Language Without Speech: Segregating Distinct Circuits in the Human Brain.

Language is a fundamental part of human cognition. The question of whether language is processed independently of speech, however, is still heavily discussed. The absence of speech in deaf signers offers the opportunity to disentangle language from speech in the human brain. Using probabilistic tractography, we compared brain structural connectivity of adult deaf signers who had learned sign language early in life to that of matched hearing controls. Quantitative comparison of the connectivity profiles revealed that the core language tracts did not differ between signers and controls, confirming that language is independent of speech. In contrast, pathways involved in the production and perception of speech displayed lower connectivity in deaf signers compared to hearing controls. These differences were located in tracts towards the left pre-supplementary motor area and the thalamus when seeding in Broca's area, and in ipsilateral parietal areas and the precuneus with seeds in left posterior temporal regions. Furthermore, the interhemispheric connectivity between the auditory cortices was lower in the deaf than in the hearing group, underlining the importance of the transcallosal connection for early auditory processes. The present results provide evidence for a functional segregation of the neural pathways for language and speech.

A C-terminal nonsense mutation links PTPRQ with autosomal-dominant hearing loss, DFNA73.

PurposeHearing loss is genetically extremely heterogeneous, making it suitable for next-generation sequencing (NGS). We identified a four-generation family with nonsyndromic mild to severe hearing loss of the mid- to high frequencies and onset from early childhood to second decade in seven members.MethodsNGS of 66 deafness genes, Sanger sequencing, genome-wide linkage analysis, whole-exome sequencing (WES), semiquantitative reverse-transcriptase polymerase chain reaction.ResultsWe identified a heterozygous nonsense mutation, c.6881G>A (p.Trp2294*), in the last coding exon of PTPRQ. PTPRQ has been linked with recessive (DFNB84A), but not dominant deafness. NGS and Sanger sequencing of all exons (including alternatively spliced 5' and N-scan-predicted exons of a putative "extended" transcript) did not identify a second mutation. The highest logarithm of the odds score was in the PTPRQ-containing region on chromosome 12, and p.Trp2294* cosegregated with hearing loss. WES did not identify other cosegregating candidate variants from the mapped region. PTPRQ expression in patient fibroblasts indicated that the mutant allele escapes nonsense-mediated decay (NMD).ConclusionKnown PTPRQ mutations are recessive and do not affect the C-terminal exon. In contrast to recessive loss-of-function mutations, c.6881G>A transcripts may escape NMD. PTPRQTrp2294* protein would lack only six terminal residues and could exert a dominant-negative effect, a possible explanation for allelic deafness, DFNA73, clinically and genetically distinct from DFNB84A.

[Cochlear Implants in Children and Adults]. / Kochleaimplantatversorgung von Kindern und Erwachsenen.

About 4000 patients receive a cochlear implant in Germany every year. Adults have become the main group for cochlear implantation candidacy.Elderly people with progressive and profound hearing loss experienced high communication restraints due to the limits of conventional hearing aids in the past. Nowadays cochlear implants can be offered as a well-established extension to the supply chain of hearing systems. The option of cochlear implantation for children with profound hearing impairment has led to a paradigm shift in hearing rehabilitation. However, there is still great variability in spoken language outcomes in CI children. These range from communicative and linguistic competencies similar to normal hearing children to clearly limited receptive and productive language skills. Besides age of implantation and the quality of previous auditory stimulation, there are additional factors that likely influence the outcome after cochlea implantation. Among them are the quality of caregivers' linguistic input and an intensive post-operative rehabilitation and intervention program. Ideally, children with CI are included into mainstream educational settings. Considering the high incidence of additional disabilities in children with hearing loss, it seems obvious that this goal cannot be achieved by all implanted children. Therefore, highly complex and individualized rehabilitation concepts are needed, which, in addition to oral language intervention programs, include total communication approaches as well.

The Basis for Language Acquisition: Congenitally Deaf Infants Discriminate Vowel Length in the First Months after Cochlear Implantation.

One main incentive for supplying hearing impaired children with a cochlear implant is the prospect of oral language acquisition. Only scarce knowledge exists, however, of what congenitally deaf children actually perceive when receiving their first auditory input, and specifically what speech-relevant features they are able to extract from the new modality. We therefore presented congenitally deaf infants and young children implanted before the age of 4 years with an oddball paradigm of long and short vowel variants of the syllable /ba/. We measured the EEG in regular intervals to study their discriminative ability starting with the first activation of the implant up to 8 months later. We were thus able to time-track the emerging ability to differentiate one of the most basic linguistic features that bears semantic differentiation and helps in word segmentation, namely, vowel length. Results show that already 2 months after the first auditory input, but not directly after implant activation, these early implanted children differentiate between long and short syllables. Surprisingly, after only 4 months of hearing experience, the ERPs have reached the same properties as those of the normal hearing control group, demonstrating the plasticity of the brain with respect to the new modality. We thus show that a simple but linguistically highly relevant feature such as vowel length reaches age-appropriate electrophysiological levels as fast as 4 months after the first acoustic stimulation, providing an important basis for further language acquisition.

Estimation of insertion depth angle based on cochlea diameter and linear insertion depth: a prediction tool for the CI422.

Beside the cochlear size, the linear insertion depth (LID) influences the insertion depth angle of cochlear implant electrode arrays. For the specific implant CI422 the recommended LID is not fixed but can vary continuously between 20 and 25 mm. In the current study, the influence of cochlea size and LID on the final insertion depth angle was investigated to develop a prediction tool for the insertion depth angle by means of cochlea diameter and LID. Preoperative estimation of insertion depth angles might help surgeons avoid exceeding an intended insertion depth, especially with respect to low-frequency residual hearing preservation. Postoperative, high-resolution 3D-radiographs provided by Flat Panel Computed Volume Tomography (FPCT) were used to investigate the insertion depth angle in 37 CI422 recipients. Furthermore, the FPCT images were used to measure linear insertion depth and diameter of the basal turn of the cochlea. A considerable variation of measured insertion depth angles ranging from 306° to 579° was identified. The measured linear insertion depth ranged from -18.6 to 26.2 mm and correlated positively with the insertion depth angle. The cochlea diameter ranged from 8.11 to 10.42 mm and correlated negatively with the insertion depth angle. The results suggest that preoperatively measured cochlea diameter combined with the option of different array positions by means of LID may act as predictors for the final insertion depth angle.

Targeted and genomewide NGS data disqualify mutations in MYO1A, the "DFNA48 gene", as a cause of deafness.

MYO1A is considered the gene underlying autosomal dominant nonsyndromic hearing loss DFNA48, based on six missense variants, one small in-frame insertion, and one nonsense mutation. Results from NGS targeting 66 deafness genes in 109 patients identified three families challenging this assumption: two novel nonsense (p.Tyr740* and p.Arg262*) and a known missense variant were identified heterozygously not only in index patients, but also in unaffected relatives. Deafness in these families clearly resulted from mutations in other genes (MYO7A, EYA1, and CIB2). Most of the altogether 10 MYO1A mutations are annotated in dbSNP, and population frequencies (dbSNP, 1000 Genomes, Exome Sequencing Project) above 0.1% contradict pathogenicity under a dominant model. One healthy individual was even homozygous for p.Arg262*, compatible with homozygous Myo1a knockout mice lacking any overt pathology. MYO1A seems dispensable for hearing and overall nonessential. MYO1A adds to the list of "erroneous disease genes", which will expand with increasing availability of large-scale sequencing data.

Contribution of paranasal sinuses to the acoustic properties of the nasal tract.

BACKGROUND: The contribution of the nasal and paranasal cavities to the vocal tract resonator properties is unclear. Here we investigate these resonance phenomena of the sinonasal tract in isolation in a cadaver and compare the results with those gained in a simplified brass tube model. METHODS: The resonance characteristics were measured as the response to sine sweep excitation from an earphone. In the brass model the earphone was placed at the closed end and in the cadaver in the epipharynx. The response was picked up by a microphone placed at the open end of the model and at the nostrils, respectively. A shunting cavity with varied volumes was connected to the model and the effects on the response curve were determined. In the cadaver, different conditions with blocked and unblocked middle meatus and sphenoidal ostium were tested. Additionally, infundibulotomy was performed allowing direct access to and selective occlusion of the maxillary ostium. RESULTS: In both the brass model and the cadaver, a baseline condition with no cavities included produced response curves with clear resonance peaks separated by valleys. Marked dips occurred when shunting cavities were attached to the model. The frequencies of these dips decreased with increasing shunting volume. In the cadaver, a marked dip was observed after removing the unilateral occlusion of the middle meatus and the sphenoidal ostium. Another marked dip was detected at low frequency after removal of the occlusion of the maxillary ostium following infundibulotomy. CONCLUSION: Combining measurements on a simplified nasal model with measurements in a cadaveric sinonasal tract seems a promising method for shedding light on the acoustic properties of the nasal resonator.

Relationship between epilarynx tube shape and the radiated sound pressure level during phonation is gender specific.

OBJECTIVE/HYPOTHESIS: The aim of the study was to measure the morphology of the epilaryngeal tube during sustained phonation as a function of loudness variation and to compare subjects of different genders. STUDY DESIGN: This is a prospective study. METHODS: Five female and five male classically trained singers were recorded by magnetic resonance imaging with simultaneous audio recordings while sustaining phonation at three different loudness conditions. Three-dimensional subsections of the vocal tract were segmented on multi-image-based cross-sections. Different volume and area measures were determined and their relation to sound pressure level and loudness condition was analyzed. RESULTS: Male singers tended to narrow the epilaryngeal tube when increasing sound pressure level whereas female singers did not. CONCLUSION: Strategies of vocal tract adjustments during loudness variation in classical singing appear to be gender specific.

The inside-out surgical anatomy of the paraglottic space a video-guided endoscopic dissection.

Objectives: The paraglottic space is an essential anatomic compartment of the larynx. It is central to the spread of laryngeal cancer and to the choice of conservative laryngeal surgery and many phonosurgical procedures. Since its description, 60 years ago, the surgical anatomy of the paraglottic space was sparsely revisited. Amid the era of endoscopic and transoral microscopic functional surgery of the larynx, we provide here a long-awaited description of the inside-out anatomy of the paraglottic space. Methodology: Using an endoscope equipped with a 3D camera, we dissected 10 hemilarynges from 5 fresh frozen cadavers from the inside out. Before dissection, we labeled the vessels through injecting them with colored latex. We explored the paraglottic space emphasizing its shape, boundaries, and contents. We documented our findings through endoscopic photography and video recordings. Results: The paraglottic space is a spacious tetrahedral space located parallel not only to the glottic, but also to the subglottic and the supraglottic compartments of the laryngeal lumen. It has musculo-cartilaginous, musculo-fibrous, and mucosal boundaries. It is separated from the pyriform sinus only by mucosa. A cushion of fat surrounds its vascular and to a lesser extent its neural contents. Harbored intrinsic laryngeal muscles are endoscopically identifiable within the space, namely the thyroarytenoid, the lateral, and posterior cricoarytenoid muscles. Conclusion: The endoscopic description of the paraglottic space partly fills the knowledge gap on the laryngeal anatomy from the inside out. It opens the door for novel diagnostic methods and for ultraconservative functional laryngeal interventions under endoscopic control. Level of Evidence: N/A.

Inside-Out Surgical Anatomy of Superior Laryngeal Artery Endoscopic Dissection and Proposal for Nomenclature of Branches.

Objectives: To describe the inside out surgical anatomy of the superior laryngeal artery and to resolve the ambiguities in the nomenclature of its main branches. Study Design: Endoscopic dissection of the superior laryngeal artery in the paraglottic space of larynges of fresh frozen cadavers and a review of the literature. Setting: A center for anatomy encompassing facilities for latex injection into the cervical arteries of human donor bodies and a laryngeal dissection station equipped with a video-guided endoscope and a 3-dimensional camera. Methods: Video-guided endoscopic dissection of 12 hemilarynges in fresh frozen cadavers whose cervical arteries were injected with red latex. Description of the inside-out surgical anatomy of the superior laryngeal artery and its main branches. Review of the previous reports describing the anatomy of the superior laryngeal artery. Results: From inside the larynx, the artery was exposed upon its entry through the thyrohyoid membrane or through the foramen thyroideum. It was traced ventrocaudally in the paraglottic space exposing its branches to the epiglottis, the arytenoid, and the laryngeal muscles and mucosa. Its terminal branch was followed until it left the larynx through the cricothyroid membrane. Branches of the artery, previously described under different names, appeared to supply the same anatomical domains. Conclusion: Mastering the inside out anatomy of the superior laryngeal artery is mandatory to control any intraoperative or postoperative hemorrhage during transoral laryngeal microsurgery or during transoral robotic surgery. Naming the artery's main branches according to their domain of supply would resolve the ambiguities resulting from various nomenclatures.

For Fiddlers on the Roof and in the Pit: Healthcare and Epidemiology of Playing-Related Problems in Violinists.

Musicians, specifically violinists, may experience pain or loss of function as a result of poor playing hygiene, environmental factors, and insufficient guidance from teachers, parents, conductors, and physicians. The causal occurrence of these pathologies and the efficacy of general treatment, along with referral to other specialists for adjunct therapies and rehabilitation, were examined in this literature review. A systematic PubMed search was conducted in May 2022 to gain insight into the current state of the published literature. Inclusion criteria were cross-sectional or comparative studies that address relevant pathologies among violinists by specialty. The search resulted in 25 cross-sectional, 5 comparative studies, and 1 case review including a total of 6010 musicians, among them at least 920 violinists. Orthopedists, neurologists, dentists/orthodontists, dermatologists, and audiologists are most commonly consulted to treat violinists' complaints. Other internists and various paramedical specialists can equally be affected and should be sensitive to the complaints of musicians. In conclusion, the field of performing arts medicine is extremely wide and requires multidisciplinary specialist attention. The studies chosen highlight that violinists may suffer great discomfort at the hands of their instrument and merit adequate guidance from physicians in order to be able to continue playing in a safe, ergonomic way.

Pre-adolescent children exhibit lower aerosol particle volume emissions than adults for breathing, speaking, singing and shouting.

Speaking and singing are activities linked to increased aerosol particle emissions from the respiratory system, dependent on the utilized vocal intensity. As a result, these activities have experienced considerable restrictions in enclosed spaces since the onset of the COVID-19 pandemic due to the risk of infection from the SARS-CoV-2 virus, transmitted by virus-carrying aerosols. These constraints have affected public education and extracurricular activities for children as well, from in-person music instruction to children's choirs. However, existing risk assessments for children have been based on emission measurements of adults. To address this, we measured the particle emission rates of 15 pre-adolescent children, all eight to ten years old, with a laser particle counter for the test conditions: breathing at rest, speaking, singing and shouting. Compared with values taken from 15 adults, emission rates for breathing, speaking and singing were significantly lower for children. Particle emission rates were reduced by a factor of 4.3 across all conditions, whereas emitted particle volume rates were reduced by a factor of 4.8. These data can supplement SARS-CoV-2 risk management scenarios for various school and extracurricular settings.

Infants' vocalizations at 6 months predict their productive vocabulary at one year.

Long before their first words, children communicate by using speech-like vocalizations. These protophones might be indicative of infants' later language development. We here examined infants' (n = 56) early vocalizations at 6 months (vocal reactivity scale of the IBQ-R) as a predictor of their expressive and receptive language at 12 months (German version of the CDI). Regression analyses revealed vocalizations to significantly predict expressive, but not receptive language. Our findings in German-learning 6-month-olds extend previous predictive evidence of early vocalizations reported for older infants. Together these findings are informative in light of early assessments monitoring typical and atypical language development.

Aerosol emission in professional singing of classical music.

In this study, emission rates of aerosols emitted by professional singers were measured with a laser particle counter under cleanroom conditions. The emission rates during singing varied between 753 and 6093 particles/sec with a median of 1537 particles/sec. Emission rates for singing were compared with data for breathing and speaking. Significantly higher emission rates were found for singing. The emission enhancements between singing and speaking were between 4.0 and 99.5 with a median of 17.4, largely due to higher sound pressure levels when singing. Further, significant effects of vocal loudness were found, whereas there were no significant differences between the investigated voice classifications. The present study supports the efforts to improve the risk management in cases of possible aerogenic virus transmission, especially for choir singing.

T1a Glottic Cancer: Advances in Vocal Outcome Assessment after Transoral CO2-Laser Microsurgery Using the VEM.

Patients with unilateral vocal fold cancer (T1a) have a favorable prognosis. In addition to the oncological results of CO2 transoral laser microsurgery (TOLMS), voice function is among the outcome measures. Previous early glottic cancer studies have reported voice function in patients grouped into combined T stages (Tis, T1, T2) and merged cordectomy types (lesser- vs. larger-extent cordectomies). Some authors have questioned the value of objective vocal parameters. Therefore, the purpose of this exploratory prospective study was to investigate TOLMS-associated oncological and vocal outcomes in 60 T1a patients, applying the ELS protocols for cordectomy classification and voice assessment. Pre- and postoperative voice function analysis included: Vocal Extent Measure (VEM), Dysphonia Severity Index (DSI), auditory-perceptual assessment (GRB), and 9-item Voice Handicap Index (VHI-9i). Altogether, 51 subjects (43 male, eight female, mean age 65 years) completed the study. The 5-year recurrence-free, overall, and disease-specific survival rates (Kaplan-Meier method) were 71.4%, 94.4%, and 100.0%. Voice function was preserved; the objective parameter VEM (64 ± 33 vs. 83 ± 31; mean ± SD) and subjective vocal measures (G: 1.9 ± 0.7 vs. 1.3 ± 0.7; VHI-9i: 18 ± 8 vs. 9 ± 9) even improved significantly (p < 0.001). The VEM best reflected self-perceived voice impairment. It represents a sensitive measure of voice function for quantification of vocal performance.

Aerosol emission of adolescents voices during speaking, singing and shouting.

Since the outbreak of the COVID-19 pandemic, singing activities for children and young people have been strictly regulated with far-reaching consequences for music education in schools and ensemble and choir singing in some places. This is also due to the fact, that there has been no reliable data available on aerosol emissions from adolescents speaking, singing, and shouting. By utilizing a laser particle counter in cleanroom conditions we show, that adolescents emit fewer aerosol particles during singing than what has been known so far for adults. In our data, the emission rates ranged from 16 P/s to 267 P/s for speaking, 141 P/s to 1240 P/s for singing, and 683 P/s to 4332 P/s for shouting. The data advocate an adaptation of existing risk management strategies and rules of conduct for groups of singing adolescents, like gatherings in an educational context, e.g. singing lessons or choir rehearsals.

Voice classification and vocal tract of singers: a study of x-ray images and morphology.

This investigation compares vocal tract dimensions and the classification of singer voices by examining an x-ray material assembled between 1959 and 1991 of students admitted to the solo singing education at the University of Music, Dresden, Germany. A total of 132 images were available to analysis. Different classifications' values of the lengths of the total vocal tract, the pharynx, and mouth cavities as well as of the relative position of the larynx, the height of the palatal arch, and the estimated vocal fold length were analyzed statistically, and some significant differences were found. The length of the pharynx cavity seemed particularly influential on the total vocal tract length, which varied systematically with classification. Also studied were the relationships between voice classification and the body height and weight and the body mass index. The data support the hypothesis that there are consistent morphological vocal tract differences between singers of different voice classifications.

Establishing a mental lexicon with cochlear implants: an ERP study with young children.

In the present study we explore the implications of acquiring language when relying mainly or exclusively on input from a cochlear implant (CI), a device providing auditory input to otherwise deaf individuals. We focus on the time course of semantic learning in children within the second year of implant use; a period that equals the auditory age of normal hearing children during which vocabulary emerges and extends dramatically. 32 young bilaterally implanted children saw pictures paired with either matching or non-matching auditory words. Their electroencephalographic responses were recorded after 12, 18 and 24 months of implant use, revealing a large dichotomy: Some children failed to show semantic processing throughout their second year of CI use, which fell in line with their poor language outcomes. The majority of children, though, demonstrated semantic processing in form of the so-called N400 effect already after 12 months of implant use, even when their language experience relied exclusively on the implant. This is slightly earlier than observed for normal hearing children of the same auditory age, suggesting that more mature cognitive faculties at the beginning of language acquisition lead to faster semantic learning.

Effects of professional singing education on vocal vibrato--a longitudinal study.

Vocal vibrato is regarded as one of the essential characteristics of voice quality in classical singing. Professional singers seem to develop vibrato automatically, without actively striving to acquire it. In this longitudinal investigation, the vocal vibrato of 22 singing students was examined at the beginning of and after 3 years of professional singing education. Subjects sang an ascending-descending triad pattern in slow tempo on vowel [a:] at a comfortable pitch level twice at soft (piano) and twice at medium (mezzoforte) loudness. The top note of the triad pattern was sustained for approximately 5s. The mean and the standard deviation (SD) of the vibrato rate were measured for this note. Results revealed that after 3 years of training, voices with vibrato slower than 5.2 Hz were found to have a faster vibrato, and voices with vibrato faster than 5.8 Hz were found to have a slower vibrato. Standard deviation of vibrato rate was higher in soft than in medium loudness, particularly before the education. Also high values of SD of vibrato rate, exceeding 0.65 Hz, had decreased after the education. These findings confirm that vibrato characteristics can be affected by singing education.

Three-dimensional Vocal Tract Morphology Based on Multiple Magnetic Resonance Images Is Highly Reproducible During Sustained Phonation.

OBJECTIVES: The extraction of a three-dimensional (3D) morphology of the human vocal tract (VT) from magnetic resonance imaging (MRI) during sustained phonation can be used for various analyses like numerical simulations or creating physical models. The precision of visualizing techniques nowadays allows for very targeted acoustical simulation evaluating the influence of subsections of the VT for the transfer function. The aim of the study was to assess the accuracy of the 3D geometry based on MRI data in repetitive trials. STUDY DESIGN: This is a prospective study. METHODS: Four experienced singers underwent an MRI while repeating a specific vocal task 20 times consecutively. Audio recordings were made by means of an optical microphone. Images were restacked and subsections of the VT were segmented on multi-image-based cross sections using a semiautomatic algorithm. Different volume and area measures were evaluated. RESULTS: A high reproducibility of the morphologic data based on multiple images by means of the applied segmentation method could be shown with an overall variation of around 8%. CONCLUSIONS: 3D modeling of the VT during sustained phonation involves a complex experimental setting and elaborate image processing techniques. Functional comparative analysis or acoustical simulations based on such data should take the found variability into account.