Faculty Bibliography

OBJECTIVES/HYPOTHESIS: To evaluate the efficacy of image-guided surgical navigation (IGSN) in otologic surgery and establish practice guidelines. STUDY DESIGN: Prospective study. METHODS: Between January 2003 and January 2010, all patients requiring complicated surgery for chronic otitis media, glomus jugulare, atresia, cerebrospinal fluid leak with or without encephalocele, and cholesterol granuloma of the petrous apex were offered IGSN. The accuracy of IGSN relative to pertinent pathology and 11 anatomic landmarks was established. Additionally IGSN-related operative time, complications, and surgical outcome were recorded. RESULTS: In the study period there were 820 otologic procedures, among 94 patients (96 ears) with disease meeting proposed criteria. Thirteen patients (15 procedures) consented to the use of IGSN. All patients had a minimum 6 months of follow-up. The average additional operative time required was 36.7 minutes. The mean accuracy error was 1.1 mm laterally at the tragus but decreased to 0.8 mm medially at the level of the oval window. The mean accuracy of IGSN was within 1 mm in 10 of the 11 targeted surgical anatomic landmarks. CONCLUSIONS: Interactive image-guided surgical navigation during complex otologic surgery may improve surgical outcome and decrease morbidity by providing an accurate real-time display of surgical instrumentation relative to patient anatomy and pathology. In select cases, the extra cost of imaging immediately prior to surgery and extra operating room time may be compensated by enhancing the ability to distinguish distorted anatomy relative to disease, potentially improving surgical outcome. IGSN, although useful, does not replace surgical expertise and experience.

Congenital cytomegalovirus infection is the most frequent nongenetic cause of pediatric hearing loss in the United States, affecting approximately 8,000 children each year. Due in part to variable cytomegalic involvement of the auditory cortex, cochlear implantation outcomes have varied widely. Functional magnetic resonance imaging (fMRI) has the potential to assist in determining candidacy for cochlear implantation through the detection of intact auditory pathways including the cortex. We report a case of a 21-month-old girl with cytomegalovirus-related deafness and diffuse white matter involvement in which fMRI was a determining factor for cochlear implantation and side selection.

BACKGROUND: The head and neck is the most common site of mucosal melanoma, a cancer with poor prognosis. In contrast to cutaneous melanoma, mucosal melanoma of the head and neck (MMHN) is uncommon, with limited data regarding outcomes and prognostic factors drawn from small, single-institution case series. In order to identify factors predictive of survival, we analyzed MMHN outcomes in a large US cohort. METHODS: MMHN cases (n = 815) diagnosed in the USA between 1973 and 2007 were analyzed in the Surveillance, Epidemiology, and End Results registry, and cause of death was individually determined in 778 (95.5%) cases. Kaplan-Meier survival analysis and Cox proportional hazards regression were used to analyze prognostic variables. RESULTS: Disease-specific survival status was determined in 778 (95.5%) cases. The 5- and 10-year rates of overall survival (OS) were 25.2 and 12.2%; disease-specific survival (DSS), 32.4 and 19.3%. On multivariable analysis, anatomic primary site was an independent predictor of OS and DSS, with tumors in the nasal cavity and oral cavity associated with survival superior to tumors in the nasopharynx and paranasal sinuses. Age > 70 years, tumor size, nodal status, and distant metastasis status were additional independent predictors of poorer survival. CONCLUSIONS: In this large cohort of patients with MMHN, we have identified several novel factors robustly predictive of overall and melanoma-specific survival.

OBJECT: The aim of this study was to determine whether patients with neurofibromatosis Type 2 (NF2) who have intact ipsilateral cochlear nerves can have open-set speech discrimination following cochlear implantation. METHODS: Records of 7 patients with documented NF2 were reviewed to determine speech discrimination outcomes following cochlear implantation. Outcomes were measured using consonant-nucleus-consonant words and phonemes; Hearing in Noise Test sentences in quiet; and City University of New York sentences in quiet and in noise. RESULTS: Preoperatively, none of the patients had open-set speech discrimination. Five of the 7 patients had previously undergone excision of ipsilateral vestibular schwannoma (VS). One of the patients who received a cochlear implant had received radiation therapy for ipsilateral VS, and another was undergoing observation for a small ipsilateral VS. Following cochlear implantation, 4 of 7 patients with NF2 had open-set speech discrimination following cochlear implantation during extended follow-up (15-120 months). Two of the 3 patients without open-set speech understanding had a prolonged period between ipsilateral VS resection and cochlear implantation (120 and 132 months), and had cochlear ossification at the time of implantation. The other patient without open-set speech understanding had good contralateral hearing at the time of cochlear implantation. Despite these findings, 6 of the 7 patients were daily users of their cochlear implants, and the seventh is an occasional user, indicating that all of the patients subjectively gained some benefit from their implants. CONCLUSIONS: Cochlear implantation can provide long-term auditory rehabilitation, with open-set speech discrimination for patients with NF2 who have intact ipsilateral cochlear nerves. Factors that can affect implant performance include the following: 1) a prolonged time between VS resection and implantation; and 2) cochlear ossification

HYPOTHESIS: When cochlear implant (CI) users are allowed to self-select the 'most intelligible' frequency-to-electrode table, some of them choose one that differs from the default frequency table that is normally used in clinical practice. BACKGROUND: CIs reproduce the tonotopicity of normal cochleas using frequency-to-electrode tables that assign stimulation of more basal electrodes to higher frequencies and more apical electrodes to lower frequency sounds. Current audiologic practice uses a default frequency-to-electrode table for most patients. However, individual differences in cochlear size, neural survival, and electrode positioning may result in different tables sounding most intelligible to different patients. No clinical tools currently exist to facilitate this fitting. METHODS: A software tool was designed that enables CI users to self-select a most intelligible frequency table. Users explore a 2-dimensional space that represents a range of different frequency tables. Unlike existing tools, this software enables users to interactively audition speech processed by different frequency tables and quickly identify a preferred one. Pilot testing was performed in 11 long-term, postlingually deaf CI users. RESULTS: The software tool was designed, developed, tested, and debugged. Patients successfully used the tool to sample frequency tables and to self-select tables deemed most intelligible, which for approximately half of the users differed from the clinical default. CONCLUSION: A software tool allowing CI users to self-select frequency-to-electrode tables may help in fitting postlingually deaf users. This novel approach may transform current methods of CI fitting

OBJECTIVE: To investigate whether children implanted in the first year of life show higher levels of speech perception than later-implanted children, when compared at the same ages and to investigate the time course of sensitive periods for developing speech perception skills. More specifically, to determine whether faster gains in speech perception are made by children implanted before 1 year old relative to those implanted at 2 or 3 years. STUDY DESIGN: Retrospective cohort study. SETTING: Tertiary academic referral center. PATIENTS: 117 children with congenital profound bilateral sensorineural hearing loss, with no additional identified disabilities. INTERVENTION: Cochlear implantation in the first, second, or third year of life. MAIN OUTCOME MEASURE: Development curves showing Lexical Neighborhood Test (LNT) word identification scores as a function of age. RESULTS: Children implanted within the first year of life have a mean advantage of 8.2% LNT-easy word scores over those implanted in the second year (p < 0.001) and a 16.8% advantage in LNT-easy word scores over those implanted in the third year of life (p < 0.001). These advantages remained statistically significant after accounting for sex, residual hearing, and bilateral cochlear implant use. When speech perception scores were expressed as a function of 'hearing age' rather than chronological age, however, there were no significant differences among the 3 groups. CONCLUSION: There is a clear speech perception advantage for earlier-implanted children over later-implanted children when compared at the same age but not when compared at the same time after implantation. Thus, the sensitive period for developing word identification seems to extend at least until age 3 years