Cochlear Implantation in Patients With Menière's Disease: Does Disease Activity Affect the Outcome?

OBJECTIVE: Menière's disease (MD) is characterized by episodes of vertigo, tinnitus, and sensorineural hearing loss. In the setting of bilateral deafness due to MD alone or contralateral pathology, cochlear implantation (CI) improves hearing. Active MD is characterized by fluctuating auditory symptoms and vertigo; whereas remittance of vertiginous symptoms and severe, permanent sensorineural hearing loss characterizes the inactive disease state. This study evaluates outcomes for MD patients compared with the general CI population and assesses if disease activity affects implant outcomes. STUDY DESIGN: Retrospective chart review. SETTING: Tertiary referral center. PATIENTS: Twenty-four patients with MD that received CI (7 active, 16 inactive, and 1 Probable Menière's), and 24 age-matched controls. INTERVENTIONS: Cochlear implantation. MAIN OUTCOME MEASURES: Word Recognition Score, Sentence Recognition Score (SRS), and Speech Reception Threshold. RESULTS: Best-aided preoperative and postoperative audiometric data were compared per ear between MD patients and controls and stratified by disease status using descriptive statistics with mixed-effects modeling. Patients with MD derived significantly more benefit from CI than controls when comparing differences between preoperative and postoperative levels for Word Recognition Score (12.2%, p = 0.0236), SRS (12.8%, p = 0.0375), and Speech Reception Threshold (-14.4 dB, p = 0.0188). Active disease status does not negatively impact CI outcomes and patients with active MD may benefit from greater gains in SRS (23.5%, p = 0.0107). CONCLUSIONS: CI provides greater gains in functional hearing for patients with MD compared with age-matched controls. Patients with active MD seem to perform better with respect to SRS following CI than patients with inactive status.

Long-term outcomes of endolymphatic sac shunting with local steroids for Meniere's disease.

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.

Successful endovascular management of venous sinus thrombosis complicating trans-labyrinthine removal of vestibular schwanomma.

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.

Bilateral Sequential Cochlear Implantation in Patients With Enlarged Vestibular Aqueduct (EVA) Syndrome.

OBJECTIVES: To analyze audiometric outcomes after bilateral cochlear implantation in patients with isolated enlarged vestibular aqueduct (EVA) syndrome and associated incomplete partition (IP) malformations. Secondary objective was to analyze rate of cerebrospinal fluid (CSF) gusher in patients with IP-EVA spectrum deformities and compare this with the existing literature. STUDY DESIGN: Retrospective chart review. METHODS: Thirty-two patients with EVA syndrome who received unilateral or bilateral cochlear implants between June 1999 and January 2014 were identified in the University Hospitals Case Medical Center cochlear implant database. Isolated EVA (IEVA) and Incomplete Partition Type II (IP-II) malformations were identified by reviewing high-resolution computed tomography (HRCT) imaging. Demographic information, age at implantation, surgical details, postimplantation audiometric data including speech reception thresholds (SRT), word, and sentence scores were reviewed and analyzed. Intra- and postoperative complications were analyzed as well and compared with the literature. RESULTS: Seventeen patients (32 implanted ears) had pediatric cochlear implantation for EVA-associated hearing loss. Data from 16 controls (32 implanted ears) were used to compare audiometric and speech outcomes of EVA cohort. Mean age at implantation was 6.8 years for EVA cohort and 6.0 years for controls. There was no statistically significant difference in long-term postoperative SRT, monaurally aided word scores, and binaurally tested word scores between pediatric EVA group and controls. The EVA patients had a long-term mean sentence score of 85.92%. A subset of EVA patients implanted at mean age of 3.18 years (n = 15 ears) had similar audiometric outcomes to another control group with Connexin 26 mutations (n = 20 ears) implanted at a similar age. Further subset analysis revealed no significant differences in age at implantation, SRT, and word scores in patients with IEVA and IP-II malformation. There was no significant association between size of vestibular aqueduct and age at implantation. There was no CSF gusher or other intra- or postoperative complications reported in our series. CONCLUSION: Bilateral sequential cochlear implantation can be performed safely in patients with EVA. Audiometric outcomes are excellent and comparable to pediatric cochlear implant patients with no malformations. CSF gusher rates can be minimized by trans-round window approach. Further long-term studies are needed to identify differences within IP-EVA spectrum deformities, audiometric outcomes, and proportions of EVA patients who will need cochlear implantation for hearing rehabilitation.

Retrosigmoid versus translabyrinthine approach to acoustic neuroma resection: A comparative cost-effectiveness analysis.

OBJECTIVES/HYPOTHESIS: Approach-specific economic data of acoustic neuroma (AN) resection is lacking. The purpose of this study was to analyze and compare adjusted total hospital costs, hospital and intensive care unit (ICU) length of stay (LOS), and associated factors in AN patients undergoing resection by translabyrinthine (TL) approach versus retrosigmoid (RS) approach. STUDY DESIGN: Retrospective chart review. METHODS: A total of 113 patients with AN undergoing TL (N = 43) or RS (N = 70) surgical resection between 1999 and 2012 were analyzed. Data including age, health status, preoperative hearing, tumor size, postoperative complications, hospital, ICU LOS, and disposition after discharge were collected from medical records and compared between both groups. Cost data was obtained from the hospital finance department and adjusted based on the Consumer Price Index for 2013. RESULTS: There were no significant differences in demographic data, preoperative hearing, preoperative health status, or postoperative complication rate. Total hospital LOS and ICU LOS were significantly longer in the RS compared to the TL group (4.3 ± 3.6 vs. 2.6 ± 1.1 days; P < 0.001, and 1.5 ± 1.1 vs. 1.0 ± 0.5 days; P = 0.015, respectively). Tumors were larger in RS compared to the TL group (2.1 ± 1.0 cm vs. 1.5 ± 0.7 cm, respectively; P = 0.002). When patients were stratified by tumor size < or ≥ 2 cm, the total hospital LOS remained greater in the RS group in both subgroups (< and ≥ 2 cm, P < 0.001, and P = 0.031, respectively). However, there was no difference in the total ICU LOS between both subgroups. The adjusted mean total hospital cost was higher in the RS compared to the TL group ($25,069 ± 14,968 vs. $16,799 ± 5,724; P < 0.001). The adjusted mean total hospital cost was greater in the RS group with tumor < 2 cm (P < 0.001) but not significantly different in patients with tumors ≥ 2 cm. Univariate analysis showed that greater tumor size, poorer preoperative health status, the presence of major postoperative complications, and the RS approach were independently significantly associated with higher total hospital LOS (P = 0.001, P = 0.009, P = 0.001, and P < 0.001, respectively) and a higher adjusted total hospital cost (P < 0.001, P = 0.002, P = 0.014, and P < 0.001, respectively). CONCLUSION: Hospital LOS and total adjusted costs are significantly less for patients undergoing translabyrinthine acoustic neuroma resection compared to the retrosigmoid approach. Many factors appear to influence these differences. Economic considerations in addition to tumor characteristics and surgeon preference should be considered in future acoustic neuroma resections. LEVEL OF EVIDENCE: 2c.

Ipsilateral single stage conversion from BAHA to cochlear implant.

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.

Endolymphatic sac tumors.

Endolymphatic sac tumors (ELST) are slow-growing, locally aggressive, low-grade malignancies that originate from the epithelium of the endolymphatic duct and sac. ELST often present with sensorineural hearing loss, tinnitus, and vertigo, which may mimic Meniere disease. Large tumors may present with additional cranial neuropathies. Management is primarily via microsurgical excision. Radiation therapy has a limited role for residual or unresectable disease. Early detection may enable hearing preservation techniques. ELST have an association with von Hippel-Lindau disease.

Predictive factors and outcomes of cochlear implantation in patients with connexin 26 mutation: a comparative study.

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.

A Genetic Murine Model of Endolymphatic Hydrops: The Phex Mouse.

Animal models of endolymphatic hydrops (ELH) provide critical insight into the pathophysiology of Meniere's disease (MD). A new genetic murine model, called the Phex mouse, circumvents prior need for a time and cost-intensive surgical procedure to create ELH. The Phex mouse model of ELH, which also has X-linked hypophosphatemic rickets, creates a postnatal, spontaneous, and progressive ELH whose phenotype has a predictable decline of vestibular and hearing function reminiscent of human MD. The Phex mouse enables real-time histopathologic analysis to assess diagnostic and therapeutic interventions as well as further our understanding of ELH's adverse effects. Already the model has validated electrocochleography and cervical vestibular evoked myogenic potential as useful diagnostic tools. New data on caspase activity in apoptosis of the spiral ganglion neurons may help target future therapeutic interventions. This paper highlights the development of the Phex mouse model and highlights its role in characterizing ELH.

Conservative management of vestibular schwannoma: expectations based on the length of the observation period.

OBJECTIVE: Evaluate long-term prevalence of tumor growth and need for further treatment in patients with a vestibular schwannoma treated with conservative management. STUDY DESIGN: Retrospective chart review. SETTING: Private neurotologic tertiary referral center. PATIENTS: Vestibular schwannoma patients undergoing conservative management and previously studied (N = 114). Mean time to last magnetic resonance imaging was 4.8 years and to last follow-up was 6.4 years (maximum, 18.5 yr). INTERVENTION: Serial gadolinium-enhanced magnetic resonance imaging with size measurement. MAIN OUTCOME MEASURES: Change in maximum tumor dimension of 2 mm or higher (growth), further treatment, audiologic measures-pure-tone average, word recognition, AAO-HNS (American Academy of Otolaryngology-Head and Neck Surgery) hearing class. RESULTS: Thirty-eight percent of tumors demonstrated growth; an average of 6.5 mm (SD, 3.8) at a mean rate of 3.1 mm per year. Of patients with no growth at 1 year or less, 20% grew by last follow-up. Overall, 31% had further treatment after a mean of 3.8 years (SD, 3.5; maximum, 18.5 yr). Of those followed for 5 to 10 years, 18% eventually had further treatment. Only 56% of growing tumors had further treatment by last follow-up; 14.8% with nongrowing tumors also had further treatment. Pure-tone average declined more in tumors that grew (mean Δ = 28.8 dB) than those that did not (mean Δ = 16.5 dB) (p ≤ 0.025), but there was no correlation between the amount of change in hearing and in the size of the tumor. Of patients with an initial AAO-HNS hearing Class A, 85.7% retained serviceable hearing. CONCLUSION: For patients electing an observation approach to treatment of vestibular schwannoma, about 31% may eventually undergo further treatment. Of those followed for 5 to 10 years, 18% eventually had further treatment. However, some patients are followed with radiologic evaluations for many years without necessity for further treatment.

A mouse model validates the utility of electrocochleography in verifying endolymphatic hydrops.

Endolymphatic hydrops (ELH) is a disorder of the inner ear that causes tinnitus, vertigo, and hearing loss. An elevated ratio of the summating potential (SP) to the action potential (AP) measured by electrocochleography has long been considered to be the electrophysiological correlate of ELH-related clinical conditions, such as Meniere's disease, but in vivo confirmation and correlation between an elevated SP/AP ratio and ELH has not yet been possible. Confirming this relationship will be important to show that elevated SP/AP ratio is indeed diagnostic of ELH. Here, we sought to confirm that an elevated SP/AP ratio is associated with ELH and test the hypothesis that severity of ELH and hearing loss would also correlate with the SP/AP ratio in vivo using the Phex(Hyp-Duk)/Y mouse model of postnatal ELH. In addition, we describe a minimally invasive approach for electrocochleography in mice. Auditory brainstem responses and electrocochleography data were collected from controls and Phex(Hyp-Duk)/Y mutants at postnatal day 21 and the mice (all male) were euthanized immediately for cochlear histology. Our results show that (1) the SP/AP ratio was significantly elevated in mice with histological ELH compared to controls, (2) the SP/AP ratio was not correlated with the severity of histological ELH or hearing loss, and (3) the severity of hearing loss correlated with the severity of histological ELH. Our study demonstrates that an elevated SP/AP ratio is diagnostic of ELH and that the severity of hearing loss is a better predictor of the severity of ELH than is the SP/AP ratio.

Hearing and vertigo outcomes after congenital labyrinthine cholesteatoma resection.

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.

Meningoencephalocele of the temporal bone: pictorial essay on transmastoid extradural-intracranial repair.

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.

Characterization of neuronal cell death in the spiral ganglia of a mouse model of endolymphatic hydrops.

HYPOTHESIS: Spiral ganglion neurons (SGN) in the Phex male mouse, a murine model of postnatal endolymphatic hydrops (ELH) undergo progressive deterioration reminiscent of human and other animal models of ELH with features suggesting apoptosis as an important mechanism. BACKGROUND: Histologic analysis of the mutant's cochlea demonstrates ELH by postnatal Day (P) 21 and SGN loss by P90. The SGN loss seems to occur in a consistent topographic pattern beginning at the cochlear apex. METHODS: SGN were counted at P60, P90, and P120. Semiquantitative reverse transcriptase-polymerase chain reaction (RT-PCR), quantitative PCR, and immunohistochemical analyses of activated caspase-3, caspase-8, and caspase-9 were performed on cochlear sections obtained from mutants and controls. Terminal deoxynucleotidyl transferase-mediated dUTP-biotin nick-end labeling assay (TUNEL) was carried out on 2 mutants and 2 controls. RESULTS: Corrected SGN counts in control mice were greater in the apical turn of the cochleae at P90 and P120, respectively (p < 0.01). Increased expression of activated caspase-3, caspase-8, and caspase-9 was seen in the mutant. At later time points, activated caspase expression gradually declined in the apical turns and increased in basal turns of the cochlea. Quantitative and semiquantitative PCR analysis confirmed increased expression of caspase-3, caspase-8, and caspase-9 at P21 and P40. TUNEL staining demonstrated apoptosis at P90 in the apical and basal turns of the mutant cochleae. CONCLUSION: SGN degeneration in the Phex /Y mouse seems to mimic patterns observed in other animals with ELH. Apoptosis plays an important role in the degeneration of the SGN in the Phex male mouse.

Surgical duration of cochlear implantation in an academic university-based practice.

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.

Prevention of postoperative cerebrospinal fluid leaks with multilayered reconstruction using titanium mesh-hydroxyapatite cement cranioplasty after translabyrinthine resection of acoustic neuroma.

OBJECT: Several prophylactic surgical methods have been tried to prevent CSF leakage after translabyrinthine resection of acoustic neuroma (TLAN). The authors report an improvised technique for multilayer watertight closure using titanium mesh-hydroxyapatite cement (HAC) cranioplasty in addition to dural substitute and abdominal fat graft after TLAN. METHODS: The study was limited to 42 patients who underwent TLAN at University Hospitals Case Medical Center using this new technique from 2006 to 2012. Systematic closure of the surgical wound in layers using temporalis fascia, dural substitute, dural sealant, adipose graft, titanium mesh, and then HAC was performed in each case. Temporalis muscle and eustachian tube obliteration were not used. The main variables studied were patient age, tumor size, tumor location, cosmetic outcome, length of hospitalization, and the incidence of CSF leak, pseudomeningocele, and infection. RESULTS: Excellent cosmetic outcome was achieved in all patients. There were no cases of postoperative CSF rhinorrhea, incisional CSF leak, or meningitis. Cosmetic results were comparable to those achieved using HAC alone. This cost-effective technique used only a third of the HAC required for traditional closure in which the entire mastoid defect is filled with cement, predisposing to infection. Postoperative CT and MRI showed excellent bony contouring and dural reconstitution, respectively. CONCLUSIONS: The authors report on successful use of titanium mesh-HAC cranioplasty in preventing postoperative CSF leak after TLAN in all cases in their series. The titanium mesh provides a well-defined anatomical dissection plane that would make reoperation easier than working through scarred soft tissue. The mesh bolsters the fat graft and keeps HAC out of direct contact with mastoid air cells, thereby reducing the risk of infection. The cement cranioplasty does not preclude subsequent implantation of a bone-anchored hearing aid.

Cochlear implantation outcomes in patients with far advanced otosclerosis.

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.

Hearing preservation in Guinea pigs with long-standing endolymphatic hydrops.

HYPOTHESIS: Interruption of the excitotoxic and inflammatory pathways implicated in endolymphatic hydrops (ELH)-associated hearing loss (HL) should afford hearing protection at the neuronal level. BACKGROUND: Previous work in our laboratory in the mouse model of ELH shows that dimethyl sulfoxide (DMSO), an anti-inflammatory solvent, can slow the progression of HL before neuronal degeneration occurs. Riluzole, a glutamate release inhibitor, may provide synergistic benefit. This study was designed to quantify the effects of DMSO and riluzole in a long-term model. METHODS: Guinea pigs with surgically induced ELH were sorted into 3 groups: riluzole+DMSO (Group 1), DMSO alone (Group 2), and untreated controls (Group 3). Animals in Groups 1 and 2 received daily injections of the study drug(s). All animals underwent auditory-evoked brainstem response evaluation every 4 weeks until 24 weeks, when they were sacrificed. Cochleae were preserved; spiral ganglion density was quantified. Animals without hydrops were excluded from the study as surgical failures. RESULTS: Animals from all groups developed unilateral HL. At the end of the experiment, HL was significantly lower in Group 1 relative to Group 3 (p = 0.049) and trended toward lower in Group 2 relative to Group 3 (p = 0.097). Groups 1 and 2 were not different (p = 0.311). At the cellular level, there is no evidence of neuronal degeneration in either treated group, whereas there is a significant neuronal degeneration in the untreated group. CONCLUSION: These results confirm the hearing protection observed with DMSO in short-term studies. However, unlike the previous study, which showed no additive benefit to riluzole, the combined treatment group in this study showed a hearing-protective effect at 24 weeks. This indicates a potential additive benefit conferred by riluzole toward long-term hearing protection. The study also finds evidence of statistically significant neuronal protection with both treatment groups. Overall, study provides additional evidence that DMSO and riluzole may preserve or slow the long-term progression of ELH-associated HL.

Transmastoid extradural-intracranial approach for repair of transtemporal meningoencephalocele: a review of 31 consecutive cases.

OBJECTIVE: To review the clinical presentation, surgical techniques, and outcomes of the transmastoid extradural-intracranial (TMEDIC) approach for the treatment of transtemporal meningoencephalocele. HYPOTHESIS: The TMEDIC is a safe and effective approach to repair meningoencephalocele originating from the middle or posterior cranial fossa. STUDY DESIGN: Retrospective chart review. SETTING: Academic neurotologic tertiary referral center. PATIENTS: Thirty-one consecutive patients diagnosed with transpetrous meningo(encephalo)cele, with or without cerebrospinal fluid leak, between January of 2003 and October of 2010. INTERVENTION: TMEDIC approach for repairing herniated neural tissue through the tegmen or posterior fossa plate using the combination of autologous cartilage, fascia, and tissue sealant. MAIN OUTCOME MEASURES: Anatomic location, size, and number of defects, presence of herniated brain tissue, pre- and postoperative hearing thresholds, and failure rate. RESULTS: Mean age was 62 ± 26 years. The etiology was spontaneous in 25/31 (80%), congenital in 3/31 (10%), chronic otitis media in 2/31 (6%), and posttraumatic in 1/31 (4%). Posttympanostomy tube clear otorrhea was the presenting sign in 21/31 (68%) of patients. The mean duration of symptoms was 26 months (range: 1-240). The defect involved the middle fossa (MF) floor in 25/31 (90%). Both the tegmen tympani and mastoideum were involved in 12/31 (39%) of patients and multiple dehiscences were seen in 7/31 (22%). In 17/31 (55%) of cases the size exceeded 1 cm. No recurrences were seen. CONCLUSION: The TMEDIC is a safe and effective method to repair transtemporal meningoencephalocele obviating the need for a middle fossa craniotomy in certain cases.

Ménière's disease: a challenging and relentless disorder.

Ménière's disease (MD) is characterized by episodic vertigo, fluctuating hearing loss and tinnitus, and by the presence of endolymphatic hydrops on postmortem examination. This disease continues to be a diagnostic and therapeutic challenge. Patients with MD range from minimally symptomatic, highly functional individuals to severely affected, disabled patients. Current management strategies are designed to control the acute and recurrent vestibulopathy but offer minimal remedy for the progressive cochlear dysfunction. Recent research highlights the role of neurotoxicity in the pathogenesis of the cochleovestibular deterioration. This article discusses a patient with MD, and provides an algorithm for the management of this disease.