Vestibular Preservation in Pediatric Cochlear Implantation.

OBJECTIVES: Evaluate the rate of preserved vestibular function in pediatric cochlear implant surgery. STUDY DESIGN: Retrospective case review. METHODS: Pre- and post-operative vestibular tests were compared in children who underwent cochlear implantation at a tertiary level pediatric hospital over a 4-year period. RESULTS: Data from 59 implanted ears in 44 children was included. Median age was 2.8 years at initial testing (range 7 months - 21 years) with 1:1 male/female ratio. Implant surgeries were 26 unilateral, 13 bilateral simultaneous, and 5 bilateral sequential. The majority were implanted with slim, non-styletted electrodes (86.4%) via a round window approach (91.5%). Normal pre-operative results were preserved post-operatively on rotary chair testing in 75% (21/28) of patients, cervical vestibular evoked myogenic potential testing in (75%) 30/40 of ears tested, ocular vestibular evoked myogenic potential testing in 85.7% (6/7) of ears tested, video head impulse testing in 100% (9/9) of ears tested, and computerized dynamic posturography in 100% (5/5) of patients tested. Overall, 62.5% of patients had no new deficits on any vestibular test performed post-operatively. CONCLUSIONS: Preservation rates of vestibular function following cochlear implant surgery were higher in this cohort than what has been reported in many earlier studies. Contemporary, less traumatic electrodes and insertion techniques may be a significant factor. The risk of causing a new, severe bilateral vestibular loss with long-term functional impacts appears to be low. Further study is warranted on the impacts of different cochlear implant electrode designs and insertion approaches on post-operative vestibular preservation. LEVEL OF EVIDENCE: 4, Case Series Laryngoscope, 134:1913-1918, 2024.

Benign paroxysmal vertigo of childhood.

Benign paroxysmal vertigo of childhood (or recurrent vertigo of childhood) is the most common cause of vertigo in young children. It is considered a pediatric migraine variant or precursor disorder, and children with the condition have an increased likelihood of developing migraine later in life than the general population. Episodes are typically associated with room-spinning vertigo in conjunction with other migrainous symptoms (e.g. pallor, nausea, etc.), but it is rarely associated with headaches. Episodes typically only last for a few minutes and occur with a frequency of days to weeks without interictal symptoms or exam/test abnormalities. Treatment is rarely necessary, but migraine therapy may be beneficial in cases where episodes are particularly severe, frequent, and/or prolonged. An appreciation of the typical presentation and characteristics of this common condition is essential to any provider responsible for the care of children with migraine disorders and/or dizziness. This chapter will review the current literature on this condition, including its proposed pathophysiology, clinical presentation, and management. This chapter also includes a brief introduction to pediatric vestibular disorders, including relevant anatomy, physiology, embryology/development, history-taking, physical examination, testing, and a review of other common causes of pediatric dizziness/vertigo.

Evidence of vestibular dysfunction in children with enlarged vestibular aqueduct.

OBJECTIVE: To investigate the occurrence and characteristics of balance and vestibular deficits in pediatric patients with enlarged vestibular aqueduct (EVA). MATERIALS AND METHODS: Retrospective review of 53 children with EVA who underwent a comprehensive vestibular evaluation in our pediatric balance and vestibular program. Laboratory testing included videonystagmography (VNG), rotary chair, video head impulse testing (vHIT), vestibular evoked myogenic potential (VEMP), subjective visual vertical (SVV) and Sensory Organization Test (SOT) in posturography. RESULTS: The mean age of these children, 31 girls and 22 boys, was 7.1 years (SD = 4.8). Among these 53 children, 16 had unilateral EVA (7 on the left side and 9 on the right side) and 37 had bilateral EVA, in which genetic testing confirmed 5 cases of Pendred syndrome. Abnormal testing results were found in 58% (11/19) on SOT, 67% (32/48) on rotary chair, 55% (48/88 of ears) on VEMP, 30% (8/27) on vHIT, 39% (7/18) on SVV, and 8% (4/53) on VNG. CONCLUSIONS: Vestibular dysfunction may be a common finding in children with EVA. Clinicians who provide medical care for children with EVA need to be familiar with signs of potential balance and vestibular impairments. Although performing vestibular evaluation on young children with EVA can be difficult, objective testing is important in order to identify any potential vestibular deficit in these pediatric patients so that proper vestibular rehabilitation and balance retraining can be provided.

Characteristics of Benign Paroxysmal Positional Vertigo in Young Children.

OBJECTIVES: This study aimed to determine the clinical characteristics of benign paroxysmal positional vertigo (BPPV) in young children. STUDY DESIGN: Retrospective case review. METHODS: All children <10 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. RESULTS: 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. CONCLUSIONS: 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. LEVEL OF EVIDENCE: 4, Case Series Laryngoscope, 133:694-699, 2023.

Vergence and Accommodation Deficits in Children and Adolescents with Vestibular Disorders.

SIGNIFICANCE: The high frequency of vergence and accommodation deficits coexisting in patients with a vestibular diagnosis merits a detailed visual function examination. PURPOSE: 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. METHODS: 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. RESULTS: 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). CONCLUSIONS: 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.

Evaluation and management of paediatric vertigo.

PURPOSE OF REVIEW: This review summarizes the most current information on cause, evaluation and treatment of dizziness in children. RECENT FINDINGS: 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. SUMMARY: 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.

Pain at the Cochlear Implant Site Requiring Device Removal in Pediatric Patients.

OBJECTIVES: 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. STUDY DESIGN: Retrospective chart review. METHODS: 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. RESULTS: 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. CONCLUSIONS: The majority of patients with idiopathic pain in this cohort ultimately requiring CI explantation had chronic bacterial colonization. LEVEL OF EVIDENCE: 4 (Case series) Laryngoscope, 132:2044-2049, 2022.

Specialty-Specific Diagnoses in Pediatric Patients With Postconcussion Syndrome: Experience From a Multidisciplinary Concussion Clinic.

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.

Peripheral Vestibular Dysfunction Is a Common Occurrence in Children With Non-syndromic and Syndromic Genetic Hearing Loss.

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.

Persistent Postural-Perceptual Dizziness in Children and Adolescents.

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 Bárány 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.

Endoscopic Repair of Traumatic Perilymphatic Fistula in Children: A Case Series.

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.

Development of video otoscopy quiz using a smartphone adaptable otoscope.

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.

Benign Paroxysmal Positional Vertigo in Children and Adolescents With Concussion.

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.

Multifactorial Characteristics of Pediatric Dizziness and Imbalance.

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.

Severe motion sickness in infants and children.

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.

Auditory Quality-of-Life Measures in Patients With Traumatic Brain Injury and Normal Pure Tone Audiometry.

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.

Histopathological changes to the peripheral vestibular system following meningitic labyrinthitis.

OBJECTIVE: While cochlear ossification is a common sequalae of meningitic labyrinthitis, less is known about the effects of meningitis on peripheral vestibular end organs. Herein, we investigate histopathologic changes in the peripheral vestibular system and cochlea in patients with a history of meningitic labyrinthitis. METHODS: Temporal bone (TB) specimens from patients with a history of meningitis were evaluated and compared to age-matched controls. Specimens were evaluated by light microscopy and assessed for qualitative changes, including the presence of vestibular and/or cochlear endolymphatic hydrops, presence and location of inflammatory cells, new bone formation, and labyrinthitis ossificans; and quantitative changes, including Scarpa's ganglion neuron (ScGN) and spiral ganglion neuron (SGN) counts. RESULTS: Fifteen TB from 10 individuals met inclusion and exclusion criteria. Presence of inflammatory cells and fibrous tissue was found in 5 TB. Of these, evidence of labyrinthitis ossificans was found in 2 TB. In the peripheral vestibular system, mild to severe degeneration of the vestibular membranous labyrinth was identified in 60% of cases (n = 9 TBs). There was a 21.2% decrease (range, 3%-64%) in the mean total count of ScGN in patients with meningitis, compared to age-matched controls. In the cochlea, there was a 45% decrease (range, 25.3%-80.9%) in the mean total count of SGN compared to age-matched controls (n = 14 TBs). CONCLUSIONS: Otopathologic analysis of TB from patients with a history of meningitic labyrinthitis demonstrated distinct peripheral vestibular changes. Future research may help to delineate potential mechanisms for the observed otopathologic changes following meningitis. LEVEL OF EVIDENCE: N/A.

Evolution of Cochlear implant mapping and vestibular function in a pediatric case of Labyrinthitis.

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.

Torticollis in children with enlarged vestibular aqueducts.

OBJECTIVES: To evaluate the association between torticollis and enlarged vestibular aqueduct (EVA). METHODS: 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. RESULTS: 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. CONCLUSION: 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.