Vestibular Hypofunction Screening in Older Cochlear Implant Candidates.

OBJECTIVE: Vestibular loss is associated with increasing age and hearing loss. Cochlear implantation (CI) may be performed in these patients; however, CI can induce vestibular hypofunction (VH) postoperatively. If CI is performed in the "better balancing ear," patients may experience vestibulopathy from new bilateral VH. The objective of this study was to evaluate the rates of VH in older CI candidates, thereby helping to identify patients at increased risk for bilateral VH after CI. STUDY DESIGN: Retrospective cohort study from 2019 to 2022 of patients age 60 to 80 years old who underwent videonystagmography (VNG). SETTING: Tertiary care neurotology practice. METHODS: VNG, including spontaneous nystagmus, cervical vestibular evoked myogenic potential (cVEMP), caloric stimulation, and rotary chair, was reviewed and stratified by CI candidacy. Patients with prior CI or known vestibular diagnosis were excluded. RESULTS: Thirty-three patients were CI candidates and 184 patients were controls. cVEMP demonstrated a significantly greater rate of unilateral VH in CI candidates (P = .018). Caloric stimulation demonstrated an elevated rate of bilateral VH and presbyvestibulopathy in CI candidates (P = .057 and P = .036, respectively). Rotary chair demonstrated a significantly higher rate of bilateral VH and incomplete vestibular compensation based on reduced gain and gain asymmetry, respectively, in CI candidates (P < .001 and P = .043, respectively). Mean bithermal slow phase velocity sum and rotary chair gain were significantly lower in the CI candidate group (P = .002 and P < .001, respectively). Preoperative identification of VH determined the side of implantation in 4 patients (15%). CONCLUSION: VH and incomplete vestibular compensation are common and more frequently seen in CI candidates compared to age-matched controls. Vestibular screening can play a role in surgical counseling and planning, and should be considered in older patients undergoing CI.

Isolated Fracture of the Malleus: An Overlooked Cause of Conductive Hearing Loss?

OBJECTIVE: To report the largest case series of isolated malleus fractures with systematic review to characterize the disease's presentation and natural history, and provide suggestions for management. DATA SOURCES: PubMed, Embase, Cochrane Library. REVIEW METHODS: Retrospective cohort study was performed on 12 patients with isolated malleus fractures. History, physical exam, pre- and post-treatment audiograms, and imaging were obtained. Systematic review of the literature was performed. RESULTS: Including the cases herein, 58 isolated malleus fractures were identified, the majority of which were published in the 21st century. Mean time to presentation after injury was 34.4 months. Most common etiology was external auditory canal (EAC) manipulation. Physical exam and imaging did not identify any abnormality at presentation in 16% and 21% of cases, respectively. The majority of fractures involved the manubrium. Air-bone gap (ABG) at initial presentation ranged from 16 to 26 dB, and was greater at higher frequencies. Thirty-six cases underwent surgery. ABG improvement was greater at all frequencies for those who underwent surgery. Final ABG was significantly less than initial ABG at nearly every frequency for those who underwent surgery (p < 0.05), while not at any frequency for those who were observed. CONCLUSIONS: Isolated malleus fractures may occur more often than historical data suggests, and are perhaps underdiagnosed. Abrupt removal of a finger from the EAC with pain and hearing loss is nearly pathognomonic. Conductive hearing loss with ABG greater at higher frequencies is most often observed. Observation is unlikely to produce spontaneous improvements in hearing, while surgery demonstrates reliable decreases in ABG. Laryngoscope, 134:1032-1041, 2024.

Ménière's disease is a disorder of the inner ear.

PURPOSE OF REVIEW: Ménière's disease is a disorder characterized by recurrent episodes of vertigo, hearing loss, tinnitus, and pressure in the ear. Its pathogenesis and optimal management continue to be the subject of ongoing debate. Although it is classically believed to be a primary disorder of the inner ear, some clinicians have hypothesized that it is a form of vestibular migraine. RECENT FINDINGS: Evidence supporting vestibular migraine in the differential of Ménière's disease includes overlap of symptoms, high prevalence of migraines in patients with Ménière's disease, and the efficacy of migraine treatments for symptoms of Ménière's disease in some patients. However, there is far more evidence to support Ménière's disease as a disorder of the inner ear, including genetic and histologic changes of hydrops, imaging studies showing hydropic changes, predominance of low-frequency hearing loss, clinical efficacy of treatments targeted at inflammatory change/hydrops, and clinical efficacy of ablating the vestibular end-organ. SUMMARY: Although there is conflicting evidence regarding the cause and treatment of Ménière's disease, current evidence favors it as a disorder of the inner ear. Further research is needed to fully understand the mechanisms of Ménière's disease and how to improve diagnosis and treatment outcomes.

Hearing Outcomes After Surgical Manipulation of the Membranous Labyrinth During Superior Semicircular Canal Dehiscence Plugging or Posterior Semicircular Canal Occlusion.

OBJECTIVE: To analyze audiometric data after surgical manipulation of the membranous labyrinth during plugging of superior semicircular canal dehiscence (SSCD) or posterior semicircular canal occlusion (PSCO) for benign paroxysmal positional vertigo. STUDY DESIGN: Retrospective chart review. SETTING: Tertiary referral center. PATIENTS: Patients undergoing plugging of SSCD or PSCO between 2009 and 2019. MAIN OUTCOME MEASURES: Pre- and postoperative audiometric data were collected per AAO-HNS guidelines. Hearing outcomes at initial and last follow-up were compared. Subanalyses were performed for surgical approach and age. RESULTS: Eighty-seven total procedures in 76 patients including 43 middle cranial fossa for SSCD, 29 transmastoid SSCD, and 15 PSCO. Mean preoperative air-conduction-pure-tone averages was 21.1±14.9 dB compared with 26.1 ±â€Š19.6 dB at initial follow-up and 24.4 ±â€Š18.6 dB at last follow-up (p = 0.006). Mean preoperative bone-conduction-pure-tone average was 14.3 ±â€Š11.9 dB compared with 18.3 ±â€Š15.6 dB at initial follow-up and 18.5 ±â€Š16.9 dB at last follow-up (p < 0.001). There were five cases of hearing loss >20 dB including one case of profound sensorineural hearing loss >55 dB. PSCO resulted in the most hearing loss at initial follow-up but largely resolves with time. Transmastoid approaches for SSCD resulted in more hearing loss compared with middle cranial fossa. Hearing outcomes were generally stable for SSCD approaches but showed improvement over time for PSCO. Age >50 was associated with greater hearing loss of 5.2 ±â€Š11.1 dB compared with 1.3 ±â€Š10.5 dB but did not reach statistical significance (p = 0.110). CONCLUSIONS: Surgical manipulation of the membranous labyrinth results in statistically significant hearing loss in a pooled analysis. Transient hearing loss is observed in PSCO and TM SSCD plugging was associated with postoperative hearing loss. There was a trend toward increased hearing loss in patients >50 years old.

Pre-existing Vestibular Symptoms Are Associated With Acute Vestibular Symptoms After Gamma Knife Therapy for Vestibular Schwannoma.

OBJECTIVE: To identify pretreatment variables associated with the development of acute vestibular symptoms after Gamma Knife (GK) treatment for Vestibular Schwannoma (VS). STUDY DESIGN: Retrospective case series. SETTING: Tertiary neurotology referral center. PATIENTS: Patients treated with GK radiosurgery for VS between March 2007 and March 2017 were considered for this study. Patients with neurofibromatosis type II, previous VS surgery, follow-up less than 6 months, or the lack of T2 magnetic resonance imaging (MRI) sequences from the day of treatment were excluded. MAIN OUTCOME MEASURES: The presence of acute vestibular symptoms arising within 6 months after GK was the main outcome variable. Tumor, patient, and treatment characteristics were gathered from the medical record. RESULTS: In total, 98 patients met inclusion criteria. The incidence of acute vestibular symptoms occurring within 6 months after GK treatment was 46.9%. Post-GK vestibular symptoms were reported at a significantly higher frequency among subjects who had reported vestibular symptoms before their treatment (p = 0.001). Tumor size was not associated with a propensity to develop acute vestibular symptoms (p = 0.397). The likelihood of receiving a referral to vestibular rehabilitation services was not significantly different among patients with larger versus smaller tumor size, as defined by 1.6 cm and 1.4 cm thresholds (p = 0.896, p = 0.654). CONCLUSIONS: Inquiries aimed at revealing a history of vestibular complaints may prove useful in counseling patients on the likelihood of experiencing acute vestibular symptoms after treatment of Vestibular Schwannoma with Gamma Knife therapy.

Hearing Outcomes With a Novel Total Ossicular Replacement Prosthesis.

INTRODUCTION: A total ossicular replacement prosthesis (TORP) is used to reconstruct the ossicular chain in the absence of the stapes suprastructure. The Wildcat prosthesis is a novel TORP that eliminates the need for a separate footplate shoe prosthesis and aims to improve ease-of-use and stability. This study evaluates hearing outcomes using the Wildcat prosthesis. STUDY DESIGN: Case series with chart review. SETTING: Tertiary neurotology referral center. METHODS: Retrospective chart review of 64 patients undergoing ossicular chain reconstruction using the Wildcat TORP. Hearing outcomes after surgery were assessed with air conduction pure-tone average, bone conduction pure-tone average, air-bone gap (ABG), speech recognition threshold , and word recognition score as primary outcome measures. The stability of hearing outcomes was evaluated on subsequent long-term follow-up. RESULTS: At mean short-term follow-up of 4.4 ±â€Š2.7 months, ABG improved from 31.0 ±â€Š13.0 dB preoperatively to 22.5 ±â€Š10.0 dB (p < 0.001) with 51.6% achieving ABG less than 20 dB. No significant difference in any primary outcome measures was found when analyzing outcomes by initial versus revision surgery, use of cartilage graft, or type of mastoidectomy. The only exception was a smaller reduction in ABG of 4.2 dB for patients with canal wall down mastoidectomy compared with a 13.7 dB ABG closure in patients with canal wall up mastoidectomy (p = 0.039). CONCLUSION: Total ossicular chain reconstruction using the Wildcat demonstrates versatility in challenging cases to provide hearing outcomes that are comparable to published data using TORPs.

A Protective Cap: Fundal Fluid Cap Facilitates a Reduction in Inner Ear Radiation Dose in the Radiosurgical Treatment of Vestibular Schwannoma.

OBJECTIVE: Assess inner ear radiation dose magnitude as it relates to fundal cap length and hearing outcomes in the radiosurgical treatment of vestibular schwannoma. STUDY DESIGN: Retrospective case series. SETTING: Tertiary neurotology referral center. PATIENTS: Patients treated with Gamma Knife radiosurgery for vestibular schwannoma between March 2007 and March 2017 were considered for this study. Exclusion criteria included pretreatment pure-tone average (PTA) >90 dB, neurofibromatosis type II, history of previous surgical resection, and follow-up less than 1 year. MAIN OUTCOME MEASURE: (s): Hearing function was assessed by maintenance of class A/B hearing level and maintenance of baseline hearing (≤20 dB change in PTA following Gamma Knife radiosurgery). RESULTS: Lower radiation doses delivered to the inner ear were associated with longer fundal cap lengths: mean cochlear dose (r = -0.130; p = 0.184), mean labyrinth dose (r = -0.406; p < 0.001), max cochlear dose (r = -0.326; p = 0.001), and max labyrinth dose (r = -0.360; p < 0.001). Kaplan-Meier analysis with log-rank testing revealed that patients with a mean labyrinth dose < 3 Gy achieved higher rates of preserving baseline hearing (≤20 dB change in PTA) following radiosurgery, compared to patients with a mean labyrinth dose ≥3 Gy (p < 0.001). A fundal fluid cap length of 2.5 mm was associated with the 3 Gy mean labyrinth dose threshold. CONCLUSIONS: We report that fundal cap presence facilitated the creation of treatment plans with a lower dose delivered to the labyrinth. By affording this dose reduction, a fundal cap may be associated with a slight improvement in hearing outcomes.

Fundal Fluid Cap Is Associated With Hearing Preservation in the Radiosurgical Treatment of Vestibular Schwannoma.

OBJECTIVE: To evaluate the relationship between fundal fluid and hearing outcomes after treatment of vestibular schwannoma (VS) with Gamma Knife radiosurgery (GKRS). STUDY DESIGN: Retrospective case series. SETTING: Tertiary neurotology referral center. PATIENTS: Patients treated with GKRS for vestibular schwannoma between March 2007 and March 2017 were considered for this study. Exclusion criteria included pretreatment pure-tone average (PTA) >90 dB, neurofibromatosis type II, history of previous surgical resection, and follow-up less than 1 year. MAIN OUTCOME MEASURE(S): Hearing function was assessed both by preservation of serviceable hearing and by preservation of baseline hearing (≤20 dB change in PTA) after GKRS. Hearing preservation comparisons were made between groups of patients with and without a fundal fluid cap. RESULTS: Patients with a fundal cap had significantly higher rates of baseline hearing preservation (≤20 dB change in PTA) according to Kaplan-Meier survival analysis of all 106 patients (p = 0.006). By the 3rd year posttreatment, 70.9% of patients with a fundal cap had maintained a ≤20 dB change in PTA, while only 43.6% of patients without a fundal fluid cap achieved this outcome (p = 0.004). CONCLUSIONS: Fundal fluid present on pretreatment magnetic resonance imaging is predictive of improved baseline hearing preservation rates in patients undergoing GKRS for vestibular schwannoma when considering all patients with PTA ≤90 dB. Fundal fluid cap presence may serve as a favorable prognostic indicator to help set hearing expectations and guide patient selection efforts.

A Critical Analysis of the Information Available Online for Ménière's Disease.

OBJECTIVE: Patients increasingly rely on online resources for medical information; however, the Internet is unregulated and prone to misinformation. This study analyzes the reliability, quality, and readability of websites for Ménière's disease. STUDY DESIGN: A Google search was performed using keywords Ménière's disease. The first 5 pages (50 results) were reviewed. Websites were sorted into 5 categories: academic institutions, government agencies, professional organizations, medical information websites, and miscellaneous. The reliability, quality, and readability of each website were evaluated using the DISCERN instrument and Flesch-Kincaid Grade Level (FKGL). DISCERN assesses reliability and quality by scoring 15 questions on a scale from 1 (low) to 3 (high). The reliability score emphasizes clear objectives and sources, as well as lack of bias, whereas the quality score emphasizes information on treatment options. The FKGL of each website was calculated using a formula to determine the equivalent US grade reading level. SETTING: Ambulatory. SUBJECTS: None. RESULTS: Forty-two websites were analyzed. Academic institutions were the most common (n = 13, 31%) but scored the lowest using DISCERN at 1.75 ± 0.13. Medical information websites scored highest at 2.24 ± 0.09 (P = .024 compared to academic institutions). The average FKGL of all websites was 10.12 ± 0.57 with medical information websites being the easiest to read at 8.84 ± 0.83. Only 5 (13%) of websites scored below the eighth-grade reading level. CONCLUSIONS: Most top online search results for Ménière's disease are deficient in quality and readability. Medical information websites are generally the most reliable and easy to read.

Surgical Explantation of Bone-Anchored Hearing Devices: A 10-year Single Institution Review.

OBJECTIVE: To identify clinical variables associated with the decision to surgically discontinue bone-anchored hearing device function. STUDY DESIGN: Retrospective chart review. SETTING: Tertiary neurotology referral center. SUBJECTS AND METHODS: This study examines surgical interventions performed on existing bone-anchored hearing devices at a single institution from 2008 to 2018. Patient characteristics, indications for implantation, and complications prompting surgical intervention were assessed. RESULTS: Seventy-seven cases were included in this study. Among patients in the younger cohort (<37 years old), 100% (13 of 13) of those discontinuing their device had a contralateral normal-hearing ear. Conversely, 0% (0 of 14) of the younger patients with bilateral hearing loss surgically discontinued their devices. Within the older cohort (≥37 years old), female patients (P = .002) and those with an increased body mass index (P = .035) were more likely to surgically discontinue their devices. Multivariate analysis revealed that a contralateral normal-hearing ear (P = .001) and infection without soft tissue overgrowth of the abutment (P = .026) were the strongest predictors of device discontinuation, after adjusting for potential confounders. CONCLUSION: Surgical discontinuation is associated with several clinical variables. Targeted interventions that are viable alternatives to removal, such as device relocation, should be presented to younger patients with a contralateral normal-hearing ear who experience persistent complications. Patients with persistent infection in the absence of soft tissue overgrowth would especially benefit from enhanced counseling on proper hygiene.

Incidence of Prolonged Systemic Steroid Treatment after Surgery for Acoustic Neuroma and Its Implications.

Objectives To determine the incidence of prolonged postoperative systemic corticosteroid therapy after surgery for acoustic neuroma as well as the indications and associated risk factors that could lead to prolonged steroid administration, and the incidence of steroid-related adverse effects. Study Designs Retrospective chart review. Methods Retrospective chart review of patients undergoing resection of acoustic neuroma between 2010 and 2017 at two tertiary care medical centers. Patient and tumor characteristics, operative approach, hospital length of stay, initial postoperative taper length, number of discrete postoperative steroid courses, and postoperative complications were analyzed. Results There were 220 patients (99 male, 121 female) with an average age of 49.4 (range 16-78). There were 124 left-sided tumors and 96 right-sided tumors. Within the group, 191 tumors were operated through a retrosigmoid approach, 25 tumors through a translabyrinthine approach, and 4 tumors with a combined retrosigmoid-translabyrinthine approach under the same anesthetic. In total, 35 (15.9%) patients received an extended initial course of postoperative systemic steroids, defined as a taper longer than 18 days. Twenty six (11.8%) patients received additional courses of systemic steroids after the initial postoperative taper. There were 5 (2.3%) patients who required an extended initial taper as well as additional courses of steroids. Aseptic meningitis, often manifested as headache, was the most common indication for additional steroids (14 cases of prolonged taper and 17 cases of additional courses). None of the patient or tumor factors including age, gender, side, size, and approach were statistically significantly associated with either a prolonged initial steroid taper or additional courses of steroids. An extended hospital length of stay was associated with a prolonged initial steroid taper ( p = 0.03), though the initial taper length was not predictive of additional courses of steroids. The cumulative number of days on steroids was associated with need for additional procedures ( p < 0.01) as well as steroid-related side effects ( p = 0.05). The administration of steroids was not found to significantly improve outcomes in postoperative facial paresis. Steroid-related complications were uncommon, seen in 9.26% of patients receiving steroids, with the most common being psychiatric side effects such as agitation, anxiety, and mood lability. Conclusions Systemic corticosteroids are routinely administered postoperatively for patients undergoing craniotomy for the resection of acoustic neuromas. In a review of 220 patients operated by a single neurotologist, no patient or tumor factors were predictive of requiring prolonged initial steroid taper or additional courses of steroids. The cumulative number of days on systemic steroids was associated with undergoing additional procedures and steroid-related side effects. The most common indications for prolonged or additional steroids were aseptic meningitis, cerebrospinal fluid leak, and facial paresis. Additional steroids for postoperative facial paresis did not significantly improve outcomes. Patient-reported steroid-related complications were infrequent and were most commonly psychiatric including agitation, anxiety, and mood lability.

Stapes prosthesis length and hearing outcomes.

OBJECTIVES: First, to determine if prosthesis length plays a role in optimizing successful hearing outcomes in stapedotomy surgery; and second, to determine if patient factors such as height are correlated with prosthesis length. STUDY DESIGN: Retrospective chart review. METHODS: Retrospective chart review of patients undergoing stapedotomy surgeries with adequate follow up. Length of prosthesis, pre-/postoperative audiograms, and follow-up data were obtained. RESULTS: The primary group consisted of 227 cases. The prosthesis length ranged from 3.75 mm to 4.75 mm (median 4.25 mm). The greatest improvement in postoperative air-bone gap (ABG) occurred in the 4.25 mm group, and the least in the 3.75 mm group. Patient height showed a positive, although weak, correlation with prosthesis length. No findings reached statistical significance. CONCLUSION: Accurate measurement of prosthesis length is important for successful postoperative hearing outcomes. There is a positive but not significant correlation between patient height and prosthesis length. LEVEL OF EVIDENCE: 4. Laryngoscope, 128:722-726, 2018.

Stapedotomy With Adipose Tissue Seal: Hearing Outcomes, Incidence of Sensorineural Hearing Loss, and Comparison to Alternative Techniques.

OBJECTIVE: To evaluate hearing outcomes and incidence of sensorineural hearing loss (SNHL) after stapedotomy with adipose tissue seal, and to compare adipose tissue sealing with alternative techniques. STUDY DESIGN: Retrospective chart review. SETTING: Tertiary referral center. PATIENTS: Primary stapedotomy was performed in 179 patients using a KTP laser, microdrill, piston prosthesis, and adipose tissue seal of the oval window fenestra between 1991 and 2015. An additional 123 patients underwent stapes surgery with alternative techniques. INTERVENTIONS: Stapes surgery for otosclerosis. Audiograms were performed preoperatively and 3 months postoperatively. MAIN OUTCOME MEASURES: Hearing outcomes were measured as percentage of cases with closure of the air-bone gap (ABG) to within 10 and 15 dB. SNHL was identified as cases with decrement in bone conduction thresholds greater than 10 or 5 dB. The median postoperative ABG and change in bone conduction thresholds were compared between the primary stapedotomy group (KTP laser with adipose tissue seal) and various subgroups with alternative techniques. RESULTS: We found excellent hearing outcomes after primary stapedotomy with adipose tissue seal. The ABG closed to within 10 dB in 81.01% of cases and within 15 dB in 93.30% of cases. We observed a low rate of SNHL without any cases of profound hearing loss. 1.67% of cases had a decrement in bone conduction greater than 10 dB while 6.14% of cases had a decrement greater than 5 dB. Stapedotomy with adipose tissue seal compared favorably with alternative techniques with respect to hearing outcomes and SNHL. CONCLUSIONS: Adipose tissue is an effective and cost-efficient tissue seal during stapedotomy with excellent hearing outcomes and low incidence of SNHL.

The Tightrope Facial Nerve-An Unsupported Mastoid Segment After Resection of Recidivistic Cholesteatoma.

OBJECTIVES/HYPOTHESIS: Cholesteatomas are epidermal inclusion cysts containing stratified squamous epithelium that arise in the middle ear and mastoid cavities resulting in a persistent inflammatory state. Complications include chronic otorrhea, granulation tissue, and bony erosion. Cholesteatoma growth patterns predict frequent involvement of the Fallopian canal of the facial nerve. Extensive disease may extend to the posterior and middle fossa dura, the otic capsule, the carotid artery, and the jugular bulb. Dehiscence of the Fallopian canal and direct involvement of the facial nerve epineurium by cholesteatoma are risk factors for intraoperative facial nerve injury during tympanomastoid surgery by exposing the facial nerve to mechanical trauma and inflammation during microdissection. We present two cases of recidivistic cholesteatoma with unusual medial involvement of the vertical segment of the facial nerve that resulted in an unsupported mastoid segment after resection. The outcomes from these two patients suggest that facial nerve function may be preserved despite circumferential exposure with appropriate preoperative planning and intra-operative techniques. STUDY DESIGN: Case studies. METHODS: Two patients undergoing revision tympanomastoidectomy for extensive recidivistic cholesteatoma with medial involvement of the facial nerve in the mastoid segment. In each case, the facial nerve was circumferentially exposed during the resection of the cholesteatoma. RESULTS: Normal facial nerve function after surgery was confirmed in both patients at 18 months and 6 years, respectively. CONCLUSIONS: Surgical resection of extensive cholesteatoma with medial involvement of the vertical segment of the facial nerve resulted in an unsupported mastoid segment in two patients with normal postoperative facial nerve function.

GDNF overexpression fails to provoke muscle recovery from botulinum toxin poisoning: a preliminary study.

Glial cell line-derived neurotrophic factor (GDNF) has potent axonal growth and survival effects on motoneurons. This study used transgenic Myo-GDNF mice to assess the effects of targeted GDNF overexpression on functional recovery after botulinum toxin type A (BTxA) chemodenervation. BTxA (0.1 U) was injected into the tibialis anterior (TA) muscle of wild-type CF1 and transgenic Myo-GDNF mice. On days 1, 7, 14, and 21 after injection, evoked muscle force production and muscle mass were measured (n = 6, for each group at each time point). Greater maximal tetanic force and calculated specific force were evoked in Myo-GDNF animals when compared with control CF1 animals at days 1, 7, and 21. However, the differences were not statistically significant. Similarly, modest reductions in muscle atrophy in the Myo-GDNF group at all time points were not statistically significant. Targeted overexpression of GDNF in the muscles of Myo-GDNF mice did not improve motor recovery in the first 21 days after BTxA chemodenervation.