Objective:This study aimed to compare the effects of cochlear implantation（CI） on vestibular function in patients with large vestibular aqueduct syndrome（LVAS） and in patients with extremely severe deafness with normal inner ear structure. Methods:A total of 28 LVAS patients and 28 patients with normal inner ear structure who suffered from extremely severe deafness were selected. The parameters of caloric tests, bone conduction evoked cervical vestibular-evoked myogenic potentials（cVEMP）, bone conduction evoked ocular vestibular-evoked myogenic potentials（oVEMP） and video head impulse tests（v-HIT） were compared between the two groups before and after CI. The data were analyzed using SPSS 26.0 software. Results:There was no significant difference in the results of the preoperative caloric test, v-HIT, and oVEMP（P1, N1） between the LVAS group and the control group（P>0.05）. Compared to the control group, the LVAS group exhibited a shorter cVEMP P1[（13.41±0.71）ms vs （16.28±0.89）ms, P<0.000 1], shorter N1[（19.83±0.54）ms vs （28.18±1.56）ms, P<0.000 1], higher amplitude[（123.60±83.80）μV vs （73.92±79.85）μV, P=0.049 4] and higher oVEMP amplitude[（16.60±13.87）μV vs （9.96±10.47）μV, P=0.028 5] before CI. The abnormal rate of caloric test increased in both groups after CI（25.00% vs 57.14%, P=0.028 8, 32.14% vs 82.14%, P=0.000 3, respectively）. There was no significant difference in the v-HIT parameters in both groups before and after the operation. As for the LVAS group, there was no statistically significant difference in cVEMP and oVEMP induction rates before and after operation. In the control group, there was a decrease in cVEMP induction rate（96.42% vs 64.28%, P=0.005 2） and oVEMP induction rate（96.42% vs 57.14%, P=0.000 9） after CI. LVAS group showed a shorter cVEMP P1[（13.41±0.71）ms vs （10.30±0.60）ms, P<0.000 1]; shorter cVEMP N1[（19.86±0.53）ms vs （18.97±1.33）ms, P=0.004 7]; decreased amplitude[（124.50±84.86）μV vs （64.35±61.57）μV, P=0.001 0] and shorter oVEMP amplitude[（15.92±13.03）μV vs （9.16±9.20）μV, P=0.009 9] after CI. The oVEMP N1 in the control group was longer than that before operation[（11.73 ± 0.91）ms vs （13.35 ± 2.60）ms, P=0.019 6], whereas there was no significant difference in other VEMP parameters after CI. Conclusion:Before CI, there was no significant difference in the results of the caloric test and v-HIT between the LVAS group and the control group, but the LVAS group exhibited increased sensitivity to acoustic stimulation-induced myogenic potentials. After CI, the function of the semicircular canal was impaired in both groups in the low-frequency area, and remained largely unaffected in the high-frequency area. Additionally, the function of the otolith in the LVAS group was less affected than that in the control group after CI, which may be related to the fact that the enlarged vestibular aqueduct of the LVAS patients acted as the third window of the inner ear.
Humans ; Vestibular Aqueduct/physiopathology* ; Cochlear Implantation ; Male ; Female ; Vestibular Evoked Myogenic Potentials ; Deafness/physiopathology* ; Child ; Adolescent ; Adult ; Young Adult ; Hearing Loss, Sensorineural/physiopathology* ; Vestibular Function Tests

OBJECTIVE: To investigate the clinical and diagnostic characteristics of audiometric findings and vestibular-evoked myogenic potentials in patients with large vestibular aqueduct syndrome (LVAS). METHOD: Thirty LVAS subjects (60 ears) recruited received pure tone audiometry, acoustic immittance, auditory brain stem responses (ABRs), distortion-product otoacoustic emission (DPOAE), Vestibular evoked myogenic potentials (VEMP) and caloric test, and the diagnostic significance of the results was analyzed. RESULT: All 30 cases (60 ears) showed progressive and fluctuating hearing loss, while 16 cases experienced dizziness when hearing fluctuated. Most of our cases showed sensorineural hearing loss, and 47 ears (94.0%) showed air-bone gap in the low frequencies, with mean gaps of (43 +/- 17) dB HL at 250 Hz, (33 +/- 18) dB HL at 500 Hz, in which the middle ear function showed normal. The acoustically evoked short latency negative response (ASNR) with medium latency (3.06 +/- 0.52) ms was elicited from 18 ears (64.3%). The mean amplitude of vestibular evoked myogenic potentials (VEMP) of 42 ears was (147.10 +/- 107.55) microv, and the threshold of VEMP of 19 ears was 75 dB nHL, of 7 ears was 65 dB nHL. CONCLUSION Characteristics of hearing performance, such as progressive and fluctuating hearing loss, air-bone gap at the low frequencies with normal middle ears, the ASNR, and increased amplitude and decreased threshold of the VEMPs, will help clinicians make initial diagnosis of LVAS, and provide a reference for further imaging examination.
Adolescent ; Adult ; Audiometry, Pure-Tone ; Child ; Child, Preschool ; Female ; Humans ; Male ; Syndrome ; Vestibular Aqueduct ; physiopathology ; Vestibular Evoked Myogenic Potentials ; Young Adult

OBJECTIVE: To analyse the clinical audiological significance in the diagnosis of large vestibular aqueduct syndrome (LVAS) by the auditory brain stem response (ABR) testing. METHOD: Patients with sensorineural hearing loss were examined by temporal bone CT scanning from January, 2008 to September, 2009. The result of CT scanning of 70 cases inner ear malformation were analysed. Patients were divided into two groups, LVAS group including 38 cases (76 ears) and other inner ear malformation group including 32 cases (62 ears). All patient accepted clinical audiology analysis and auditory brainstem response (ABR) test. RESULT: Twenty-four cases (41 ears) of LVAS group were detected with ASNR in 2 3 cm by the ABR testing, the positive rate was 54%, while ASNR was not detected in patients of other inner ear malformations group. There was significant differences (P=0.01) of the ASNR between two groups. CONCLUSION There is high incidence of LVAS on the patients with non-syndromic deafness. ASNR by ABR testing could help diagnosing the LVAS.
Child ; Child, Preschool ; Ear, Inner ; abnormalities ; Evoked Potentials, Auditory, Brain Stem ; Female ; Hearing Loss, Sensorineural ; diagnosis ; physiopathology ; Humans ; Infant ; Infant, Newborn ; Male ; Syndrome ; Vestibular Aqueduct ; abnormalities

OBJECTIVETo evaluate the hearing and speech recognition in patients with large vestibular aqueduct syndrome undergoing multi-channel cochlear implantation.

METHODSFrom 2005 to 2008, multi-channel cochlear implantation surgeries were performed in 22 patients with large vestibular aqueduct syndrome. All the patients received multi-channel cochlear implantation through transmastoid facial recess approach. The postoperative outcomes of the patients were compared with those of 22 patients with non-malformed cochlear.

RESULTSSevere gusher occurred in 3 cases during the implantation, and perilymph fluctuation was found in another 15 cases, with a rate of anomalies of 81.8%. All the electrodes were totally inserted into the cochlear, and no facial paralysis or cerebrospinal fluid leakage occurred after the operation. The hearing threshold in these patients was similar to that in patients with normal cochlear receiving the implantation. After speech rehabilitation for over 6 months, all the patients showed improved hearing and verbal ability.

CONCLUSIONMulti-channel cochlear implantation can be performed in patients with large vestibular aqueduct syndrome, but preoperative hearing assessment and radiographic examinations should be performed.

Adolescent ; Adult ; Child ; Child, Preschool ; Cochlear Implantation ; Electrodes ; Female ; Hearing ; Hearing Loss ; diagnostic imaging ; physiopathology ; surgery ; Humans ; Infant ; Magnetic Resonance Imaging ; Male ; Speech ; Tomography, X-Ray Computed ; Vestibular Aqueduct ; diagnostic imaging ; pathology ; physiopathology ; Young Adult

OBJECTIVE: To explore the disease incidence, clinical symptoms, prevention and treatment measures of the large vestibular aqueduct syndrome (LVAS). METHOD: Retrospective analyse the medical history, hearing, vestibular function examination and treatment of 76 LVAS patients who were diagnosed in our department of Otolaryngology from 2002 to 2008. RESULT: Most patients (93.4%) showed sensorineural hearing loss. Part of patients (61.8%) showed air-bone conduction gap in low frequency. The hearing loss of 43 ears is > 40-60 dB HL, > 60 -80 dB HL 47 ears, > 80 dB HL 62 ears. Decline curve is the characteristic of the Audiogram. The decline in high-frequency 112 ears, flat curve in 29 ears, island hearing in 11 ears. Forty-six patients were conducted the vestibular function examination, which showed low vestibular function. Tympanogram showed that 141 ears are type A, 11 ears are type C. High-resolution CT scan revealed that vestibular aqueduct minimum diameter is 2.2 mm and the largest is 6.2 mm, with a wide opening and deep narrower, and showed the "triangle" or "flared". Forty-two cases of this group were simple dilatation of the vestibular aqueduct, and no large vestibular semicircular canal malformation or cochlear malformation. There was no intellectual and other development disorders. In accordance with the degree of hearing loss, 20 cases of patients restored hearing after drug treatment. Eleven were cases fit a suitable hearing aid and carried out the language rehabilitation training. Forty-five very severe patients were implanted the cochlear and mapping one month later. CONCLUSION Fluctuative and progressive hearing loss is the main clinical symptoms of large vestibular aqueduct syndrome. The patients should be examined by high resolution CT scan of the temporal bone. There is no precise and effective treatment for the disease. It is very important for the deaf children who have residual hearing to fit hearing aids and carry out the language rehabilitation training as soon as possible. As for the patients who suffer from hearing loss severely and the hearing aid cannot achieve effective compensation, the cochlear implant should be considered.
Adolescent ; Child ; Child, Preschool ; Female ; Hearing Loss ; diagnosis ; therapy ; Humans ; Male ; Retrospective Studies ; Syndrome ; Temporal Bone ; diagnostic imaging ; Tomography, X-Ray Computed ; Vestibular Aqueduct ; physiopathology ; Vestibular Diseases ; diagnosis ; diagnostic imaging ; therapy ; Young Adult

OBJECTIVE: The purposes of this study was to analyse the significant of low frequency air-bone gap in enlarged vestibular aqueduct (EVA) patients according to the pure tone audiogram and the benefit for clinic diagnosis of the EVA. METHOD: The audiology testing include (1) play audiometry or pure tone audiometry, tympanometry, middle ear muscle reflex thresholds and stapedius muscle reflex; (2) High-resolution computed tomography (CT) scan of the temporal bone and magnetic resonance imaging was used for diagnoses the EVA. RESULT: All the 78 patients (154 ears) were diagnosed as the typical EVA by CT or MRI. Inner ear malformations were found in 3 ears otherwise the structures of middle ear in all the patients were absolutely normal. The audiology analysis showed 154 ears were type A tympanogram including 126 ears with typical A, 25 ears with As, 3 ears with Ad type. In the 250 Hz pure-tone test:A-B gap were observed in 126 ears(126/154,81. 8%) with the different hearing loss degree: 1 ear mild, 11 ears moderate, 19 ears moderate severe, 40 ears severe and 53 ears profound. In the 500 Hz pure-tone test: A-B gap were found in 102 ears(102/154, 66. 2%) with the different hearing loss degree: 9. ears moderate, 17 ears moderate severe, 35 ears severe and 41 ears profound. CONCLUSION Our study suggested a 66. 2% -81. 8% possibilities to find the EVA through the pure-tone audiometry firstly in the basis of the normal tympanograms with the significant A-B gap.
Acoustic Impedance Tests ; Adolescent ; Audiometry, Pure-Tone ; Child ; Child, Preschool ; Female ; Humans ; Infant ; Male ; Vestibular Aqueduct ; Vestibular Diseases ; physiopathology