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.

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In Memoriam: John K. Niparko, MD, 1954 to 2016

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Mary Gantz, 1949–2016, A Friend to So Many…

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An Unusual Complication of Stapes Surgery

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Book Review

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Cochlear Patency After Transmastoid Labyrinthectomy for Ménière's Syndrome

Objective: Labyrinthectomy is considered the “gold standard” in the treatment of intractable vertigo attacks because of Ménière's Disease (MD) but sacrifices all residual hearing. Interest in auditory rehabilitation has lead to cochlear implantation in some patients. Concern remains that the cochlear lumen may fill with tissue or bone after surgery. This study sought to determine the incidence of obliteration of the cochlea after transmastoid labyrinthectomy. Study Design: Retrospective observational study. Setting: Tertiary referral center. Patients: Eighteen patients with intractable vertigo from MD who underwent surgery. Interventions: Transmastoid labyrinthectomy between 2008 and 2013. Cochleas were imaged with unenhanced, heavily T2-weighted magnetic resonance imaging (MRI). Main Outcome Measure: Presence of symmetrical cochlear fluid signals on MRI. Results: There was no loss of fluid signal in the cochleas of operated ear compared with the contralateral, unoperated ear in any subject an average of 3 years (standard deviation [SD]: 1.2) after surgery. Five of 18 patients had the vestibule blocked with bone wax at the time of surgery. Blocking the vestibule with bone wax did not change the cochlear fluid signal. Conclusion: The risk of cochlear obstruction after labyrinthectomy for MD is very low. The significance of this finding is that patients with MD who undergo labyrinthectomy will likely remain candidates for cochlear implantation in the labyrinthectomized ear long after surgery if this becomes needed. Immediate cochlear implantation or placement of a cochlear lumen keeper during labyrinthectomy for MD is probably not necessary.

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Botulinum Toxin Treatment of Objective Tinnitus Because of Essential Palatal Tremor: A Systematic Review

Introduction: In contrast to subjective tinnitus, objective tinnitus can be heard by the examiner as well as by the patient. It can be triggered by, among many other etiologies, idiopathic muscular tremor in the soft palate, the essential palatal tremor (EPT). Many treatment modalities have been investigated, of which only Botulinum toxin (BT) injections have shown promising results. Goal: The aim of this study was to evaluate the effect of BT treatment on objective tinnitus due to EPT by a systematic review of the literature. Methods: In accordance with PRISMA guideline a systematic literature search in three databases was performed. Results: Twenty-two studies fulfilled the inclusion criteria, mainly case reports and case series. A total of 51 BT treated patients diagnosed with EPT were identified in the literature. The studies were evaluated with focus on diagnostics, injection technique and BT dose, follow-up, effect on objective tinnitus, complications, and adverse effects. Conclusions: The included studies suffer from an extremely low evidence level with several sources of bias. When optimally injected, BT seems to be an effective treatment of objective tinnitus due to EPT, with few adverse effects and complications. We suggest BT injections as first choice in case of EPT and present a guideline regarding diagnostics, treatment, and follow-up.

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