Clinical Decision Making for Intraoperative Auditory Brainstem Response Testing in Children following Tympanostomy Tube Placement.

Intraoperative auditory brainstem response (ioABR) testing following tympanostomy tube (TT) placement may be biased due to temporary threshold shifts (TTS). The purpose of the study was to assess the evidence for TTS in children who have undergone ioABR using prolonged latencies of wave I (males > 1.95 ms, females > 1.88 ms) as a marker of a persisting air-bone gap. Eighty-three children underwent ioABR following surgical procedures at University Hospital Bonn, Germany. The primary outcome measure was the latency of wave I at 80-dB SPL. The total sample consisted of 66 males (79.5%) and 17 females (20.5%) with a mean (SD) age of 46.4 (26.6) months. Of 163 operated ears (83 children), 72 (44.2%) had no middle ear fluid, 19 (11.6%) serous fluid, and 72 (44.2%) mucoid fluid. The risk of having a prolonged latency of wave I at 80-dB SPL was OR 4.61 (95% CI 2.01-10.59; p < 0.001) in those with mucoid fluid as compared to those without mucoid fluid. Intraoperative ABR results should account for sex differences and be interpreted with caution and be verified. Ultimately, parents should be engaged in a preoperative discussion to decide if an ioABR should be postponed if mucoid fluid was found.

[Identification of speaker characteristics by listener groups of different ages and expertise]. / Identifizierung von Sprechermerkmalen durch Zuhörer unterschiedlicher Altersstufen und Expertise.

OBJECTIVE: In this study we investigated to what extent listeners can identify some unknown speakers' characteristics by listening only to their voice recordings (physical characteristics, accent/dialect, smoking habits, level of education, personality, outer appearance). Several listener groups of different ages and expertise took part. The aim of the study was to compare the voice perception of children/adolescents and adults as well as naive listeners and phonetically trained listeners. MATERIAL AND METHODS: A total of 197 subjects, divided into 4 groups (primary school pupils, secondary school pupils, university students, specialists in phoniatrics and pediatric audiology), listened to voice recordings of 23 speakers. They were instructed to fill in a questionnaire that asked for the speakers' characteristics. RESULTS: With regard to the individual characteristics, the listeners achieved variable results and identified the speakers' gender, age, foreign accents and partly level of education fairly accurate, while they were less efficient judging other categories such as height, body mass index, dialects and smoking habits.The primary school listeners achieved significantly less correct answers in all but 2 categories than each of the other listener groups; however, they already made some correct judgments above chance levels. The results achieved by the specialists and students did not differ significantly in any category. CONCLUSIONS: It has been confirmed that voice perception or the required skills are age dependent. The expert listeners did not perform significantly better than the naive listeners.

Early detection of hearing loss.

The universal newborn hearing screening (UNHS) is currently spreading in Germany, as well, even though there can be no talk of a comprehensive establishment. The introduction of UNHS in several federal states such as Hamburg, Hessen, and Schleswig-Holstein can be ascribed to the personal commitment of individual pediatric audiologists. Apart from the procurement of the screening equipment and the training of the staff responsible for the examination of the newborns, the tracking, i.e. the follow-up on children with conspicuous test results, is of utmost importance. This involves significant administration effort and work and is subject to data protection laws that can differ substantially between the various federal states. Among audiologists, there is consensus that within the first three months of a child's life, a hearing loss must be diagnosed and that between the age of 3 and 6 months, the supply of a hearing aid must have been initiated. For this purpose, screening steps 1 (usually a TEOAE measurement) and 2 (AABR testing) need to be conducted in the maternity hospital. The follow-up of step 1 then comprises the repetition of the TEOAE- and AABR measurement for conspicuous children by a specialized physician. The follow-up of step 2 comprises the confirmatory diagnostics in a pediatric audiological center. This always implies BERA diagnostics during spontaneous sleep or under sedation. The subsequent early supply of a hearing aid should generally be conducted by a (pediatric) acoustician specialized on children.

Office-based laryngoscopic observations of recurrent laryngeal nerve paresis and paralysis.

OBJECTIVES: To evaluate the endoscopic criteria of recurrent laryngeal nerve disorders, we performed a retrospective evaluation of videolaryngoscopic recordings from 50 patients with recurrent laryngeal nerve disorders. METHODS: The videolaryngoscopic examination was performed with rigid and flexible endoscopes. The range of motion of three laryngeal structures was assessed: the vocal ligament, the vocal process, and the arytenoid "hump" (mainly the corniculate region). RESULTS: Comparison of movement of these three structures revealed discrepancies. In 16 of 45 patients (36%) rigid endoscopy showed movements of the arytenoid hump associated with absence of any mobility of the vocal process and vocal ligament. In 5 patients the extent of movement of the vocal process and vocal ligament was less than that of the arytenoid hump. Only in 24 of 45 cases were the ratings for the vocal process, vocal ligament, and arytenoid hump identical. The findings of fiberscopy were comparable. CONCLUSIONS: In assessing recurrent laryngeal nerve disorders via laryngoscopy, sole judgment of the arytenoid hump movement can mislead. Our interpretation suggests that visible movement of the mucosa covering the arytenoid and accessory cartilages is not always paralleled by movement of the arytenoid cartilage itself. It was shown that the best criterion to rely on in endoscopy is movement of the vocal process or the vocal ligament.

Impaired calmodulin binding of myosin-7A causes autosomal dominant hearing loss (DFNA11).

Both myosin 7A (MYO7A) and calmodulin (CaM) are required for transduction and adaptation processes in inner ear hair cells. We identified a novel heterozygous missense mutation (c.2557C>T; p.R853C) in a family with autosomal dominant non-syndromic hearing loss that changes an evolutionarily invariant residue of the fifth IQ motif (IQ5), a putative calmodulin (CaM) binding domain, of MYO7A. Functional effects of the p.R853C mutation were investigated in a physiological cellular environment by expressing MYO7A IQ5-containing peptides in smooth muscle cells of microarteries, in which overexpression of wildtype IQ5 (with intact calmodulin binding) would be expected to compete with myosin light chain kinase (MLCK) for CaM binding. Indeed, analysis of calmodulin-dependent vasoconstriction suggests constitutive binding of CaM to the wildtype, but not the p.R853C-mutated IQ5 motif at all physiologically relevant Ca2+ concentrations. Thus our data suggest a disturbed CaM/MYO7A binding of the p.R853C mutant, this amino acid change may result in impaired adaptation to environmental stimuli and progressive deterioration of hearing transduction in heterozygotes. A defect in CaM/MYO7A interaction represents a novel pathomechanism for genetic hearing loss. It provides an attractive molecular target for therapeutic interventions aimed to delay or prevent the onset of hearing loss in families with mutations in myosin IQ domains.

Phenotypic variability of non-syndromic hearing loss in patients heterozygous for both c.35delG of GJB2 and the 342-kb deletion involving GJB6.

Mutations in GJB2, encoding the gap junction protein connexin 26, are the most common cause of inherited non-syndromic hearing loss (NSHL), with a broad spectrum of mutations leading to recessive as well as dominant forms. It has been shown that patients who are compound heterozygous for a 342-kb deletion (Delta(GJB6-D13S1830)) involving a large portion of the 5'-part of GJB6, encoding connexin 30, and a GJB2 mutation develop NSHL due to a trait with a digenic pattern of inheritance. We have used a mutation-specific polymerase chain reaction assay to screen NSHL patients for the presence of Delta(GJB6-D13S1830) and identified two families segregating both c.35delG in GJB2 and Delta(GJB6-D13S1830). Remarkably, the severity of hearing loss due to heterozygosity for c.35delG in GJB2 in conjunction with Delta(GJB6-D13S1830) is considerably different in members of the two families, ranging from congenital deafness in one to moderate/severe hearing loss with congenital onset in the other case.

Imaging of the larynx--extending the use of stroboscopy-related techniques.

Three methods (I-III) are presented to demonstrate how technical modifications in the use of stroboscopy-related techniques can be applied to further analyze vocal fold vibrations. (I) With double exposure videostroboscopy, dynamic properties of vocal folds can be visualized within one single image. This allows for estimations of horizontal vibratory velocity of the vocal fold margins. (II) Stroboscopic transillumination of the larynx makes it possible to localize initial vocal fold opening in the horizontal glottal plane and to visualize different vibratory opening patterns. Bidirectional color-coded endoscopic imaging of the larynx facilitates a semi-automated, digital measurement of the glottal area. (III) Stroboscopic illumination using four light emitting diodes (LEDs) clipped on a rigid endoscope and triggered by a portable 'pocket-sized', battery-powered electronic control device offers new prospects for performing stroboscopy outside of specialized clinical facilities.