Faculty Bibliography

OBJECTIVES AND IMPORTANCE: Children with certain congenital malformations of the inner ear, including those with a common cavity defect, have a higher incidence of spontaneous cerebrospinal fluid (CSF) leak and resulting meningitis. However, they may also benefit from cochlear implantation. We suggest that surgical management may be possible that both prevents meningitis and provides hearing rehabilitation during the same procedure. CLINICAL PRESENTATION: A 2-year-old girl with bilateral common cavity defects who had previously undergone cochlear implantation developed contralateral CSF leak resulting in meningitis. INTERVENTION: After resolution of the infection, cochlear implantation was performed at the same time as definitive CSF leak repair. Simultaneous cochlear implantation and repair of the CSF leak successfully decreased the chance of recurrent meningitis in this case. She has been deriving hearing benefit from the bilateral implants. CONCLUSION: This case suggests a role for cochlear implantation to be combined with simultaneous CSF leak repair in children with a cochlear malformation. Furthermore, bilateral cochlear implantation at an early age may be warranted in these patients before CSF leaks and meningitis have occurred.

OBJECTIVES/HYPOTHESIS: This pilot study details the use of a software tool that uses continuous impedance measurement during electrode insertion, with the eventual potential to assess and optimize electrode position and reduce insertional trauma. STUDY DESIGN: Software development and experimental study with human cadaveric cochleae and two live surgeries. METHODS: A prototype program to measure intracochlear electrode impedance and display it graphically in real time has been developed. The software was evaluated in human cadaveric temporal bones while simultaneously making real-time fluoroscopic recordings and in two live surgeries during intracochlear electrode insertion. RESULTS: Impedance changes were observed with various scalar positions, and values were consistent with those obtained using clinically available software. Using Contour Advance electrodes, impedance values increased after stylet removal, particularly when using the monopolar mode. CONCLUSIONS: Impedance values seem systematically affected by electrode position, with higher values being associated with proximity to the cochlear wall. The new software is capable of acquiring impedance measurements during electrode insertion, and these data may be useful to guide surgeons to achieve optimal and atraumatic electrode insertion, to guide robotic electrode insertion, and to provide insights about electrode position in the cochlea.

OBJECTIVE: To analyze the effects of residual hearing on postoperative speech performance in traditional cochlear implant (CI) patients implanted with a conventional electrode. STUDY DESIGN: Retrospective review. SETTING: Academic tertiary referral center. PATIENTS: A total of 129 adults implanted by a single surgeon at a tertiary care facility between June 2005 and November 2010 with measurable preoperative pure tone thresholds at any frequency were included. INTERVENTION: Cochlear implantation with a conventional electrode via an anterior inferior cochleostomy. MAIN OUTCOME MEASURE: Speech perception using monosyllabic word scores in quiet and sentences in quiet and noise in the electric (CI-only) condition of the implanted ear. Preservation of hearing was defined as complete for postoperative thresholds within 10 dB of preimplant values and partial if greater than 11 dB. Pure tone audiometry and speech perception testing were performed preoperatively and at regular intervals postoperatively, with the 1-year evaluation being the final outcome period. RESULTS: Preservation at any frequency or level was not a factor in speech perception outcome, although preservation was more common in low frequencies. Hearing preservation was correlated with younger age at implantation, but was not related to length of hearing loss, cause of deafness, device type, sex, preoperative speech performance, or low-frequency pure-tone average. CONCLUSION: Hearing can be preserved in traditional CI patients implanted with a conventional electrode. Although preservation of hearing may have implications for future technology, it is not currently correlated with speech performance in the CI-only condition.

Objective To determine whether transcranial motor-evoked potential (TCMEP) monitoring of the facial nerve (FN) during cerebellopontine angle (CPA) tumor resection can predict both immediate and long-term postoperative FN function. Design Retrospective review. Setting Tertiary referral center. Main Outcome Measures DeltaTCMEP (final-initial) and immediate and long-term facial nerve function using House Brackmann (HB) rating scale. Results Intraoperative TCMEP data and immediate and follow-up FN outcome are reported for 52 patients undergoing CPA tumor resection. Patients with unsatisfactory facial outcome (HB >2) at follow-up had an average deltaTCMEP of 57 V, whereas those with HB I or II had a mean deltaTCMEP of 0.04 V (t = -2.6, p < 0.05.) Intraoperative deltaTCMEP did not differ significantly between groups with satisfactory (HB I, II) and unsatisfactory (HB > 2) facial function in the immediate postoperative period. Conclusion Intraoperative TCMEP of the facial nerve can be a valuable adjunct to conventional facial nerve electromyography during resection of tumors at the CPA. Intraoperative deltaTCMEP >57 V may be worrisome for long-term recovery of satisfactory facial nerve function.

This single-institution phase II study was performed to estimate the response rate to lapatinib in neurofibromatosis type 2 (NF2) patients with progressive vestibular schwannoma (VS). Twenty-one eligible patients were enrolled. Brain and spine MRIs, including 3-dimensional volumetric tumor analysis, and audiograms were performed once at baseline and again every 12 weeks. The primary response end point was evaluable in 17 patients and defined as >/=15% decrease in VS volume. Hearing was evaluable as a secondary end point in 13 patients, with responses defined as an improvement in the pure tone average of at least 10 dB or a statistically significant increase in word recognition scores. Four of 17 evaluable patients experienced an objective volumetric response (23.5%; 95% confidence interval [CI], 10%-47%), with median time to response of 4.5 months (range, 3-12). In responders, reduction in VS volumes ranged from -15.7% to -23.9%. Four of 13 patients evaluable for hearing met hearing criteria for response (30.8%; 95% CI, 13%-58%). One sustained response exceeded 9 months in duration. Median time to overall progression (ie, volumetric progression or hearing loss) was 14 months. The estimated overall progression-free survival and volumetric progression-free survival at 12 months were 64.2% (95% CI, 36.9%-82.1%) and 70.6% (95% CI, 43.1%-86.6%), respectively. Toxicity was generally minor, and no permanent dose modifications were required. Lapatinib carries minor toxicity and has objective activity in NF2 patients with progressive VS, including volumetric and hearing responses. Future studies could explore combination therapy with other molecular targeted agents such as bevacizumab.

OBJECTIVE: To describe and characterize diplopia resulting from skew deviation after cerebellopontine angle (CPA) surgery and labyrinthectomy. PATIENTS AND INTERVENTIONS: Retrospective case series of 4 patients who developed vertical diplopia from skew deviation after resection of tumors in the CPA or labyrinthectomy MAIN OUTCOME MEASURE: Complete neuro-opthalmologic examination including opticokinetic testing, confrontational visual field assessment, color plate, pupillary reflex, slit lamp examination, and head tilt test. RESULTS: Four patients with residual hearing preoperatively developed skew deviation immediately after surgical intervention, including translabyrinthine (n = 1) and retrosigmoid (n = 2) approaches to the CPA and labyrinthectomy (n = 1). Neuro-ophthalmologic examination demonstrated intact extraocular movements, and 2- to 14-mm prism diopter hypertropia on both primary gaze and head tilt testing. In all cases, skew deviation resolved spontaneously with normalization of the neuro-ophthalmologic examination within 10 weeks. CONCLUSION: Patients undergoing CPA surgery or labyrinthectomy can develop postoperative diplopia due to skew deviation as a consequence of acute vestibular deafferentation. Patients with significant hearing preoperatively, a probable marker for residual vestibular function, may be especially at risk for developing skew deviation postoperatively.