The effect of Tai Chi on balance and functional mobility in children with congenital sensorineural hearing loss.

Background: The aim of the study was to examine the effect of Tai Chi on balance and functional mobility in children with congenital sensorineural hearing loss.Methods: The study included 39 children, aged 10-14 years, with congenital sensorineural hearing loss. The participants were divided into three groups as the Tai Chi group, conventional exercise group, and control group. The Tai Chi group and the conventional exercise group received a 1-h exercise program twice a week for 10 weeks. The balance function of the children was assessed using the Pediatric Balance Scale, the balance subtest of Bruininks-Oseretsky Test 2-Short Form, and the Functional Reach Test. The Timed Up and Go Test and the Timed Up and Down Stairs Test were used to assess functional mobility. The Wilcoxon rank, Kruskal-Wallis. and Mann-Whitney U-tests were used for statistical analyses.Results: When the pre-training values of the groups were compared, with the exception of the Timed Up and Go test, there was no statistically significant difference with respect to demographic data, balance, and functional mobility parameters (p > 0.05). After training, the overall balance and functional mobility tests improved compared to pre-training values in both the Tai Chi and conventional exercise groups (p < 0.05). When the post-training values were compared between the groups, with the exception of the Functional Reach Test and the Timed Up and Down Stairs Test, the results of both exercise groups were superior to those of the control group (p < 0.05).Conclusions: The results of this study indicate that Tai Chi and conventional exercise programs have positive effects on balance and functional mobility in children with congenital sensorineural hearing loss. However, no superiority of Tai Chi or the conventional exercise programs was determined over the other. Both Tai Chi and conventional exercise programs could be used to improve balance and functional mobility in children with congenital sensorineural hearing loss.Implications for rehabilitationTai Chi and conventional exercises are effective on balance in children with congenital sensorineural hearing loss.Tai Chi and conventional exercises are effective on functional mobility in children with congenital sensorineural hearing loss.Tai Chi may be added to the rehabilitation program for children with congenital sensorineural hearing loss.

[Impact of the Overlap Region Between Acoustic and Electric Stimulation]. / Einfluss des Überlappungsbereichs zwischen akustischer und elektrischer Stimulation.

Patients with residual hearing in the low frequencies and ski-slope hearing loss with partial deafness at medium and high frequencies receive a cochlear implant treatment with electric-acoustic stimulation (EAS, "hybrid" stimulation). In the border region between electric and acoustic stimulation a superposition of the 2 types of stimulation is expected. The area of overlap is determined by the insertion depth of the stimulating electrode and the lower starting point of signal transmission provided by the CI speech processor. The study examined the influence of the variation of the electric-acoustic overlap area on speech perception in noise, whereby the width of the "transmission gap" between the 2 different stimulus modalities was varied by 2 different methods. The results derived from 9 experienced users of the MED-EL Duet 2 speech processor show that the electric-acoustic overlapping area and with it the crossover frequency between the acoustic part and the CI should be adjusted individually. Overall, speech reception thresholds (SRT) showed a wide variation of results in between subjects. Further studies shall investigate whether generalized procedures about the setting of the overlap between electric and acoustic stimulation are reasonable, whereby an increased number of subjects and a longer period of acclimatization prior to the conduction of hearing tests deemed necessary.

Hemispheric asymmetries in subcortical visual and auditory relay structures in congenital deafness.

Neuroplasticity - the capacity of the brain to change as a response to internal and external pressures - has been studied from a number of different perspectives. Perhaps one of the most powerful models is the study of populations that have been congenitally deprived of a sense. It has been shown that the right Auditory Cortex (AC) of congenitally deaf humans is neuroplastically modified in order to represent visual properties of a stimulus. One unresolved question is how this visual information is routed to the AC of congenitally deaf individuals. Here, we performed volumetric analysis of subcortical auditory and visual brains regions - namely the thalamus (along with three thalamic nuclei: the pulvinar, the lateral geniculate nucleus and the medial geniculate nucleus), and the inferior and superior colliculi - in deaf and hearing participants in order to identify which structures may be responsible for relaying visual information toward the altered AC. Because there is a hemispheric asymmetry in the neuroplastic changes observed in the AC of the congenitally deaf, we reasoned that subcortical structures that also showed a similar asymmetry in their total volume could have been enlisted in the effort of relaying visual information to the neuroplastically altered right AC. We show that for deaf, but not for hearing individuals, the right thalamus, right lateral geniculate nucleus and right inferior colliculus are larger than their left counterparts. These results suggest that these subcortical structures may be responsible for rerouting visual information to the AC in congenital deafness.

Hearing screening using auditory steady state responses obtained by simultaneous air- and bone-conduction stimuli.

INTRODUCTION AND OBJECTIVES: Minimising false positives rates is an important goal of universal newborn hearing screening programmes. An adequate way for reaching that goal could be differentiating between transient conductive hearing losses (false positives) and permanent sensorineural hearing impairments (true positives) by means of a methodology that studies electrophysiological responses obtained using both air- and bone-conduction stimuli. Our objective was to evaluate the efficiency of an automated hearing screening test based on auditory steady state responses obtained using simultaneous air- and bone-conduction stimuli. METHODS: A sample of 80 high risk babies lees than 2 months of born were screened using the automatic screening test. A confirmatory clinical and electrophysiological evaluation was used as the gold standard. RESULTS: The estimated diagnostic efficiency of this screening test was equivalent (100% sensitivity and 97.7% specificity) to the efficiency reported for otoacoustic emissions and automated auditory brainstem responses. The introduction of bone conduction in the screening reduced the false positive rate from 13.3% to 2.2%. The test duration was 5.3 (± 1.9)min. In 34% of babies only one repetition of the test was needed to raising the result. CONCLUSIONS: The screening test performed quite well in this initial clinical trial, differentiating transient conductive hearing losses from permanent neurosensory impairments and improving the diagnostic efficiency of auditory steady state responses.

A case series of paediatric hearing preservation cochlear implantation: a new treatment modality for children with drug-induced or congenital partial deafness.

OBJECTIVE: To investigate the use of hearing preservation cochlear implantation in children with partial deafness. PATIENTS AND METHODS: Five children with either drug-induced or congenital partial deafness were enrolled in a pilot study. The patients ranged in age from 13 months to 14 years. Implantation was performed using a hearing preservation technique. A Flex EAS electrode (MED-EL, Innsbruck, Austria) was used in all full insertions. RESULTS: Low frequency hearing was preserved in all patients with postoperative bone conduction within 10 dB of the preoperative hearing levels. These changes were preserved over the follow-up period of 12 months. There were significant improvements in speech perception. CONCLUSION: Hearing preservation cochlear implantation is a new effective modality in children with partial deafness.

Outcome of a universal newborn hearing-screening programme based on multiple transient-evoked otoacoustic emissions and clinical brainstem response audiometry.

CONCLUSION: This universal newborn hearing-screening (UNHS) programme revealed high efficacy. The proportion of congenital sensorineural hearing loss was higher in left ears and in males than in right ears and females, which was in line with the systematic ear asymmetries and sex differences in transient-evoked otoacoustic emission (TEOAE) pass percentage. OBJECTIVES: To study the long-term outcome of a UNHS programme based on multiple TEOAEs and clinical click-evoked auditory brainstem response (ABR). METHOD: The study included all the newborns that were screened during a 6-year period (n = 31 092). TEOAE pass/fail was analysed in detail. In an assessment performed 10 years after the start of the 6-year UNHS, prevalence, degree and type of congenital hearing loss were studied. RESULTS: The proportion of screened newborns was high, i.e. 98%. Multiple TEOAE recordings minimized the need for clinical ABR. Fifty-seven (0.18%) subjects showed bilateral hearing loss (exceeding ≈ 30 dB HL); median ABR threshold = 60 dB nHL (at 2.5 months of age). Bilateral and unilateral sensorineural hearing loss was found in 0.17% (n = 52; 56% males) and 0.06% (n = 18; 61% left ears, 56% males) of the screened newborns, respectively. Higher TEOAE pass percentages (p < 0.01) were demonstrated in right ears and in females than in left ears and males.

Risk factors for sensorineural hearing loss in NICU infants compared to normal hearing NICU controls.

OBJECTIVES: To evaluate independent etiologic factors associated with sensorineural hearing loss in infants who have been admitted to the neonatal intensive care unit compared to normal hearing controls. METHOD: Between 2004 and 2009, 3366 infants were admitted to the neonatal intensive care unit of Sophia Children's Hospital, of which 3316 were screened with AABR. A total of 103 infants were referred for auditory brainstem response analysis after failure on neonatal hearing screening. We included all infants diagnosed with sensorineural hearing loss. Each patient was matched with two normal hearing controls from the neonatal intensive care unit of the same gender and postconceptional age. The following risk factors were studied: birth weight, dysmorphic features, APGAR scores (at 1, 5 and 10 min), respiratory distress (IRDS), CMV infection, sepsis, meningitis, cerebral bleeding, cerebral infarction, hyperbilirubinemia requiring phototherapy, peak total bilirubin level, furosemide, dexamethason, vancomycin, gentamycin and tobramycin administration. RESULTS: Fifty-eight infants were diagnosed with sensorineural hearing loss: 26 girls and 32 boys. The incidence of dysmorphic features (P=0.000), low APGAR score (1 min) (P=0.01), sepsis (P=0.003), meningitis (P=0.013), cerebral bleeding (P=0.016) and cerebral infarction (P=0.000) were significantly increased in infants with sensorineural hearing loss compared to normal hearing controls (n=116). CONCLUSION: Dysmorphic features, low APGAR scores at 1 min, sepsis, meningitis, cerebral bleeding and cerebral infarction are associated with sensorineural hearing loss independent of neonatal intensive care unit admittance.

Concomitant hypoparathyroidism, sensorineural deafness, and renal agenesis: a case of Barakat syndrome.

Barakat syndrome, also known as hypoparathyroidism, sensorineural deafness, and renal dysplasia syndrome is an extremely rare congenital disorder. Different etiologies are described for the syndrome but the definite pathophysiology remains unclear. Hereby, we present a case of Barakat syndrome who was diagnosed on the basis of clinical and molecular data.

Hearing threshold estimation in infants using auditory steady-state responses.

Successful early intervention in children with permanent hearing loss requires assessment techniques that can accurately reflect the behavioral audiogram in infancy. This retrospective study compared auditory steady-state response (ASSR) findings from subjects tested in the first three months of life with subsequently obtained behavioral hearing levels. ASSR audiograms were established using amplitude and frequency modulated tones at octave frequencies (500 Hz to 4 kHz). Results obtained from 575 subjects including 285 with normal hearing, 271 with sensorineural hearing loss, and 19 with auditory neuropathy-type hearing loss are presented. ASSR and behavioral hearing thresholds for subjects in the normal and sensorineural groups were highly correlated, with Pearson r values exceeding 0.95 at each of the test frequencies. In contrast, ASSR thresholds in children with AN-type hearing loss did not accurately reflect the behavioral audiogram. Overall, the findings indicate that ASSR testing can offer useful insights into the hearing acuity of children tested in infancy.

Detection of hearing loss in infants.

Permanent Congenital Hearing Impairment (PCHI) is a major cause of delay in speech and language development. Average age of identification of PCHI and subsequent fitting of a hearing aid is currently 18 months. Technology is available to screen successfully infants in the neonatal period for PCHI. Universal screening of all infants prior to discharge has been shown to be the most equitable and efficient method of identifying PCHI. Targeted neonatal screening is a good alternative if universal screening is not currently available.

[The effect of perinatal factors on development of congenital sensorineural hearing loss in children]. / Vliianie perinatal'nykh faktorov na razvitie vrozhdennoi neirosensornoi tugoukhosti u detei.

Combined treatment with hyperbaric oxygenation (HBO), antihypoxants, correctors of microcirculatory, liquor-dynamic and metabolic disorders in the internal ear was used in 99 children aged 1 month to 2.5 years with perinatal neurosensory hypoacusis. A 10-15 dB decrease in auditory thresholds was achieved in 10% of the patients. HBO application in combined treatment of the above patients was pathogenetically validated.

Sensorineural hearing loss alters recovery from short-term adaptation in the C57BL/6 mouse.

Several strains of laboratory mouse (Mus musculus) have a pattern of hearing loss which resembles that found in humans. The C57BL/6 strain of mouse has a genetic defect that results in degeneration of the organ of Corti, originating in the basal, high-frequency region and then proceeding apically over time. The end result is a severe-to-profound sensorineural hearing loss (SNHL) by 14 months of age. In contrast, auditory function of the CBA strain remains normal through its early life span then slowly declines later in life, much like that typified by human presbycusis. The purpose of the present study was to compare ABR (peak 5) forward masking recovery functions in young, normal-hearing CBA and C57BL/6 mice to hearing-impaired C57BL/6 mice. ABR audiograms were obtained prior to collecting the tone-on-tone forward masking data. Masking was defined as a 50% reduction in the P5 component of the ABR, elicited and masked by 12 kHz tone bursts, using masker/probe time delays from 0 to 100 ms. Time constants were computed from an exponential model fit to the recovery functions (masker level vs. time delay). In hearing-impaired animals there was a significant increase in recovery from short-term adaptation as measured by the time constants, as well as a significant latency shift in the P5 component. The effects of SNHL on the recovery of the P5 component from short-term adaptation was comparable to that reported behaviorally for human hearing-impaired listeners and physiologically from the inferior colliculus (IC) of chinchillas suffering permanent threshold shifts.

Síndrome de Waardenburg tipo II: a propósito de dos casos clínicos / Waardenburg's syndrome type II: a propose of 2 clinical cases

El Síndrome de Waardenburg representa la forma más común de sordera congénita; es una condición pleitrópica, autosómica dominante, con penetrancia y expresividad variable. Se describen dos casos clínicos de Síndrome de Waardenburg tipo II. Las principales manifestaciones son la sordera sensorioneural congénita, alteraciones de la pigmentación pilosa y cutánea, puente nasal ancho, hipertricosis de las cejas, mandíbula cuadrada, encanecimiento prematuro y alteraciones neurológicas. Se revisan los criterios de diagnóstico de la enfermedad y los hallazgos asociados descritos de la literatura

Early detection of hearing impairment: what role is there for behavioural methods in the neonatal period?

A survey of the use of behavioural methods for neonatal hearing screening in 1985 (1) concluded that the future for automated methods was quite promising. Since then several studies have assessed the two main automated behavioural tests: the Auditory Response Cradle (ARC) and the Crib-o-Gram (COG). As a screen targeted at neonatal intensive care unit (NICU) babies and other high risk groups (at present the most cost-effective form of neonatal hearing screening), the ARC is shown to have low sensitivity, even for severe hearing impairments, and the COG has an unacceptably low specificity. Any future for behavioural testing during this period must therefore rely on new implementations flowing out of a fundamental understanding of (a) the way in which neonates respond to sound and (b) the ways in which a behavioural test might complement screening with Auditory Brainstem Responses (ABR) or Evoked Oto-acoustic Emissions (EOAE). A clearer understanding of the relative benefits of detecting different degrees of hearing impairment at birth in both the NICU population and the unrestricted population is urgently needed. To determine what role should be played by specific screening programmes such benefits need to be balanced against the total costs of screening assessment and rehabilitation, in which false positives (low specificity) play a large part.