Translational research in aphasia: from neuroscience to neurorehabilitation.

PURPOSE: In this article, the authors encapsulate discussions of the Language Work Group that took place as part of the Workshop in Plasticity/NeuroRehabilitation Research at the University of Florida in April 2005. METHOD: In this narrative review, they define neuroplasticity and review studies that demonstrate neural changes associated with aphasia recovery and treatment. The authors then summarize basic science evidence from animals, human cognition, and computational neuroscience that is relevant to aphasia treatment research. They then turn to the aphasia treatment literature in which evidence exists to support several of the neuroscience principles. CONCLUSION: Despite the extant aphasia treatment literature, many questions remain regarding how neuroscience principles can be manipulated to maximize aphasia recovery and treatment. They propose a framework, incorporating some of these principles, that may serve as a potential roadmap for future investigations of aphasia treatment and recovery. In addition to translational investigations from basic to clinical science, the authors propose several areas in which translation can occur from clinical to basic science to contribute to the fundamental knowledge base of neurorehabilitation. This article is intended to reinvigorate interest in delineating the factors influencing successful recovery from aphasia through basic, translational, and clinical research.     

Dichotic abilities in children, normal adults and aphasic adults for open- and closed-context words

School children (N: 147, distributed across grades from K through 5), 15 normal adults aged 19-30 yrs, and 11 aphasic adults with stabilized aphasia either of traumatic (N: 5) or cerebrovascular (N: 6) origin were used to explore the non-acoustic element of cognition as an influence on dichotic behavior. All Ss were audiometrically normal. Dichotic performances were assessed across age and condition. Pairs of words from the Peabody Picture Vocabulary Test (open-context) and common color names (closed-context) were presented simultaneously to both ears at about 70 db SPL by stereo tapes and earphones. Each pair was adjusted to +/- 3 msec for time-of-onset and +/- 4 db in level. S reported words heard. Performance was assessed for single- and for double-correct responses and for order-of-report (L-first, R-first) bias. Consistent with earlier findings, children showed R-ear-advantage in the single-correct and order-of-report measures for both stimulus sets. Double-correct scores were considerably and significantly higher for all Ss for the closed-context words. The older children (Grade 5) performed at adult levels on the double-correct measure for closed-context words, but not on any measure for the open-context words. The children's order-of-report biases were nearly identical to the normal adults'. As expected, the aphasic subgroups performed poorer than any normal subgroups, except K, and those of traumatic etiology yielded responses generally poorer than those of cerebrovascular origin. From the stabilized aphasic patient data we conclude that the R hemisphere appears to have a compensatory capacity for language acquisition subsequent to L hemisphere damage, and that dichotic testing is sensitive not only in aphasia generally, but can distinguish between certain aphasiogenic categories.     

Facial paralysis in children. A review of 150 cases

The in-patient records at the Hospital for Sick Children were reviewed for the 10-year period 1960 to 1970. All charts in which were coded a primary diagnosis of Bell's palsy, facial paralysis or paresis, or a secondary diagnosis of facial nerve functional disturbance were pulled and reviewed. As there was no out-patient diagnostic index in that period, it proved impossible to identify the numbers or diagnosis of patients with the physical sign of those who were not admitted to the hospital. There were 150 patients in whom a facial nerve disorder was recorded. The distribution and a broad etiological classification is shown in Table I. It will be seen that there were significantly more lower motor neurone than upper motor neurone lesions, although it is likely that many upper motor neurone facial pareses were not recorded because they formed but a small part of a more serious generalized neurological disorder. The upper motor neurone pareses which were recorded had a variety of causes. The infections included meningitis and Guillain-Barré syndrome. Three of the four infective cases made a complete recovery, as did all of the traumatic cases. In one of the hydrocephalic children the facial paresis diminished as the primary lesion responded to treatment. The presence of an upper motor neurone facial paresis is therefore not necessarily a sign of irreversible disease. Trauma due to birth injury, accident or motor vehicle injury was the most common cause of lower motor neurone paresis. The birth injuries usually cleared spontaneously. Bell's palsy was the next most common cause of facial palsy. The sexes were equally affected and the age distributions even throughout childhood. Bell's palsy in children, as in adults, may not clear spontaneously, and should be treated with respect. Facial palsy caused by acute inflammatory disease responds in most instances to antibiotic treatment, although simple mastoidectomy may be necessary. The outcome in this group is excellent. The remarkably few cases of facial palsy complicating chronic middle ear disease are a reflection of the changing pattern of disease. Operative causes were surprisingly mainly due to parotid surgery, and the indication for surgery was always excision of parotid hemangioma. In a 10-year period there were only two facial palsies attributable to temporal bone surgery. There is a need for a detailed prospective study of all children with a facial paralysis and particularly the Bell's palsy group. Only in this way will accurate figures be obtained about the prognosis and thus allow appropriate regimens to be established.     

Otitis media with effusion and chronic upper respiratory tract infection in children: a randomized, placebo-controlled clinical study.

This study was performed to investigate the course of spontaneous recovery from otitis media with effusion in children with chronic rhinosinusitis treated in various ways. One hundred forty-one children between 3 and 10 years of age were selected for the presence of chronic rhinosinusitis and unilateral or bilateral otitis media with effusion. The children were assigned at random to one of four treatment groups, i.e., placebo, amoxicillin combined with xylometazoline hydrochloride nose drops, maxillary sinus drainage, or a combination of the latter two forms of therapy. The follow-up period was 6 months. Drainage of the maxillary sinus had no effect on either the recovery of the chronic upper respiratory tract infection or otitis media with effusion. Amoxicillin combined with xylometazoline nose drops had no significant effect on recovery from the upper respiratory tract infection, but did have a small but significant effect on recovery from otitis media with effusion. However, the general tendency of the upper respiratory tract and ears to recover was poor. Persistence of the chronic upper respiratory tract infection during the follow-up period proved to be a negative prognostic factor with respect to cure of otitis media with effusion. Children with chronic rhinosinusitis as defined in this study appear to have a high risk of developing chronic otitis media with effusion. The results of the study are discussed.     

Inferior turbinectomy in children

BACKGROUND: Inferior turbinectomy on patients of all ages is a controversial procedure. Its effect on children has been reported little in the literature and the few studies that are available involved relatively older children, i.e., >10 years old. Nasal obstruction caused by extensive hypertrophy of the inferior turbinates is not an uncommon observation in the pediatric population. The clinical manifestations might present as snoring, noisy breathing, mouth breathing, and, possibly, sleep apnea. METHODS: In this study, we followed 227 children <10 years of age who underwent inferior turbinectomy (27 children also underwent a revision of an earlier adenoidectomy), of whom 179 children had significant relief of nasal obstruction at the 1-year follow-up. RESULTS: Nocturnal breathing was reported to be more regular and otherwise improved in the 36 children with a suspected history of sleep apnea. Forty-two of 47 children who had thick nasal secretions and did not respond to antibiotic therapy before the operation had significant relief postoperatively. Postoperative complications were few and their number did not exceed that of adults. CONCLUSIONS: A complete inferior turbinectomy should be considered in children <10 years of age who have hypertrophied inferior turbinates that cause major interference with nasal breathing.     

Pediatric facial nerve paralysis: patients, management and outcomes

OBJECTIVE: To characterize the causes and treatment of facial nerve paresis (FNP) in pediatric patients. METHOD: Retrospective study in a tertiary care pediatric hospital. Thirty-four patients identified with partial or complete FNP evaluated between 1997 and 2003. A review of the medical records including sex, age, laterality, etiology, therapy, severity of paralysis according to House-Brackman (HB) six-point grading scale, duration, and degree of recovery. RESULTS: Thirty-five cases of FNP. Causes of FNP were infectious (13), traumatic (7), iatrogenic (5), congenital (4), Bell's/Idiopathic (3), relapsing (2) and neoplastic (1). Peak age distributions for both infectious and traumatic etiologies were bimodal: 1-3 and 8-12 years. Of the 13 infectious cases, 11 were associated with acute otitis media with effusion (AOME). Four (4/11) were bacterial-culture negative. Seven (7/11) were bacterial-culture positive, four (4/7) of which required prolonged, broth-medium culture. Bacteria cultured predominantly included Staphylococcus non-aureus species (5/7) and Propionobacterium acnes (3/7). One (1/13) was viral culture positive (Herpes Simplex Virus). All six patients who received intravenous steroids for OME-associated FNP received the doses within the first week of presentation and had complete recovery (HB I/VI); three of five patients who did not receive steroids had complete recovery. There were five iatrogenic cases; two (2/5) were planned surgical sacrifices and three (3/5) were complications of middle ear/mastoid surgery. Facial nerve function associated with infection returned in 0.5-2 months while, when associated with trauma, returned in 0.25-30 months. CONCLUSIONS: In infectious or traumatic FNP, children aged 1-3 and 8-12 years are the primary groups involved. In AOME FNP, culture-identified organisms may not be representative of traditional pathogens. Infectious FNP averaged 1 month for recovery while traumatic FNP averaged 9 months. Intravenous steroid therapy may improve the outcome. Recovery was complete (HB I/VI) in 8/10 infectious and 4/6 traumatic cases.     

Comparison of the recovery patterns of language and cognitive functions in patients with post-traumatic language processing deficits and in patients with aphasia following a stroke

In this study we investigated the recovery patterns of language and cognitive functions in patients with post-traumatic language processing deficits and in patients with aphasia following a stroke. The correlation of specific language functions and cognitive functions was analyzed in the acute phase and 6 months later.Significant recovery of the tested functions was observed in both groups. However, in patients with post-traumatic language processing deficits the degree of recovery of most language functions and some cognitive functions was higher. A significantly greater correlation was revealed within language and cognitive functions, as well as between language functions and other aspects of cognition in patients with post-traumatic language processing deficits than in patients with aphasia following a stroke.Our results show that patients with post-traumatic language processing deficits have a different recovery pattern and a different pattern of correlation between language and cognitive functions compared to patients with aphasia following a stroke.Learning outcomes: (1) Better understanding of the differences in recovery of language and cognitive functions in patients who have suffered strokes and those who have experienced traumatic brain injury. (2) Better understanding of the relationship between language and cognitive functions in patients with post-traumatic language processing deficits and in patients with aphasia following a stroke. (3) Better understanding of the factors influencing recovery.     

Children with a history of acquired aphasia: residual language and academic impairments

Fifteen children and adolescents with a history of acquired aphasia were administered a battery of language and academic tests, 1-10 years postonset. As a group, these children performed significantly more poorly than non-brain-injured subjects on the language measures, with deficits in word, sentence, and paragraph comprehension; naming; oral production of complex syntactic constructions; and word fluency. One particular language deficit or cluster of deficits did not characterize the group as a whole. For individual brain-injured subjects, language deficits ranged from no or only mild impairment to significant language deficits. All brain-injured subjects were functional verbal communicators at the time of the study; that is, all were oral and primarily used grammatical sentences as their means of communication. Academic difficulties were characteristic of this population. Two thirds of the brain-injured group were receiving academic assistance of some kind at the time of the study. Poor performance on arithmetic calculations was typical. The brain-injured group was heterogeneous with regard to age at onset, etiology, extent of damage, length of recovery, and outcome profiles. Careful and comprehensive assessment of a range of language and academic abilities is essential to adequately identify needs and appropriate intervention strategies for this population.     

Wegener's granulomatosis in childhood. A clinical report based on 3 cases

Wegener's granulomatosis is a very rare disease in children; only 10 cases have been reported in the literature. The present report describes 3 cases where Wegener's granulomatosis developed at an early stage, beginning with upper respiratory tract symptoms. All the 3 children have been successfully treated with a combination of azathioprine and corticosteroids.     

The surgical management of drooling—a 15 year follow-up

Drooling is rarely seen in the normal child after the age of 6 months, but an estimated 10% of children with neurological impairment suffer significant interference with everyday living due to excessive drooling. Submandibular duct relocation is a procedure that involves the dissection and re-routing of the submandibular ducts to the posterior tonsillar pillar. This procedure has been carried out on 53 patients over the past 15 years at the Childrens Hospital, Dublin. All patients have been followed up with a detailed questionnaire to determine symptomatic improvement, parent satisfaction and complications. Parental satisfaction regarding this procedure is high, with 94% of parents stating that their child had benefited from the operation and over half the parents reported complete cessation of all drooling within 3 months of the operation. The major complication of post-operative pneumonia presumed secondary to salivary aspiration occurred in three children. These patients all made a full recovery. Early minor complications occurred in two children, involving post-operative submandibular gland swelling, and the late complication of a ranula was seen in four patients. We believe this is a safe and highly successful procedure that can significantly improve the quality of life of these children.     

Hyaluronic acid and upper airway inflammation in pediatric population: A systematic review

BackgroundInflammatory disease of upper airway in pediatric population represents a social problem for both the pharmaco-economic impact and a burden for the family. To date, the use of topical therapies represents a significant therapeutic progress because they are able to reduce mucosal inflammation and improve tissue healing. Topical Hyaluronic Acid (HA) is a promising molecule that has been recently proposed as adjuvant treatment in the inflammatory disease of the upper aerodigestive tract (UADT) infections.AimsThe aim of our work was to review the published literature regarding all the potential therapeutic effects of HA in the inflammatory disease of upper airway in pediatric population and evaluate the effectiveness of HA, alone or in combination, in children affected by cystic fibrosis.MethodsRelevant published studies about use of HA in UADT in pediatrics were searched in Pubmed, Google Scholar, Ovid using various keywords with no limit for the year of publication. Studies based on the use of HA with nasal packing and with invasive administration of HA were excluded.ResultsAt the end of our selection process, four publications have been included: one of them in children with recurrent upper respiratory tract infections, one of them in children with bacterial acute rhinopharyngitis, two of them in children affected by cystic fibrosis.ConclusionsTopical administration of HA plays a pivotal role in all the children suffering from UADT inflammatory disease, and positive results are generally observed in children with cystic fibrosis.     

Neonatal septoplasty: case report and review of the literature

Treatment of nasal septal deformity in childhood has received growing acceptance in recent years. Traditionally, concern about the role of the septum in the overall growth of the midface has led otolaryngologists to take a very cautious approach to correction of septal deformities in children. However, a great deal of evidence now suggests that severe traumatic septal deviation can and should be corrected early in childhood to prevent future nasal and systemic complications. Closed manipulation of the septum in the first 1–2 days of an infant's life has been performed by many otolaryngologists with good results. The use of this technique, however, is usually limited to those subluxations of anterior cartilage which are diagnosed immediately or very shortly after birth. We present a case of severe traumatic nasal deformity presenting with obstructive asleep and awake apnea and cyanosis at the age of 8 days. The child underwent limited septoplasty using endoscopic techniques at age 14 days with resolution of both the apneic and cyanotic episodes immediately post-operatively. This unusual presentation and the literature surrounding infant nasal/septal surgery are discussed.     

Angiomatous ethmoidochoanal polyp in an infant: case report

Nasal polyps are rare in children younger than 10 years. We describe the case of an infant girl who had undergone a traumatic intubation at birth that had resulted in nasal bleeding. At the age of 5 months, she was brought to us with an obstructive left nasal mass. Imaging revealed the presence of an ethmoidochoanal polyp, as well as a fracture of the posterior cribriform plate and a small associated meningocele. Four months later, the polyp was excised, and the meningocele was corrected with endoscopic nasal surgery. Pathologic evaluation identified the lesion as an angiomatous polyp, which was probably related to the previous traumatic episode. We discuss the clinical aspects of a pathologic entity that has not been previously reported in an infant.     

Congenital high airway obstruction syndrome and airway reconstruction: an evolving paradigm

OBJECTIVES: To refine the classic definition of, and provide a working definition for, congenital high airway obstruction syndrome (CHAOS) and to discuss the various aspects of long-term airway reconstruction, including the range of laryngeal anomalies and the various techniques for reconstruction. DESIGN: Retrospective chart review. PATIENTS: Four children (age range, 2-8 years) with CHAOS who presented to a single tertiary care children's hospital for pediatric airway reconstruction between 1995 and 2000. CONCLUSIONS: To date, CHAOS remains poorly described in the otolaryngologic literature. We propose the following working definition for pediatric cases of CHAOS: any neonate who needs a surgical airway within 1 hour of birth owing to high upper airway (ie, glottic, subglottic, or upper tracheal) obstruction and who cannot be tracheally intubated other than through a persistent tracheoesophageal fistula. Therefore, CHAOS has 3 possible presentations: (1) complete laryngeal atresia without an esophageal fistula, (2) complete laryngeal atresia with a tracheoesophageal fistula, and (3) near-complete high upper airway obstruction. Management of the airway, particularly in regard to long-term reconstruction, in children with CHAOS is complex and challenging.     

Hearing aid management in young children with residual hearing and implications for use of cochlear implants]

Hearing aid fitting in children with profound sensorineural hearing loss (greater than 90 dB) has been regarded as problematic because of the risk of a further hearing aid induced damage. Maximal output levels of 125 dB (SPL) were the upper limit in such cases. We believe, that the danger of a further damage to hearing as a consequence of too high output levels has been overestimated. We therefore offer a strategy which may make it possible, to benefit from high output levels (up to 140 dB (SPL)) and at the same time may reduce the risk of further hearing damage. Furthermore the limit between hearing aid fitting an cochlear implantation is discussed.     

Tracheostomy in human immuno-deficiency virus infected children at the Red Cross War Memorial Children's Hospital, Cape Town, South Africa

Tracheostomy in adults with HIV/AIDS has been reported to be associated with both high and early mortality of 47-100%. There is minimal data regarding the role of tracheostomy in HIV infected children. We did a retrospective analysis of HIV positive children that underwent tracheostomy at our institution over a 5-year period, 2002-2006. A total of 70 tracheostomies were done during the period and 15 (21.4%) of these children were confirmed as HIV infected. The average age at presentation for HIV infected children with upper airway obstruction resulting eventually in tracheostomy was 9.4 months and 60% were under 1 year of age. Only three (20%) were on Anti-Retroviral Therapy (ART) prior to presentation. The cause of upper airway obstruction was croup in 14 (93%) of these 15 children. Following tracheostomy all were treated with ART. To date six children have been successfully decannulated (40%) and there have been three deaths (20%) which were unrelated to tracheostomy. CONCLUSION: Tracheostomy in HIV positive children is not associated with the high mortality that has been reported in adults provided such children are started on treatment with antiretroviral therapy.     

Prognostic factors of sudden hearing loss in children

OBJECTIVE: Sudden hearing loss in children under 15 years old is rare and its pathophysiology remains unclear. The aim of this retrospective study was to define prognostic factors of sudden hearing loss in children under 15 years old. METHODS: Among the patients referred to our institution during the period 1990-1999 for sudden hearing loss, 12 children were considered eligible for this study (mean age=9.5 years old). Patients were divided into three groups according to hearing recovery and seven putative prognostic factors were analyzed. RESULTS: Total hearing recovery (group I: 90-100%) occurred in 28.5% of cases, partial hearing recovery (group II: 11-89%) occurred in 28.5% of cases and absence of recovery (group III: 0-10%) was reported in 43% of cases. Factors such as age, sex, and bilaterality of hearing loss were not correlated with hearing recovery. CONCLUSIONS: Initial severe hearing loss, associated vertigo and 'downward' audiometric curve were three negative prognostic factors of hearing recovery. Tinnitus has only been reported in children with partial or total hearing recovery.     

Methylene blue toxicity following infusion to localize parathyroid adenoma

The parathyroid glands are small, inconspicuous, and variable in number, colour and position. Their identification is vital for excision of hyper-functioning glands and for preservation of normally functioning ones in patients undergoing thyroidectomy. Intravenous infusion of methylene blue at a dose of 7.5 mg/kg is commonly used to aid visualization of the parathyroid glands intra-operatively. Methylene blue is generally considered benign, and there are only two cases published in the literature reporting toxicity following intravenous infusion--such toxicity is a diagnosis of exclusion. We report a case of methylene blue toxicity resulting in expressive aphasia, confusion and disinhibition following infusion for parathyroid adenoma localization. The patient made a complete recovery over 48 hours. Methaemoglobinaemia was excluded as a cause. We suggest that the mechanism of toxicity was a direct effect of methylene blue, although an adverse interaction with serotonin re-uptake inhibitors could not be excluded. In keeping with the UK National Poisons Information Service recommendations, we have altered our practice and now use methylene blue at a dose not exceeding 4 mg/kg. This has not affected our success rate for identification of parathyroid glands. We report this case to highlight the rare occurrence of methylene blue toxicity when used at a dose of 7.5 mg/kg.     

Tympanometric characteristics of Chinese school-aged children

OBJECTIVES: To investigate the tympanometric characteristics of Chinese school-aged children with normal middle ear function. DESIGN: Measurements were made for four tympanometric variables [peak, compensated static acoustic admittance (peak Ytm); equivalent ear canal volume (Vec); tympanometric width (TW); and tympanometric peak pressure] from 278 Chinese children aged between 6 and 15 yrs. Data from the right ear were compared across age groups with those of Chinese young adults and with Western children of comparable ages. Data from the left ear were used to examine specificity using tympanometric screening criteria suggested in the present study. RESULTS: The developmental pattern in tympanometric variables found with the Chinese school-aged children in the study was similar to that found with white children in Western studies. Increasing age was accompanied by an increase in peak Ytm and Vec values, a decrease in TW values, and less negative and less varied tympanometric peak pressure values. The lower limit of peak Ytm 90% range of the Chinese school-aged children in the study was lower and their TW values were wider than those of white children. Age-specific data also suggested that the upper Vec limits of children between 6 and 7 yrs of age differed from those of older children. Racial differences in peak Ytm and TW values were noted, in that the Chinese school-aged children had a lower peak Ytm limit and wider TW values than white children. The use of ASHA 1997 guidelines for identifying ears for referral with respect to Chinese school-aged children may therefore not be highly sensitive and specific. Gender differences noted in peak Ytm and Vec values were too small to be of clinical significance. CONCLUSIONS: To increase the accuracy of tympanometry in determining ears to be referred for further assessment, the use of the tympanometric characteristics observed in the Chinese school-aged children in the present study (i.e., peak Ytm lower limit < 0.2 mmhos and Vec upper limit > 1.5 cm3) should be considered in addition to ASHA 1997 tympanometric screening guidelines.     

Pediatric chronic rhinosinusitis

PURPOSE OF REVIEW: Pediatric sinusitis is prevalent, and the debate continues regarding how best to care for these children. Although acute sinusitis is commonly associated with an upper respiratory infection, the focus of this paper is on chronic rhinosinusitis in children. Research is often more difficult in children than adults, so many times one can learn from the adult literature and determine whether there can be application to the childhood population. RECENT FINDINGS: This paper looks at both medical and surgical treatment of chronic rhinosinusitis. "Maximal medical management" is often cited in the literature, but what this should consist of has never been clearly proved in the literature. Alternative medicine approaches as well as irrigation as an adjunct to care are discussed. Biomaterials are also be discussed. Recent outcome data are put in perspective. SUMMARY: Hopefully the reader will find the presentation stimulating. The paper does not promote surgery as a "cure all," and in the end, analysis will hopefully leave the reader more cautious but with a better understanding of this complex disease.