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.

OBJECTIVES:Cochlear implant array malpositioning is associated with impaired speech perception, vertigo, and facial nerve stimulation. Tip fold-over is a subset of malpositioning that occurs more often with perimodiolar electrodes, but historically it has not been characterized due to lack of knowledge regarding electrode movements of the electrode within the cochlea. The aim of this study was to characterize the mechanics of tip fold-over events and their associated insertion pressure profiles. METHODS:Cadaveric human heads were surgically prepared with a mastoidectomy and facial recess. Fiberoptic pressure sensors were inserted into the scala vestibuli and tympani to measure intracochlear pressures. Perimodiolar CI electrodes (Cochlear Slim-Modiolar, CI532) were inserted via round window under fluoroscopy. RESULTS:Three types of tip fold-over events were observed: anterior-posterior C-shaped, medial-lateral C-shaped, and S-shaped roll-overs. The largest transient pressures occurred with anterior-posterior and S-type roll-over, and were associated with rotation or twisting inside the cochlea. CONCLUSIONS:Results demonstrate at least three subtypes of tip fold-overs. Elevated pressure transients were noted before and during the tip fold-over event related to electrode twisting. The characterization of tip fold-over into subtypes is novel and may aid identification of tip fold-over events intraoperatively in the future. It remains important to identify tip fold-over events, and they should be recognized early using a multimodal verification system. Further investigation is still required to determine the significance of these changes and other possible patterns of intracochlear electrode movement. LEVEL OF EVIDENCE:NA: Cadaver study Laryngoscope, 135:1795-1802, 2025.

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.

Background: Uncompensated vestibular hypofunction can result in symptoms of dizziness, imbalance, and/or oscillopsia, gaze and gait instability, and impaired navigation and spatial orientation; thus, may negatively impact an individual's quality of life, ability to perform activities of daily living, drive, and work. It is estimated that one-third of adults in the United States have vestibular dysfunction and the incidence increases with age. There is strong evidence supporting vestibular physical therapy for reducing symptoms, improving gaze and postural stability, and improving function in individuals with vestibular hypofunction. The purpose of this revised clinical practice guideline is to improve quality of care and outcomes for individuals with acute, subacute, and chronic unilateral and bilateral vestibular hypofunction by providing evidence-based recommendations regarding appropriate exercises. Methods: These guidelines are a revision of the 2016 guidelines and involved a systematic review of the literature published since 2015 through June 2020 across 6 databases. Article types included meta-analyses, systematic reviews, randomized controlled trials, cohort studies, case-control series, and case series for human subjects, published in English. Sixty-seven articles were identified as relevant to this clinical practice guideline and critically appraised for level of evidence. Results: Based on strong evidence, clinicians should offer vestibular rehabilitation to adults with unilateral and bilateral vestibular hypofunction who present with impairments, activity limitations, and participation restrictions related to the vestibular deficit. Based on strong evidence and a preponderance of harm over benefit, clinicians should not include voluntary saccadic or smooth-pursuit eye movements in isolation (ie, without head movement) to promote gaze stability. Based on moderate to strong evidence, clinicians may offer specific exercise techniques to target identified activity limitations and participation restrictions, including virtual reality or augmented sensory feedback. Based on strong evidence and in consideration of patient preference, clinicians should offer supervised vestibular rehabilitation. Based on moderate to weak evidence, clinicians may prescribe weekly clinic visits plus a home exercise program of gaze stabilization exercises consisting of a minimum of: (1) 3 times per day for a total of at least 12 minutes daily for individuals with acute/subacute unilateral vestibular hypofunction; (2) 3 to 5 times per day for a total of at least 20 minutes daily for 4 to 6 weeks for individuals with chronic unilateral vestibular hypofunction; (3) 3 to 5 times per day for a total of 20 to 40 minutes daily for approximately 5 to 7 weeks for individuals with bilateral vestibular hypofunction. Based on moderate evidence, clinicians may prescribe static and dynamic balance exercises for a minimum of 20 minutes daily for at least 4 to 6 weeks for individuals with chronic unilateral vestibular hypofunction and, based on expert opinion, for a minimum of 6 to 9 weeks for individuals with bilateral vestibular hypofunction. Based on moderate evidence, clinicians may use achievement of primary goals, resolution of symptoms, normalized balance and vestibular function, or plateau in progress as reasons for stopping therapy. Based on moderate to strong evidence, clinicians may evaluate factors, including time from onset of symptoms, comorbidities, cognitive function, and use of medication that could modify rehabilitation outcomes. Discussion: Recent evidence supports the original recommendations from the 2016 guidelines. There is strong evidence that vestibular physical therapy provides a clear and substantial benefit to individuals with unilateral and bilateral vestibular hypofunction. Limitations: The focus of the guideline was on peripheral vestibular hypofunction; thus, the recommendations of the guideline may not apply to individuals with central vestibular disorders. One criterion for study inclusion was that vestibular hypofunction was determined based on objective vestibular function tests. This guideline may not apply to individuals who report symptoms of dizziness, imbalance, and/or oscillopsia without a diagnosis of vestibular hypofunction. Disclaimer: These recommendations are intended as a guide to optimize rehabilitation outcomes for individuals undergoing vestibular physical therapy. The contents of this guideline were developed with support from the American Physical Therapy Association and the Academy of Neurologic Physical Therapy using a rigorous review process. The authors declared no conflict of interest and maintained editorial independence. Video Abstract available for more insights from the authors (see the Video, Supplemental Digital Content 1, available at: http://links.lww.com/JNPT/A369).

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.

OBJECTIVE:Nervus intermedius (NI) dysfunction after the management of vestibular schwannoma (VS) is underreported and is experienced by patients undergoing stereotactic radiosurgery and surgical resection. The aim of this study was to present NI outcomes in a series of patients who underwent all treatment modalities for VS and to review the existing literature.METHODS:We performed a retrospective review of all patients with VS who were treated at our institution between January 1, 2008, and December 31, 2018. Demographic data, tumor size, Koos grade, treatment modality, extent of resection, postoperative facial nerve function, and hearing function were collected. NI outcomes were determined from phone interview using a previously published functional questionnaire.RESULTS:Of 222 patients who were reviewed, 98 patients responded to the questionnaire. Patients were stratified into 3 groups: group 1, 54 patients who underwent radiation; group 2, 27 patients who underwent surgical treatment; group 3, 17 patients who underwent both radiation and surgery. Of patients, 28% presented with preoperative NI dysfunction, most commonly dry eye followed by taste dysfunction and lacrimation dysfunction. Following treatment, 79% of patients experienced NI dysfunction, most commonly dry eye. Statistical differences in dry eye and taste dysfunction were observed when comparing the treatment groups.CONCLUSIONS:NI dysfunction is common following treatment for VS and should be included in pretreatment counseling of patients, as it may impact treatment choice and quality of life. Additional studies are warranted to fully characterize NI dysfunction after treatment.

Abstract Objectives To understand the presence of transient autophony symptoms in patients being treated for severe anorexia nervosa (AN), and whether those symptoms were due to patulous eustachian tube (PET). Methods A prospective observational study was performed in patients requiring admission for treatment of severe AN. All enrolled patients completed The Eustachian Tube Dysfunction Questionnaire (ETDQ‐7) and were screened for symptoms of autophony. If patients reported autophony and had a score of ≥14.5 on the ETDQ‐7 they were asked to undergo comprehensive audiological testing and an evaluation with an otolaryngologist. Results Of the 73 patients enrolled in the study, 35 patients (44%) reported autophony and 36 (49%) scored 14.5 or higher on the ETDQ‐7. Of the 16 (22%) patients who had both autophony and an ETDQ‐7 score of 14.5 or higher, 7 patient s (representing 11 symptomatic ears) underwent evaluations by audiology and otolaryngology. Every evaluation of a symptomatic ear revealed objective evidence of PET. Nine of 11 (81.8%) symptomatic ears had subjectively resolved within 12 days of admission after nutritional rehabilitation and weight gain. Conclusion Transient autophony in severe AN patients is due to PET, and was present in at least 8% of patients within our cohort. Further study is warranted to understand the quality of life impact and pathophysiology of transient PET in this patient population.

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. 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. 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. 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.

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. 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. 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). 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.

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.

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.

Objectives: To investigate novel variants in hearing loss genes and clinical factors affecting audiometric outcomes of cochlear implant (CI) patients. Background: Approximately 50% of hearing loss has a genetic etiology, with certain genetic variants more prevalent in specific ethnic groups. Different variants and some clinical variables including inner ear malformations result in different prognoses or clinical outcomes after CI. Methods: Medical and genetic testing records of pediatric CI patients were reviewed for clinical variables. Minor allele frequencies of variants were obtained from Genome Aggregation Database (gnomAD) and variants were classified for pathogenicity. Standard statistical testing was done using Fisher's exact, Wilcoxon, and Spearman correlation tests. Results: Eighteen CI patients with genetic test results had pathogenic variants, including six patients with syndromic hearing loss and six patients with known GJB2 variants. Novel pathogenic variants were noted in CHD7, ADGRV1, and ARID1B, with variants in the latter two genes identified in Hispanic patients. Overall, carriage of genetic variants was associated with better pre-CI audiometric thresholds at 2000 Hz (p = 0.048). On the other hand, post-CI thresholds were significantly worse in patients with inner ear malformations, particularly in patients with atretic cochlear nerve canals. Conclusion: Four novel pathogenic variants were identified, which contributes to knowledge of allelic spectrum for hearing loss especially in Hispanic patients. In this cohort, carriage of pathogenic variants particularly of GJB2 variants was associated with better pre-CI audiometric thresholds, while patients with inner ear malformations had worse post-CI audiometric thresholds.

Objectives: To compare contralateral to ipsilateral stimulation with percutaneous and transcutaneous bone conduction implants. Background: Bone conduction implants (BCIs) effectively treat conductive and mixed hearing losses. In some cases, such as in single-sided deafness, the BCI is implanted contralateral to the remaining healthy ear in an attempt to restore some of the benefits provided by binaural hearing. While the benefit of contralateral stimulation has been shown in at least some patients, it is not clear what cues or mechanisms contribute to this function. Previous studies have investigated the motion of the ossicular chain, skull, and round window in response to bone vibration. Here, we extend those reports by reporting simultaneous measurements of cochlear promontory velocity and intracochlear pressures during bone conduction stimulation with two common BCI attachments, and directly compare ipsilateral to contralateral stimulation. Methods: Fresh-frozen whole human heads were prepared bilaterally with mastoidectomies. Intracochlear pressure (P-IC) in the scala vestibuli (P-SV) and tympani (P-ST) was measured with fiber optic pressure probes concurrently with cochlear promontory velocity (V-Prom) via laser Doppler vibrometry during stimulation provided with a closed-field loudspeaker or a BCI. Stimuli were pure tones between 120 and 10,240 Hz, and response magnitudes and phases for P-IC and V-Prom were measured for air and bone conducted sound presentation. Results: Contralateral stimulation produced lower response magnitudes and longer delays than ipsilateral in all measures, particularly for high-frequency stimulation. Contralateral response magnitudes were lower than ipsilateral response magnitudes by up to 10 to 15 dB above 2 kHz for a skin-penetrating abutment, which increased to 25 to 30 dB and extended to lower frequencies when applied with a transcutaneous (skin drive) attachment. Conclusions: Transcranial attenuation and delay suggest that ipsilateral stimulation will be dominant for frequencies over 1 kHz, and that complex phase interactions will occur during bilateral or bimodal stimulation. These effects indicate a mechanism by which bilateral users could gain some bilateral advantage.

Background: Combined electrical-acoustical stimulation (EAS) has gained popularity as patients with residual hearing are increasingly undergoing cochlear implantation. Preservation of residual hearing correlates with hearing outcomes, but loss of hearing occurs in a subset of these patients. Several mechanisms have been proposed as causing this hearing loss; we have previously described high amplitude pressure transients, equivalent to high-level noise exposures, in the inner ear during electrode insertion. The source of these transients has not been identified. Methods: Cadaveric human heads were prepared with an extended facial recess. Fiber-optic pressure sensors were inserted into the scala vestibuli and scala tympani to measure intracochlear pressures. Two cochlear implant (CI) electrode styles (straight and perimodiolar) were inserted during time-synced intracochlear pressures and video fluoroscopy measurements. Results: CI electrode insertions produced pressure transients in the cochlea up to 160 to 170 dB pSPL equivalent for both styles, consistent with previous results. However, the position of the electrode within the cochlea when transients were generated differed (particularly contact with the medial or lateral walls). Conclusions: These results begin to elucidate the insertion pressure profiles of CI electrodes, which can be used to improve CI electrode designs and facilitate "silent-insertions" to improve chances of hearing preservation.

Previous genetic studies on susceptibility to otitis media and airway infections have focused on immune pathways acting within the local mucosal epithelium, and outside of allergic rhinitis and asthma, limited studies exist on the overlaps at the gene, pathway or network level between the upper and lower airways. In this report, we compared [1] pathways identified from network analysis using genes derived from published genome-wide family-based and association studies for otitis media, sinusitis, and lung phenotypes, to [2] pathways identified using differentially expressed genes from RNA-sequence data from lower airway, sinus, and middle ear tissues, in particular cholesteatoma tissue compared to middle ear mucosa. For otitis media, a large number of genes (n = 1,806) were identified as differentially expressed between cholesteatoma and middle ear mucosa, which in turn led to the identification of 68 pathways that are enriched in cholesteatoma. Two differentially expressed genes CR1 and SAA1 overlap in middle ear, sinus, and lower airway samples and are potentially novel genes for otitis media susceptibility. In addition, 56 genes were differentially expressed in both tissues from the middle ear and either sinus or lower airways. Pathways that are common in upper and lower airway diseases, whether from published DNA studies or from our RNA-sequencing analyses, include chromatin organization/remodeling, endocytosis, immune system process, protein folding, and viral process. Taken together, our findings from genetic susceptibility and differential tissue expression studies support the hypothesis that the unified airway theory wherein the upper and lower respiratory tracts act as an integrated unit also applies to infectious and nonallergic airway epithelial disease. Our results may be used as reference for identification of genes or pathways that are relevant to upper and lower airways, whether common across sites, or unique to each disease.

Objective: Current clinical practice for fixation of the cochlear implant receiver-stimulator (RS) may not follow recommended manufacturer's guidelines. We investigated short-and long-term RS migration using a subperiosteal pocket technique via serial objective position measurements since previous literature provided only subjective or short-term evaluation. Study Design: Retrospective review. Setting: Tertiary referral center. Patients: We reviewed all patients who underwent cochlear implantation by the senior author between 2012 and 2018. At least two comparison measurements were available for 73 implants in 62 patients, 72.6% adults and 27.4% children. Interventions: RS placement using a subperiosteal pocket technique. Main Outcome Measure: Distance between the pinna and RS magnet in the early (<6 mo) and late (>6 mo) postoperative period. Results: In the early postoperative period, mean RS distance was 57.0mm (SD 9.4mm) from the pinna compared with baseline intraoperative distance of 55.8mm (SD 8.4 mm), p = 0.44. With some shifts closer and some farther from the pinna, there was a 3.0mm mean absolute value migration (median 2mm, SD 3.8mm, range 0-15 mm). Fourteen implants (25.9%) migrated >5mm in the early period, 35.7% closer, and 64.3% farther from pinna. In the late postoperative period, mean RS final distance was 56.6mm (SD 10.1 mm), compared with its baseline of 59.0mm (SD 9.2mm), p = 0.29. Seven implants (19.4%) demonstrated shifts >5mm, with a mean 3.6mm shift (median 2mm, SD 4.5mm, range 0-17mm). Conclusion: A subperiosteal pocket technique demonstrated objective RS migration in 20 to 25% of adult and pediatric patients, nearly all of which were not clinically apparent unless measured. Additionally, none of these patients experienced associated symptoms or device failures.

Objective To compare outcomes for patients undergoing a transmastoid approach versus a middle fossa craniotomy approach with plugging and/or resurfacing for repair of superior semicircular canal dehiscence. Outcome measures include symptom resolution, hearing, operative time, hospital stay, complications, and revision rates. Study Design Multicenter retrospective comparative cohort study. Settings Three tertiary neurotology centers. Subjects and Methods All adult patients undergoing repair for superior canal dehiscence between 2006 and 2017 at 3 neurotology centers were included. Demographics and otologic history collected by chart review. Imaging, audiometric data, and vestibular evoked myogenic potential measurements were also collected for analysis. Results A total of 68 patients (74 ears) were included in the study. Twenty-one patients underwent middle fossa craniotomy repair (mean age, 47.9 years), and 47 underwent transmastoid repair (mean age, 48.0 years). There were no significant differences in age or sex distribution between the groups. The transmastoid group experienced a significantly shorter duration of hospitalization and lower recurrence rate as compared with the middle fossa craniotomy group (3.8% vs 33%). Both groups experienced improvement in noise-induced vertigo, autophony, pulsatile tinnitus, and nonspecific vertigo. There was no significant difference among symptom resolution between groups. Additionally, there was no significant difference in audiometric outcomes between the groups. Conclusion Both the transmastoid approach and the middle fossa craniotomy approach for repair of superior canal dehiscence offer symptom resolution with minimal risk. The transmastoid approach was associated with shorter hospital stays and lower recurrence rate as compared with the middle fossa craniotomy approach.

A genetic basis for otitis media is established, however, the role of rare variants in disease etiology is largely unknown. Previously a duplication variant within A2ML1 was identified as a significant risk factor for otitis media in an indigenous Filipino population and in US children. In this report exome and Sanger sequencing was performed using DNA samples from the indigenous Filipino population, Filipino cochlear implantees, US probands, Finnish, and Pakistani families with otitis media. Sixteen novel, damaging A2ML1 variants identified in otitis media patients were rare or low-frequency in population-matched controls. In the indigenous population, both gingivitis and A2ML1 variants including the known duplication variant and the novel splice variant c.4061 + 1 G>C were independently associated with otitis media. Sequencing of salivary RNA samples from indigenous Filipinos demonstrated lower A2ML1 expression according to the carriage of A2ML1 variants. Sequencing of additional salivary RNA samples from US patients with otitis media revealed differentially expressed genes that are highly correlated with A2ML1 expression levels. In particular, RND3 is upregulated in both A2ML1 variant carriers and high-A2ML1 expressors. These findings support a role for A2ML1 in keratinocyte differentiation within the middle ear as part of otitis media pathology and the potential application of ROCK inhibition in otitis media.