Hearing loose

OBJECTIVES: Cochlear implant (CI) recipients are at a severe disadvantage compared with normal-hearing listeners in distinguishing consonants that differ by place of articulation because the key relevant spectral differences are degraded by the implant. One component of that degradation is the upward shifting of spectral energy that occurs with a shallow insertion depth of a CI. The present study aimed to systematically measure the effects of spectral shifting on word recognition and phoneme categorization by specifically controlling the amount of shifting and using stimuli whose identification specifically depends on perceiving frequency cues...

OBJECTIVES: There are no approved pharmacologic therapies for chronic sensorineural hearing loss (SNHL). The combination of CHIR99021+valproic acid (CV, FX-322) has been shown to regenerate mammalian cochlear hair cells ex vivo. The objectives were to characterize the cochlear pharmacokinetic profile of CV in guinea pigs, then measure FX-322 in human perilymph samples, and finally assess safety and audiometric effects of FX-322 in humans with chronic SNHL. STUDY DESIGNS: Middle ear residence, cochlear distribution, and elimination profiles of FX-322 were assessed in guinea pigs...

OBJECTIVES: To describe audiologic outcomes following hydroxyapatite bone cement fixation of stapedotomy prostheses. METHODS: A retrospective case review at a tertiary neurotology referral center was performed of patients undergoing primary or revision stapedotomy between 2010 and 2017. Patients with hydroxyapatite bone cement fixation of stapes prostheses were assessed. Pre- and postoperative hearing was compared, consisting of air conduction (AC) and bone conduction (BC) pure-tone averages (PTA), air-bone gap (ABG), and word recognition scores (WRS)...

Objective: The research is to study the expression and distribution of matrix metalloproteinase (MMPs)-2 and -9 in the guinea pig cochlea after noise exposure, and to explore the role of MMPs in the blood-labyrinth-barrier (BLB). In addition, the role of MMPs inhibitor doxycycline in noise-induced BLB trauma was studied as well, which provides the basis for further studies and prophylaxis of noise-induced hearing loss. Methods: A total of 45 healthy adult guinea pigs were randomly divided into the control group (15 received intraperitoneal injection of 0...

On the basis of multiple skeletal specimens from Liaoning, China, we report a new genus and species of Cretaceous stem therian mammal that displays decoupling of hearing and chewing apparatuses and functions. The auditory bones, including the surangular, have no bone contact with the ossified Meckel's cartilage; the latter is loosely lodged on the medial rear of the dentary. This configuration probably represents the initial morphological stage of the definitive mammalian middle ear. Evidence shows that hearing and chewing apparatuses have evolved in a modular fashion...

OBJECTIVE: To describe the first reported case of a fracture of the long process of the incus due to digital manipulation of the ear canal and to discuss diagnostic markers for ossicular fractures. PATIENT: A 46-year-old woman with incessant clicking and crunching in her left ear, and hearing loss after digital manipulation of the ear canal. INTERVENTION: Diagnostic evaluation and therapeutic ossiculoplasty. MAIN OUTCOME MEASURE(S): Audiometric and wideband acoustic immittance (WAI) measurements were made before surgery to investigate the cause of clicking sounds and mild conductive hearing loss (CHL)...

A Ca2+ current transient block (ICa TB) by protons occurs at some ribbon-type synapses after exocytosis, but this has not been observed at mammalian hair cells. Here we show that a robust ICa TB occurs at post-hearing mouse and gerbil inner hair cell (IHC) synapses, but not in immature IHC synapses, which contain non-compact active zones, where Ca2+ channels are loosely coupled to the release sites. Unlike ICa TB at other ribbon synapses, ICa TB in mammalian IHCs displays a surprising multi-peak structure that mirrors the EPSCs seen in paired recordings...

Triclosan (TCS) is a prevalent anthropogenic contaminant in aquatic environments and its chronic exposure can lead to a series of neurotoxic effects in zebrafish. Both qRT-PCR and W-ISH identified that TCS exposure resulted in significant up-regulation of miR-137, but downregulation of its regulatory genes (bcl11aa, MAPK6 and Runx1). These target genes are mainly associated with neurodevelopment and the MAPK signaling pathway, and showed especially high expression in the brain. After overexpression or knockdown treatments by manual intervention of miR-137, a series of abnormalities were induced, such as ventricular abnormality, bent spine, yolk cyst, closure of swim sac and venous sinus hemorrhage...

Noonan syndrome (NS), the most common of the RASopathies, is a developmental disorder caused by heterozygous germline mutations in genes encoding proteins in the RAS-MAPK signaling pathway. Noonan-like syndrome with loose anagen hair (NSLH, including NSLH1, OMIM #607721 and NSLH2, OMIM #617506) is characterized by typical features of NS with additional findings of macrocephaly, loose anagen hair, growth hormone deficiency in some, and a higher incidence of intellectual disability. All NSLH1 reported cases to date have had an SHOC2 c...

BACKGROUND: Theoretical disadvantages are associated with the use of heat-crimped piston prostheses in stapedotomy. Loose crimping could result in prosthesis displacement or slippage. Alternatively, overly tight crimping may cause trauma to the incus with resultant necrosis. OBJECTIVES: 1) Analyze outcomes of patients undergoing stapedotomy with heat-activated piston-style prostheses, and 2) compare outcomes between prostheses made by different manufacturers. STUDY DESIGN: Retrospective...

Tprn encodes the taperin protein, which is concentrated in the tapered region of hair cell stereocilia in the inner ear. In humans, TPRN mutations cause autosomal recessive nonsyndromic deafness (DFNB79) by an unknown mechanism. To determine the role of Tprn in hearing, we generated Tprn-null mice by clustered regularly interspaced short palindromic repeat/Cas9 genome-editing technology from a CBA/CaJ background. We observed significant hearing loss and progressive degeneration of stereocilia in the outer hair cells of Tprn-null mice starting from postnatal day 30...

Xia-Gibbs syndrome (XGS) is a recently described neurodevelopmental disorder due to heterozygous loss-of-function AHDC1 mutations. XGS is characterized by global developmental delay, intellectual disability, hypotonia, and sleep abnormalities. Here we report the clinical phenotype of five of six individuals with XGS identified prospectively at the Children's Hospital of Philadelphia, a tertiary children's hospital in the USA. Although all five patients demonstrated common clinical features characterized by developmental delay and characteristic facial features, each of our patients showed unique clinical manifestations...

This manuscript introduces a novel concept for measuring coil coupling for extremely loose-coupled coils (coupling factors k<10-6; mutual inductance values M<10-10 H). Such a coupling is found everywhere where the ratio of solenoid diameter to coil spacing is >50. Measuring these quantities with a low-power technology requires a sophisticated setup that goes beyond the sensitivity of state-of-the art approaches. The methodology is validated using laboratory measurements with three sets of solenoids (two ferrite-cored, one air-cored) and numerical simulations with COMSOL Multiphysics 5...

Normal blood supply to the cochlea is critical for hearing. Noise damages auditory sensory cells and has a marked effect on the microvasculature in the cochlear lateral wall. Pericytes in the stria vascularis (strial pericytes) are particularly vulnerable and sensitive to acoustic trauma. Exposure of NG2DsRedBAC transgenic mice (6-8 weeks old) to wide-band noise at a level of 120 dB for 3 h per day for 2 consecutive days produced a significant hearing threshold shift and caused pericytes to protrude and migrate from their normal endothelial attachment sites...

The anterior inferior cerebellar artery (AICA) usually runs loosely within the cerebellopontine cistern; in rare cases, however, it is firmly adherent to the petrous dura mater.1,2 Recognizing this variation is particularly important in vestibular schwannoma surgery via the retrosigmoid transmeatal approach to prevent the high morbidity associated with vascular injury. This video demonstrates a surgical technique to effectively mobilize the AICA when firmly adherent to the petrous dura mater. A 39-year-old man presented with a history of progressive right-sided hearing loss without facial weakness or other associated symptoms3...

The given and family names of all the co-authors are incorrect in the published article. The correct names should read as follows.

OBJECTIVE: To evaluate long-term hearing results of stapedotomy and analyze the influence of patient-, disease-, and procedure-related variables. STUDY DESIGN: Retrospective case series. SETTING: Tertiary referral center. PATIENTS: 230 ears (202 patients, 10-74 years) underwent stapedotomy for otosclerosis between January 2008 and August 2014. All cases had early postoperative follow-up (4 weeks post-surgery) and 181 cases had late postoperative follow-up (≥ 1 year, average 32...

The protracted maturational process of temporal processing in layer 4 (L4) of primary auditory cortex (A1) has been extensively studied. Accumulating evidences show that layer 5 (L5) receives direct thalamic inputs as well. How the temporal responses in L5 may developmentally emerge remains unclear. Using in vivo loose-patch recordings in rat A1, we found that putative pyramidal (Pyr) neurons in developing L5 exhibited adult-like stimulus-following ability but less bursting shortly after hearing onset. L5 Pyr neurons in adult A1 exhibited phase-locking similar to L4 neurons, while L5 fast-spiking (FS) neurons showed greater phase-locking at 7 and 12...

The Adenovirus (Ad) genome within the capsid is tightly associated with a virus-encoded, histone-like core protein-protein VII. Two other Ad core proteins, V and X/μ, also are located within the virion and are loosely associated with viral DNA. Core protein VII remains associated with the Ad genome during the early phase of infection. It is not known if naked Ad DNA is packaged into the capsid, as with dsDNA bacteriophage and herpesviruses, followed by the encapsidation of viral core proteins, or if a unique packaging mechanism exists with Ad where a DNA-protein complex is simultaneously packaged into the virion...

INTRODUCTION: Primary synovial osteochondromatosis of the hip is a benign disorder, which is not frequently seen clinically. The characteristic of this disease is proliferation of synovium and formation of loose bodies inside the joint. It is known that only the loose bodies derived from synovium were called synovial osteochondromatosis. Although it can take place in any joint, the knee is most commonly affected, involvement of the hip joint is relatively rare. We report a young man who has multiple synovial osteochondromatosis in his left hip...