Objectives/Hypothesis To report a series of patients with neurofibromatosis type 2 (NF2), where each patient underwent both cochlear implantation and auditory brainstem implantation for hearing rehabilitation, and to discuss factors influencing respective implant success. Study Design Retrospective case series. Methods Ten NF2 patients with both cochlear implantations and auditory brainstem implantations were retrospectively reviewed. Speech testing for auditory brainstem implants (ABIs) and cochlear implants (CIs) was performed separately. Scores at last follow‐up were obtained for Iowa vowels and consonants, Northwestern University Children's Perception of Speech (NU‐CHIPS), and City University of New York (CUNY) sentences. Results Mean age at time of implant was 37 years for cochlear implantation and 40 years for auditory brainstem implantation ( P = .790, t test). Nine of 10 patients had a CI and ABI on contralateral sides, and one had both devices on the same side. Mean duration of deafness in the implanted ear was 4.3 years for both cochlear implantation and auditory brainstem implantation ( P = .491, t test). Follow‐up range was 1 to 28 years. CI performance on NU‐CHIPS was 32% to 100%, and sound + lip‐reading CUNY was 56% to 100%. Four patients experienced an eventual decline in CI function to unusable levels. ABI performance on NU‐CHIPS was 40% to 80%, and sound + lip‐reading CUNY was 38% to 94%. There was no notable decline in ABI function over time. Conclusions If the cochlear nerve is intact, cochlear implantation can be an effective strategy for hearing rehabilitation in NF2. However, a significant proportion experience a decline in CI performance related to growing vestibular schwannoma or tumor treatment. Auditory brainstem implantation remains the standard option for surgical hearing rehabilitation in NF2, but peak performance is generally lower than that achievable with cochlear implantation. Level of Evidence 4. Laryngoscope , 128:2163–2169, 2018

Program Description:The goals of cholesteatoma surgery are the complete removal of disease while minimizing the risk of recurrence. A secondary goal is to optimize postoperative hearing. This miniseminar features distinguished experts in the field who will present well‐established methods of cholesteatoma surgery. Important topics, such as intact‐canal‐wall versus canal‐wall‐down surgery, recurrence prevention, use of cartilage, and management of labyrinthine fistula will then be discussed in a case‐based format. The role of endoscopes and use of magnetic resonance imaging for detection of recurrence will be discussed. Finally, ossiculoplasty techniques will be presented.Educational Objectives:(1) Perform cholesteatoma surgery as practiced by experts in the field. (2) Examine advantages and disadvantages of endoscopic techniques. (3) Incorporate various methods for preventing recurrence.

Program Description: Although there are well-established surgical techniques to reconstruct the ossicular chain, these methods remain varied, often based more on local tradition than on scientific research. The increasing number of available prostheses further increases the number of potential surgical variables. Successful ossiculoplasty as a part of tympanoplasty therefore continues to pose a significant challenge to the otolaryngologist. The goal of this miniseminar is to present the current best surgical technique for ossiculoplasty. Toward this end, we have assembled a panel of distinguished otologists who will draw on significant advances in basic research and ossicular prosthesis design, in addition to their vast clinical experience. Following a brief introduction, each panelist will present his preferred surgical technique of ossiculoplasty for reconstruction of the ossicular chain. The remaining time will be devoted to case presentations to identify the best ossiculoplasty method in specific clinical situations. This miniseminar will provide information that should improve the ability of each participant to perform successful ossiculoplasty.

Program DescriptionAlthough there are well‐established surgical techniques for tympanoplasty to repair chronic tympanic membrane perforation, surgical methods remain varied, often based more on local traditions than on scientific research. Successful tympanoplasty continues to pose a significant challenge to the otolaryngologist. The goal of this miniseminar is to present the best surgical technique of this common procedure. Toward these ends, we have assembled a panel of respected and experienced otologists from the East coast, Midwest, South, and West coast of the US. Following a brief introduction, each panelist will present his preferred surgical technique of tympanoplasty for tympanic membrane perforation that works best whether media, lateral, medio‐lateral, or cartilage graft method. The remaining time will be devoted to case presentations to discuss on best graft method for particular cases. This miniseminar will provide information that should improve the ability of each participant to perform successful tympanoplasty for chronic tympanic membrane perforation in different clinical situations.Educational Objectives1) Learn best grafting method in tympanoplasty for reconstruction of chronic tympanic membrane perforation. 2) Understand to use different method grafting depending on the location of tympanic membrane perforation. 3) Be able to perform better tympanoplasty in different clinical situations.

Objective: To assess the duration of hearing preservation from time of vestibular schwannoma middle fossa decompression with short-term and 1-year hearing preservation rates. Study Design: Retrospective case review. Setting: Tertiary referral center. Patients: Patients with and without neurofibromatosis type 2 who underwent middle fossa decompression. We excluded patients without tumor size or audiograms at initial diagnosis or follow-up less than 3 months. Interventions: Middle cranial fossa decompression, audiometry, and magnetic resonance imaging. Main Outcome Measures: Period of hearing maintenance (from surgery to longest time point that preoperative American Academy of Otolaryngology-Head and Neck Surgery [AAO-HNS] hearing class maintained or improved), short-term hearing preservation (within 3 mo of surgery), and 1-year hearing preservation. Results: A total of 49 patients underwent middle fossa decompression of vestibular schwannoma. Approximately 90% of patients had documented hearing loss before surgery, and more than 50% of patients exhibited significant tumor growth before surgery. Of these surgeries, more than 90% were performed in patients with hearing loss in an only hearing ear, and more than 90% were patients with neurofibromatosis type 2. The mean period of hearing maintenance was 2.1 years. The short-term hearing preservation rate as measured by the change from preoperative AAO-HNS hearing class was approximately 90%. The 1-year hearing preservation rate as measured by change from preoperative AAO-HNS hearing class was 63%. Conclusion: Middle fossa decompression for vestibular schwannoma can prolong hearing in patients with hearing changes in an only hearing ear. Understanding the duration of hearing preservation can enable more effective counseling of patients considering middle cranial fossa decompression for vestibular schwannoma.

Objective: To describe the diagnosis, management, and treatment outcome of jugular foramen (JF) tumors.Study Design: Retrospective chart review.Methods: Charts of the 83 patients diagnosed with JF tumors between January 1997 and May 2008 were reviewed. Presenting symptoms, otologic and neurotologic examination, audiologic thresholds, treatment procedure, surgical technique, tumor size and classification, and postoperative complications were recorded. Facial nerve function was graded using the House-Brackmann scale. Extent of tumor removal was determined at time of surgery, followed by routine radiographic follow-up.Results: The mean age of patients with JF tumors was 48.5 years (standard deviation, 16.3 yr), and women (79.5%) outnumbered men (20.5%). Most had glomus jugulare (GJ) tumors (n = 67, 80.7%); 9 patients had lower cranial nerve schwannomas (10.8%), and 7 patients had meningiomas (8.4%). The most frequent initial symptoms included pulsatile tinnitus (84.3%), conductive hearing loss (75.9%), and hoarseness (34.9%). Sixty-one patients (73.5%) underwent surgery, 18.1% had radiotherapy, and 8.4% were observed. Total tumor removal was achieved in 81% of surgery cases. New lower cranial nerve (CN) deficits occurred after surgery in 18.9% of GJ, 22.2% of schwannoma, and 50% of the 4 meningiomas. At last follow-up, 88.1% of surgical patients had normal or near-normal (House-Brackmann I or II) facial function.Conclusion: Total resection of GJ tumors, meningiomas, and lower CN schwannomas can be a curative treatment. However, subtotal removal may be required to preserve CN function, vital vascular structures, and the brainstem. Postoperative radiotherapy is used to control residual tumor. When postoperative complications develop in patients, early rehabilitation is important to decrease mortality and morbidity. Therefore, patients should be closely followed.

Objective: Review publications reporting radiation treatment of vestibular schwannomas (VS) and describe how data, patient characteristics, and study endpoints were reported. Data Sources: PubMed search for English language articles on radiation treatment of VS published from January 2002 to July 2007. Study Selection: Studies presenting outcomes were selected, yielding 56 articles (58 studies) in journals of neurosurgery (30), oncology (18), otolaryngology (6), and other (2). Data Extraction/Synthesis: Data included type of study, number of subjects, demographics, follow-up times, type of radiation, tumor size, tumor control definition, control rates, facial nerve function measure and outcome, type of hearing and vestibular testing and outcomes, and complications. Descriptive statistics were performed. Results: Studies (72.9%) were retrospective reviews with stated sample sizes ranging from 5 to 829. Gamma-knife (49.2%), linear accelerator (35.6%), and proton beam (6.8%) were used with various doses. Average follow-up was less than 5 years in 79.6% of studies, and 67.4% included patients at less than or equal to 1 year. Tumor size was reported as diameter (23.7%), volume (49.2%), both (11.9%), other (3.4%), or not reported (11.9%). Definition of tumor control varied: less than or equal to 2 mm growth (22.0%), no visible/measurable change (16.9%), required surgery (10.2%), other (17.0%), and not clearly specified (33.9%). Facial nerve outcome was reported as House-Brackmann (64.4%), normal/abnormal (11.9%), other (1.7%), or was not reported (22%). Conclusion: The lack of uniform reporting criteria for tumor control, facial function and hearing preservation, and variability in follow-up times make it difficult to compare studies of radiation treatment for VS. We recommend consideration of reporting guidelines such as those used in otology for reporting VS resection results.

OBJECTIVE:The penetrating electrode auditory brainstem implant (PABI) is an extension of auditory brainstem implant (ABI) technology originally developed for individuals deafened by neurofibromatosis type 2. Whereas the conventional ABI uses surface electrodes on the cochlear nuclei, the PABI uses 8 or 10 penetrating microelectrodes in conjunction with a separate array of 10 or 12 surface electrodes. The goals of the PABI were to use microstimulation to reduce threshold current levels, increase the range of pitch percepts, and improve electrode selectivity and speech recognition. PATIENTS AND PROTOCOL: In a prospective clinical trial, 10 individuals, all with neurofibromatosis type 2, received a PABI after vestibular schwannoma removal via a translabyrinthine approach. All study participants met strict requirements for informed consent as part of a Food and Drug Administration clinical trial. Approximately 8 weeks after implantation, PABI devices were activated and tested at our tertiary clinical and research facility. Mean follow-up time was 33.8 months.STUDY DESIGN:Using a single-subject design, we measured thresholds and dynamic ranges, electrode-specific pitch percepts, and speech perception performance at regular intervals.RESULTS:Penetrating electrodes produced auditory thresholds at substantially lower charge levels than surface electrodes, a wide range of electrode-specific pitch sensations, and minimal cross-electrode interference and could be used in speech maps either alone or in combination with surface electrodes. However, less than 25% of penetrating electrodes resulted in auditory sensations, whereas more than 60% of surface electrodes were effective. Even after more than 3 years of experience, patients using penetrating electrodes did not achieve improved speech recognition compared with those using surface electrode ABIs. In patients with usable penetrating electrodes, City University of New York Sentence Test scores with sound and visual information were 61.6% in the PABI group and 64.7% in a surface ABI cohort (p = not significant).CONCLUSION:The PABI met the goals of lower threshold, increased pitch range, and high selectivity, but these properties did not result in improved speech recognition.

This paper discusses the development and implementation of three novel implantable technologies that have advanced the communication abilities of hearing-impaired individuals who cannot benefit from conventional hearing aids. This paper will discuss clinical indications and outcomes and include current technological limitations and future research efforts.

OBJECTIVE Hypoglossal–facial neurorrhaphy has been widely used for reanimation of paralyzed facial muscles after irreversible proximal injury of the facial nerve. However, complete section of the hypoglossal nerve occasionally results in hemiglossal dysfunction and interferes with swallowing and speech. To reduce this morbidity, a modified technique with partial section of the hypoglossal nerve after mastoid dissection of the facial nerve (HFM) has been used. We report our experience with the HFM technique, retrospectively comparing the outcome with results of the classic hypoglossal-facial neurorrhaphy. METHODS A retrospective review was performed in 36 patients who underwent hypoglossal-facial neurorrhaphy with the classic (n = 12) or variant technique (n = 24) between 2000 and 2006. Facial outcome was evaluated with the House-Brackmann grading system, and tongue function was evaluated with a new scale proposed to quantify postoperative tongue alteration. The results were compared, and age and time between nerve injury and surgery were correlated with the outcome. RESULTS There was no significant difference between the two techniques concerning facial reanimation. A worse outcome of tongue function, however, was associated with the classic technique (Mann-Whitney U test; P < 0.05). When HFM was used, significant correlations defined by the Spearman test were identified between preoperative delay (ρ = 0.59; P = 0.002) or age (ρ = 0.42; P = 0.031) and results of facial reanimation evaluated with the House-Brackmann grading system. CONCLUSION HFM is as effective as classic hypoglossal-facial neurorrhaphy for facial reanimation, and it has a much lower morbidity related to tongue function. Better results are obtained in younger patients and with a shorter interval between facial nerve injury and surgery.