TTC7A mutation must be considered in patients with repeated intestinal atresia associated with early inflammatory bowel disease: Two new case reports and a literature review.

TTC7A mutations cause multiple neonatal intestinal atresias with early inflammatory bowel disease and severe combined immunodeficiency. There are no treatment protocols for this rare disease. Two new cases are described for which radical early treatment measures - total enterectomy, home parenteral nutrition, immunoglobulin therapy and intravenous antibiotic prophylaxis - have allowed both patients to develop optimally.

Development of auditory asymmetry in transient evoked otoacoustic emissions in pre-term infants.

We previously reported that transient evoked otoacoustic emissions (TEOAEs) continue to develop after the onset of cochlear function in pre-term infants from 34 to 39 weeks of conceptional age (Morlet et al., 1996). The time-course of development differed between genders.

Preoperative speech and pure-tone audiometry in four types of patients with acoustic neuroma.

Purpose: The goal of this study was to compare pure-tone and speech audiometry in 4 groups of patients with confirmed unilateral acoustic neuroma in which auditory brainstem responses and transient-evoked otoacoustic emissions were either both normal, abnormal, or one of the tests was abnormal.

Material And Methods: This study was realized during a preoperative assessment of 65 patients (29 men, 36 women) from 25 to 78 years of age suffering from unilateral acoustic neuroma. The assessment, preceded by tympanometry, included recordings of auditory brainstem responses, transient-evoked otoacoustic emissions, pure-tone audiometry, speech recognition thresholds, and speech discrimination.

Stacked tone-burst-evoked auditory brainstem response (ABR): preliminary findings.

In 1997, Don and his co-workers described a new method for evaluating the auditory brainstem response (ABR)--yielding the stacked-wave-V ABR--which may permit the reliable detection of even small vestibular schwannomas (VSs). However, this method requires a masking technique that may not be readily available to the clinician. Furthermore, relatively high-level noise is required and may be annoying to the patient.

[Systematic screening of deafness at a maternity ward using evoked otoacoustic emissions: practical aspects and parental attitudes].

Objective: Evaluation of the feasibility and parental attitudes towards a hearing screening programme using evoked otoacoustic emissions, implemented in a maternity ward in France.

Methods: A hearing screening test using transiently evoked otoacoustic emissions (TEOAE) was proposed to each baby, and an anonymous questionnaire was given to parents to assess their attitudes towards the screening procedure.

Results: Although the refusal of the test reached 16% (mainly during the first two weeks of the program), more than 92% of parents judged the test as being useful, and 65% wished it to be systematically done.

EEG bispectral index and hypnotic component of anaesthesia induced by sevoflurane: comparison between children and adults.

This prospective study was designed to evaluate the correlation between the electroencephalographic bispectral index (BIS) and the hypnotic component of anaesthesia (CA) induced by sevoflurane in 27 children and 27 adult patients. BIS and CA were compared at loss of consciousness (LOC) and on recovery of consciousness (ROC). Mean (SD) BIS decreased significantly at LOC in children and adults from 94 (2.

[Hearing disorders screening in neonates at risk].

The present report concerns a three year, eight month hearing screening in 1 531 high-risk neonates by means of two successive transient evoked otoacoustic emissions (TEOAE) recordings followed, cin cases of suspected hearing loss, by brainstem auditory evoked potential (BAEP) recording and otolaryngology (ORL) consultation. After TEOAE1 and 2 and BAEP testing, 1 361 infants (88.9%) were declared normal, and 170 (11%) suspected of hearing loss.

Effect of sleep stages on synchronized spontaneous otoacoustic emissions in pre-term neonates.

Objectives: In infants, auditory tests are mainly performed during sleep, since they spend most of their time asleep, and because quiet is required for the duration of the recording session to obtain a precise and reliable response. The aim of this study was to investigate the effect of sleep stages on synchronized spontaneous otoacoustic emissions (sSOAEs) in pre-term neonates at the age where the sleep states begin to be well established and auditory screening can be performed in a neonatology unit before discharge.

Methods: Synchronized SOAEs were repeatedly recorded during a polygraphic sleep recording using the Otodynamic ILO88 system in 10 pre-term neonates at 36 weeks post-conception.

Acuteness of preoperative factors to predict hearing preservation in acoustic neuroma surgery.

Objectives: To determine in patients with acoustic neuromas the predictive factors of hearing preservation according to clinical, radiological, and electrophysiological parameters and to evaluate, for each of these predictive factors, the percentage of patients with preserved hearing.

Study Design: The study involved 107 candidates for hearing preservation attempt. Mean age was 49.

Development of human cochlear active mechanism asymmetry: involvement of the medial olivocochlear system?

To study the functional development of the medial olivocochlear system, transient-evoked otoacoustic emission suppression experiments were conducted in 73 ears of 38 pre-term and 11 full-term neonates. The continuous contralateral stimulation was a broad band white noise, presented at 70 dB SPL. Efferent suppression was determined by subtracting the without-contralateral stimulation condition from the with-contralateral stimulation condition.

Vestibular evoked myogenic potentials in humans: a review.

The human vestibule has preserved an ancestral sound sensitivity and it has been suggested that a reflex could originate from this property, thus inducing cervical muscle microcontractions secondary to strong acoustic stimulations. This reflex is assumed to originate in the saccule, the afferent pathways being either the vestibulocochlear nerve or the inferior vestibular nerve, and the efferent pathways the vestibulospinal tract. Averaging these muscular responses allows vestibular evoked myogenic potentials (VEMPs) to be obtained.

Autoregulation of human inner ear blood flow during middle ear surgery with propofol or isoflurane anesthesia during controlled hypotension.

Unlabelled: We used controlled hypotension to obtain a bloodless cavity during middle ear surgery under an optical microscope. No previous study has assessed the effect of controlled hypotension on inner ear blood flow (IEF) autoregulation in humans receiving propofol or isoflurane anesthesia. In the present study, the IEF autoregulation was determined using laser Doppler flowmetry in combination with transient evoked otoacoustic emissions (TEOAEs) during controlled hypotension with sodium nitroprusside in 20 patients randomly anesthetized with propofol or isoflurane.

Auditory screening in high-risk pre-term and full-term neonates using transient evoked otoacoustic emissions and brainstem auditory evoked potentials.

The present report concerns a 3 year, 8 month hearing screening in 1531 high-risk neonates by means of two successive transient evoked otoacoustic emission (TEOAE) recordings followed, in cases of suspected hearing loss, by brainstem auditory evoked potential (BAEP) recording and otolaryngology (ORL) consultation. After TEOAE 1 and 2 and BAEP testing, 1361 infants (88.9%) were declared normal, and 170 (11.

Effect of anesthesia on transient evoked otoacoustic emissions in humans: a comparison between propofol and isoflurane.

The influence of general anesthesia (GA) on auditory brainstem responses (ABRs) has been widely studied in humans whereas few studies have been devoted as yet to its effect on cochlear micromechanical properties. This study was aimed at evaluating: (1) the effect of GA on transient evoked otoacoustic emissions (TEOAEs) in humans (n=10), and (2) to compare the effects induced by two different anesthetic agents: propofol (n=5) and isoflurane (n=5). The TEOAEs were continuously monitored together with hemodynamic patterns describing various measures of blood pressure.

Is the presence of transient evoked otoacoustic emmissions in ears with acoustic neuroma significant?

One hundred sixty-eight patients admitted for acoustic neuroma removal were involved in this study. In all cases, the size of the tumor and the presence or absence of tinnitus and vertigo or dizziness were evaluated. Investigated functional procedures included bilateral measures of pure-tone audiometry (PTA), auditory brainstem response (ABR), electronystagmography (ENG), and transient evoked otoacoustic emissions (TEOAEs).

Cochleovestibular afferent pathways of trapezius muscle responses to clicks in human.

Brief intense clicks cause short-latency cervical muscles microcontractions which are supposed to be of vestibular origin. Averaging these microcontractions allows myogenic vestibular evoked potentials (MVEP) to be obtained. MVEP from the trapezius muscles were investigated in normal subjects, cochleovestibular nerve-damaged patients and patients with a vestibular or a cochlear lesion.

Myogenic vestibular-evoked potentials in normal subjects: a comparison between responses obtained from sternomastoid and trapezius muscles.

Brief intense clicks cause short latency microcontraction of cervical muscles. Several studies have supported the hypothesis that these microcontractions are of vestibular origin. Averaging these muscular responses enables us to obtain myogenic vestibular evoked potential (MVEP).

[Rotatory impulse test does not replace caloric tests].

We sought to determine whether the rotatory impulsional test was capable of exploring the canalar function with sufficient precision to replace the caloric test, as it has been recently affirmed. We first compared the observed preponderance from this test with that measured during a sinusoidal (20 and 4 s) test. We observed that, in the case of a significant preponderance for a given test, there was complete redondance with the preponderance observed with any other test.

Sympathetic nerve regulation of cochlear blood flow during increases in blood pressure in humans.

The purpose of this work was to show that regulation of the blood flow to the cochlea by the sympathetic nervous system occurs in humans at the level of the cochlear microcirculation during increases in blood pressure and that its involvement depends on the pressure level. Eight anaesthetized patients undergoing tympanoplasty for hearing disease took part in a pharmacological protocol of stimulation and inhibition of the autonomic nervous system (ANS) to provide variations in systolic blood pressure (BPS) and cochlear blood flow (CBF). The CBF was measured by laser-Doppler flowmetry.

Development of cochlear active mechanisms in humans differs between gender.

Despite onset of function early during the third term of gestation, the human auditory system demonstrates continued maturation, thought previously to occur primarily at the neural level. The electromotile properties of outer hair cells appear to contribute substantially to hearing sensitivity and frequency selectivity and lead to the generation of otoacoustic emissions (OAEs). This report demonstrates continued development of cochlear active mechanisms (i.

Clinical interest of brainstem auditory evoked potentials in 72 children with inadequate language development.

The present study is of brainstem auditory evoked potentials (BAEP) in 72 children, of 2 to 13 years of age, showing inadequate language development. The age of the children was mainly below the age of 4 (39 cases = 54%), peaking between 3 and 4 years of age (33 cases = 46%). Even so, 82% of our patients were 3 years old or over: 64% were boys, 36% girls, the proportion of boys falling off with age 26% (19 cases) of BAEPs recorded were normal, 74% (53 cases) pathological.

Transient evoked otoacoustic emissions in nonsurgical ear.

Several studies have reported contralateral hearing deficits following ear surgery. This study aimed to evaluate changes in micromechanical cochlear properties which could occur in the contralateral ear following ear surgery, using transient evoked otoacoustic emission (TEOAE) recording. Surgery involved tympanic membrane surgery in 13 cases and middle ear surgery in 16 cases.

Type of initial brainstem auditory evoked potentials (BAEP) impairment and risk factors in premature infants.

Brainstem auditory evoked potentials (BAEPs) were recorded in 89 premature infants aged between 34 and 52 weeks. 47.2% had normal and 52.