Multicenter US Clinical Trial With an Electric-Acoustic Stimulation (EAS) System in Adults: Final Outcomes.

OBJECTIVE: To demonstrate the safety and effectiveness of the MED-EL Electric-Acoustic Stimulation (EAS) System, for adults with residual low-frequency hearing and severe-to-profound hearing loss in the mid to high frequencies. STUDY DESIGN: Prospective, repeated measures. SETTING: Multicenter, hospital. PATIENTS: Seventy-three subjects implanted with PULSAR or SONATA cochlear implants with FLEX electrode arrays. INTERVENTION: Subjects were fit postoperatively with an audio processor, combining electric stimulation and acoustic amplification. MAIN OUTCOME MEASURES: Unaided thresholds were measured preoperatively and at 3, 6, and 12 months postactivation. Speech perception was assessed at these intervals using City University of New York sentences in noise and consonant-nucleus-consonant words in quiet. Subjective benefit was assessed at these intervals via the Abbreviated Profile of Hearing Aid Benefit and Hearing Device Satisfaction Scale questionnaires. RESULTS: Sixty-seven of 73 subjects (92%) completed outcome measures for all study intervals. Of those 67 subjects, 79% experienced less than a 30 dB HL low-frequency pure-tone average (250-1000 Hz) shift, and 97% were able to use the acoustic unit at 12 months postactivation. In the EAS condition, 94% of subjects performed similarly to or better than their preoperative performance on City University of New York sentences in noise at 12 months postactivation, with 85% demonstrating improvement. Ninety-seven percent of subjects performed similarly or better on consonant-nucleus-consonant words in quiet, with 84% demonstrating improvement. CONCLUSION: The MED-EL EAS System is a safe and effective treatment option for adults with normal hearing to moderate sensorineural hearing loss in the low frequencies and severe-to-profound sensorineural hearing loss in the high frequencies who do not benefit from traditional amplification.

Facial Nerve Outcome and Tumor Control Rate as a Function of Degree of Resection in Treatment of Large Acoustic Neuromas: Preliminary Report of the Acoustic Neuroma Subtotal Resection Study (ANSRS).

BACKGROUND: Patients with large vestibular schwannomas are at high risk of poor facial nerve (cranial nerve VII [CNVII]) function after surgery. Subtotal resection potentially offers better outcome, but may lead to higher tumor regrowth. OBJECTIVE: To assess long-term CNVII function and tumor regrowth in patients with large vestibular schwannomas. METHODS: Prospective multicenter nonrandomized cohort study of patients with vestibular schwannoma ≥2.5 cm who received gross total resection, near total resection, or subtotal resection. Patients received radiation if tumor remnant showed signs of regrowth. RESULTS: Seventy-three patients had adequate follow-up with mean tumor diameter of 3.33 cm. Twelve received gross total resection, 22 near total resection, and 39 subtotal resection. Fourteen (21%) remnant tumors continued to grow, of which 11 received radiation, 1 had repeat surgery, and 2 no treatment. Four of the postradiation remnants (36%) required surgical salvage. Tumor regrowth was related to non-cystic nature, larger residual tumor, and subtotal resection. Regrowth was 3 times as likely with subtotal resection compared to gross total resection and near total resection. Good CNVII function was achieved in 67% immediately and 81% at 1-year. Better immediate nerve function was associated with smaller preoperative tumor size and percentage of tumor left behind on magnetic resonance image. Degree of resection defined by surgeon and preoperative tumor size showed weak trend toward better late CNVII function. CONCLUSION: Likelihood of tumor regrowth was 3 times higher in subtotal resection compared to gross total resection and near total resection groups. Rate of radiation control of growing remnants was suboptimal. Better immediate but not late CNVII outcome was associated with smaller tumors and larger tumor remnants. ABBREVIATIONS: CNVII, cranial nerve VIIGTR, gross total resectionHB, House-BrackmannMRI, magnetic resonance imageNTR, near total resectionSTR, subtotal resection.

Intracranial Facial Nerve Grafting in the Setting of Skull Base Tumors: Global and Regional Facial Function Analysis and Possible Implications for Facial Reanimation Surgery.

BACKGROUND: Reconstructive surgeons may encounter patients presenting after intracranial facial nerve resection and grafting in the setting of skull base tumors, who inquire regarding progression, final facial function, and need for future operations. Study goals were to analyze global and regional facial function using established grading systems and videography, while examine variables possibly affecting outcomes. METHODS: Between 1997 and 2012, 28 patients underwent intracranial nerve grafting. Fifteen were prospectively evaluated by three facial nerve physical therapists with the Facial Nerve Grading System 2.0 and the Sunnybrook Facial Grading Score for function and the Facial Disability Index for quality of life. Still photographs and videography were used to assess quality of motion and tone, while demographic and medical variables were analyzed regarding their effect on end results. RESULTS: Average patient age was 41.9 years (range, 22 to 66 years), and there were 10 women and five men. Average time interval between nerve grafting and evaluations was 42.9 months (range, 12 to 146 months). Both grading scores demonstrated best outcomes in the periorbita and worst outcomes in the brow. Buccinator muscle tone also improved. The average total Facial Disability Index was 67.5 percent. Although not statistically significant, the data suggest that nerve gap length affected total resting symmetry and voluntary movement, whereas preoperative palsy and age may affect total resting symmetry. Perioperative radiation therapy, tumor type, donor nerve, and coaptation technique were not found to affect outcomes. CONCLUSIONS: Intracranial facial nerve grafting largely provides better resting tone and facial symmetry, potentially improving end results of future intervention; however, overall voluntary facial motion is poor. CLINICAL QUESTION/LEVEL OF EVIDENCE: Therapeutic, IV.

En bloc resection of the temporal bone and temporomandibular joint for advanced temporal bone carcinoma.

Advanced skin malignancies involving the temporal bone can involve the temporomandibular joint and glenoid fossa. Many of these tumors can be removed with a lateral temporal bone resection; however, extensive involvement of the glenoid fossa should include an en bloc resection of the temporal bone, glenoid fossa, and condyle. We describe a novel surgical approach that is an extension of a temporal bone resection that includes the glenoid fossa and condyle in an en bloc resection with the temporal bone. This procedure has been performed in 7 patients with advanced carcinoma of the temporal bone involving the glenoid fossa. There were no short-term complications as a result of the surgical approach. The addition of a middle fossa craniotomy and inclusion of the glenoid fossa and condyle as part of an en bloc resection of the temporal bone can be performed safely.

Utility of MRIs in adult cochlear implant evaluations.

OBJECTIVES: To determine the prevalence of MRI abnormalities in adults undergoing cochlear implantation and to correlate abnormalities to audiology data. STUDY DESIGN: Case series. SETTING: Academic medical center. METHODS: Adult patients (>18 yr old) undergoing cochlear implant evaluation from January 2008 to December 2012 were identified based on CPT code search. Demographics, preoperative MRI findings, operative findings, and audiometric data were collected. RESULTS: The study included 188 patients. Seventeen (9%) patients had significant otic capsule or vestibulocochlear nerve pathologies: 5 vestibular schwannomas, 4 enlarged vestibular aqueducts, 2 hypoplastic cochlear nerves, 2 labyrinthitis ossificans, 1 cochlear aplasia, 1 posterior semicircular canal malformation, 1 calcified meningioma, and 1 cholesterol granuloma. MRI results were normal (65%) or with findings not directly related to hearing loss (incidental findings, 25%) in the remaining patients. Mean pure tone average (PTA) differences (between the implanted and contralateral ear) did not significantly vary between normal-incidental and abnormal MRI scans (-6.6 dB versus -6.7 dB, p = 0.99) nor did speech discrimination scores (SDS) scores (8.5% versus 8.4%, p = 0.99). No significant difference was found in HINT scores for patients with a normal versus an abnormal MRI (19% versus 16,%, p = 0.62). CONCLUSION: Although the majority of precochlear implantation MRIs were normal or demonstrated incidental findings, such as white matter changes, significant MRI findings affecting implantation site and patient counseling were found in almost 10% of patients. Audiogram findings did not correlate with abnormal findings on MRIs. Routine use of MRI in adult cochlear implant candidates may be warranted.

Clinical practice guideline: acute otitis externa.

OBJECTIVE: This clinical practice guideline is an update and replacement for an earlier guideline published in 2006 by the American Academy of Otolaryngology-Head and Neck Surgery Foundation. This update provides evidence-based recommendations to manage acute otitis externa (AOE), defined as diffuse inflammation of the external ear canal, which may also involve the pinna or tympanic membrane. The variations in management of AOE and the importance of accurate diagnosis suggest a need for updating the clinical practice guideline. The primary outcome considered in this guideline is clinical resolution of AOE. PURPOSE: The primary purpose of the original guideline was to promote appropriate use of oral and topical antimicrobials for AOE and to highlight the need for adequate pain relief. An updated guideline is needed because of new clinical trials, new systematic reviews, and the lack of consumer participation in the initial guideline development group. The target patient is aged 2 years or older with diffuse AOE. Differential diagnosis will be discussed, but recommendations for management will be limited to diffuse AOE, which is almost exclusively a bacterial infection. This guideline is intended for primary care and specialist clinicians, including otolaryngologists-head and neck surgeons, pediatricians, family physicians, emergency physicians, internists, nurse practitioners, and physician assistants. This guideline is applicable in any setting in which patients with diffuse AOE would be identified, monitored, or managed. ACTION STATEMENTS: The development group made strong recommendations that (1) clinicians should assess patients with AOE for pain and recommend analgesic treatment based on the severity of pain and (2) clinicians should not prescribe systemic antimicrobials as initial therapy for diffuse, uncomplicated AOE unless there is extension outside the ear canal or the presence of specific host factors that would indicate a need for systemic therapy. The development group made recommendations that (1) clinicians should distinguish diffuse AOE from other causes of otalgia, otorrhea, and inflammation of the external ear canal; (2) clinicians should assess the patient with diffuse AOE for factors that modify management (nonintact tympanic membrane, tympanostomy tube, diabetes, immunocompromised state, prior radiotherapy); (3) clinicians should prescribe topical preparations for initial therapy of diffuse, uncomplicated AOE; (4) clinicians should enhance the delivery of topical drops by informing the patient how to administer topical drops and by performing aural toilet, placing a wick, or both, when the ear canal is obstructed; (5) clinicians should prescribe a non-ototoxic preparation when the patient has a known or suspected perforation of the tympanic membrane, including a tympanostomy tube; and (6) clinicians should reassess the patient who fails to respond to the initial therapeutic option within 48 to 72 hours [corrected] to confirm the diagnosis of diffuse AOE and to exclude other causes of illness.

Clinical practice guideline: acute otitis externa executive summary.

The American Academy of Otolaryngology--Head and Neck Surgery Foundation (AAO-HNSF) has published a supplement to this issue featuring the updated Clinical Practice Guideline: Acute Otitis Externa, as a supplement to Otolaryngology-Head and Neck Surgery. To assist in implementing the guideline recommendations, this article summarizes the rationale, purpose, and key action statements. The 8 recommendations developed address appropriate diagnosis of acute otitis externa (AOE) and the use of oral and topical antimicrobials and highlight the need for adequate pain relief. An updated guideline is needed due to new clinical trials, new systematic reviews, and the lack of consumer participation in the initial guideline development group.

Elevated intracranial pressure in patients with spontaneous cerebrospinal fluid otorrhea.

OBJECTIVES/HYPOTHESIS: To determine the prevalence of elevated intracranial hypertension in patients with spontaneous cerebrospinal fluid otorrhea (SCSFO). STUDY DESIGN: Case series with chart review at a tertiary care academic medical center following institutional review board approval. METHODS: A retrospective review was performed of patients undergoing operative repair of SCSFO between January 2007 and May 2012. RESULTS: Thirty-eight patients underwent operative repair of SCSFO. Of these, 22 underwent postoperative lumbar puncture with measurement of opening pressure. The opening pressure was elevated (> 20 cm/H2 0) in eight patients (36.4%). Preoperative magnetic resonance imaging was available for review by a neuroradiologist in 27 patients. Radiographic evidence of elevated intracranial pressure (ICP) was present in 48.1% of patients. CONCLUSION: Elevated ICP is common in patients with SCSFO. However, as only a minority of patients have elevated ICP, it is not the sole factor in the development of SCSFO.

Ciprofloxacin 0.3% + dexamethasone 0.1% for the treatment for otitis media.

INTRODUCTION: Ciprofloxacin 0.3% with dexamethasone 0.1% (ciprofloxacin/dexamethasone) is an ototopical preparation for acute otitis externa, otorrhea with tympanostomy tubes, and is frequently used to treat chronic suppurative otitis media (CSOM). The advantage of topical therapy is the ability to deliver higher concentration of antibiotics to the treatment site when compared with oral or parenteral antibiotics. The delivery of a high concentration of antibiotics significantly decreases treatment failure and makes the development of resistant organisms unlikely. Previous ototopical preparations contained antibiotics such as aminoglycosides that are known to be ototoxic making treatment of otic infections without an intact tympanic membrane difficulty. AREAS COVERED: A literature search of PubMed was performed as the basis for a literature-based discussion on the clinical efficacy of ciprofloxacin/dexamethasone compared to oral antibiotics and ototopical therapy without a steroid component. The potential ototoxicity of ototopical therapies is discussed, including evidence demonstrating the lack of ototoxicity of fluoroquinolone and dexamethasone containing drops. EXPERT OPINION: Because multiple studies have demonstrated that fluoroquinolones are not ototoxic, fluoroquinolone ototopical drops should be a first-line treatment for otorrhea without an intact membrane. The addition of dexamethasone 0.1% to ciprofloxacin 0.3% has been shown to decrease granulation tissue, improve clinical cure and achieve greater rates of bacterial eradication when compared to ciprofloxacin 0.3% alone.

A tribute to a remarkably sound solution.

For their work on the development of the modern cochlear implant, which bestows hearing to individuals with profound deafness, Ingeborg Hochmair, Graeme Clark, and Blake Wilson are the 2013 recipients of the Lasker∼DeBakey Clinical Medical Research Award.

Pediatric stapedectomy: does cause of fixation affect outcomes?

OBJECTIVE: To compare outcomes of stapedectomy in patients with congenital stapes fixation versus juvenile otosclerosis. METHODS: A retrospective chart review was performed from January 1, 1999 until January 1, 2011 to identify patients under 18 years old who underwent a stapedectomy. Age, gender, pre- and postoperative audiograms, intraoperative findings including etiology of stapes fixation, prosthesis type, and complications were recorded. RESULTS: Twenty-two children were identified who had undergone a stapedectomy (two patients underwent sequential bilateral surgery) resulting in a total of 24 ears. The cause of fixation included juvenile otosclerosis (n=7) and congenital stapes fixation (n=17). The overall mean pre-operative air-bone gap (ABG) was 34.7 dB (SD: 13.5) compared to a postoperative mean ABG of 9.0 (SD: 9.3) (p<0.001). The mean postoperative ABG of 9.6 (SD: 10.5) in the congenital stapes fixation group was similar to the mean postoperative ABG of 7.2dB (SD: 5.4) in children with juvenile otosclerosis (p=0.6). Two patients developed delayed profound sensorineural hearing loss approximately two weeks after surgery. One patient with profound sensorineural hearing loss recovered to a profound mixed hearing loss with a speech discrimination score of 80%. CONCLUSIONS: Pediatric stapedectomy has comparable results to stapedectomy in adults regardless of the cause of stapes fixation; however, delayed sensorineural hearing loss may be higher in the pediatric population.

A comparative study of psychosocial development in children who receive cochlear implants.

OBJECTIVES: This study assessed self-reported quality of life of children with a cochlear implant (CI), comparing results with two published reports from the past decade. METHODS: Participants included 33 pediatric CI recipients with a mean age of 10.12 years (SD = 3.59), mean implantation age of 1.36 years (SD = 0.46), and mean CI experience of 6.23 years (SD = 1.75). Children in all three studies completed a self-report quality-of-life questionnaire that included seven benefit and six problem items, rated on a 5-point Likert scale. RESULTS: Outcomes revealed agreement across studies in overall benefit and problem ratings. Environmental awareness and frustration reduction were the most and least positively rated outcomes, respectively. Items contributing to overall ratings differed across studies. Current CI recipients rated speech production, making new friends, and understanding speech more positively and taking extra care of the device more negatively than previous generations of pediatric CI users. DISCUSSION: Overall, benefits outweigh problems of the device, according to children using CI. Differences in issues motivating self-report ratings reflect changes in CI candidacy, technology, and social participation over the past decade. These findings emphasize the need for clinicians to address not only communication needs, but also quality-of-life issues to optimize outcomes in children using CI.

Draining ears and tympanostomy tubes: a survey of pediatric otolaryngologists and pediatric emergency medicine physicians.

INTRODUCTION: Posttympanostomy tube otorrhea also known as acute otitis media with tympanostomy tubes (AOMT) occurs in 15% to 80% of children with tympanostomy tubes. Its management is fairly standardized among pediatric ear, nose, and throat (ENT) physicians owing to recommendations published by the American Academy of Otolaryngology-Head and Neck Surgery. Pediatric emergency medicine (EM) physicians have no such guidelines. OBJECTIVE: This study aimed to compare management of AOMT by pediatric ENT and EM physicians. METHODS: A 27-question online survey was disseminated via SurveyMonkey.com using e-mail addresses of ENT and EM physicians via organization directories and professional listserves. RESULTS: A total of 175 and 174 responses were received from EM and ENT physicians, respectively. Higher proportion of EM physicians used oral antibiotics to treat AOMT (54% [n = 94] vs 9% [n = 16], P < 0.001). Virtually all ENT physicians used topical antibiotics, compared with 87% of EM physicians. Only 6% (n = 10) of EM physicians used suction to clean ear canals (aural toilet) before instilling topical antibiotics, compared with 81% (n = 138) of ENT physicians. Most ENT physicians (80% [n = 138]) instructed patients to keep the treated ear up for 10 to 60 seconds after instilling the drops and to use the tragal pump technique to direct the medication down the ear canal and through the tube (92% [n = 157]). Only 56% (n = 98) and 24% (n = 41) of EM physicians did the same. CONCLUSIONS: There are large differences between ENT and EM physicians with respect to: the use of systemic antibiotics, techniques of using ototopical antibiotics, methods of aural toilet in treating AOMT, and directions given to patients.

Vascular trauma during cochlear implantation: a contributor to residual hearing loss?

OBJECTIVE: This study was performed to investigate the vascular structures of the cochlea that are potentially vulnerable to mechanical trauma during cochlear implant surgery. BACKGROUND: Despite improvements in surgical technique and electrode design, residual hearing is lost in a significant percentage of cochlear implant patients. Although a variety of factors may contribute, it is widely believed that mechanical trauma plays an important role. This study focused on the vasculature of scala tympani and its potential susceptibility to injury during implant surgery. METHODS: Anatomic study of normal human temporal bones prepared by either conventional cross sectioning or by microdissection for examination by light or scanning electron microscopy. RESULTS: Blood vessels located at or near the perilymphatic surface of scala tympani are predominately of the venous type, and they are situated so as to be at risk for trauma during implantation. Those vessels include veins on the lateral wall and floor of scala tympani, the modiolar wall and the undersurface of the osseous lamina and basilar membrane. CONCLUSION: Injury or occlusion of blood vessels associated with scala tympani may adversely affect inner ear function, potentially contributing to hearing loss following cochlear implantation.

Superior semicircular canal dehiscence in patients with spontaneous cerebrospinal fluid otorrhea.

OBJECTIVE: To determine the prevalence of superior semicircular canal dehiscence (SCD) in patients with spontaneous cerebrospinal fluid (CSF) otorrhea. STUDY DESIGN: Case series with chart review. SETTING: Tertiary care referral center. SUBJECTS AND METHODS: Patients included have undergone a middle fossa craniotomy for repair of spontaneous CSF otorrhea between January 2007 and December 2011. The main outcome measure is the presence or absence of SCD observed during spontaneous CSF leak repair. Computed tomography (CT) imaging was also reviewed to determine the diagnostic accuracy of this modality. RESULTS: Thirty-three ears in 31 patients underwent surgical repair for spontaneous CSF otorrhea via a middle fossa craniotomy. The average age at the time of repair was 60.5 years, and 80.6% of patients were female. A dehiscence of the superior canal was observed in 15.2% of ears (16.1% of individuals). No significant difference in age, body mass index, or sex was noted between those patients with or without a superior canal dehiscence. For the diagnosis of SCD, coronal CT was 100% sensitive and 91.7% specific. The positive predictive value and negative predictive value of CT were 66.7% and 100%, respectively. CONCLUSION: The prevalence of superior semicircular canal dehiscence in ears with spontaneous otorrhea is 15.2%. This prevalence is greater than the 0.5% reported in a temporal bone study of ears not selected for CSF otorrhea.

Management of electrode exposure after cochlear implantation.

BACKGROUND: Exposure of a cochlear implant electrode array in the middle ear or external auditory canal is an uncommon complication. We report a series of 7 patients with electrode array exposure, 5 of whom were managed without surgical intervention. OBJECTIVE: To report the outcomes of patients with electrode array exposure after cochlear implantation. STUDY DESIGN: Retrospective case series. SETTING: Tertiary neurotology practice. PATIENTS: Patients with cochlear implant electrode exposure in the middle ear or external auditory canal. MAIN OUTCOME MEASURE: Length of observation without deterioration of implant function and without complications. RESULTS: Five patients were managed without revision surgery. One patient presented with a poorly functioning implant, but after reprogramming, the implant returned to its baseline performance. Four other patients required no reprogramming and were managed medically without deterioration of implant function. Follow-up time was between 2 and 6 years. There were no serious complications in any patient related to their cochlear implant. CONCLUSION: Careful observation of patients with an exposed electrode array after cochlear implantation and a functioning implant seems to be a reasonable management option in select cases.

Cochlear implant device failure secondary to cholesterol granuloma-mediated cochlear erosion.

STUDY DESIGN: Case report. SETTING: A tertiary care pediatric hospital. PATIENT: A 7-year-old boy complained of retroauricular pain 3.5 years after cochlear implantation. Temporal bone computed tomographic scan revealed a soft tissue density filling the mastoid and middle ear space. There was extensive osseous erosion involving the cochlear promontory that resulted in lateral displacement electrode array into the middle ear space. INTERVENTION: Mastoidectomy with removal of cochlear implant. RESULTS: Pathologic examination of the soft tissue filling the mastoid cavity was consistent with a cholesterol granuloma. The cultures of the mastoid cavity demonstrated no growth. Examination of the explanted device revealed a cell layer covering the length of the electrode. The stylet tract was filled with cells that grew out through the silicone component. No birefringent cholesterol crystals were detected in the tissue surrounding the electrode. Biofilms were not identified. CONCLUSION: The authors describe a unique case of a cholesterol granuloma of the middle ear and mastoid in a cochlear implant recipient causing erosion of the otic capsule and displacement of the cochlear implant electrode array into the middle ear space.