Thierry Morlet

Prevalence of vestibular and balance disorders in children.

Objective: Determine the prevalence of vestibular and balance disorders in children, rate of complaints of imbalance, and odds ratio of related diagnoses. Patients and Methods: Retrospective review of pediatric health system during a 4-year period for International Classification of Diseases, 9th Revision, codes related to balance disorders. Identified records were searched for chief complaints related to balance and for codes of related otologic and neuro-otologic diagnoses. Results: A total of 561,151 distinct patient encounters were found. Unspecified dizziness was diagnosed in 2,283 patients (0.4%). Also, 22% presented with balance complaints. Peripheral disorders were diagnosed in 159, and central disturbances were diagnosed in 109 (prevalence < 0.0002%). Cumulative prevalence of diagnoses related to balance was 0.45% (2,546/561,151). Of all patients, 5,793 (1.03%) had chief complaint related to balance, and 2,076 (35.84%) were also diagnosed with vestibular disorder. Moreover, 38% with peripheral disturbances and 21% with central disturbances had balance complaints. Odds ratio of syncope was 21× higher than the general pediatric population in patients with unspecified dizziness, and sensorineural hearing loss was 43 times higher in those with peripheral vestibular disorders. In patients with central disorders headache was 16× higher (p < 0.05). Conclusion: The prevalence of balance disorders in children is low. Children diagnosed with these disorders typically do not present with chief complaint related to balance. Significant associations exist between sensorineural hearing loss, syncope, and headache in children diagnosed with balance disorders.

Comprehensive vestibular and balance testing in the dizzy pediatric population

Objective. To describe the spectrum of balance disease in a large population of children presenting to a tertiary care vestibular and balance laboratory. Study Design. Case series with chart review. Setting. Tertiary care pediatric hospital. Main Outcome Measures. Results of audiometric, vestibular, and balance tests and final diagnosis. Subjects and Methods. Retrospective review of audiometric, vestibular, balance testing, and final diagnosis from a patient database. Results. Between September 2003 and September 2007, 132 children were evaluated at the Alfred I. duPont Hospital for Children Vestibular Disorders Program. Sixty-nine of the patients were boys and 63 were girls. The average age was 9.7 ± 5.0 years (range, 1-17 years). Although not all were able to complete the entire test battery (99 children completed at least 50% of the tests in the protocol), a diagnosis was achieved in most cases. The most common diagnoses were peripheral vestibulopathy (29.5%), migraine/benign recurrent vertigo of childhood (24.2%), motor/developmental delay (10.6%), traumatic brain injury (9.8%), and central nervous system structural lesion (9.1%). Conclusions. Peripheral vestibular deficits and migraine disease account for most of the pathology in the pediatric population. With a multidisciplinary approach, diagnosis of the source of vertigo and imbalance is possible in most children.

Development of the Vestibular System and Balance Function: Differential Diagnosis in the Pediatric Population

Dizziness is a rare complaint among children. In this article, the authors present the embryology and development of the vestibular system, and offer a rational approach to taking a careful history and ordering and interpreting appropriate vestibular and balance testing in children. A differential diagnosis is presented, so that the likely cause of the balance disorder can be elucidated even in the most complex pediatric patients.

Clinical characteristics of children with cochlear nerve dysplasias

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

Pediatric Cartilage Interleave Tympanoplasty

OBJECTIVE: To describe a novel technique of cartilage tympanoplasty, and review surgical and hearing results in children. STUDY DESIGN AND SETTING: Retrospective chart review of all patients who had undergone tympanoplasty at a pediatric tertiary care hospital from August 2002 to July 2005. Forty-two patients were identified with a minimum follow-up time of 12 months. RESULTS: Mean preoperative perforation size was 21.3 percent (range 10%-90%), and mean patient age was 7.9 years (range 3-16 years). Median clinical follow-up was 24 months. Tympanic membrane closure and graft integration were achieved in 40 of 42 patients (95.2%), and 35 of 42 (85.7%) patients maintained an intact, stable tympanic membrane on long-term follow-up. A total of 93.8 percent of patients achieved a postoperative air-bone gap of less than or equal to 20 dB, and mean improvement in the air-bone gap was 10.7 dB. CONCLUSION AND SIGNIFICANCE: Cartilage interleave tympanoplasty is a versatile, stable, and effective technique for tympanic membrane repair in children.

Vestibular and balance testing in childhood migraine

INTRODUCTION The prevalence of benign recurrent vertigo of childhood (BRVC) and migraine in school-age children is estimated to range from 2% to 10.6%. Both conditions can have a significant impact on health, and in 10% of patients, migraine significantly hinders school attendance and activities of daily living. Seventy percent of children with BRVC experience peripheral vestibular disturbances; however, the pathophysiology of their vertiginous symptoms is unclear. Despite the importance of this clinical issue, documentation of comprehensive vestibular and balance testing in these patients is scant. The aim of this study was therefore to report the audiometric findings and both objective and subjective measures of vestibular and balance testing in pediatric patients diagnosed with migraine, BRVC, or other migraine equivalent.

Hearing loss in children with mitochondrial disorders.

OBJECTIVE At least 1-5 children per 1000 suffer from congenital hearing loss, and 50% of these cases can be attributed to genetic causes. It has been estimated that 1% of pre-lingual hearing loss is due to mutations in mitochondrial DNA. Previous literature reports audiometric data for few patients, usually less than 20 per study. The goal of this study was to characterize the hearing loss associated with mitochondrial mutations and determine whether previously characterized patterns of hearing loss in these patients (progressive, sensorineural, high frequency losses) are found in our population as well. METHODS An IRB-approved retrospective chart review of the electronic medical records in the Nemours/Alfred I. dupont Hospital for Children system from January 2004 to October 2009 (a five-year period) was undertaken using ICD-9 codes 277.87 (mitochondrial disorder) and 359.89BA (mitochondrial myopathy). These 149 records were then evaluated for audiologic data, resulting in 26 charts with both a mitochondrial disorder and hearing evaluation. RESULTS Of 26 patients with known mitochondrial disorders and audiometric documentation, 15 (58%) had hearing loss, and 11 patients had normal hearing (42%). Ten patients had sensorineural hearing loss (38%), two patients had conductive hearing loss (7.7%), one patient had a mixed hearing loss (3.8%), and two patients had an as yet undefined hearing loss (ABR had not yet been performed at the time of this study) (7.7%). CONCLUSION In comparison with previous studies, generally including less than 20 patients, this is one of the largest collections of audiometric data on children with mitochondrial disorders. Unlike prior studies describing a progressive, sensorineural loss across all frequencies or mainly affecting high frequencies, the hearing loss in our patients was more variable including low frequency losses, mid-frequency losses, and conductive losses and was often not progressive or even improved. Our overall 38% rate of sensorineural hearing loss correlates well with previous case series; this study clearly justifies the use of routine audiometric screening in children with mitochondrial disorders, including use of ABR and OAEs as ASND can be seen in this population, as well as repeat testing over time to evaluate for progression.

Ophthalmologic Disorders in Children With Syndromic and Nonsyndromic Hearing Loss

OBJECTIVE To determine the rate of ophthalmologic anomalies among patients with syndromic and nonsyndromic, congenital sensorineural hearing loss (SNHL) to assess the need for comprehensive ophthalmologic evaluation in these children. DESIGN Retrospective medical chart review of children with SNHL who underwent comprehensive evaluation by pediatric ophthalmologists and geneticists. SETTING Tertiary care pediatric hospital. PATIENTS Seventy-seven patients with SNHL. MAIN OUTCOME MEASURES Degree of hearing loss (HL) and presence of ophthalmologic and genetic disorders. RESULTS The overall rate of ophthalmologic disorders was 32% (25 of 77 patients). When children with multisystem genetic disorders known to be related to visual loss were excluded, the rate fell to 23% (12 of 53 vs 13 of 24; P = .006). There was no statistically significant difference in the degree of HL between patients with and without eye disorders (mean [SD], 46.5 [29.9] vs 49.1 [32.3] dB HL; P = .75). Patients with eye disorders were significantly more likely to have a multisystem genetic disorder (13 of 25 [52%] vs 11 of 52 [21%]; P = .006). No patients with ocular abnormalities had isolated otologic disorders, but 9 of 52 (17%) of those patients without ocular abnormalities did. CONCLUSIONS Comprehensive ophthalmologic examination revealed a rate of ophthalmologic disorders in children with SNHL in the lower end of the previously reported rates of 31% to 61%. Children with nonsyndromic SNHL have an approximately 2- to 3-fold increase in ocular abnormalities compared with the general pediatric population. Ophthalmologic and genetic consultations are warranted in patients with congenital SNHL.

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

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

Neurophysiology of hearing in patients with mucopolysaccharidosis type IV

BACKGROUND Hearing impairment is a common problem in patients with mucopolysaccharidosis IV (MPS IV) throughout their life. Many of the adult patients with MPS IV exhibit permanent or severe hearing loss. However, there has been no systematic review of detailed audiological test results in MPS IV. MATERIALS AND METHODS Fourteen individuals with MPS IV (13 MPS IVA and 1 MPS IVB; aged between 12 and 38 years old) participated in the current study. We obtained auditory neurophysiological responses (auditory brainstem responses and otoacoustic emissions test) in addition to pure-tone audiometry and middle ear function tests (tympanometry and acoustic reflexes). RESULTS The results indicated various levels and types of hearing loss with abnormal neurophysiological responses even in those patients with MPS IVA with normal pure tone thresholds. We also found a strong relationship between height (short stature is an indicator of skeletal severity) and hearing sensitivity as well as a strong relationship between height and outer hair cell function in the inner ear (measured by otoacoustic emissions) among MPS IVA patients. CONCLUSION The strong correlation between reduced height and hearing loss indicates that patients with severe skeletal dysplasia may be at higher risk of developing more severe hearing loss. More importantly, the spectrum of hearing disorders indicates that MPS IV patients should have annual neurophysiological hearing tests in addition to audiometric testing from an early age regardless of their skeletal severity to more carefully monitor disease progression.