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Purpose: To better characterize the frequency and patterns of hearing dysfunction in patients who have received teprotumumab to treat thyroid eye disease., Design: Noncomparative case series., Participants: Patients who underwent audiology testing before and after completion of teprotumumab infusions., Methods: A review of patients who underwent audiology testing before and after completion of teprotumumab infusions was carried out. Additional audiogram testing during treatment was included when available. Hearing function was analyzed using audiogram data measuring threshold hearing levels at specific frequencies. Basic demographic data as well as information regarding otologic symptoms also were obtained and analyzed., Main Outcome Measures: Hearing loss demonstrated by a significant change in decibel hearing thresholds or that meets criteria for ototoxicity., Results: Twenty-two patients (44 ears) were included in the study, with baseline and most recent audiology testing after treatment ranging from 84 days before to 496 days after treatment. Fifteen patients (30 ears) also underwent testing during treatment starting after the second infusion up until the day of, but before, the eighth infusion. Hearing loss after treatment met criteria for ototoxicity in 17 of the 44 ears (38.6%), with 11 of the 22 patients (50.0%) meeting criteria in at least 1 ear. The pure-tone average decibel hearing levels (HLs) across all 44 ears demonstrated hearing loss after treatment (P = 0.0029), specifically at high (P = 0.0008) and middle frequencies (P = 0.0042), but not at low frequencies (P = 0.8344). Patients who were older also were more likely to experience hearing loss after treatment (P = 0.0048)., Conclusions: Audiometric data demonstrate that teprotumumab influences hearing function, most significantly at higher frequencies and in older patients. Audiometric testing is critical for counseling patients regarding teprotumumab treatment. A protocol for monitoring hearing during treatment is needed to detect and manage hearing changes associated with teprotumumab use., Financial Disclosure(s): Proprietary or commercial disclosure may be found in the Footnotes and Disclosures at the end of this article., (Copyright © 2023 American Academy of Ophthalmology. Published by Elsevier Inc. All rights reserved.)

Objective: Minimally traumatic surgical techniques and advances in cochlear implant (CI) electrode array designs have allowed acoustic hearing present in a CI candidate prior to surgery to be preserved postoperatively. As a result, these patients benefit from combined electric-acoustic stimulation (EAS) postoperatively. However, 30% to 40% of EAS CI users experience a partial loss of hearing up to 30 dB after surgery. This additional hearing loss is generally not severe enough to preclude use of acoustic amplification; however, it can still impact EAS benefits. The use of electrocochleography (ECoG) measures of peripheral hair cell and neural auditory function have shed insight into the pathophysiology of postimplant loss of residual acoustic hearing. The present study aims to assess the long-term stability of ECoG measures and to establish ECoG as an objective method of monitoring residual hearing over the course of EAS CI use. We hypothesize that repeated measures of ECoG should remain stable over time for EAS CI users with stable postoperative hearing preservation. We also hypothesize that changes in behavioral audiometry for EAS CI users with loss of residual hearing should also be reflected in changes in ECoG measures., Design: A pool of 40 subjects implanted under hearing preservation protocol was included in the study. Subjects were seen at postoperative visits for behavioral audiometry and ECoG recordings. Test sessions occurred 0.5, 1, 3, 6, 12 months, and annually after 12 months postoperatively. Changes in pure-tone behavioral audiometric thresholds relative to baseline were used to classify subjects into two groups: one group with stable acoustic hearing and another group with loss of acoustic hearing. At each test session, ECoG amplitude growth functions for several low-frequency stimuli were obtained. The threshold, slope, and suprathreshold amplitude at a fixed stimulation level was obtained from each growth function at each time point. Longitudinal linear mixed effects models were used to study trends in ECoG thresholds, slopes, and amplitudes for subjects with stable hearing and subjects with hearing loss., Results: Preoperative, behavioral audiometry indicated that subjects had an average low-frequency pure-tone average (125 to 500 Hz) of 40.88 ± 13.12 dB HL. Postoperatively, results showed that ECoG thresholds and amplitudes were stable in EAS CI users with preserved residual hearing. ECoG thresholds increased (worsened) while ECoG amplitudes decreased (worsened) for those with delayed hearing loss. The slope did not distinguish between EAS CI users with stable hearing and subjects with delayed loss of hearing., Conclusions: These results provide a new application of postoperative ECoG as an objective tool to monitor residual hearing and understand the pathophysiology of delayed hearing loss. While our measures were conducted with custom-designed in-house equipment, CI companies are also designing and implementing hardware and software adaptations to conduct ECoG recordings. Thus, postoperative ECoG recordings can potentially be integrated into clinical practice., Competing Interests: B.J.G. is a consultant for Cochlear Ltd. M.R.H. is cofounder and Chief Medical Officer for iotaMotion, Inc. V.D.T. is a consultant for iotaMotion, Inc. The remaining authors have no conflicts of interest to disclose., (Copyright © 2023 Wolters Kluwer Health, Inc. All rights reserved.)

Viral vector gene therapy is an attractive strategy to treat hearing loss. Since hearing loss is due to a variety of pathogenic signaling cascades in distinct cells, viral vectors that can express large or multiple genes in a cell-type specific manner are needed. Helper-dependent adenoviral vectors (HdAd) are safe viral vectors with a large packaging capacity (-36 kb). Despite the potential of HdAd, its use in the inner ear is largely unexplored. Therefore, to evaluate the utility of HdAd for inner ear gene therapy, we created two HdAd vectors that use distinct cellular receptors for transduction: HdAd Serotype Type 5 (HdAd5), the Coxsackie-Adenovirus Receptor (CAR) and a chimeric HdAd 5/35, the human CD46+ receptor (hCD46). We delivered these vectors through the round window (RW) or scala media in CBA/J, C57Bl6/J and hCD46 transgenic mice. Immunostaining in conjunction with confocal microscopy of cochlear sections revealed that multiple cell types were transduced using HdAd5 and HdAd 5/35 in all mouse models. Delivery of HdAd5 via RW in the C57Bl/6 J or CBA/J cochlea resulted in transduced mesenchymal cells of the peri‑lymphatic lining and modiolar region while scala media delivery resulted in transduction of supporting cells and inner hair cells. Hd5/35 transduction was CD46 dependent and RW delivery of HdAd5/35 in the hCD46 mouse model resulted in a similar transduction pattern as HdAd5 in the peri‑lymphatic lining and modiolar region in the cochlea. Our data indicate that HdAd vectors are promising vectors for use in inner ear gene therapy to treat some causes of hearing loss., (Copyright © 2023. Published by Elsevier B.V.)

Objectives/hypothesis: To determine the timing of acoustic hearing changes among hearing preservation Cochlear implant (CI) recipients. To determine differences in hearing outcomes based on device type and demographic factors. To determine if there is a relationship between the extent of early hearing loss after CI and the subsequent rate of continued hearing loss., Study Design: Prospective, single subject study., Methods: Two hundred and eleven subjects who received a hearing preservation CI were included in the study-80 Nucleus Hybrid L24 (Cochlear), 47 422/522 (Cochlear), 24 S8 (Cochlear), 14 S12 (Cochlear), 6 SRW (Cochlear), 21 SLIM J (Advanced Bionics), and 19 Flex (Med-EL). Of these, 127 were included in the subsequent analyses. Audiometric thresholds (low frequency pure-tone-averages) were collected and compared pre and postoperatively., Results: Long-term hearing preservation rates were 65% (52/80) for L24, 83% (20/24) for S8, 79% (11/14) for S12, 83% (5/6) for SRW, 54% (25/47) for 422/522, 91% (21/23) for SLIM J, and 84% (16/19) for Flex. Hearing loss was not related to device type (P = .9105) or gender (P = .2169). Older subjects (age ≥65) had worse hearing outcomes than younger subjects after initial device activation (age <65, P = .0262). There was no significant difference in rate of hearing loss over time between older and younger patients (P = .0938). Initial postoperative hearing loss was not associated with the rate of long-term hearing loss., Conclusions: Long-term low frequency hearing preservation is possible for CI recipients and is not associated with gender or device type. Rate of hearing loss over time is not dependent on patient age. Early hearing loss after CI does not predict the rate of long-term hearing loss., Level of Evidence: 3 Laryngoscope, 132:2036-2043, 2022., (© 2021 The American Laryngological, Rhinological and Otological Society, Inc.)

Understanding genetic causes of hearing loss can determine the pattern and course of a patient's hearing loss and may also predict outcomes after cochlear implantation. Our goal in this study was to evaluate genetic causes of hearing loss in a large cohort of adults and children with cochlear implants. We performed comprehensive genetic testing on all patients undergoing cochlear implantation. Of the 459 patients included in the study, 128 (28%) had positive genetic testing. In total, 44 genes were identified as causative. The top 5 genes implicated were GJB2 (20, 16%), TMPRSS3 (13, 10%), SLC26A4 (10, 8%), MYO7A (9, 7%), and MT-RNR1 (7, 5%). Pediatric patients had a higher diagnostic rate. This study lays the groundwork for future studies evaluating the relationship between genetic variation and cochlear implant performance.

Acoustic hearing can be preserved after cochlear implant (CI) surgery, allowing for combined electric-acoustic stimulation (EAS) and superior speech understanding compared to electric-only hearing. Among patients who initially retain useful acoustic hearing, 30-40 % experience a delayed hearing loss that occurs 3 or more months after CI activation. Increases in electrode impedances have been associated with delayed loss of residual acoustic hearing, suggesting a possible role of intracochlear inflammation/fibrosis as reported by Scheperle et al. (Hear Res 350:45-57, 2017) and Shaul et al. (Otol Neurotol 40(5):e518-e526, 2019). These studies measured only total impedance. Total impedance consists of a composite of access resistance, which reflects resistance of the intracochlear environment, and polarization impedance, which reflects resistive and capacitive properties of the electrode-electrolyte interface as described by Dymond (IEEE Trans Biomed Eng 23(4):274-280, 1976) and Tykocinski et al. (Otol Neurotol 26(5):948-956, 2005). To explore the role of access and polarization impedance components in loss of residual acoustic hearing, these measures were collected from Nucleus EAS CI users with stable acoustic hearing and subsequent precipitous loss of hearing. For the hearing loss group, total impedance and access resistance increased over time while polarization impedance remained stable. For the stable hearing group, total impedance and access resistance were stable while polarization impedance declined. Increased access resistance rather than polarization impedance appears to drive the increase in total impedances seen with loss of hearing. Moreover, access resistance has been correlated with intracochlear fibrosis/inflammation in animal studies as observed by Xu et al. (Hear Res 105(1-2):1-29, 1997) and Tykocinski et al. (Hear Res 159(1-2):53-68, 2001). These findings thus support intracochlear inflammation as one contributor to loss of acoustic hearing in our EAS CI population., (© 2021. Association for Research in Otolaryngology.)

Purpose of Review: Advancements in cochlear implant surgical approaches and electrode designs have enabled preservation of residual acoustic hearing. Preservation of low-frequency hearing allows cochlear implant users to benefit from electroacoustic stimulation, which improves performance in complex listening situations, such as music appreciation and speech understanding in noise. Despite the relative high rates of success of hearing preservation, postoperative acoustic hearing outcomes remain unpredictable., Recent Findings: Thin, flexible, lateral wall arrays are preferred for hearing preservation. Both shortened and thin, lateral wall arrays have shown success with hearing preservation and the optimal implant choice is an issue of ongoing investigation. Electrocochleography can monitor cochlear function during and after insertion of the electrode array. The pathophysiology of hearing loss acutely after cochlear implant may differ from that involved in delayed hearing loss following cochlear implant. Emerging innovations may reduce cochlear trauma and improve hearing preservation., Summary: Hearing preservation is possible using soft surgical techniques and electrode arrays designed to minimize cochlear trauma; however, a subset of patients suffer from partial to total loss of acoustic hearing months to years following surgery despite evidence of residual apical hair cell function. Early investigations in robotic-assisted insertion and dexamethasone-eluting implants show promise., (Copyright © 2021 Wolters Kluwer Health, Inc. All rights reserved.)

Changes in cochlear implant (CI) design and surgical techniques have enabled the preservation of residual acoustic hearing in the implanted ear. While most Nucleus Hybrid L24 CI users retain significant acoustic hearing years after surgery, 6-17 % experience a complete loss of acoustic hearing (Roland et al. Laryngoscope. 126(1):175-81. (2016), Laryngoscope. 128(8):1939-1945 (2018); Scheperle et al. Hear Res. 350:45-57 (2017)). Electrocochleography (ECoG) enables non-invasive monitoring of peripheral auditory function and may provide insight into the pathophysiology of hearing loss. The ECoG response is evoked using an acoustic stimulus and includes contributions from the hair cells (cochlear microphonic-CM) as well as the auditory nerve (auditory nerve neurophonic-ANN). Seven Hybrid L24 CI users with complete loss of residual hearing months after surgery underwent ECoG measures before and after loss of hearing. While significant reductions in CMs were evident after hearing loss, all participants had measurable CMs despite having no measurable acoustic hearing. None retained measurable ANNs. Given histological data suggesting stable hair cell and neural counts after hearing loss (e.g., Quesnel et al. Hear Res. 333:225-234. (2016)), the loss of ECoG and audiometric hearing may reflect reduced synaptic input. This is consistent with the theory that residual CM responses coupled with little to no ANN responses reflect a "disconnect" between hair cells and auditory nerve fibers (Fontenot et al. Ear Hear. 40(3):577-591. 2019). This "disconnection" may prevent proper encoding of auditory stimulation at higher auditory pathways, leading to a lack of audiometric responses, even in the presence of viable cochlear hair cells.

Objectives/hypothesis: The use of a short 10-mm/10-electrode cochlear implant to preserve low-frequency residual hearing was investigated. This report describes the 12-month outcomes of this multicenter clinical trial., Study Design: Single-subject design., Methods: Twenty-eight subjects with low-frequency hearing at or better than 60 dB HL at 500 Hz and severe high-frequency hearing loss were implanted with a Nucleus Hybrid S12 implant in their poorer ear. Speech perception in quiet using Consonant-Nucleus-Consonant (CNC) words and sentences in noise using AzBio sentences was collected pre- and postoperatively at 3, 6, and 12 months. Subjective reporting using the Speech, Spatial, and Qualities of Hearing Scale (SSQ) questionnaire was also collected pre- and postoperatively., Results: Functional hearing preservation was accomplished in 96% of subjects. At 3 and 6 months, 86% of the 28 subjects had maintained functional hearing. By 12 months, 23 out of 27 subjects (85%) had maintained functional hearing (one subject with functional hearing at 6 months withdrew from the study prior to the 12-month visit). Speech perception results demonstrated that 81% of the participants on CNC words and 77% with AzBio sentences in noise had significant improvements using their everyday listening condition at 12 months compared to preoperative performance with bilateral hearing aids. Furthermore, preoperative to 12 months postoperative subjective ratings showed significant improvements for the SSQ., Conclusions: This study demonstrates that a high degree of hearing preservation enabling acoustic-electric hearing and improvement in speech understanding in quiet and in noise can be accomplished using a short-electrode 10-mm cochlear implant., Level of Evidence: 2c Laryngoscope, 130:E548-E558, 2020., (© 2020 The American Laryngological, Rhinological and Otological Society, Inc.)

Objective: Few studies have investigated the stability of residual hearing and speech perception outcomes in individuals who were implanted with a shorter electrode device., Study Design: Longitudinal, single-subject design., Methods: Fifty subjects who received a Nucleus Hybrid (Cochlear, Sydney, Australia) short electrode cochlear implant (CI) and had a minimum of 2 years (and up to 15 years) of postoperative longitudinal experience were included in this study. Twenty-three subjects received a Nucleus Hybrid S8 (S8); 14 subjects received a Nucleus Hybrid L24 (L24); and 13 received a Nucleus Hybrid S12 (S12). Audiometric thresholds and consonant-nucleus-consonant (CNC) words were collected pre- and postoperatively for up to 15 years for the S8 subjects and for up to 7 years for the S12 and L24 subjects. AzBio Sentences in multi-talker babble was collected for up to 7 years on the S12 and L24 subjects., Results: Longitudinally, 83% of the S8 subjects, 92% of the S12 subjects, and 86% of the L24 subjects maintained a functional hearing pure-tone average (PTA) (125-500 Hz). Predicted change using a piecewise linear mixed model in PTA over time showed a postoperative linear decrease in hearing for each group until 0.5 years, after which the PTA stabilizes and is maintained. The averaged individual data for CNC and AzBio sentences show a significant improvement in scores by 0.25 to 0.5 years postimplantation, after which scores start to reach their maximum., Conclusion: This long-term study demonstrates that acoustic-electric processing hearing and improvement in speech understanding in quiet and in noise can be accomplished and sustained for many years with a short electrode CI., Level of Evidence: 2C. Laryngoscope, 128:473-481, 2018., (© 2017 The American Laryngological, Rhinological and Otological Society, Inc.)

This retrospective review explores delayed-onset hearing loss in 85 individuals receiving cochlear implants designed to preserve acoustic hearing at the University of Iowa Hospitals and Clinics between 2001 and 2015. Repeated measures of unaided behavioral audiometric thresholds, electrode impedance, and electrically evoked compound action potential (ECAP) amplitude growth functions were used to characterize longitudinal changes in auditory status. Participants were grouped into two primary categories according to changes in unaided behavioral thresholds: (1) stable hearing or symmetrical hearing loss and (2) delayed loss of hearing in the implanted ear. Thirty-eight percent of this sample presented with delayed-onset hearing loss of various degrees and rates of change. Neither array type nor insertion approach (round window or cochleostomy) had a significant effect on prevalence. Electrode impedance increased abruptly for many individuals exhibiting precipitous hearing loss; the increase was often transient. The impedance increases were significantly larger than the impedance changes observed for individuals with stable or symmetrical hearing loss. Moreover, the impedance changes were associated with changes in behavioral thresholds for individuals with a precipitous drop in behavioral thresholds. These findings suggest a change in the electrode environment coincident with the change in auditory status. Changes in ECAP thresholds, growth function slopes, and suprathreshold amplitudes were not correlated with changes in behavioral thresholds, suggesting that neural responsiveness in the region excited by the implant is relatively stable. Further exploration into etiology of delayed-onset hearing loss post implantation is needed, with particular interest in mechanisms associated with changes in the intracochlear environment., (Copyright © 2017 Elsevier B.V. All rights reserved.)

Purpose: To determine if two-dimensional and volumetric imaging parameters in vestibular schwannomas (VS) correlate with hearing loss at presentation., Study Design: Retrospective., Methods: Forty-one cases of pathologically confirmed sporadic VS were analyzed. Maximal tumor dimensions in anteroposterior (AP), coronal (ML), and craniocaudal (CC) dimensions were obtained along with tumor-fundus distance and internal auditory canal (IAC) porus diameters. Volumetric analysis was done on 37 cases. Tumors volumes were calculated through both 3-D volumetric and ABC/2 [AP × ML × CC/2] methodology. With the 3-D method, total tumor volume (TTV), and cerebellopontine angle (CPA) tumor volume were separately calculated and IAC volumes obtained by subtracting CPA tumor volumes from TTV. Pure tone average (PTA) and speech discrimination scores (SDS) were correlated with tumor dimensions., Results: Non-volumetric analysis was performed on 41 tumors. The AP and ML dimensions correlated with both PTA and SDS (p < 0.05). No significant correlations were seen between hearing loss and tumor-fundus distance or porus diameters. The tumor volume calculated through ABC/2 methodology correlated with PTA and SDS (p < 0.05). The 3-D TTV and CPA volumes only correlated with PTA. IAC tumor volumes did not correlate with hearing loss., Conclusion: Maximal AP and ML dimensions are the only non-volumetric variables, which significantly correlate with hearing loss. Tumor volume calculated through ABC/2 method significantly correlates with hearing impairment while the 3-D TTV and CPA tumor volumes only correlated significantly with PTA scores but not SDS scores.

Objective: Characterize hearing loss (HL) after hearing preservation cochlear implantation and determine the association between high charge electrical stimulation (ES) and late loss of acoustic hearing., Methods: A retrospective cohort analysis of all hearing preservation implantees at our center (n = 42) assayed HL as a function of maximum charge. We analyzed serial audiometry from 85 patients enrolled in the multicenter Hybrid S8 trial to detail the hearing loss greater than 1 month after implantation. Cochleotypic explant cultures were used to assess susceptibility to high levels of ES., Results: Early HL after implantation tends to be mild and averages 12.2 dB. After activation of the Hybrid S8 device, 17 (20%) of 85 patients experienced acceleration of HL. Compared with the majority of patients who did not lose significant hearing after activation, these patients experienced more severe HL at 1 year. Five patients implanted at our center experienced acceleration of HL after high charge exposure. In patients implanted at our center, high charge was associated with late HL (Pearson 0.366, p = 0.016). In rat cochleotypic explants, high voltage ES damaged afferent nerve fibers, reflected by blebbing and a 50% reduction in the number of fibers innervating the organ of Corti. In contrast, hair cells displayed only minor differences in cell number and morphology., Conclusions: Based on clinical and in vitro data, we theorize that the combination of acoustic amplification and ES in the setting of intact hair cells and neural architecture may contribute, in part, to cochlear toxicity, perhaps by damaging the afferent innervation.

Objective: Residual low-frequency acoustic hearing benefits cochlear implantees in difficult listening situations such as understanding speech in noise and music appreciation. Most subjects retain functional residual hearing in the operated ear. A small number of patients, however, will lose significant ipsilateral residual hearing after short-electrode cochlear implantation. The objectives of this retrospective series are to determine whether predisposition to hearing loss after implantation exists in a subset of patients and to assess the functional impact of this hearing loss on clinical measures of combined electric and acoustic hearing., Study Design: Retrospective case series., Setting: Multicenter clinical trial; tertiary care facility., Patients: Hearing preservation cochlear implant recipients., Main Outcome Measure: Frequency-averaged ipsilateral hearing loss at 1 year after activation., Results: Eighty-five patients from the Hybrid S8 FDA trial had serial postoperative audiometric measurements. Twenty-two of these patients, implanted at the home institution, provided additional medical data. Univariate analysis (Pearson's, Spearman's, Student's t test) showed that the severity of hearing loss at 1 year after activation was significantly correlated with age, male gender, and noise-induced hearing loss as the etiology of hearing impairment. A multivariate regression model corroborated these variables. No other medical factors were predictive. Clinical measures of speech perception (Consonant-Nucleus-Consonant and Hearing in Noise Test) worsened with hearing loss in ipsilateral but not bilateral listening conditions., Conclusion: Age, male gender, and a history of noise-induced hearing loss correlate with the severity of hearing loss at 1 year after activation. Even the most severely affected patients benefit from bilateral electric and acoustic inputs.

Background: Up to 7% of patients with severe-to-profound deafness do not benefit from cochlear implantation. Given the high surgical implantation and clinical management cost of cochlear implantation (>$1 million lifetime cost), prospective identification of the worst performers would reduce unnecessary procedures and healthcare costs. Because cochlear implants bypass the membranous labyrinth but rely on the spiral ganglion for functionality, we hypothesize that cochlear implant (CI) performance is dictated in part by the anatomic location of the cochlear pathology that underlies the hearing loss. As a corollary, we hypothesize that because genetic testing can identify sites of cochlear pathology, it may be useful in predicting CI performance., Methods: 29 adult CI recipients with idiopathic adult-onset severe-to-profound hearing loss were studied. DNA samples were subjected to solution-based sequence capture and massively parallel sequencing using the OtoSCOPE(®) platform. The cohort was divided into three CI performance groups (good, intermediate, poor) and genetic causes of deafness were correlated with audiometric data to determine whether there was a gene-specific impact on CI performance., Results: The genetic cause of deafness was determined in 3/29 (10%) individuals. The two poor performers segregated mutations in TMPRSS3, a gene expressed in the spiral ganglion, while the good performer segregated mutations in LOXHD1, a gene expressed in the membranous labyrinth. Comprehensive literature review identified other good performers with mutations in membranous labyrinth-expressed genes; poor performance was associated with spiral ganglion-expressed genes., Conclusions: Our data support the underlying hypothesis that mutations in genes preferentially expressed in the spiral ganglion portend poor CI performance while mutations in genes expressed in the membranous labyrinth portend good CI performance. Although the low mutation rate in known deafness genes in this cohort likely relates to the ascertainment characteristics (postlingual hearing loss in adult CI recipients), these data suggest that genetic testing should be implemented as part of the CI evaluation to test this association prospectively., (Copyright © 2012 Elsevier B.V. All rights reserved.)

Objective: Evaluate factors affecting outcomes of small acoustic neuroma (AN) removal via a middle cranial fossa (MCF) approach, and compare results to published data on observation and radiation therapy., Study Design: Retrospective chart review., Setting: Academic tertiary referral center., Methods: 162 consecutive patients (ages 19-70) with unilateral AN (0.2-2.5 cm in largest dimension) removed through a MCF approach were reviewed focusing on preservation of hearing, facial nerve function and complications. One hundred thirteen patients had pre-operative word recognition scores (WRS)>70%., Results: Both tumor size and pre-operative WRS were related to post-operative WRS (p<0.01). Overall, at least some hearing was preserved in 94 (60%) of the 156 patients who had hearing before surgery. If the WRS was also >70% (N=113), 56 (50%) maintained WRS>70%. Importantly, WRS for 12 others improved to >70% after surgery. When the patients were stratified by tumor size, the patients with small tumors (2-10 mm) faired better than the overall group. At least some hearing was preserved in 65 (72%) of the 90 patients. If the WRS was also >70% (N=66), 39 (59%) maintained WRS>70%. WRS for eight others improved to >70% after surgery. When the tumor was 1.1-1.4 cm (N=34), the chance of preserving some hearing decreased to 42% (14/33). If the WRS was also >70% (N=23), 9 (39%) maintained WRS>70%. WRS for three others improved to >70% after surgery. When the tumor reached 1.5-2.5 cm (N=35), the hearing preservation rate was 43%. If the WRS was also >70% (N=24), only eight (33%) maintained WRS of 70%, and one other improved to >70%. The addition of intra-operative whole eighth nerve near field monitoring improved results during small tumor (70% WRS in 76% (22/29) of those with >70% pre-operative WRS. Good facial nerve function (HB I-II) was achieved in 97% (86% HB I). When tumor size was