Can Imaging Predict Hearing Outcomes in Children With Cochleovestibular Nerve Abnormalities?

OBJECTIVES/HYPOTHESIS: To identify the imaging characteristics associated with better hearing outcomes found in cochleovestibular nerve (CVN) abnormalities treated with hearing aids and/or cochlear implantation (CI). STUDY DESIGN: Retrospective review. METHODS: A retrospective review was undertaken of 69 ears with CVN abnormalities seen on magnetic resonance imaging (MRI) treated at a tertiary referral academic center analyzing the clinical features, imaging characteristics, and hearing data. We searched for associations among the hearing and imaging data, hypothesizing that the imaging data was not a good indicator of hearing function. RESULTS: In univariable analysis of all those who underwent aided testing (hearing aid and CI), health status (P = .016), internal auditory canal (IAC) midpoint diameter (P < .001), and number of nerves in the IAC (P < .001) were predictors of positive hearing outcome. Modiolar abnormalities, cochlear aperture diameter, cochlear malformations, vestibular malformations, and nerves in the cerebellar cistern did not predict hearing outcome (P = .79, .18, .59, .09, .17, respectively). For patients who received CI, health status (P = .018), IAC midpoint (P = .024), and number of nerves in the IAC (P = .038) were significant. When controlling for health status, IAC midpoint diameter (P < .001) and number of nerves in the IAC (P < .001) remained significant. In our cohort, one out of the eight ears (13%) with Birman class 0 or 1 exhibited responses to sound compared to nine out of 13 ears (70%) with Birman class 2-4. CONCLUSIONS: Current imaging modalities cannot accurately depict the status of the cochleovestibular nerve or predict a child's benefit with a CI. Cochlear implantation should be considered in children with abnormal cochleovestibular nerves. LEVEL OF EVIDENCE: 3 Laryngoscope, 132:S1-S15, 2022.

Exploring Factors Responsible for Delay in Pediatric Cochlear Implantation.

OBJECTIVE: To identify and characterize demographic and socioeconomic factors associated with delays in cochlear implantation (CI) in children. STUDY DESIGN: Retrospective. SETTING: Tertiary pediatric CI referral center. PATIENTS: All patients under 18 years of age receiving CI between March 2018 and February 2020. INTERVENTIONS: CI. MAIN OUTCOME MEASURES: Primary outcome measures included age at implantation and time from hearing loss diagnosis and candidacy evaluation to CI. RESULTS: Seventy-two patients were identified (44% women, average age at implantation 4.9 yr). Age at implantation was older in patients with public, rather than private, insurance (6.0 ±â€Š0.8 yr versus 3.1 ±â€Š0.7 yr, p = 0.007) and those from low-income areas (8.6 ±â€Š7.6 yr versus 2.4 ±â€Š3.0 yr, p = 0.007). Time between hearing loss diagnosis and implantation was longer in publicly insured patients (4.1 ±â€Š0.6 yr versus 2.2 ±â€Š0.5 yr, p = 0.014). Time between identification as a CI candidate and implantation was longer in publicly insured patients (721 ±â€Š107d versus 291 ±â€Š64 d, p = 0.001). Among children with congenital profound hearing loss, publicly insured patients continued to be older at implantation (1.9 ±â€Š0.2 versus 1.0 ±â€Š0.2 yr, p = 0.008). Latinx children were more often publicly insured whereas white children were more often privately insured (p < 0.05). Publicly insured patients had delays in the pre-CI workup, including, in no particular order, vestibular evaluation (621 ±â€Š132 d versus 197 ±â€Š67 d, p = 0.007), developmental evaluation (517 ±â€Š106 d versus 150 ±â€Š56 d, p = 0.003), speech evaluation (482 ±â€Š107 d versus 163 ±â€Š65 d, p = 0.013), and children's implant profile (ChIP) assessment (572 ±â€Š107d versus 184 ±â€Š59d, p = 0,002). On ChIP evaluation, concerns regarding educational environment and support were higher in Spanish-speaking children (p = 0.024; p = 2.6 × 10-4) and children with public insurance (p = 0.016; p = 0.002). CONCLUSIONS: Disparities in access to CI continue to affect timing of pediatric cochlear implantation.

Trend correlations for coastal eutrophication and its main local and whole-sea drivers - Application to the Baltic Sea.

Coastal eutrophication is a major environmental issue worldwide. In the Baltic Sea, eutrophication affects both the coastal waters and the open sea. Various policy frameworks aim to hinder its progress but eutrophication-relevant water quality variables, such as chlorophyll-a concentrations, still exhibit opposite temporal trends in various Baltic Sea marine and coastal waters. In this study, we investigate the temporal-trend linkages of measured water quality variables and their various anthropogenic, climatic and hydrospheric drivers over the period 1990-2020 with focus on the Swedish coastal waters and related marine basins in the Baltic Sea. We find that it is necessary to distinguish more and less isolated coastal waters, based on their water exchanges with the open sea, to capture different coastal eutrophication dynamics. In less isolated coastal waters, eutrophication is primarily related to nitrogen concentrations, while it is more related to phosphorus concentrations in more isolated coastal waters. In the open sea, trends in eutrophication conditions correlate best with trends in climatic and hydrospheric drivers, like wind speed and water salinity, respectively. In the coastal waters, driver signals are more mixed, with considerable influences from anthropogenic land-based nutrient loads and sea-ice cover duration. Summer chlorophyll-a concentration in the open sea stands out as a main change driver of summer chlorophyll-a concentration in less isolated coastal waters. Overall, coastal waters are a melting pot of driver influences over various scales, from local land-based drivers to large-scale total catchment and open sea conditions. The latter in turn depend on long-term integration of pathway-dependent influences from the various coastal parts of the Baltic Sea and their land-based nutrient load drivers, combined with overarching climate conditions and internal feedback loops. As such, our results challenge any unidirectional local source-to-sea paradigm and emphasize a need for concerted local land-catchment and whole-sea measures for robust coastal eutrophication management.

Factors Associated With Unilateral Hearing Loss and Impact on Communication in US Adults.

OBJECTIVE: To investigate the factors associated with unilateral hearing loss (UHL) and its impact on communication in US adults. STUDY DESIGN: Cross-sectional study. SETTING: Nationally representative sample of US adults. METHODS: We analyzed data from the 2011-2012 and 2015-2016 National Health and Nutritional Examination Survey, in which participants aged 20 to 69 years completed an audiometric evaluation (n = 8138). UHL was defined as a speech frequency pure-tone average ≥25 dB in the worse hearing ear and <25 dB in the better hearing ear. Logistic regression was used to examine the association between UHL and relevant factors. RESULTS: The prevalence of UHL was 8.1% (95% CI, 7.3%-9.0%) in US adults. Factors associated with UHL included older age, male sex, white race, lower level of education, diabetes, cardiovascular disease, and off-work noise exposure. Among adults with UHL, 40% (95% CI, 32%-48%) reported subjective trouble with hearing, a rate higher than the 12% (95% CI, 11%-14%) among normal-hearing adults. After adjusting for relevant factors, adults with UHL were more likely to report difficulties with following conversations with noise (odds ratio [OR], 1.7; 95% CI, 1.2-2.5) and frustration when talking to family and friends (OR, 3.0; 95% CI, 1.9-4.6). Higher levels of communication difficulties were observed with worsening level of UHL. CONCLUSIONS: Adults with UHL report significant communication difficulties in comparison to normal-hearing adults. Further research is needed to understand the psychosocial impact of UHL on adults and ways to improve communication support for adults with UHL.

Successful treatment of highly recurrent facial baroparesis in a frequent high-altitude traveler: a case report.

BACKGROUND: Facial baroparesis is a palsy of the seventh cranial nerve resulting from increased pressure compressing the nerve along its course through the middle ear cavity. It is a rare condition, most commonly reported in barotraumatic environments, in particular scuba diving and high-altitude air travel. We report here an unusual case of highly frequent baroparesis, workup, and successful treatment. CASE PRESENTATION: A 57-year-old Caucasian male frequent commercial airline traveler presented with a 4-year history of recurrent episodes of right-sided facial paralysis and otalgia, increasing in both frequency and severity. Incidents occurred almost exclusively during rapid altitude changes in aircraft, mostly ascent, but also during rapid altitude change in an automobile. Self-treatment included nasal and oral decongestants, nasal corticosteroids, and warm packs. Temporal bone computed tomography (CT) scan revealed possible right-sided dehiscence of the tympanic bone segment; audiogram and magnetic resonance imaging of the internal auditory canals were unremarkable. After a diagnosis of facial nerve baroparesis was made, the patient underwent myringotomy with insertion of a pressure equalization tube (PET) into the right tympanic membrane. Despite re-exposure to altitude change multiple times weekly post-treatment, the patient reported being symptom-free for more than 6 months following intervention. CONCLUSIONS: Prompt PET insertion may represent the preferred treatment for individuals who suffer recurrent episodes of facial baroparesis. Education regarding this rare condition may prevent unnecessary testing and treatment of affected patients. Future studies should explore the pathophysiology and risk factors, compare therapeutic options, and provide follow-up data to optimize the management of affected patients.

Abnormal cochleovestibular anatomy and imaging: Lack of consistency across quality of images, sequences obtained, and official reports.

OBJECTIVES: There are significant variations across centers on how to acquire and interpret imaging of children with congenital sensorineural hearing loss and cochleovestibular abnormalities. This study assesses the quality of imaging, sequences included, and accuracy of official radiology reports, to determine if these children are being assessed appropriately. METHODS: This study is retrospective review of CTs and MRIs from 40 pediatric patients diagnosed with profound sensorineural hearing loss and cochleovestibular structure/nerve abnormalities presenting to a tertiary referral academic center. Images were reviewed by two experienced neuroradiologists and a neurotologist. Findings were compared to official reports, when available. RESULTS: Twelve (30%) patients had an MRI only, while 28 (70%) had both an MRI and a CT. There were 3 (10.7%) CTs and 7 (17.5%) MRIs noted to be of poor quality. Children received an average of 6.8 (±2.7) CT acquisitions and 10.9 (±5.7) MRI acquisitions. There was non-concordance between the official report and expert review for 27 (71.1%) ears on CT and 27 (56.3%) ears on MRI. CONCLUSIONS: These data demonstrate high variability in protocols and quality of medical imaging of children with sensorineural hearing loss. Interpretation of images is highly discordant between official reports and tertiary review. Given these results, we recommend that these children be imaged and evaluated at centers with neuroradiologists who are experienced in interpreting congenital abnormalities of the cochleovestibular system.

Genes Implicated in Rare Congenital Inner Ear and Cochleovestibular Nerve Malformations.

OBJECTIVE: A small subset of children with congenital hearing loss have abnormal cochleovestibular nerves (i.e., absent, aplastic, or deficient cochlear nerves), with largely unknown etiology. Our objective was to investigate the underlying pathways and identify novel genetic variants responsible for cochleovestibular malformations and nerve abnormalities. It is our hypothesis that several cochleovestibular nerve abnormalities might share common causative pathways. DESIGN: We used a family-based exome sequencing approach to study 12 children with known rare inner ear and/or cochleovestibular nerve malformations. RESULTS: Our results highlight a diverse molecular etiology and suggest that genes important in the developing otic vesicle and cranial neural crest, e.g., MASP1, GREB1L, SIX1, TAF1, are likely to underlie inner ear and/or cochleovestibular nerve malformations. CONCLUSIONS: We show that several cochleovestibular nerve malformations are neurocristopathies, which is consistent with the fact that cochleovestibular nerve development is based on otic placode-derived neurons in close association with neural crest-derived glia cells. In addition, we suggest potential genetic markers for more severely affected phenotypes, which may help prognosticate individual cochlear implantation outcomes. Developing better strategies for identifying which children with abnormal nerves will benefit from a cochlear implantation is crucial, as outcomes are usually far less robust and extremely variable in this population, and current neuroimaging and electrophysiologic parameters cannot accurately predict outcomes. Identification of a suitable treatment early will reduce the use of multiple interventions during the time-sensitive period for language development.

A de novo SIX1 variant in a patient with a rare nonsyndromic cochleovestibular nerve abnormality, cochlear hypoplasia, and bilateral sensorineural hearing loss.

BACKGROUND: Childhood hearing impairment affects language and cognitive development. Profound congenital sensorineural hearing impairment can be due to an abnormal cochleovestibular nerve (CVN) and cochleovestibular malformations, however, the etiology of these conditions remains unclear. METHODS: We used a trio-based exome sequencing approach to unravel the underlying molecular etiology of a child with a rare nonsyndromic CVN abnormality and cochlear hypoplasia. Clinical and imaging data were also reviewed. RESULTS: We identified a de novo missense variant [p(Asn174Tyr)] in the DNA-binding Homeodomain of SIX1, a gene which previously has been associated with autosomal dominant hearing loss (ADHL) and branchio-oto-renal or Branchio-otic syndrome, a condition not seen in this patient. CONCLUSIONS: SIX1 has an important function in otic vesicle patterning during embryogenesis, and mice show several abnormalities to their inner ear including loss of inner ear innervation. Previous reports on patients with SIX1 variants lack imaging data and nonsyndromic AD cases were reported to have no inner ear malformations. In conclusion, we show that a de novo variant in SIX1 in a patient with sensorineural hearing loss leads to cochleovestibular malformations and abnormalities of the CVN, without any other abnormalities. Without proper interventions, severe to profound hearing loss is devastating to both education and social integration. Choosing the correct intervention can be challenging and a molecular diagnosis may adjust intervention and improve outcomes, especially for rare cases.

Standardization of the Punch Technique for the Implantation of Bone Anchored Auditory Devices: Evaluation of the MIPS Surgical Set.

OBJECTIVE: To describe and assess intraoperative and postoperative outcomes in the insertion of osseointegrated auditory implants with a newly designed surgical instrumentation set through a punch type technique. STUDY DESIGN: Retrospective case series. METHODS: Patients who underwent bone anchored auditory implant surgery using the Minimally Invasive Ponto Surgery (Oticon Medical, Somerset, NJ) surgical set through a punch technique at nine neurotology tertiary referral based practices were identified. Demographic data, skin thickness at implant site, implant used, duration of surgery, adverse intraoperative events, and postoperative outcomes were recorded. RESULTS: Seventy-five patients comprised the study cohort (32 males, 43 females). Most patients (57. 3%) were aged 51 to 75 years while 30.7% of the cohort comprised those aged 18 to 50 years and 12% were over 75 years. All but two patients received 4 mm fixtured implants and 68% received the Oticon Medical BioHelix implant. Two patients received 3 mm fixture implants and 32% received the Oticon Medical Wide Ponto implant. Mean surgical time was 12.2 minutes (6-45 min, standard deviation of 6.88 min). In three instances, surgery was converted to a linear incision to control brisk bleeding. Skin condition was Holgers 0 to 1 in 91.8%, while 5.5% had Holgers 2, and 2.7% had Holgers 3 at the first postoperative visit. At second postoperative visit, 94.3% had Holgers 0 to 1, 4.3% had Holgers 2, and 1.4% had Holgers 3. All instances of adverse skin reactions were treated with topical or systemic antibiotics and/or local debridement. There were no instances of implant loss. One patient had his implant traumatically displaced to a 45-degree angle necessitating implant replacement at a second site. CONCLUSION: Punch technique placement of osseointegrated auditory implants using the Minimally Invasive Ponto Surgery surgical set represents a safe technique that further simplifies a progressively minimally invasive surgery.

De novo variants in GREB1L are associated with non-syndromic inner ear malformations and deafness.

Congenital inner ear malformations affecting both the osseous and membranous labyrinth can have a devastating impact on hearing and language development. With the exception of an enlarged vestibular aqueduct, non-syndromic inner ear malformations are rare, and their underlying molecular biology has thus far remained understudied. To identify molecular factors that might be important in the developing inner ear, we adopted a family-based trio exome sequencing approach in young unrelated subjects with severe inner ear malformations. We identified two previously unreported de novo loss-of-function variants in GREB1L [c.4368G>T;p.(Glu1410fs) and c.982C>T;p.(Arg328*)] in two affected subjects with absent cochleae and eighth cranial nerve malformations. The cochlear aplasia in these affected subjects suggests that a developmental arrest or problem at a very early stage of inner ear development exists, e.g., during the otic pit formation. Craniofacial Greb1l RNA expression peaks in mice during this time frame (E8.5). It also peaks in the developing inner ear during E13-E16, after which it decreases in adulthood. The crucial function of Greb1l in craniofacial development is also evidenced in knockout mice, which develop severe craniofacial abnormalities. In addition, we show that Greb1l-/- zebrafish exhibit a loss of abnormal sensory epithelia innervation. An important role for Greb1l in sensory epithelia innervation development is supported by the eighth cranial nerve deficiencies seen in both affected subjects. In conclusion, we demonstrate that GREB1L is a key player in early inner ear and eighth cranial nerve development. Abnormalities in cochleovestibular anatomy can provide challenges for cochlear implantation. Combining a molecular diagnosis with imaging techniques might aid the development of individually tailored therapeutic interventions in the future.

Abnormal Cochleovestibular Anatomy and Hearing Outcomes: Pediatric Patients with a Questionable Cochleovestibular Nerve Status May Benefit from Cochlear Implantation and/or Hearing Aids.

OBJECTIVE: Imaging characteristics and hearing outcomes in children with cochleovestibular or cochleovestibular nerve (CVN) abnormalities. STUDY DESIGN: Retrospective, critical review. SETTING: Tertiary referral academic center. PATIENTS: Twenty-seven children with CVN abnormalities with magnetic resonance (MRI) and/or computed tomography (CT). Study Intervention(s): None. MAIN OUTCOME MEASURE(S): Determine the likely presence or absence of a CNV and auditory stimulation responses. RESULTS: Two of 27 cases had unilateral hearing loss, and all others had bilateral loss. Eleven (46%) were identified with a disability or additional condition. Twenty-two (42%) ears received a cochlear implant (CI) and 9 ears (17%) experienced no apparent benefit from the device. MRI acquisition protocols were suboptimal for identification of the nerve in 22 (42%) ears. A likely CVN absence was associated with a narrow cochlear aperture and internal auditory canal and cochlear malformation. Thirteen (48%) children with an abnormal nerve exhibited normal cochleae on the same side. Hearing data were available for 30 ears, and 25 ears (83%) exhibited hearing with or without an assistive device. One child achieved closed set speech recognition with a hearing aid, another with a CI. One child achieved open set speech recognition with a CI. CONCLUSIONS: Current imaging cannot accurately characterize the functional status of the CVN or predict an assistive device benefit. Children who would have otherwise been denied a CI exhibited auditory responses after implantation. A CI should be considered in children with abnormal CVN. Furthermore, imaging acquisition protocols need standardization for clear temporal bone imaging.

Prevalence of Hearing Loss and Hearing Care Use Among Asian Americans: A Nationally Representative Sample.

OBJECTIVE: To assess the prevalence of hearing loss and factors affecting hearing care use among Asian Americans, using the first nationally representative sample of Asian Americans. STUDY DESIGN: National cross-sectional survey. SETTING: Ambulatory examination centers. PATIENTS: Three thousand six hundred twelve adults (522 Asian American) aged 20 to 69 in the 2011 to 2012 National Health and Examination Survey with pure-tone audiometry. MAIN OUTCOME MEASURE(S): Percentage with hearing loss, undertaking a hearing test before the study, and hearing aid use. Hearing loss was defined as better hearing ear speech frequency pure-tone average ≥25 dBHL. Analyses incorporated sampling weights to account for complex sampling design. RESULTS: The prevalence of hearing loss was 6.0% [95% CI 3.1-8.9%] among Asian Americans, comparable to White, Black, and Hispanic groups, and increased substantially with age (OR: 2.25 [95% CI: 1.6-3.2]). After adjusting for age and pure-tone average, Asian Americans with hearing loss were less likely to have received a hearing test compared with White (OR: 0.27 [95% CI: 0.20-0.36, p = <0.001]) and Black groups (OR: 0.26 [95% CI: 0.16-0.38, p<0.001]), less likely to use hearing aids compared with Whites (OR: 0.06 [95% CI: 0.01-0.64], p = 0.02), and less likely to self-report poor hearing compared with Whites (OR: 0.30 [95% CI: 0.10-0.90], p = 0.03). Among Asian Americans, using more non-English than English, being foreign-born, less education, being married, and not having insurance were associated with lower levels of receiving a hearing test. CONCLUSION: The nationally representative sample of Asian Americans with hearing data suggests that hearing loss prevalence is similar to other races/ethnicities. However, hearing aid adoption by Asian Americans tends to be less frequent.

Evaluation of the increased use of partial resection of large vestibular schwanommas: facial nerve outcomes and recurrence/regrowth rates.

OBJECTIVE: To determine whether partial tumor removal in large vestibular schwannoma improves facial nerve outcomes while maintaining a low risk of tumor regrowth/recurrence. STUDY DESIGN: Retrospective chart review and prospective database. SETTING: Tertiary neurotologic referral center. PATIENTS: Four hundred patients with a vestibular schwannoma of 2.5 cm or greater in maximum diameter undergoing translabyrinthine microsurgical resection from 2001 to 2011. There were 325 gross total resections (GTR), 44 near total resections (NTR), and 31 subtotal resections (STR), with an overall mean tumor size of 3.2 cm (standard deviation, 0.7). INTERVENTION(S): Translabyrinthine surgical tumor resection. MAIN OUTCOME MEASURES: House-Brackmann (H-B) facial nerve grade postoperatively and at 1 year, tumor regrowth/recurrence (≥2 mm), additional treatment, and complications. RESULTS: Higher rates of H-B facial nerve Grades I and II were achieved at both the postoperative and 1-year follow-ups in the NTR (78%, 97%) and STR (71%, 96%) groups compared with GTR (53%, 77%) (p ≤ 0.001). Eye treatment, medical or surgical, was required more often in GTR (28.0%) than NTR and STR (8% and 21%, respectively, p ≤ 0.04), with no other differences in complications. The NTR and STR groups had a significantly higher rate of regrowth than GTR resection (21% and 22% versus 3%) (p ≤ 0.001) at average follow-up times of 3.7, 3.7, and 5.1 years, respectively, and need for further treatment occurred at a higher rate, although infrequently, in NTR and STR (2% and 10% versus 0%) (p ≤ 0.001). CONCLUSION: Near total and subtotal removal in large tumors are viable treatment options to maintain facial nerve function. During the follow-up period examined in this study, there was a low risk of need for further treatment. Longer-term follow-up is needed to better assess the need for retreatment in patients treated with NTR and STR.

Hearing preservation: microsurgery.

PURPOSE OF REVIEW: To evaluate the recent and significant contributions to the literature that examine hearing preservation outcomes and prognostic factors in vestibular schwannoma microsurgery. RECENT FINDINGS: Hearing preservation rates overall range considerably between 2 and 93% in recent studies. There are a number of factors that have been reported to be significant in the prediction of hearing preservation. Characteristics such as approach, results of preoperative neurophysiological testing, tumor size and nerve of origin have long been reported. A more recent contribution to the literature has included the association between MRI T2 signal in the fundus of the internal auditory canal and hearing preservation. This review provides a summary of some of the landmark studies in conjunction with more recent work detailing the prognostic factors for hearing preservation in the surgical management of vestibular schwannoma. SUMMARY: Hearing preservation in vestibular schwannoma surgery has undergone tremendous evolution over the past 50 years. In this review, we outline the prognostic factors that predict hearing preservation and describe recent contributions.

Comparison of traditional 2-dimensional endoscopic pituitary surgery with new 3-dimensional endoscopic technology: intraoperative and early postoperative factors.

BACKGROUND: Traditionally, endoscopic transsphenoidal pituitary surgery is performed using 2-dimensional (2D) endoscopes, which lack depth of field and contribute to image distortion. Recently, a new generation of 3D endoscopes has been introduced for improved endoscopic depth perception. Little data exist comparing surgical outcomes with 2D vs 3D endoscopic systems. This study examines perioperative and postoperative factors in patients undergoing pituitary surgery using 2D vs 3D endoscopes. The objective of this work was to determine the differences in perioperative and postoperative factors in patients undergoing pituitary surgery using 2D vs 3D endoscopy. METHODS: Retrospective chart review at a tertiary academic referral center. Statistical comparison was undertaken for perioperative (estimated blood loss, operative time) and postoperative factors (length of stay, complications, and readmission rate). RESULTS: A total of 58 patients underwent endoscopic pituitary surgery during the 24-month study period (22 functional, 36 nonfunctional lesions). The 2D endoscopic system was used for pituitary tumor resection in 32 patients (55%), and the 3D endoscopic system in 26 patients (45%). No significant difference existed between 2D and 3D endoscopic systems for operative time (p = 0.275) or estimated blood loss (p = 0.312). Additionally, no difference was found between groups for cerebrospinal fluid (CSF) leak rate (p = 0.581), postoperative endocrine complications (p = 0.081), length of hospital stay (p = 0.934), or hospital readmission rate (p = 1.0). CONCLUSION: 3D endoscopy affords the surgeon improved depth of field and stereoscopic vision. Our data demonstrate that 3D endoscopy does not result in significantly different perioperative or postoperative outcomes vs 2D endoscopic surgery.

Transtemporal management of temporal bone encephaloceles and CSF leaks: review of 56 consecutive patients.

CONCLUSIONS: This report details our experience in the management of 56 consecutive patients with mastoid encephaloceles and cerebrospinal fluid (CSF) leaks. The majority were managed through the transmastoid route with temporalis fascia and calvarial bone graft. Among the patients operated on at least 18 months before analysis, there was a median follow-up of 54 months with no intracranial complications. The average body mass index (BMI) of patients with spontaneous CSF leaks was found to be statistically significantly greater than that of patients with CSF leaks with traumatic, infectious or iatrogenic etiologies. OBJECTIVES: To investigate the long-term follow-up of patients with spontaneous, iatrogenic, and traumatic temporal bone encephaloceles and CSF leaks to determine the effectiveness of the repair, late intracranial complications, and recurrent lesions. METHODS: This is a retrospective review from a tertiary care center of 56 consecutive patients managed with CSF otorrhea, encephaloceles, and spontaneous pneumocephalus. All patients were repaired through transtemporal, middle cranial fossa or combined approaches for extradural repair and bone grafting. RESULTS: Of the 56 consecutive cases, 12 had CSF otorrhea alone, 19 had encephaloceles, and 23 had both. Two patients presented with spontaneous pneumocephalus without CSF leak. Thirty-three patients had spontaneous onset of their lesion without any history of temporal bone surgery or trauma. The average BMI of patients with spontaneous CSF leaks was found to be greater than the average BMI of patients with CSF leaks due to traumatic, infectious or iatrogenic causes. This difference was found to be statistically significant. Six of these had either preceding or subsequent contralateral mastoid or anterior fossa (sphenoid, ethmoid) CSF leak. Intracranial pressure was evaluated in these cases and most underwent VP shunting to reduce their intracranial pressure. Sixteen cases followed previous mastoid surgery or chronic ear disease and seven were attributed to previous temporal bone trauma. Lesions were repaired with the transtemporal approach (42), middle fossa (4), and combined (5) approaches. Five cases required petrosectomy and fat obliteration. There was one recurrent CSF leak 4 years after initial repair; the leak was anterior to the previously placed bone graft.

Treatment of sinus headache as migraine: the diagnostic utility of triptans.

OBJECTIVE: To determine the response rate to triptans in alleviating "sinus headache" in patients with endoscopy- and computed tomography (CT)-negative sinus examinations. STUDY DESIGN: Prospective clinical trial. METHODS: Patients who presented to a tertiary care center Otolaryngology Department with primary complaints of facial pain, pressure, or headache localized over the area of the sinuses, and a self- or physician-diagnosis of "sinus headache" were enrolled. Patients underwent directed history, physical examination, rigid nasal endoscopy, a sinus CT scan, and completed a headache questionnaire. Those patients that had negative findings were treated empirically with triptans. Patients completed a headache diary for each headache, using a visual analogue scale to rate the headache before and after triptan use. Response was considered as significant improvement (greater than 50% reduction of pain), partial (25-50% reduction), and no response. RESULTS: Fifty-four patients were enrolled. Thirty-eight (69%) completed follow-up, 63% were women. The mean age was 41 years (23-70). Thirty-one patients (82%) had significant reduction of headache pain with triptan use. Thirty-five patients (92%) had a significant reduction in headache pain in response to migraine-directed therapy. Seventeen patients (31%) withdrew or failed to follow-up, often reluctant to accept a diagnosis of migraine. CONCLUSIONS: This study demonstrated that the demographics of patients with self-described "sinus headaches" who did not have findings of sinusitis on endoscopy and CT scan closely reflected the demographics of patients afflicted with migraines. It also showed that 82% of these patients had a significant response to empiric treatment for sinus headaches with triptans. These findings support that "sinus headaches" may represent migraines, and response to triptans may aid in diagnosis.

Toll-like receptor 7-dependent loss of B cell tolerance in pathogenic autoantibody knockin mice.

Systemic lupus erythematosus (SLE) is characterized by the production of autoantibodies that are frequently directed against nucleic acid-associated antigens. To better understand how B cells reactive with such antigens are regulated, we generated a model system in which heavy and light chain genes encoding 564 immunoglobulin have been targeted to the heavy and light chain loci of the nonautoimmune C57BL/6 mouse strain. This antibody recognizes RNA, single-stranded DNA, and nucleosomes. We show that B cells expressing this immunoglobulin were activated, producing class-switched autoantibody in vivo despite the apparently normal induction of anergy. This autoantibody production was largely dependent on Toll-like receptor 7 (TLR7). We further show that production of these autoantibodies was sufficient to cause kidney pathology in these mice. These results demonstrate that the particular threat of nucleic acid-containing autoantigens lies in their ability to bind both antigen receptor and TLR7.