Contrasting results of tests of peripheral vestibular function in patients with bilateral large vestibular aqueduct syndrome.

OBJECTIVE: To analyze and summarize the effect of bilateral large vestibular aqueducts in peripheral vestibular organ function. METHODS: Eighteen patients with bilateral large vestibular aqueduct syndrome (LVAS; Study Group) and 18 healthy volunteers (Control Group) were investigated using audiometry, caloric test, sensory organization test (SOT), and vestibular-evoked myogenic potential (VEMP) tests. RESULTS: All 18 patients (36 ears) exhibited sensorineural hearing loss. For cervical VEMP (cVEMP), the Study Group showed lower thresholds (Study Group vs. CONTROL GROUP: 71.4vs. 75.3dBnHL; p=0.006), N1 latencies (24.1vs. 25.2ms; p=0.026) and shorter P1 (15.3vs. 16.6ms; p=0.003), and higher amplitudes (400.7vs. 247.2µV; p<0.001) than the Control Group. For ocular VEMP (oVEMP), the Study Group had lower thresholds (79.3vs. 81.8dBnHL; p=0.046) and higher amplitudes (40.6vs. 14.4µV; p<0.001) than the Control Group. Fourteen of 16 patients (87.5%) who completed caloric tests had abnormal results, and 10 of 18 patients (55.6%) exhibited abnormal results in SOTs. CONCLUSIONS: The hyperfunction of vestibular test in otolithic organs and the hypofunction of vestibular test in semicircular canals, as well as the dysfunction in the balance test were demonstrated in patients with LVAS. SIGNIFICANCE: Our findings can help clinicians gain a better understanding of the characteristics of vestibular organ function in patients with LVAS, which can facilitate optimal targeted treatment.

Development and evaluation of the Nurotron 26-electrode cochlear implant system.

Although the cochlear implant has been widely acknowledged as the most successful neural prosthesis, only a fraction of hearing-impaired people who can potentially benefit from a cochlear implant have actually received one due to its limited awareness, accessibility, and affordability. To help overcome these limitations, a 26-electrode cochlear implant has been developed to receive China's Food and Drug Administration (CFDA) approval in 2011 and Conformité Européenne (CE) Marking in 2012. The present article describes design philosophy, system specification, and technical verification of the Nurotron device, which includes advanced digital signal processing and 4 current sources with multiple amplitude resolutions that not only are compatible with perceptual capability but also allow interleaved or simultaneous stimulation. The article also presents 3-year longitudinal evaluation data from 60 human subjects who have received the Nurotron device. The objective measures show that electrode impedance decreased within the first month of device use, but was stable until a slight increase at the end of two years. The subjective loudness measures show that electric stimulation threshold was stable while the maximal comfort level increased over the 3 years. Mandarin sentence recognition increased from the pre-surgical 0%-correct score to a plateau of about 80% correct with 6-month use of the device. Both indirect and direct comparisons indicate indistinguishable performance differences between the Nurotron system and other commercially available devices. The present 26-electrode cochlear implant has already helped to lower the price of cochlear implantation in China and will likely contribute to increased cochlear implant access and success in the rest of the world. This article is part of a Special Issue entitled .

Up-regulation of cochlear Hes1 expression in response to noise exposure.

Hes1, a hairy and enhancer of split homolog, negatively regulates inner ear hair cell differentiation. The main objective of this study was to investigate the status of the Hes1 gene in the noise-damaged cochlea in relation to the degree of noise-induced hearing loss (NIHL). Adult albino guinea pigs were exposed to white-band noise (115 dB sound pressure level). Noise exposure for either 1 or 3 hours induced significant elevations of threshold in auditory brainstem response (ABR) compared with unexposed controls. Succinate dehydrogenase staining showed that white-band noise exposure caused significant outer hair cell losses. In addition, we found significant up-regulations of cochlear Hes1 mRNA and protein expressions following acoustic trauma, and Hes1 mRNA expression was positively correlated with NIHL. These findings suggest that up-regulation of Hes1 expression in response to noise exposure may be one of the underlying mechanisms of NIHL.

Use of the loud sound stimulation test in diagnosis of semicircular canal dehiscence syndrome.

Semicircular canal dehiscence (SCD) syndrome is rare, and its diagnosis is a significant challenge in clinical practice. Our aim was to explore application of the loud sound stimulation test for diagnosing SCD syndrome. Eight cases of superior semicircular canal dehiscence (SSCD), among them two patients had bilateral dehiscences and one case of lateral semicircular canal dehiscence (LSCD). A total of 11 dehiscences were studied retrospectively. Loud sounds (pure tones, 100 dB, 110 dB nHL) at frequencies of 500, 1,000, and 2,000 Hz were used to stimulate both ears for 5 s. A temporal bone computed tomography (CT) scan with semicircular canal reconstruction was performed in all patients. Vertigo was present in seven of nine cases following loud sound stimulation. In addition, the patient with LSCD demonstrated horizontal eye movement following loud sound stimulation, whereas six patients with SSCD showed rotational eye movement. Among them, two patients with bilateral superior canal dehiscence showed a positive response to the loud sound stimulation in only one ear. The diagnoses of all patients were confirmed with a high-resolution temporal bone CT with corresponding multi-planar reconstruction of the affected semicircular canals with various size dehiscences. We conclude that the characteristic eye movement following loud sound stimulation is valuable for diagnosing SCD syndrome. In addition, the loud sound stimulation test has unique advantages, especially for confirming the affected ear and the corresponding semicircular canal.

Intratympanic dexamethasone as initial therapy for idiopathic sudden sensorineural hearing loss: Clinical evaluation and laboratory investigation.

OBJECTIVE: To evaluate the effect of intratympanic dexamethasone (ITD) as initial therapy for idiopathic sudden sensorineural hearing loss (ISSHL) as well as to determine the concentration-dependent time course distribution of dexamethasone in the inner ear. METHODS: Sixty-six patients with profound ISSHL were included. Twenty-two were treated with ITD and the rest as control. Audiograms were performed before the treatment and one month afterwards. In the animal study, dexamethasone of different concentrations (5, 10 and 20mg/ml) was injected into the tympanums of three groups of SD rats (Groups A, B and C), their inner ears dissected free at various postinjection survival intervals. Immunofluorescence was applied to detect the locations of dexamethasone. RESULTS: The overall rate of good prognosis was 77.27% in ITD group, which was not significantly different from 81.82% in the control group. In the animal study, the higher local concentration and longer lasting period was found in Groups B and C. CONCLUSIONS: ITD at 5mg/ml did not add effect to systemic steroids in improving hearing outcomes in patients with ISSHL. An increase in dexamethasone concentration led to large variations in pharmacokinetics in animal study, showing potential value in optimizing the drug delivery protocols and improving the therapeutic results.

Experimental study on thermic effects, morphology and function of guinea pig cochlea: a comparison between the erbium:yttrium-aluminum-garnet laser and carbon dioxide laser.

BACKGROUND AND OBJECTIVE: Surgery of the inner ear requires atraumatic techniques to preserve the morphology of the inner ear. Recent experiment and clinical studies have demonstrated that several laser systems are suitable for cochleostomy. The goal of this study was to quantify the thermic effects, morphology and function of guinea pig cochlea in vivo by comparing the erbium:yttrium-aluminum-garnet (Er:YAG) laser and carbon dioxide (CO(2)) laser and to determine the optimum laser parameters for safe clinical treatment. STUDY DESIGN/MATERIALS AND METHODS: A fenestration in the basal cochlear turn of guinea pigs was created. A type K thermocouple was placed on the membrane of round window to detect the local temperature change during laser irradiation. The auditory evoked brainstem response (ABR) was measured before and after laser application. Confocal laser microscopy and scanning electron microscopy (SEM) was used for cochlear morphology. RESULTS: An increased hearing loss immediately and 4 weeks later after irradiation was observed in animals with the higher power CO(2) laser in accordance with a higher temperature increase during laser application. In contrast, a wider safety scope of Er:YAG application in cochleostomy was presented with little temperature increase. These findings were correlated with the ultrastructural changes in guinea pig cochlea. CONCLUSION: The Er:YAG and CO(2) lasers are shown to be safe if the total amount of energy is kept within the limits applied in this study. In addition, on this preliminary basis by guinea pig laser cochleostomy, Er:YAG laser maybe less damaging to inner ear structures than CO(2) laser with a larger safety scope and less thermic effects.

Taurine attenuates aminoglycoside ototoxicity by inhibiting inducible nitric oxide synthase expression in the cochlea.

This study explored the potential protective role of taurine against aminoglycoside ototoxicity and the involvement of inducible nitric oxide synthase (iNOS) in the process. Guinea pigs, which received a single intravenous administration of gentamycin (100 mg/kg) followed by one large dose of furosemide (90 mg/kg, intravenously) were used as subjects. A rapid and profound hearing loss was seen in these animals 3-day posttreatment with iNOS expression upregulated in the cochlea. However, pretreatment with taurine prevented the hearing damage with decreased iNOS expression, similar to the effect of aminoguanidine, a selective inhibitor of iNOS. These findings suggest that taurine provides protection against the acute gentamycin/furosemide ototoxicity possibly by downregulating iNOS expression in the cochlea.

Reanimation of paralyzed laryngeal muscles by electrical stimulation synchronized with inspiration.

OBJECTIVE: To determine the effects of electrical stimulation on denervated cat posterior cricoarytenoid (PCA) muscle. STUDY DESIGN AND SETTING: This study was conducted on six cats with PCA muscle denervation. All animals were sacrificed 12 weeks after surgery and the glottal area in the live animals and the fiber diameters of PCA muscle were obtained. RESULTS: Signals synchronized with inspiration were recorded and transmitted to stimulate PCA muscle. The abduction of the paralyzed vocal cord during inspiration was observed; this allows enough flow of air through the larynx to maintain the respiration. The stimulated fiber diameters of PCA muscle were different from that of nonstimulated (P < 0.01). Although all denervated muscles were degenerated, electrical stimulation was used to prevent muscular atrophy. CONCLUSIONS: This study indicates that electrical stimulation of the PCA muscle synchronized with inspiration could restore the abduction of a paralyzed vocal cord and prevent the denervated muscles from atrophying. SIGNIFICANCE: Electrical stimulation synchronized with inspiration may lead to reanimation of paralyzed laryngeal muscles.

Effects of hyperbaric therapy on function and morphology of Guinea pig cochlea with endolymphatic hydrops.

OBJECTIVE: The objective of this study was to investigate the effect on experimental endolymphatic hydrops in guinea pigs after hyperbaric therapy. BACKGROUND: The histopathologic character of Ménière's disease is the presence of endolymphatic hydrops. Endolymphatic hypertension could be one of the factors resulting from endolymphatic hydrops. Some treatments of Ménière's disease are aimed toward preventing the endolymphatic hypertension. Exposure to pressure change has risen in recent years. METHODS: Thirty-two guinea pigs were operated on the right ears to induce endolymphatic hydrops by obliterating the endolymphatic sac through an extradural posterior cranial fossa approach. After 5 weeks' survival, 12 guinea pigs were put into a chamber with an absolute atmospheric pressure of 2.2 for 3 weeks (90 minutes once a day 5 times a week). We observed the morphologic and functional changes in guinea pig cochleae of the pressure group, 4-week hydrops group (n = 10), 8-week hydrops group (n = 10), and the normal group (n = 10). We measured the hearing threshold of the auditory brainstem response, the 70-dB SPL action potential (AP) latency, the ratio of 70-dB SPL summating potential magnitude to action potential magnitude (-SP/AP) of the electrocochleogram, and the maximum scala media area (SMA) ratio, respectively. RESULTS: The average 70-dB SPL-SP/AP magnitude of right ears (0.29 +/- 0.09) and the average maximum SMA ratio (2.23 +/- 0.20) in the pressure group were significantly less than that in the 8-week hydrops group (0.69 +/- 0.15 and 4.04 +/- 0.52, respectively) with the same survival time (p < 0.05). The results in the pressure group were almost as similar as that in the 4-week hydrops group (0.29 +/- 0.13 and 2.22 +/- 0.20, respectively) (p > 0.05). The average hearing threshold of ABR of right ears in the pressure group (36.67 +/- 14.30-dB SPL) was lower than that of the 8-week hydrops group (44 +/-1 4.30-dB SPL), but the difference was insignificant (p > 0.05). The average 70-dB SPL AP latency of right ears in the pressure group was not significantly different from those of the 8-week hydrops group, the 4-week hydrops group, or the normal group (p > 0.05). CONCLUSIONS: Our findings suggest hyperbaric therapy can significantly suppress the development of endolymphatic hydrops and improve cochlear function in guinea pigs. This study provided strong evidence for the development of pressure treatment of Ménière's disease without destroying the inner ear.