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Structural/audiometric correlations in a human inner ear with noise-induced hearing loss.

A morphological analysis was performed on a human cochlea removed during skull base surgery. The patient experienced a noise-induced hearing loss following 30 years of mechanical exposure. The tissue was processed according to the block surface technique and the organ of Corti, osseous spiral lamina and spiral ganglion were analyzed at different levels. There was a circumscribed lesion approx. 10 mm from the round window extending to about 13 mm. At this site, the dominant pathological feature was the loss of outer hair cells that was comprehensive in the centermost area and partial in the peripheral region of the damage. The degradation of inner hair cells was less severe with signs of cell atrophy yet with limited loss. Outer pillar cells were often collapsed leading to deformation of the acoustic ridge. The Deiters cells were often present and physically interactive with remaining nerve fibers. In the reticular lamina, surgical manipulation and dissection resulted in tears which may be attributed to a reduction of intercellular strength between cells. In the damaged area, there was a 45% loss of myelinated nerve fibers measured at the osseous spiral lamina. Pathological changes could not be observed in the spiral ganglion with certainty although the type II cells innervating the outer hair cells were often difficult to discern.

Audiometry↗

Continuous intratympanic infusion of gentamicin via a microcatheter in Menière's disease.

Between 1995 and 1998, 11 patients with disabling Menière's disease were treated at our institution with a continuous gentamicin infusion into the middle ear via a microcatheter. The patients had frequent attacks of vertigo and vomiting (functional levels 3-5). Hearing threshold on the affected side was significantly worse than on the healthy side (stage 4+5). Gentamicin was applied by a high-precision insulin pump with a flow rate of 40 mg per day directly in front of the round window. Application was stopped as soon as signs of vestibular affection appeared. A good overall control of vertiginous spells was achieved in 8 patients. Eight patients experienced complete hearing loss on the affected side, 1 experienced a slight worsening, and 1 had no hearing change. There was no correlation between the cumulative gentamicin dosage and the hearing loss. Our findings show that in terms of hearing loss and hospitalization time the continuous gentamicin application is inferior to other applications presented in the literature.

Administration, Topical↗

[Multichannel cochlear implant: personal experience].

Cochlear Implantation is a recognized procedure for acoustical rehabilitation of the profoundly or totally deaf unable to effectively use a traditional hearing aid. Since 1989, 30 patients (27 postlingual and 3 perilingual) have been operated on by the senior Author with the implantation of a multichannel device: Med-El (3 cases), Clarion (3 case) and Nucleus Mini-22 (24 cases). The first 24 Nucleus cases, with a multipeak speechcoding strategy, are the object of this retrospective report. Selection of patients was performed according to an international protocol which includes PTA and Speech Audiometry, CT and/or MRI imaging of the ear, the Promontorial Test, an evaluation of lip reading ability, a psychological evaluation, a trial with two recent and currently available aids. The operation was carried out under general anesthesia. An extended endaural approach was adopted. The internal package of the device was firmly seated onto the temporalis squama and the electrode array was introduced into the tympanic cleft via a posterior tympanotomy. The electrode system was inserted into the scala tympani through a cochleotomy drilled out at the anteroinferior border of the round window. The whole system was fixed to the bony walls of the mastoid and squama using an ionomeric bone bonding agent. Starting at the second postoperative month and up to the 24th month of utilization of the device, each patient was evaluated with regard to his or her ability to recognize environmental sounds, to detect prosody transitions and to understand speech with and without visual cues. Selected tests, either close or open set, were used from the MAC and the Iowa Cochlear Implant Battery, as well as the Helen test. Telephone conversation was also evaluated. Results were very encouraging in all cases, showing that the multichannel cochlear device provides the properly selected patient a substantial auditory rehabilitation allowing him or her to understand speech in most instances without any visual cue.

Adolescent↗

AAV-mediated delivery of the caspase inhibitor XIAP protects against cisplatin ototoxicity.

HYPOTHESIS: Delivery of the gene encoding X-linked inhibitor of apoptosis (XIAP) using an adeno-associated viral (AAV) vector can protect against cisplatin-mediated ototoxicity. BACKGROUND: Cisplatin is a widely used chemotherapeutic agent with significant ototoxic side effects. One possible mechanism of toxicity is apoptotic death of many cochlear cell types. Acute treatment with inhibitors of caspases- enzymes critical for apoptosis- has been shown to prevent hearing loss in vivo, but is too short-acting for therapeutic use. Gene therapy provides a specific and chronic means of delivering potential therapeutic gents. Introducing an anti-apoptotic gene into the cochlea could provide long-term prophylaxis against the ototoxic effects of cisplatin. METHOD: Two groups of rats were treated with unilateral injection into the round window of AAV harboring a gene encoding either XIAP or green fluorescent protein (GFP). After at least two months of gene expression, auditory-brainstem-response (ABR) threshold shifts and outer-hair-cell (OHC) number were measured in these two groups of animals after 72-hour treatment with cisplatin. RESULTS: Consistent with previous reports, uninjected and AAV.GFP-injected ears displayed profound ABR threshold elevations and OHC loss after cisplatin treatment. Ears that had been injected with AAV encoding XIAP, however, were significantly protected from these effects: cisplatin-induced ABR-threshold shift and hair-cell loss were attenuated by as much as 78% and 45%, respectively, when compared with contralateral (untreated) ears. CONCLUSION: XIAP delivery to the cochlea can protect against the audiometric changes and hair-cell loss associated with cisplatin ototoxicity. The efficacy, specificity, and duration of the protective effects make this a potentially attractive therapeutic paradigm.

Animals↗

Pressure gradients affecting the labyrinth during hypobaric pressure. Experimental study.

Hypobaric effects on the perilymph pressure were investigated in 18 cats. The perilymph, tympanic cavity, cerebrospinal fluid, and systemic and ambient pressure changes were continuously recorded relative to the atmospheric pressure. The pressure equilibration of the eustachian tube and the cochlear aqueduct was studied, as well as the effects of blocking these channels. During ascent, the physiologic opening of the eustachian tube reduced the pressure gradients across the tympanic membrane. The patent cochlear aqueduct equilibrated perilymph pressure to cerebrospinal fluid compartment levels with a considerable pressure gradient across the oval and round windows. With the aqueduct blocked, the pressure decrease within the labyrinth and tympanic cavities was limited, resulting in large pressure gradients toward the chamber and the cerebrospinal fluid compartments, respectively. We conclude that closed cavities with limited pressure release capacities are the cause of the pressure gradients. The strain exerted by these pressure gradients is potentially harmful to the ear.

Animals↗

Improved auditory performance of cochlear implant patients using the middle fossa approach.

The preliminary results of insertion of a cochlear implant via the middle fossa in nine patients with profound bilateral hearing loss are described. Aetiologies included a bilateral radical mastoidectomy cavity, adhesive otitis media, autoimmune inner ear disease, previous cranial trauma, genetic pre-lingual deafness, and otosclerosis. A classic middle fossa approach was adopted. A small cochleostomy measuring 1.5 mm in diameter was performed on the most superficial part of the basal turn. A Nucleus 24M cochlear implant system (Cochlear Corporation) was inserted in four patients, a Lauraflex implant (Philips Hearing Implants) was used in three patients and a Combi 40+ (Med-el) with a double electrode array in two. Single electrode arrays were inserted from the cochleostomy to the cochlear apex and occupied a portion of the basal turn, as well as the middle and apical turns. Double electrode arrays were inserted, one towards the apex and one into the basal turn of the cochlea towards the round window. The receiver stimulator was positioned in a bone well previously drilled in the temporal squama and the electrode carrier was inserted in the fenestrated cochlea. The activity of the inserted electrodes was tested by means of telemetry and intraoperative recording of electrically evoked auditory brainstem responses (EABR). Speech recognition tests, performed over a period of time ranging from one to six months after cochlear implant activation, yielded better results in these patients compared with those obtained in postlingually deaf patients operated on via the traditional transmastoid route. Cochlear implant insertion via the middle fossa approach is a technique which is suitable for the implantation of patients with bilateral radical mastoidectomy cavities, chronic middle ear disease, middle ear malformations, or with partial obliteration of the cochlea in the basal turn. However, the main advantage of inserting the implant through the middle fossa cochleostomy consists in the possibility of stimulating, with the single array, areas of the cochlea, i.e. part of the basal, middle and apical turns, where a greater survival rate of spiral ganglion cells is known to occur. In addition, with the double array total occupation of the cochlea is possible, providing the possibility of replicating the tonotopic organization of the cochlea. This new approach has led to major improvements in speech recognition in all patients compared with patients operated on via the transmastoid approach and, given the present state of the art, may be the elective approach for optimal implantation outcomes.

Adolescent↗

Cochlear prostheses: stimulation-induced damage.

The effects of 4 weekly, three-hour exposures to continuous sinusoidal (l kHz) electrical stimulation of the inner ear at various current levels were assessed in the chronically implanted guinea pig. With scala tympani stimulation, histopathological damage, including new bone growth, was observed for currents at and above 100 microA rms. No changes were observed in similarly implanted, but not stimulated cochleas. At equal current levels, less damage was found in subjects stimulated via electrodes placed on the round window and promontory, as compared to the scala tympani. Consistent reversible changes in threshold and suprathreshold features of the electrically evoked auditory brainstem response (EABR) were found. The magnitude of EABR change was directly related to exposure stimulus current level and to cochlear stimulation site. Suprathreshold features of the EABR were more sensitive to continuous stimulation exposures than threshold measures. Reversible EABR changes were found in the presence and absence of stimulation-induced histopathology.

Animals↗

Unexplained conductive hearing loss.

We report a series of unusual clinical cases which exhibit what we have referred to as unexplained conductive hearing loss. Audiometrically, these cases typically display mild to moderate conductive or mixed-type hearing loss and good word discrimination (80% to 100%). The otologic evaluations also lend support to the audiometric findings. When polytome roentgenograms were obtained thr results showed a normal inner ear system. Most often, the otologists' clinical impression was a fixed malleus or congenital stapes fixation, and exploratory tympanotomy with inspection of the ossicular chain was recommended. In each case, the ossicular chain was found to be intact. When the incudostapedial joint was separated the malleus and incus were seen to be mobile. Further, palpation of the stapes resulted in good round window reflexes. Possible explanations for this phenomenon are considered.

Adult↗

Acoustic modulation of electrically evoked otoacoustic emission in chickens.

Electrically evoked otoacoustic emissions (EEOAEs) can be elicited from the chicken inner ear. Since lesion studies implicate hair cells are the source of EEOAEs, we hypothesized that acoustic stimuli would modulate EEOAE amplitude at cochlear locations where the acoustic and electrical stimuli overlap. To assess this interaction, EEOAEs were measured as the frequency and amplitude of the acoustic stimuli were varied. EEOAEs, evoked by AC current (3-250 microA rms) delivered to the round window had a broad band pass response (1-6 kHz) with a peak between 3 and 4 kHz and maximum amplitude of 27 dB SPL. EEOAE suppression/enhancement tuning curves were measured at 2, 3, 4 and 6 kHz by varying the frequency of a 70 dB SPL tone and measuring the change in EEOAE amplitude. EEOAE tuning curves were characterized by a tip; a narrow range of frequencies where EEOAE amplitude was suppressed by as much as 5 dB, and by sidebands, a range of frequencies above and below the tip where EEOAE amplitude was enhanced by as much as 1.5 dB. The best suppression frequency, or characteristic frequency, was close to the frequency of the EEOAE elicited by the 3- or 4-kHz electric stimulus. However, the characteristic frequency was displaced towards higher frequencies for the 2-kHz electric stimulus, and towards lower frequencies for the 6-kHz electric stimulus. EEOAE suppression increased approximately linearly with acoustic level. These results suggest that EEOAEs evoked by round window stimulation are predominantly generated by hair cells near the 3- to 4-kHz region of the cochlea.

Acoustics↗

Acoustic to electric pitch comparisons in cochlear implant subjects with residual hearing.

The aim of this study was to assess the frequency-position function resulting from electric stimulation of electrodes in cochlear implant subjects with significant residual hearing in their nonimplanted ear. Six cochlear implant users compared the pitch of the auditory sensation produced by stimulation of an intracochlear electrode to the pitch of acoustic pure tones presented to their contralateral nonimplanted ear. Subjects were implanted with different Clarion electrode arrays, designed to lie close to the inner wall of the cochlea. High-resolution radiographs were used to determine the electrode positions in the cochlea. Four out of six subjects presented electrode insertions deeper than 450 degrees . We used a two-interval (one acoustic, one electric), two-alternative forced choice protocol (2I-2AFC), asking the subject to indicate which stimulus sounded the highest in pitch. Pure tones were used as acoustic stimuli. Electric stimuli consisted of trains of biphasic pulses presented at relatively high rates [higher than 700 pulses per second (pps)]. First, all electric stimuli were balanced in loudness across electrodes. Second, acoustic pure tones, chosen to approximate roughly the pitch sensation produced by each electrode, were balanced in loudness to electric stimuli. When electrode insertion lengths were used to describe electrode positions, the pitch sensations produced by electric stimulation were found to be more than two octaves lower than predicted by Greenwood's frequency-position function. When insertion angles were used to describe electrode positions, the pitch sensations were found about one octave lower than the frequency-position function of a normal ear. The difference found between both descriptions is because of the fact that these electrode arrays were designed to lie close to the modiolus. As a consequence, the site of excitation produced at the level of the organ of Corti corresponds to a longer length than the electrode insertion length, which is used in Greenwood's function. Although exact measurements of the round window position as well as the length of the cochlea could explain the remaining one octave difference found when insertion angles were used, physiological phenomena (e.g., stimulation of the spiral ganglion cells) could also create this difference. From these data, analysis filters could be determined in sound coding strategies to match the pitch percepts elicited by electrode stimulation. This step might be of main importance for music perception and for the fitting of bilateral cochlear implants.

Acoustic Stimulation↗

Rupture of the round window membrane.

A case of sudden deafness due to rupture of the round window membrane is presented. Nineteen similar cases have previously been reported in the literature. In a review of these twenty patients, it is noted that a history of concurrent physical effort or barotrauma was present in eighteen. This supports the view that the injury is produced by pressure changes acting either along the cochlear aqueduct (the explosive route) or, directly on the middle ear structures (the implosive route). At operation, the rupture may be difficult to see, and a separate leak from the oval window may be present. The timing of any surgical intervention is important. The authors recommend that this should be deferred for one week after the onset of symptoms, as the fistula may heal spontaneously. If no definite improvement has occurred at the end of this time, then tympanotomy should be undertaken during the next week.

Adult↗

[Methylprednisolone pharmacokinetics in perilymph and plasma of guinea pig after topical administration and intracardial injection].

OBJECTIVE: To determine pharmacokinetics profiles of methylprednisolone (MTH) in perilymph and plasma of guinea pig after topical and systemic administration. METHOD: A granule gelfoam soaked with MTH (40 mg/ml) was placed in the niche of round window of guinea pig; otocyst was infused with MTH (40 mg/ml); 4 mg/kg of MTH was injected intracardially. Samples of blood and perilymph of the scala tympani were obtained 1-6 h after administration of two routes, respectively. High-performance liquid chromatography was used to assay MTH in the samples. RESULT: After intracardial injection of MTH, concentrations of drug in plasma were obviously higher than those of in perilymph in every hour while drug in perilymph could not be obtained in 4-6 h, and the results were significantly different. After niche was placed with granule gelfoam with drug or otocyst was infused with MTH,drug could not be obtained in plasma, concentrations of drug in perilymph were obviously higher than those of in plasma after intracardial injection,and the highest concentrations in perilymph were manifested in 1 h 20 min. The concentrations of drug in perilymph after otocyst was infused with MTH were higher than those of after niche was placed with granule gelfoam soaked with MTH in every hour, and there were significant differences in between except those in 4 and 6 h. The half-life time of MTH in perilymph after otocyst was infused was 1.51 h, and the one after niche was placed with granule gelfoam soaked with MTH was 1.55 h. CONCLUSION: MTH could penetrate effectively through membrane of round window into the cochlear fluid and reach a much higher concentration following topical administration as compared with systemic application. The concentrations of drug in perilymph after otocyst was infused with MTH were higher.

Administration, Topical↗

[Clinical evaluation of congenital cholesteatoma of the middle ear].

We conducted a retrospective study to identify the clinical features and surgical observations of congenital cholesteatoma. Sixty patients were diagnosed and underwent surgery for congenital cholesteatoma between April 1987 and May 2002. All diagnoses were made on the basis of two operative findings: 1. the tympanic membrane manifested neither retraction, perforation, nor granulation. 2. the tympanic membrane was not continuous with the cholesteatoma. In this series, congenital cholesteatoma accounted for 7% of all cholesteatomas (853 ears). The patient age ranged from 2 to 48 years. The male to female ratio was 4:1. Seventeen patients had multiple cholesteatoma. Fifty-three patients exhibited closed-type cholesteatomas, while the remaining 7 patients had open-type cholesteatomas that had formed as a flat surface of the epidermis. Patients with open-type cholesteatomas presented with a much more pronounced conductive hearing loss and ossicular erosion or malformation. Twenty-two patients with relatively small cholesteatomas were analyzed to estimate the origin of their cholesteatomas. Of the 22 patients, 13 had anterior superior quadrant (ASQ-type) and 9 had posterior superior quadrant (PSQ-type) cholesteatomas. The mean age at the time of detection was older in the PSQ-type group than in the ASQ-type group and the frequency of ossicular erosion or malformation was more prominent in the PSQ-type group than in the ASQ-type group. The primary site of origin was thought to be the portion between the tympanic ostium of the auditory canal and the semicanal for tensor tympani in the ASQ-type group and near the incudostapedial joint in the PSQ-type group. A planned staged procedure was performed in 29 patients, 15 patients (52%) had residual lesions situated mostly on the oval window, the round window, an exposed facial nerve or an exposed lateral semicircular canal. The frequency of residual lesions in patients who presented with extended, multiple cholesteatoma and those with ossicular malformation was comparable to the frequency of patients who did not present with these features.

Adolescent↗

Sinus tympani: anatomic considerations, computed tomography, and a discussion of the retrofacial approach for removal of disease.

Surgical access to the sinus tympani remains a challenge for otologic surgeons. Usually, the retrotympanum is approached through the middle ear in an anterior to posterior direction during chronic ear surgery. Whether this is via a posterior tympanotomy or after canal wall down tympanomastoidectomy, visualization of the most posterior recess of the sinus tympani is often inadequate. The purpose of this investigation is two fold: (1) to describe the highly variable anatomy of the posterior tympanic cavity and (2) to review the retrofacial approach to the sinus tympani. Histologic sections, cadaver dissections, and diagrammatic illustrations are combined with computed tomographic (CT) imaging to provide a three-dimensional understanding of the sinus tympani and adjacent labyrinthine structures. Viewed from the mastoid, the anatomic structures that define the boundaries of the retrofacial approach include the facial nerve and stapedius muscle laterally, the lateral semicircular canal superiorly, the posterior semicircular canal posteromedially, the vestibule anteromedially, and the jugular bulb inferiorly. When the sinus tympani is well developed, saucerization within these boundaries gives wide access into the sinus and round window niche. The authors suggest that preoperative imaging can select patients who are candidates for a retrofacial approach to expose and remove disease in the sinus tympani. Contraindications to this approach include axial CT image measurements showing a contracted space between the posterior semicircular canal and the medial aspect of the facial nerve, lack of posterior expansion of the sinus tympani, and in cases where these measurements are marginal, the presence of a high jugular bulb or anteriorly positioned sigmoid sinus.

Ear Diseases↗

Age-related changes in cochlear vascular conductance in mice.

Vascular changes contribute to age-related hearing loss but the mechanisms involved in microvascular reactivity, particularly in the aged ear, are still incompletely understood. In this study, possible age-related changes in cochlear blood flow (CBF) and vascular reactivity were studied in presbycusic mice (C57BL/6) and young, age-matched, and old controls (CBA/J) without presbycusis. Reactivity was monitored by laser Doppler flowmetry and assessed by change in cochlear vascular conductance (VC) (defined as the ratio of CBF to blood pressure) in response to round window-applied sodium nitroprusside, a vasodilating agent. Mean VC response of C57BL/6 mice differed from controls both in maximum response and in post-drug recovery time. In C57BL/6 mice, mean VC increased about 28%, in contrast to an increase of over 40% in young and age-matched CBA/J controls. A less elevated VC response, similar to that of the presbycusic mice, was shown by aged (20-21 month) controls. Also, VC response in C57BL/6 mice was sustained throughout the 60 min observation period, while response of most CBA/J controls recovered in 50 min or less. These changes suggest age-dependent, pathologically-related altered responsiveness in cochlear vascular reactivity.

Aging↗

Needle placement with transtympanic electrocochleography.

Electrocochleography (ECoG) is an objective, electrophysiologic test useful in the clinical diagnosis of endolymphatic hydrops, or Meniere's disease. The purpose of this study was to determine if the position of the needle, using transtympanic methodology, gives a variable SP/AP (summating potential/action potential) response. SP/AP ratios were obtained during routine tympanoplasty procedures. After the tympanic membrane remnant was removed using a lateral graft technique, precise needle placement was obtained at the medial and lateral round window niches, as well as on the promontory. SP/AP ratios were obtained in these three needle positions. There was no significant difference in the SP/AP ratio responses despite the location of needle placement. The use of transtympanic electrocochleography can give very good wave form morphology and consistent results. Therefore, if elevated SP/AP ratios do occur, they are thought to be due to a pathologic process of the ear and not needle placement.

Audiometry, Evoked Response↗

Surgical technique for implantation of the totally ossified cochlea.

OBJECTIVES: Among the technical challenges of cochlear implant surgery is electrode insertion into the fully ossified cochlea. Earlier drill-out techniques have two significant drawbacks: 1. up to one half fail when the electrode pulls away from the cochlea; and 2. extended radical mastoidectomy, abdominal fat graft for obliteration, and closure of the external auditory meatus are required. A simplified technique is described that allows positive fixation of the cochlear implant electrode and in selected cases avoids a radical cavity with obliteration. STUDY DESIGN: Technical description with case reports and hearing outcomes. METHODS: Ten cadaver temporal bones were dissected to determine middle ear landmarks that overlie the basal turn of the cochlea from the transcanal approach and to establish the feasibility of the intact canal wall procedure. Surgery was performed on four patients who had preoperative imaging evidence of full ossification, two with the canal wall down, and two with canal wall up. Standard measures of speech recognition were used to evaluate hearing. RESULTS: Critical surgical landmarks are the round window, carotid artery, cochleariform process, and oval window. The procedure was successfully performed on four patients and open-set speech recognition is present in each. CONCLUSIONS: This canal wall up procedure allows long electrode insertion without radical cavity/obliteration in patients with fully ossified cochleas and prevents distraction of the electrode from the cochlea. While open-set word recognition was achieved by all subjects, results are poorer than expected for patients with limited or no ossification.

Audiometry, Speech↗

The effects of hyperbaric oxygen on experimental noise damage to the ears.

The effect of hyperbaric oxygen on cochlear microphonics, action potential of the auditory nerve, and brain stem response damaged by short exposure to noise is varied. Of 26 guinea pigs 14 showed a positive influence as measured by amplitudes. In 12 animals oxygen had little or no effect. The reason for this could lie in the individual reaction to the anesthetic of the section of the auditory nerve tested. The reaction of postmortem cochlear microphonics after hyperbaric oxygen treatment indicates oxygen diffusion through the round window.

Animals↗