Distortion product emissions (DPEs) at 2f1 f2 frequencies were measured in 53 human ears; 21 of them exhibited cochlear hearing loss. DPEs were obtained as a function of stimulus level (DPE growth curves) at seven frequency regions between 707 Hz and 5656 Hz. Several distinctly different shapes or patterns of DPE growth curves were observed. These included single-segment monotonic growth curves...

Dehiscence of the cochlear otic capsule has recently been described as a pathologic entity. We describe two cases of cochlear-facial dehiscence, which are the first reported: a 69-year-old male who complained of hearing loss, autophony, and pulsatile tinnitus and a 41-year-old female who complained of left-sided hearing loss, pulsatile tinnitus, and vertigo. In both, computed tomography (CT) sh...

Background: Congenital hearing loss delays many aspects of a child's development, including speech and socio-cognitive development. The aim of this study was determine the results of infant hearing screening with transient evoked otoacoustic emission (TEOAE) in Moradi Hospital, Rafsanjan, Iran: 2014. Materials and Methods: In this descriptive cross-sectional study from 6017 infants born in Nik...

OBJECTIVE To confirm the clinical efficacy and safety of a direct acoustic cochlear implant. STUDY DESIGN Prospective multicenter study. SETTING The study was performed at 3 university hospitals in Europe (Germany, The Netherlands, and Switzerland). PATIENTS Fifteen patients with severe-to-profound mixed hearing loss because of otosclerosis or previous failed stapes surgery. INTERVENTIO...

Identifying the multiple contributors to the audiometric loss of a hearing impaired (HI) listener at a particular frequency is becoming gradually more useful as new treatments are developed. Here, we infer the contribution of inner (IHC) and outer hair cell (OHC) dysfunction to the total audiometric loss in a sample of 68 hearing aid candidates with mild-to-severe sensorineural hearing loss, an...

cochlear implantation has become an increasingly common procedure in the rehabilitation of selected cases of profound deafness.' patients should have profound total bilateral sensorineural hearing loss. sound is transformed into small electric currents which stimulate the auditory nerves in the cochlea and generate the hearing sensation. the nucleus cochlear implant is the result of more t...

Background Harboyan syndrome, defined as congenital corneal dystrophy associated with progressive sensorineural hearing loss, was first described by Harboyan in 1971. It is a hereditary disease manifested by eye lesions consistent with corneal endothelial dystrophy and progressive sensorineural hearing loss. There is bilateral symmetric progressive hearing loss, which may be either dominant or ...

Cyclodextrins are sugar compounds that are increasingly finding medicinal uses due to their ability to complex with hydrophobic molecules. One cyclodextrin in particular, 2-hydroxypropyl-β-cyclodextrin (HPβCD), is used as a carrier to solubilize lipophilic drugs and is itself being considered as a therapeutic agent for treatment of Niemann-Pick Type C disease, due to its ability to mobilize cho...

perrault's syndrome (p.s.) is rare. the combination of gonadal dysgenesis and hearing loss was accompanied by 46,xxkaryotype in three sisters with parental consanguineous marriage. genetic investigation revealed normal female karyotype, positive liarr and negative fluorescence ipj-bodies, which was confirmed by molecular study on the basis of fluorescence in situ hybridization (pish), with appl...

Excessive exposure to noise damages the principal cochlear structures leading to hearing impairment. Inflammatory and immune responses are central mechanisms in cochlear defensive response to noise but, if unregulated, they contribute to inner ear damage and hearing loss. Transforming growth factor β (TGF-β) is a key regulator of both responses and high levels of this factor have been associate...