Maroun T. Semaan

Geniculate ganglion hemangiomas are rare lesions of the facial nerve. Identification of these lesions takes a high index of suspicion, and diagnosis can be aided by several diagnostic modalities. The current chapter reviews the presentation, workup, diagnosis, management, and expected outcomes of patients with geniculate ganglion hemangiomas.

Objective: Characterize the sensitivity and specificity of TransImpedance Matrix (TIM) recordings compared with x-rays in detecting cochlear implant tip foldovers. Study Design: Retrospective. Setting: Multi-institutional, academic and private surgery centers. Patients: Patients 6 months and older undergoing cochlear implantation. Interventions: After cochlear implant array insertion, intraoperative TIM recordings and x-rays were conducted to confirm appropriate placement. If a foldover was identified, repeat insertion, followed by TIM and x-ray, was performed. Main Outcome Measures: Presence of tip foldovers as determined by TIM recordings and x-rays. Results: There were 13 tip foldovers out of 484 insertions, yielding a 2.7% tip foldover rate. Using x-rays as the “gold standard,” TIM recordings showed 100% sensitivity, 99.6% specificity, 84.6% positive predictive value (PPV), and 100% negative predicative value in detecting tip foldovers. The x-ray images for two TIM-identified tip foldovers were interpreted as normal intraoperatively (apparent false positives), but then were interpreted as foldovers when presented to the operating surgeon postoperatively in a blinded fashion. If these false positives had been reclassified as true positives, TIM specificity and PPV would both improve to 100%. Conclusions: These results provide further support regarding the utility of TIM recordings to evaluate appropriate cochlear implant electrode array position. Despite the low tip foldover rate reported across the literature and within this study, this multicenter patient group provided a large sample size to calculate the sensitivity and specificity of TIM recordings in identifying tip foldovers. Compared with the reference-standard, x-ray imaging, TIM recordings show equivalent, and in some cases superior, performance in identification of tip foldovers. Thus, use of TIM can potentially preclude the need for routine intraoperative x-rays without compromising patient care.

Objectives: To analyze audiometric outcomes after bilateral cochlear implantation in patients with isolated enlarged vestibular aqueduct (EVA) syndrome and associated incomplete partition (IP) malformations. Secondary objective was to analyze rate of cerebrospinal fluid (CSF) gusher in patients with IP-EVA spectrum deformities and compare this with the existing literature. Study Design: Retrospective chart review. Methods: Thirty-two patients with EVA syndrome who received unilateral or bilateral cochlear implants between June 1999 and January 2014 were identified in the University Hospitals Case Medical Center cochlear implant database. Isolated EVA (IEVA) and Incomplete Partition Type II (IP-II) malformations were identified by reviewing high-resolution computed tomography (HRCT) imaging. Demographic information, age at implantation, surgical details, postimplantation audiometric data including speech reception thresholds (SRT), word, and sentence scores were reviewed and analyzed. Intra- and postoperative complications were analyzed as well and compared with the literature. Results: Seventeen patients (32 implanted ears) had pediatric cochlear implantation for EVA-associated hearing loss. Data from 16 controls (32 implanted ears) were used to compare audiometric and speech outcomes of EVA cohort. Mean age at implantation was 6.8 years for EVA cohort and 6.0 years for controls. There was no statistically significant difference in long-term postoperative SRT, monaurally aided word scores, and binaurally tested word scores between pediatric EVA group and controls. The EVA patients had a long-term mean sentence score of 85.92%. A subset of EVA patients implanted at mean age of 3.18 years (n = 15 ears) had similar audiometric outcomes to another control group with Connexin 26 mutations (n = 20 ears) implanted at a similar age. Further subset analysis revealed no significant differences in age at implantation, SRT, and word scores in patients with IEVA and IP-II malformation. There was no significant association between size of vestibular aqueduct and age at implantation. There was no CSF gusher or other intra- or postoperative complications reported in our series. Conclusion: Bilateral sequential cochlear implantation can be performed safely in patients with EVA. Audiometric outcomes are excellent and comparable to pediatric cochlear implant patients with no malformations. CSF gusher rates can be minimized by trans-round window approach. Further long-term studies are needed to identify differences within IP-EVA spectrum deformities, audiometric outcomes, and proportions of EVA patients who will need cochlear implantation for hearing rehabilitation.

Objectives/Hypothesis: Approach‐specific economic data of acoustic neuroma (AN) resection is lacking. The purpose of this study was to analyze and compare adjusted total hospital costs, hospital and intensive care unit (ICU) length of stay (LOS), and associated factors in AN patients undergoing resection by translabyrinthine (TL) approach versus retrosigmoid (RS) approach.Study Design: Retrospective chart review.Methods: A total of 113 patients with AN undergoing TL (N = 43) or RS (N = 70) surgical resection between 1999 and 2012 were analyzed. Data including age, health status, preoperative hearing, tumor size, postoperative complications, hospital, ICU LOS, and disposition after discharge were collected from medical records and compared between both groups. Cost data was obtained from the hospital finance department and adjusted based on the Consumer Price Index for 2013.Results: There were no significant differences in demographic data, preoperative hearing, preoperative health status, or postoperative complication rate. Total hospital LOS and ICU LOS were significantly longer in the RS compared to the TL group (4.3 ± 3.6 vs. 2.6 ± 1.1 days; P < 0.001, and 1.5 ± 1.1 vs. 1.0 ± 0.5 days; P = 0.015, respectively). Tumors were larger in RS compared to the TL group (2.1 ± 1.0 cm vs. 1.5 ± 0.7 cm, respectively; P = 0.002). When patients were stratified by tumor size < or ≥ 2 cm, the total hospital LOS remained greater in the RS group in both subgroups (< and ≥ 2 cm, P < 0.001, and P = 0.031, respectively). However, there was no difference in the total ICU LOS between both subgroups. The adjusted mean total hospital cost was higher in the RS compared to the TL group ($25,069 ± 14,968 vs. $16,799 ± 5,724; P < 0.001). The adjusted mean total hospital cost was greater in the RS group with tumor < 2 cm (P < 0.001) but not significantly different in patients with tumors ≥ 2 cm. Univariate analysis showed that greater tumor size, poorer preoperative health status, the presence of major postoperative complications, and the RS approach were independently significantly associated with higher total hospital LOS (P = 0.001, P = 0.009, P = 0.001, and P < 0.001, respectively) and a higher adjusted total hospital cost (P < 0.001, P = 0.002, P = 0.014, and P < 0.001, respectively).Conclusion: Hospital LOS and total adjusted costs are significantly less for patients undergoing translabyrinthine acoustic neuroma resection compared to the retrosigmoid approach. Many factors appear to influence these differences. Economic considerations in addition to tumor characteristics and surgeon preference should be considered in future acoustic neuroma resections.Level of Evidence: 2c. Laryngoscope, 126:S5–S12, 2016

Animal models of endolymphatic hydrops (ELH) provide critical insight into the pathophysiology of Meniere’s disease (MD). A new genetic murine model, called the Phex mouse, circumvents prior need for a time and cost-intensive surgical procedure to create ELH. The Phex mouse model of ELH, which also has X-linked hypophosphatemic rickets, creates a postnatal, spontaneous, and progressive ELH whose phenotype has a predictable decline of vestibular and hearing function reminiscent of human MD. The Phex mouse enables real-time histopathologic analysis to assess diagnostic and therapeutic interventions as well as further our understanding of ELH’s adverse effects. Already the model has validated electrocochleography and cervical vestibular evoked myogenic potential as useful diagnostic tools. New data on caspase activity in apoptosis of the spiral ganglion neurons may help target future therapeutic interventions. This paper highlights the development of the Phex mouse model and highlights its role in characterizing ELH.

Endolymphatic hydrops (ELH) is a disorder of the inner ear that causes tinnitus, vertigo, and hearing loss. An elevated ratio of the summating potential (SP) to the action potential (AP) measured by electrocochleography has long been considered to be the electrophysiological correlate of ELH-related clinical conditions, such as Meniere’s disease, but in vivo confirmation and correlation between an elevated SP/AP ratio and ELH has not yet been possible. Confirming this relationship will be important to show that elevated SP/AP ratio is indeed diagnostic of ELH. Here, we sought to confirm that an elevated SP/AP ratio is associated with ELH and test the hypothesis that severity of ELH and hearing loss would also correlate with the SP/AP ratio in vivo using the Phex ^Hyp-Duk /Y mouse model of postnatal ELH. In addition, we describe a minimally invasive approach for electrocochleography in mice. Auditory brainstem responses and electrocochleography data were collected from controls and Phex ^Hyp-Duk /Y mutants at postnatal day 21 and the mice (all male) were euthanized immediately for cochlear histology. Our results show that (1) the SP/AP ratio was significantly elevated in mice with histological ELH compared to controls, (2) the SP/AP ratio was not correlated with the severity of histological ELH or hearing loss, and (3) the severity of hearing loss correlated with the severity of histological ELH. Our study demonstrates that an elevated SP/AP ratio is diagnostic of ELH and that the severity of hearing loss is a better predictor of the severity of ELH than is the SP/AP ratio.