Faculty Bibliography

Objective Preservation of residual low-frequency hearing has become a priority in cochlear implantation. The purpose of this study was to compare rates of hearing preservation and effects on performance of loss of low-frequency acoustic hearing with two different length electrodes. Study design Retrospective chart review. Setting Tertiary Care Hospital. Patients Twelve patients were implanted with the CI422 a slim-straight electrode; the second group consisted of 10 patients implanted with the Hybrid-L, a shorter hearing preservation electrode. Main outcome measure Audiometric thresholds and speech perception measures. Results At 1 year, 3/10 (30%) patients with the Hybrid-L and 7/12 (58%) patients with the CI422 lost residual acoustic hearing resulting in a profound hearing loss in the implanted ear. In comparing these patients in particular, mean CNC words in the implanted ear were 72% in the CI422 electrode group and 15% in the Hybrid-L electrode group at 1 year (P = 0.03). While hearing preservation rates with the Hybrid-L tended to be better, among recipients who lost residual hearing, speech perception was better in those with the longer CI422 electrode. Conclusions With emphasis on preservation of residual hearing, patients need to be counseled regarding possible outcomes and options should loss of residual hearing occur following implantation. While shorter electrodes may have better rates of hearing preservation, the patients with the longer straight electrode in our study had significantly better speech understanding following the loss of residual hearing.

OBJECTIVES/HYPOTHESIS: Trauma to intracochlear structures during cochlear implant insertion is associated with poorer hearing outcomes. One way surgeons can influence insertion trauma is by choosing the surgical approach. We seek to compare cochleostomy (CO), peri-round window (PRW), and round window (RW) approaches using a fresh frozen temporal bone model. STUDY DESIGN: Experiments using fresh frozen temporal bones. METHODS: Cochlear implant insertions using the three aforementioned approaches were performed on 15 fresh frozen human temporal bones using a Cochlear 422 electrode. Insertions were evaluated by examining fluoroscopic recordings of histologic sections. RESULTS: Five cochlear implant insertions were performed using each of the three aforementioned approaches. Fluoroscopic examination revealed that none of the CO or PRW insertions contacted the modiolus during insertion, whereas three of five RW insertions did. RW insertions were less linear during insertion when compared to CO and PRW insertions (P < .05). CO insertions had significantly larger angular depth of insertion (487 degrees ) when compared to PRW (413 degrees ) and RW (375 degrees ) (P < .05). Histologic examination revealed one RW insertion resulted in osseous spiral lamina fracture, whereas the remaining insertions had no evidence of trauma. In the damaged specimen, the inserted electrode was observed to rest in the scala vestibuli, whereas the remaining electrodes rested in the scala tympani. CONCLUSIONS: Due to variability in RW anatomy, a CO or PRW window surgical approach appears to minimize the risk for insertion trauma. However, with favorable anatomy, a Cochlear 422 electrode can be inserted with any of the three approaches. LEVEL OF EVIDENCE: NA Laryngoscope, 125:966-971, 2015.

OBJECTIVE: The aim of this study was to develop a multidimensional metric for assessing quality of life (QoL) in patients with neurofibromatosis type 2 (NF2). STUDY DESIGN: Electronically distributed questionnaire. SETTING: University tertiary care hospital, NF2 support groups. SUBJECTS AND METHODS: Structured interviews with NF2 providers and patients identified relevant domains. Items in each domain were extracted from validated QoL modules, then combined with items unique to NF2 and pretested on NF2 providers and patients. The final 61-item questionnaire was administered electronically to patients with NF2 (N = 118). The form assessed overall QoL and 11 additional domains, including hearing, balance, facial function, vision, oral intake, future uncertainty, psychosocial, cognition, sexual activity, pain, and vocal communication. Responses were compared with reference values for the general population, patients with head and neck cancer, and patients with brain cancer. RESULTS: Overall, QoL in patients with NF2 was lower than that of the general population (P < .01) and similar to that of patients with cancer. Patients with more facial weakness, hearing loss, and imbalance reported significantly lower QoL. However, domains most predictive of overall QoL were psychosocial, future uncertainty, and pain. Compared with patients with head and neck and brain cancer, patients with NF2 demonstrated significantly higher levels of psychosocial stressors, including disease-related anxiety, personal and financial stress, and lack of social support (P < .01). CONCLUSION: Psychosocial stress and pain significantly affect QoL in NF2, indicating that mental health, pain management, and financial counseling could have an important impact on QoL in this population.

OBJECTIVE: To assess whether recombinant growth factor (hGH) therapy has an effect on cochlear implant (CI) performance. PATIENTS: Two pediatric CI recipients (S1, S2) who underwent treatment with hGH for short stature were identified for review. S1 has bilateral labyrinthine dysplasia and received implants at ages 10 months (right) and 4 years 3 months (left). S2 was diagnosed with severe to progressive sensorineural hearing loss bilaterally and received a CI at age 9 years 10 months (left). INTERVENTION(S): Case series. MAIN OUTCOME MEASURE(S): Cochlear implant, hGH, and speech perception data were collected. Phonetically Balanced Kindergarten (PBK) and Consonant Nucleus Consonant (CNC) word recognition scores were reviewed to assess auditory perception. Electrode impedances, threshold levels, and comfort levels were also reviewed. RESULTS: After 4 months of hGH, word recognition scores for S1 were observed to decrease from 90 to 72% (right) and were stable at 40% (left). Despite troubleshooting, performance continued to decline bilaterally to 52% (right) and 28% (left), and the decision was made to discontinue hGH. One month after cessation of hGH, word recognition scores began improving to 74% (right) and 68% (left). Word recognition scores for S2 were observed to have decreased from 92% the previous year to 82% after taking hGH for 2 months. Given both our previous experience with S1 and discussions with S2's parents, hGH was discontinued after 10 months of therapy. Two months after cessation of hGH, S2's word recognition had improved to 86% (left). CONCLUSIONS: Our case studies illustrate that implanted children undergoing treatment with hGH may experience a decrease in speech perception, which recovers after the cessation of treatment. Since hGH use has become more prevalent in recent years, it is important to inquire whether children undergoing, or who have undergone, implantation are receiving hGH so that they may be appropriately monitored.

OBJECTIVE: Is conscious sedation an effective, safe, and efficient anesthetic choice in patients undergoing select neurotologic and otologic procedures? STUDY DESIGN: Retrospective case review. SETTING: Tertiary referral center. PATIENTS: Six patients underwent eight neurotologic procedures including cerebrospinal fluid leak and encephalocele repair, and primary and revision cochlear implant surgery. Patients were deemed poor candidates for general anesthesia secondary to medical comorbidities. These were compared to 11 control patients who underwent same procedures under general anesthesia with intubation. INTERVENTION: Dexmedetomidine infusion was utilized as the primary agent for conscious sedation in this high-risk patient population because, unlike other commonly used sedatives, it preserves normal respirations while providing adequate analgesia. MAIN OUTCOME MEASURES: Preoperative Physiological and Operative Severity Score for the enUmeration of Mortality and Morbidity (P-POSSUM) scores were calculated for the study group. We measured total anesthesia, and perioperative and recovery times. Cost of anesthetic agents was considered and any adverse effects were noted. Patient satisfaction with the operative experience was assessed with telephone surveys. RESULTS: P-POSSUM scores were high for the study group. Postoperative anesthesia was shorter for the study patients undergoing conscious sedation. Difference in cost of anesthetic agents was negligible. The adverse effects were few and as expected for the type of procedure. Patients reported satisfaction and comfort with their operative experience. CONCLUSION: Select neurotologic and otologic procedures can be safely, effectively, and efficiently performed under conscious sedation with dexmedetomidine infusion as the primary anesthetic choice for patients who are deemed poor medical candidates for general endotracheal anesthesia.

OBJECTIVE: The preferential delayed enhancement of the perilymphatic space enables detection of the non-enhancing endolymphatic hydrops present in patients with Meniere's disease. The aim of this study was to evaluate the diagnostic utility of delayed postcontrast 3D FLAIR images and a color map of fused postcontrast FLAIR and constructive interference steady state (CISS) images in the identification of endolymphatic hydrops in patients with clinically diagnosed Meniere's disease. STUDY DESIGN: Case control, blinded study. SETTING: Tertiary referral center. PATIENTS: Ten patients with Meniere's disease and five volunteer controls. INTERVENTION: Diagnostic. MAIN OUTCOME MEASURE: Two neuroradiologists blinded to the clinical history independently evaluated for the presence of endolymphatic hydrops on the images of both inner ears for test and control subjects. Both the standard gray-scale FLAIR images and the fused color map images were independently reviewed. RESULTS: The gray-scale 3D FLAIR images demonstrated 68.2% sensitivity and 97.4% specificity, and the fused color map images demonstrated 85.0% sensitivity and 88.9% specificity in the identification of endolymphatic hydrops in Meniere's disease. There was significant correlation between the gray-scale 3D FLAIR images and fused color map images with the categorization of involvement (p = 0.002). Inter-evaluator reliability was excellent (kappa = 0.83 for gray-scale images, kappa = 0.81 for fused color map). CONCLUSION: Delayed 3D FLAIR and fused 3D FLAIR-CISS color map images of the inner ears after intravenous contrast administration are potentially useful diagnostic tools in the evaluation of patients with suspected Meniere's disease.

PURPOSE OF REVIEW: To summarize the recent medical literature related to the feasibility of hearing preservation after cochlear implantation and to identify variables that may influence hearing preservation in the implanted ear as well as the hearing perception outcomes. RECENT FINDINGS: The published data provide evidence that hearing preservation in the implanted ear yields significantly higher levels of speech recognition in complex listening environments than having just monaural acoustic hearing. There is ample evidence that hearing preservation is feasible and the reported rate is 50-89% in the low frequencies. This rate tends to be stable over time, except for a small percentage that diminishes around 3 months of follow-up. However, factors or variables which influence hearing preservation are yet unknown. Variables such as patient age, degree of residual hearing, insertion depth, insertion speed, duration of profound high frequency deafness and electrode design have been proposed. SUMMARY: Much attention has been focused on hearing preservation in the implanted ear. Thus, investigations into electrode array design, surgical technique and pharmacological management have been undertaken. Minimizing damage in the inner ear not only enhances the possibility for hearing preservation and better performance in complex listening scenarios but also conserves inner ear structures for future treatments.