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.

Rationale for the Development of a Novel Clinical Grading Scale for Postoperative Facial Nerve Function: Results of a Multidisciplinary International Working Group.

OBJECTIVE: The objective of the current study was to present the results of an international working group survey identifying perceived limitations of existing facial nerve grading scales to inform the development of a novel grading scale for assessing early postoperative facial paralysis that incorporates regional scoring and is anchored in recovery prognosis and risk of associated complications. STUDY DESIGN: Survey. SETTING: A working group of 48 multidisciplinary clinicians with expertise in skull base, cerebellopontine angle, temporal bone, or parotid gland surgery. RESULTS: House-Brackmann grade is the most widely used system to assess facial nerve function among working group members (81%), although more than half (54%) agreed that the system they currently use does not adequately estimate the risk of associated complications, such as corneal injury, and confidence in interrater and intrarater reliability is generally low. Simplicity was ranked as the most important attribute of a novel postoperative facial nerve grading system to increase the likelihood of adoption, followed by reliability and accuracy. There was widespread consensus (91%) that the eye is the most critical facial region to focus on in the early postoperative setting. CONCLUSIONS: Members were invited to submit proposed grading systems in alignment with the objectives of the working group for subsequent validation. From these data, we plan to develop a simple, clinically anchored, and reproducible staging system with regional scoring for assessing early postoperative facial nerve function after surgery of the skull base, cerebellopontine angle, temporal bone, or parotid gland.

Imaging of Alternobaric Facial Palsy: An Etiologic Hypothesis.

OBJECTIVE: To report a case of alternobaric facial palsy (AFP) with a hyperpneumatized temporal bone and to review the relevant literature in order to propose a new facet of AFP pathogenesis. METHODS: A patient with a hyperpneumatized temporal bone was found to have recurrent episodes of facial palsy associated with air travel. Systematic review of PubMed, Cochrane Library, and Embase characterized all articles related to transient facial palsy associated with altitude changes or diving with a focus on demographics and imaging findings. RESULTS: A 25 year-old male was referred for recurrent facial palsy associated with commercial air travel. Imaging demonstrated a hyperpneumatized and well-aerated temporal bone. Total air cell volume was calculated to be 8.59 cc on the affected side. A systematic review of the literature identified 49 cases of AFP. Mean age was 34 years old and 80% were male. Twelve cases had imaging of the temporal bone for evaluation, of which 85% demonstrated at least 1 area of hyperpneumatization. Hyperpneumatization was most frequently encountered in the mastoid, zygomatic root, and inferior petrous apex. CONCLUSION: Hyperpneumatization of the temporal bone is critical to AFP etiology as it predicts higher middle ear pressure, if atmospheric pressure changes cannot be equilibrated, via reduced efficacy of normal tympanic membrane pressure buffering. This hypothesis helps to better account for the rarity of the condition and male preponderance.

Otosclerosis in an Urban Population.

OBJECTIVE: To investigate the evolving prevalence of otosclerosis in a large urban population. METHODS: A retrospective review of patients in a large, urban, public health system was conducted from January 2010 to August 2019 to identify subjects with otosclerosis. Diagnostic testing included audiometry and computed tomography scans. Sex, age at diagnosis, treatment received, race, ethnicity, and country of birth were analyzed for each subject and compared with all eligible patients in the reference population. RESULTS: A total of 134 patients from a reference population of 672,839 were diagnosed with otosclerosis and analyzed. The otosclerosis patients were predominantly Hispanic (73%), of which the majority were foreign born (87%). The average age at onset was 46 years and 59% were women. The overall prevalence of otosclerosis was 20 of 100,000 patients. The crude prevalence of otosclerosis by ethnicity was 43 of 100,000 for Hispanics, 12.6 of 100,000 for Caucasians, and 3 of 100,000 for African Americans. Within the Hispanic population, the prevalence of otosclerosis was 60 of 100,000 for foreign-born individuals and 16 of 100,000 for those born in the USA (odds ratio [OR] = 3.69, [95% confidence interval [CI], 2.02-6.76], p < 0.0001). Prevalence was not significantly different among Caucasians and US-born Hispanics. CONCLUSION: Otosclerosis in the studied population was most common among Hispanic patients, though it was strongly influenced by country of birth. The "imported" otosclerosis cases are best explained by environmental influence rather than ethnic susceptibility. This discrepancy is likely due to variance in measles immunization rates among North and Central American countries before 1990.

The Rise and Fall of Otosclerosis: A Population-based Study of Disease Incidence Spanning 70 Years.

OBJECTIVE: Although it is commonly held that otosclerosis has become increasingly uncommon over recent decades, no population-based data exist to characterize this trend. Moreover, because most large epidemiologic databases within the United States primarily include cancer data, even the modern incidence of otosclerosis is unknown. The chief objective of the current work was to characterize the trend in the incidence of otosclerosis over 70 years using the unique resources of the Rochester Epidemiology Project. STUDY DESIGN: Population-based study. PATIENTS: Residents of Olmsted County, Minnesota diagnosed with otosclerosis. MAIN OUTCOME MEASURE: Disease incidence from 1950 to 2017. RESULTS: From 1950 to 2017, 614 incident cases of otosclerosis were identified. The incidence rose from 8.9 per 100,000 person-years in the 1950s to a peak of 18.5 from 1970 to 1974. From this peak, the incidence significantly declined to 6.2 per 100,000 person-years by the early-1990s and reached a nadir of 3.2 from 2015 to 2017 (p<0.001). From 1970 to 2017, age at diagnosis (p = 0.23) and the proportion of bilateral cases (p = 0.16) did not significantly change; pure-tone average at diagnosis did not clinically appreciably change over the study period (median difference <5 dB across decades, p = 0.034). CONCLUSIONS: The incidence of otosclerosis drastically declined since the early-1970s. Historically considered one of the most common causes of acquired hearing loss, the low modern incidence of otosclerosis renders it legally a "rare disease" within the United States. These trends require consideration when determining trainee case requirements and developing practice guidelines.

The Potential for Preserved Cochleovestibular Function in the Setting of Prolonged Pneumolabyrinth.

OBJECTIVE: To discuss the natural history and management of pneumolabyrinth, with particular attention to illustrative cases where hearing improved after a fluid-filled vestibule was restored. PATIENTS: Adults at two academic medical centers with documented pneumolabyrinth on computed tomography (CT) imaging. INTERVENTION: Middle ear surgery or observation. MAIN OUTCOME MEASURES: Cochlear and vestibular symptoms are reported. Inner ear function was documented with audiometry and vestibular testing. Resolution of pneumolabyrinth was documented on imaging for a subset of patients. RESULTS: All patients presented with severe hearing loss following temporal bone fracture (three cases), penetrating trauma (one case), or stapedectomy (three cases). The radiographic finding was typically seen when imaging was obtained in the first few days after the injury. In two cases, vestibular symptoms resolved and partial recovery of hearing was seen despite a relatively long time interval between the inciting event and repair. CONCLUSION: Management of pneumolabyrinth is individualized based on the mechanism of injury, severity of symptoms, and timing of the imaging study. In most cases, the barrier between the middle ear and inner ear is expected to heal with rapid resolution of pneumolabyrinth. Late repair is demonstrated to result in favorable recovery in selected cases. The presence of air in the vestibule cannot be assumed to be indicative of irreversible inner ear damage.

A Systematic Review of Cochlear Implant Outcomes in Prelingually-deafened, Late-implanted Patients.

OBJECTIVE: To analyze outcomes of cochlear implantation (CI) in prelingually-deafened, late-implanted patients. DATA SOURCES: A search of MEDLINE and EMBASE was performed in February 2018 using SCOPUS for the intersection of "cochlear implant," "prelingual," "deaf," and "delay." REVIEW METHODS: Two independent reviewers screened all abstracts and titles for relevance, with conflicts resolved by either the primary or senior author. All articles passing this screen were subjected to a full-text review, during which the primary and senior author each examined manuscripts for inclusion and exclusion criteria. The Cochrane Risk of Bias Assessment Tool was used to assess potential sources of systematic error, and postoperative clinical outcomes were collected at the latest clinical follow-up. RESULTS: Twenty-eight articles were yielded in the final systematic review, accounting for 542 patients. For open-set sentence scores, 10 studies representing 240 patients showed an overall estimated improvement of 44.6% (95% CI: 38.0-51.2%). In terms of quality of life, studies generally showed improvement when looking at specific emotional, social, or hearing-specific domains, but not in global measures. Nonuser rates ranged from 0 to 9.5%. CONCLUSION: Despite performance that is generally poorer than what is generally seen in "traditional" candidates, prelingually-deafened, late-implanted (PL-LI) CI users can experience benefit in terms of both QOL and audiometric scores. The wide range of performance that is reported in the literature highlights the importance of careful patient counseling.

Microvascular free tissue transfer and cochlear implants: A case series and literature review.

OBJECTIVE: The use of microvascular free tissue transfer (MVFTT) for defects of the scalp and lateral temporal bone in patients with cochlear implants (CI) is uncommon. Herein, we report our experience with the utility, indications, and outcomes for MVFTT in patients with cochlear implants. METHODS: A retrospective review of patients at our institution from September 2016 to December 2017 identified subjects with coexistent cochlear implant and ipsilateral MVFTT of the lateral temporal bone or scalp. Information including demographics, indication for MVFTT, timing of CI and MVFTT, donor site, and previous radiation to the head and neck was collected. To assess the current literature on MVFTT in CI patients, a MEDLINE search was performed using key search terms. RESULTS: Two patients with cochlear implants and MVFTT of the ipsilateral temporal bone or scalp were identified. One patient underwent MVFTT for advanced device extrusion with stable audiometric parameters rather than locoregional reconstruction or device explantation. The second patient had primary cochlear implantation at oncologic lateral temporal bone resection (LTBR) and MVFTT for locally advanced squamous cell carcinoma and concurrent profound sensorineural hearing loss (SNHL). A literature review identifies MVFTT as an option for advanced device extrusion, treatment of osteoradionecrosis, and reconstruction after primary oncologic surgery. CONCLUSION: MVFTT is an important reconstructive tool for patients with functional, exposed cochlear implants. Cochlear implantation for severe to profound SNHL should be considered at the time of primary oncologic surgery and MVFTT of the lateral temporal bone or scalp. LEVEL OF EVIDENCE: IV Laryngoscope, 130:1552-1557, 2020.

The Role of Obesity in Iatrogenic Encephaloceles of the Temporal Bone.

OBJECTIVE: Acquired encephaloceles of the temporal bone may be traumatic, spontaneous, or from chronic ear disease or previous surgery. Iatrogenic encephaloceles arise in the setting of previous mastoidectomy and traditionally involve both bony dehiscence and dural injury. We aim to classify and analyze the pathogenesis, clinical presentation, and treatment options for patients with iatrogenic tegmen defects and encephaloceles of the temporal bone. STUDY DESIGN: Retrospective chart review. SETTING: Single tertiary academic center. PATIENTS: Subjects with iatrogenic tegmen defects and encephaloceles of the temporal bone were included. INTERVENTION: Patient demographics, history, symptoms, radiographic data, intraoperative findings, management, follow-up, and outcomes were recorded. OUTCOME MEASURES: Primary outcome measures included patient characteristics, time from primary otologic surgery to surgical repair, location of the defect, and management strategy including surgical approach, methods, and follow-up. RESULTS: Iatrogenic tegmen injuries or encephaloceles were identified in 18 patients and divided into intentional or unintentional. The latter group presented immediately, early, or late, as determined by intraoperative identification or from delayed symptoms. Eleven patients presented late with previously unrecognized encephaloceles. Compared with patients presenting with incidentally noted tegmen dehiscence during revision mastoidectomy, a significant proportion of patients with late encephaloceles had BMI ≥30 kg/m (p = 0.03). CONCLUSION: The majority of iatrogenic encephaloceles are unintentional, unrecognized, and may present many years after primary surgery. Similar to spontaneous encephaloceles of the temporal bone, obesity is associated with iatrogenic encephaloceles, although the rate of progression and timing is unclear. We recommend primary repair of any unintentional tegmen defect encountered during mastoid surgery, especially in obese patients.

A Start Codon Variant in NOG Underlies Symphalangism and Ossicular Chain Malformations Affecting Both the Incus and the Stapes.

We performed exome sequencing to evaluate the underlying molecular cause of a patient with bilateral conductive hearing loss due to multiple ossicular abnormalities as well as symphalangism of the fifth digits. This leads to the identification of a novel heterozygous start codon variant in the NOG gene (c.2T>C:p.Met1?) that hinders normal translation of the noggin protein. Variants in NOG lead to a spectrum of otologic, digit, and joint abnormalities, a combination suggested to be referred to as NOG-related-symphalangism spectrum disorder (NOG-SSD). Conductive hearing loss from such variants may stem from stapes footplate ankylosis, fixation of the malleoincudal joint, or fixation of the incus short process. In this case, the constellation of both stapes and incus fixation, an exceptionally tall stapes suprastructure, thickened long process of the incus, and enlarged incus body was encountered, leading to distinct challenges during otologic surgery to improve hearing thresholds. This case highlights multiple abnormalities to the ossicular chain in a patient with a start codon variant in NOG. We provide detailed imaging data on these malformations as well as surgical considerations and outcomes.

Imaging of labyrinthine fistula after repair with bone pate.

OBJECTIVES/HYPOTHESIS: To demonstrate imaging of labyrinthine fistula due to cholesteatoma and illustrate outcome following repair with bone pate. STUDY DESIGN: Retrospective review. METHODS: Patients with labyrinthine fistula due to cholesteatoma were identified, and pre- and postoperative imaging was assessed. Method of repair was recorded. The fistula site was examined for patency and bone density after repair. RESULTS: Twenty-nine fistulae were seen in a cohort of 375 cholesteatoma cases (8%). Preoperative computed tomography (CT) imaging for fistula detection showed sensitivity of 96% and specificity of 90%. The lateral semicircular canal was the most common site of dehiscence occurring in all cases. Facial nerve dehiscence is observed in 91% of fistula cases and stapes erosion in 69%. Severe hearing loss was present preoperatively in four (14%) cases. Bone pate was used to repair the fistula with CT-documented restoration of the otic capsule in 11/13 cases with postoperative imaging. When bone pate was not used, the otic capsule defect persisted. CONCLUSIONS: Spontaneous repair of the otic capsule following treatment of cholesteatoma is expected to be an uncommon occurrence. Repair of the fistula with bone can result in long-lasting restoration of the otic capsule. LEVEL OF EVIDENCE: 4. Laryngoscope, 128:1643-1648, 2018.

Iatrogenic cholesteatoma arising from the vascular strip.

OBJECTIVES/HYPOTHESIS: To highlight the phenomenon of cholesteatoma arising from the vascular strip following tympanoplasty and tympanomastoidectomy. STUDY DESIGN: Multi-institutional retrospective chart review. METHODS: Consecutive adult and pediatric patients evaluated between 2000 and 2015 with acquired cholesteatoma arising from the skin of a prior vascular strip were identified. Patients with evidence of residual or recurrent cholesteatoma elsewhere in the middle ear, mastoid, or ear canal were excluded. RESULTS: Seventeen cases (71% female, 53% right-sided) were identified, and the mean age at presentation was 39.2 years. Patients presented on average 12.9 years following prior otologic surgery, which was most commonly tympanoplasty without mastoidectomy (59%). However, younger patients presented with symptoms sooner following prior surgery (r = 0.61, r2 = 0.37, P = 0.0095). The most common presenting symptom was otorrhea (76%). All patients were found to have mastoid cholesteatoma with bony erosion, and three patients exhibited additional tegmen or posterior fossa bone defects. The middle ear was not involved with cholesteatoma in any case. A canal wall down procedure was performed due to extensive bony canal erosion in 29% of cases. CONCLUSION: The vascular strip is an uncommon source of iatrogenic cholesteatoma that can present years following an otherwise uncomplicated otologic surgery. The findings presented herein highlight the importance of careful vascular strip orientation at the conclusion of otologic surgery. LEVEL OF EVIDENCE: 4. Laryngoscope, 127:698-701, 2017.

Diagnostic Criteria for Detection of Vestibular Schwannomas in the VA Population.

OBJECTIVE: To investigate the prevalence of vestibular schwannoma (VS) and asymmetric sensorineural hearing loss in the Veterans Administration hospital population and analyze a more efficient method of diagnosing VS in a population with significant noise exposure. STUDY DESIGN: Retrospective review of South Central (VISN 16) Veterans Administration hospitals. METHODS: Record query for ICD-9 codes for asymmetric sensorineural hearing loss or VS between 1999 and 2012. Patient demographics, signs and symptoms at presentation, audiogram and imaging data, and management data were collected and analyzed. Audiograms from tumor patients were compared with controls matched for age, sex, combat experience, and medical comorbidity (2:1 control to case ratio). RESULTS: The prevalence of VS was 1 per 1,145 patients in this population, with average age at diagnosis of 62. Patients with VS presented more commonly with unilateral tinnitus, rollover, and absent acoustic reflexes when compared with matched controls, but positive predictive value was low. Published criteria for defining hearing asymmetry showed variable sensitivity (51-89%) and low specificity (0-42%) for the detection of VS in this population. Criteria meeting the definitions of significant asymmetry with specificity for VS of 80% or greater were as follows: >15 dB threshold difference at 3 kHz and unilateral tinnitus, ≥45 dB threshold difference at 3 kHz regardless of tinnitus, or when the word recognition score difference was ≥80%. With serial audiograms 2.5 years apart or greater, a ≥10 dB threshold increase at any frequency between 0.5 and 4 kHz had a 100% sensitivity for tumor and a ≥10 dB increase at 3 kHz had a specificity of 84%. The majority of patients were observed, whereas only 30% had surgery. Patients who were observed were older than those treated with surgery or radiation (p <0.001). CONCLUSION: Typical audiometric screening criteria should be modified in the veteran population to improve cost efficiency of diagnosis. Observation is the primary management strategy in the veteran population because of age.

Early practice: neurotology.

Besides technical and surgical proficiency, some of the most important skills for a young Neurotologist to refine include communication and critical thinking abilities. This Early Practice article provides perspectives on common challenges and career development from a current Neurotology fellow and his mentor.

Workforce analysis of neurotologists in the United States.

OBJECTIVE: To analyze current ratio of neurotologists to the population and examine regional variation. STUDY DESIGN: Population survey examining hospital referral regions (HRRs) as defined in the Dartmouth Atlas of Health Care Project and location of members of the American Neurotology Society. MAIN OUTCOME MEASURE: Ratio of neurotologists to the population within designated HRRs. RESULTS: The national estimate of practicing neurotologists per population in 2006 was 1:922664 persons. The ratio is not uniform across HRRs, indicating that referral patterns for neurotologic care are wider than the care for indicators used in designating HRRs. Distribution of specialists is closely correlated with population. Estimated procedures per population are stable or declining for lateral cranial base surgery, implying current or impending oversupply of neurotologists. CONCLUSION: The number of specialists in Neurotology likely exceeds projected needs. The current supply of trainees predicts this trend will continue for decades. Development and maintenance of lateral cranial base surgical skills will be difficult for some practitioners.

Chondroblastoma-like chondroma of soft tissue: report of the first case in the base of skull.

Chondroblastoma-like chondroma (CLC) of soft tissue is a rare benign neoplasm that usually involves the soft tissues of the hand. This report describes the first case of CLC of soft tissue arising in the base of the skull. A 33-year-old man was seen with a slow growing mass in the right parotid region of his face. The noncontrast computed tomographic scans showed an 8.5-cm mass with calcifications involving the right masticator space and extending through the bone into the middle cranial fossa. The radiologic differential diagnosis included osteosarcoma, leiomyosarcoma, chondrosarcoma, and giant cell tumor. During surgery, the large lateral skull base tumor appeared to involve the middle and infratemporal fossae and eroded the surrounding bone. Although the tumor was removed piecemeal, total excision was performed. On microscopic examination, the tumor displayed lobules of mature hyaline cartilage with numerous chondroblasts, coarse calcifications including chicken wire calcifications, and scattered osteoclasts. No atypia, mitoses, necrosis, or osteoid formation was seen. The tumor was diagnosed as chondroma with chondroblastoma features of the soft tissue. His postoperative clinical course was uneventful; however, after 7 months, he had a local recurrence identified on follow-up magnetic resonance imaging. He underwent repeat surgical excision of the tumor, which showed similar histology as the previous excision. This large skull based tumor eroding the bone, which clinically and radiologically mimicked a malignant process, was an unusual presentation of a benign cartilaginous neoplasm. Pathologists should be aware that CLC may occur in the base of the skull and this lesion should be differentiated from the other benign or malignant tumors arising in this area. These lesions have a potential for local recurrence; hence, a close follow-up is recommended.