Longitudinal outcomes of Haemophilus influenzae meningitis in school-age children.

The purpose of this study was to investigate long-term outcomes of Haemophilus influenzae Type b meningitis in a cohort of school-age survivors. Findings from an initial assessment at mean age 10 years revealed neuropsychological, achievement, and behavioral sequelae in the children with neurologic complications during the acute-phase illness (H. Taylor, C. Schatschneider, & D. Rich, 1992). Here, the cohort was reassessed 1 and 2 years after the initial evaluation to investigate age-related influences on disease sequelae. After excluding children with hearing loss, the sample was divided into 2 groups: an affected group of 39 children with acute-phase neurologic complications and an unaffected group of 73 children without these complications. Growth-curve modeling showed poorer outcomes at the final assessment and less rapid improvement at follow-up for the affected group. Later age at assessment and later age at illness were associated with larger group differences in some outcomes. Results suggest that children with diffuse early brain insults are at risk for later-emerging sequelae. (PsycINFO Database Record (c) 2010 APA, all rights reserved)     

Fenestration of porencephalic cysts to the lateral ventricle: experience with a new technique for treatment of seizures

BACKGROUND: Porencephalic cysts are brain cavities resulting from perinatal vascular occlusion and are commonly associated with severe neurological deficits and medically intractable epilepsy. Thirty-seven children presenting to the University of Münster with intractable seizures because of a porencephalic cyst were treated by uncapping and fenestration of these cysts to the lateral ventricle between 1978 and 1992. We conducted the following study to determine the efficacy and safety of the uncapping and fenestration procedure for the treatment of seizures. METHODS: We reviewed all cases retrospectively and assessed the outcome of these patients with regard to seizures, paresis, and perioperative complications. RESULTS: Of 37 children, 23 (62%) were seizure-free postoperatively. Nine patients (24%) had a reduction of seizures and five children (14%) remained unchanged. Of 30 patients with preoperative hemiparesis, 11 (30%) improved after the operation. The leading postoperative problem was a subcutaneous/subgaleal cushion of CSF, which affected 12 children (12 of 37). A dural patch covering the iatrogenic dural defect could not prevent or reduce postoperative CSF leakage, but prolonged the postoperative fever period. Postoperative fever occurred in 36 children (36 of 37) and was caused by an aseptic meningitis. CONCLUSIONS: Children with intractable seizures and porencephalic cysts benefit from uncapping and cyst fenestration to the lateral ventricle. Concomitant perioperative complications are mild and are easily treated.     

Long-term sequelae of pneumococcal meningitis in children

The purpose of this study was to assess the long-term effects of pneumococcal meningitis in children. From 1967 to 1988, a total of 90 children were admitted to the Hospital for Infectious Diseases, Thessaloniki, Greece, with the diagnosis of pneumococcal meningitis. Sixteen patients died in the hospital as a direct result of meningitis. Eleven others were excluded from the study (neurologic deficits prior to onset of meningitis, two; death subsequent to hospitalization, two; recurrent meningitis, seven). Of the remaining 63 survivors, we were able to evaluate 47 patients (75%). Evaluation was performed 4 to 23 years (mean 12.3 +/- 5.8 years) after discharge. Forty patients returned to hospital for evaluation, and seven were evaluated by their primary physicians, who sent information by a standardized questionnaire. The following examinations were carried out: history, physical and neurologic examination, ophthalmologic and hearing evaluation, and psychometric testing. Fourteen patients (30%) had at least one neurologic handicap; nine (19%) had mental retardation, eight (17%) hearing loss, seven (15%) seizure disorder, five (11%) motor defects, and one each (2%) behavioral problems and visual impairment. The presence of coma was the strongest predictor of increased morbidity. The high frequency of long-term sequelae observed in our study supports the need of an effective vaccine.     

Elective cervical discectomy in California: postoperative in-hospital complications and their risk factors

STUDY DESIGN: A retrospective cohort study of short-term outcomes after elective cervical discectomy in California hospitals. OBJECTIVES: To compare the frequency of elective cervical discectomy across population strata, to determine the frequency of adverse outcomes in the early postoperative period, and to identify risk factors for such outcomes. SUMMARY OF BACKGROUND DATA: Previous cervical discectomy series have been too small to analyze risk factors for early complications, and have originated from centers that may not adequately represent the population. METHODS: Computerized hospital discharge abstracts were obtained from the California Office of Statewide Health Planning and Development. Inclusion and exclusion criteria were applied to identify 10,416 routine discectomies at 257 hospitals in 1990-1991. Several categories of postoperative complications were identified, along with inpatient deaths, early reoperations, and nursing home transfers. Logistic regression was used to estimate the independent effects of patient characteristics on short-term outcomes. RESULTS: After adjustment for age and gender, blacks were 51% and Hispanics were 24% as likely as whites to undergo elective cervical discectomy. Overall, 6.7% of patients had one or more reported postoperative complications: 1.8% had noninfectious surgical complications, 1.8% had infectious complications, 4.0% had other medical complications, and 0.35% had unplanned reoperations before discharge. Fourteen inpatient deaths were reported (0.13%). Congestive heart failure, alcohol/drug abuse, chronic lung disease, previous spine surgery, psychological disorders, and chronic musculoskeletal disorders were independently associated with postoperative complications. Even after adjustment, risk was higher with advancing age, higher among women than among men, and higher after posterior fusion than after discectomy without fusion. CONCLUSIONS: The ethnic disparity in cervical discectomy rates suggests overuse among whites or underuse among minority populations. The complication rates reported here are similar to those synthesized from previous literature, except that the lower incidence of neurologic complications reflects our inability to distinguish preoperative from postoperative deficits. Important comorbidities should be identified and treated, if appropriate, before cervical spine surgery.     

Anticarcinogenic effect of palustran on development of tumors induced by 3-(1-alpha-L-arabinopyranosyl)-1-methyl-1-nitrosourea (AMNU) in rats

Clinical manifestations of superior sagittal sinus (SSS) thrombosis are nonspecific but characterized by headache, papilledema, seizures, focal deficits, progressive coma and death. Recurrent transient focal neurologic deficit is an extremely rare manifestation in superior sagittal sinus thrombosis and the mechanism is unknown. A 45-year-old man presented with headache for two weeks and four episodes of transient (5-10 minutes) right or left hemiparesis for two days. Magnetic resonance image and transfemoral cerebral angiography revealed superior sagittal sinus thrombosis with numerous prominent collateral venous channels. There was no parenchymal lesion. After four days of heparinization, no further transient focal neurologic deficits developed. Follow-up angiography showed partial recanalization of the SSS. Possible mechanism of transient ischemic attacks in this patient is thought to be a transient functional disturbance due to a temporal reduction of tissue perfusion in the process of operating fully-enough collateral channels.     

Long-term effect of perinatal and postnatal asphyxia on developing human auditory brainstem responses: peripheral hearing loss

Peripheral hearing was assessed by examining brainstem auditory evoked responses (BAER) in children who suffered perinatal or postnatal asphyxia, particularly in those with residual neurodevelopmental deficits, to investigate the long-term effect of asphyxia on the developing auditory sensitivity. The BAER data were collected at least 6 months after the episode of asphyxia. Of the children who suffered perinatal asphyxia, hearing loss was found more frequently in those who exhibited residual neurodevelopmental deficits (17.1%) than in those who did not (6.3%), but this difference did not reach statistical significance. This implied that the long-term effect of perinatal asphyxia on the peripheral auditory system and its effect on the central nervous system may be relatively discrete or not closely correlated. The occurrence of hearing loss did not appear to be closely related to the degree of perinatal asphyxia, although hearing loss occurred more frequently in the children after severe asphyxia compared to those after mild asphyxia. There was no evidence for permanent hearing loss in the children who survived severe, prolonged postnatal asphyxia and exhibited residual neurodevelopmental deficits. These findings suggest that a critical period of particular sensitivity to the effect of hypoxia may exist during the development of the human peripheral auditory system. This period may range from some time prenatally to some time shortly after birth, probably the third postnatal month. After that, hypoxia is unlikely to lead to permanent hearing loss.     

Clinicopathologic characteristics of replication error-positive gastric carcinoma

OBJECTIVES: To determine the incidence of skull fracture (SF) and intracranial injury (ICA) among children younger than 2 years evaluated in a pediatric emergency department for head trauma; whether historical features and/or physical findings are predictive of injury type; and whether clinical criteria could allow a selective approach to radiographic imaging. DESIGN: Retrospective medical record review. SETTING: Tertiary pediatric emergency department. PATIENTS: Case series of 278 children aged younger than 24 months evaluated for head injury. MAIN OUTCOME MEASURES: Presence of SF and/or ICA. RESULTS: Diagnoses at discharge included 227 minor head injuries, 39 isolated SF, 9 ICA with SF, and 3 isolated ICA. Children younger than 12 months had the highest incidence of SF/ICA (29%) vs 4% for children aged 13 to 24 months (P<.001). Seven percent of complications from SF/ICA resulted from falls 3 ft (0.9 m) or less [corrected]. Incidence of behavioral change, loss of consciousness, emesis, and seizures did not differ significantly between those with minor head injuries and those with SF/ICA. Scalp abnormalities were more common in children with SF/ICA (P<.001). Sixty-two percent of children with isolated SF and 58% of children with ICA had no history of loss of consciousness, emesis, seizure, or behavioral change. Ninety-two percent of children with isolated SF and 75% of children with ICA had normal levels of consciousness and nonfocal neurologic examinations at diagnosis. Among children who fell 3 ft or less (0.9 m) [corrected] and had no loss of consciousness, emesis, seizure, behavioral change, or scalp abnormality, none of 31 (95% confidence interval [CI], 0-0.10) children younger than 24 months and none of 20 (95% CI, 0-0.15) children younger than 12 months had SF/ICA. CONCLUSIONS: Both SF and ICA are common in children younger than 2 years evaluated for head trauma. Children younger than 12 months are at highest risk. Injuries resulted from relatively minor falls and occurred in alert, neurologically normal children. Clinical signs and symptoms were insensitive predictors of SF/ICA; however, a grouping of features (fall < or = 3 ft [0.9 m], no history of neurologic symptoms, and normal scalp physical examination results) identified a subset of children at low risk for complications.     

Neonatal hyperbilirubinemia and long-term outcome: another look at the Collaborative Perinatal Project

OBJECTIVE: To examine the association between neonatal bilirubin levels and subsequent neurodevelopmental outcome. DESIGN: Prospective cohort study. SETTING: 12 US medical centers from 1959 (first births) to 1974 (last follow-up). PARTICIPANTS: 41,324 singleton white or black infants with birth weight > or = 2500 g who had neonatal bilirubin measurements recorded and survived at least 1 year. MAIN OUTCOME MEASURES: Wechsler Intelligence Scale for Children Intelligence Quotient (IQ) at age 7 years, blinded neurologic examination at age 7 years, and sensorineural hearing loss at age 8 years. RESULTS: There was no association between IQ and bilirubin. For example, comparing children who had maximum bilirubin levels > or = 342 mumol/L (20 mg/dL) with those who had lower bilirubin levels, adjusted mean IQs were 105.0 and 103.4 in whites (difference + 1.6; 95% confidence interval [CI]: -0.4 to +3.5) and 91.0 and 93.3 in blacks (difference -2.3; 95% CI: -4.8 to +0.2). Abnormal neurologic examination results were reported in 12 of 268 children (4.5%) with bilirubin > or = 342 mumol/L (20 mg/dL) compared with 1249 of 33,004 children (3.8%) with lower levels (relative risk [RR] = 1.2; 95% CI: 0.7 to 2.1). The frequency of abnormal or suspicious neurologic examination results increased in a stepwise fashion with increasing bilirubin level (P < .001), from 4346/29,258 (14.9%) of those with bilirubin levels < 171 mumol/L (10 mg/dL) to 60/268 (22.4%) of those with bilirubin levels. > or = 342 mumol/L (20 mg/dL), apparently due to increasing minor motor abnormalities at higher bilirubin levels. Sensorineural hearing loss was not associated with high bilirubin levels (RR = 1.0; 95% CI: 0.3 to 3.0). CONCLUSIONS: Neonatal bilirubin levels seem to have little effect on IQ, definite neurologic abnormalities, or hearing loss. Higher bilirubin levels are associated with minor motor abnormalities, but the clinical importance of this finding is limited by the weakness of the association, the mild nature of the abnormalities, and the lack of evidence that they are prevented by treatment.     

Transient focal neurologic deficits associated with hypoglycaemia in children with insulin-dependent diabetes mellitus. Italian Collaborative Paediatric Diabetologic Group

We describe 54 transient focal neurologic deficits (TFND) episodes in 44 children under 18 y observed retrospectively during a 5-y period (1991-96). Mean age and duration of insulin-dependent diabetes mellitus (IDDM) were 8.4 and 3.4 y, respectively. None of the children had a history of seizure disorder and only one had a personal history of migraine. Twenty-nine episodes were characterized by right- and 25 by left-sided hemiparesis. Three of six patients who presented more than one event had alternate episodes of right- and left-sided hemiparesis. On 8 occasions the episode was preceded by a brief convulsion, in 39 it was not witnessed, and in 7 it was certainly absent. Hypoglycaemia (< or = 2.77 mmol/l) was documented on 26 occasions. On 18 of these 26 occasions, the episodes did not resolve promptly after sugar administration. The clinical course was benign, all patients remained neurologically normal and none developed migraine at follow up. Episodes of TFND were associated with hypoglycaemia in the majority of our cases and we do not consider invasive investigations to be mandatory, since the long-term prognosis was invariably good.     

Granulomatous prostatitis on needle biopsy

Sixty-four children with malignant brain tumours diagnosed at less than 3 years of age were reported to the Finnish Cancer Registry from 1975 to 1993. The survival rate has improved significantly: the 5-year survival rate was 26% for all children, 13% for children diagnosed during 1975-85 (n = 30) and 40% for those diagnosed during 1986-93 (n = 34). Of the surviving children in 1986-93, 43% were categorized in Bloom's group I or II and could lead active lives without major disabilities. The remaining children had severe neurologic late complications, such as hemiplegia, intractable seizures, and mental retardation.     

Early neurologic complications following allogeneic bone marrow transplant for leukemia: a prospective study

BACKGROUND: Bone marrow transplant (BMT) is used for both neoplastic and nonneoplastic diseases. Following BMT, particularly during the first 3 months, patients have a number of neurologic complications. We evaluated the early neurologic complications following BMT and their influence on survival. METHODS: We prospectively followed 115 consecutive patients having BMT for leukemia, for a median period of 90 days after transplantation. RESULTS: Sixty-four patients (56%) had neurologic complications. Sixteen developed more than one complication. Twenty-seven patients (25%) had major neurologic complications: metabolic encephalopathy (8), seizures (8), psychiatric symptoms (3), cerebral hemorrhage (1), cerebral abscess (1), leukemic meningitis (1), peripheral neuropathies (5), and myopathies (2). Forty patients (35%) had minor complications, including headache (16) and tremor (31). Major neurologic complications occurred after engraftment in most patients. Metabolic encephalopathy correlated with graft-versus-host disease (GVHD) (p < 0.03). Seven percent of patients had generalized seizures that occurred without signs of structural cerebral lesions. Probability of survival at day 90 was lower in patients with than in those without major central nervous system complications (63% versus 87.5%, p < 0.01). CONCLUSIONS: Neurologic complications are frequent during the first 3 months following BMT and affect patient survival. Drug neurotoxicity and acute GVHD are the main factors influencing their occurrence.     

Family studies and human leukocyte antigen class II typing in Indian probands with seizures in association with single small enhancing computed tomography lesions

PURPOSE: To define the clinical features of the syndrome of seizures associated with single, small, enhancing computed tomography (CT) lesions (SSELs) in 235 Indian probands and seizure types among their family members. Human leukocyte antigen (HLA) class II genomic typing in randomly selected 41 probands was done to identify the role of hereditary factors in this syndrome. METHODS: The seizure types among 235 probands, their clinical outcome, and seizures in their family members were studied. Family data were collected on relatives of 212 additional probands with neurologic diseases other than epilepsy. HLA class II antigens were studied by using polymerase chain reaction (PCR) amplified DNA and sequence-specific oligonucleotide probe (PCR-SSOP) hybridization. RESULTS: The seizures in 86% were partial with or without generalization; 77% had fewer than five seizures before the first CT scan. Evanescent focal neurologic deficits after seizures were noted in 40%. Most patients (97%) were treated with a single antiepileptic drug (AED). Significant resolution of the CT scan lesion was noted within 6 months in 125 (53%) of 235 cases. Two thirds of patients had no seizures while taking a single AED, and an additional 18% had no seizures even after their AEDs were discontinued. Epilepsy among relatives of Indian probands having seizures in association with SSELs was more common as compared with relatives of probands with other neurologic diseases. A family history of seizures was noted in 21% probands, the ratio of affected first- to second-degree relatives was 4.3:1, and 60% of affected sibs had syndromic concordance with probands. There was a positive association of HLA-DRB1*13 (Pc = 0.036) with this syndrome. CONCLUSIONS: The syndrome of seizures in association with SSELs seems to be a benign localization-related epileptic syndrome. Our results of HLA studies point to an inherited susceptibility to an infective agent, which in most cases is of cysticercal etiology.     

Hearing capacity and speech production in 417 children with facial cleft abnormalities

Children with cleft palates often suffer from chronic conductive hearing losses, delayed language acquisition and speech disorders. This study presents results of speech and language outcomes in relation to hearing function and types of palatal malformations found. 417 children with cleft palates were examined during followup evaluations that extended over several years. Disorders were studied as they affected the ears, nose and throat, audiometry and speech and language pathology. Children with isolated cleft lips were excluded. Among the total group, 8% had normal speech and language development while 92% had speech or language disorders. 80% of these latter children had hearing problems that predominantly consisted of fluctuating conductive hearing losses caused by otitis media with effusion. 5% had sensorineural hearing losses. Fifty-eight children (14%) with rhinolalia aperta were not improved by speech therapy and required velopharyngoplasties, using a cranial-based pharyngeal flap. Language skills did not depend on the type of cleft palate presents but on the frequency and amount of hearing loss found. Otomicroscopy and audiometric follow-ups with insertions of ventilation tubes were considered to be most important for language development in those children with repeated middle ear infections. Speech or language therapy was necessary in 49% of the children.     

The unrecognized epidemic of blunt carotid arterial injuries: early diagnosis improves neurologic outcome

OBJECTIVE: To determine the benefit of screening for blunt carotid arterial injuries (BCI) in patients who are asymptomatic. SUMMARY BACKGROUND DATA: Blunt carotid arterial injuries have the potential for devastating complications. Published studies report 23% to 28% mortality rates, with 48% to 58% of survivors having permanent severe neurologic deficits. Most patients have neurologic deficits when the injury is diagnosed. The authors hypothesized that screening patients who are asymptomatic and instituting early therapy would improve neurologic outcome. METHODS: The Trauma Registry of the author's Level I Trauma Center identified patients with BCI from 1990 through 1997. Beginning in August 1996, the authors implemented a screening for BCI. Arteriography was used for diagnosis. Patients without specific contraindications were anticoagulated. Endovascular stents were deployed in the setting of pseudoaneurysms. RESULTS: Thirty-seven patients with BCI were identified among 15,331 blunt-trauma victims (0.24%). During the screening period, 25 patients were diagnosed with BCI among 2902 admissions (0.86%); 13 (52%) were asymptomatic. Overall, eight patients died, and seven of the survivors had permanent severe neurologic deficits. Excluding those dying of massive brain injury and patients admitted with coma and brain injury, mortality associated with BCI was 15%, with severe neurologic morbidity in 16% of survivors. The patients who were asymptomatic at diagnosis had a better neurologic outcome than those who were symptomatic. Symptomatic patients who were anticoagulated showed a trend toward greater neurologic improvement at the time of discharge than those who were not anticoagulated. CONCLUSIONS: Screening allows the identification of asymptomatic BCI and thereby facilitates early systemic anticoagulation, which is associated with improved neurologic outcome. The role of endovascular stents in the treatment of blunt traumatic pseudoaneurysms remains to be defined.     

Long-term outcomes after radiosurgery for acoustic neuromas

BACKGROUND: Stereotactic radiosurgery is the principal alternative to microsurgical resection for acoustic neuromas (vestibular schwannomas). The goals of radiosurgery are the long-term prevention of tumor growth, maintenance of neurologic function, and prevention of new neurologic deficits. Although acceptable short-term outcomes have been reported, long-term outcomes have not been well documented. METHODS: We evaluated 162 consecutive patients who underwent radiosurgery for acoustic neuromas between 1987 and 1992 by means of serial imaging tests, clinical evaluations, and a survey between 5 and 10 years after the procedure. The average dose of radiation to the tumor margin was 16 Gy, and the mean transverse diameter of the tumor was 22 mm (range, 8 to 39). Resection had been performed previously in 42 patients (26 percent); in 13 patients the tumor represented a recurrence of disease after a previous total resection. Facial function was normal in 76 percent of the patients before radiosurgery, and 20 percent had useful hearing. RESULTS: The rate of tumor control (with no resection required) was 98 percent. One hundred tumors (62 percent) became smaller, 53 (33 percent) remained unchanged in size, and 9 (6 percent) became slightly larger. Resection was performed in four patients (2 percent) within four years after radiosurgery. Normal facial function was preserved in 79 percent of the patients after five years (House-Brackmann grade 1), and normal trigeminal function was preserved in 73 percent. Fifty-one percent of the patients had no change in hearing ability. No new neurologic deficits appeared more than 28 months after radiosurgery. An outcomes questionnaire was returned by 115 patients (77 percent of the 149 patients still living). Fifty-four of these patients (47 percent) were employed at the time of radiosurgery, and 37 (69 percent) remained so. Radiosurgery was believed to have been successful by all 30 patients who had undergone surgery previously and by 81 (95 percent) of the 85 who had not. Thirty-six of the 115 patients (31 percent) described at least one complication, which resolved in 56 percent of those cases. CONCLUSIONS: Radiosurgery can provide long-term control of acoustic neuromas while preserving neurologic function.     

Consequences of neonatal seizures in the rat: morphological and behavioral effects

Whereas neonatal seizures are a predictor of adverse neurological outcome, there is controversy regarding whether seizures simply reflect an underlying brain injury or can cause damage. We subjected neonatal rats to a series of 25 brief flurothyl-induced seizures. Once mature the rats were compared with control littermates for spatial learning and activity level. Short-term effects of recurrent seizures on hippocampal excitation were assessed by using the intact hippocampus formation preparation and long-term effects by assessing seizure threshold. Brains were analyzed for neuronal loss, sprouting of granule cell axons (mossy fibers), and neurogenesis. Compared with controls, rats subjected to neonatal seizures had impaired learning and decreased activity levels. There were no differences in paired-pulse excitation or inhibition or duration of afterdischarges in the intact hippocampal preparation. However, when studied as adults, rats with recurrent flurothyl seizures had a significantly lower seizure threshold to pentylenetetrazol than controls. Rats with recurrent seizures had greater numbers of dentate granule cells and more newly formed granule cells than the controls. Rats with recurrent seizures also had sprouting of mossy fibers in CA3 and the supragranular region. Recurrent brief seizures during the neonatal period have long-term detrimental effects on behavior, seizure susceptibility, and brain development.     

Children with minimal sensorineural hearing loss: prevalence, educational performance, and functional status

OBJECTIVE: This study was designed to determine the prevalence of minimal sensorineural hearing loss (MSHL) in school-age children and to assess the relationship of MSHL to educational performance and functional status. DESIGN: To determine prevalence, a single-staged sampling frame of all schools in the district was created for 3rd, 6th, and 9th grades. Schools were selected with probability proportional to size in each grade group. The final study sample was 1218 children. To assess the association of MSHL with educational performance, children identified with MSHL were assigned as cases into a subsequent case-control study. Scores of the Comprehensive Test of Basic Skills (4th Edition) (CTBS/4) then were compared between children with MSHL and children with normal hearing. School teachers completed the Screening Instrument for Targeting Education Risk (SIFTER) and the Revised Behavior Problem Checklist for a subsample of children with MSHL and their normally hearing counterparts. Finally, data on grade retention for a sample of children with MSHL were obtained from school records and compared with school district norm data. To assess the relationship between MSHL and functional status, test scores of all children with MSHL and all children with normal hearing in grades 6 and 9 were compared on the COOP Adolescent Chart Method (COOP), a screening tool for functional status. RESULTS: MSHL was exhibited by 5.4% of the study sample. The prevalence of all types of hearing impairment was 11.3%. Third grade children with MSHL exhibited significantly lower scores than normally hearing controls on a series of subtests of the CTBS/4; however, no differences were noted at the 6th and 9th grade levels. The SIFTER results revealed that children with MSHL scored poorer on the communication subtest than normal-hearing controls. Thirty-seven percent of the children with MSHL failed at least one grade. Finally, children with MSHL exhibited significantly greater dysfunction than children with normal hearing on several subtests of the COOP including behavior, energy, stress, social support, and self-esteem. CONCLUSIONS: The prevalence of hearing loss in the schools almost doubles when children with MSHL are included. This large, education-based study shows clinically important associations between MSHL and school behavior and performance. Children with MSHL experienced more difficulty than normally hearing children on a series of educational and functional test measures. Although additional research is necessary, results suggest the need for audiologists, speech-language pathologists, and educators to evaluate carefully our identification and management approaches with this population. Better efforts to manage these children could result in meaningful improvement in their educational progress and psychosocial well-being.     

Developmental outcomes in children receiving resection surgery for medically intractable infantile spasms

Two-year postsurgical developmental outcomes were assessed in 24 children with infantile spasms who underwent resective surgery. The mean age of onset of infantile spasms was 12.0 weeks and the mean age at surgery was 20.8 months. Developmental outcomes were assessed using the Vineland Adaptive Behavior Scales (VABS). There was a significant increase in developmental level at 2 years postsurgery compared with presurgical levels. At 2 years postsurgery only one of the children in this series was severely retarded. The developmental outcomes of patients in the series were better than those in prior studies of symptomatic patients receiving medical treatment for infantile spasms. It is surprising that the children in the UCLA series frequently had developmental outcomes equal to and sometimes superior to other groups of children with infantile spasms, since all the UCLA patients were symptomatic, had neurologic deficits and had failed to respond to adrenocorticotrophic hormone (ACTH) and antiepileptic drugs. The 2-year postsurgery developmental outcomes were best for the children who received surgery when they were relatively young and who had the highest level of developmental attainments presurgically.     

Late presentation of biotinidase deficiency with acute visual loss and gait disturbance

An unusual presentation of biotinidase deficiency is described. The disorder classically presents in infancy or early childhood with intractable seizures, hypotonia, ataxia, hearing loss, dermatitis, and alopecia. A 5-year-old girl developed acute visual loss associated with optic atrophy, and disturbance of gait with predominantly lower-limb pyramidal signs. She had no seizures, and skin, hair, hearing, and intellect were normal. Biotinidase deficiency was confirmed biochemically and she responded well to biotin therapy. A diagnosis of biotinidase deficiency should be considered in children with unexplained bilateral optic neuropathy, particularly when there is accompanying gait disorder.