (3.3 [SD, 1.7]) or without BPPV (2.8 [SD, 1.6]; P = 0.13). Conclusion: BPPV is fairly common in pediatric concussion, occurring in one-third of the patients studied. BPPV is often not diagnosed and treated until many weeks after the injury. Increased awareness of the evaluation and management of BPPV among pediatric concussion providers may help expedite resolution of dizziness and hasten overall recovery in affected patients. Clinical Relevance: BPPV is a treatable cause of dizziness caused by minor head injuries and is more common than previously reported in pediatric patients with concussion. Improved awareness of BPPV by concussion providers may expedite recovery.

Introduction Susceptibility to severe motion sickness has not been well described in the pediatric population, particularly in very young children. This study aimed to describe and evaluate risk factors and treatment responses in a group of children with severe motion sickness, including infants and toddlers. Methods We conducted a retrospective review of patients less than 18 years of age seen in our pediatric vestibular program for evaluation of motion sickness over a 6-year period. Results A total of 23 patients with motion sickness were identified. Age of onset ranged from 0 to 15 years old, with a mean age of 6.6 ± 4.2 years. Eleven patients (47.8%) were diagnosed with a migraine variant. Vestibular deficits were identified in four out of 17 patients (23.5%) who underwent formal vestibular testing. Other frequent comorbid conditions included recurrent/chronic otitis media (n = 9; 39.1%) and a history of motor delay (n = 7; 30.4%). A high proportion of patients reported symptom improvement when treated with meclizine, ondansetron, cyproheptadine, or vestibular rehabilitation. Discussion Motion sickness can impact children even in infancy. Common comorbid conditions that may contribute to pediatric motion sickness include migraine disorders, vestibular impairment, otitis media, and motor delay. Treatments such as cyproheptadine and vestibular rehabilitation may be helpful but require further study.

Idiopathic pain at the cochlear implant (CI) site outside of the immediate postoperative period is an uncommon occurrence but may necessitate device explantation. Our objective was to describe the clinical course for pediatric patients with CI site pain who ultimately required device explantation.Retrospective chart review.We performed a retrospective database review of CIs performed at a tertiary referral center for pediatric cochlear implantation. We specifically evaluated pediatric patients who presented with pain at or near the CI device site and ultimately required explantation.Fifteen patients (16 CIs) had pain at or near the CI site requiring device explantation. Cultures taken during site exploration or device explantation identified bacteria in 86% and 81% of procedures, respectively. Propionibacterium acnes and Staphylococcus non-aureus were the most commonly identified organisms.The majority of patients with idiopathic pain in this cohort ultimately requiring CI explantation had chronic bacterial colonization.4 (Case series) Laryngoscope, 132:2044-2049, 2022.

Hearing loss (HL) is the most common sensory deficit in humans and is frequently accompanied by peripheral vestibular loss (PVL). While often overlooked, PVL is an important sensory dysfunction that may impair development of motor milestones in children and can have a significant negative impact on quality of life. In addition, many animal and in vitro models of deafness use vestibular hair cells as a proxy to study cochlear hair cells. The extent of vestibular end organ dysfunction associated with genetic pediatric hearing loss is not well-understood. We studied children with a known genetic cause of hearing loss who underwent routine preoperative vestibular testing prior to cochlear implantation between June 2014 and July 2020. Vestibular testing included videonystagmography, rotary chair, video head impulse testing, and/or vestibular evoked myogenic potentials. Etiology of HL was determined through history, physical examination, imaging, laboratory testing, and/or genetic testing. Forty-four children (21 female/23 male) met inclusion criteria; 24 had genetic non-syndromic and 20 had genetic syndromic forms of HL. Mean age at the time of testing was 2.8 ± 3.8 years (range 7 months−17 years). The most common cause of non-syndromic HL was due to mutations in GJB2 (n = 13) followed by MYO15A (3), MYO6 (2), POU3F4 (2), TMPRSS3 (1), CDH23 (1), TMC1 (1), and ESRRB (1). The most common forms of syndromic HL were Usher syndrome (4) and Waardenburg (4), followed by SCID/reticular dysgenesis (3), CHARGE (2), CAPOS (1), Coffin-Siris (1), Jervell and Lange-Nielsen (1), Noonan (1), peroxisome biogenesis disorder (1), Perrault (1), and Trisomy 21 (1). Overall, 23 patients (52%) had PVL. A larger proportion of children with syndromic forms of HL had PVL (12/20, 60%) compared with children with genetic non-syndromic HL (11/24, 46%), though without statistical significant (p = 0.3). The occurrence of PVL varied by affected gene. In conclusion, PVL is a common finding in children with syndromic and non-syndromic genetic HL undergoing vestibular evaluation prior to cochlear implantation. Improved understanding of the molecular physiology of vestibular hair cell dysfunction is important for clinical care as well as research involving vestibular hair cells in model organisms and in vitro models.

Background Vestibular symptoms such as vertigo and imbalance are known to occur in some cochlear implant patients during the immediate postoperative period; however, acute vertigo in implanted children occurring remotely from the postoperative period has not been previously well-described. Case presentation A three-year-old girl with a history of bilateral sequential cochlear implantation presented with acute labyrinthitis associated with sudden onset of vertigo, balance impairment, and decline in right cochlear implant function 2 years after her most recent implant surgery. We describe her audiological and vestibular testing results during both the acute phase and following medical management and recovery. Conclusion Acute labyrinthitis should be considered when sudden onset vertigo and/or imbalance presents in children with cochlear implants outside of the perioperative period. Such symptoms should prompt early assessment of cochlear implant function, so that the device can be reprogrammed accordingly.

Understand the prevalence of vestibular symptoms in US children.Cross-sectional analysis.2016 National Health Interview Survey.Responses from the 2016 National Health Interview Survey for children ages 3 to 17 years were examined to determine the prevalence of vestibular symptoms and provider-assigned diagnoses.Dizziness or imbalance was reported in 3.5 (95% confidence interval, 3.1-3.9) million patients (5.6%) with a mean age of 11.5 years. Dizziness was reported in 1.2 million patients (2.0%) with a mean age of 12.7 years and balance impairment in 2.3 million patients (3.7%) with a mean age of 10.6 years. Prevalence of dizziness and imbalance did not vary by sex (The national prevalence of childhood vestibular symptoms is more common than previously thought. Reported diagnoses varied greatly from the literature, suggesting a need for increased awareness of causes of vestibular symptoms in children.

Vestibular disorders can be difficult to accurately diagnose and manage. A careful history and focused physical examination are typically adequate to establish a diagnosis and initiate medical treatment. Vestibular testing is an important component of the work-up, but it is particularly essential for patients being considered for surgical intervention for a vestibular disorder, where the testing can be used to more definitively confirm a suspected diagnosis and to determine baseline vestibular organ function. In this article, we will first briefly review key components of the history and physical examination of patients with vestibular complaints. We will then discuss the most commonly used vestibular tests and their role in the preoperative assessment of patients undergoing vestibular surgery, including nystagmography, caloric testing, rotary chair testing, video head impulse testing, and vestibular evoked myogenic potential testing.

Traumatic perilymphatic fistula (PLF) is an uncommon cause of acute vestibular symptoms and hearing loss following head injury in children. We describe the management of 3 pediatric patients with traumatic PLF using an endoscopic ear surgery (EES) approach. Three pediatric patients with traumatic PLF underwent repair via an EES approach between August and October 2018. Patients included a 14-year-old female (oval window), a 13-year-old male (round window), and a 10-month-old male (oval and round window). Ossicular chain injury was identified and repaired in 2 patients. The 10-month-old patient required a second-stage surgery that included lumbar drain placement and a post-auricular, endoscopic-assisted approach due to an especially brisk leak. All patients had complete resolution of vestibular symptoms post-operatively with no recurrence at a mean follow-up of 8.3 months. Traumatic PLF can be safely and effectively diagnosed and managed via an EES approach in children, though an endoscopic-assisted approach may be necessary in select cases due to factors such as patient age and leak severity.

Auditory complaints are commonly reported following traumatic brain injury (TBI). However, few studies have examined patient-reported auditory symptomatology and quality-of-life metrics in individuals with TBI. We hypothesize that following TBI, individuals can experience auditory symptoms even with hearing thresholds in the normal range. Adult patients with normal auditory thresholds and a history of TBI were evaluated for subjective hearing loss, tinnitus, aural fullness, hyperacusis, and autophony. Hearing Handicap Inventory for Adults, Tinnitus Handicap Inventory, and Hyperacusis Questionnaire were administered. Thirty-one patients were prospectively recruited. Twenty-eight TBI participants (90%) reported ≥1 auditory symptoms at the time of survey intake. Mild to severe handicap in the Hearing Handicap Inventory for Adults and Tinnitus Handicap Inventory was reported in 71.4% and 40% of the participants with hearing loss and tinnitus, respectively. Hyperacusis handicap was considered significant in 41.1% of the participants who complained of hyperacusis and completed the survey. Despite normal hearing thresholds, individuals with TBI experience decrements in auditory quality-of-life metrics. Level of evidence: 3.

OBJECTIVES To examine the relative prevalence of individual diagnoses in children and adolescents presenting with dizziness and/or imbalance, and to assess the proportion of patients assigned multiple contributing diagnoses. STUDY DESIGN Retrospective cohort study. METHODS We retrospectively reviewed our internal database of all patients seen at our pediatric vestibular program between January 2012 and March 2019 to determine the incidence of common diagnoses and groups of diagnoses for patients ages 21 or younger. RESULTS One thousand twenty-one patients were included with a mean age of 12.5 ± 4.9 years (range: 9 months-21 years). Of this total, 624 patients were female and 397 were male. Common diagnoses included vestibular migraine (VM; 35.0%), benign paroxysmal positional vertigo (BPPV; 21.6%), primary dysautonomia (15.7%), anxiety disorder (13.5%), and persistent postural perceptual dizziness (PPPD; 11.2%). A high proportion of patients (44.4%) received multiple contributing diagnoses. VM was frequently diagnosed with BPPV or PPPD, and 22 patients were diagnosed with all three concurrently. CONCLUSION The causes of dizziness and imbalance in the pediatric population are diverse, and many patients have multiple diagnoses that are often interrelated. It is important that providers recognize that the causes of vestibular symptoms in children and adolescents may be multifactorial and may span across multiple specialties. LEVEL OF EVIDENCE 4 Laryngoscope, 131:E1308-E1314, 2021.

Objective To examine peak head velocity in video head impulse testing of pediatric patients and its effect on vestibulo-ocular reflex (VOR) gain. Study design Retrospective case review. Setting Tertiary referral center. Patients Pediatric patients ages 3 to 18 years old who underwent video head impulse testing during clinical evaluation of dizziness and/or imbalance. Intervention(s) Conducted video head impulse test (vHIT) in horizontal, left anterior-right posterior (LARP), and right anterior-left posterior (RALP) planes. Main outcome measure(s) Peak head velocity and mean VOR gain for individual semicircular canals in different age groups. Results For the horizontal plane, vHIT peak head velocity greater than 150 degrees/s was achieved in 43 to 63% of 219 patients. In contrast, vHIT peak head velocity greater than 150 degrees/s was achieved in only 3 to 14% for the LARP and RALP planes. Analyses of differences in mean VOR gains among three ranges of peak head velocity (100-125 degrees/s, 126-150 degrees/s, and >150 degrees/s) by paired-t tests were not significant (p > 0.05) within individual patients. Mean VOR gains of three vertical canals in younger patients were significantly lower with higher peak head velocities compared with older children (p Conclusions Peak head velocities of greater than 150 degrees/s may not be feasible in a large proportion of children and adolescents, especially in LARP and RALP planes. However, such high velocities may not be necessary in the pediatric population since VOR gain values obtained at lower peak head velocities of 100 to 150 degrees/s were found to be comparable to those observed at higher velocities.

Objective To review peripheral vestibular disorders in pediatric patients with dizziness following concussion. Study Design Case series with chart review. Setting Pediatric vestibular clinic and pediatric multidisciplinary concussion clinic at a tertiary level pediatric hospital. Subjects and Methods We retrospectively reviewed 109 patients seen for dizziness following a concussion between September 2012 and July 2015. Patients were ≤20 years of age at the time of concussion. Incidences of specific peripheral vestibular disorders were assessed along with timing of diagnosis relative to the date of injury, diagnostic test findings, and treatment interventions associated with those diagnoses. Results Twenty-eight patients (25.7%) were diagnosed with peripheral vestibular disorders. None of these disorders were diagnosed prior to evaluation in our pediatric vestibular clinic or our multidisciplinary concussion clinic, which occurred a mean of 133 days (95% confidence interval, 89.2-177.3) after injury. Benign paroxysmal positioning vertigo was diagnosed in 19 patients, all of whom underwent successful canalith repositioning maneuvers. Other diagnoses included temporal bone fracture (n = 3), labyrinthine concussion (n = 2), perilymphatic fistula (n = 2), and superior semicircular canal dehiscence (n = 2). Both patients with perilymphatic fistula and 1 patient with superior semicircular canal dehiscence underwent successful surgical management, while 1 patient with superior semicircular canal dehiscence was managed nonsurgically. Conclusion Peripheral vestibular disorders may occur in pediatric patients with dizziness following concussion, but these disorders may not be recognized until symptoms have persisted for several weeks. An algorithm is proposed to guide the diagnosis and management of peripheral vestibular disorders in pediatric patients with concussion.

A 15-year-old boy was diagnosed with a cystic parotid mass, which was initially thought to be a first branchial cleft cyst. The mass was treated with antibiotics and fully resolved on examination and imaging. The mass returned, and a superficial parotidectomy was performed to remove the suspected branchial cleft cyst. Final pathology demonstrated a B-cell lymphoblastic lymphoma. Bilateral bone marrow biopsies and peripheral blood counts were negative for any malignancy. This case demonstrates a rare presentation of primary parotid B-cell lymphoblastic lymphoma that began as a fluctuating cystic parotid mass consistent in appearance with a first branchial cleft cyst. Laryngoscope, 128:998-1001, 2018.

OBJECTIVE To describe the collaborative findings across a broad array of subspecialties in children and adolescents with postconcussion syndrome (PCS) in a pediatric multidisciplinary concussion clinic (MDCC) setting. DESIGN Retrospective analysis. SETTING Multidisciplinary concussion clinic at a pediatric tertiary-level hospital. PATIENTS Fifty-seven patients seen in MDCC for evaluation and management of PCS between June 2014 and January 2016. INTERVENTIONS Clinical evaluation by neurology, sports medicine, otolaryngology, optometry, ophthalmology, physical therapy, and psychology. MAIN OUTCOME MEASURES Specialty-specific clinical findings and specific, treatable diagnoses relevant to PCS symptoms. RESULTS A wide variety of treatable, specialty-specific diagnoses were identified as potential contributing factors to patients' postconcussion symptoms. The most common treatable diagnoses included binocular vision dysfunction (76%), anxiety, (57.7%), depression (44.2%), new or change in refractive error (21.7%), myofascial pain syndrome (19.2%), and benign paroxysmal positional vertigo (17.5%). CONCLUSIONS Patients seen in a MDCC setting receive a high number of treatable diagnoses that are potentially related to patients' PCS symptoms. The MDCC approach may (1) increase access to interventions for PCS-related impairments, such as visual rehabilitation, physical therapy, and psychological counseling; (2) provide patients with coordinated medical care across specialties; and (3) hasten recovery from PCS.

To evaluate the association between torticollis and enlarged vestibular aqueduct (EVA).An online/phone survey was administered to parents of 133 children diagnosed with the following disorders: EVA, GJB2 (Connexin 26) mutations associated congenital hearing loss and epistaxis (control). The survey included questions regarding symptoms of torticollis, vertigo, and hearing loss.Patients with EVA had a 10-fold greater odds of having torticollis than controls (31% vs. 4%; OR = 10.6; 95% CI: 2.9, 39.2). No patients with GJB2 had a reported history of torticollis. Torticollis preceded the diagnosis of hearing loss in most (87%) patients with EVA who had a reported history of torticollis. EVA patients were more likely to have reported motor delay than controls (40% vs. 15%; p = 0.002). EVA patients with prior torticollis (80%; 12/15) were more likely to have balance impairment than EVA patients without prior torticollis (12%; 4/33; p 0.001). Twelve patients had a reported history of paroxysmal torticollis, all of whom had EVA.Torticollis in infants may be a marker of EVA. Infants with torticollis should be monitored closely for hearing loss and motor delay, especially when the torticollis is paroxysmal.

Objective Epidemiologic studies have shown Vestibular migraine (VM) to be the most common cause of vertigo in children, but little is known about the typical presentation and response to treatment of this disorder in the pediatric population. The aim of this study was to evaluate the diagnostic features and response to therapy of VM in children managed at a pediatric vestibular clinic. Methods Twenty-eight patients ≤18 years old with a diagnosis of VM were identified from 208 patients seen at the Balance and Vestibular Program at Boston Children's Hospital from July 2012–July 2014, after excluding 12 patients with a history of major otologic or neurologic surgery, recent concussion, or additional vestibular disorders. Patients' electronic medical records and testing results were retrospectively reviewed. Results Patients ranged in age from 9 to 18 years old (mean 14.48). All included patients met criteria for definite (n = 25) or probable (n = 3) VM as defined by the International Classification of Headache Disorders. Rotary chair (n = 17), caloric (n = 8), cervical vestibular evoked myogenic potential (n = 16), and video head impulse (n = 3) tests were normal. Medications effectively reduced reported vestibular symptoms in 88% of those treated with tricyclics (n = 8), 86% of those treated with cyprohepatadine (n = 7), 80% of those treated with topiramate (n = 5), 80% of those treated with triptans (n = 10), and 25% of those treated with gabapentin (n = 4). Conclusions Vestibular migraine is a common cause of vertigo in the pediatric population that is frequently responsive to medical therapy.

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.Results using the ICS Impulse VHIT device in 33 patients20 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.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 of0.7 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 gain0.7 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.).VHIT is an effective test for evaluating semicircular canal function in children and offers major potential advantages over rotary chair and caloric testing.

Objectives/Hypothesis 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. Study Design Prospective, controlled study. Methods Thirty-three children aged 7 to 18 years with (n = 21) and without (n = 12) dizziness underwent static SVV. History, exam, rotary chair, and caloric testing were used to categorize subjects with dizziness into groups with peripheral vestibular loss (PVL), benign paroxysmal positioning vertigo (BPPV), central vertigo (CV), and nonvestibular dizziness (NVD). Results Mean SVV deviation was significantly higher in the peripheral vestibular loss group (n = 4; 2.1 ± 1.5) compared to BPPV (n = 5; 0.5 ± 0.3), CV (n = 7; 0.4 ± 0.3), NVD (n = 5; 0.6 ± 0.4), and control (n = 12; 0.7 ± 0.5) groups by one-way analysis of variance (P = .002). SVV deviation >2° demonstrated a sensitivity of 100%, specificity of 75%, positive predictive value of 100%, and negative predictive value of 97% for PVL. Conclusions SVV is a simple, noninvasive test that provides a valuable contribution to the assessment of peripheral vestibular function in children. Level of Evidence 3b Laryngoscope, 126:727–731, 2016

Objective To analyze spontaneous nystagmus recorded in pediatric patients and determine its diagnostic value. Materials and methods Retrospective review of 86 patients (mean age = 13.9 ± 4.7 years) with definitive spontaneous nystagmus recorded by videonystagmography (VNG). Spontaneous nystagmus was categorized and analyzed based on its characteristics. Correlations between spontaneous nystagmus and established clinical diagnoses were explored. Results Among the patients seen in a period of three years at our pediatric vestibular clinic, 15.4% (86 out of 560) were found to have spontaneous nystagmus and half of them had horizontal nystagmus with a fixed-direction. The majority of cases with spontaneous nystagmus (77%, 66/86) were present without fixation only, while the presence of spontaneous nystagmus with and without fixation was found in 16 patients (19%). Most patients with spontaneous nystagmus without fixation only (68%) were diagnosed with a peripheral vestibular disorder (e.g., vestibular neuritis) or vestibular migraine. In contrast, vertical nystagmus was found in 15 patients, most of whom had central vestibular disorders. Other rare forms of nystagmus or abnormal eye movements, such as pendular nystagmus, ocular oscillation or flutter were recorded in only four patients. Conclusions Spontaneous nystagmus in children and young adults with possible vestibular impairments may vary in clinical presentation. Identification of its characteristics may help to make an accurate clinical diagnosis. While spontaneous nystagmus associated with peripheral vestibular disorders can be readily recognized, spontaneous nystagmus in pediatric patients with vestibular migraine appears to be more complex and variable in form.

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, 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. Language: en

This study aimed to characterize the clinical features and outcomes of benign paroxysmal positional vertigo (BPPV) in the pediatric population.Retrospective case review.Tertiary care center.One hundred ten patients, aged 5 to 19 years old, diagnosed with BPPV.Patient demographics, comorbidities, canal involvement, response to treatment, and incidence of recurrence.BPPV was diagnosed in 19.8% of patients seen for dizziness during the study period. Patient age ranged 5 to 19 years old (mean =13.4 ± 3.4 yr). Female:male ratio was 3:2. The most prevalent comorbidities were concussion (n = 42, 38.2%) and migraine disorders (n = 33, 30.0%). Average time to diagnosis from symptom onset was 178.2 ± 190.8 days. The posterior canal was most frequently affected (n = 80, 72.7%), followed by the lateral canal (n = 37, 33.6%) and superior canal (n = 21, 19.1%), and 36.4% (n = 40) of patients had multiple canals affected. Treatment requiring more than or equal to five maneuvers to achieve resolution was observed in 11.8% of cases (n = 13). Recurrence was observed in 18.2% of cases. A logistic regression analysis demonstrated that patients with vestibular migraine or benign paroxysmal vertigo of childhood had five times higher odds of recurrence of BPPV, p = 0.003, 95% [1.735, 15.342], than those who did not have either.BPPV is a relatively common cause of dizziness in the pediatric population. Children and adolescents with BPPV can be successfully treated with repositioning maneuvers but may be at risk for treatment resistance and recurrence. Increased awareness of BPPV in pediatric patients may reduce delays in identification and treatment.

Introduction Migraine variant disorders of childhood include benign paroxysmal torticollis of infancy (BPTI) and benign paroxysmal vertigo of childhood (BPVC). This study aimed to review our experience with BPTI and BPVC and determine the incidence of children transitioning between each of these disorders and to vestibular migraine (VM). Methods We retrospectively reviewed the medical records of patients seen at the Balance and Vestibular Program at Boston Children's Hospital between January 2012 and December 2016 who were diagnosed with BPTI, BPVC, and/or VM. Results Fourteen patients were diagnosed with BPTI, 39 with BPVC, and 100 with VM. Abnormal rotary chair testing was associated with progression from BPTI to BPVC (n = 8, p = 0.045). Eight (57.1%) patients with BPTI and 11 (28.2%) with BPVC had motor delay. Eleven (78.6%) patients with BPTI and 21 (53.8%) with BPVC had balance impairment. Six BPTI patients developed BPVC (42.9%), six BPVC patients developed VM (15.4%), and two patients progressed through all three disorders (2%). One BPTI patient progressed directly to VM. Discussion Most patients with BPTI will experience complete resolution in early childhood, but some will progress to BPVC, and similarly many patients with BPVC will progress to VM. Parents of children with these disorders should be made aware of this phenomenon, which we refer to as “the vestibular march.” Children with BPTI and BPVC should also be screened for hearing loss, otitis media, and motor delay.

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.Multi-institutional prospective cohort study.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.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, p0.01) and significant nonotologic injury (RR = 2.7, p0.01) 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.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.

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.

Objective To review our institution's 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 children's 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.

Patients commonly present to the otolaryngologist for management of benign facial lesions. It is important to be able to both recognize these lesions and offer patients the most effective therapies currently available. Advances in the understanding and management of three of the more common benign skin lesions affecting the face will be addressed in this review, with a particular emphasis on the most current therapeutic options for each lesion.Actinic keratosis can now be treated with photodynamic therapy or with many topical agents, as alternatives to traditional surgical techniques. Seborrheic keratosis, as well as actinic keratosis and rosacea, are now often treated with laser therapy. In addition, rosacea management now includes nonantimicrobial topical agents, such as azelaic acid, and a wider armamentarium of systemic agents.An understanding of these options will allow the clinician to provide patients with optimal treatment outcomes while minimizing unfavorable side effects.

Background. 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. Methods. We describe the case of an infant with a rapidly enlarging, painless neck mass. MRI revealed a 4-cm-diameter mass deep to the paraspinal muscles, in close proximity to the C2 vertebral foramen. Review of literature, diagnostic methods, and genetics of lipomatous tumors are discussed. Results. Complete surgical excision via a posterior cervical approach demonstrated irregular lobules of immature fat cells separated by a loose, myxoid connective tissue. Histology and genetic analysis confirmed the diagnosis of lipoblastoma. Conclusion. Cervical lipoblastoma is rare, and typically presents as an asymptomatic, painless mass, rarely causing air- way obstruction or nerve compression. MRI can be helpful in identifying the lipomatous nature of the mass, but the findings can be inconsistent due to variable maturity of fat cells and the mesenchymal content of the tumor. Chromosomal analysis is useful in differentiating lipoblastoma from liposarcoma. Recom- mended treatment is complete surgical excision. V C 2007 Wiley Periodicals, Inc. Head Neck 29: 1055-1060, 2007

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.

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.

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 of2° 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.Prospective, controlled study.Thirty-three children aged 7 to 18 years with (n = 21) and without (n = 12) dizziness underwent static SVV. History, exam, rotary chair, and caloric testing were used to categorize subjects with dizziness into groups with peripheral vestibular loss (PVL), benign paroxysmal positioning vertigo (BPPV), central vertigo (CV), and nonvestibular dizziness (NVD).Mean SVV deviation was significantly higher in the peripheral vestibular loss group (n = 4; 2.1 ± 1.5) compared to BPPV (n = 5; 0.5 ± 0.3), CV (n = 7; 0.4 ± 0.3), NVD (n = 5; 0.6 ± 0.4), and control (n = 12; 0.7 ± 0.5) groups by one-way analysis of variance (P = .002). SVV deviation2° demonstrated a sensitivity of 100%, specificity of 75%, positive predictive value of 100%, and negative predictive value of 97% for PVL.SVV is a simple, noninvasive test that provides a valuable contribution to the assessment of peripheral vestibular function in children.3b Laryngoscope, 126:727-731, 2016.

Pediatric germ cell tumors (GCT) of the head and neck are rare lesions with a highly variable presentation. Teratomas are benign GCT that often present at birth, frequently resulting in significant upper airway obstruction and a high risk of mortality. Malignant GCT represent a minority of GCT of the head and neck in the pediatric population and typically respond well to a combination of surgical resection and chemotherapy. This chapter will discuss the pathophysiology and variable clinical presentation of both benign and malignant pediatric GCT of the head and neck as well as the diagnosis and management of these lesions. Particular emphasis is given to the prenatal diagnosis and perinatal management of neonatal teratomas of the head and neck, including prenatal MRI, the ex utero intrapartum treatment (EXIT) procedure, and airway management.

Lipomas of the cerebellopontine angle (CPA) are unusual tumors that typically present with hearing loss, tinnitus, dizziness, and occasionally facial neuropathies. We describe the case of a healthy 42-year-old woman who presented with left-sided hearing loss and facial synkinesis. T1-weighted magnetic resonance imaging revealed an enhancing lesion of the left CPA with no signal on fat suppression sequences. Despite conservative therapy, the patient developed progressive hemifacial spasm, and a suboccipital craniotomy approach was used to debulk the tumor, which encased cranial nerves V, VII, VIII, IX, X, and XI. Surgical histopathology demonstrated mature adipocytes, consistent with lipoma. Two years after surgery, the patient remains free of facial nerve symptoms. Cerebellopontine angle lipomas are rare lesions of the skull base and are reliably diagnosed with T1-weighted and fat suppression magnetic resonance sequences, which we recommend in the routine radiologic workup of CPA tumors. Accurate preoperative diagnosis is crucial because most CPA lipomas should be managed conservatively. Partial surgical resection is indicated only to alleviate intractable cranial neuropathies or relieve brainstem compression.

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.

Introduction:Acquired nasopharyngeal stenosis typically occurs as a result of surgery or irradiation of the nasopharynx. Sarcoidosis has numerous manifestations in the head and neck region, although an association with nasopharyngeal stenosis has not previously been reported.Case report:A 40-year-old man with sarcoidosis developed severe acquired nasopharyngeal stenosis. This was successfully managed with balloon dilatation, followed by pharyngoplasty with local pharyngeal flap reconstruction.Conclusion:This report is intended to prompt consideration of nasopharyngeal stenosis as a potential cause of nasal obstruction in patients with sarcoidosis, and to draw attention to the need to consider sarcoidosis in the differential diagnosis of patients with acquired nasopharyngeal stenosis. We also demonstrate the viability of pharyngoplasty in the management of nasopharyngeal stenosis in the setting of sarcoidosis.