Factors Affecting Speech Discrimination After Vestibular Schwannoma Resection.

OBJECTIVE: Investigate the relationship between word recognition score (WRS) and pure tone average (PTA) after hearing preservation surgery for vestibular schwannomas (VS) as well as evaluate the consistency of hearing classification systems. STUDY DESIGN: A retrospective chart review was performed. SETTING: This study included patients from a single academic tertiary referral hospital. PATIENTS: Patients with VS and serviceable hearing who underwent hearing preservation surgery 2014-2023. Patients excluded for neurofibromatosis 2 and lacking pre/postop audiograms. INTERVENTIONS: All patients underwent resection of vestibular schwannoma. MAIN OUTCOME MEASURES: Pre/postop WRS, PTA, and AAO-HNS, Gardner-Robertson (GR), and WRS Class (WRSC) hearing classifications. RESULTS: Seventy-five patients were included. Average preop and postop PTA and WRS were 26 ± 12 dB, 79 ± 39 dB, 92 ± 12%, and 33 ± 43%, respectively. Postop PTAs were distributed along the complete testable decibel range, while the postop WRS displayed a bimodal distribution, with WRS >50% or <20%. Worsening intraop ABR changes were significantly associated with poorer hearing outcomes (p = 0.005). With increasing Koos grades, intraop ABRs were significantly more likely to exhibit changes (p = 0.005). AAO-HNS and GR classified patients nearly identically, while the WRSC resulted in more class I and fewer class II. The cutoff of serviceable hearing was comparable across all classification systems. CONCLUSIONS: Effects on the brainstem component of Koos 3-4 tumors may particularly disturb speech processing. This effect seems amplified by surgical dissection. AAO-HNS, GR, and WRSC hearing classifications are comparable in describing serviceable hearing in vestibular schwannoma patients.

Nestin-expressing cells are mitotically active in the mammalian inner ear.

Nestin expression is associated with pluripotency. Growing evidence suggests nestin is involved in hair cell development. The objective of this study was to investigate the morphology and role of nestin-expressing cells residing in the early postnatal murine inner ear. A lineage-tracing nestin reporter mouse line was used to further characterize these cells. Their cochleae and vestibular organs were immunostained and whole-mounted for cell counting. We found Nestin-expressing cells present in low numbers throughout the inner ear. Three morphotypes were observed: bipolar, unipolar, and globular. Mitotic activity was noted in nestin-expressing cells in the cochlea, utricle, saccule, and crista. Nestin-expressing cell characteristics were then observed after hair cell ablation in two mouse models. First, a reporter model demonstrated nestin expression in a significantly higher proportion of hair cells after hair cell ablation than in control cochleae. However, in a lineage tracing nestin reporter mouse, none of the new hair cells which repopulated the organ of Corti after hair cell ablation expressed nestin, nor did the nestin-expressing cells change in morphotype. In conclusion, Nestin-expressing cells were identified in the cochlea and vestibular organs. After hair cell ablation, nestin-expressing cells did not react to the insult. However, a small number of nestin-expressing cells in all inner ear tissues exhibited mitotic activity, supporting progenitor cell potential, though perhaps not involved in hair cell regeneration.

Debilitating Gentamicin Ototoxicity: Case Report and Recommendations Against Routine Use in Surgical Prophylaxis.

INTRODUCTION: Aminoglycoside antibiotics such as gentamicin are bactericidal and effective against gram negative organisms and act synergistically against gram positive organisms, including Staphylococcus aureus. However, they have serious adverse effects such as nephrotoxicity and ototoxicity. Gentamicin ototoxicity may occur after a single dose and results in decreased vestibular function, which is frequently debilitating and often permanent. OBJECTIVE: To emphasize the risk of gentamicin ototoxicity and suggest alternative antibiotics in penicillin-allergic patients undergoing surgery. CASE SUMMARY: We present a case of a woman with preexisting Meniere's Disease who received gentamicin 400 mg perioperatively for a sigmoidectomy due to a penicillin allergy listed in the patient's medical record. The patient developed severe ototoxicity preventing her from working or driving. Physical examination was remarkable for a broad-based gait requiring assistance to walk and bilateral corrective saccades. Vestibular testing revealed high-grade bilateral vestibular loss associated with all semicircular canals, a considerable decline compared to her function 3 years prior. DISCUSSION: Gentamicin is indicated for surgical prophylaxis when a patient has a true allergy to penicillins and cannot receive cephalosporins, though alternatives exist. True allergies include IgE-mediated illness (anaphylaxis, bronchospasm, or urticaria 30-60 minutes after administration) or exfoliative reactions (Stevens-Johnson Syndrome or Toxic Epidermal Necrolysis). The authors encourage more prudent use of gentamicin, especially in patients susceptible for debilitating otologic insults, and offer recommendations for alternative agents prior to using gentamicin.

Rare Coding Variants in Patients with Non-Syndromic Vestibular Dysfunction.

Vertigo due to vestibular dysfunction is rare in children. The elucidation of its etiology will improve clinical management and the quality of life of patients. Genes for vestibular dysfunction were previously identified in patients with both hearing loss and vertigo. This study aimed to identify rare, coding variants in children with peripheral vertigo but no hearing loss, and in patients with potentially overlapping phenotypes, namely, Meniere's disease or idiopathic scoliosis. Rare variants were selected from the exome sequence data of 5 American children with vertigo, 226 Spanish patients with Meniere's disease, and 38 European-American probands with scoliosis. In children with vertigo, 17 variants were found in 15 genes involved in migraine, musculoskeletal phenotypes, and vestibular development. Three genes, OTOP1, HMX3, and LAMA2, have knockout mouse models for vestibular dysfunction. Moreover, HMX3 and LAMA2 were expressed in human vestibular tissues. Rare variants within ECM1, OTOP1, and OTOP2 were each identified in three adult patients with Meniere's disease. Additionally, an OTOP1 variant was identified in 11 adolescents with lateral semicircular canal asymmetry, 10 of whom have scoliosis. We hypothesize that peripheral vestibular dysfunction in children may be due to multiple rare variants within genes that are involved in the inner ear structure, migraine, and musculoskeletal disease.

Effect of Head Position and Magnetic Resonance Sequence on Cochlear Implant-Related Artifact Size and Internal Auditory Canal Visibility.

HYPOTHESIS: To assess whether cochlear implant (CI)-related magnetic resonance imaging (MRI) artifact and visibility of the internal auditory canal (IAC) can be improved with head positioning and select MRI sequences. BACKGROUND: CI-related MRI artifact limits the use of CIs in otherwise good candidates because of the need for serial MRIs for monitoring of pathology. This most notably impacts patients with tumors of the cerebellopontine angle and IAC. METHODS: Two cadaver heads were implanted with either a Med-El CONCERT (fixed magnet) or SYNCHRONY (rotating magnet) device. Each head was imaged in a 1.5T scanner in 11 different positions. The SYNCHRONY-implanted head was also imaged in a 3.0T scanner in seven positions. Artifact size and IAC visibility (graded on a Likert scale) were measured for each head position by a neuroradiologist. RESULTS: The CONCERT CI produced significantly smaller artifact than the SYNCHRONY CI (effect size, 14.65 mm; p < 0.001). There was no significant difference between CI models in regard to IAC visibility. No head positions were statistically significantly better than neutral position for minimizing artifact size or IAC visibility, although some positions resulted in significantly larger artifact (effect sizes, 2.1-14.3 mm; p < 0.05) or significantly poorer IAC visibility (effect size, 1.4; p < 0.01). The T2 three-dimensional (CISS/FIESTA) sequence demonstrated significantly smaller artifact than T1 sequences, whereas T1 sequences demonstrated significantly better IAC visibility than T2 sequences. CONCLUSION: Head positioning and magnetic resonance sequence selection impact CI-related artifact size and IAC visibility.

Efficacy of Cochlear Implantation in Neurofibromatosis Type 2 Related Hearing Loss.

OBJECTIVE: To investigate the results of cochlear implantation in subjects with neurofibromatosis type 2 (NF2) and bilateral vestibular schwannomas (VS). STUDY DESIGN: Retrospective case series. SETTING: University-based tertiary referral center. SUBJECTS: Five subjects with NF2 and severe-to-profound sensorineural hearing loss. INTERVENTION: Cochlear implantation. MAIN OUTCOME MEASURE: Surgical outcomes and audiometric performance after cochlear implantation. RESULTS: Five subjects (3 female, 2 male) were included in the study. The mean age at the time of implantation was 54 years old (range 35-78 years). Follow-up after cochlear implantation averaged 38 months (range 21-106 months). In the 5 implanted ears, 2 had no prior treatment, 1 had undergone prior radiation therapy, 1 underwent prior microsurgical removal, and 1 underwent prior microsurgical removal with adjuvant radiation therapy. The mean ipsilateral VS dimensions at time of implantation were 14 mm × 7.2 mm × 6.1 mm (mediolateral × anteroposterior × craniocaudal). Following cochlear implant activation, all 5 subjects achieved sound awareness, open set speech recognition, and 4 continue to be daily users of the devices. CONCLUSION: Cochlear implantation is a viable hearing rehabilitation option for subjects with NF2 and severe-to-profound sensorineural hearing loss. All subjects reported benefit with their cochlear implant, including open set speech recognition, enhanced lip-reading skills and environmental awareness of sound. Four subjects continued to demonstrate improved open-set speech recognition at the time of their last evaluations.

Effects of Varying Laser Parameters During Laser Stapedotomy on Intracochlear Pressures.

OBJECTIVE: Sensorineural hearing loss is a known complication of stapes surgery. We previously showed that laser stapedotomy can result in intracochlear pressures that are comparable to high sound pressure levels. Optimizing laser settings to those that correspond with the lowest pressure changes may mitigate risk for postoperative hearing loss. Here we quantify the effects of various laser parameters on intracochlear pressures and test the hypothesis that intracochlear pressure changes are proportional to the laser energy delivered. STUDY DESIGN: Basic and translational science. SETTING: Cadaveric dissection and basic science laboratory. METHODS: Cadaveric human heads underwent mastoidectomies. Intracochlear pressures were measured via fiber-optic pressure probes placed in scala vestibuli and tympani. Pulses of varied stimulus power and duration from a 980-nm diode laser were applied to the stapes footplate. RESULTS: Sustained high-intensity pressures were observed in the cochlea during all laser applications. Observed pressure magnitudes increased monotonically with laser energy and rose linearly for lower stimulus durations and powers, but there was increased variability for laser applications of longer duration (200-300 ms) and/or higher power (8 W). CONCLUSIONS: Results confirm that significant pressure changes occur during laser stapedotomy, which we hypothesize may cause injury. Overall energy delivered depends predictably on duration and power, but surgeons should use caution at the highest stimulus levels and longest pulse durations due to the increasing variability in intracochlear pressure under these stimulus conditions. While the risk to hearing from increased intracochlear pressures from laser stapedotomy remains unclear, these results affirm the need to optimize laser settings to avoid unintended injury.

Hydroxyapatite cement cranioplasty for reconstruction of translabyrinthine approach: aesthetic results, long-term satisfaction, quality of life, and complications.

BACKGROUND: Translabyrinthine approaches (TLAs) for resection of skull base neoplasms are complex with variable reconstruction techniques. Fat grafts in conjunction with hydroxyapatite bone cement techniques have seldom been described in terms of possible superiority to other skull base reconstruction techniques. We sought to determine the impact of this skull base reconstruction technique on clinical outcomes and patient's satisfaction. METHOD: We performed a retrospective analysis of all patients who underwent translabyrinthine approaches for resection of CPA lesions over a 5-year period. Both post-op objective and subjective markers of reconstruction, as well as postoperative complications, were collected and analyzed. RESULTS: Sixty-nine patients were included, 34 underwent reconstruction with hydroxyapatite and fat (rHAC) and 35 with fat alone (rF). rHAC was associated with fewer cranial wound superficial infection/dehiscence (0% vs 14.3%; p = 0.029) and shorter length of stay (mean ± standard deviation) (6.9 ± 7.4 vs 4.4 ± 3.7 days, p = 0.008). Postoperative subjective characterization of rHAC demonstrated improved satisfaction scores (1.38 ± 0.5 vs 1.83 ± 1; p = 0.049) and fewer reports of post-operative irregularities (11.5% vs 37.5%; p = 0.017). CONCLUSION: The use of hydroxyapatite for cranial reconstruction after translabyrinthine approaches has improved patient satisfaction and decreased cranial defects in our small series. Postoperative complications are consistent with other described methods, but with shorter hospital stay, decreased risk of superficial wound dehiscence/infection, and a perceived superiority in preventing percutaneous post-op CSF leaks.

Novel candidate genes for cholesteatoma in chronic otitis media.

Cholesteatoma is a rare and benign disease, but its propensity to cause erosive damage through uninhibited growth can be detrimental to hearing and health. Prior reports indicated a genetic component to pathogenesis in at least a subset of patients. In this study, we aimed to identify rare DNA variants in affected patients. The salivary DNA of six patients whose middle ear tissues were obtained during tympanoplasty/mastoidectomy surgeries were submitted for exome sequencing. Tissue samples from the same patients were previously submitted for mRNA sequencing and analyzed for differentially expressed genes (DEGs). From the generated exome sequence data, rare predicted-to-be-damaging variants were selected within previously identified DEGs, and the candidate genes within which these rare variants lie were used for network analysis. Exome sequencing of six DNA samples yielded 5,078 rare variants with minor allele frequency <.001. A total of 510 variants were predicted to be deleterious and 52 were found to lie within previously identified DEGs. After selecting variants based on quality control measures, 12 variants were identified all from one pediatric patient. Network analysis identified ten significant cellular pathways, including protein transport, viral process, regulation of catalytic activity and cell cycle, and apoptotic and rhythmic processes. We hypothesize that the candidate genes identified in this study may be part of key signaling pathways during the mucosal response to middle ear infection. The occurrence of multiple rare variants may play a role in earlier onset of cholesteatoma formation in chronic otitis media.

Selective ablation of cochlear hair cells promotes engraftment of human embryonic stem cell-derived progenitors in the mouse organ of Corti.

BACKGROUND: Hearing loss affects 25% of the population at ages 60-69 years. Loss of the hair cells of the inner ear commonly underlies deafness and once lost this cell type cannot spontaneously regenerate in higher vertebrates. As a result, there is a need for the development of regenerative strategies to replace hair cells once lost. Stem cell-based therapies are one such strategy and offer promise for cell replacement in a variety of tissues. A number of investigators have previously demonstrated successful implantation, and certain level of regeneration of hair and supporting cells in both avian and mammalian models using rodent pluripotent stem cells. However, the ability of human stem cells to engraft and generate differentiated cell types in the inner ear is not well understood. METHODS: We differentiate human pluripotent stem cells to the pre-placodal stage in vitro then transplant them into the mouse cochlea after selective and complete lesioning of the endogenous population of hair cells. RESULTS: We demonstrate that hair cell ablation prior to transplantation leads to increased engraftment in the auditory sensory epithelium, the organ of Corti, as well as differentiation of transplanted cells into hair and supporting cell immunophenotypes. CONCLUSION: We have demonstrated the feasibility of human stem cell engraftment into an ablated mouse organ of Corti.

Reversible Canalith Jam of the Horizontal Semicircular Canal Mimicking Cupulolithiasis.

OBJECTIVE: To describe a case of benign paroxysmal positional vertigo (BPPV) resulting in reversible horizontal semicircular canalith jam successfully treated with horizontal canal occlusion. A brief literature review of similar cases was performed. METHODS: Case report and literature review. RESULTS: A 68-year-old female presented with apogeotropic positional nystagmus, attributed to reversible horizontal canalith jam mimicking cupulolithiasis that was refractory to tailored repositioning maneuvers across months. She was unable to work due to the severity of her symptoms. She underwent surgical occlusion of the affected canal with immediate resolution of her symptoms. A literature review revealed similar cases of canalith jam mimicking cupulolithiasis. CONCLUSIONS: Reversible canalith jam, in which particles moving with horizontal head position alternate between obstructing the semicircular canal and resting on the cupula, can mimic signs of cupulolithiasis. This variant of BPPV can be effectively managed with surgical canal occlusion should symptoms fail to resolve after tailored repositioning maneuvers.

Sex difference in the efferent inner hair cell synapses of the aging murine cochlea.

Efferent innervation of the inner hair cells changes over time. At an early age in mice, inner hair cells receive efferent feedback, which helps fine-tune tonotopic maps in the brainstem. In adulthood, inner hair cell efferent innervation wanes but increases again in older animals. It is not clear, however, whether age-related inner hair cell efferents increase along the entire range of the cochlear frequencies, or if this increase is restricted to a particular frequency-region, and whether this phenomenon occurs in both sexes. Age-related hearing loss, presbycusis, affects men and women differently. In mice, this difference is also strain specific. In aging black six mice, the auditory brainstem response thresholds increase in females earlier than in males. Here, we study age-related increase of the inner hair cell efferent innervation throughout the cochlea before hearing onset, in one month old and in ten months old and older male and female black six mice. We collected confocal images of immunostained inner hair cell efferents and quantified the labeled terminals in the entire cochlea using a machine learning algorithm. The overall number of the inner hair cell efferents in both sexes did not change significantly between age-groups. The distribution of the inner hair cell efferent innervation did not differ across frequencies in the cochlea. However, in females, inner hair cells received on average up to four times more efferent innervation than in males per each of the frequency regions tested. Sex differences were also found in the oldest age-group tested (≥ 10 months) where on average inner hair cells received six times more efferents in females than in males of matching age. Our findings emphasize the importance of including both sexes in sensorineural hearing loss research.

Impact of vestibular nerve preservation on facial and hearing outcomes in small vestibular schwannoma surgery: a technical feasibility study.

BACKGROUND: Management of small vestibular schwannomas (VSs) remains controversial. When surgery is chosen, the preservation of facial and cochlear nerve function is a priority. In this report, we introduce and evaluate a technique to anatomically preserve the vestibular nerves to minimize manipulation and preserve the function of the facial and cochlear nerves. METHODS: The vestibular nerve preservation technique was prospectively applied to resect small VS tumors in patients with serviceable preoperative hearing (American Academy of Otolaryngology-Head and Neck Surgery (AAO-HNS) class A or B). Clinical and radiological data were recorded and analyzed. RESULTS: Ten patients met the inclusion criteria. The mean (SD) age was 40.4 (12.5) years. Follow-up ranged from 6 weeks to 2 years. The maximum tumor diameter parallel to the internal auditory canal ranged from 10 to 20 mm (mean, 14.9 (3.1) mm). There were three Koos grade 3 and seven Koos grade 2 tumors. Gross total resection was achieved in all cases. Both the facial and cochlear nerves were anatomically preserved in all cases. Postoperatively, 7 patients (70%) remained in the AAO-HNS class A or B hearing category. None of the patients had new vestibular symptoms, and all had House-Brackmann grade 1 facial function. Nervus intermedius dysfunction was observed in 1 patient preoperatively, which worsened postoperatively. Two patients had new nervus intermedius symptoms postoperatively. CONCLUSION: Improvement of facial nerve and hearing outcomes is feasible through the intentional preservation of the vestibular nerves in the resection of small VSs. Longer follow-up is required to rule out tumor recurrence.

The FUT2 Variant c.461G>A (p.Trp154*) Is Associated With Differentially Expressed Genes and Nasopharyngeal Microbiota Shifts in Patients With Otitis Media.

Otitis media (OM) is a leading cause of childhood hearing loss. Variants in FUT2, which encodes alpha-(1,2)-fucosyltransferase, were identified to increase susceptibility to OM, potentially through shifts in the middle ear (ME) or nasopharyngeal (NP) microbiotas as mediated by transcriptional changes. Greater knowledge of differences in relative abundance of otopathogens in carriers of pathogenic variants can help determine risk for OM in patients. In order to determine the downstream effects of FUT2 variation, we examined gene expression in relation to carriage of a common pathogenic FUT2 c.461G>A (p.Trp154*) variant using RNA-sequence data from saliva samples from 28 patients with OM. Differential gene expression was also examined in bulk mRNA and single-cell RNA-sequence data from wildtype mouse ME mucosa after inoculation with non-typeable Haemophilus influenzae (NTHi). In addition, microbiotas were profiled from ME and NP samples of 65 OM patients using 16S rRNA gene sequencing. In human carriers of the FUT2 variant, FN1, KMT2D, MUC16 and NBPF20 were downregulated while MTAP was upregulated. Post-infectious expression in the mouse ME recapitulated these transcriptional differences, with the exception of Fn1 upregulation after NTHi-inoculation. In the NP, Candidate Division TM7 was associated with wildtype genotype (FDR-adj-p=0.009). Overall, the FUT2 c.461G>A variant was associated with transcriptional changes in processes related to response to infection and with increased load of potential otopathogens in the ME and decreased commensals in the NP. These findings provide increased understanding of how FUT2 variants influence gene transcription and the mucosal microbiota, and thus contribute to the pathology of OM.

Do Patients Benefit From a Cochlear Implant When They Qualify Only in the Presence of Background Noise?

OBJECTIVE: To compare the difference in pre- to postoperative speech performance of patients qualifying for a cochlear implant (CI) in quiet, +10 dB signal-to-noise ratio (SNR), and +5 dB SNR. STUDY DESIGN: Retrospective. SETTING: Tertiary referral center. PATIENTS: Fifty-eight post-lingually deafened, unilateral CI recipients from three Groups were included: 1) those who met CI candidacy criteria with AzBio sentences in quiet, 2) in noise at +10 dB SNR but not in quiet, 3) and in noise at +5 dB SNR but not in quiet or +10 dB SNR. INTERVENTION: Unilateral CI. MAIN OUTCOME MEASURES: Pre- and 1 year postoperative speech recognition scores. RESULTS: Best-aided AzBio speech recognition of individuals in Group 1 improved significantly for all test conditions and improved significantly for Groups 2 and 3 in the +10 and +5 dB SNR test conditions postoperatively. When tested with their CI alone however, while AzBio speech recognition of individuals in Group 1 and Group 2 improved significantly in the quiet and +10 dB SNR conditions, speech recognition was not significantly changed postoperatively under any testing condition for individuals in Group 3. CONCLUSIONS: While individuals qualifying for a CI only in the +5 dB SNR condition may derive significant benefit from implantation in best aided conditions, speech understanding outcomes can be more variable thus warranting additional counseling before implantation and case-by-case consideration of listening needs and goals.

Multi-omic studies on missense PLG variants in families with otitis media.

Otitis media (OM), a very common disease in young children, can result in hearing loss. In order to potentially replicate previously reported associations between OM and PLG, exome and Sanger sequencing, RNA-sequencing of saliva and middle ear samples, 16S rRNA sequencing, molecular modeling, and statistical analyses including transmission disequilibrium tests (TDT) were performed in a multi-ethnic cohort of 718 families and simplex cases with OM. We identified four rare PLG variants c.112A > G (p.Lys38Glu), c.782G > A (p.Arg261His), c.1481C > T (p.Ala494Val) and c.2045 T > A (p.Ile682Asn), and one common variant c.1414G > A (p.Asp472Asn). However TDT analyses for these PLG variants did not demonstrate association with OM in 314 families. Additionally PLG expression is very low or absent in normal or diseased middle ear in mouse and human, and salivary expression and microbial α-diversity were non-significant in c.1414G > A (p.Asp472Asn) carriers. Based on molecular modeling, the novel rare variants particularly c.782G > A (p.Arg261His) and c.2045 T > A (p.Ile682Asn) were predicted to affect protein structure. Exploration of other potential disease mechanisms will help elucidate how PLG contributes to OM susceptibility in humans. Our results underline the importance of following up findings from genome-wide association through replication studies, preferably using multi-omic datasets.

The Changing Paradigm of Head and Neck Paragangliomas: What Every Otolaryngologist Needs to Know.

BACKGROUND: Recommendations regarding head and neck paragangliomas (HNPGL) have undergone a fundamental reorientation in the last decade as a result of increased understanding of the genetic and pathophysiologic basis of these disorders. OBJECTIVE: We aim to provide an overview of HNPGL and recent discoveries regarding their molecular genetics, along with updated recommendations on workup, treatment, and surveillance, and their implications for otolaryngologists treating patients with these disorders. RESULTS: SDHx susceptibility gene mutations, encoding subunits of the enzyme succinate dehydrogenase (SDH), give rise to the Hereditary Pheochromocytoma/Paraganglioma Syndromes. SDHA, SDHB, SDHC, SDHD, and SDHAF2 mutations each result in unique phenotypes with distinct penetrance and risk for variable tumor development as well as metastasis. Genetic and biochemical testing is recommended for every patient with HNPGL. Multifocal disease should be managed in multi-disciplinary fashion. Patients with SDHx mutations require frequent biochemical screening and whole-body imaging, as well as lifelong follow-up with an expert in hereditary pheochromocytoma and paraganglioma syndromes. CONCLUSION: Otolaryngologists are likely to encounter patients with HNPGL. Keeping abreast of the latest recommendations, especially regarding genetic testing, workup for additional tumors, multi-disciplinary approach to care, and need for lifelong surveillance, will help otolaryngologists appropriately care for these patients.

The Combined Endoscopic Endonasal Far Medial and Open Postauricular Transtemporal Approaches As a Lesser Invasive Approach to the Jugular Foramen: Anatomic Morphometric Study With Case Illustration.

BACKGROUND: Access to the jugular foramen (JF) requires extensive approaches. An endoscopic endonasal far medial (EEFM) approach combined with a postauricular transtemporal (PTT) approach may provide adequate exposure with limited morbidities. OBJECTIVE: To provide a quantitative anatomic comparison of the EEFM, the PTT, and the combined EEFM/PTT approaches. A clinical case of the combined approach is presented. METHODS: Five cadaveric heads were dissected. Each specimen received PTT and EEFM approaches on opposite sides followed by an EEFM approach on the side of the PTT approach. Morphometric and quadrant analyses were conducted. Three groups were obtained and compared: PTT (group A), EEFM (group B), and combined (group C). RESULTS: Group B had a significantly higher area of exposure of the JF as compared to group A (112.3 and 225 mm2, respectively, P = .004). The average degree of freedom (DOF) in the cranio-caudal plane for groups A and B was 63.6 and 12.6 degrees, respectively (P < .00001). Group A had a higher DOF in the medial-lateral plane than group B (49 vs 13.4 degrees, respectively, P < .00001. The average volume of exposure in groups A and B was 1469.2 and 1897.4 mm3, respectively (P = .02). By adding an EEFM approach to the PTT approach, an additional 56.1% of the anterior quadrant was exposed, representing a 584.4% increase in the anterior exposure. CONCLUSION: The PTT and EEFM approaches provide optimal exposures to different aspects of the JF and in combination may constitute a less invasive alternative to the more extensive approaches.

Practical aspects of inner ear gene delivery for research and clinical applications.

The application of gene therapy is widely expanding in research and continuously improving in preparation for clinical applications. The inner ear is an attractive target for gene therapy for treating environmental and genetic diseases in both the auditory and vestibular systems. With the lack of spontaneous cochlear hair cell replacement, hair cell regeneration in adult mammals is among the most important goals of gene therapy. In addition, correcting gene defects can open up a new era for treating inner ear diseases. The relative isolation and small size of the inner ear dictate local administration routes and carefully calculated small volumes of reagents. In the current review, we will cover effective timing, injection routes and types of vectors for successful gene delivery to specific target cells within the inner ear. Differences between research purposes and clinical applications are also discussed.

Time-based Assessment of Hearing Preservation Rates After Microsurgical Resection of Vestibular Schwannomas: A Systematic Review.

OBJECTIVE: To determine short- and intermediate-term hearing preservation rates after microsurgical resection of vestibular schwannoma (VS). DATA SOURCES: Systematic review of the Ovid, Cochrane, EMBASE, and Web of Science databases. STUDY SELECTION: This study was restricted to full-text English-language articles detailing VS resection via the middle cranial fossa or retrosigmoid approaches. Documentation of pre- and posttreatment hearing outcomes with American Academy of Otolaryngology-Head and Neck Surgery, Gardner-Robertson, or word recognition score scales, as well as time to follow-up were required. Duplicate data sets, studies with >10% of patients with neurofibromatosis two, previous or nonsurgical VS treatment, case reports with