Developmental Auditory and Speech-Language Performance in Pediatric Cochlear Implantation Recipients with Stable White Matter Lesions.

To analyze the association between stable asymptomatic white matter lesions (WMLs) and the cochlear implantation (CI) effect in congenitally deaf children, 43 CI children with stable asymptomatic WMLs determined via preoperative assessments and 86 peers with normal white matter were included. Outcome measurements included closed-set Mandarin Chinese (tone, disyllable, and sentence) recognition tests; categories of auditory performance (CAPs); and speech intelligibility rating (SIR) scales at 1, 12, and 24 months post-CI. Generalized estimating equation (GEE) models were used to analyze the association between WML and outcomes. In the WML group (control group), median CAP and SIR scores were 5 (5) and 4 (4) with mean rates of tone, disyllable, and sentence recognition of 84.8% (89.0%), 87.9% (89.7%), and 85.8% (88.0%) at 24 months post-CI, respectively. Auditory and speech performance improved significantly with implant use. Compared to their peers in the control group, for the participants with stable asymptomatic WMLs, auditory and speech abilities were not significantly different (p > 0.05). Stable asymptomatic WMLs might not be associated with poor auditory and speech intelligibility post-CI, which indicates that it is feasible to use comprehensive assessments to screen suitable candidates with WMLs who are likely to present with a good prognosis.

Diffuse-type Tenosynovial Giant Cell Tumor Invading the Temporal Bone: Three Cases.

Diffuse-type tenosynovial giant cell tumor (D-TSGCT) is a destructive benign tumor-like proliferative disease that occurs in synovial tissue characterized by villous nodular hyperplasia of joints, tendon sheaths, and synovium. D-TSGCT invading the temporal bone originating from the temporomandibular joint (TMJ) is very rare. Here, we report 3 cases of temporal bone D-TSGCT originating from the TMJ. The tumors in the three cases were originating from the TMJ and further invading the middle ear, the carotid foramen or the temporal lobe respectively. The second patient clearly involved the carotid foramen. The third patient clearly affected the temporal lobe. Lesions were completely removed in 3 cases, and all 3 patients were followed up for 30, 20, and 7 months, and none had recurrence. There are very few reports describing such cases. Although this report is not representative of most scenarios, there is still a potential that it provides a relatively reliable surgical idea for similar cases.

[Effect of cochlear implantation on tinnitus and the prognosis mode analysis].

Objective:To describe the effects of the possible related factors in unilateral cochlear implantation（CI） on tinnitus，and analysis the hearing and speech ability in different tinnitus prognosis mode. Method:The 70 post-lingual deafness CI patients（27.73±14.032 years old） in the clinical trial for LCI-20PI cochlear implant and LSP-20A sound processor project by a fast questionnaire about the tinnitus positive or negative respectively before the CI, 3rd, 6th, 9th and 12th months after the first mapping. 6 modes about tinnitus development were record: Type A（-to-）, no tinnitus before the CI and continued negative until the last follow-up; Type B（+ to-）, have tinnitus before CI and disappeared after the mapping; Type C（-to +）, no tinnitus before but appeared after the surgery; Type D（+ to-to +）, have tinnitus before the CI and disappeared during the continue follow-up, but finally the tinnitus show up again at last; Type E（-to + to-）, no tinnitus before and suffered from tinnitus after the CI surgery, but the noise disappeared at last; Type F（+ to +）, have the tinnitus in all the duration. Then we briefly analyzed the factors like age, gender, the duration of hearing loss, and the duration of hearing aids usage. Compare the characteristics in all the modes of tinnitus prognosis. Result:In this research CI treatment effect rate on tinnitus is 80%. The mean age of Type A （tinnitus from -to- ） group is （20.79 ±11.364） years old; Type B （tinnitus from + to-） group is （32.69±10.606） years old; Type C（tinnitus from-to +） group is （40.25±2.217） years old; Type D（tinnitus from + to-to +） group is （28.00±0） years old; Type E （tinnitus from-to + to-） group is （52.50±6.364） years old; Type F （tinnitus from + to +） group is （30.33±11.015） years old. And P<0.05 between the groups, while the severe-deaf-duration intergroup differences （P>0.05）. The mean speech discrimination rates are all elevate after 12 months and no statistical significance between the groups. But the E group has a lowst elevation in mean pure tone threshold （22.50±3.535 ） dB HL, when the F group has the best promotion （56.04±10.649 ） dB HL, and the difference between the groups is statistically significant. Conclusion:The cochlear implantation could eliminate tinnitus in 80% patients in this research. The better elevation of hearing and speech ability in the patients with persist tinnitus pre-and post-CI usage may related to amount and functions of the residual auditory nerves. Age may be an important factor in tinnitus generation, which may need more explanation and attention during the rehabilitation period.

Disrupted functional brain connectome in unilateral sudden sensorineural hearing loss.

Sudden sensorineural hearing loss (SSNHL) is generally defined as sensorineural hearing loss of 30 dB or greater over at least three contiguous audiometric frequencies and within a three-day period. This hearing loss is usually unilateral and can be associated with tinnitus and vertigo. The pathogenesis of unilateral sudden sensorineural hearing loss is still unknown, and the alterations in the functional connectivity are suspected to involve one possible pathogenesis. Despite scarce findings with respect to alterations in brain functional networks in unilateral sudden sensorineural hearing loss, the alterations of the whole brain functional connectome and whether these alterations were already in existence in the acute period remains unknown. The aim of this study was to investigate the alterations of brain functional connectome in two large samples of unilateral sudden sensorineural hearing loss patients and to investigate the correlation between unilateral sudden sensorineural hearing loss characteristics and changes in the functional network properties. Pure tone audiometry was performed to assess hearing ability. Abnormal changes in the peripheral auditory system were examined using conventional magnetic resonance imaging. The graph theoretical network analysis method was used to detect brain connectome alterations in unilateral sudden sensorineural hearing loss. Compared with the control groups, both groups of unilateral SSNHL patients exhibited a significantly increased clustering coefficient, global efficiency, and local efficiency but a significantly decreased characteristic path length. In addition, the primary increased nodal strength (e.g., nodal betweenness, hubs) was observed in several regions primarily, including the limbic and paralimbic systems, and in the auditory network brain areas. These findings suggest that the alteration of network organization already exists in unilateral sudden sensorineural hearing loss patients within the acute period and that the functional connectome of unilateral SSNHL patients is characterized by a shift toward small-worldization. Additionally, we hope that these findings will help to elucidate unilateral SSNHL through a new research perspective and provide insight for the potential pathophysiology of unilateral SSNHL.

Altered Contralateral Auditory Cortical Morphology in Unilateral Sudden Sensorineural Hearing Loss.

OBJECTIVE: To investigate the cerebral gray matter volume alterations in unilateral sudden sensorineural hearing loss patients within the acute period by the voxel-based morphometry method, and to determine if hearing impairment is associated with regional gray matter alterations in unilateral sudden sensorineural hearing loss patients. STUDY DESIGN: Prospective case study. SETTING: Tertiary class A teaching hospital. PATIENTS: Thirty-nine patients with left-side unilateral sudden sensorineural hearing loss and 47 patients with right-side unilateral sudden sensorineural hearing loss. INTERVENTION: Diagnostic. MAIN OUTCOME MEASURE: To compare the regional gray matter of unilateral sudden sensorineural hearing loss patients and healthy control participants. RESULTS: Compared with control groups, patients with left side unilateral sudden sensorineural hearing loss had significant gray matter reductions in the right middle temporal gyrus and right superior temporal gyrus, whereas patients with right side unilateral sudden sensorineural hearing loss showed gray matter decreases in the left superior temporal gyrus and left middle temporal gyrus. A significant negative correlation with the duration of the sudden sensorineural hearing loss (R = -0.427, p = 0.012 for left-side unilateral SSNHL and R = -0.412, p = 0.013 for right-side unilateral SSNHL) was also found in these brain areas. There was no region with increased gray matter found in both groups of unilateral sudden sensorineural hearing loss patients. CONCLUSIONS: This study confirms that detectable decreased contralateral auditory cortical morphological changes have occurred in unilateral SSNHL patients within the acute period by voxel-based morphometry methods. The gray matter volumes of these brain areas also perform a negative correlation with the duration of the disease, which suggests a gradual brain structural impairment after the progression of the disease.

Evaluation of the implanted cochlear implant electrode by CT scanning with three-dimensional reconstruction.

CONCLUSIONS: Computed tomography (CT) scan with three-dimensional (3D) reconstruction of the inner ear provides a more accurate image of the relationship of the electrode within the cochlear canal, with direct demonstration of electrode insertion depth in the cochlea in comparison with X-ray plain film. OBJECTIVE: This study was designed to evaluate the value of spiral CT scans with 3D reconstruction in determining the insertion site and depth of implanted cochlear implant electrodes. METHODS: A total of 172 cochlear implant recipients were involved in this study. The implanted electrodes of all patients were examined by X-ray plain film, and 157 cochlear recipients were examined by spiral CT scans with axial 1 mm image slices. The data from the CT scans were transferred to a workstation for 3D reconstruction (direct volume rendering) of the inner ear. The pseudocolor technique was used to display the electrode. RESULTS: The insertion depth of the electrode could be evaluated indirectly by the X-ray plain film. In contrast, the stereoscopic images from a CT scan with 3D reconstruction of the inner ear demonstrated the shape, position, and insertion depth of the electrode more accurately.

Muscarinic acetylcholine receptor subtype expression in type vestibular hair cells of guinea pigs.

Recent studies have demonstrated that five subtypes (M1-M5) of muscarinic acetylcholine receptor (mAChR) are expressed in the vestibular periphery. However, the exact cellular location of the mAChRs is not clear. In this study, we investigated whether there is the expression of M1-M5 muscarinic receptor mRNA in isolated type II vestibular hair cells of guinea pig by using single-cell RT-PCR. In vestibular end-organ, cDNA of the expected size was obtained by RT-PCR. Moreover, mRNA was identified by RT-PCR from individually isolated type II vestibular hair cells (single-cell RT-PCR). Sequence analysis confirmed that the products were M1-M5 mAChR. These results demonstrated that M1-M5 mAChR was expressed in the type II vestibular hair cells of the guinea pig, which lends further support for the role of M1-M5 mAChR as a mediator of efferent cholinergic signalling pathway in vestibular hair cells.

Muscarinic Acetylcholine Receptor Subtype Expression in Type Vestibular Hair Cells of Guinea Pigs / 华中科技大学学报（医学）（英德文版）

Recent studies have demonstrated that five subtypes (M1-M5) of muscarinic acetylcholine receptor (mAChR) are expressed in the vestibular periphery.However,the exact cellular location of the mAChRs is not clear.In this study,we investigated whether there is the expression of M1-M5 muscarinic receptor mRNA in isolated type Ⅱ vestibular hair cells of guinea pig by using single-cell RT-PCR.In vestibular end-organ,cDNA of the expected size was obtained by RT-PCR.Moreover,mRNA was identified by RT-PCR from individually isolated type Ⅱ vestibular hair cells (single-cell RT-PCR).Sequence analysis confirmed that the products were M1-M5 mAChR.These results demonstrated that M1-M5 mAChR was expressed in the type Ⅱ vestibular hair cells of the guinea pig,which lends further support for the role of M1-M5 mAChR as a mediator of efferent cholinergic signalling pathway in vestibular hair cells.

Integrated profile to assess auditory nerve-auditory pathway integrity.

PURPOSE: To evaluate the Integrated Cochlear Profile for Assessing Auditory Nerve-Auditory Pathway Integrity (ICP-API), as proposed by our group, in the selection of cochlear implant candidates. PROCEDURES: The API of the candidates for cochlear implantation were assessed with the ICP-API, which consists of 5 categories including: audiological testing; radiological imaging study; ear-canal electric response audiometry; response to environmental sounds; speech development level. The auditory rehabilitation effects of the cochlear implantation receivers were evaluated postoperatively. RESULTS: Sixty-six of 68 candidates who met the criteria of the ICP-API received cochlear implantation with improved hearing and speech development postoperatively. The remaining 2 of the 68 candidates were diagnosed with bilateral auditory nerves aplasia, and therefore failed cochlear implants were avoided. CONCLUSION: The ICP-API is valuable and feasible for the selection of cochlear implant candidates. The API should be considered as one of the most important criteria for cochlear implant candidate selection.

[Properties of cholinergic receptor-mediated ion channels on type I vestibular hair cells of guinea pigs].

To confirm the existence of cholinergic receptors on type I vestibular hair cells (VHCs I) of guinea pigs and to study the properties of the cholinergic receptor-mediated ion channels on VHCs I, electrophysiological responses of isolated VHCs I to external ACh were examined by means of whole-cell patch-clamp recordings. The results showed that 7.5% (21/279) VHCs I were found to be sensitive to ACh (10-1000 µmol/L). ACh generated an outward current in a steady, slow, dose-dependent [EC(50) was (63.78±2.31) µmol/L] and voltage-independent manner. In standard extracellular solution, ACh at the concentration of 100 µmol/L triggered a calcium-dependent current of (170±15) pA at holding potential of -50 mV, and the current amplitude could be depressed by extracellularly added calcium-dependent potassium channel antagonist TEA. The time interval for the next complete activation of ACh-sensitive current was no less than 1 min. The ion channels did not shut off even when they were exposed to ACh for an extended period of time (8 min). The results suggest that dose-dependent, calcium-dependent and voltage-independent cholinergic receptors were located on a few of the VHCs I investibular epithelium of guinea pigs. The cholinergic receptors did not show desensitization to ACh. This work reveals the existence of efferent neurotransmitter receptors on VHCs I and helps in understanding the function of vestibular efferent nervous system, and may provide some useful information on guiding the clinical rehabilitative treatment of vertigo.

[Diagnosis and therapy of bronchial foreign body in aged patients].

OBJECTIVE: To explore the proper diagnosis and therapy method in aged patients with bronchial foreign body. METHOD: A retrospective analysis was performed on 15 aged patients with bronchial foreign body by clinical characteristics. The experience of diagnosis and therapy in these cases were summarized. RESULT: Misdiagnosed or missed diagnosis were found in 6 of 15 aged patients with bronchial foreign body. Three bronchial foreign bodies were taken out successfully under topical anesthesia. Twelve bronchial foreign bodies were taken out under topical anesthesia and balanced anesthesia. No complications happened in all case. CONCLUSION: Clinical manifestation of bronchial foreign body is often unrepresentative in most aged patients. The chest X-ray of most cases can not show actual appearance of foreign bodies. In addition, history of foreign body in bronchus is often uncertain. So bronchial foreign bodies have often been misdiagnosed or missed diagnosed in aged patients. Suitable anesthesia method is the key to taking out bronchial foreign body in aged patients.

Expression of nicotinic acetylcholine receptor subunit alpha9 in type II vestibular hair cells of rats.

AIM: To explore the cell specific existence of alpha 9 AChR in the vestibular type II hair cells (VHC II) of rats. METHODS: To detect the expression of alpha 9 AChR messenger RNA (mRNA) in the vestibular endorgans and single VHC II of rats by using the reverse transcription polymerase chain reaction (RT-PCR) technique and the single cell RT-PCR technique, respectively. RESULTS: It was shown that alpha 9 AChR mRNA was detected in the vestibular endorgans. By using single-cell RT-PCR, mRNA encoding alpha 9 AChR was also detected in the VHC II of the rats. Sequence analysis of the PCR products confirmed identity to corresponding cDNA sequence in the predicted region. CONCLUSION: We established a method which could effectively detect the cell specific expression of mRNA in an individual VHC. Present data confirm that alpha 9 AChR mRNA is expressed in the VHC II of rats and indicates that alpha 9 AChR may function as a mediator of efferent cholinergic signaling in mammalian VHC.

The effect of the mtDNA4834 deletion on hearing.

Mutations in mitochondrial DNA (mtDNA) are associated with diverse pathological states in humans, notably sensorineural deafness. In humans, mtDNA4977 deletion, known as common deletion, is thought to play a critical role in presbyacusis. A similar mtDNA deletion occurs in the naturally aging rats is mtDNA4834 deletion. Today, it is still obscure about the effect of common mtDNA deletion on the presbyacusis and hearing loss. We establish a model of rat associated with mtDNA4834 deletion in inner ear by d-galactose. It was found that the malondialdehyde (MDA) increased with superoxide dismutase (SOD) decreasing in the inner ear of the rat treated with d-galactose than of the control. However, there was no significant difference in elevation of ABR threshold between the rat with mtDNA4834 deletion induced by d-galactose and control. After aminoglycoside antibiotic injected, the hearing threshold of the rats carrying mtDNA4834 deletion increased significantly compared with the rats without mtDNA4834 deletion. The results show that resembled accelerated aging in the inner ear of the rat could be induced by injecting d-galactose. Moreover, those suggest that mtDNA4834 deletion can not directly induce the hearing loss, but acting as a predisposing factor which can greatly enhance the sensitivity of the inner ear to the aminoglycoside antibiotic.

[The comparison of three ways for extracting nucleic acid from rats hair].

OBJECTIVE: To discuss the method of extracting nucleic acid from rat's hair. METHOD: The method with PCR buffer, SDS-proteinase K or Chelex-100 were used to extract the nucleic acid from rat's hair separately, and the isolated nucleic acid was analyzed by PCR and electrophoresis. RESULT: The success rate of antracting mt DNA from the hair gollcle, we gound the method with SDS-prot einase kis lower than the ather two methods (chi1(2) = 42.421, chi2(2) = 28.800, P<0.01). This is same as the result ot entracting nuclear DNA (chi1(2) = 49.091, chi2(2) = 30.767, P<0.01). While no alifference has been bound between the method with PCR buffer and with chelex-100 (mtDNA: chi(2) = 0.296; nuclear DNA: chi(2) = 0.048, P>0.05). The method with PCR buffer can't extract nucleic acid from hair shafts, the method with SDS-proteinase K can't extract mtDNA from one or two hairs, the method with Chelex-100 can extract mtDNA and nuclear DNA from single rat's hair or hair shafts. CONCLUSION: The method with Chelex-100 is suitable for extracting nucleic acid from hair.

[Expression of gamma-aminobutyric acid receptor gamma 1 subunit in the end-organs of rat vestibule].

OBJECTIVE: To investigate the expression of the gamma-aminobutyric acid receptor gamma 1 subunit in the end-organs of rat vestibule. METHOD: Using a combination of reverse transcription followed by polymerase chain reaction, expression of the gamma-aminobutyric acid receptor gamma 1 subunit in the end-organs of rat vestibule was examined. The rat brain RNA was used as positive control. RESULT: PCR amplification products representing subunit gene expression for gamma-aminobutyric acid receptor alpha 1 subunit were amplified. CONCLUSION: The results showed that gamma-aminobutyric acid receptor gamma 1 subunit was expressed in the rat vestibule, indicating that GABA is one of the important neurotransmitters in the vestibular system.

[Integrated methods for assessing auditory nerve-auditory pathway integrity].

OBJECTIVE: To evaluate the feasibility and value of integrated methods to assess auditory pathway integrity. METHOD: Twenty-four cases of bilateral profoundly-deafened individuals who were considered as the candidates of cochlear implantation were included in this study. Auditory pathway integrity from these candidates of cochlear implantation were assessed with the integrated methods established by our team, which consist of 5 categories including 1. audiological test; 2. radiological imaging study; 3. ear-canal electric audiometry; 4. response to sound in daily life; and 5. speech development. RESULT: Twenty-three candidates who meet the criteria of auditory nerve-auditory pathway integrity received cochlear implantation with improved hearing and speech development postoperatively. The remainder one of the 24 candidates was diagnosed as bilateral absence of auditory nerve. The fault of cochlear implantation was avoided. CONCLUSION: The integrated methods for assessing auditory pathway integrity is feasible and valuable. Auditory nerve-auditory pathway integrity should be considered and included as one of the most important criteria for cochlear implantation candidate.

[Mitochondrial DNA mutations and non-syndromic sensorineural hearing loss].

OBJECTIVE: To analyze the incidence of three types of mitochondrial DNA mutations in the non-syndromic sensorineural hearing loss (NSSNHL) patients and control subjects in order to investigate the possible role of mitochondrial DNA mutations in NSSNHL. METHODS: Sixty-one sporadic NSSNHL patients (from 3 to 84 years old) and 19 control subjects matched for age were selected. DNA was extracted from isolated blood leukocytes. Interrupt polymerase chain reaction (PCR) and primer-shift PCR were used to detect the mtDNA4977 deletion; mtDNA1555A-->G and mtDNA3243A-->G point mutation were detected by PCR and restriction fragment length polymorphism (RFLP) analysis. PCR products were sequenced by automated laser fluorescent DNA sequencer. RESULTS: The detection rate of mtDNA4977 deletion in deafness groups and control groups are 68.85%(42/61) vs. 5.26%(1/19). Among all the samples, neither any mtDNA1555A-->G mutation nor mtDNA3243A-->G point mutation was detected. CONCLUSIONS: MtDNA4977 deletion had a high detection rate in patients with NSSNHL. MtDNA1555A-->G mutation and mtDNA3243A-->G point mutation may not be common mutations in patients with NSSNHL.