Unilateral hearing loss in children: a retrospective study and a review of the current literature.

Despite the introduction of universal newborn hearing screening (UNHS), unilateral hearing loss (UHL) is sometimes recognized late. This diagnostic delay has adverse repercussions, given the importance of binaural hearing for the development of normal auditory processing. It is incorrect to maintain that unilateral hearing is the minimum requirement for adequate speech development and that hearing aid provision is consequently unnecessary. In our retrospective study, hearing aid provision resulted in improved directional and selective hearing (quiet and noisy environments) and, compared with their chronically ill counterparts, the children in our study displayed superior health-related quality of life (HRQoL) scores in all areas. On the basis of the results, the authors conclude that even mild hearing losses (from an auditory threshold of 30 to 40 dB) should have the opportunity for hearing aid provision. A selective literature review was conducted in PubMed and textbooks and with reference to national and international guidelines. Early diagnosis and treatment of UHL have a positive effect on verbal-cognitive, linguistic, communicative, and socio-emotional development, as demonstrated by neurophysiological studies. Among the treatment modalities with differing effects on the quality of binaural hearing, cochlear implants are now used increasingly in children with hearing loss bordering on deafness. CONCLUSION: Published evidence and clinical experience support early diagnosis and treatment. Wherever feasible, hearing aid provision before or at the end of the first year of life is recommended for children with UHL. What is Known: • Almost 30 years ago, poor academic performance was reported in children with unilateral hearing loss (UHL). • Despite improvements in treatment options, it is traditionally held that unilateral hearing is the minimum requirement for adequate speech development and hearing aid provision is unnecessary. What is New: • Academic and behavioral deficits in children with UHL may be mediated by deficiencies in the default mode network. • Published evidence supports the recommendation for hearing aid provision before or at the end of the first year of life in children with UHL.

Distinct mutations in STXBP2 are associated with variable clinical presentations in patients with familial hemophagocytic lymphohistiocytosis type 5 (FHL5).

Familial hemophagocytic lymphohistiocytosis (FHL) is a genetically determined hyperinflammatory syndrome caused by uncontrolled immune response mediated by T-lymphocytes, natural killer (NK) cells, and macrophages. STXBP2 mutations have recently been associated with FHL5. To better characterize the genetic and clinical spectrum of FHL5, we analyzed a cohort of 185 patients with suspected FHL for mutations in STXBP2. We detected biallelic mutations in 37 patients from 28 families of various ethnic origins. Missense mutations and mutations affecting 1 of the exon 15 splice sites were the predominant changes detectable in this cohort. Patients with exon 15 splice-site mutations (n = 13) developed clinical manifestations significantly later than patients with other mutations (median age, 4.1 year vs 2 months) and showed less severe impairment of degranulation and cytotoxic function of NK cells and CTLs. Patients with FHL5 showed several atypical features, including sensorineural hearing deficit, abnormal bleeding, and, most frequently, severe diarrhea that was only present in early-onset disease. In conclusion, we report the largest cohort of patients with FHL5 so far, describe an extended disease spectrum, and demonstrate for the first time a clear genotype-phenotype correlation.

The Relation Between Endoscopic and Subjective Laryngopharyngeal Reflux Signs, Vocal Tract Discomfort, Voice Handicap, and Voice Disorder Type: Same Yet Different?

OBJECTIVES: This study aimed to investigate the relation between subjective voice-related symptoms and endoscopic findings in patients with different etiology of voice disorder and vocally healthy subjects with and without laryngopharyngeal reflux (LPR). STUDY DESIGN: Retrospective cross-sectional study. METHODS: The study involved 149 participants (106 female, 43 male) including 125 with various voice disorders (functional, structural, and neurogenic) and 24 vocally healthy individuals. For self-rating the German versions of the Voice Handicap Index (VHI), Vocal Tract Discomfort (VTD) Scale, and Reflux Symptom Index (RSI) were applied, while endoscopic evaluations utilized the Reflux Finding Score (RFS) and Reflux Sign Assessment (RSA). Statistical analyses incorporated ANOVA with Bonferroni posthoc tests to identify group variations. Correlations between VTD Scale, VHI, RSI, RFS, and RSA were evaluated using Pearson's correlation coefficient. To examine test sensitivity and specificity for the VTD Scale and RSA, we performed a receiver operating characteristics analysis. Youden's-Index was applied to determine the cut-off-value with best discriminatory abilities. The diagnosis of LPR was assumed when the criteria of RFS > 7 AND RSI > 13 was met. RESULTS: Significant differences for all voice diagnosis groups and vocally healthy individuals for RFS and all three self-rating questionnaires were found. Moreover, there was significant correlation between VTD Scale and VHI and RSI as well as RSI and RFS, which was moderate, negative in the group of persons with LPR. However, there was no significant difference for RSA results between the vocally healthy or any diagnosis group. CONCLUSION: Thus, the RFS may be more suitable to predict reflux and voice-related symptoms. The VTD Scale is a useful instrument in screening voice disorders but also LPR and can therefore be used as a tool for decision-making when transferring to a specialist.

The anisotropic nature of the human vocal fold: an ex vivo study.

The purpose of this study was to measure the relationship between the shear elastic properties of vocal fold with respect to the direction of applied stress. There is extensive published material that quantifies the shear viscoelastic properties of the vocal fold, but as much of these data were obtained using rotating parallel plate rheometers, which are unable to resolve out difference of the shear elastic behaviour with respect to direction, there is very little data that indicates anisotropic behaviour. To overcome this gap in knowledge, the team devised an apparatus that is capable of applying a shear stress in a known direction. A series of measurements were taken at the mid-membranous position, in the transverse and longitudinal directions. Point-specific measurements were performed using fourteen human cadaver excised larynges, which were hemi-sectioned to expose the vocal fold. An extremely low sinusoidal shear force of 1 g was applied tangentially to the membrane surface in both the longitudinal and transverse direction, and the resultant shear strain was measured. With the probe applied to the intact vocal fold, the average ratio of the elasticity in the transverse with respect to the longitudinal direction was 0.55. Further investigation using histological staining of collagens in the lamina propria indicates that there is a visible difference in the general alignment of collagen fibres when comparing the coronal and the sagittal sections. Our conclusion is that there is a quantifiable difference between the shear elastic response of the lamina propria in the longitudinal and transverse directions, and that this could be explained by the difference in alignment of collagen fibres within the lamina propria.

Cross-Validation and Normative Values for the German Vocal Tract Discomfort Scale.

Purpose The Vocal Tract Discomfort Scale (VTD Scale) is a self-rating questionnaire investigating physical symptoms in the larynx associated with vocal pathology. The aim of this work was to investigate the reliability, validity, sensitivity, and specificity of the first German version and to provide normative data with thresholds for pathology and a scaling scheme. Study Design A retrospective multicenter study was performed. Method A total of 571 participants (409 female and 162 male), with a mean age of 47.2 years, were recruited at three German centers; of these, there were 447 participants with voice disorder and 124 vocally healthy participants. The clinical examination consisted of patient history, visual laryngeal examination, acoustic and aerodynamic assessment, perceptual analysis by the Grading-Roughness-Breathiness-Asthenia-Strain Scale, and subjective evaluation using the VTD Scale and the Voice Handicap Index (VHI). Statistics included group comparisons (t test and analysis of variance), Pearson correlation coefficient (between VTD Scale and VHI), and Cronbach's alpha to assess validity and reliability. Analysis of receiver operating characteristics was performed to examine VTD Scale's discriminatory ability and provide a cutoff score. Additionally, percentiles were applied to provide VTD Scale ranges. Results There were highly significant differences between healthy participants and participants with voice disorder regarding the total score and both subscales of the VTD Scale. Internal consistency was excellent (α = .928). We found moderate, positive correlation between the VTD Scale and VHI (ρ = .596, p < .001). Receiver operating characteristics analysis showed an area under the curve of 0.876 (p < .001, 95% confidence interval [0.846, 0.906]). VTD Scale ranges were no (score: 0-13), mild (score: 14-26), moderate (score: 27-40), and severe (score: 41-96) disorder. Conclusions Results confirm an excellent reliability and validity of the German VTD Scale. It provides additional and independent diagnostic information and is a useful instrument to complement voice assessment. The scaling into four severity subgroups allows the tool to be used for screening patients and considers a transferral to a voice specialist.

Interdisciplinary approach to design, performance, and quality management in a multicenter newborn hearing screening project. Discussion of the results of newborn hearing screening in Hamburg (part II).

Previously presented results of the newborn hearing screening in Hamburg and the perspectives are subsequently discussed. Minimum standards referring a participation of 95% of the neonates and a fail rate of less than 4% hearing-impaired children at the primary screening are fulfilled in Hamburg. Systematic screening of newborn hearing by an interdisciplinary approach provides early identification and intervention for children with permanent unilateral and bilateral hearing loss. But a newborn hearing screening on a voluntary basis alone cannot be maintained in the long run. Further, an anonymous data collection is not sufficient in regard to an uninterrupted tracking of conspicuous and unscreened neonates. A lost-to-follow-up rate of 31.3% at primary screening in Hamburg is much too high and emphasizes the need for a public health approach to a population-based newborn hearing screening with an elaborate and name-based tracking system. The legislation and implementation of a nationwide newborn hearing screening program in Germany and the association of German newborn hearing screening centers are highlighting long efforts of hearing professionals. But the implementation of a newborn hearing screening only makes sense if there exists an efficient tracking system. Sad to say, we are still a long way from the implementation of such a tracking system.

Interdisciplinary approach to design, performance, and quality management in a multicenter newborn hearing screening project: introduction, methods, and results of the newborn hearing screening in Hamburg (Part I).

From the actual point of view, the "sensitive period" for the effects of hearing impairment on speech and language development is within the first year of life. Early exposure to acoustic or electric stimulation can compensate for the acoustic deficit. A regional-based, specifically designed concept of a universal newborn hearing screening (UNHS) was started in Hamburg in the year 2002. For the first time in Germany, a comprehensive protocol including screening measurement, follow-up procedures, tracking, and early intervention was implemented. An interdisciplinary approach from the very beginning could be realized. Sixty-three thousand, four hundred fifty-nine out of 65,466 births were registered during the period August 2002 to July 2006, 93% were primarily screened. 3.3% failed the test and 31.3% were lost to follow-up. A total of 118 children were diagnosed with hearing loss in the follow-up. The median age at time of diagnosis was 3.5 months. Seventy-four children received hearing aids. Out of these 74 children, 6 were subsequently supplied with cochlear implants. The high lost-to-follow-up rate is the biggest challenge for the tracking. Our results will be discussed in part II.

Quantification of change in vocal fold tissue stiffness relative to depth of artificial damage.

OBJECTIVES: To quantify changes in the biomechanical properties of human excised vocal folds with defined artificial damage. METHODS: The linear skin rheometer (LSR) was used to obtain a series of rheological measurements of shear modulus from the surface of 30 human cadaver vocal folds. The tissue samples were initially measured in a native condition and then following varying intensities of thermal damage. Histological examination of each vocal fold was used to determine the depth of artificial alteration. The measured changes in stiffness were correlated with the depth of cell damage. RESULTS: For vocal folds in a pre-damage state the shear modulus values ranged from 537 Pa to 1,651 Pa (female) and from 583 Pa to 1,193 Pa (male). With increasing depth of damage from the intermediate layer of the lamina propria (LP), tissue stiffness increased consistently (compared with native values) following application of thermal damage to the vocal folds. The measurement showed an increase of tissue stiffness when the depth of tissue damage was extending from the intermediate LP layer downwards. CONCLUSIONS: Changes in the elastic characteristics of human vocal fold tissue following damage at defined depths were demonstrated in an in vitro experiment. In future, reproducible in vivo measurements of elastic vocal fold tissue alterations may enable phonosurgeons to infer the extent of subepithelial damage from changes in surface elasticity.

Frequency-specific Animal Sound Test (FAST) 4: A valid method for hearing screening.

OBJECTIVES: It is essential to monitor hearing status in children not only as a mandatory requirement during universal newborn hearing screening (UNHS), but also later during preschool and school-age development. The present study considers the appropriateness of the Frequency-specific Animal Sound Test (FAST4) for use in children between the ages of 2.5 and 10 years; the comparability of hearing thresholds determined using FAST4 and those measured by pure tone audiometry (PTA); and the clinical and diagnostic utility of FAST4 in a variety of pediatric settings. METHODS: 322 children aged 2.6-14.1 years and 41 adults were tested with FAST4. Four animal sounds were presented via headphones and a hearing threshold was determined for the high and low frequency range. In addition, the hearing threshold of each child was measured by PTA. RESULTS: Results were analyzed from 156 normal-hearing and hearing-impaired children, mostly above the age of 4 years. In general, FAST4 yielded hearing levels comparable with those from PTA in children and in adults. FAST4 frequently had to be halted prematurely in children under 4 years old. CONCLUSIONS: FAST4 is a strong candidate for use as an instrument for preschool hearing screening and offers several advantages over other hearing tests. FAST4 permits simple, swift and efficient determination of the hearing threshold and the test can be administered by staff without specialist training. A number of improvements have already been integrated into the successor model known as mFAST.

Ventricular-fold dynamics in human phonation.

PURPOSE: In this study, the authors aimed (a) to provide a classification of the ventricular-fold dynamics during voicing, (b) to study the aerodynamic impact of these motions on vocal-fold vibrations, and (c) to assess whether ventricular-fold oscillations could be sustained by aerodynamic coupling with the vocal folds. METHOD: A 72-sample database of vocal gestures accompanying different acoustical events comprised high-speed cinematographic, audio, and electroglottographic recordings of 5 subjects. Combining the physiological correlates with a theoretical model of phonation, the vocal-ventricular aerodynamic interactions were investigated. RESULTS: A ventricular-fold motion is found during (de)crescendos, shout, throat singing, yodel, growls, and glides with transitions between registers. Three main types of dynamics are identified: slow nonoscillatory motion and fast oscillatory motion with aperiodical or periodical vibrations. These patterns accompany a change in voice quality, pitch, and/or intensity. Alterations of glottal-oscillatory amplitude, frequency, and contact were predicted. It is shown that a ventricular oscillation can be initiated and sustained by aerodynamic coupling with the vocal folds. CONCLUSIONS: Vocal-ventricular aerodynamic interactions can alter, enhance, or suppress vocal-fold vibrations or leave them unchanged, depending on the ventricular-fold dynamics involved. Depending on its variation in time, a similar level of ventricular-fold adduction impacts the glottal vibratory magnitude and contact much differently.

Uncommon lesions in the internal auditory canal (IAC): review of the literature and case report.

BACKGROUND: Despite the relatively frequent occurrence of multiple primary tumors, namely, 10% of intracranial tumors, metastasis is a rare occurrence within the internal auditory canal (IAC) and cerebellopontine angle (CPA). Intracanalicular metastases of adenocarcinoma are documented, but a primary adenocarcinoma remains unreported. We provide a review of uncommon lesions in the IAC and describe to our knowledge the first instance of a primary adenocarcinoma. CASE REPORT: A 60-year-old man presented with nausea and vomiting. Cranial computed tomography scan revealed bilateral nonspecific periventricular and subcortical vascular lesions. He presented 8 months later with left-sided tinnitus, progressive hearing loss, and attacks of vertigo. Magnetic resonance imaging (MRI) showed an extra-axial mass most likely representing a left-sided vestibular schwannoma with characteristic contrast enhancement in the IAC. The follow-up MRI showed an unchanged pattern of contrast enhancement. Due to progressive headaches and dizziness, the patient underwent a left transtemporal craniotomy with subtotal tumor resection. Histological examination revealed blennogenic cylindrical adenocarcinoma. The investigations for the primary tumor site were all negative. The patient's condition deteriorated gradually. MRI showed an increase of the residual tumor and meningeosis carcinomatosa, and cerebrospinal fluid (CSF) examination was positive for tumor cells. The patient was treated with intrathecal chemotherapy. He died of multiple organ failure. DISCUSSION: The discussion focuses on the incidence of extra-axial CPA and IAC lesions with their clinical presentations and their radiological findings. We address the issue of a possible regulation of CPA lesion laterality by asymmetrically expressed genes. In view of the sparse literature on treatment of single intracanalicular metastases, the review is broadened to the current treatment recommendations of single brain metastases. CONCLUSIONS: The differentiation between benign and malign lesions in the CPA and IAC is important, as it requires diverse treatment protocols. For the physician this differentiation represents a clinical and radiological challenge. For the developmental research the left-right asymmetry might be a field of research.