Changing Management of Intravestibular Schwannomas in the Era of Cochlear Implantation for Single-Sided Deafness.

OBJECTIVE: Intralabyrinthine schwannomas (ILSs) are a rare cause of deafness. Patients with ILS confined to the semicircular canals and the vestibule (intravestibular schwannomas) are potential candidates for cochlear implantation for hearing rehabilitation, a new option for patients with unilateral hearing loss since the 2019 FDA approval of cochlear implant (CI) for single-sided deafness. In this report, we describe an evolving management approach for ILSs causing hearing loss. PATIENTS: Adults (≥18 years) who underwent simultaneous ILS resection and CI between January 2019 and June 2023 (n = 3). INTERVENTION: Transmastoid labyrinthectomy with simultaneous cochlear implantation. MAIN OUTCOME MEASURES: Hearing performance with cochlear implantation measured as CNC Word Recognition scores and AzBio Sentence scores. RESULTS: Three patients with ILS confined to the semicircular canals and vestibule underwent simultaneous tumor resection via labyrinthectomy with CI placement. In all cases, complete tumor resection and full CI insertion were achieved. No patients experienced postoperative complications. Patients 1 and 2 underwent 6- and 9-month postactivation testing, respectively, with CNC scores 64% to 80% and AzBio 81% to 99% in the implanted ears. Patient 3 scored 0% on CNC and AzBio testing at 3 months and deferred her 6-month audiometry. CONCLUSIONS: Patients with ILS confined to the vestibule and semicircular canals can be considered for simultaneous tumor resection and CI placement.

Superior Canal Dehiscence Syndrome Affecting 3 Families.

Importance: Superior canal dehiscence syndrome (SCDS) is an increasingly recognized cause of hearing loss and vestibular symptoms, but the etiology of this condition remains unknown. Objective: To describe 7 cases of SCDS across 3 families. Design, Setting, and Participants: This retrospective case series included 7 patients from 3 different families treated at a neurotology clinic at a tertiary academic medical center from 2010 to 2014. Patients were referred by other otolaryngologists or were self-referred. Each patient demonstrated unilateral or bilateral SCDS or near dehiscence. Interventions: Clinical evaluation involved body mass index calculation, audiometry, cervical vestibular evoked myogenic potential testing, electrocochleography, and multiplanar computed tomographic (CT) scan of the temporal bones. Zygosity testing was performed on twin siblings. Main Outcomes and Measures: The diagnosis of SCDS was made if bone was absent over the superior semicircular canal on 2 consecutive CT images, in addition to 1 physiologic sign consistent with labyrinthine dehiscence. Near dehiscence was defined as absent bone on only 1 CT image but with symptoms and at least 1 physiologic sign of labyrinthine dehiscence. Results: A total of 7 patients (5 female and 2 male; age range, 8-49 years) from 3 families underwent evaluation. Family A consisted of 3 adult first-degree relatives, of whom 2 were diagnosed with SCDS and 1 with near dehiscence. Family B included a mother and her child, both of whom were diagnosed with unilateral SCDS. Family C consisted of adult monozygotic twins, each of whom was diagnosed with unilateral SCDS. For all cases, dehiscence was located at the arcuate eminence. Obesity alone did not explain the occurrence of SCDS because 5 of the 7 cases had a body mass index (calculated as weight in kilograms divided by height in meters squared) less than 30.0. Conclusions and Relevance: Superior canal dehiscence syndrome is a rare, often unrecognized condition. This report of 3 multiplex families with SCDS provides evidence in support of a potential genetic contribution to the etiology. Symptomatic first-degree relatives of patients diagnosed with SCDS should be offered evaluation to improve detection of this disorder.

Surgical treatment of hearing loss when otosclerosis coexists with superior semicircular canal dehiscence syndrome.

OBJECTIVE: Document a case of bilateral otosclerosis with coexisting bilateral superior semicircular canal dehiscence syndrome and the treatment of hearing loss in this setting. PATIENT: A 33-year-old woman presented with bilateral mixed hearing loss; worse in the left ear. This was gradual in onset, and she denied dizziness. Computerized tomographic scan revealed fenestral otosclerosis and a large dehiscence of the superior semicircular canal bilaterally. She declined amplification. INTERVENTION: Sequential laser-assisted stapedotomy with insertion of a Kurz titanium CliP Piston prosthesis. MAIN OUTCOME MEASURE: Comparison of audiovestibular symptoms, hearing thresholds, and neurodiagnostic testing results preoperatively and postoperatively. RESULTS: Hearing improved bilaterally with closure of the air-bone gaps at most frequencies, and she has not had permanent vestibular symptoms. Postoperative follow-up time is 37 months for the left ear and 13 months for the right ear. CONCLUSION: When otosclerosis and superior semicircular canal dehiscence syndrome coexist and hearing loss is the dominant symptom, stapes surgery can be effective for improving hearing without permanent vestibular symptoms.

Iatrogenic cholesteatoma arising at the bony-cartilaginous junction of the external auditory canal: a late sequela of intact canal wall mastoidectomy.

OBJECTIVE: The objective of this study is to describe the presentation and management of a rare site of cholesteatoma recurrence at the bony-cartilaginous junction after intact canal wall (ICW) mastoidectomy. STUDY DESIGN: Retrospective case series SETTING: Tertiary referral center PATIENTS: Patients with cholesteatoma formation arising from the bony-cartilaginous (BC) junction of the external auditory canal (EAC) requiring surgical intervention were retrospectively identified across a 5-year period. INTERVENTION(S): All patients were treated surgically to eradicate the disease and reconstruct the bony defect when possible. MAIN OUTCOME MEASURE(S): This observational study details the presentation, risk factors, and management of a rare site of cholesteatoma recurrence after ICW mastoidectomy. RESULTS: After ICW mastoidectomy, eight patients were identified with fistulae in the lateral EAC near the BC junction. Seven patients had associated iatrogenic cholesteatomas arising at this site, and one patient had a dry fistula with bony stenosis of the EAC. All patients had a history of chronic otitis media and previous surgery. Patients averaged 9 years between surgery and recidivism. Reconstruction of the bony defect was completed using hydroxyapatite reconstruction plates in four patients with 75% success, soft wall reconstruction in two patients using temporalis muscle, and canal wall down mastoidectomy in two patients who had extensive disease and exposed dura. No recurrent disease was evident during an average follow-up of 16 months. EAC reconstruction was successful in 83% of cases. CONCLUSION: This case series reports a novel pattern of iatrogenic cholesteatoma formation near the BC junction of the EAC that can occur years after ICW mastoidectomy. In properly selected cases, this condition can be managed with revision ICW mastoidectomy and reconstruction.

Durability of hearing preservation after microsurgical treatment of vestibular schwannoma using the middle cranial fossa approach.

OBJECT: The middle cranial fossa (MCF) approach is a microsurgical technique described as a primary option in the treatment of small, intracanalicular schwannomas involving the eighth cranial nerve. Excellent rates of complete tumor resection, hearing preservation, preservation of facial nerve function, and low complication rates have been reproduced using this technique. However, the durability of hearing preservation attained using the various treatment options has not been adequately assessed. The purpose of this study was to evaluate the durability of long-term hearing preservation in patients with vestibular schwannoma (VS) treated via the MCF approach. The authors hypothesize that hearing preservation in these patients will prove to be durable years after treatment in a high percentage of cases. METHODS: Retrospective medical chart review was performed in 103 consecutive patients undergoing resection of VS via a modified MCF approach between 1999 and 2008. Patients in whom surgical goals were gross-total resection and hearing preservation were included. Preoperative and postoperative hearing assessment was performed using standard audiometric testing, and classified according to American Academy of Otolaryngology-Head and Neck Surgery (AAO-HNS) guidelines as a primary outcome measure. Outcomes and neurological complications initially, and at 1, 3, and 5 years following operation were analyzed. RESULTS: Initial hearing preservation rates were in keeping with the best previously published results. At initial postoperative audiometric follow-up, of the patients presenting with Class A hearing, 67% remained Class A, 17% were Class B, 1% were Class C, and 15% were Class D. Of patients presenting with Class B hearing, 24% were Class A, 53% remained Class B, 6% were Class C, and 18% were Class D. Of patients presenting with Class C hearing, 100% remained Class C. To assess the durability of hearing preservation in our patients, the authors evaluated hearing function at regular intervals after the initial postoperative audiometric follow-up. Audiometric data were available for 56 patients at 5-year follow-up. Of the 20 patients with Class A hearing at initial postoperative follow-up with 5-year follow-up, 13 (65%) remained Class A, 6 (30%) were Class B, and 1 (5%) was Class C. Of the 12 patients with Class B hearing at initial postoperative follow-up with 5-year follow-up, 4 (33%) were Class A, 4 (33%) remained Class B, and 4 (33%) were Class C. Of the 3 patients with Class C hearing at initial postoperative follow-up with 5-year follow-up, all 3 (100%) remained Class C. CONCLUSIONS: A majority of patients with preserved hearing following the MCF approach for treatment of VS experience durability of their preserved hearing at 5-year follow-up. The initial AAO-HNS classification was preserved in 13 (65%) of the 20 patients who had Class A hearing at 5 years, and in 8 (67%) of the 12 who had Class B hearing at 5 years. Overall, a decline in AAO-HNS classification was noted in 15% of patients with preserved Class A hearing, and in 33% of those with preserved Class B hearing. Facial nerve function was preserved in 91% of cases. Superior hearing preservation as well as good outcomes in facial nerve function and few serious complications can be accomplished using the MCF approach for resection of small VSs.

Squamous cell carcinoma involving the temporal bone: lateral temporal bone resection as primary intervention.

OBJECTIVE: To provide an up-to-date review of treatment and outcomes of patients with squamous cell carcinoma (SCCA) involving the temporal bone. DESIGN: Retrospective cohort study of all patients treated at our institution for SCCA of the temporal bone between 1995 and 2007 with follow-up until 2011. Factors evaluated were demographics, presenting findings, follow-up time, previous treatment, workup, stage, surgical intervention, adjuvant therapy, histopathologic findings, recurrence, and survival. SETTING: Tertiary care academic medical center. PATIENTS: Thirty patients with SCCA of the temporal bone, originating from the external auditory canal and adjacent sites. INTERVENTION: Surgical resection ± adjuvant therapy. Lateral temporal bone resection was the primary and most aggressive procedure performed. MAIN OUTCOME MEASURES: Disease-free survival. RESULTS: The overall disease free survival for this series when considering both external auditory canal and adjacent site SCCAs was 70%. When evaluated by tumor stage, disease-free survival was as follows: T1 tumors = 100%, T2 tumors = 100%, T3 tumors = 67%, and T4 tumors = 56%. Aggressive tumors of this series originating at periauricular sites behaved in a similar manner to primary canal tumors. Need for surgical resection of CN VII was associated with diminished survival on multivariate analysis. CONCLUSION: Lateral temporal bone resection provides comparable disease free survival rates to more radical surgical therapy. Such resection is appropriate for many SCCAs of the external auditory canal and adjacent sites, as these tumors are similar in disease progression and prognosis. CN VII involvement portends a poor outcome.

Transcanal labyrinthectomy for intractable vertigo after unilateral cochlear implantation.

OBJECTIVE: Document the use of transcanal labyrinthectomy to treat disabling attacks of vertigo after unilateral cochlear implantation. PATIENT: A 46-year-old woman with severe-profound bilateral sensorineural hearing loss secondary to enlarged vestibular aqueducts underwent cochlear implantation for her right ear with a Nucleus Freedom device. The surgery was uneventful, and postoperative imaging confirmed that the electrode was positioned properly. She developed episodic vertigo 10 to 14 days after the implant surgery, which failed to improve with aggressive vestibular rehabilitation therapy. Plugging of the round window for possible perilymphatic fistula did not relieve her symptoms. INTERVENTION: Right transcanal labyrinthectomy supplemented by filling the vestibule with gentamicin-soaked Gelfoam and then a customized vestibular rehabilitation program. MAIN OUTCOME MEASURE: Comparison of vestibular symptoms and cochlear implant performance before and after transcanal labyrinthectomy. RESULTS: The patient had immediate relief of symptoms, and the function of the cochlear implant was not adversely affected. CONCLUSION: Transcanal labyrinthectomy may be an effective method to ablate the vestibular end organ after unilateral cochlear implantation. It can offer relief of disabling vertigo without adversely affecting the performance of the implant.

Facial nerve outcomes in middle cranial fossa vs translabyrinthine approaches.

OBJECTIVE: To compare the final facial nerve outcomes between middle cranial fossa (MCF) vs translabyrinthine (TL) resection of size-matched vestibular schwannomas. STUDY DESIGN AND SETTING: Retrospective case review at a tertiary care hospital. All patients who underwent resection utilizing either MCF or TL approaches with tumors 18 mm or smaller and complete data were included in the analysis. One hundred twenty-four patients were identified meeting the above criteria, with sixty-three in the translabyrinthine group and sixty-one in the middle fossa group. One-week-postoperative and final facial nerve examinations were compared in the two surgical groups. Patients were separately analyzed in subgroups: tumors smaller than 10 mm and those that were between 10 and 18 mm. RESULTS: The tumor size range for the MCF group was 3-18 mm while it was 4-18 mm for the TL group. No statistically significant difference was found in facial nerve outcomes between the two surgical groups, at the first postoperative visit week and at last follow-up. CONCLUSION: Facial nerve outcomes are similar using TL and MCF approaches for resection of vestibular schwannomas up to 18 mm in size. SIGNIFICANCE: Patients undergoing the MCF approach for hearing preservation can be counseled that there is no increased risk of permanent facial nerve weakness, compared to the TL approach.

Corticosteroid response and supporting cell antibody in autoimmune hearing loss.

OBJECTIVE: To determine whether antibodies to supporting cells are associated with response to corticosteroids in patients with autoimmune sensorineural hearing loss. DESIGN: Prospective analysis of antibody to inner-ear antigens. SETTING: Collaborating otology practices in Pennsylvania, Michigan, and Indiana. PATIENTS: Sixty-three patients with rapidly progressive unilateral or bilateral sensorineural hearing loss of unknown cause suggestive of autoimmune sensorineural hearing loss. INTERVENTIONS: Pretreatment audiometry, serum analysis by Western blot (WB) and immunofluorescence (IF) tests, corticosteroid therapy, and follow-up audiometry. MAIN OUTCOME MEASURES: Antibody reactivity and audiogram changes were analyzed for association with response to treatment. RESULTS: More than half of the patients (37/63) had antibodies to both a 68- to 72-kDa protein and to inner-ear supporting cells, 16 patients had positive results on one assay only, and 10 had negative results on both. Twenty-eight patients improved and 35 did not. The WB findings did not correlate with response. Of the WB-positive patients, 49% (21/43) improved, as did 35% (7/20) of the WB-negative patients (P = .30). In contrast, 53% (25/47) of IF-positive patients improved, compared with only 19% (3/16) in the IF-negative group (P = .02). Of those who improved, 89% (25/28) were IF positive. CONCLUSIONS: Antibody to an inner-ear supporting cell antigen was significantly associated with hearing improvement after corticosteroid therapy (relative rate, 2.8). Patients with IF-positive serum are nearly 3 times more likely to experience improved hearing with corticosteroid treatment than those who are IF negative. Antibodies to inner-ear supporting cell antigen may have value in diagnosis and treatment of patients with autoimmune sensorineural hearing loss.

Chronic Pseudomonas infections of cochlear implants.

OBJECTIVE: To discuss chronic, refractory Pseudomonas infections of cochlear implants and their management. DESIGN: Retrospective case series. SETTING: Two university-based cochlear implant programs. PATIENTS: Twenty-eight-year-old (Case 1) and 4-year-old (Case 2), different devices. INTERVENTIONS: Medical and surgical management. MAIN OUTCOME MEASURES: Clinical course. RESULTS: Both patients had delayed presentations, 4 months and 3 years postimplantation, respectively, with fluctuating scalp edema and pain resistant to multiple courses of oral antibiotics. Infections began as localized granulation and progressed to complete encasement of both devices with rubbery, poorly vascularized tissue. In each case, two different strains of multiresistant Pseudomonas aeruginosa were cultured. Infections progressed despite local debridement and targeted antipseudomonal antibiotic coverage, and sensitive organisms continued to appear in cultures of refractory granulation tissue. Both patients underwent partial explantation, with the electrode array left in the cochlea, then received 2 to 3 more months of further medical therapy and observation and then were reimplanted successfully with new devices. Both have shown excellent performance and no sign of recurrent infection. CONCLUSIONS: Infections of cochlear implants are uncommon, and cases of successful conservative management without device explantation have been reported. However, our experience and the implanted device literature suggest that chronic Pseudomonas infections may represent a distinct clinical entity, likely to fail protracted therapy and ultimately require device removal. Fortunately, successful reimplantation is possible.

Prediction of long-term facial nerve outcomes with intraoperative nerve monitoring.

OBJECTIVE: Evaluate the utility of a statistical equation using two independent intraoperative monitoring parameters in predicting long-term facial nerve function. STUDY DESIGN: Retrospective case review. SETTING: Tertiary care hospital. PATIENTS: Sixty patients undergoing resection of vestibular schwannomas with intraoperative facial nerve monitoring at a single institution. INTERVENTION: All patients underwent microsurgical resection of vestibular schwannomas with the use of intraoperative cranial nerve monitoring. MAIN OUTCOME MEASURE: Final facial nerve outcome measured using the House-Brackmann scale at least 6 months after microsurgical resection. RESULTS: Five out of 60 (8.3%) patients demonstrated significant long-term weakness (i.e., House-Brackmann grade III or worse). Intraoperative monitoring parameters (proximal stimulation threshold, proximal-to-distal response amplitude ratio) were accurate in predicting increased risk of long-term facial nerve dysfunction when used in a logistic regression model. A Student's t test confirmed the equation result was statistically significant in differentiating long-term facial nerve outcomes. CONCLUSION: Patients with immediate weakness are at higher risk of having long-term poor facial nerve function. The use of intraoperative monitoring parameters was reliable in predicting facial nerve outcomes. Patients with permanent facial nerve dysfunction often require rehabilitative procedures. The ability to predict facial nerve outcomes with intraoperative monitoring may allow early rehabilitative procedures to improve quality of life and prevent ocular complications.

Cochlear implantation in the chronically diseased ear.

PURPOSE OF REVIEW: The presence of chronic ear disease in patients with profound SNHL presents a challenge to the otologic surgeon and was considered, in the past, a contraindication to cochlear implantation. This review discusses options for cochlear implantation in patients with chronically diseased ears. RECENT FINDINGS: Several management options are available for cochlear implantation in patients with chronic suppurative otitis media (CSOM) and severe to profound sensorineural hearing loss (SNHL). CONCLUSION: Cochlear implantation can be safely achieved in patients with CSOM. The approach chosen should be individualized based on clinical findings.

External auditory canal closure in cochlear implant surgery.

OBJECTIVE: To evaluate surgical techniques and complications associated with external auditory canal (EAC) closure in cochlear implant surgery. STUDY DESIGN: Retrospective case review. SETTING: Tertiary referral center with a large cochlear implant program. PATIENTS: Twenty-eight patients (8 adults and 20 children) underwent multichannel cochlear implantation with EAC closure. The follow-up periods ranged between 1 and 10 years. Closure of the EAC was performed in conjunction with implantation of ears with chronic suppurative otitis media or cochlear drill-out procedures for ossification, or for access to the cochlea in patients with abnormal temporal bone anatomy. A modified Rambo technique was used for EAC closure in all but one case. INTERVENTIONS: Cochlear implantation with EAC closure and subsequent clinical and radiologic follow-up. MAIN OUTCOME MEASURES: Postoperative complications or the development of cholesteatoma in the implanted ear. RESULTS: Cholesteatoma developed in the implanted ear in two patients. Breakdown of EAC closure occurred in one of these patients. The details of these patients are reviewed. CONCLUSION: Closure of EAC can be done with relatively low risk. Close and careful follow-up is required for early detection of a developing cholesteatoma.