[Cochlear implant in patients with congenital malformation of the inner ear].

OBJECTIVE: To summarize the clinical experience with multi-channel cochlear implantation in patients with inner ear malformations and evaluate and the outcomes of speech rehabilitation. METHODS: A retrospective study was conducted in 295 patients receiving cochlear implantation from 1998 to 2007, including 25 patients with large vestibular aqueduct syndrome (LVAS), 9 with Modini malformation, and 5 with common cavity deformity. All the patients received the Nucleus24 cochlear implants. In LVAS cases, 4 had Nucleus 24R (ST) implants, 8 had Contuor implants, 10 had Contuor Advance, and the remaining cases used Nucleus24(M) straight-electrode implants. RESULTS: Severe gusher appeared in 3 cases of LVAS, and perilymph fluctuation were seen in other 15 cases. Four patients with Mondini malformation and 2 with common cavity malformation also experienced severe gusher, but the electrodes were inserted smoothly in all the patients without postoperative facial paralysis or cerebrospinal fluid leakage. The hearing threshold in these patients was similar to that in patients with normal cochlear structure. After speech rehabilitation for over 6 months, the abilities of speech discrimination and spoken language improved in all the cases in comparison with the preoperative lingual functions. CONCLUSION: Multi-channel cochlear implantation can be performed in patients with inner ear malformation, but should not be attempted in patients with poor cochlear and cochlear nerve development. A comprehensive pre-operative radiographic and audiological evaluation is essential.

[Cochlear implantation in patients with large vestibular aqueduct syndrome].

OBJECTIVE: To evaluate the hearing and speech recognition in patients with large vestibular aqueduct syndrome undergoing multi-channel cochlear implantation. METHODS: From 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. RESULTS: Severe 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. CONCLUSION: Multi-channel cochlear implantation can be performed in patients with large vestibular aqueduct syndrome, but preoperative hearing assessment and radiographic examinations should be performed.

[Effect of surgical reconstruction of congenital aural atresia via the mastoid antrum approach: analysis of 48 cases].

OBJECTIVE: To assess the effect of surgical reconstruction of congenital aural atresia via the mastoid antrum approach and investigate method for preventing postoperative atresia of the reconstructed aural canal. METHODS: From 2000 to 2008, aural canal reconstruction and tympanoplasty was performed via the mastoid antrum approach. In 48 patients with congenital aural atresia (54 ears, including 45 ears of type II, 9 ears of type III). All the patients were followed-up for 18 months to assess the therapeutic effect. RESULTS: The mastoid antrum was located uneventfully for all the 54 ears, all showing ossicular chain anomalies involving most frequently the malleus and the incus followed by the upper structures of the stapes. Facial nerve abnormalities were seen in 23 ears (42.6%). Hearing improvement to over 20 dB was achieved in 45 ears (83.3%) and to over 25 dB in 25 ears (46.2%) one year later. CONCLUSION: The mastoid antrum approach for surgical reconstruction of congenital aural atresia is safe and reliable. Maintenance of the width of the aural canal and prevention of lateral healing of the transplanted tympanic membrane are crucial in the treatment of congenital aural atresia.

[Audiological assessment in 79 patients with tympanosclerosis].

OBJECTIVE: To conduct audiological assessment in patients with tympanosclerosis. METHODS: A retrospective review was conducted in 79 patients with tympanosclerosis (involving 79 ears) with complete records, including 30 patients (30 ears) with fixed Malleus-incus complex, 29 (29 ears) with fixed stapes, and 20 (20 ears) with fixations of both the stapes footplate and the Malleus-incus complex. Audiometry was performed for all the patients one or two days before operation, and the audiological features of the patients were compared between the 3 groups. RESULTS: Most of the patients (65.8%) suffered conductive hearing loss, 32.9% had mixed deafness, and one patient had sensorineural hearing loss. No statistically significant differences was noted in the speech frequency (0.5, 1, and 2 kHz) air conduction pure tone average (PTA) or the air-bone gap (ABG) in the 3 groups (P<0.05). CONCLUSION: Most of the patients with tympanosclerosis suffer conductive hearing loss, and the severity of hearing loss is not associated with the site of tympanosclerosis.