Dispersed DNA variants underlie hearing loss in South Florida's minority population.

BACKGROUND: We analyzed the genetic causes of sensorineural hearing loss in racial and ethnic minorities of South Florida by reviewing demographic, phenotypic, and genetic data on 136 patients presenting to the Hereditary Hearing Loss Clinic at the University of Miami. In our retrospective chart review, of these patients, half self-identified as Hispanic, and the self-identified racial distribution was 115 (86%) White, 15 (11%) Black, and 6 (4%) Asian. Our analysis helps to reduce the gap in understanding the prevalence, impact, and genetic factors related to hearing loss among diverse populations. RESULTS: The causative gene variant or variants were identified in 54 (40%) patients, with no significant difference in the molecular diagnostic rate between Hispanics and Non-Hispanics. However, the total solve rate based on race was 40%, 47%, and 17% in Whites, Blacks, and Asians, respectively. In Non-Hispanic Whites, 16 different variants were identified in 13 genes, with GJB2 (32%), MYO7A (11%), and SLC26A4 (11%) being the most frequently implicated genes. In White Hispanics, 34 variants were identified in 20 genes, with GJB2 (22%), MYO7A (7%), and STRC-CATSPER2 (7%) being the most common. In the Non-Hispanic Black cohort, the gene distribution was evenly dispersed, with 11 variants occurring in 7 genes, and no variant was identified in 3 Hispanic Black probands. For the Asian cohort, only one gene variant was found out of 6 patients. CONCLUSION: This study demonstrates that the diagnostic rate of genetic studies in hearing loss varies according to race in South Florida, with more heterogeneity in racial and ethnic minorities. Further studies to delineate deafness gene variants in underrepresented populations, such as African Americans/Blacks from Hispanic groups, are much needed to reduce racial and ethnic disparities in genetic diagnoses.

Comparison of packing material in an animal model of middle ear trauma.

PURPOSE: To compare the performance of absorbable gelatin sponge (AGS) with polyurethane foam (PUF) as middle ear packing material after mucosal trauma. MATERIALS AND METHODS: Using a randomized, controlled and blinded study design fifteen guinea pigs underwent middle ear surgery with mucosal trauma performed on both ears. One ear was packed with either PUF or AGS while the contralateral ear remained untreated and used as non-packed paired controls. Auditory brainstem response (ABR) thresholds were measured pre-operatively and repeated at 1, 2, and 6weeks postoperatively. Histological analysis of middle ear mucosa was done in each group to evaluate the inflammatory reaction and wound healing. Another eighteen animals underwent middle ear wounding and packing in one ear while the contralateral ear was left undisturbed as control. Twelve guinea pigs were euthanized at 2weeks postoperatively, and six were euthanized at 3days post-operatively. Mucosal samples were collected for analysis of TGF-ß1 levels by enzyme-linked immunosorbent assay. RESULTS: ABR recordings demonstrate that threshold level changes from baseline were minor in PUF packed and control ears. Threshold levels were higher in the AGS packed ears compared with both control and PUF packed ears for low frequency stimuli. Histological analysis showed persistence of packing material at 6weeks postoperatively, inflammation, granulation tissue formation, foreign body reaction and neo-osteogenesis in both AGS and PUF groups. TGF-ß1 protein levels did not differ between groups. CONCLUSION: PUF and AGS packing cause inflammation and neo-osteogenesis in the middle ear following wounding of the mucosa and packing.

Middle fossa vestibular neurectomy for refractory vertigo: less is more.

OBJECTIVE: This study aimed to evaluate outcomes of the middle fossa (MF) superior vestibular neurectomy in unilateral Ménière's disease. PATIENTS AND METHODS: Case series with preoperative and postoperative analysis of the 1995 American Academy of Otolaryngology hearing stage and vertigo class, gait instability, and results of vestibular-evoked myogenic potentials (VEMP). RESULTS: Four out of the 5 patients had total vertigo control (class A) and 1 had near total control (class B) by the last visit (mean follow-up, 23.6 months). There were no changes in hearing thresholds and hearing stage. Four patients had resolution of their gait instability by 2 months after surgery. Postoperative VEMP responses were preserved in all 3 patients with positive VEMP preoperatively. CONCLUSION: This is the first report of the anatomical and functional preservation of the inferior vestibular nerve in vestibular neurectomy for the treatment of refractory vertigo in unilateral Ménière's disease, with VEMP testing before and after vestibular neurectomy. The modified technique limits the surgical dissection and may help avoid complications such as postoperative hearing loss and persistent gait instability. This approach is indicated when other more conservative measures have failed, and patient selection is paramount to avoid long-term complications.

A schwannoma of the greater petrosal nerve located within the petrous apex and treated with stereotactic radiotherapy.

A 26 year-old female experienced progressive left sided pulsatile tinnitus and conductive hearing loss for two years, which following an extensive clinical workup, was diagnosed as a left greater petrosal nerve schwannoma located within the petrous apex of the temporal bone. Between neurosurgical management and radiation therapy, multiple therapeutic options were presented to the patient, who ultimately chose stereotactic radiotherapy as an alternative to surgical resection due to the potential morbidity associated with surgery. The patient received three fractions of 600 cGy without subsequent worsening of her symptoms, new onset neurologic symptoms or radiation induced side effects reported at a 3, 6 and 12 month clinic visits. A follow-up MRI at 6 and 12 months post radiation administration demonstrated no further tumor growth.

Genotype-phenotype associations in paragangliomas of the temporal bone in a multi-ethnic cohort.

BACKGROUND: Temporal bone paragangliomas are rare tumours with variable presentation that can be hereditary. Identification of clinical and genetic factors of aggressive tumour behaviour is important. OBJECTIVE: To determine the underlying genetic mutations and genotype/phenotype correlations in a multi-ethnic population of South Florida with sporadic temporal bone paragangliomas. METHODS: In a cohort of glomus tympanicum (GT) and glomus jugulare (GJ) cases, we assessed the frequency of pathogenic single nucleotide variants, insertions, deletions, and duplications in coding exons of genes that have been associated with paragangliomas (SDHB, SDHC, SDHD, SDHA, SDHAF2, RET, NF1, VHL, TMEM127, and MAX). RESULTS: None of the 12 GT cases had mutations. Among 13 GJ cases, we identified four mutation carriers (31%); two in SDHC, one in SDHB, and one in SDHD. All patients with pathogenic mutations were of Hispanic ethnicity, presented at a younger age (mean 27.5 versus 52.11 years), and with more advanced disease when compared to mutation-negative GJ cases.Conclusions and Significance: Mutations in the SDH genes are found in 31% of sporadic GJ. SDH-associated GJ had advanced disease and a 50% risk of metastasis. Our data supports emerging recommendations for genetic screening in all populations with GJ tumours as the genetic status informs management.

Ethnic disparity in skin complications following bone-anchored hearing aid implantation.

OBJECTIVES: Sound processor loading after implantation of a bone-anchored hearing aid is often delayed by skin-site complications. This study examined the frequency of skin-site complications in various ethnic groups and determined factors that may lead to higher rates of skin-site complications resulting in delayed processor loading. METHODS: Adult, English-speaking patients who underwent implantation of a bone-anchored hearing aid between 2007 and 2010 were reviewed. Demographic data including ethnicity, tobacco use, diabetes mellitus, immunosuppression, and long-term steroid use were determined. Major and minor skin-site complications and the time to processor loading were recorded. RESULTS: The mean time to processor loading was 9.5 weeks, and the mean follow-up time was 23 months. There were no cases of osseointegration failure. African American patients had a significantly higher rate of major skin-site complications (p < 0.005) and a longer time to processor loading (mean, 17.6 weeks; p < 0.05) than the other ethnic groups. There was no significant difference in minor skin complication rates. There was no correlation between diabetes mellitus, long-term immunosuppression, or tobacco use and skin-site complications. CONCLUSIONS: Skin complications can delay processor loading following implantation of a bone-anchored hearing aid. There is a higher rate of major skin-site complications in African American patients, and these often delay processor loading. The risk of skin-site complications is not correlated with smoking, diabetes mellitus, or immunosuppression. An increased risk of skin-site complications is an important consideration for preoperative counseling.

Prophylactic valacyclovir in a patient with recurrent vestibular disturbances secondary to vestibular neuritis.

A 57-year-old woman with herpes labialis and previously diagnosed with vestibular neuritis experienced recurrences of vertigo and disequilibrium. Initially preceded by oral herpes outbreaks or upper respiratory infections, these recurrences became spontaneous and more frequent. Vestibular function demonstrated a 25% decrease in energy function in the right and the patient had left beating nystagmus on positional maneuver. Her reoccurrences of vestibular disturbances were followed up. Concurrently, she was prescribed daily valacyclovir (500 mg, 1 per day) given for the prevention of herpes labialis outbreaks by her primary care physician. Recurrences of disequilibrium stopped completely as well as oral herpes outbreaks.

Mesenchymal Stem Cells for Treatment of Delayed-Healing Tympanic Membrane Perforations Using Hyaluronate-based Laminas as a Delivery System: An Animal Model With Histopathologic Study.

HYPOTHESIS: Bone marrow derived-mesenchymal stem cells (BM-MSCs) improve the healing of chronic tympanic membrane perforations (cTMPs) in an animal model. BACKGROUND: cTMPs generate significant morbidity and reduced quality of life, usually requiring surgical assistance. With growing interest in alternative therapies, we sought to evaluate the effect of BM-MSC-therapy on the healing of cTMPs. METHODS: Sixty cTMPs were established in C57Bl/6 mice and randomized into four groups: hyaluronate scaffold as graft plus BM-MSCs (n = 19 ears), scaffold plus cell culture media (n = 16), scaffold plus phosphate-buffered saline (PBS, n = 12), and no intervention (n = 13). Hyaluronate scaffolds with or without BM-MSCs were applied on 8-week perforated eardrums. After a blinded assessment of perforation sizes at baseline and 2 weeks after treatment, mean perforation reduction rates (%) were compared. Histology characterization was then performed. RESULTS: Mean perforation size reduction rates were significantly higher for cTMPs that received scaffolds plus BM-MSCs (Student's t test, p = 0.0207, 12.3% [95% CI: 7.8-16.7]) and scaffolds plus cell culture media (p = 0.0477, 11.3% [95% CI: 4.4-18.2]) when compared with no intervention (4.2% [95% CI: 1.2-7.2]). This was not observed when treating eardrums with scaffolds plus PBS (7.3% [95% CI: 2.7-11.9]). On histology, BM-MSC-treated eardrums demonstrated restoration of the trilaminar configuration and reduced inflammatory changes, while other groups developed tissue architecture disorganization and hypercellular infiltrates surrounding the perforation site. CONCLUSIONS: BM-MSCs and cell culture media equivalently increased cTMP healing rates. Cell-based therapy conferred a restoration of the trilaminar configuration of the eardrum with relatively compact and organized fibrous layers.

Safety and Facial Nerve Outcomes of Intracisternal Papaverine Irrigation for Vestibular Schwannoma Resection.

BACKGROUND: Papaverine, a vasodilator approved for use by the U.S. Food and Drug Administration, has shown efficacy in treating vasospasm in cardiology, urology, and nephrology. The vasodilatory effect of papaverine is also hoped to protect the facial nerve from ischemic damage and nerve manipulation during vestibular schwannoma surgery. Our institution uses intracisternal papaverine irrigation during vestibular schwannoma resection to protect the facial nerve in patients with neuromonitoring changes. Our objective was to investigate the safety and facial nerve outcomes of intracisternal papaverine irrigation during vestibular schwannoma resection. METHODS: We retrospectively reviewed patients who underwent resection of vestibular schwannoma at our institution between 2008 and 2021. Patients received papaverine if the intraoperative facial nerve stimulation threshold increased above 0.05 mA. Postoperative outcomes were compared with control patients who did not receive papaverine. RESULTS: A total of 283 cases were included in our analysis. Patients who received papaverine (n = 60) had lower immediate postoperative House-Brackmann (HB) grades than did control individuals (mean, 1.54 vs. 1.95; P = 0.029) and a lower likelihood of immediate postoperative HB grade >1 (odds ratio, 0.514; P = 0.039). At long-term follow-up, there was no significant difference in HB grade. Papaverine use was not associated with increased rates of perioperative complications (P = 0.24). CONCLUSIONS: The off-label use of intracisternal papaverine irrigation during vestibular schwannoma resection can certainly be used safely for select cases. It is associated with improved immediate postoperative facial nerve outcomes, similar long-term facial nerve outcomes, and no significant increase in complications.

Microsurgical Management of Vestibular Schwannoma (Acoustic Neuroma): Facial Nerve Outcomes, Radiographic Analysis, Complications, and Long-Term Follow-Up in a Series of 420 Surgeries.

OBJECTIVE: The purpose of this study is to retrospectively evaluate the clinical and surgical outcomes of a large surgical series of vestibular schwannoma from North America over 20 years. METHODS: After institutional review board approval a retrospective review of the senior author's personal case logs to identify patients who had operations for vestibular schwannoma was performed. The clinical notes, operative record, preoperative and postoperative imagings, and long-term clinical follow-up notes were evaluated. RESULTS: A total of 415 patients who underwent 420 surgeries were identified from the years 1998-2021. The average length of follow-up was 3 years and 9 months. Overall, at last follow-up the rate of "good" facial nerve outcomes (House-Brackmann [HB] score I and II) was 86% and "poor" facial nerve outcomes (HB III-VI) was 14%. The amount of cerebellopontine angle extension (P = 0.023), tumor volume (P = 0.015), facial nerve consistency (P < 0.001), preoperative HB score (P < 0.001), and FN stimulation threshold at the end of the procedure (P < 0.001) were correlated to facial nerve function at the last follow-up. CONCLUSIONS: This study represents one of the largest recently reported surgical series of vestibular schwannoma in North American literature with available long term follow-up. Facial nerve outcomes correlated with cerebellopontine angle extension, tumor volume, facial nerve stimulation threshold, facial nerve consistency, preoperative facial nerve function, and history of a prior resection. Tumor recurrence remains significantly higher after subtotal resection. We believe the data supports a continuation of a strategy of general intent of gross total resection, greatly modifiable by intraoperative findings and judgment.

Catchment Profile of Large Cochlear Implant Centers in the United States.

OBJECTIVE: To characterize the catchment area and patient profile of large cochlear implant (CI) centers in the United States. STUDY DESIGN: Multi-institutional retrospective case series. SETTING: Tertiary referral CI centers. METHODS: Patients who underwent CI surgery at 7 participating CI centers between 2015 and 2020 were identified. Patients' residential zip codes were used to approximate travel distances and urban vs rural residential areas. RESULTS: Over the 6-year study period (2015-2020), 6313 unique CI surgical procedures occurred (4529 adult, 1784 pediatric). Between 2015 and 2019, CI procedures increased by 43%. Patients traveled a median 52 miles (interquartile range, 21-110) each way; patients treated at rural CI centers traveled greater distances vs those treated at urban centers (72 vs 46 miles, P < .001). Rural residents represented 61% of the patient population and traveled farther than urban residents (73 vs 24 miles, P < .001). Overall, 91% of patients lived within a 200-mile radius of the institution, while 71% lived within a 100-mile radius. In adults, multiple regression analysis redemonstrated an association between greater travel distances and (1) older age at the time of CI and (2) residential rural setting (both P < .001, r2 = 0.2). CONCLUSIONS: While large CI centers serve geographically dispersed populations, most patients reside within a 200-mile radius. Strategies to expand CI utilization may leverage remote programming, telemedicine, and strategic placement of new centers and satellite clinics to ameliorate travel burden.

Head-shaking nystagmus predicts greater disability in unilateral peripheral vestibulopathy.

OBJECTIVES: The objective of this study was to determine the association of the bedside test of head-shaking nystagmus (HSN) with patients' self-perceived dizziness handicap as well as this test's sensitivity and specificity in unilateral peripheral vestibular hypofunction. STUDY DESIGN: A retrospective case-control study was performed. SETTING: The study was held at an academic, tertiary referral center. METHODS: Fifty-three adult patients with unilateral peripheral hypofunction defined by the caloric test of the videonystagmography with documented bedside HSN and who had completed questionnaires of self-perceived dizziness handicap were included. The sensitivity and specificity of the bedside HSN in patients and 10 healthy controls in diagnosing unilateral vestibular hypofunction defined by videonystagmographic caloric testing and by abnormal gain and symmetry of the vestibular-ocular reflex by rotary chair testing were determined. Scores of the screening test of the Dizziness Handicap Index and Functional Level Scale questionnaires were taken. RESULTS: When using the caloric irrigation test as the reference standard for unilateral vestibular hypofunction, the sensitivity, specificity, and positive predictive value of the bedside HSN were 31%, 96%, and 97%, respectively. When comparing with results of rotational chair testing (vestibular-ocular reflex gain and symmetry), the sensitivity of the HSN test increases to 71%. Patients with positive bedside HSN had higher scores (greater self-perceived dizziness handicap) of the Dizziness Handicap Index (P = .049) and higher (worse) scores of the Functional Level Scale (P = .0377) than those with negative bedside HSN (Wilcoxon rank test). CONCLUSIONS: Greater perceived handicap was correlated with a positive bedside HSN in patients with unilateral peripheral vestibulopathy. The HSN has sufficient sensitivity to be used as screening test of uncompensated vestibulopathy in this series. However, a negative HSN alone does not rule out the diagnosis of peripheral vestibular dysfunction.

The Epidemiological and Financial Effects of Pneumococcal Vaccination on Otitis Media Related Admissions in the United States.

OBJECTIVE: To quantify the prevalence of hospital admissions, the financial impact, and the trends in surgical procedure rates for AOM and CAOM for all ages before and after 13-valent pneumococcal conjugate vaccine (PCV13) introduction. METHODS: Retrospective analysis of the National Inpatient Sample (NIS) from 1998 to 2013 to determine the prevalence of AOM/CAOM related admissions and weighted frequencies of AOM/CAOM related International Classification of Diseases, ninth revision (ICD-9) hospital diagnoses. Prevalence of surgical procedures to treat CAOM, cost of admission, length of stay, and cost per day of admission were tabulated. Trend analysis of this data was performed. RESULTS: A total of 46 580 patients were hospitalized with AOM in the designated time period, of which 37 366 had CAOM. The prevalence of hospital admission due to AOM had the most pronounced decrease from pre-vaccine era (1998) to post-PCV13 implementation (2013) in age group 0 to 4 (32%) followed by age group 5 to 19 (7%). Age groups 20-64 and 65+ showed slight increases in prevalence. The trend in prevalence of admissions due to CAOM mirrors that of overall admissions with an 18% and 5.8% decrease in age groups 0-4 and 5-19, respectively, and a 1% increase in ages 20+. The inflation adjusted mean cost of admission did not significantly increase between 2001 and 2013. The total cost per admission was $4428 and $7546 for those with AOM and CAOM, respectively. Mastoidectomy rates increased by 17% in hospitalized children during the post-vaccine era but decreased in the elderly population. CONCLUSION: The prevalence of AOM/CAOM hospital admissions decreased from the pre-vaccine era (1998) to post-PCV13 implementation (2013) in pediatric patients. Surgical procedure utilization and cost of hospital admission for AOM/CAOM did not increase throughout the study period.

Malleus handle: determinant of success in ossiculoplasty.

OBJECTIVE: The purpose of this study is to identify prognostic factors affecting outcome in ossicular chain reconstruction (OCR). STUDY DESIGN AND SETTING: This study is a retrospective case series of electronic database at an academic institution. MATERIALS AND METHODS: We reviewed 209 cases of chronic supportive otitis media performed from January 2000 through December 2007 and collected demographic, clinical, audiologic, and outcome information. Univariate analyses of group differences in terms of postoperative air-bone gap (ABG) changes were evaluated by analysis of variance. Multiple regression analyses were used to examine the relationship between postoperative ABG and the independent variables. RESULTS: There were 105 cases of OCR the met the inclusion criteria (44 primary and 61 revision tympanoplasties), with an average follow-up of 19 months. The diagnoses were chronic suppurative otitis media without cholesteatoma in 36 cases and cholesteatoma in 69 cases. The mean preoperative ABG was 34 +/- 15 dB, and the mean postoperative ABG was 20 +/- 14 dB (P < 0.001). Of the independent variables analyzed, the type of procedure (ie, OCR performed during second-look tympanoplasty vs canal wall up vs canal wall down), preoperative ABG, and status of malleus handle were predictive of the success of OCR. CONCLUSIONS: Favorable prognostic factors in OCR include smaller preoperative ABG and the presence of an intact malleus handle.

A clinical protocol for predicting outcomes with an implantable prosthetic device (Baha) in patients with single-sided deafness.

BACKGROUND: The Baha implant is increasingly becoming a common form of treatment for individuals with single-sided deafness (SSD). However, evidence-based guidelines for determining candidacy in these patients are not yet established. PURPOSE: The purpose of this study was to investigate the clinical utility of speech-in-noise testing as a part of the preoperative evaluation of the Baha device in patients with SSD. RESEARCH DESIGN: The study design was a prospective cohort of 24 English-speaking adults comparing preoperative results on speech-in-noise measures using the Baha Cordelle II headband stimulator to postoperative results using the patient's external Baha processor. INTERVENTION: Outcome measures included signal-to-noise ratio (SNR) loss as measured by the QuickSIN™ and scores of self-reported disability questionnaires. RESULTS: Wilcoxon signed-rank test resulted in no significant difference between the preoperative and postoperative methods for measuring benefit on listening in noise tasks. Passing Bablok regression analysis showed the preoperative and postoperative results to be statistically equivalent, which suggests that postoperative results can be predicted during preoperative testing. Wilcoxon signed-rank test showed significant improvements in self-reported disability postoperatively. CONCLUSIONS: The results support the use of speech-in-noise measures as an accurate predictor of overall benefit in patients with SSD prior to implantation.

Animal Model of Chronic Tympanic Membrane Perforation.

Chronic tympanic membrane perforations (TMP) can be a source of significant morbidity from hearing loss, recurrent middle ear infections, changes in lifestyle, and risk of cholesteatoma formation. Laboratory experiments of TMP have been fraught by the rapid and high rate of spontaneous healing observed in animal models. There is controversy on the minimal time that perforations in animal models must have in order to be considered chronic TMP and thus have clinical relevance, with authors suggesting time periods of perforation patency of 8-12 weeks. In this article, we sought to create a clinically significant experimental model that could yield a high rate of perforation patency for at least 8 weeks. Animals undergoing acute TMP were exposed to three different experimental situations to delay the healing of the perforation: fractionated radiation, topical lipopolysaccharide application, and a combined dexamethasone and mitomycin C (DXM/MC) solution. In our study, the use of DXM/MC reliably produced TMP lasting at least 8 weeks in 86.48% of the cases without the need to reopen the perforation, infolding the edges of the membrane, or using physical barriers to prevent TMP closure. Histologically, the resulting perforated tympanum showed hyaline changes of the remnant tympanum and hyperkeratosis of the squamous epithelia of the external auditory canal. We believe that this model is reproducible and has potential use in experiments of delayed healing of TMP. Anat Rec, 303:619-625, 2020. © 2019 American Association for Anatomy.

Imaging correlation of children with DFNB1 vs non-DFNB1 hearing loss.

OBJECTIVES: To evaluate temporal bone CT findings in GJB2-related deafness (DFNB1) hearing loss and non-DFNB1 hearing loss children. STUDY DESIGN: Case-control series. SUBJECTS AND METHODS: Children with nonsyndromic hearing loss diagnosed as DFNB1 or non-DFNB1 after screening GJB2 allele variants and the large GJB6 deletion. Temporal bone CT images compared in a cohort of nine DFNB1 children with 10 non-DFNB1 children. Visual criteria and absolute measurements were compared against established normative values. RESULTS: Visual inspection failed to identify two patients with abnormalities identified by using absolute measurements. Only one of nine DFNB1 children had an ear anomaly versus seven of 10 non-DFNB1 (odds ratio 16.33; 95% CI, 1.35, 197.78; P = 0.050). The non-DFNB1 group had a mean vestibule width that was significantly larger, and a mean lateral semicircular canal island width and vestibular aqueduct that were significantly smaller than the DFNB1 group. CONCLUSIONS: Visual inspection of temporal bone CT images alone may not identify all anomalies and should be used with absolute CT measurements. Abnormal temporal bone CT findings are significantly less likely in children with DFNB1 compared with non-DFNB1 children despite similar age and degree of hearing loss.

Cervical VEMP tuning changes by Meniere's disease stages.

OBJECTIVE: To determine if changes in cervical vestibular-evoked myogenic potential (cVEMP) testing reflect the different stages of cochlea-saccular hydrops in Meniere's disease (MD). METHODS: This is a case-control retrospective series. Forty-seven patients with unilateral MD by American Academy of Otolaryngology-Head and Neck Surgery diagnostic and staging criteria, and 30 with non-MD vertigo as control. Meniere patients were further classified based on symptoms at the time of testing as active or stable. Subsequently, patients underwent cVEMP testing by tone-burst stimuli at 500 and 1,000 Hz. The main outcome measure was to compare the cVEMP 1,000 and 500 Hz amplitude ratio in ears with MD and non-MD vertigo, and in active versus stable MD. RESULTS: The cVEMP 1,000/500 Hz amplitude ratio was higher in Meniere's ears (mean = 1.14 µV, SD = 0.25) than in non-Meniere's ears (mean = 0.96 µV, SD = 0.2) (Student's t test, P = .001), and higher in active (mean = 1.22 µV, SD = 0.25) than in stable MD (mean = 1.00 µV, SD = 0.18) (P = .0035). The diagnostic value of cVEMP 1,000/500 Hz amplitude ratio to differentiate MD versus non-MD vertigo was evaluated with a receiver-operating characteristics (ROC) curve and the area under the curve (AUC) was 0.716 (95% confidence interval [CI] [0.591, 0.829]). The ideal cutoff point was 0.9435 with sensitivity and specificity values of 83% and 53%, respectively. The sensitivity and specificity values for this test to differentiate active versus stable MD were 68% and 81%, respectively, with AUC 0.746 (95% CI [0.607, 0.885]) and cutoff value of 1.048. In all ears, the 1,000/500 Hz amplitude ratio increased by a decrease of the 500 Hz amplitude with increasing age. CONCLUSION: The cVEMP 1,000/500 Hz amplitude ratio is elevated in ears with MD but not in those with non-MD vertigo. After corrected by age, this ratio is higher in active but not in stable MD, probably reflecting dynamic changes in saccular membrane motion mechanics in hydrops, and may be a useful marker of disease progression and the effect of therapy. LEVEL OF EVIDENCE: IV.

The pattern of hearing outcome following surgery of the semicircular canals.

OBJECTIVE: To analyze demographic, clinical, surgical, and audiometric factors that may affect hearing outcome following surgery for the semicircular canals (SCC). METHOD: This is a retrospective case review of adults who underwent surgeries for superior SCC (SSCC), lateral SCC (LSCC), or posterior SCC (PSCC) and whose data were extracted and analyzed for factors affecting the hearing outcome in these procedures. RESULTS: Thirteen patients underwent surgery for SSCC, seven cases for the LSCC, one for the PSCC, and one case of combined PSCC/SSCC surgery. The mean age was 49.8 ± 12 years (21-66). There was no difference between the preoperative and postoperative pure tone average (PTA) thresholds at 0.5-3 kHz. Higher thresholds were noted at 4, 6, and 8 kHz postoperatively. Deterioration (>10 dB) in the bone-conduction (BC) PTA was demonstrated in 3 of 22 (13.6%) cases with no significant difference in the demographic, clinical, surgical, and preoperative audiometric parameters relative to the cases without PTA BC change. A significantly larger difference in PTA BC (pre- vs. postoperative) was seen for males. Small effect size was noted for Air conduction (AC) PTA in males, and moderate effect size for Word Recognition Score (WRS) in surgery for the LSCC compare to SSCC. CONCLUSIONS: SCC surgeries carry a relatively low risk of deterioration in PTA BC. High frequency thresholds should also be included in postoperative hearing outcome assessment. Cases of LSCC for intractable Meniere's disease and surgery in males carry higher risk of poor postoperative hearing outcomes. Level of Evidence: 4.

Caloric function after endolymphatic sac surgery.

OBJECTIVE: To evaluate the effect of endolymphatic sac surgery on vestibular functions using caloric testing on electronystagmography (ENG). STUDY DESIGN: Retrospective chart review. METHODS: The medical records of 21 adult patients with unilateral Ménière's disease who underwent endolymphatic sac surgery between 1998 and 2004 were reviewed. With use of ENG, the absolute value of the caloric response of the operated ear (i.e., the cool + warm irrigation response) and the degree of reduced vestibular response (RVR) rates as indicators of caloric functions were compared before and after surgery. Average follow-up was 17 (6-52) months. RESULTS: The mean change in RVR after surgery was found to be 2.9%. In total, there were six (28.5%) patients who had an RVR increase more than 10%. Of those, there were three (14.2%) patients who demonstrated an RVR increase more than 20%. Only one (4.8%) patient had an RVR increase more than 30%. Total loss of vestibular function was not observed in any of the patients. There were three (14.2%) patients who exhibited a decrease of more than 10% in their RVR. In two (9%) patients, the contralateral ear was shown to have less vestibular function than the operated side on ENG postoperatively (in one case, the absolute caloric nystagmus response remained the same in the operated ear, and in the other case, the response increased on the surgical side). When we evaluated the absolute caloric responses of the operated ear only, we found no statistical difference between the pre- and postoperative values (P = .219). Early results of vertigo control and hearing outcomes were comparable with those in the literature. CONCLUSION: Endolymphatic sac surgery does not appear to be a vestibular destructive procedure, and it is a therapeutic alternative for patients with Ménière's disease who have failed medical treatment. This is important given the possibility of bilateral disease in some patients.