The Bonebridge (BB) was the first active transcutaneous implantation system for bone conduction. The main indications are conductive or mixed hearing loss and single-sided deafness. Treacher-Collins syndrome (TCS) is a rare genetic disease that affects craniofacial development. The disorder results in deformations of facial structure including ear malformations, especially microtia and ear canal atresia. These patients suffer from conductive hearing loss. CT scans often show unfavorable temporal bone anatomy making placement of an implant difficult. For implantable hearing rehabilitation, patients may decide for conduction implants, such as a BAHA, a Ponto, a Vibrant Soundbridge, or a Bonebridge. In this case report, we present 2 patients with TCS implanted with the Bonebridge system, their audiological results, and quality of life.
- Klíčová slova
- active transcutaneous bone conduction, bonebridge, children, hearing rehabilitation, treacher-collins syndrome,
- MeSH
- implantace protézy * metody MeSH
- kostní vedení zvuku MeSH
- kvalita života MeSH
- lidé MeSH
- mandibulofaciální dysostóza * komplikace MeSH
- převodní nedoslýchavost * chirurgie etiologie rehabilitace MeSH
- sluchové pomůcky * MeSH
- výsledek terapie MeSH
- Check Tag
- lidé MeSH
- mužské pohlaví MeSH
- ženské pohlaví MeSH
- Publikační typ
- časopisecké články MeSH
- kazuistiky MeSH
Auditory processing in mammals begins in the peripheral inner ear and extends to the auditory cortex. Sound is transduced from mechanical stimuli into electrochemical signals of hair cells, which relay auditory information via the primary auditory neurons to cochlear nuclei. Information is subsequently processed in the superior olivary complex, lateral lemniscus, and inferior colliculus and projects to the auditory cortex via the medial geniculate body in the thalamus. Recent advances have provided valuable insights into the development and functioning of auditory structures, complementing our understanding of the physiological mechanisms underlying auditory processing. This comprehensive review explores the genetic mechanisms required for auditory system development from the peripheral cochlea to the auditory cortex. We highlight transcription factors and other genes with key recurring and interacting roles in guiding auditory system development and organization. Understanding these gene regulatory networks holds promise for developing novel therapeutic strategies for hearing disorders, benefiting millions globally.
- Klíčová slova
- auditory cortex, cochlea, cochlear nucleus, inferior colliculus, medial geniculate body, superior olivary complex,
- MeSH
- lidé MeSH
- mozek metabolismus růst a vývoj MeSH
- sluch * fyziologie MeSH
- sluchová dráha * fyziologie MeSH
- sluchové korové centrum metabolismus fyziologie MeSH
- zvířata MeSH
- Check Tag
- lidé MeSH
- zvířata MeSH
- Publikační typ
- časopisecké články MeSH
- přehledy MeSH
BACKGROUND: During otosclerosis surgery, operative trauma can lead to decreased bone conduction. AIMS: The study aims to observe the bone conduction changes after otosclerosis operations and analyse possible factors affecting the postoperative decrease in bone conduction. MATERIAL AND METHODS: Authors retrospectively processed the data of 109 patients and evaluated pure tone audiometry before surgery and consequently 2 days, 1 month and 1 year after surgery. RESULTS: We noted a deterioration of bone conduction >5 dB on the second postoperative day in 28% (30/109) of patients, which persisted one year after the surgery in 9% (10/109) cases. Analysis of individual factors affecting bone conduction loss revealed a higher risk of permanent loss of bone conduction in patients with early postoperative loss in higher frequencies, in older patients and patients with a preoperative threshold of bone conduction >20 dB. Revision surgery was not a statistically significant factor. CONCLUSION AND SIGNIFICANCE: The bone conduction decrease after otosclerosis surgery is usually temporary. The recovery of bone conduction is influenced by the age of patients and the level of bone conduction before the surgery. The early postoperative decrease of bone conduction in higher frequencies is a negative predictive factor for permanent hearing loss.
- Klíčová slova
- otosclerosis, sensorineural hearing loss, surgical trauma,
- MeSH
- audiometrie čistými tóny * MeSH
- chirurgie třmínku * MeSH
- dospělí MeSH
- kostní vedení zvuku * MeSH
- lidé středního věku MeSH
- lidé MeSH
- otoskleróza * chirurgie patofyziologie komplikace MeSH
- percepční nedoslýchavost * etiologie patofyziologie MeSH
- pooperační komplikace * etiologie patofyziologie MeSH
- retrospektivní studie MeSH
- senioři MeSH
- Check Tag
- dospělí MeSH
- lidé středního věku MeSH
- lidé MeSH
- mužské pohlaví MeSH
- senioři MeSH
- ženské pohlaví MeSH
- Publikační typ
- časopisecké články MeSH
Computer models of the individual components of the peripheral auditory system - the outer, middle, and inner ears and the auditory nerve - have been developed in the past, with varying level of detail, breadth, and faithfulness of the underlying parameters. Building on previous work, we advance the modeling of the ear by presenting a complete, physiologically justified, bottom-up computer model based on up-to-date experimental data that integrates all of these parts together seamlessly. The detailed bottom-up design of the present model allows for the investigation of partial hearing mechanisms and their defects, including genetic, molecular, and microscopic factors. Also, thanks to the completeness of the model, one can study microscopic effects in the context of their implications on hearing as a whole, enabling the correlation with neural recordings and non-invasive psychoacoustic methods. Such a model is instrumental for advancing quantitative understanding of the mechanism of hearing, for investigating various forms of hearing impairment, as well as for devising next generation hearing aids and cochlear implants.
- Klíčová slova
- Auditory nerve, Cochlear amplifier, Computer modeling, Hair cell specialization, Mechano-electrical transduction, Peripheral auditory system, Ribbon synapse,
- MeSH
- akustická stimulace MeSH
- lidé MeSH
- nedoslýchavost * MeSH
- nervus cochlearis MeSH
- sluch * MeSH
- zevní ucho MeSH
- Check Tag
- lidé MeSH
- Publikační typ
- časopisecké články MeSH
- práce podpořená grantem MeSH
Swept-sines provide a tool for fast and high-resolution measurement of evoked otoacoustic emissions. During the measurement, a response to swept-sine(s) is recorded by a probe placed in the ear canal. Otoacoustic emissions can then be extracted by various techniques, e.g., Fourier analysis, the heterodyne method, and the least-square-fitting (LSF) technique. This paper employs a technique originally proposed with exponential swept-sines, which allows for direct emission extraction from the measured intermodulation impulse response. It is shown here that the technique can be used to extract distortion-product otoacoustic emissions (DPOAEs) evoked with two simultaneous swept-sines. For proper extraction of the DPOAE phase, the technique employs previously proposed adjusted formulas for exponential swept-sines generating so-called synchronized swept-sines (SSSs). Here, the SSS technique is verified using responses derived from a numerical solution of a cochlear model and responses measured in human subjects. Although computationally much less demanding, the technique yields comparable results to those obtained by the LSF technique, which has been shown in the literature to be the most noise-robust among the emission extraction methods.
- MeSH
- Fourierova analýza MeSH
- kochlea * fyziologie MeSH
- lidé MeSH
- otoakustické emise spontánní * fyziologie MeSH
- zvukovod fyziologie MeSH
- Check Tag
- lidé MeSH
- Publikační typ
- časopisecké články MeSH
- práce podpořená grantem MeSH
INTRODUCTION: Currently, it is possible to preserve the auditory nerve in a large number of cases, but the preservation of the hearing itself is unpredictable. Apart from wait and scan strategy and stereoradiotherapy, hearing after vestibular schwannoma surgery is considered to remain stable even in long-term follow-up. MATERIALS AND METHODS: Twenty-eight patients had preserved hearing after retrosigmoid suboccipital microsurgery of the vestibular schwannoma between 2008 and 2014. A standard audiological protocol was performed together with an magnetic resonance imaging evaluation of the fluid content of the inner ear. RESULTS: The mean difference in pure-tone average between the direct and final postsurgical examination was 12.758 dB ( p = 2.5E - 06). The word recognition score deteriorated by 17.45% ( p = 0.03516). The mean American Academy of Otolaryngology-Head and Neck Surgery score on the second examination was 2.5, and that on the second examination was 3.111 ( p = 0.00483). There was no significant deterioration in the healthy ear.The signal intensity ratio in the basal turn of the cochlea increased by an average of 0.13 points ( p < 0.05).Patients with persistent tumor or nodular enhancement in the internal acoustic meatus deteriorated significantly in hearing according to the American Academy of Otolaryngology-Head and Neck Surgery scale compared with patients without any finding in the meatus ( p = 0.01299). CONCLUSIONS: There is a discrete but gradual deterioration of the hearing in the postoperative period. Hearing impairment is more pronounced in patients with a nodular process in the internal acoustic meatus, regardless of whether it is growth active. After surgery, the pathological content of the inner ear normalizes (evaluated on T2 magnetic resonance imaging sequences).
- MeSH
- kochlea MeSH
- lidé MeSH
- nedoslýchavost * etiologie MeSH
- retrospektivní studie MeSH
- sluch MeSH
- vestibulární schwannom * chirurgie MeSH
- vnitřní ucho * chirurgie MeSH
- výsledek terapie MeSH
- Check Tag
- lidé MeSH
- Publikační typ
- časopisecké články MeSH
PURPOSE: This study aimed to determine hearing thresholds in an otologically normal population without occupational noise exposure aged 18 to 64 years using extended high-frequency audiometry (EHFA). METHODS: Individuals from the general population who have never had hearing problems and whose job was not associated with noise exposure were included in the study and classified by age into 5 categories: 18-24 and, further, by 10 years of age. Each of these groups was further divided according to gender. All subjects underwent tympanometry, conventional pure-tone audiometry within the 0.125-8 kHz range, and extended high-frequency audiometry within the 9-16 kHz range, performed according to the standards. The significance level for statistical testing was set at 5%. RESULTS: Here, we established hearing thresholds in an otologically healthy population within the extended high-frequency (EHF) range (9-16 kHz). We found the EHFA to be a highly sensitive method for early detection of hearing loss, with hearing thresholds decreasing as soon as 35 years of age. In males, the hearing thresholds grew with age more rapidly than in women. The ability to respond at EHF gradually decreased with age and increasing frequency. CONCLUSION: Our results can help improve the knowledge of EHF hearing thresholds for individual sexes and age groups. So far, the standard 7029:2017 is not binding and, moreover, it only reaches up to the frequency of 12.5 kHz. EHFA is a highly sensitive method for the evaluation of hearing loss depending on age and sex.
- Klíčová slova
- Audiogram, Audiometry, Extended high-frequency audiometry, Hearing loss, Hearing test, Hearing threshold,
- MeSH
- akustické impedanční testy MeSH
- audiometrie čistými tóny MeSH
- audiometrie MeSH
- dítě MeSH
- dospělí MeSH
- hluchota * MeSH
- lidé MeSH
- nedoslýchavost z hluku * diagnóza MeSH
- nedoslýchavost * diagnóza MeSH
- sluch MeSH
- sluchový práh MeSH
- Check Tag
- dítě MeSH
- dospělí MeSH
- lidé MeSH
- mužské pohlaví MeSH
- ženské pohlaví MeSH
- Publikační typ
- časopisecké články MeSH
The cranial window technique has proven to be an effective method for in vivo imaging of cortical activity. However, given the invasive nature of this procedure, possible side effects could be expected in the nervous system. In this study, we evaluated the effects of unilateral cranial window surgery on auditory function in C57BL6 mice using electrophysiological and behavioral approaches. We found that one week after implantation, mice exhibited both increased thresholds and decreased amplitudes of their auditory brainstem responses. These changes were accompanied by a decrease in distortion product otoacoustic emissions, indicating a deterioration in cochlear function. In addition, behavioral testing of these mice revealed reduced suppression of their acoustic startle response by gap prepulse, suggesting a deficit in auditory processing or possibly the presence of tinnitus. The changes in auditory function appeared to be only partially reversible within four weeks after surgery. Thus, our findings suggest that cranial window implantation causes long-term functional changes in the auditory system that should be considered when interpreting data from optical imaging techniques.
- Klíčová slova
- Auditory, Chronic cranial window, Hearing loss, Surgery trauma,
- MeSH
- myši inbrední C57BL MeSH
- myši MeSH
- protézy a implantáty MeSH
- sluch * MeSH
- sluchové kmenové evokované potenciály MeSH
- úleková reakce * MeSH
- zvířata MeSH
- Check Tag
- myši MeSH
- zvířata MeSH
- Publikační typ
- časopisecké články MeSH
- práce podpořená grantem MeSH
The basilar membrane in the cochlea can be modeled as an array of fluid coupled segments driven by stapes vibration and by the undamping nonlinear force simulating cochlear amplification. If stimulated with two tones, the model generates additional tones due to nonlinear distortion. These distortion products (DPs) can be transmitted into the ear canal and produce distortion-product otoacoustic emissions (DPOAEs) known to be generated in the healthy ear of various vertebrates. This study presents a solution for DPs in a two-dimensional nonlinear cochlear model with cochlear roughness-small irregularities in the impedance along the basilar membrane, which may produce additional DPs due to coherent reflection. The solution allows for decomposition of various sources of DPs in the model. In addition to the already described nonlinear-distortion and coherent-reflection mechanisms of DP generation, this study identifies a long-latency DPOAE component due to perturbation of nonlinear force. DP wavelets that are coherently reflected due to impedance irregularities travel toward the stapes across the primary generation region of DPs and there evoke perturbation of the nonlinear undamping force. The ensuing DP wavelets have opposite phase to the wavelets arising from coherent reflection, which results in partial cancellation of the coherent-reflection DP wavelets.
OBJECTIVES: Hearing preservation after vestibular schwannoma (VS) surgery remains a surgical challenge. In some patients with preserved inner ear function, hearing improvement is achievable. As it is currently impossible to determine which patients will present this outcome, predictions must rely on previously published reports. Our case report describes a patient who experienced hearing improvement from an unuseful level to a useful one after vestibular schwannoma surgery. METHODS: Surgery was performed via suboccipital retrosigmoid approach. The patient underwent a basic audiovestibular protocol before and after the surgery - pure tone and speech audiometry, otoacoustic emissions, auditory brainstem responses, electronystagmography - together with a detailed questionnaire study. Usefulness of hearing was evaluated using the AAO-HNS guidelines, supplemented by a frequency of 4 kHz. RESULTS: Hearing was preserved and even improved from an unuseful level to a useful one. Based on the available literature, the most informative predictive factors for such a result seem to be: sudden sensorineural hearing loss prior to surgery, elicitable otoacoustic emissions and the origin from the superior vestibular nerve. CONCLUSION: There are a limited number of studies on this topic and it is still impossible to regularly improve hearing in properly selected patients. Furthermore, the importance of postoperative hearing quality compared to other symptoms and complications remains debatable.
- Klíčová slova
- hearing improvement, quality of life, retrosigmoid approach, vestibular schwannoma,
- MeSH
- audiometrie čistými tóny MeSH
- lidé MeSH
- retrospektivní studie MeSH
- sluch fyziologie MeSH
- vestibulární schwannom * komplikace chirurgie MeSH
- výsledek terapie MeSH
- Check Tag
- lidé MeSH
- Publikační typ
- časopisecké články MeSH
- kazuistiky MeSH
- přehledy MeSH
