Auditory Brainstem Implantation in Chinese Patients With Neurofibromatosis Type II: The Hong Kong Experience.

OBJECTIVE: To describe our experience and outcomes of auditory brainstem implantation (ABI) in Chinese patients with Neurofibromatosis Type II (NF2). STUDY DESIGN: Retrospective case review. SETTING: Tertiary referral center. PATIENTS: Patients with NF2 who received ABIs. RESULTS: Between 1997 and 2014, eight patients with NF2 received 9 ABIs after translabyrinthine removal of their vestibular schwannomas. One patient did not have auditory response using the ABI after activation. Environmental sounds could be differentiated by six (75%) patients after 6 months of ABI use (mean score 46% [range 28-60%]), and by five (63%) patients after 1 year (mean score 57% [range 36-76%]) and 2 years of ABI use (mean score 48% [range 24-76%]). Closed-set word identification was possible in four (50%) patients after 6 months (mean score 39% [range 12-72%]), 1 year (mean score 68% [range 48-92%]), and 2 years of ABI use (mean score 62% [range 28-100%]). No patient demonstrated open-set sentence recognition in quiet in the ABI-only condition. However, the use of ABI together with lip-reading conferred an improvement over lip-reading alone in open-set sentence recognition scores in two (25%) patients after 6 months of ABI use (mean improvement 46%), and five (63%) patients after 1 year (mean improvement 25%) and 2 years of ABI use (mean improvement 28%). At 2 years postoperatively, three (38%) patients remained ABI users. CONCLUSION: This is the only published study to date examining ABI outcomes in Cantonese-speaking Chinese NF2 patients and the data seems to show poorer outcomes compared with English-speaking and other nontonal language-speaking NF2 patients. Environmental sound awareness and lip-reading enhancement are the main benefits observed in our patients. More work is needed to improve auditory implant speech-processing strategies for tonal languages and these advancements may yield better speech perception outcomes in the future.

Contemporary solutions for patients with microtia and congenital aural atresia - Hong Kong experience.

Managing microtia patients is always a challenge. Multidisciplinary approach, good family support, well established doctor-patient relationship and well organised patient-support groups are the essential elements for success. With the advancement of implantable hearing devices, more options will be available for the microtia patients. Otologists play a leading role in the whole management process. They not only provide proper guidance to the patients in choosing the correct path of the treatment, but also play a key role in organising and maintaining a cost-effective multidisciplinary rehabilitation team for the microtia patients.

The BAHA hearing system for hearing-impaired postirradiated nasopharyngeal cancer patients: a new indication.

BACKGROUND: Radiation for patients who have nasopharyngeal cancer (NPC) often renders them hearing challenged and facing difficulties from treatment sequelae such as chronic suppurative otitis media and osteoradionecrosis. Conventional hearing aids aggravate otorrhea, and ear moulds traumatize osteoradionecrosis ulcers in the ear canal. The bone-anchored hearing aid (BAHA) hearing system might represent an excellent hearing solution. OBJECTIVE: To investigate the BAHA benefit and osseointegration results for hearing-impaired postirradiated NPC patients. STUDY DESIGN: A prospective longitudinal study. SETTING: Tertiary university center. PATIENTS: Eleven hearing-impaired postirradiated NPC patients were studied from October 2002 to October 2006. METHODS: Two-stage BAHA surgeries were performed. Assessments include pure-tone and speech audiometry, implant integrity, periabutment audit, and patient satisfaction analysis during a 24-month period. Radiation dosimetric analysis and bone sampling at the fixture implant sites were studied. RESULTS: No implant fixtures were lost (follow-up, 13-58 mo). Average patient satisfaction scores were 84.4%, with 80% using their BAHA everyday and 90% using their devices for more than 8 hours. Dosimetric analysis of the implant site revealed that all fixtures were outside the irradiated field. There was a reduction in otorrhea rates after BAHA use over the course of the study. CONCLUSION: Successful osseointegration was demonstrated in postirradiated NPC patients. Improved subjective hearing clarity, reduced ear discharge rates, and extended BAHA usage times accounted for high patient satisfaction with the BAHA hearing system. This is the first study to demonstrate long-term osseointegration and hearing benefit in postirradiated NPC patients. We recommend the BAHA hearing system for the treatment of chronic suppurative otitis media-related hearing problems in NPC patients.

Tone perception results with Harmony and HiRes 120 in Cantonese-speaking subjects.

The goal of this study was to examine tonal language perception in adults with cochlear implants who are native speakers of Cantonese. Ten adult subjects were implanted with HiRes 90K devices and participated in the study. Baseline data were obtained with standard HiRes and compared with HiRes 120. Subjects were evaluated using a tone identification test and a questionnaire. While objective outcome measures with the tone identification test did not show significant differences between standard HiRes and HiRes 120, subjective evaluation with the questionnaire showed significant differences in the overall satisfaction perceived by subjects. HiRes 120 received a significantly higher rating and the majority of subjects preferred HiRes 120.

Age and outcome of cochlear implantation for patients with bilateral congenital deafness in a Cantonese-speaking population.

OBJECTIVE: To evaluate the effect of age at implantation by assessment of speech perception in cochlear implant users with bilateral congenital deafness. DESIGN: A retrospective cohort analysis of 60 cochlear implant users (age at implantation, 1.01 to 22.0 yr) who have at least 2 yr of experience. Their outcome performance was defined by the change in i) speech perception category (SPC) score based on postoperative assessment results and ii) the type of education attended after implantation. The association of age at implantation with SPC scores was analyzed at different ages at implantation (2, 3, 4, 5, and 6 yr old). The SPC scores for a particular age at implantation were compared at 6, 12, and 24 mo after implantation. The impact of age at implantation on choice of education was evaluated by analyzing the transition from a school for the deaf to mainstream education for the 45 children who were operated on before the age of 10, because older children are less likely to make such a change. RESULTS: Children implanted at the ages of 2, 3, 4, 5, and 6 yr all obtained significant improvements in SPC scores 24 mo after implantation. The greatest improvement was noted at 24 mo after implantation among those operated on before age 3. For all age groups, improvement at 24 mo after implantation is greater than at 12 mo, whereas the latter is greater than the improvement noted at 6 mo after implantation. Comparison of children implanted before the age of 3 and between ages 3 and 10 showed a significant difference in the choice of education after implantation. Children who were implanted before the age of 3 were more likely to attend mainstream education after implantation. CONCLUSION: Results from the present study are consistent with the current belief that implantation at a younger age provides greater benefit. The proportion of children attending mainstream education was significantly higher for those implanted before age 3, which may be a potential benefit to early implantation for relieving the burden of governments in providing special education.