Promiscuous signaling by a regulatory system unique to the pandemic PMEN1 pneumococcal lineage.

Streptococcus pneumoniae (pneumococcus) is a leading cause of death and disease in children and elderly. Genetic variability among isolates from this species is high. These differences, often the product of gene loss or gene acquisition via horizontal gene transfer, can endow strains with new molecular pathways, diverse phenotypes, and ecological advantages. PMEN1 is a widespread and multidrug-resistant pneumococcal lineage. Using comparative genomics we have determined that a regulator-peptide signal transduction system, TprA2/PhrA2, was acquired by a PMEN1 ancestor and is encoded by the vast majority of strains in this lineage. We show that TprA2 is a negative regulator of a PMEN1-specific gene encoding a lanthionine-containing peptide (lcpA). The activity of TprA2 is modulated by its cognate peptide, PhrA2. Expression of phrA2 is density-dependent and its C-terminus relieves TprA2-mediated inhibition leading to expression of lcpA. In the pneumococcal mouse model with intranasal inoculation, TprA2 had no effect on nasopharyngeal colonization but was associated with decreased lung disease via its control of lcpA levels. Furthermore, the TprA2/PhrA2 system has integrated into the pneumococcal regulatory circuitry, as PhrA2 activates TprA/PhrA, a second regulator-peptide signal transduction system widespread among pneumococci. Extracellular PhrA2 can release TprA-mediated inhibition, activating expression of TprA-repressed genes in both PMEN1 cells as well as another pneumococcal lineage. Acquisition of TprA2/PhrA2 has provided PMEN1 isolates with a mechanism to promote commensalism over dissemination and control inter-strain gene regulation.

Comparison of Outcomes with the Use of Different Heat-Activated Crimping Prostheses in Stapedotomy.

OBJECTIVE: To determine differences in failure rate and hearing outcomes of a completely encircling heat-activated crimping prosthesis (SMart 360°) compared to partially encircling prosthesis (SMart). STUDY DESIGN: Retrospective chart review. SETTING: Private neurotology tertiary referral center. PATIENTS: Patients who underwent stapedotomies performed by the senior authors from 2008 to 2019 using the SMart prosthesis and SMart 360° prothesis. INTERVENTIONS: Stapedotomy operations with placement of a SMart or SMart 360° prosthesis. MAIN OUTCOME MEASURES: Incidence of early failure requiring revision surgery. Differences in preoperative air-bone gap (ABG) compared to postoperative ABG at 3 months, 1 year, and 2 years after surgery. RESULTS: A total of 228 stapedotomies were performed (SMart n = 48 and SMart 360° n = 180). Mean preoperative ABG for SMart and SMart 360° were 26.15 and 29 dB, respectively. The mean difference in ABG for the SMart at 3 months, 1 year, and 2 years were 17, 18, and 11 dB, respectively. The mean difference in ABG for the SMart 360° at 3 months, 1 year, and 2 years were 20, 20, and 19 dB. ABG differences at 3 months (p = 0.10) and 1 year (p = 0.36) were not statistically different. The failure rate for the SMart prosthesis was 12.5% and for the SMart 360° 2.2% (p = 0.002). CONCLUSIONS: There were no statistically significant differences in ABG changes for SMart compared to SMart 360°. The Smart 360 corrects the problem with early failure seen with the Smart prosthesis. PROFESSIONAL PRACTICE GAP AND EDUCATIONAL NEED: Determination of most efficacious stapes prosthesis. LEARNING OBJECTIVE: Which stapes prosthesis produces better hearing results with fewer failures. DESIRED RESULT: To disseminate information necessary to choose the best stapes prosthesis for patients. LEVEL OF EVIDENCE: Level III. INDICATE IRB OR IACUC: 2022-029-agh.

Outcomes of Mastoidectomy With Antibiotic Catheter Irrigation for Patients With Draining Ventilation Tubes.

OBJECTIVES: To evaluate the effectiveness of mastoidectomy with antibiotic catheter irrigation in patients with chronic tympanostomy tube otorrhea. METHODS: A chart review of adult and pediatric patients with persistent tympanostomy tube otorrhea who had failed outpatient medical management and underwent mastoidectomy with placement of a temporary indwelling catheter for antibiotic instillation was performed. Patients were retrospectively followed for recurrent drainage after 2 months and outcomes were categorized as resolution (0-1 episodes of otorrhea or otitis media with effusion during follow-up), improvement (2-3 episodes), or continued episodic (>3 episodes). RESULTS: There were 22 patients and 23 operated ears. Median age was 46 years (interquartile range, IQR = 29-65). The median duration of otorrhea from referral was 5.5 months (IQR = 2.8-12). Following surgery, 14 ears had resolution of drainage, 6 had improvement, and 3 had episodic. The observed percentage of resolved/improved ears (87%) was significant (P = .0005, 95% CI = 67.9%-95.5%). Median follow-up time was 25 months (IQR = 12-59). Pre and postoperative pure tone averages improved (difference of medians = -3.3 dB, P = .02) with no significant difference in word recognition scores (P = .68). Methicillin-resistant Staphylococcus aureus was the most common isolated microbe while no growth was most frequently noted on intraoperative cultures. CONCLUSIONS: Mastoidectomy with antibiotic catheter irrigation may be an effective surgical strategy, and single stage alternative to intravenous antibiotics, for select patients with persistent tube otorrhea who have failed topical and oral antibiotics.

Fibrin Glue-Coated Collagen Complex Patch: A Novel Material for Surgical Repair of Spontaneous Temporal Bone Cerebrospinal Fluid Leak and Encephalocele.

OBJECTIVE: To compare outcomes of surgical repair of temporal bone encephalocele and cerebrospinal fluid (CSF) leak using fibrin glue-coated collagen (FGCC) complex patch versus other materials for repair of dura. STUDY DESIGN: Retrospective chart review. SETTING: Tertiary care hospital. PATIENTS: Fifty-two adult patients undergoing transmastoid (TM), middle fossa (MF) or combined approach repair of spontaneous MF CSF leak between 2016 and 2020. INTERVENTIONS: Exposure of bony defect via TM approach, MF craniotomy, or combined TM/MF, and repair of the associated dura defect with FGCC complex patch, or other materials (acellular collagen matrix, bovine collagen, autologous fascia, fibrin tissue sealant). MAIN OUTCOME MEASURES: Successful repair without recurrent CSF leak or encephalocele throughout follow-up. Cost of materials used in duraplasty. RESULTS: Sixty-four percent of patients were female. Mean (standard deviation) age at repair was 61.4 (12.1) years. Mean (standard deviation) body mass index was 35.0 (8.3) kg/m 2 . Forty-nine (94%) patients had successful repair without known recurrence of CSF leak or encephalocele over a median follow-up interval of 11.7 months. Average duraplasty material cost was significantly lower with FGCC in comparison with other nonautologous materials (FGCC+: $1259.94, FGCC-: $1652.58; p = 0.004). No significant differences in recurrence risk (FGCC+: 6.9%, FGCC-: 6.9%; p > 0.999) or operative time (FGCC+: 153.7 min, FGCC-: 155.4 min; p = 0.88) were detected based on material used for duraplasty. CONCLUSIONS: All materials studied demonstrate effective and sustained means of repair for MF CSF leak and encephalocele, including in the presence of multiple defects. Use of FGCC for duraplasty produces noninferior surgical results to other nonautologous materials in repair of spontaneous CSF leaks of the temporal bone and may be more cost-effective.

A Deep Learning Model for Automated Classification of Intraoperative Continuous EMG.

OBJECTIVE: Intraoperative neurophysiological monitoring (IONM) is the use of electrophysiological methods during certain high-risk surgeries to assess the functional integrity of nerves in real time and alert the surgeon to prevent damage. However, the efficiency of IONM in current practice is limited by latency of verbal communications, inter-rater variability, and the subjective manner in which electrophysiological signals are described. METHODS: In an attempt to address these shortcomings, we investigate automated classification of free-running electromyogram (EMG) waveforms during IONM. We propose a hybrid model with a convolutional neural network (CNN) component and a long short-term memory (LSTM) component to better capture complicated EMG patterns under conditions of both electrical noise and movement artifacts. Moreover, a preprocessing pipeline based on data normalization is used to handle classification of data from multiple subjects. To investigate model robustness, we also analyze models under different methods for processing of artifacts. RESULTS: Compared with several benchmark modeling methods, CNN-LSTM performs best in classification, achieving accuracy of 89.54% and sensitivity of 94.23% in cross-patient evaluation. CONCLUSION: The CNN-LSTM model shows promise for automated classification of continuous EMG in IONM. SIGNIFICANCE: This technique has potential to improve surgical safety by reducing cognitive load and inter-rater variability.

The Role of Mastoidectomy in Draining Ventilation Tubes.

OBJECTIVES: To discuss the indication for performing a mastoidectomy with catheter placement in patients with chronic tympanostomy tube otorrhea. METHODS: The Medical Literature Analysis and Retrieval System Online was searched via PubMed for relevant articles using serous mastoiditis, mastoidectomy, chronic otorrhea, tube otorrhea, tympanostomy tubes, and biofilm as keywords. RESULTS: Further understanding of the pathophysiology of otorrhea and the development of ototopical fluoroquinolones have made a draining tympanostomy tube more manageable. Nevertheless, chronic otorrhea refractory to an otolaryngologist's traditional treatment algorithm still occurs and may benefit from a mastoidectomy with antibiotic irrigation using a catheter in certain cases. We theorize that resolution of otorrhea results from this technique by decreasing the burden of diseased mucosa and providing a larger concentration or dose of antibiotic to the middle ear cleft through the antrum. High-resolution images of the technique and catheter placement are included in this review. CONCLUSIONS: Despite being an uncommon management strategy, the literature suggests an indication for performing a mastoidectomy in a small percentage of patients with a chronically draining tympanostomy tube.

Long-term outcomes of cartilage-buttressed T-tube tympanoplasty for prolonged middle ear ventilation.

OBJECTIVES/HYPOTHESIS: To report on the safety and efficacy of cartilage-buttressed T-tube tympanoplasty for long-term middle ear ventilation, specifically by examining duration of tube survival, as well as adverse events associated with prolonged middle ear intubation, including persistent tympanic membrane perforation. STUDY DESIGN: Retrospective case series of patients undergoing cartilage-buttressed T-tube tympanoplasty between January 2005 and December 2016 in a tertiary-care neurotology private practice. METHODS: Patients who underwent cartilage T-tube tympanoplasty with complete pre- and postoperative audiometric data and a minimum follow-up duration of 12 months were analyzed. T-tube survival and adverse events including persistent tympanic membrane perforation were recorded and compared to published data for other long-term middle ear ventilation techniques. RESULTS: The study cohort included 72 cartilage-buttressed T-tube tympanoplasties in 68 patients. Median tube survival was 34 months (range, 2-131 months). Incidence of persistent tympanic membrane perforation (n = 1) was 1.4% CONCLUSIONS: Cartilage-buttressed T-tube tympanoplasty is a safe and effective means of accomplishing long-term middle ear ventilation with a considerably lower rate of persistent tympanic membrane perforation as compared to alternative methods of prolonged middle ear ventilation. LEVEL OF EVIDENCE: 4 Laryngoscope, 129:203-208, 2019.

Deletion of genes involved in the ketogluconate metabolism, Entner-Doudoroff pathway, and glucose dehydrogenase increase local and invasive virulence phenotypes in Streptococcus pneumoniae.

Streptococcus pneumoniae displays increased resistance to antibiotic therapy following biofilm formation. A genome-wide search revealed that SP 0320 and SP 0675 (respectively annotated as 5-keto-D-gluconate-5-reductase and glucose dehydrogenase) contain the highest degree of homology to CsgA of Myxococcus xanthus, a signaling factor that promotes cell aggregation and biofilm formation. Single and double SP 0320 and SP 0675 knockout mutants were created in strain BS72; however, no differences were observed in the biofilm-forming phenotypes of mutants compared to the wild type strain. Using the chinchilla model of otitis media and invasive disease, all three mutants exhibited greatly increased virulence compared to the wild type strain (increased pus formation, tympanic membrane rupture, mortality rates). The SP 0320 gene is located in an operon with SP 0317, SP 0318 and SP 0319, which we bioinformatically annotated as being part of the Entner-Doudoroff pathway. Deletion of SP 0317 also resulted in increased mortality in chinchillas; however, mutations in SP 0318 and SP 0319 did not alter the virulence of bacteria compared to the wild type strain. Complementing the SP 0317, SP 0320 and SP 0675 mutant strains reversed the virulence phenotype. We prepared recombinant SP 0317, SP 0318, SP 0320 and SP 0675 proteins and confirmed their functions. These data reveal that disruption of genes involved in the degradation of ketogluconate, the Entner-Doudoroff pathway, and glucose dehydrogenase significantly increase the virulence of bacteria in vivo; two hypothetical models involving virulence triggered by reduced in carbon-flux through the glycolytic pathways are presented.

Combined petrosal approaches in the management of temporal bone meningiomas.

OBJECTIVES: To evaluate the indications and outcomes of the combined petrosal approaches in the surgical management of temporal bone meningiomas. STUDY DESIGN: Retrospective chart review. SETTING: University teaching hospital. PATIENTS: Adults with temporal bone meningiomas. INTERVENTION(S): Meningioma removal using a combined petrosal approach. MAIN OUTCOME MEASURE(S): Cranial nerve outcomes, complications, completeness of resection, and recurrence rates. RESULTS: Forty-nine patients underwent surgical excision of a temporal bone meningioma between 1996 and 2004 at our institution. Nineteen of these patients required a combined petrosal approach for excision. The most common presenting complaints were balance disturbance, 11 (58%); hearing loss, 10 (53%); headache, 10 (53%); and tinnitus, 9 (47%). The most common tumor origin was of the petrous ridge (14; 74%). Average tumor size was 3.1 cm. Complete resection was possible in 17 (89%) patients. Upper cranial nerve (III-VI) function was improved in two (11%) patients and worsened in three (16%) patients. Lower cranial nerve (IX-XII) function improved in one (5%) patient and was worsened in one (5%) patient. Postoperative facial nerve function was Grades I to II in 16 (84%) patients and Grades III to IV in 1 (5%) patient at last follow-up. Hearing data were available in 14 patients. Of those patients, 11 (85%) had serviceable hearing after surgery. The most common surgical complication was a cerebrospinal fluid leak, with three (16%) incidences. There were no reported incidents of stroke, death, or meningitis in the cohort. CONCLUSION: The use of the combined petrosal approach for temporal bone meningioma resection results in favorable outcomes for the patient. The incidence of complications is acceptably low, and cure rates are high.

Fungal skull base osteomyelitis: Emerging microbial identification techniques.

Culture-based pathogen identification in skull base osteomyelitis, particularly for fungi, is often inaccurate. We report the case of patient with fungal skull base osteomyelitis cured by sustained antifungal therapy after 16 months of debilitating illness. Due to medical complications, a strong clinical rationale was needed to justify long-term antifungal therapy. The offending fungus was identified by experimental molecular technology (Ibis T5000 universal biosensor); invasive fungal disease was corroborated by biochemical assays. Our discussion will help familiarize the otolaryngologist with existing biochemical and molecular diagnostics for invasive fungal disease. We encourage future investigators to study their application in cases of skull base osteomyelitis. Laryngoscope, 127:E5-E7, 2017.

Hydroxyapatite cement cranioplasty following translabyrinthine approach: Long-term study of 369 cases.

OBJECTIVE: To report the authors' experience with hydroxyapatite cement (HAC) cranioplasty and analyze the material's long-term safety and efficacy in repairing translabyrinthine skull-base defects by examining adverse events, specifically cerebrospinal fluid (CSF) leaks and surgical site infections. STUDY DESIGN: Retrospective case-control study (primary study arm); prospective cross-sectional study of patients not examined within the last 5 years (secondary arm). SETTING: tertiary-care neurotology private practice and academic practice (two centers). METHODS: Hydroxyapatite cement implanted following translabyrinthine approach, with or without fat graft, was included. Combined approaches were excluded. Implant-associated adverse events were defined as 1) CSF leaks requiring reoperation or spinal drainage, and (2) infections requiring reoperation. Patients not examined within 5 years were interviewed by telephone to update their condition. Incidence of adverse events was compared to published data for translabyrinthine cranioplasty using fat graft alone. Implant survival analysis was performed. RESULTS: The study cohort included 369 HAC implants in the same number of patients. There were seven CSF leaks and seven infections. Combined (n = 14) incidence of adverse events was 3.8% (2.09%, 6.28%). Compared to fat graft alone, the adverse events associated with HAC were fewer (P < 0.001). Up to 15 years (5,475 days), HAC cement maintained 95% adverse event-free survival. There were no cases of meningitis. CONCLUSION: Cranioplasty using HAC with autologous fat following translabyrinthine skull-base surgery is safer and more effective than fat graft alone, up to 15 years after surgery. LEVEL OF EVIDENCE: 4. Laryngoscope, 127:2120-2125, 2017.

An evaluation of the risk of cerebrospinal fluid leakage as a function of the surgical approach to the cochlear nerve.

OBJECTIVE: Direct implantation into the cochlear nerve can bypass the potential limitations of conventional cochlear implants. Implantable electrical arrays increase the number of stimulation sites, broaden frequency selectivity, require less activating current, and are not dependent on cochlear anatomy. Cochlear nerve implantation demands a unique surgical exposure to the modiolus. This study comprises an assessment of the risk of generating an intraoperative cerebrospinal fluid (CSF) leak using this approach. METHODS: Five fresh cadavers were obtained for dissection. Using dyed normal saline as an indicator, the central nervous system was pressurized to physiological parameters. Surgical approaches to the cochlear nerve were initiated. Leakage of indicator fluid for each approach was quantitatively and qualitatively assessed. Indicator dye in the surgical field was considered a CSF breech. Indicator fluid was collected and quantified. RESULTS: Eighty percent of the surgical approaches caused quantifiable leaks with rates ranging from 0.1 mL/minute to 6 mL/minute. For directly implanted microarrays, the utilization of a modified facial recess approach with cochleostomy and modiolar drillout carries significant risk for generating a CSF leak during the procedure.

Visually enhanced vestibulo-ocular reflex: a diagnostic tool for migraine vestibulopathy.

OBJECTIVES: 1) To compare the incidence of elevated visually enhanced vestibular-ocular reflex (VVOR) rotational gain during rotational chair testing in a normal control group versus a group of patients diagnosed with migraine vestibulopathy; 2) to discuss the possible application of VVOR gain during rotational chair testing for diagnosing migraine vestibulopathy. STUDY DESIGN: Prospective normal control group and retrospective cohort comparison. METHODS: 1) Prospective rotational chair studies including VVOR in 20 normal control patients; 2) retrospective review of vestibular studies including VVOR in 100 consecutive patients with migraine vestibulopathy. RESULTS: Twenty of the normal controls and 69 of the migraine vestibulopathy patients met all inclusion criteria. One of 20 (5%) normal control patients had elevated VVOR gain, whereas 49 of 69 (71%) of migraine vestibulopathy patients had elevated VVOR gain. CONCLUSIONS: VVOR gain normal criteria were appropriate in 95% of our normal control test group. VVOR gain was more frequently elevated in migraine vestibulopathy patients than in the normal controls, and the difference was significant (P < .001). VVOR gain elevation was the most common vestibular test abnormality in this cohort of patients with migraine vestibulopathy. Because VVOR measures visual-vestibular interaction and its central connections, this parameter may be a useful diagnostic tool for migraine vestibulopathy in patients manifesting disequilibrium and motion sensitivity.

Stapedectomy in congenital stapes fixation: are hearing outcomes poorer?

OBJECTIVE: To evaluate outcomes of stapedectomy surgery for congenital stapes fixation. STUDY DESIGN: Retrospective chart review. METHODS: The charts of 463 stapedectomies performed by 1 surgeon from 1996 to 2003 were reviewed. Patients with a history of childhood hearing loss and operative findings consistent with congenital fixation of the stapes were included. Patients with a history of trauma or chronic otitis media and those with otosclerosis were excluded. Thirty-six patients underwent stapedectomy for congenital fixation. Revision cases and those with inadequate postoperative bone or air conduction data were excluded. Inclusion criteria were met for 25 patients. Operative findings and hearing outcomes were evaluated. RESULTS: The study group was comprised of 25 stapedectomies. Closure of the air-bone gap to less than 10 dB was achieved in 48% of cases, and 80% had closure within 20 dB. A gap of more than 30 dB remained in 3 cases. There were 2 cases of sensorineural hearing loss with worsening of the bone conduction thresholds by 15 dB and 30 dB. There were no instances of perilymph gusher. In addition to an excluded case that was not reconstructed because of facial nerve position, 3 of the included patients were found to have a dehiscent facial nerve at surgery. There were no facial nerve injuries, and fixation of the malleus or incus was not found in any of the included patients. When hearing outcomes were compared with our results for otosclerosis over the same time period, rates of closure to within 10 dB and 20 dB were significantly worse in the congenital group. CONCLUSION: Closure of the air-bone gap in this population differs from our results in stapedectomy done for otosclerosis. This difference likely reflects subtle anatomic variations in the congenital group that affect the effectiveness of the prosthesis. Despite the difference in results, stapedectomy for congenital fixation remains an effective method to achieve significant hearing improvement in the majority of patients. EBM RATING: B-3b.

Reversible peripheral vestibulopathy: the treatment of superior canal dehiscence.

OBJECTIVE: Superior canal dehiscence (SCD) is a recently described disorder that results from absence of bone over the superior semicircular canal. We have reviewed 30 cases of SCD found at our institution and report their presentation, workup, and response to therapy. STUDY DESIGN: Retrospective chart review of all patients diagnosed with SCD from 1999 to 2004 at the University of Utah. RESULTS: Thirty patients were identified with SCD. Patients presented with chronic disequilibrium (63%), Tullio's phenomenon (41%), pressure evoked vertigo (44%), hearing loss (30%), and pulsatile tinnitus (7%). ENG performed early in our series revealed abnormal nystagmus with sound presentation, Valsalva, or tympanogram; however, history and CT examination alone was used to identify this condition in most of our patients. Twenty-seven of the 30 patients had some symptoms related to SCD; the other 3 were found to have incidental SCD on CT examination. Of these patients, 14 had severe enough symptoms to warrant operative intervention. All, but one had resolution of their symptoms after completion of intervention. CONCLUSIONS: Superior canal dehiscence is a highly treatable form of vestibulopathy once recognized. When patients present with typical symptoms, workup with CT is reliable and accurate. Surgical intervention results in reversal of symptoms in most cases with low morbidity. EBM RATING: C-4.

Identification and Characterization of msf, a Novel Virulence Factor in Haemophilus influenzae.

Haemophilus influenzae is an opportunistic pathogen. The emergence of virulent, non-typeable strains (NTHi) emphasizes the importance of developing new interventional targets. We screened the NTHi supragenome for genes encoding surface-exposed proteins suggestive of immune evasion, identifying a large family containing Sel1-like repeats (SLRs). Clustering identified ten SLR-containing gene subfamilies, each with various numbers of SLRs per gene. Individual strains also had varying numbers of SLR-containing genes from one or more of the subfamilies. Statistical genetic analyses of gene possession among 210 NTHi strains typed as either disease or carriage found a significant association between possession of the SlrVA subfamily (which we have termed, macrophage survival factor, msf) and the disease isolates. The PittII strain contains four chromosomally contiguous msf genes. Deleting all four of these genes (msfA1-4) (KO) resulted in a highly significant decrease in phagocytosis and survival in macrophages; which was fully complemented by a single copy of the msfA1 gene. Using the chinchilla model of otitis media and invasive disease, the KO strain displayed a significant decrease in fitness compared to the WT in co-infections; and in single infections, the KO lost its ability to invade the brain. The singly complemented strain showed only a partial ability to compete with the WT suggesting gene dosage is important in vivo. The transcriptional profiles of the KO and WT in planktonic growth were compared using the NTHi supragenome array, which revealed highly significant changes in the expression of operons involved in virulence and anaerobiosis. These findings demonstrate that the msfA1-4 genes are virulence factors for phagocytosis, persistence, and trafficking to non-mucosal sites.

Implantation of the ossified cochlea: management with the split electrode array.

OBJECTIVES/HYPOTHESIS: To describe indications for, the surgical technique required, and the expected functional results of split electrode array cochlear implants. STUDY DESIGN: Retrospective chart review. METHODS: Data collected included etiology of deafness, radiographic findings, pre- and postoperative aided pure tone thresholds, and speech perception testing. Adult speech perception outcomes were measured using the Consonant Nucleus Consonant (CNC) monosyllable words and Hearing in Noise Test (HINT) in quiet/noise (+10 dB). The children were assessed using the Infants and Toddlers Meaningful Auditory Integration Scale. RESULTS: Five patients were implanted with a split electrode array. This included two adults and three children. Both adults had preoperative binaural aided pure tone averages worse than 50 dB and scores of 0% on both HINT quiet and CNC words. The children had undetectable preoperative aided thresholds and scored an average 4/40 on the IT-MAIS. Postimplant, the average threshold gain was 38.5 dB in the adults and 81.5 dB in the children. One adult improved to score 51%/22% on HINT quiet/noise at 6 months and 72%/30% at 12 months. The other adult continued to score 0% on HINT at 12 months but claimed substantial subjective auditory improvement after the first year of device use. The children averaged 28/40 on the IT MAIS at 6 months after implantation. Forty-two of 48 implanted electrodes were functional. CONCLUSIONS: The split electrode array is a useful alternative to traditional cochlear implants in treating deafened patients with cochlear ossification. Patients implanted with the split array show marked improvement in sound and speech perception.

Jugular foramen schwannomas: diagnosis, management, and outcomes.

OBJECTIVES/HYPOTHESIS: To describe the presentation, radiographic findings, and surgical management of seven patients who have been diagnosed and treated with jugular foramen schwannomas at the University of Utah. STUDY DESIGN: Retrospective chart review. METHODS: The charts of seven patients diagnosed with jugular foramen schwannomas were reviewed for presentation symptoms, radiographic findings, and physical examination findings. For the six who underwent surgical excision, the surgical procedure used, cranial nerve function results, audiometric results, perioperative complications, and other follow-up data are presented. RESULTS: Seven patients were identified from ages 24 to 69 years. Six of the seven underwent surgical excision. Primary presentation symptoms included dizziness, hearing loss, dysphagia, diplopia, tongue paresis, and hoarseness. The choice of surgical approach was based on the size and location of the tumor. All patients had complete excision of their tumors. The nerve of origin included the glossopharyngeal, vagus, and spinal accessory nerves. Preoperative cranial nerve dysfunction continued postoperatively for lower cranial nerves but resolved in patients who were noted to have preoperative dysfunction of cranial nerve V and VI. The rate of new lower cranial nerve injury was 15% and was only seen in the cranial nerves that were determined to be the nerve of origin. In two cases, a temporary feeding tube was required. No recurrences have been noted to date. CONCLUSIONS: Jugular foramen schwannomas can be successfully diagnosed preoperatively with computed tomography and magnetic resonance imaging. These tumors can be successfully managed with surgery and low morbidity.

Dominant optic atrophy, sensorineural hearing loss, ptosis, and ophthalmoplegia: a syndrome caused by a missense mutation in OPA1.

PURPOSE: To describe the clinical features of and identify the disease-causing mutation in a large Utah family segregating a dominantly inherited syndrome of optic atrophy, sensorineural hearing loss, ptosis, and ophthalmoplegia. DESIGN: Observational case series. METHODS: Thirty individuals at risk for a syndrome of optic atrophy, sensorineural hearing loss, ptosis, and ophthalmoplegia in a single family underwent clinical examinations and venipuncture. Linkage analysis and mutation screening of the optic atrophy 1 gene (OPA1) were performed. RESULTS: Eighteen individuals demonstrated characteristics of the syndrome. Genetic analysis identified a G-->A substitution at nucleotide position 1334 in exon 14 of OPA1 causing an arginine-to-histidine change (R445H) in all affected members of the family. This change segregated with the disease phenotype in the study family with a LOD score of 7.02 at theta; = 0 and was not found in 200 normal control subjects. Analysis of an unrelated Belgian family with a similar phenotype revealed the same R445H mutation segregating with the disease phenotype. CONCLUSIONS: This study describes a mutation in OPA1 causing a unique syndrome of optic atrophy, sensorineural hearing loss, ptosis, and ophthalmoplegia. These results expand the spectrum of human disease associated with mutations of OPA1 and indicate that ophthalmologists caring for patients with optic atrophy should inquire about possible associated hearing loss. Although OPA1 is a nuclear gene, the gene product localizes to mitochondria, suggesting that mitochondrial dysfunction may be the final common pathway for many forms of syndromic and nonsyndromic optic atrophy, hearing loss, and external ophthalmoplegia.

Ossicular chain reconstruction: titanium versus plastipore.

OBJECTIVE: To compare the complication rate and hearing results of a new, lightweight, titanium ossicular replacement prosthesis with Plastipore prostheses (Xomed, Jacksonville, FL). STUDY DESIGN: Retrospective. METHODS: Charts were reviewed for type of operation, type of prosthesis used, extrusion rate, prostheses failure rate, and hearing thresholds at multiple frequencies and at multiple follow-up points. The dependant variable for hearing results was the four-frequency average air-bone gap. RESULTS: There were 84 patients undergoing tympanoplasty with the Plastipore prosthesis and 53 with the titanium. There was one extrusion in the titanium group. There was an additional single incidence of prosthesis failure in the titanium group. Overall hearing results were comparable with an air-bone gap average of 19.3 dB in the Plastipore group compared with the titanium group with an air-bone gap of 22.0 dB (P =.08). Sixty percent of patients had a postoperative air-bone gap of 20 dB or less in the Plastipore group. In the titanium group, 45.3% achieved a 20 dB or less postoperative air-bone gap. Plastipore had a lower air-bone gap than the titanium when a canal wall up operation was performed (17.8 vs. 23.9 dB) and tended toward a lower air-bone gap when a total ossicular prosthesis was needed (22 vs. 27 dB) (P <.07). CONCLUSION: The titanium prosthesis is a new ossicular replacement prosthesis that provides excellent visualization during insertion and provides hearing results that are comparable with Plastipore. The Plastipore prosthesis performed better in canal wall up mastoidectomy situations and tended toward better performance when a total ossicular replacement prosthesis was needed.