Conductive Hearing Loss Associates With Dementia, and Middle Ear Reconstruction Mitigates This Association: A Multinational Database Study.

OBJECTIVE: To test the hypothesis that conductive hearing loss (CHL) is associated with dementia, and that middle ear reconstruction (MER) associates with improved outcomes for these measures in a multinational electronic health records database. STUDY DESIGN: Retrospective cohort study with propensity-score matching (PSM). SETTING: TriNetX is a research database representing about 110 million patients from the United States, Taiwan, Brazil, and India. PATIENTS: Subjects older than 50 years with no HL and any CHL (ICD-10: H90.0-2). Subjects of any age with and without any MER (CPT: 1010174). MAIN OUTCOME MEASURES: Odds ratios (ORs) and hazard ratios with 95% confidence intervals (95% CIs) for incident dementia (ICD-10: F01, F03, G30). RESULTS: Of 103,609 patients older than 50 years experiencing any CHL, 2.74% developed dementia compared with 1.22% of 38,216,019 patients with no HL (OR, 95% CI: 2.29, 2.20-2.37). Of patients experiencing CHL, there were 39,850 who received MER. The average age was 31.3 years, with 51% female patients. A total of 343,876 control patients with CHL were identified; 39,900 patients remained in each cohort after 1:1 PSM for HL- and dementia-related risk factors. Matched risk for developing dementia among MER recipients was 0.33% compared with 0.58% in controls (OR: 0.58, 0.46-0.72). CONCLUSIONS: CHL increases the odds for dementia, and MER improves the odds for incident dementia. This study represents the first population study on the topic of CHL, MER, and dementia.

Comparing Approaches for Repair of Superior Semicircular Canal Dehiscence.

OBJECTIVE: Compare outcomes for subjects who underwent middle cranial fossa (MCF) or transmastoid (TM) repair of superior semicircular canal dehiscence (SSCD). STUDY DESIGN: Retrospective cohort study. SETTING: Quaternary-care, academic neurotology practice. METHODS: Subjects who underwent MCF or TM repair of SSCD between December 1999 and April 2023 were identified. Main outcome measures included demographic data, length of surgery and hospital stay, clinical presentation, and audiometric testing. RESULTS: Ninety-three subjects (97 ears) who underwent surgery for SSCD met inclusion criteria: 58.8% (57) via MCF, 39.2% (38) via TM, and 2.0% (2) via TM + MCF. Median operative time was shorter for the TM (35) compared to the MCF (29) approach (118 vs 151 minutes, P < .001). Additionally, median hospital stays were shorter for TM (36) compared to the MCF (56) approach (15.3 vs 67.7 hours, P < .001). Overall, 92% (49/53) of MCF and 92% (33/36) of TM surgeries resulted in an improvement or resolution of one or more symptoms (P = .84). There was no significant preoperative to postoperative change in the median air conduction pure-tone average (PTA), air-bone gap, or word recognition score in both the MCF and TM groups (P > .05). Improvements of >10 dB in the pre- to postoperative absolute change in bone conduction PTA were noted in 3 subjects in the MCF group and 4 subjects in the TM group (P = .49). CONCLUSION: The TM approach for SSCD demonstrates shorter operative times and length of hospital stay. The TM and MCF approaches have comparable audiometric and clinical outcomes.

Staged implantation of active transcutaneous bone conduction hearing devices (BCHD) after explantation of older generation bone anchored hearing aids (BAHAs): Surgical outcomes and approach to management.

PURPOSE: To evaluate outcomes following explantation of percutaneous or transcutaneous bone conduction implants (pBCIs or tBCIs) and subsequent implantation of transcutaneous active bone conduction hearing devices (BCHDs); to provide guidance regarding staging of surgery and adjunctive procedures. MATERIALS AND METHODS: Retrospective chart review of eight adult subjects (ten ears) with pBCIs or tBCIs who underwent explantation of their device and subsequent implantation with a BCHD [MED-EL BONEBRIDGE™ (n = 7, 70 %) or Cochlear™ Osia® (n = 3, 30 %)]. RESULTS: Reasons for pBCI or tBCI explantation were pain (60 %, 6/10), infection (60 %, 6/10), skin overgrowth (50 %, 5/10), and inability to obtain new processors (20 %, 2/10). Median time between pBCI or tBCI removal and BCHD staged implant was 4.7 (IQR 2.2-8.1) months. Two subjects developed complications following BCHD implantation. One had a persistent wound overlying the osseointegrated screw after removal of the pBCI abutment, requiring removal and temporalis rotational flap. Staged Osia® implantation was performed, but ultimately wound dehiscence developed over the device. The second subject experienced an infection after BONEBRIDGE™ implantation (32 days after pBCI explant), necessitating washout and treatment with intravenous antibiotics. There was subsequent device failure. CONCLUSION: The transition from a pBCI or tBCI to a novel transcutaneous device is nuanced. Staged pBCI or tBCI explantation and novel BCHD implantation with sufficient time for wound healing is vital. Adjunctive procedures to augment soft tissue in cases of prior attenuation may be required to avoid complications with larger internal devices.

MRI Safety of Stapes Prostheses: A Systematic Review.

OBJECTIVE: To assess magnetic resonance imaging (MRI) safety of stapes prostheses. DATA SOURCES: Ovid MEDLINE, Embase, Cochrane Central Register of Controlled Trials, Web of Science, and Scopus databases were searched from inception to November 2021 following PRISMA guidelines. REVIEW METHODS: Studies reporting evidence of stapes prosthesis displacement or interaction in adult or pediatric implant recipients undergoing MRI. Cadaveric, animal, and basic studies with nonhuman data were also included. RESULTS: From an initial search of 123 articles, 42 full-text studies were evaluated for eligibility and 19 studies that met the inclusion criteria were included. Motion artifact was reported in a few stainless steel prosthesis types in vitro; however, such displacement was not observed in human cadaver temporal bone studies and had no adverse reported outcomes. A small subgroup of patients in the 1980s received a ferromagnetic stainless steel stapes implant that was recalled and has not been used since 1987. Patients with implants performed in the 1980s should be directed to 1.5T scanners from an abundance of caution. CONCLUSION: Modern (post-1987) stapes prostheses do not pose a risk in vivo when exposed to the magnetic fields of MRI scanners.

Clinical Practice Guideline: Immunotherapy for Inhalant Allergy.

OBJECTIVE: Allergen immunotherapy (AIT) is the therapeutic exposure to an allergen or allergens selected by clinical assessment and allergy testing to decrease allergic symptoms and induce immunologic tolerance. Inhalant AIT is administered to millions of patients for allergic rhinitis (AR) and allergic asthma (AA) and is most commonly delivered as subcutaneous immunotherapy (SCIT) or sublingual immunotherapy (SLIT). Despite its widespread use, there is variability in the initiation and delivery of safe and effective immunotherapy, and there are opportunities for evidence-based recommendations for improved patient care. PURPOSE: The purpose of this clinical practice guideline (CPG) is to identify quality improvement opportunities and provide clinicians trustworthy, evidence-based recommendations regarding the management of inhaled allergies with immunotherapy. Specific goals of the guideline are to optimize patient care, promote safe and effective therapy, reduce unjustified variations in care, and reduce the risk of harm. The target patients for the guideline are any individuals aged 5 years and older with AR, with or without AA, who are either candidates for immunotherapy or treated with immunotherapy for their inhalant allergies. The target audience is all clinicians involved in the administration of immunotherapy. This guideline is intended to focus on evidence-based quality improvement opportunities judged most important by the guideline development group (GDG). It is not intended to be a comprehensive, general guide regarding the management of inhaled allergies with immunotherapy. The statements in this guideline are not intended to limit or restrict care provided by clinicians based on their experience and assessment of individual patients. ACTION STATEMENTS: The GDG made a strong recommendation that (Key Action Statement [KAS] 10) the clinician performing allergy skin testing or administering AIT must be able to diagnose and manage anaphylaxis. The GDG made recommendations for the following KASs: (KAS 1) Clinicians should offer or refer to a clinician who can offer immunotherapy for patients with AR with or without AA if their patients' symptoms are inadequately controlled with medical therapy, allergen avoidance, or both, or have a preference for immunomodulation. (KAS 2A) Clinicians should not initiate AIT for patients who are pregnant, have uncontrolled asthma, or are unable to tolerate injectable epinephrine. (KAS 3) Clinicians should evaluate the patient or refer the patient to a clinician who can evaluate for signs and symptoms of asthma before initiating AIT and for signs and symptoms of uncontrolled asthma before administering subsequent AIT. (KAS 4) Clinicians should educate patients who are immunotherapy candidates regarding the differences between SCIT and SLIT (aqueous and tablet) including risks, benefits, convenience, and costs. (KAS 5) Clinicians should educate patients about the potential benefits of AIT in (1) preventing new allergen sensitizations, (2) reducing the risk of developing AA, and (3) altering the natural history of the disease with continued benefit after discontinuation of therapy. (KAS 6) Clinicians who administer SLIT to patients with seasonal AR should offer pre- and co-seasonal immunotherapy. (KAS 7) Clinicians prescribing AIT should limit treatment to only those clinically relevant allergens that correlate with the patient's history and are confirmed by testing. (KAS 9) Clinicians administering AIT should continue escalation or maintenance dosing when patients have local reactions (LRs) to AIT. (KAS 11) Clinicians should avoid repeat allergy testing as an assessment of the efficacy of ongoing AIT unless there is a change in environmental exposures or a loss of control of symptoms. (KAS 12) For patients who are experiencing symptomatic control from AIT, clinicians should treat for a minimum duration of 3 years, with ongoing treatment duration based on patient response to treatment. The GDG offered the following KASs as options: (KAS 2B) Clinicians may choose not to initiate AIT for patients who use concomitant beta-blockers, have a history of anaphylaxis, have systemic immunosuppression, or have eosinophilic esophagitis (SLIT only). (KAS 8) Clinicians may treat polysensitized patients with a limited number of allergens.

Executive Summary of Clinical Practice Guideline on Immunotherapy for Inhalant Allergy.

OBJECTIVE: Allergen immunotherapy (AIT) is the therapeutic exposure to an allergen or allergens selected by clinical assessment and allergy testing to decrease allergic symptoms and induce immunologic tolerance. Inhalant AIT is administered to millions of patients for allergic rhinitis (AR) and allergic asthma (AA) and is most commonly delivered as subcutaneous immunotherapy (SCIT) or sublingual immunotherapy (SLIT). Despite its widespread use, there is variability in the initiation and delivery of safe and effective immunotherapy, and there are opportunities for evidence-based recommendations for improved patient care. PURPOSE: The purpose of this clinical practice guideline is to identify quality improvement opportunities and provide clinicians trustworthy, evidence-based recommendations regarding the management of inhaled allergies with immunotherapy. Specific goals of the guideline are to optimize patient care, promote safe and effective therapy, reduce unjustified variations in care, and reduce risk of harm. The target patients for the guideline are any individuals aged 5 years and older with AR, with or without AA, who are either candidates for immunotherapy or treated with immunotherapy for their inhalant allergies. The target audience is all clinicians involved in the administration of immunotherapy. This guideline is intended to focus on evidence-based quality improvement opportunities judged most important by the guideline development group. It is not intended to be a comprehensive, general guide regarding the management of inhaled allergies with immunotherapy. The statements in this guideline are not intended to limit or restrict care provided by clinicians based on their experience and assessment of individual patients. ACTION STATEMENTS: The guideline development group made a strong recommendation that (Key Action Statement [KAS] 10) the clinician performing allergy skin testing or administering AIT must be able to diagnose and manage anaphylaxis. The guideline development group made recommendations for the following KASs: (KAS 1) Clinicians should offer or refer to a clinician who can offer immunotherapy for patients with AR with or without AA if their patients' symptoms are inadequately controlled with medical therapy, allergen avoidance, or both, or have a preference for immunomodulation. (KAS 2A) Clinicians should not initiate AIT for patients who are pregnant, have uncontrolled asthma, or are unable to tolerate injectable epinephrine. (KAS 3) Clinicians should evaluate the patient or refer the patient to a clinician who can evaluate for signs and symptoms of asthma before initiating AIT and for signs and symptoms of uncontrolled asthma before administering subsequent AIT. (KAS 4) Clinicians should educate patients who are immunotherapy candidates regarding the differences between SCIT and SLIT (aqueous and tablet) including risks, benefits, convenience, and costs. (KAS 5) Clinicians should educate patients about the potential benefits of AIT in (1) preventing new allergen sensitization, (2) reducing the risk of developing AA, and (3) altering the natural history of the disease with continued benefit after discontinuation of therapy. (KAS 6) Clinicians who administer SLIT to patients with seasonal AR should offer pre- and co-seasonal immunotherapy. (KAS 7) Clinicians prescribing AIT should limit treatment to only those clinically relevant allergens that correlate with the patient's history and are confirmed by testing. (KAS 9) Clinicians administering AIT should continue escalation or maintenance dosing when patients have local reactions to AIT. (KAS 11) Clinicians should avoid repeat allergy testing as an assessment of the efficacy of ongoing AIT unless there is a change in environmental exposures or a loss of control of symptoms. (KAS 12) For patients who are experiencing symptomatic control from AIT, clinicians should treat for a minimum duration of 3 years, with ongoing treatment duration based on patient response to treatment. The guideline development group offered the following KASs as options: (KAS 2B) Clinicians may choose not to initiate AIT for patients who use concomitant beta-blockers, have a history of anaphylaxis, have systemic immunosuppression, or have eosinophilic esophagitis (SLIT only). (KAS 8) Clinicians may treat polysensitized patients with a limited number of allergens.

Plain Language Summary: Immunotherapy for Inhalant Allergy.

The plain language summary explains allergen immunotherapy to patients, families, and caregivers. The summary is for patients aged 5 years and older who are experiencing symptoms from inhalant allergies and are considering immunotherapy as a treatment option. It is based on the 2024 "Clinical Practice Guideline: Immunotherapy for Inhalant Allergy." This plain language summary is a companion publication to the full guideline, which provides greater detail for health care providers. Guidelines and their recommendations may not apply to every patient, but they can be used to find best practices and quality improvement opportunities.

Salvage Microsurgery Following Failed Primary Radiosurgery in Sporadic Vestibular Schwannoma.

Importance: Management of sporadic vestibular schwannoma with radiosurgery is becoming increasingly common globally; however, limited data currently characterize patient outcomes in the setting of microsurgical salvage for radiosurgical failure. Objective: To describe the clinical outcomes of salvage microsurgery following failed primary stereotactic radiosurgery (SRS) or fractionated stereotactic radiotherapy (FSRT) among patients with sporadic vestibular schwannoma. Design, Setting, and Participants: This was a cohort study of adults (≥18 years old) with sporadic vestibular schwannoma who underwent salvage microsurgery following failed primary SRS/FSRT in 7 vestibular schwannoma treatment centers across the US and Norway. Data collection was performed between July 2022 and January 2023, with data analysis performed between January and July 2023. Exposure: Salvage microsurgical tumor resection. Main Outcomes and Measures: Composite outcome of undergoing less than gross total resection (GTR) or experiencing long-term facial paresis. Results: Among 126 patients, the median (IQR) age at time of salvage microsurgery was 62 (53-70) years, 69 (55%) were female, and 113 of 117 (97%) had tumors that extended into the cerebellopontine angle at time of salvage. Of 125 patients, 96 (76%) underwent primary gamma knife SRS, while 24 (19%) underwent linear accelerator-based SRS; the remaining patients underwent FSRT using other modalities. Postoperative cerebrospinal fluid leak was seen in 15 of 126 patients (12%), hydrocephalus in 8 (6%), symptomatic stroke in 7 (6%), and meningitis in 2 (2%). Each 1-mm increase in cerebellopontine angle tumor size was associated with a 13% increased likelihood of foregoing GTR (64 of 102 patients [63%]) or long-term postoperative House-Brackmann grade higher than I (48 of 102 patients [47%]) (odds ratio, 1.13; 95% CI, 1.04-1.23). Following salvage microsurgery, tumor growth-free survival rates at 1, 3, and 5 years were 97% (95% CI, 94%-100%), 93% (95% CI, 87%-99%), and 91% (95% CI, 84%-98%), respectively. Conclusions: In this cohort study, more than half of patients who received salvage microsurgery following primary SRS/FSRT underwent less than GTR or experienced some degree of facial paresis long term. These data suggest that the cumulative risk of developing facial paresis following primary SRS/FSRT by the end of the patient's journey with treatment approximates 2.5% to 7.5% when using published primary SRS/FSRT long-term tumor control rates.

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.

Epidemiology and Risk Factors for Development of Sporadic Vestibular Schwannoma.

Vestibular schwannomas (VSs) are benign, slow-growing tumors of the eighth cranial nerve. Sporadic unilateral VSs constitute approximately 95% of all newly diagnosed tumors. There is little known about risk factors for developing sporadic unilateral VS. Potential risk factors that have been reported are familial or genetic risk, noise exposure, cell phone use, and ionizing radiation, whereas protective factors may include smoking and aspirin use. More research is needed to elucidate the risk factors for development of these rare tumors.

Approach to Cochlear Implantation in Patients With Ventriculoperitoneal Shunts.

OBJECTIVE: To evaluate the safety and outcomes of cochlear implantation (CI) in patients with ventriculoperitoneal (VP) shunts to inform clinical practice. STUDY DESIGN: Historical cohort study. SETTING: Tertiary referral centers. METHODS: A multi-institutional historical cohort of patients with VP shunts and CI was identified and analyzed. RESULTS: A total of 46 patients (median age 8 years [interquratile range, IQR: 2-46]) with VP shunts and CI were identified. Of these, 41 (89%) patients had a VP shunt prior to CI. Based on institutional preference and individual patient factors, CI was performed contralateral to a pre-existing VP shunt in 24 of these 41 cases (59%) and ipsilateral in 17 (41%). Furthermore, pre-CI relocation of the VP shunt was performed in 3 cases (7%), and 2 patients (5%) underwent planned revision of their VP shunt concurrent with CI. In total, 2 of 27 pediatric patients (7%) required unanticipated revision shunt surgery, both contralateral to CI device placement, given VP shunt malfunction. One of 19 adult patients (5%) required shunt revision during CI due to shunt damage noted intraoperatively. Among 43 patients with available follow-up, 38 (88%) are regular CI users, with a median consonant-nucleus vowel-consonant word: score of 58% (IQR: 28-72). CONCLUSION: CI can be performed at low risk, either contralateral or ipsilateral, to a VP shunt, and does not mandate shunt revision in most cases. Additional considerations regarding CI receiver-stimulator placement are necessary with programmable shunts to mitigate device interaction. Preoperative planning, including coordination of care with neurosurgery, is important to achieving optimal outcomes.

WITHDRAWN: Vestibular schwannoma: from a 'certain hard body' through the 'bloody angle' to hearing preservation surgery.

The Publisher regrets that this article is an accidental duplication of an article that has already been published, http://doi.org/10.1016/j.wneu.2023.02.016. The duplicate article has therefore been withdrawn. The full Elsevier Policy on Article Withdrawal can be found at https://www.elsevier.com/about/policies/article-withdrawal.

Factors associated with hearing outcomes after a middle fossa approach in 131 consecutive patients with vestibular schwannomas.

OBJECTIVE: The middle fossa approach is an effective option for the treatment of small (Koos grade I and II) vestibular schwannomas (VSs) when the goal is hearing preservation. The authors evaluated the rates of hearing preservation and examined the factors associated with improved hearing outcomes after the middle fossa approach for VSs. METHODS: In this retrospective, single-center cohort study evaluating the clinical outcomes after resection of small VSs using the middle fossa approach, consecutive adult patients (> 18 years) who underwent surgery between January 2000 and December 2021 were included. Clinical and imaging characteristics were analyzed, including baseline hearing status, duration of surgery, anesthetic parameters, and imaging characteristics of the surgically treated tumors. RESULTS: Among the 131 included patients, 102 had valid and discoverable pre- and postoperative audiology assessments. The mean follow-up was 26 months (range 1-180 months). There were 85 patients with serviceable hearing preoperatively, defined as American Academy of Otolaryngology-Head and Neck Surgery (AAO-HNS) class A or B, of whom 78% retained class A or B hearing at the last follow-up. Binary logistic regression demonstrated that preoperative hearing AAO-HNS class (OR 0.19, 95% CI 0.05-0.77; p = 0.02), overlap between fundus and cochlea (OR 0.32, 95% CI 0.11-0.96; p = 0.04), and duration of anesthesia (OR 0.98, 95% CI 0.97-0.99; p = 0.03) were independent predictors of hearing outcomes. Additionally, 75% of patients with high diffusion-weighted imaging signal in the tumor (p = 0.009) and 67% of patients with the tumor originating at the modiolus of the cochlea (p = 0.004) had poor hearing outcomes. CONCLUSIONS: The hearing preservation rates after microsurgical resection of small VSs using the middle fossa approach are high, with 78% of patients maintaining AAO-HNS class A or B hearing. Poor hearing status at baseline, longer duration of anesthesia, and large overlap between the fundus of the internal auditory canal and the cochlea were independently associated with unfavorable hearing outcomes. Imaging characteristics can be used to stratify patients' risk of hearing loss.

Cochlear Implantation in Patients With Known Cognitive Impairment: What Are the Benefits?

OBJECTIVE: To evaluate the outcomes of cochlear implantation (CI) in adults with preoperatively diagnosed cognitive impairment. STUDY DESIGN: Retrospective cohort study. SETTING: Tertiary-care academic center. PATIENTS: Adults undergoing CI with preexisting cognitive impairment. INTERVENTIONS: Cochlear implantation. MAIN OUTCOME MEASURES: 1) Hearing improvement after CI; 2) morbidity and mortality associated with surgery. RESULTS: Eight patients met inclusion criteria with mean age 77.8 years (SD, 9.6 y) at time of implantation; 7 were included in subsequent analysis as one did not have speech recognition scores. Average preoperative MoCA cognitive score of 22.6 (SD, 3.9, ≤25 demonstrates cognitive impairment). Average follow-up was 29.0 months (SD, 33.3 mo). Two patients passed away at an average 58.0 months (SD, 31.1 mo) after surgery. Median preoperative pure tone average was 86.3 dB HL ( interquartile range 31.3 dB HL) compared with 33.8 dB HL (IQR 5.0 dB HL) postoperatively ( p = <0.001). Median preoperative speech testing score (AzBio/HINT) was 21% (IQR, 24%) compared with 44% (IQR, 21%) postoperatively ( p = <0.001). There were no observed surgical complications during the follow-up period. CONCLUSIONS: This study demonstrates that patients with cognitive impairment before CI can experience improved hearing, no increased risk of complications, and good longevity after CI. Further prospective studies are needed to further define the utility of CI in patients with impaired cognition.

Extended middle fossa approach for resection of a petroclival meningioma and vestibular schwannoma.

A 69-year-old woman with refractory left facial pain and subtle left hearing decline had a 13.0 × 8.1-mm left petrous apex/Meckel's cave meningioma and an 8.8 × 5.6-mm left intracanalicular vestibular schwannoma. She was otherwise neurologically intact. The anterior petrous and middle fossa approaches provide ideal access to these lesions individually, so an extended middle fossa approach was used to resect both in the same setting. She was neurologically stable postoperatively, except for a transient abducens palsy. Hearing was preserved on audiogram, and 4-month MRI displayed no tumors. The extended middle fossa approach provides excellent exposure of the petrous apex and internal auditory canal. The video can be found here: https://stream.cadmore.media/r10.3171/2022.1.FOCVID21258.

Perigeniculate Giant Cell Tumor of Temporal Bone.

Giant cell tumors of bone (GCTBs) are benign osteolytic neoplasms that can be treated with either gross-total resection or subtotal resection with adjuvant radiotherapy. For the rare GCTB of the temporal bone, close proximity to critical structures can produce functional deficits and make gross-total resection difficult to achieve without significant morbidity. We present the case of a 28-year-old woman with progressive facial paresis, otalgia, neck pain, imbalance, and subjective hearing loss. She was found to have a facial nerve mass centered at the geniculate ganglion extending into the labyrinthine segment and vestibule. We achieved gross-total resection with preserved facial nerve function as the tumor did not originate from the facial nerve and could be dissected free from the nerve. Final pathology was consistent with GCTB.

Is There an Inherited Contribution to Risk for Sporadic Unilateral Vestibular Schwannoma? Evidence of Familial Clustering.

OBJECT: Unlike the autosomal dominant inheritance of neurofibromatosis 2, there are no known inherited risk factors for sporadic, unilateral vestibular schwannoma (VS), which comprise most VS cases. The authors tested a hypothesis positing a genetic contribution to predisposition to these lesions by analyzing familial clustering of cases. METHODS: Familial clustering of individuals with unilateral VS was analyzed in two independent genealogical resources with linked diagnosis data: the Veterans Health Administration Genealogy Resource and the Utah Population Database. Tests for excess relatedness, estimation of relative risks (RRs) in close and distant relatives, and identification of pedigrees with a significant excess of unilateral VS among descendants were performed. RESULTS: The average pairwise relatedness of the Veterans Health Administration Genealogy Resource VS cases significantly exceeded the expected relatedness ( p = 0.016), even when close relationships were ignored ( p = 0.002). RR for third- and fifth-degree relatives developing VS were significantly elevated (RR, 60.83; p = 0.0005; 95% confidence interval [CI], 7.37-219.73) and (RR, 11.88; p = 0.013; 95% CI, 1.44-42.90), respectively. No VS-affected first-, second-, or fourth-degree relatives were observed. In the Utah Population Database population, no first- or second-degree relatives with VS were observed. RR for fifth-degree relatives developing VS was significantly elevated (RR, 2.23; p = 0.009; 95% CI, 1.15-3.90). CONCLUSION: These results provide strong evidence for an inherited predisposition to sporadic, unilateral VS. This study exhibits the value of genealogical resources with linked medical data for examining hypotheses regarding inherited predisposition. The high-risk unilateral VS pedigrees identified in two independent resources provide a powerful means of pursuing predisposition gene identification.

Cognitive and behavioral effects of hearing loss.

PURPOSE OF REVIEW: Hearing loss is one of the largest modifiable risk factors for developing dementia, accounting for up to 9% of the overall modifiable risk. The neuropsychologic and psychosocial impacts of hearing loss are becoming increasingly appreciated. The objective of this review is to explore the recent literature regarding the cognitive and behavioral effects of hearing loss and the role of hearing rehabilitation, particularly in older adults. RECENT FINDINGS: Cognitive decline may begin while patients have subclinical hearing loss, earlier than previously thought. Hearing rehabilitation, either via hearing amplification, middle ear surgery, or cochlear implantation, likely plays a role in preventing or slowing the rate of cognitive decline in patients with hearing loss. Hearing loss can increase the likelihood of social isolation, loneliness and depression in older adults, but it is unclear at this time what effect hearing rehabilitation has on these domains. SUMMARY: Hearing loss is one of the largest modifiable risk factors for cognitive decline, and hearing rehabilitation can play a significant role in preserving cognitive function. Understanding the cognitive and psychosocial impact of hearing loss can help facilitate the development of approaches for prevention and treatment.

Staged and Combined Approach for Resection of Giant Posterior Fossa and Temporal Bone Schwannoma.

Vestibular schwannomas have an estimated incidence of 1.09/100,000 people, representing 6%-10% of intracranial tumors.1-4 Rarer giant vestibular schwannomas are defined by an extrameatal diameter of ≥4 cm and can be difficult to treat because of displacement and compression of local neurovasculature and the potential for multicompartment involvement. A 20-year-old woman with history of presumed right-sided Bell palsy and unexplained hearing loss was found to have a 9 × 8 × 6-cm giant posterior fossa schwannoma on syncopal workup (Video 1). It extended from the Meckel cave anterosuperiorly to below the skull base through the jugular foramen, filling the petrous apex and compressing the cerebellum, pons, and midbrain. She had ipsilateral facial weakness (House-Brackmann 3/5), V2 numbness, tongue deviation, vocal fold paresis, and hearing loss. A combined petrosal (transotic, extended middle fossa) and retrosigmoid approach was performed through a curvilineal incision that provided access to the middle fossa, petrous apex, and posterior fossa down to the jugular foramen and included a trajectory along the long axis of the tumor (retrosigmoid). Although we hypothesize this was a vestibular schwannoma, it was difficult to ascertain the exact origin of the tumor because of its massive size. Surgery was performed in 2 stages because of the large tumor size and to limit blood loss. A gross total resection was achieved. Closure included an autologous fat and synthetic cranioplasty. The patient was neurologically stable postoperatively, except for transient swallowing difficulty due to partial cranial nerve IX/X palsies. This case illustrates important considerations when combining surgical approaches for complex tumors involving multiple intracranial compartments.