Progressive hereditary hearing impairment caused by a MYO6 mutation resembles presbyacusis.
Abstract Since deafness is the most common sensorineural disorder in humans, better understanding of the underlying causes is necessary to improve counseling and rehabilitation. A Dutch family with autosomal dominantly inherited sensorineural hearing loss was clinically and genetically assessed. The MYO6 gene was selected to be sequenced because of similarities with other, previously described DFNA22 phenotypes and a pathogenic c.3610C>T (p.R1204W) mutation was found to co-segregate with the disease. This missense mutation results in a flat configured audiogram with a mild hearing loss, which becomes severe to ...
Source: Hearing Research - January 19, 2013 Category: Audiology Authors: Oonk AM, Leijendeckers JM, Lammers EM, Weegerink NJ, Oostrik J, Beynon AJ, Huygen PL, Kunst HP, Kremer H, Snik AF, Pennings RJ Tags: Hear Res Source Type: research
Optimal Ossicular Site for Maximal Vibration Transmissions to Coupled Transducers.
In this study, vibrational responses at seven locations on the middle-ear ossicles (i.e., the malleus head, 4 different points on the incus body, middle of the incus long process, tip of the incus long process) in human temporal bones (n=6) were measured using a laser Doppler vibrometer. The measurements were repeated after separating the incudostapedial joint (ISJ). Measured displacement at each location was normalized with the sound pressure level near the tympanic membrane (TM) for representation in the form of a displacement transfer function (DTF). The normalized squared sum of the DTFs (NSSDTF) was then calculated as...
Source: Hearing Research - January 18, 2013 Category: Audiology Authors: Chung J, Song WJ, Sim JH, Kim W, Oh SH Tags: Hear Res Source Type: research
A historical to present-day account of efforts to answer the question, "what puts the brakes on mammalian hair cell regeneration?"
A historical to present-day account of efforts to answer the question, "what puts the brakes on mammalian hair cell regeneration?" Hear Res. 2013 Jan 17; Authors: Burns JC, Corwin JT Abstract Hearing and balance deficits often affect humans and other mammals permanently, because their ears stop producing hair cells within a few days after birth. But production occurs throughout life in the ears of sharks, bony fish, amphibians, reptiles, and birds allowing them to replace lost hair cells and quickly recover after temporarily experiencing the kinds of sensory deficits that are irreversible for mam...
Source: Hearing Research - January 17, 2013 Category: Audiology Authors: Burns JC, Corwin JT Tags: Hear Res Source Type: research
Detection of acoustic temporal fine structure by cochlear implant listeners: Behavioral results and computational modeling.
Abstract A test of within-channel detection of acoustic temporal fine structure (aTFS) cues is presented. Eight cochlear implant listeners (CI) were asked to discriminate between two Schroeder-phase (SP) complexes using a two-alternative, forced-choice task. Because differences between the acoustic stimuli are primarily constrained to their aTFS, successful discrimination reflects a combination of the subjects' perception of and the strategy's ability to deliver aTFS cues. Subjects were mapped with single-channel Continuous Interleaved Sampling (CIS) and Simultaneous Analog Stimulation (SAS) strategies. To compare...
Source: Hearing Research - January 17, 2013 Category: Audiology Authors: Imennov NS, Won JH, Drennan WR, Jameyson E, Rubinstein JT Tags: Hear Res Source Type: research
Dynamic properties of round window membrane in guinea pig otitis media model measured with electromagnetic stimulation.
Abstract The round window, one of two openings into the cochlea from the middle ear, plays an important role in hearing and is known to be structurally altered during otitis media. However, there have been no published studies systematically describing the changes in biomechanical properties of the round window membrane (RWM) that accompany bacterial otitis media. Here we describe the occurrence of significant changes in the dynamic properties of the RWM between normal guinea pigs and those with acute otitis media (AOM) that are detectable by electromagnetic force stimulation and laser Doppler vibrometry (LDV) mea...
Source: Hearing Research - January 16, 2013 Category: Audiology Authors: Gan RZ, Nakmali D, Zhang X Tags: Hear Res Source Type: research
A brief history of hair cell regeneration research and speculations on the future.
This article is part of a Special Issue entitled "Inner Ear Development and Regeneration". PMID: 23321648 [PubMed - as supplied by publisher] (Source: Hearing Research)
Source: Hearing Research - January 12, 2013 Category: Audiology Authors: Rubel EW, Furrer SA, Stone JS Tags: Hear Res Source Type: research
Masking patterns for monopolar and phantom electrode stimulation in cochlear implants.
Abstract Phantom electrode (PE) stimulation consists of out-of-phase stimulation of two electrodes. When presented at the apex of the electrode array, phantom stimulation is known to produce a lower pitch sensation than monopolar (MP) stimulation on the most apical electrode. The ratio of the current between the primary electrode (PEL) and the compensating electrode (CEL) is represented by the coefficient σ, which ranges from 0 (monopolar) to 1 (full bipolar). The exact mechanism by which PE stimulation produces a lower pitch sensation is unclear. In the present study, unmasked and masked thresholds were obt...
Source: Hearing Research - January 5, 2013 Category: Audiology Authors: Saoji AA, Landsberger DM, Padilla M, Litvak LM Tags: Hear Res Source Type: research
Pulmonary delivery of d-methionine is associated with an increase in ALCAR and glutathione in cochlear fluids.
This report also demonstrates the validity of the pulmonary administration of antioxidants and highlights the different contributions of d-met and ALCAR allowing to further investigate their impact on oxidative stress in the cochlear microenvironment. PMID: 23296212 [PubMed - as supplied by publisher] (Source: Hearing Research)
Source: Hearing Research - January 4, 2013 Category: Audiology Authors: Grondin Y, Cotanche DA, Manneberg O, Molina R, Treviño-Villarreal JH, Sepulveda R, Clifford R, Bortoni ME, Forsberg S, Labrecque B, Altshul L, Brain JD, Jackson RL, Rogers RA Tags: Hear Res Source Type: research
Perspective of functional magnetic resonance imaging in middle ear research.
Abstract Functional magnetic resonance imaging (MRI) studies have frequently been applied to study sensory system such as vision, language, and cognition, but have proceeded at a considerably slower speed in investigating middle ear and central auditory processing. This is due to several factors, including the intrinsic anatomy of the middle ear system and inherent acoustic noise during acquisition of MRI data. However, accumulating evidences have demonstrated that clarification of some fundamental neural underpinnings of audition associated with middle ear mechanics can be achieved using functional MRI metho...
Source: Hearing Research - January 2, 2013 Category: Audiology Authors: Chang Y, Lee SH Tags: Hear Res Source Type: research
Standardized tinnitus-specific individual cognitive-behavioral therapy: A controlled outcome study with 286 tinnitus patients.
Abstract BACKGROUND: Pharmacological treatment of tinnitus cannot be considered well established. Thus, reducing tinnitus severity through behavioral therapy is emerging as a key goal. METHODS: A total of 286 patients suffering from persistent and stable tinnitus for four months or longer participated in this controlled clinical multicenter study. The study investigated the efficacy and safety of a standardized treatment involving individual cognitive-behavioral therapy (CBT). Controls were 120 patients waiting to be treated. Therapy was standardized using manualized procedures within the setting of a specifically...
Source: Hearing Research - December 31, 2012 Category: Audiology Authors: Zenner HP, Vonthein R, Zenner B, Leuchtweis R, Plontke SK, Torka W, Pogge S, Birbaumer N Tags: Hear Res Source Type: research
Special Issue on Inner Ear Development and Regeneration.
PMID: 23276729 [PubMed - as supplied by publisher] (Source: Hearing Research)
Source: Hearing Research - December 28, 2012 Category: Audiology Authors: Heller S Tags: Hear Res Source Type: research
Development of Hyperactivity after Acoustic Trauma in the Guinea Pig Inferior Colliculus.
Abstract The time of onset of hyperactivity (increased spontaneous firing rates) was investigated by single neuron recording in the inferior colliculus (IC) of guinea pigs subjected to unilateral acoustic trauma (exposure to a loud 10kHz tone). Hyperactivity was present by 12hrs post acoustic trauma whereas data obtained within approximately 4hrs of the cessation of acoustic trauma found no evidence of hyperactivity. These data suggest that hyperactivity in the IC begins at some time between 4 and 12hrs post trauma and is a relatively rapid plastic event beginning within hours rather than days post cochlear trauma...
Source: Hearing Research - December 28, 2012 Category: Audiology Authors: Mulders WH, Robertson D Tags: Hear Res Source Type: research
The Effect of Static Force on Round Window Stimulation with the Direct Acoustic Cochlea Stimulator.
Abstract The Direct Acoustic Cochlea Stimulator Partial Implant (DACS PI, Phonak Acoustic Implants SA, Switzerland) is intended to stimulate the cochlea by a conventional stapedotomy piston that is crimped onto the DACS PI artificial incus. An alternative approach to the round window (RW) is successfully done with other devices, having the advantage of being also independent of the existence of middle ear structure (e.g. ossicles). Here the possibility of stimulating the RW with the DACS actuator is investigated including the impact of static force on sound transmission to the cochlea. The maximum equivalent sound...
Source: Hearing Research - December 28, 2012 Category: Audiology Authors: Maier H, Salcher R, Schwab B, Lenarz T Tags: Hear Res Source Type: research
Finite Element Modeling of Energy Absorbance in Normal and Disordered Human Ears.
In this study, a FE model of the human ear, including the ear canal, the middle ear and the spiral cochlea constructed from the histological sections of a human temporal bone, was used to calculate EA. The viscoelastic material properties were applied to the middle ear soft tissues. Three middle ear disorders were simulated in the FE model: otitis media, otosclerosis, and ossicular chain disarticulation. Multi-physics (acoustic, structure, and fluid) coupled analysis was conducted in the model. The FE model was first validated with the published experimental data on the middle ear input impedance and EA of the normal ear. ...
Source: Hearing Research - December 27, 2012 Category: Audiology Authors: Zhang X, Gan RZ Tags: Hear Res Source Type: research
Changes in cochlear function during acute endolymphatic hydrops development in guinea pigs.
Abstract Previous studies have injected artificial endolymph into scala media in anaesthetized guinea pigs as an acute model of endolymphatic hydrops. Here, we have injected artificial endolymph into scala media in guinea pigs at rates of 40-80 nl/min, whilst monitoring Compound Action Potential (CAP) thresholds, the Summating Potential (SP)/CAP ratio, Cochlear Microphonic (CM) distortion, low-frequency modulated Distortion Product Otoacoustic Emissions (DPOAEs), and the Endocochlear Potential (EP). We found that abrupt recovery of CAP thresholds, SP/CAP ratio, and CM and DPOAE asymmetric distortion could occ...
Source: Hearing Research - December 25, 2012 Category: Audiology Authors: Brown DJ, Chihara Y, Curthoys IS, Wang Y, Bos M Tags: Hear Res Source Type: research
Modelling detection thresholds for sounds repeated at different delays.
This study shows that a simple probabilistic model based on Neubauer and Heil (2008) predicts the increase in threshold for short temporal delays as well as the asymptotic behaviour towards longer delays. The model entails (i) a 4th-order gammatone filter with a brief impulse response and thus broad bandwidth (shorter and broader than those of a filter normally assumed), (ii) the formation of stochastic 'spikes' or 'events' whose probability of occurrence is proportional to the filter output (half-wave rectified fine-structure or amplitude envelope), raised to a power of 3, and (iii) probability summation. The same model w...
Source: Hearing Research - December 23, 2012 Category: Audiology Authors: Heil P, Verhey JL, Zoefel B Tags: Hear Res Source Type: research
Two-source interference as the major reason for auditory-threshold estimation error based on DPOAE input-output functions in normal-hearing subjects.
, Gummer AW Abstract Fine structure in the frequency response of distortion product otoacoustic emissions (DPOAEs) can severely limit the usefulness of DPOAEs in estimating auditory thresholds. Here, fine structure is removed by extracting the primary-source DPOAE component using the onset-decomposition technique (Vetešník et al., 2009) and auditory threshold estimates are compared to those obtained from DPOAEs in response to conventional, continuous two-tone stimulation. Auditory thresholds are predicted using the estimated distortion product thresholds (EDPTs), obtained from linear regression ...
Source: Hearing Research - December 23, 2012 Category: Audiology Authors: Dalhoff E, Turcanu D, Vetešník A, Gummer AW Tags: Hear Res Source Type: research
Development of response selectivity in the mouse auditory cortex.
Abstract The mouse auditory system contains neurons selective for tone duration and for a narrow range of frequency modulated (FM) sweep rates. Whether such selectivity is developmentally regulated is not known. The main goal of this study was to follow the development of neuronal responses to tones (frequency and duration tuning) and FM sweeps (direction and rate selectivity) in the core auditory cortex (A1 and AAF) of ketamine/xylazine anesthetized C57bl/6 mice. Three groups were compared: postnatal day (P) 15-20, P21-30 and P31-90. Frequency tuning bandwidth decreased during the first month indicating refinemen...
Source: Hearing Research - December 20, 2012 Category: Audiology Authors: Carrasco MM, Trujillo M, Razak K Tags: Hear Res Source Type: research
Gene therapy for the inner ear.
We describe several gene vectors useful for inner ear gene therapy and the practical aspects of introducing these vectors into the ear. We then review the progress toward using gene transfer for therapies in both auditory and balance systems, and discuss the technological milestones needed to advance to clinical application of these methods. PMID: 23265411 [PubMed - as supplied by publisher] (Source: Hearing Research)
Source: Hearing Research - December 18, 2012 Category: Audiology Authors: Fukui H, Raphael Y Tags: Hear Res Source Type: research
Measurements of three-dimensional shape and sound-induced motion of the chinchilla tympanic membrane.
This article is part of a Special Issue entitled "MEMRO 2012". Summary: A new method for simultaneously measuring the shape and sound-induced motion of the tympanic membrane is utilized to estimate the 3D motion on the membrane surface. PMID: 23247058 [PubMed - as supplied by publisher] (Source: Hearing Research)
Source: Hearing Research - December 13, 2012 Category: Audiology Authors: Rosowski JJ, Dobrev I, Khaleghi M, Lu W, Cheng JT, Harrington E, Furlong C Tags: Hear Res Source Type: research
Influence of the middle ear anatomy on the performance of a membrane sensor in the incudostapedial joint gap.
This study investigates the implantable microphone sensor and its implantation concept. It intends to quantify the influence of the sensor's insertion position on the achievable microphone sensitivity. The investigation considers anatomical and pathological variations of the middle ear geometry and its space limitations. Temporal bone experiments on a laboratory model show that anatomical and pathological variations of the middle ear geometry can prevent the sensor from being placed optimally within the incudostapedial joint. Beyond scattering of transfer functions due to anatomic variations of individual middle ears there...
Source: Hearing Research - December 12, 2012 Category: Audiology Authors: Koch M, Seidler H, Hellmuth A, Bornitz M, Lasurashvili N, Zahnert T Tags: Hear Res Source Type: research
The response properties of neurons in different fields of the auditory cortex in the rat.
We describe here the functional organization of the AC in young rats (strain Long Evans, aged 30-35 days, anesthetized with ketamine/xylazine) on the basis of the neuronal responses to acoustic stimuli. Based on the neuronal responses to broad band noise (BBN) and pure tone bursts, the AC may be divided into the primary auditory cortex (AI) and three other core fields: anterior (AAF), suprarhinal (SRAF) and posterior (PAF) as well as an unspecific region (UR) inserted between the AI and AAF. The core fields are surrounded by a belt area. Neurons in the AI, AAF, SRAF and PAF showed well defined characteristic frequencies (C...
Source: Hearing Research - December 6, 2012 Category: Audiology Authors: Profant O, Burianová J, Syka J Tags: Hear Res Source Type: research
Comodulation Masking Release induced by controlled electrical stimulation of auditory nerve fibers.
Abstract Normal-hearing listeners can perceptually segregate concurrent sound sources, but listeners with significant hearing loss or who wear a Cochlear Implant (CI) lag behind in this ability. Perceptual grouping mechanisms are essential to segregate concurrent sound sources and affect Comodulation Masking Release (CMR). Thus, CMR measurements in CI users could shed light on segregation cues needed for forming and grouping of auditory objects. CMR illustrates the fact that detection of a target sound embedded in a fluctuating masker is improved by the addition of masker energy remote from the target frequency, p...
Source: Hearing Research - December 5, 2012 Category: Audiology Authors: Zirn S, Hempel JM, Schuster M, Hemmert W Tags: Hear Res Source Type: research
Evidence of inner ear contribution in bone conduction in chinchilla.
wski JJ Abstract We investigated the contribution of the middle ear to the physiological response to bone conduction stimuli in chinchilla. We measured intracochlear sound pressure in response to air conduction (AC) and bone conduction (BC) stimuli before and after interruption of the ossicular chain at the incudo-stapedial joint. Interruption of the chain effectively decouples the external and middle ear from the inner ear and significantly reduces the contributions of the outer ear and middle ear to the bone conduction response. With AC stimulation, both the scala vestibuli Psv and scala tympani Pst sound pressu...
Source: Hearing Research - December 1, 2012 Category: Audiology Authors: Chhan D, Röösli C, McKinnon ML, Rosowski JJ Tags: Hear Res Source Type: research
Return of function after hair cell regeneration.
This article is part of a Special Issue entitled "Inner Ear Development and Regeneration". PMID: 23202051 [PubMed - as supplied by publisher] (Source: Hearing Research)
Source: Hearing Research - November 29, 2012 Category: Audiology Authors: Ryals BM, Dent ML, Dooling RJ Tags: Hear Res Source Type: research
Hearing in American leaf-nosed bats. IV: The Common vampire bat, Desmodus rotundus.
Abstract We behaviorally determined the audiograms of three Common vampire bats (Phyllostomidae, Desmodus rotundus), a species specialized to exist exclusively on blood. The bats were trained to respond to pure tones in a conditioned suppression/avoidance procedure for a blood reward and a mild punisher for failures to detect the tones. Common vampire bats have a hearing range from 716 Hz to 113 kHz at a level of 60 dB. Their best hearing is at 20 kHz where they are slightly more sensitive than other bats, and they have a second peak of good sensitivity at 71 kHz. They have unusually good ...
Source: Hearing Research - November 27, 2012 Category: Audiology Authors: Heffner RS, Koay G, Heffner HE Tags: Hear Res Source Type: research
Induction of the inner ear: Stepwise specification of otic fate from multipotent progenitors.
This article is part of a Special Issue entitled . PMID: 23194992 [PubMed - as supplied by publisher] (Source: Hearing Research)
Source: Hearing Research - November 27, 2012 Category: Audiology Authors: Chen J, Streit A Tags: Hear Res Source Type: research
Salicylate-induced cochlear impairments, cortical hyperactivity and re-tuning, and tinnitus.
Abstract High doses of sodium salicylate (SS) have long been known to induce temporary hearing loss and tinnitus, effects attributed to cochlear dysfunction. However, our recent publications reviewed here show that SS can induce profound, permanent, and unexpected changes in the cochlea and central nervous system. Prolonged treatment with SS permanently decreased the cochlear compound action potential (CAP) amplitude in vivo. In vitro, high dose SS resulted in a permanent loss of spiral ganglion neurons and nerve fibers, but did not damage hair cells. Acute treatment with high-dose SS produced a frequenc...
Source: Hearing Research - November 27, 2012 Category: Audiology Authors: Chen GD, Stolzberg D, Lobarinas E, Sun W, Ding D, Salvi R Tags: Hear Res Source Type: research
Vestibular nuclei characterized by calcium-binding protein immunoreactivity and tract tracing in Gekko gecko.
Abstract Immunohistochemical techniques were used to describe the distribution of the calcium binding proteins calretinin, calbindin and parvalbumin as well as synaptic vesicle protein 2 in the vestibular nuclei of the Tokay gecko (Gekko gecko). In addition, tract tracing was used to investigate connections between the vestibular nerves and brainstem nuclei. Seven vestibular nuclei were recognized: the nuclei cerebellaris lateralis (Cerl), vestibularis dorsolateralis (Vedl), ventrolateralis (Vevl), ventromedialis (Vevm), tangentialis (Vetg), ovalis (VeO) and descendens (Veds). Vestibular fibers entered the brainst...
Source: Hearing Research - November 27, 2012 Category: Audiology Authors: Song J, Wang W, Carr CE, Dai Z, Tang Y Tags: Hear Res Source Type: research
Beyond generalized hair cells: Molecular cues for hair cell types.
Abstract Basic helix-loop-helix (bHLH) transcription factors (TFs) are crucial for inner ear neurosensory development. The proneural TF Atoh1 regulates the differentiation of hair cells (HCs) whereas Neurog1 and Neurod1 regulate specification and differentiation of neurons, respectively, but also affect HC development. Expression of Delta and Jagged ligands in nascent HCs and Notch receptors in supporting cells induce supporting cell differentiation through the regulation of neurogenic bHLH TFs (such as Hes1, Hes5) and suppression of limited Atoh1 expression. In sensorineural hearing loss, HCs are lost followed by...
Source: Hearing Research - November 27, 2012 Category: Audiology Authors: Jahan I, Pan N, Kersigo J, Fritzsch B Tags: Hear Res Source Type: research
Intra-tympanic delivery of short interfering RNA into the adult mouse cochlea.
Abstract Trans-tympanic injection into the middle ear has long been the standard for local delivery of compounds in experimental studies. Here we demonstrate the advantages of the novel method of intra-tympanic injection through the otic bone for the delivery of compounds or siRNA into the adult mouse cochlea. First, a fluorescently-conjugated scrambled siRNA probe was applied via intra-tympanic injection into the middle ear cavity and was detected in sensory hair cells and nerve fibers as early as 6 h after the injection. The fluorescent probe was also detected in other cells of the organ of Corti, the later...
Source: Hearing Research - November 23, 2012 Category: Audiology Authors: Oishi N, Chen FQ, Zheng HW, Sha SH Tags: Hear Res Source Type: research
Sound transmission along the ossicular chain in common wild-type laboratory mice.
Abstract The use of genetically modified mice can accelerate progress in auditory research. However, the fundamental profile of mouse hearing has not been thoroughly documented. In the current study, we explored mouse middle ear transmission by measuring sound-evoked vibrations at several key points along the ossicular chain using a laser-Doppler vibrometer. Observations were made through an opening in pars flaccida. Simultaneously, the pressure at the tympanic membrane close to the umbo was monitored using a micro-pressure-sensor. Measurements were performed in C57BL mice, which are widely used in hearing researc...
Source: Hearing Research - November 23, 2012 Category: Audiology Authors: Dong W, Varavva P, Olson ES Tags: Hear Res Source Type: research
Changes in the adult vertebrate auditory sensory epithelium after trauma.
Abstract Auditory hair cells transduce sound vibrations into membrane potential changes, ultimately leading to changes in neuronal firing and sound perception. This review provides an overview of the characteristics and repair capabilities of traumatized auditory sensory epithelium in the adult vertebrate ear. Injured mammalian auditory epithelium repairs itself by forming permanent scars but is unable to regenerate replacement hair cells. In contrast, injured non-mammalian vertebrate ear generates replacement hair cells to restore hearing functions. Non-sensory support cells within the auditory epithelium play ke...
Source: Hearing Research - November 20, 2012 Category: Audiology Authors: Oesterle EC Tags: Hear Res Source Type: research
Steady-state MEG responses elicited by a sequence of amplitude-modulated short tones of different carrier frequencies.
In this study, we examined magnetoencephalographic (MEG) ASSR using a sequence of sinusoidal AM tones of 0.78 s in length with various tone frequencies of 440-990 Hz in about one octave variation. It was found that the amplitude of the ASSR was invariant with tone frequencies when the level of sound pressure was adjusted along an equal-loudness curve. The amplitude also did not depend on the existence of preceding tone or difference in frequency of the preceding tone. When the sound level of AM tones was changed with tone frequencies in the same range of 440-990 Hz, the amplitude of ASSR varied in a proporti...
Source: Hearing Research - November 19, 2012 Category: Audiology Authors: Kuriki S, Kobayashi Y, Kobayashi T, Tanaka K, Uchikawa Y Tags: Hear Res Source Type: research