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.