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Objective: Enlargement of the vestibular aqueduct (EVA) is one of the most common congenital malformations in pediatric patients presenting with sensorineural or mixed hearing loss. The relationship between vestibular aqueduct (VA) morphology and hearing loss across sex is not well characterized. This study assesses VA morphology and frequency-specific hearing thresholds with sex as the primary predictor of interest., Materials and Methods: A retrospective, longitudinal, and repeated-measures study was used. 47 patients at an academic tertiary care center with hearing loss and a record of CT scan of the internal auditory canal were candidates, and included upon meeting EVA criteria after confirmatory measurements of vestibular aqueduct midpoint and operculum widths. Audiometric measures included pure-tone average and frequency-specific thresholds., Results: Of the 47 patients (23 female and 24 male), 79 total ears were affected by EVA; the median age at diagnosis was 6.60 years. After comparing morphological measurements between sexes, ears from female patients were observed to have a greater average operculum width (3.25 vs. 2.70 mm for males, p = 0.006) and a greater average VA midpoint width (2.80 vs. 1.90 mm for males, p = 0.004). After adjusting for morphology, male patients' ears had pure-tone average thresholds 17.6 dB greater than female patients' ears (95% CI, 3.8 to 31.3 dB)., Conclusions: Though females seem to have greater enlargement of the vestibular aqueduct, this difference does not extend to hearing loss. Therefore, our results indicate that criteria for EVA diagnoses may benefit from re-evaluation. Further exploration into morphological and audiometric discrepancies across sex may help inform both clinician and patient expectations., (Copyright © 2019 Elsevier Inc. All rights reserved.)

Objectives: To evaluate the long-term efficacy of endolymphatic sac shunt techniques with and without local steroid administration., Study Design: Retrospective case series and patient survey., Setting: Tertiary university hospital., Patients: Meniere's disease (MD) patients that failed medical therapy and subsequently underwent an endolymphatic sac shunt procedure. All patients had definitive or probable MD and at least 18-months of follow-up., Interventions: Three variations on endolymphatic sac decompression with shunt placement were performed: Group A received no local steroids, Group B received intratympanic dexamethasone prior to incision, and Group C received dexamethasone via both intratympanic injection and direct endolymphatic sac instillation., Main Outcome Measure(s): Vertigo control, hearing results, and survey responses., Results: Between 2002 and 2013, 124 patients with MD underwent endolymphatic sac decompression with shunt placement. 53 patients met inclusion criteria. Groups A, B, and C had 6 patients, 20 patients, and 27 patients, respectively. Mean follow-up was 56months. Vertigo control improved in 66%, 83%, and 93% of Groups A, B, and C. Functional level improved for Group B (-2.0) and Group C (-2.2) but was unchanged in Group A. Pure-tone average and speech discrimination scores changed by +22dB and -30%, +6dB and -13%, and +6dB and -5% in Groups A, B, and C. The long-term hearing results were significantly better with steroids (Groups B and C) according to the AAO-HNS 1995 criteria but did not meet significance on non-parametric testing., Conclusions: Endolymphatic sac shunt procedures may benefit from steroid instillation at the time of shunt placement., (Copyright © 2017 Elsevier Inc. All rights reserved.)

Hypothesis: Phosphorus and vitamin D (calcitriol) supplementation in the Phex mouse, a murine model for endolymphatic hydrops (ELH), will improve otic capsule mineralization and secondarily ameliorate the postnatal development of ELH and sensorineural hearing loss (SNHL)., Background: Male Phex mice have X-linked hypophosphatemic rickets (XLH), which includes osteomalacia of the otic capsule. The treatment for XLH is supplementation with phosphorus and calcitriol. The effect of this treatment has never been studied on otic capsule bone and it is unclear if improving the otic capsule bone could impact the mice's postnatal development of ELH and SNHL., Methods: Four cohorts were studied: 1) wild-type control, 2) Phex control, 3) Phex prevention, and 4) Phex rescue. The control groups were not given any dietary supplementation. The Phex prevention group was supplemented with phosphorus added to its drinking water and intraperitoneal calcitriol from postnatal day (P) 7-P40. The Phex rescue group was also supplemented with phosphorus and calcium but only from P20 to P40. At P40, all mice underwent auditory brainstem response (ABR) testing, serum analysis, and temporal bone histologic analysis. Primary outcome was otic capsule mineralization. Secondary outcomes were degree of SNHL and presence ELH., Results: Both treatment groups had markedly improved otic capsule mineralization with less osteoid deposition. The improved otic capsule mineralized did not prevent the development of ELH or SNHL., Conclusion: Supplementation with phosphorus and calcitriol improves otic capsule bone morphology in the Phex male mouse but does not alter development of ELH or SNHL., (Copyright © 2016 Elsevier Inc. All rights reserved.)

IgG4-related disease (IgG4-RD) is increasingly being recognized as an entity effecting the head and neck region. Although most commonly seen with salivary gland or paranasal sinus involvement, IgG4-RD may also involve the temporal bone and skull base. We report a rare care of a 61-year-old female with IgG4-RD presenting as synchronous lesions of the middle ear and middle cranial fossa with polyneuropathy of cranial nerves II, VI, and VII. Initial histopathological evaluation of her resected ear mass suggested a benign inflammatory process but no specific diagnosis. Her symptoms progressed over 10months prompting re-evaluation of the specimen and consideration of the IgG4-RD diagnosis. Key pathologic features included prominent lymphoplasmacytic population, storiform fibrosis, obliterative phlebitis, and IgG4 specific staining. The patient was treated with high-dose intravenous and oral steroids but was transitioned to azathioprine secondary to steroid-induced myopathy. Radiographic studies before and after treatment reveal marked improvement of the intracranial and extracranial disease. Correspondingly, her cranial neuropathies resolved. A high degree of clinical suspicion is necessary to diagnosis IgG4-RD. The diagnosis can be supported by elevated serum IgG, elevated IgG index, and pathognomonic histopathological findings. Primary treatment is with corticosteroids. However, immunotherapy using azathioprine or rituximab can be utilized in recurrent disease or patients with steroid intolerance., (Copyright © 2016 Elsevier Inc. All rights reserved.)

Cerebral venous sinus thrombosis (CVST) is a rare complication of surgical treatment of vestibular schwanomma. We present a rare case of extensive venous sinus thrombosis after trans-labyrinthine approach that was refractory to systemic anti-coagulation. Mechanical aspiration thrombectomy was utilized to re-canalize the venous sinuses and resulted in successful resolution of neurological symptoms. Indications of utilizing endovascular approaches are discussed that will enable skull base surgeons to address this uncommon yet potentially fatal complication., (Copyright © 2016 Elsevier Inc. All rights reserved.)

Profound unilateral sensorineural hearing loss is an indication for the placement of a bone anchored hearing aid. In a few unfortunate patients who later develop contralateral hearing loss, a cochlear implant becomes a good option. We present our experience in these cases and discuss our technique for single stage conversion from a bone anchored hearing aid to a cochlear implant., (Copyright © 2015 Elsevier Inc. All rights reserved.)

Purpose: To compare hearing outcomes in patients with connexin 26 (Cx 26) mutations undergoing cochlear implantation to age matched controls and to examine whether age at implantation, gender and type of mutation were correlated with hearing outcome., Materials and Methods: Retrospective chart review of 21 patients with Cx 26 mutations that underwent cochlear implantation compared to 18 age-matched controls. Patients' characteristics, type of mutation and pre- and postoperative short and long-term hearing thresholds, word and sentence scores were analyzed., Results: There was no statistically significant difference between the Cx 26 and control group in the mean short term and mean long term post-operative pure tone averages (PTA), speech reception thresholds (SRT), word and sentence scores. Gender, age at implantation and type of connexin 26 mutation did not predict hearing outcomes., Conclusions: In patients with connexin 26 mutation, cochlear implantation provides an effective mean of auditory habilitation. Mutational status, age and gender do not seem to predict hearing outcomes., (Copyright © 2015 Elsevier Inc. All rights reserved.)

Congenital labyrinthine cholesteatoma can cause varying degrees of hearing loss and vertigo. Hearing preservation is not always possible when treatment involves total labyrinthectomy. Since 1969, there have been several case reports and case series documenting hearing preservation following surgical treatment of congenital labyrinthine cholesteatoma. However, none of the case reports or case series documents the patients' vestibular complaints after surgery. Herein, we report a case series of four patients who were treated with the goal of hearing preservation and resolution of vertigo after partial or complete removal of the bony labyrinth for congenital inner ear cholesteatoma. We will also discuss possible explanations for the mechanisms of preservation of hearing and vestibular function with a review of the current literature., (Copyright © 2014 Elsevier Inc. All rights reserved.)

Purpose: A spontaneous meningoencephalocele of the temporal bone may present with effusion in the middle ear, a cerebrospinal fluid leak, hearing loss, or rarely otitic meningitis. Repair of spontaneous encephaloceles in the temporal bone has been performed using transmastoid and transcranial middle fossa approaches or a combination of the two with varied results. The authors present a technical paper on the transmastoid extradural intracranial approach for the management of temporal lobe encephaloceles., Materials/methods: Case reports and cadaver dissections are used to provide a pictorial essay on the technique. Advantages and disadvantages compared with alternative surgical approaches are discussed., Results: Traditional transmastoid approaches are less morbid compared with a transcranial repair as they avoid brain retraction. However, in the past, there has been a higher risk of graft failure and hearing loss due to downward graft migration and a potential need for ossicular disarticulation. For the appropriate lesion, the transmastoid extradural intracranial approach lesion offers a stable meningoencephalocele repair without the comorbidity of brain retraction., Conclusion: The authors describe a transmastoid extradural intracranial technique via case reports and cadaver dissections for the repair of spontaneous meningoencephalocele defects larger than 2 cm. This approach provides more support to the graft compared to the conventional transmastoid repair., (Copyright © 2013 Elsevier Inc. All rights reserved.)

Objective: Establish the time to safely and efficiently perform cochlear implantation (CI) in a university-based academic center., Study Design: Case series with chart review., Setting: Academic neurotologic referral center., Patients: 424 patients who underwent CI surgery between 2002 and 2010., Intervention: Unilateral, bilateral or revision CI using commercially available devices approved for use in the United States., Main Outcome Measures: mean surgical duration (SD) and mean total operative room time (TORT)., Results: Overall mean SD for all 424 patients was 83 ± 30 min (min) whereas the mean TORT was 135 ± 56 min. The mean SD for unilateral CI was 84 ± 18 min for the first implant and 82 ± 22 min for the second implant (p=0.55). The SD for primary and revision CI was 83 ± 18 min and 85 ± 36 min, respectively (p=0.51). The mean SD for pediatric and adult CI was 83 ± 21 min and 83 ± 18 min, respectively (p=0.92). The mean SD without resident assistance was 74 ± 14 min whereas with the assistance of a resident the mean SD was 84 ± 20 min (p=0.02). When ossification was encountered the mean SD was 90 ± 32 min compared to 82 ± 19 min when absent (p<0.001). An association was found between TORT or SD, and the year of surgery, presence of ossification and the involvement of an assistant., Conclusion: In a university-based academic center, CI surgery can be safely and efficiently performed, supporting future cost-effectiveness analysis of its current practice., (Published by Elsevier Inc.)

Objectives: To compare hearing outcomes in patients with far advanced otosclerosis (FAO) undergoing cochlear implantation to an age-matched group of controls, to describe the effects of cochlear ossification on hearing, and to review the adverse effects of implantation in patients with FAO., Hypothesis: Hearing performance in patients with FAO after cochlear implantation is comparable to similarly treated postlingually deafened adults without FAO. Ossification or retrofenestral otosclerosis does not predict poor hearing outcomes. Modiolar-hugging technology reduces postoperative facial nerve stimulation., Study Design: Retrospective chart review., Setting: Academic neurotologic tertiary referral center., Patients: Thirty patients with FAO, who metaudiological criteria for cochlear implantation, were compared to 30 age-matched controls, postlingually deafened by non-otosclerotic causes., Main Outcome Measures: Audiometric pre- and postoperative speech reception threshold, word, and sentence scores were analyzed. The presence of retrofenestral findings on computed tomography or intraoperative cochlear ossification were noted., Results: In the FAO group, radiographic abnormalities were noted in 26.4% of patients. Intraoperative ossification requiring drillout was seen in 29.4% of patients. None developed postoperative facial nerve stimulation. There was no difference between the FAO and control groups in the mean short-term and long-term postoperative speech reception threshold, word, and sentence scores (P = .77). The presence of radiographic abnormalities did not predict hearing outcome. Intraoperative cochlear ossification was not associated with worse short-term word and sentence scores (P = .58 and 0.79, respectively), and for the long-term hearing outcome (P = .24)., Conclusions: In patients with FAO, effective and safe hearing rehabilitation can be accomplished with cochlear implantation., (Copyright © 2012 Elsevier Inc. All rights reserved.)

Subcutaneous emphysema of the head and neck after otologic surgery is exceedingly rare. The mechanism relates to the intimate relationship of the temporomandibular joint to the external auditory canal. We present a rare case of subcutaneous emphysema after otologic surgery for ear disease. An astute clinical index of suspicion coupled with prompt treatment can help minimize morbidity and improve patient outcomes., (Copyright © 2012 Elsevier Inc. All rights reserved.)

We describe the case of a 28-year-old woman who presented with an acute dense left facial paralysis. Magnetic resonance imaging demonstrated enhancement of the labyrinthine portion of the facial nerve, and Bell's palsy was the presumed initial diagnosis. After 2 months without recovery despite receiving steroid and antiviral therapy, the patient underwent further workup. Computed tomographic scan demonstrated a mass lesion adjacent to the tympanic portion of the facial nerve, and electromyography showed active denervation and prominent fibrillation potentials. Surgical excision of the tumor was performed with decompression and sparing of the facial nerve. Histologically, the tumor proved to be an inflammatory pseudotumor (IPT). At the 3-year follow-up, the patient had an improvement in her facial nerve function, progressing to a House-Brackman grade III. An IPT can masquerade as Bell's palsy with sudden complete facial paralysis. Failure to obtain even slight recovery in Bell's palsy should prompt further workup, including appropriate imaging, to assess for a mass lesion. Confusion of an IPT with a nerve-based tumor on frozen section and imaging could lead to inappropriate resection and cable grafting of the facial nerve. Therefore, the relationship between an IPT and facial nerve paralysis is vital and must be recognized for treatment and to maximize postoperative facial nerve function.

Purpose: To report an unusual case of an intracranial extension of Merkel cell carcinoma originating in the external ear canal and causing neurological deficits., Case Report: An 86-year-old woman, with a 16-month history of an external auditory canal mass, presented with hemiparesis, facial paralysis, and obtundation. Radiographic images showed an intracranial mass extending into the petrous bone., Method: The patient had a craniotomy for intracranial tumor resection with concurrent mastoidectomy for facial nerve decompression and obtundation and hemiparesis were resolved. Residual tumor was subsequently treated with adjuvant radiation therapy, and facial nerve function consequently improved., Conclusion: Merkel cell tumors rarely invade the intracranial compartments. Residual tumor and neurological deficits may respond to adjuvant radiation therapy.