Diagnosis and management of facial nerve palsy secondary to granulomatosis with polyangiitis - A systematic review.

OBJECTIVE: Granulomatosis with polyangiitis is associated with otolaryngologic complaints in 70-95 % of cases, with the most common being serous otitis media. In rare cases, patients may experience facial nerve palsy in conjunction with otologic or nasal symptoms; and, often, initially present to an otolaryngologist. It is important for healthcare professionals to be able to recognize the nuisances of facial nerve palsy as a potential presentation of granulomatosis with polyangiitis. STUDY DESIGN: Systematic review. METHODS: Following the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) Protocol, PubMed and MED-LINE Databases were queried for articles published from January 2007 to December 2022 describing facial nerve palsy in the context of Granulomatosis with polyangiitis, formerly known as Wegener's Granulomatosis. The keywords included "facial nerve palsy", "facial palsy", "granulomatosis with polyangiitis", "Wegener's granulomatosis", "ANCA positive" in the title/abstract. All full-text articles available in English were screened, including single case presentations. Abstracts, commentaries, and publications deemed outside the scope of our study aims were excluded from review. After removal of duplicate articles, a total of 85 articles were screened. After applying inclusion and exclusion criteria, 14 articles were included in the review. RESULTS: There were a total of 28 reports of facial nerve palsy in the literature in patients who were eventually diagnosed with granulomatosis with polyangiitis. The patients' ages ranged from 14 to 68 years old. None of the patients had been previously diagnosed with GPA, and a majority of them presented initially with other otologic symptoms. Hearing loss was reported in 24 patients (86 %), otalgia was present in 11 patients (39 %), and otorrhea was present in 6 patients (21 %). Bilateral facial paralysis was reported in 10 patients in the literature (36 %). In total, 16 patients underwent surgery for facial paralysis: 6 tympanomastoidectomies, 4 mastoidectomies, 2 explorative tympanotomies. Surgery was generally considered ineffective in resolving facial weakness. All patients ended up receiving some combination of steroids and immunosuppressant, most commonly prednisolone and cyclophosphamide or rituximab, which was eventually transitioned to azathioprine for maintenance. Unlike auditory thresholds, which remained decreased in two patients, all patients recovered facial function following appropriate medical treatment of their vasculitis. CONCLUSIONS: Facial nerve paralysis in patients with granulomatosis with polyangiitis is a rare but treatable phenomenon. In patients with intractable otitis media, unresolving facial palsy, or a combination of otologic issues, it is important to consider GPA as a possible source. The prognosis for facial function appears to be excellent in patients who undergo appropriate treatment for vasculitis, but further studies are needed for confirmation.

Is there a role for Eustachian tube balloon dilation in pediatric patients with refractory Eustachian tube dysfunction?

BACKGROUND: Eustachian tube dysfunction (ETD) is common in children. Over the past decade, eustachian tube balloon dilation (ETBD) has become a more widespread treatment for this condition. Data has been encouraging in the adult population, but data among the pediatric population has been sparser. This comprehensive review aims to assess current evidence for ETBD in pediatric patients. REVIEW: Studies relevant to ETBD in the pediatric population were identified by utilizing the PubMed MEDLINE database. While multiple retrospective studies were found, this search yielded two systematic reviews focused on the pediatric population as the highest level of evidence assessing ETBD in children. There was overlap of included studies, but each review contained a unique set of studies. These reviews found that ETBD was safe in children. BEST PRACTICE SUMMARY: Overall, ETBD appears safe and efficacious in children with refractory ETD. Future prospective trials confirming this conclusion are warranted. At this time, there appears to be a role for ETBD with or without ventilation tube placement in children with refractory ETD.

Otologic use of porcine small intestinal submucosal graft (biodesign): A MAUDE database review.

OBJECTIVE: To review and summarize reported adverse events related to the use of porcine small intestine submucosal grafts (Biodesign™) in otologic procedures. STUDY DESIGN: Retrospective cross-sectional analysis. SETTING: Food and Drug Administration's Manufacturer and User Facility Device Experience (MAUDE) database. MATERIAL AND METHODS: The MAUDE database was queried for all medical device reports (MDR) related to otologic use of Biodesign™ (Cook Medical, Bloomington, IN) from January 2016 to November 2022. Adverse events (AEs) were identified by reviewing all reports with the basic search term "Biodesign" and "Biodesign, Otologic". Reports were individually reviewed and categorized with special attention to AEs. RESULTS: A total of 500 reports were reviewed. Since FDA approval of Biodesign™ in 2016, there have been 5 adverse events reported for use of Biodesign™ during otologic surgery (tympanoplasty, n = 3; stapes surgery, n = 2). All reported events described patient injury, and all cases required at least one revision surgery. Four cases described significant foreign body inflammatory reactions. Complications included hearing loss (n = 3), severe otalgia (n = 2), persistent perforation (n = 2), vertigo (n = 2), and facial paralysis (n = 1). CONCLUSION: The use of porcine small intestinal submucosal graft has been thought to be a safe and effective option for otologic surgery, with the advantage of availability without graft harvest in minimally invasive endoscopic surgery. However, foreign body or granulomatous reactions have been documented and should be considered prior to its use in otologic surgery.

Head trauma and Cochlear implant displacement - A systematic review.

OBJECTIVE: Cochlear implant (CI) migration due to head trauma is a rare complication, and there is limited data summarizing this topic. This review seeks to provide a more comprehensive understanding of risk factors, clinical presentations, diagnosis, and intervention strategies for traumatic CI displacement. METHODS: The Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) Protocol was followed using the PubMed and MED-LINE databases from 2000 to 2021, with manual cross-checks of reference lists of identified articles for additional relevant studies. The keywords used for database searches included "cochlear implant", "migration", "displacement", "extrusion" and "head trauma". All full-text articles available in English were screened, including single case presentations. Abstracts, commentaries, and publications deemed outside the scope of our study aims were excluded from review. RESULTS: After the removal of duplicate articles, a total of 152 articles were screened. After applying inclusion and exclusion criteria, 11 articles were included in the review. Due to heterogeneity of the available literature, a qualitative synthesis was employed to integrate results by identifying common themes among the relevant studies. CONCLUSIONS: The incidence of traumatic CI migration is extremely low and can be due to receiver-stimulator displacement, magnet displacement, or electrode extrusion. Migration occurred anywhere from 3 months to 26 years after implantation, suggesting that risk of migration from head trauma does not depend solely on time from implantation. There are several methods for repairing migrated CI, but there is limited data comparing operative techniques, specifically in the prevention of traumatic CI migration.

Impact of Surgery for Velopharyngeal Insufficiency on Eustachian Tube Function in Children: Pharyngeal Flap Versus Sphincter Pharyngoplasty.

OBJECTIVE: Velopharyngeal insufficiency (VPI) is a common speech disorder in patients with a history of cleft palate (CP) or 22q11.2 deletion syndrome. Pharyngeal flap (PF) and sphincter pharyngoplasty (SP) are 2 common surgeries to treat this disorder by decreasing unwanted nasal air emission and hypernasal resonance. Because Eustachian tube dysfunction (ETD) in patients with CP may be more frequent after surgery for VPI, we examined whether ETD was associated with either type of surgery. DESIGN: Retrospective cohort study. SETTING: Children's hospital-based tertiary referral center. PATIENTS: A total of 225 children with VPI who underwent primary PF (201) or SP (24) between 2006 and 2017. OUTCOME MEASURES: We examined differences in risk of ETD according to both surgical groups and proxies for postoperative nasal obstruction. These proxies included postoperative resonance measures and development of obstructive sleep apnea (OSA). RESULTS: Both surgical groups had similar preoperative measures, except the PF group had higher hypernasality by PSA. Postoperatively, the PF group demonstrated lower hypernasal resonance by nasometry and PSA. There were no differences between PF and SP groups with regard to ETD. Proxies for postoperative nasal obstruction also were not predictive of postoperative ETD. Degree of CP and younger age were found to be risk factors for ETD. CONCLUSION: There was no significant difference in the effects of PF and SP on ETD in this study. Neither lower hypernasality nor incidence of OSA had any impact on ETD. Degree of CP and younger age were the only significant risk factors for ETD that this study identified.

Quality of life outcomes after transmastoid plugging of superior semicircular canal dehiscence.

PURPOSE: This study was performed to evaluate the effectiveness and impact on quality of life in patients undergoing plugging of superior semicircular canal dehiscence using the transmastoid approach. MATERIALS AND METHODS: Retrospective chart review with prospective outcomes assessment, using validated quantitative scoring systems, was performed on 10 patients (23-76 years) who underwent transmastoid plugging of superior semicircular canal dehiscence between February 2014 and February 2018 at a tertiary referral center. Pre-operative and post-operative autophony and vertigo were measured by The Autophony Index and the Dizziness Handicap Index. Overall quality of life following intervention was measured by the Glasgow Benefit Inventory. Subjective improvement, audiological changes, and subjective quality of life changes were also recorded. RESULTS: A significant reduction in the total Dizziness Handicap Index was seen following transmastoid repair of superior semicircular canal dehiscence (p = 0.0078). This was also evident when subgroup analysis of the Dizziness Handicap Index was performed, as physical (p = 0.0273), emotional (p = 0.0078), and functional subgroups were all significantly reduced (p = 0.0117). Autophony was also significantly reduced following intervention (p = 0.0312). Overall quality of life was seen to be improved following surgery as measured by the Glasgow Benefit Inventory (p = 0.0345). CONCLUSION: Our data suggest that transmastoid plugging of a dehiscence in the superior semicircular canal is a safe and effective means of improving autophony, dizziness and overall quality of life in these patients. We believe that these results should be taken into consideration in discussions regarding surgical approach for patients who are contemplating this procedure.

Functional results after cholesteatoma surgery in an adult population using the retrograde mastoidectomy technique.

In this retrospective study, we analyzed the functional results after using the retrograde mastoidectomy technique for cholesteatoma removal in an adult patient population. The described technique was used at a tertiary referral center for cholesteatoma removal in 218 adult patients, representing 242 operated ears, with an average follow-up time of 20.3 months. With the retrograde mastoidectomy technique, the cholesteatoma is removed posteriorly through the canal wall, from the epitympanic region toward the mastoid, with the option to reconstruct the posterior bony canal wall or create an open mastoid cavity, depending on the size of the defect. Primary surgery was carried out in 58.7 % ears, with the remaining 41.3 % representing revision surgery. In 151 cases, the posterior canal wall was reconstructed, and in 91 cases a classical CWD with an open mastoid cavity was created. In the majority of the cases (n = 213, 88.0 %), a primary hearing restoration was performed. There were 18 recurrences (12.7 %) in primary cases and 22 recurrences (22 %) in revision surgeries. Ninety percent of the recurrences (36 of 40 cases) occurred within 5 years. A postoperative air-bone gap of less than 20 dB was achieved in 61.6 % of the operated ears. Ears with a reconstructed posterior canal wall had significantly better hearing results than those cases in which a CWD procedure was used (air-bone gap of 17.6 versus 22.5 dB, p < 0.05). The retrograde mastoidectomy technique for cholesteatoma removal resulted in satisfying hearing results in the majority of the cases, with a recurrence rate comparable to the current literature.

Auditory brainstem implants: an analysis of adverse events in the MAUDE database.

OBJECTIVE: Auditory Brainstem Implants (ABI) are used to restore hearing in patients lacking appropriate cochlear anatomy and/or cochlear nerve. The objective of this study was to examine the Manufacture and User Facility Device Experience (MAUDE) database to analyze adverse events. STUDY DESIGN: This is a study of a multi-institutional database maintained by the US FDA. SETTING: A database analysis was performed via collaboration of multiple clinicians at tertiary referral centers. METHODS: The MAUDE database was queried for Medical Device Reports (MDRs) relating to ABIs. MDRs were identified using the advanced search term "Implant, Auditory Brainstem" and reviewing all reports with the basic search term "Brainstem Implant". All collected reports were individually reviewed. RESULTS: A total of 265 individual patient reports were reviewed, of which 55 reports met inclusion criteria. Reports regarding audiologic outcome included failure to provide hearing benefit (n = 27), implant failure/device malfunction (n = 10), and device non-use (n = 6). Postoperative complications included local skin infection (n = 3), CSF leak (n = 3), elevated ICP (n = 1), surgical site dehiscence (n = 1), swelling (n = 1), seroma formation requiring drainage (n = 1), and meningitis (n = 2). Two patients had dislodged magnets during 1.5 Tesla MRI acquisition. There were 35 instances of full explantation of the device and 1 partial removal; 13 patients had a new device implanted following explantation. CONCLUSIONS: Poor hearing results, device failure, and non-use were commonly reported causes for explanation in this analysis. This information can aid physicians in counseling patients and family members and managing device expectations.

Focal electrical stimulation as an effective sham control for active rTMS and biofeedback treatments.

A valid sham control is important for determining the efficacy and effectiveness of repetitive transcranial magnetic stimulation (rTMS) as an experimental and clinical tool. Given the manner in which rTMS is applied, separately or in combination with self-regulatory approaches, and its intended impact on brain states, a valid sham control of this type may well serve as a meaningful control for biofeedback studies, where efforts to develop a credible control have often been less than ideal. This study examined the effectiveness of focal electrical stimulation of the frontalis muscle as a sham technique for blinding participants to high-frequency rTMS over the dorso-lateral prefrontal cortex (DLPFC) at durations, intensities, and schedules of stimulation similar to many clinical applications. In this within-subjects single blind design, 19 participants made guesses immediately after receiving 54 counterbalanced rTMS sessions (sham, 10 Hz, 20 Hz); 7 (13 %) of the guesses were made for sham, 31 (57 %) were made for 10 Hz, and 16 (30 %) were made for 20 Hz. Participants correctly guessed the sham condition 6 % (CI 1, 32 %) of the time, which is less than the odds of chance (i.e., of guessing at random, 33 %); correctly guessed the 10 Hz condition 66 % (CI 43, 84 %) of the time, which was greater than chance; and correctly guessed the 20 Hz condition 41 % (CI 21, 65 %) of the time, which was no different than chance. Focal electrical stimulation therefore can be an effective sham control for high-frequency rTMS of the DLPFC, as well as for active biofeedback interventions. Participants were unaware that electrical stimulation was, in fact, sham rTMS.

Eustachian Tube Balloon Dilation: A Comprehensive Analysis of Adverse Events.

BACKGROUND: Eustachian tube balloon dilation (ETBD) has been Food and Drug Administration (FDA) approved for refractory Eustachian tube dysfunction since 2016. While ETBD is generally seen as safe, the complication profile has not been well defined. OBJECTIVE: The objective of this study was to utilize the FDA manufacturer and user facility device experience (MAUDE) database to better assess adverse events (AE) related to ETBD. METHODS: This is a study of a multiinstitutional database maintained by the U.S. FDA. A database analysis was performed via the collaboration of multiple clinicians at tertiary referral centers. The FDA MAUDE database was queried for all medical device reports (MDR) related to ETBD devices from January 2012 to November 2022. Eighty-eight unique MDR were identified, 16 of which met inclusion criteria. RESULTS: Three MDRs were classified as device-related (18.8%); none resulted in an AE. Thirteen MDRs (81.3%) were patient-related; all were classified as AEs. The most common AE was postoperative subcutaneous emphysema (n = 6, 46.2%). Of the patients with subcutaneous emphysema, there was a wide range of severity. The most severe AE (n = 1, 6.3%) was postoperative stroke secondary to carotid artery dissection. CONCLUSION: Though ETBD is generally seen as a safe procedure, there have been several concerning AEs reported to date. Increased awareness of ETBD complications can serve as a primer for improved patient education and counseling during the informed consent process and aid surgeons in clinical decision-making. Future studies with standardized reporting protocols are warranted to create a central registry for ETBD.

Pharmaceutical countermeasures have opposite effects on the utricles and semicircular canals in man.

INTRODUCTION: Sensory conflicts in the vestibular system lead to motion sickness of which space motion sickness (SMS) is a special case. SMS affects up to 70% of the astronauts during the first 3 days in space. The search for effective countermeasures has led to several nonpharmacological and pharmacological approaches. The current study focuses on the effects of lorazepam (1 mg), meclizine (25 mg), promethazine (25 mg), and scopolamine (0.4 mg) on the vestibular system, with special focus on the canal and otolith functions separately. METHODS: The study had a placebo-controlled, single blind, repeated measures design. Sixteen healthy volunteers were subjected to a total of 7 test sessions, the first and last being without intake of medication. Semicircular canal function was evaluated by means of electronystagmography and otolith function with unilateral centrifugation. The horizontal semicircular canal function was characterized by the vestibulo-ocular reflex (VOR) gain measured during earth vertical axis rotation as well as the total caloric response. The function of the utricles was represented by the utricular sensitivity, reflecting the ocular counter roll relative to the virtual induced head tilt. RESULTS: Promethazine significantly decreased the semicircular canal and utricular parameters. Both scopolamine and lorazepam caused only a decrease in the utricular sensitivity, whereas meclizine only decreased the semicircular canal-induced VOR gain. DISCUSSION: The results show that the drugs affected different areas of the vestibular system and that the effects can thus be attributed to the specific pharmacological properties of each drug. Meclizine, as an antihistaminergic and weak anticholinergic drug, only affected the VOR gain, suggesting a central action on the medial vestibular nucleus. The same site of action is suggested for the anticholinergic scopolamine since acetylcholine receptors are present and utricular fibers terminate here. The global vestibular suppression caused by promethazine is probably a consequence of its anticholinergic, antihistaminergic, and antidopaminergic properties. Based on the fact that lorazepam increased the affinity of gamma-aminobutyric acid (GABA) for the GABA(A)-receptor and its effects on the utriculi, the site of action seems to be the lateral vestibular nucleus. CONCLUSION: Meclizine, scopolamine, and lorazepam selectively suppress specific parts of the vestibular system. Selective suppression of different parts of the vestibular system may be more beneficial for alleviating (space) motion sickness than general suppressive agents. Additionally, this knowledge may help the clinician in his therapeutic management of patients with either semicircular canal or otolith dysfunction.

Assessing Patient Barriers to Cochlear Implantation.

OBJECTIVE: Evaluate barriers that deter adult patients from following through with cochlear implantation. STUDY DESIGN: Retrospective chart review and phone survey. SETTING: Single tertiary referral center. PATIENTS: Between January 2019 and August 2021, 113 patients, without a previous cochlear implant (CI), were determined to be candidates for cochlear implantation. Thirty-eight (33.6%) patients deferred cochlear implantation. Survey response rate was 61.1% (22/36). INTERVENTION: None. MAIN OUTCOME MEASURES: Demographic, socioeconomic, otologic history, and comorbidity factors associated with deferment of cochlear implantation. Patient survey assessment of factors that had the greatest impact, rated on a scale of 1 to 10 (10 being the most impactful), on their decision to defer a CI. RESULTS: Out of the 113 patients who met inclusion criteria, 75 (66.3%) underwent cochlear implantation and 38 (33.6%) patients deferred. Comparing implanted versus deferred groups, there was no statistical difference in age (67.1 y versus 68.5 y; p = 0.690) or male sex (53.3% versus 57.9%; p = 0.692). The deferred group had higher mean neighborhood disadvantage state decile (5.3 versus 4.3; p = 0.064) and national percentile (73.0 versus 66.2; p = 0.106) scores, although neither were statistically significant. A greater proportion of the deferred group were not living independently at time of CI evaluation (13.2 versus 2.7%; p = 0.017). Fear of losing residual hearing was rated the highest among the survey respondents (mean rating of 5.1), followed by general medical health (4.9) and cost and financial concerns (3.6). CONCLUSION: The greatest concern among the patients was the fear of losing residual hearing. Spending greater time educating patients about the success and failure rates of cochlear implantation may reduce patient hesitancy with implantation.

Verification of speech spectrum audibility for pediatric Baha Softband users with craniofacial anomalies.

OBJECTIVE: The purpose of this study was (1) to determine benefit of the Baha Softband coupled to the Softband for infants and children with bilateral conductive hearing loss; and (2) to verify audibility of the speech spectrum for octave frequencies 500 through 4000 Hz. DESIGN: The research design for this retrospective chart study is pretest-posttest repeated measures. SETTING: The study was conducted in the Department of Audiology and Speech Pathology, Arkansas Children's Hospital. PARTICIPANTS: Twenty-five children aged 6 months to 18 years with craniofacial disorders and bilateral conductive hearing loss participated in the study. Participants were consistent, full-time unilateral Baha users with the Baha Compact bone-conduction amplifier coupled to the head via the Softband. INTERVENTIONS: The intervention was the Baha device coupled to the head via the Softband as a prerequisite to surgical implantation. MAIN OUTCOME MEASURE(S): The primary study outcome measures used aided and unaided soundfield audiometric thresholds to calculate functional gain. Audibility of the speech spectrum was verified by comparison with target aided thresholds. RESULTS: Results revealed an improvement in soundfield thresholds with Baha amplification for the four octave frequencies. Means, standard deviations, and confidence intervals for aided and unaided thresholds are reported. Percentages of thresholds meeting target levels were significant at all frequencies, exceeding the 80% criterion. CONCLUSIONS: Benefit of the Baha in providing audibility of the speech spectrum for infants and children with bilateral congenital conductive hearing loss has been demonstrated, offering important and timely data supporting third-party reimbursement.

Comparison of traditional bone-conduction hearing AIDS with the Baha system.

BACKGROUND: Little research exists to demonstrate efficacy and verification measures of the Baha system versus traditional bone-conduction hearing aids. This study gives statistical data about 10 children who have used traditional bone-conduction hearing aids, Baha coupled to a Softband, and the Baha system implanted. PURPOSE: The purpose of this study was to compare functional gain at 500, 1000, 2000, and 4000 Hz for infants and children with bilateral conductive hearing loss who were initially fit with traditional bone-conduction devices then progressed to Baha with Softband and finally to unilateral Baha implants. RESEARCH DESIGN: Retrospective five-year chart review. STUDY SAMPLE: 10 children with bilateral conductive hearing loss due to congenital atresia and/or microtia. Participants ranged in age from 6 mo to 16 yr; three were male and seven were female. Two participants were African-American, five Caucasian, and three Hispanic. INTERVENTION: The intervention was the Baha system used in children via a Softband or implanted as compared to traditional bone-conduction hearing aids. DATA COLLECTION AND ANALYSIS: Single-factor, repeated analyses of variance were run to examine the amount of functional gain delivered by the various devices as well as the threshold measures with each device at each frequency. RESULTS: Participants in this study showed a statistically significant improvement when using the Baha Softband over traditional bone-conduction hearing aids. An implanted Baha has statistically as much gain as a bone-conduction transducer at all frequencies tested. CONCLUSIONS: The Baha system is a valid treatment in conductive hearing loss via a Softband or implanted. It statistically outperforms the traditional bone-conduction hearing aids and should be used as a first choice in intervention rather than a last option for inoperable conductive hearing loss.

T-tubes Through Cartilage Tympanoplasty: Is It Worth the Perforation Risk?

OBJECTIVE: To review a single surgeon's experience with T-tube placement through cartilage tympanoplasty versus native tympanic membrane for long-term ventilation of the chronic ear and residual perforation rates following tube removal. STUDY DESIGN: Retrospective chart review. SETTING: Two tertiary referral centers. PATIENTS: One hundred sixteen patients (4-71 yr of age) who underwent either total island cartilage tympanoplasty or posterior palisade cartilage tympanoplasty with T-tube placement primarily or secondarily from 1998 to 2016. MAIN OUTCOME MEASURES: Long-term outcome of each T-tube was recorded with respect to retention and patency, and tympanic membrane status following either tube removal or extrusion. Audiometric data, age, sex, diagnosis, and procedure(s) performed were considered. RESULTS: There were 116 patients (122 total ears and 139 total T-tubes) included: 57 ears underwent total island cartilage tympanoplasty with tube placed through cartilage and 65 ears underwent posterior island graft with tube placed through native tympanic membrane. Sixty-eight T-tubes were placed in the total island cartilage group with three (4.4%) residual perforations following removal. Seventy-one T-tubes were placed in the posterior palisade graft group with six residual perforations (8.5%) following removal or extrusion. The mean retention rate for the T-tubes was 3.93 years for the total island tympanoplasty group and 3.58 years for the posterior palisade tympanoplasty group. The mean follow-up for total island tympanoplasty and posterior palisade tympanoplasty was 5.36 and 5.66 years, respectively. CONCLUSION: Our data suggest that T-tube placement through cartilage tympanoplasty is worthwhile providing long-term ventilation to the middle ear and portends no higher risk for residual perforation than T-tube placement through native tympanic membrane.

Outcomes Following Revision Stapes Surgery in Children: A Single-Surgeon, Single-Center 15-Year Experience.

OBJECTIVE: To review a single surgeon experience with revision pediatric stapes surgery for congenital stapes fixation (CSF) and tympanosclerosis (TS). Secondly, to determine whether hearing outcomes following revision surgery may be predicted by a thorough work-up aimed at assessing whether an extruded or malpositioned prosthesis is likely to be encountered intraoperatively. SETTING: Tertiary referral center. STUDY DESIGN: Retrospective chart review. PATIENTS: Fifteen patients having revision surgery for fixation of the stapes footplate over a 15-year period. MAIN OUTCOME MEASURES: Hearing results based on pre- and post-revision pure-tone average air-bone gap (PTA-ABG) and speech recognition threshold testing (SRT). RESULTS: Overall, the mean improvement of PTA-ABG following revision surgery was 11.9 dB (standard deviation [SD] 15.2) while SRTs improved by a mean of 12.3 (SD 19.9). Outcomes were significantly better in patients who reported a history of trauma following their initial surgery, when there was otoscopic evidence of an extruding or extruded prosthesis and/or a pre-revision CT (where performed) suggested an extruded or malpositioned prosthesis. No patients had a significant postoperative sensorineural hearing loss. CONCLUSION: Revision stapes surgery in children is a safe procedure in experienced hands which nonetheless should only be contemplated in patients in whom preoperative work-up suggests an extruded or malpositioned prosthesis is likely to be encountered intraoperatively.

Long-term Outcomes of Ossiculoplasty With and Without an Intact Malleus.

OBJECTIVE: To compare long-term hearing outcomes following ossiculoplasty with cartilage tympanoplasty with (M) and without (M) the malleus present. STUDY DESIGN: Retrospective chart review. SETTING: Tertiary referral center. PATIENTS: One twenty-six patients (18-88 yr of age) undergoing ossiculoplasty with tympanoplasty or tympanomastoidectomy using cartilage tympanic membrane grafts from 1998 to 2012 with at least 5 years of documented postoperative follow-up. MAIN OUTCOME MEASURES: Short-term hearing results (pure-tone average air-bone gap [PTA-ABG] measured between 60 d and 1 yr after surgery), long-term hearing results (PTA-ABG measured ≥5 yr after surgery), Ossiculoplasty Outcome Parameter Staging (OOPS) index, and complications. RESULTS: There were 46 patients in the M group and 80 in the M group. Preoperative PTA-ABG was 23.8 dB for M and 34.5 dB for M (p = 0.00001). Short-term postoperative PTA-ABG was 19.3 dB for M and 18.5 dB for M (p = 0.727). Long-term postoperative PTA-ABG was 18.2 dB for M and 19.6 dB for M (p = 0.500). The OOPS index was 4.11 and 6.41 for M and M, respectively, (p = 0.00001). Thirteen patients (10.3%) experienced complications. CONCLUSION: Our data suggest that the malleus is not statistically significant with regard to its impact on final audiometric outcome following ossiculoplasty. This has implications in our clinic, particularly in our use of the OOPS index as a prognostic tool, and will likely lead to its revision. These data may further support the coupling theory of acoustic gain and weaken the catenary lever theory.

Outcomes of Primary Pediatric Stapedotomy.

OBJECTIVE: To review a single institution experience with pediatric stapedotomy for juvenile otosclerosis (JO), congenital stapes footplate fixation (CSFF), or tympanosclerosis. STUDY DESIGN: Retrospective chart review. SETTING: Tertiary referral center. PATIENTS: Pediatric patients undergoing surgery for stapes fixation from 2001 to 2017. MAIN OUTCOME MEASURES: Hearing result based on preoperative, first postoperative, and final postoperative pure-tone average air-bone gap (PTA-ABG). Age, sex, diagnosis, procedure performed, prosthesis, and ossicular anomalies were considered. RESULTS: A total of 59 children (4-16 years of age) underwent surgery for stapes fixation (67 ears), with an average postoperative audiogram out to 2.88 years. Final postoperative ABG for tympanosclerosis (30.4 dB ±â€Š10.9 dB) showed some improvement but the outcome was significantly worse than in CSFF (21.0 dB ±â€Š11.4 dB) (p = 0.020) and JO (22.8 dB ±â€Š14.9 dB). CONCLUSION: Our data suggest, that in our clinic, surgery for stapes fixation is safe to perform in children. While we achieved desirable results for JO and CSFF, patients with tympanosclerosis showed a statistically worse hearing outcome. Tympanosclerosis deserves special consideration and may be better served with a malleovestibulopexy, total ossicular replacement prosthesis (TORP), or amplification in lieu of traditional stapes surgery.

Cerebellopontine angle tumor in a patient with a maternally inherited SDHD gene mutation.

Acoustic neuromas are the most common tumor in the cerebellopontine angle (CPA) but are rare in the general population. Paragangliomas are rarer still and, in a minority of cases (20%), are known to be caused by errors in the SDHD gene. Mutations in this gene are highly penetrant when inherited paternally but not maternally. We present the first reported case of a patient with a CPA tumor and a maternally inherited SDHD gene mutation.

Evaluation of Intraoperative Testing During Cochlear Implantation From a Time and Cost Perspective: A Single-Center Experience in the United States.

OBJECTIVE: To measure the time spent performing intraoperative testing during cochlear implantation (CI) and determine the impact on hospital charges. STUDY DESIGN: Prospective study. SETTING: Tertiary referral hospital. PATIENTS: Twenty-two children (7 mo-18 yr) who underwent a total of 22 consecutive primary and/or revision CIs by a single surgeon from December 2016 to July 2017. INTERVENTION: The time spent performing intraoperative testing, including evoked compound action potentials (ECAP) and electrical impedances (EI), was recorded for each case. The audiologist performing the testing was unaware of the time measurement and subsequent evaluations with regard to cost data. Billing information was used to determine if the testing contributed to increased operative charges to the patient. OUTCOME MEASURES: Whether intraoperative testing had an impact on operative charges to the patient. RESULTS: The average time spent in testing (ECAPs/EIs in all cases) was 6.7 minutes (range, 2-26 min). No correlation was found between testing time and preoperative computed tomography findings, the audiologist performing testing, or the electrode type used (p > 0.05). Based on billing data, including time spent in the operating room (OR), 5/22 (23%) cases incurred greater charges than if intraoperative testing had not been performed. CONCLUSION: Our data suggest that intraoperative testing increases time in the OR and can contribute to increased hospital charges for CI patients. By using testing selectively, costs incurred by patients and hospitals may be reduced. This is of interest in a healthcare environment that is increasingly focused on cost, quality, and outcomes.