Michael B. Gluth

Long-term benefit perception, complications, and device malfunction rate of bone-anchored hearing aid implantation for profound unilateral sensorineural hearing loss.

Objective: To longitudinally evaluate short- and long-term subject satisfaction/benefit perception, device usage rates, complication rates, and external device repair rates of bone-anchored hearing aid (BAHA) implantation on a cohort of adult subjects with profound unilateral sensorineural hearing loss (PUSHL). Study Design: Prospective clinical trial. Setting: Tertiary referral center. Patients: Fifty-six adults with PUSHL, 21 of which underwent BAHA implantation (followed for an average of 3.2 years after implantation; range, 0.8-4.6 yr). Main Outcome Measures: Short- and long-term satisfaction/benefit perception outcomes consisting of the Glasgow Hearing Aid Benefit Profile, Abbreviated Profile of Hearing Aid Benefit, and Single-Sided Deafness Questionnaire, including a comparison of results between implanted and nonimplanted subjects. Short- and long-term device usage rates, complications, and device failure issues also were carefully documented. Results: There were statistically significant improvements in nearly all measures of benefit perception documented as well as a high rate of long-term device usage (81%). Although satisfaction and benefit perception outcomes generally tended to regress over time when compared with initial short-term outcomes, long-term scores still tended to be significantly improved nevertheless as compared with preoperative levels. Approximately 38% of implants experienced severe local skin reactions (Grade 2 and above) around the implant site at some point throughout the follow-up period, whereas only one (4.8%) required implant removal. 66.7% of subjects required repair of their external sound processor. Conclusion: BAHA implantation seems to provide a high level of short- and long-term perceived benefit and satisfaction in subjects with PUSHL and high rate of long-term device usage. Implant site adverse local skin reactions and repairs of the external sound processor were quite common.

Facial and Lower Cranial Neuropathies after Preoperative Embolization of Jugular Foramen Lesions with Ethylene Vinyl Alcohol

Objective To report 3 unique cases of cranial neuropathy after super-selective arterial embolization of jugular foramen vascular tumors with ethylene vinyl alcohol. Study Design Clinical capsule report. Setting Three tertiary academic referral hospitals. Patients Three patients who underwent superselective arterial embolization (SSE) of head and neck paragangliomas with ethylene vinyl alcohol are described. One individual was treated with primary SSE, whereas the remaining tumors were treated with preoperative SSE followed by surgical extirpation within 72 hours. All patients were found to have new cranial nerve deficits after SSE. Results One patient with isolated complete cranial nerve VII palsy demonstrated partial return of function at 8 months. One individual experienced cranial nerve VII, X, and XII palsies and demonstrated partial recovery of function of the involved facial nerve after 19 months. One subject experienced ipsilateral cranial nerve X and XI palsies after SSE and recovered full function of the spinal accessory nerve within 1 week but failed to demonstrate mobility of the ipsilateral true vocal fold. Conclusion We present the first report documenting facial and lower cranial neuropathies after super-selective embolization of head and neck paragangliomas with EVA. Although it is difficult to draw conclusions from this small number of cases, it is plausible that the use of ethylene vinyl alcohol during SSE may result in a higher risk of permanent cranial neuropathy than the use of other well-established and more temporary agents. Knowledge of the arterial supply to the cranial nerves can help the clinician to choose the embolization agent that will provide maximal occlusion while minimizing the risk of complications.

Outcomes of Cartilage Tympanoplasty in the Pediatric Population

Objective To justify the successful use of a patient selection algorithm based on age for primary cartilage tympanoplasty. Study Design Case series with chart review. Setting Tertiary care pediatric hospital. Subjects and Methods We performed a retrospective chart review of patients between ages 4 and 13 years who underwent cartilage tympanoplasty for tympanic membrane perforations from August 2005 to November 2011. Demographics, complication data, and auditory outcomes were collected. Results Patients were subdivided into 3 age groups. Group 1 consisted of patients younger than 7 years (n = 43); group 2, ages 7 to 10 years (n = 40); and group 3, ages 10 to 13 years (n = 36). Mean follow-up was 595 days (range, 48-1742). Complication rates respective to the 3 groups were as follows: remnant perforation (6.97%, 5.00%, 2.78%), revision tympanoplasty (2.33%, 2.50%, 0%), and need for tympanostomy tubes (4.65%, 2.50%, 0%). Logistic regression models were used to evaluate complication rates between groups. No significant differences were found (remnant perforation, P = .710; repeat tympanoplasty, P = .998; tympanostomy tubes, P = .875). No significance was found among audiological outcomes between the 3 groups. Conclusion These data suggest cartilage tympanoplasty can be performed effectively in young children when appropriate conditions exist.

Cochlear implant failure, revision, and reimplantation.

Objective To evaluate the long-term adverse cochlear implant (CI) outcomes resulting in revision surgery including CI reimplantation (CIR). Patients Pediatric and adult patients requiring revision procedures after CI placement. Intervention(s) Revision surgery on cochlear implant patients. Main Outcome Measures Device type, length of total device follow-up, time to device failure, cause for failure, peak pre-CIR and post-CIR audiometric performance, rate of surgical site complications, and operative findings. Results A total of 317 patients, receiving 439 CIs between January 2000 and April 2012, met inclusion criteria for this series. For the patients implanted at our institution, the revision surgery rate was 4.1%, with a CIR rate of 3.0%. The CIR rates among the pediatric and adult populations were 5.0% and 1.3%, respectively (p = 0.0336). The rate of revision procedures because of failed fixation or device extrusion was 0.9%. Device failure was experienced in 8 patients in our series, with 75% occurring with the CI24R (CS) device. Conclusion All reimplanted patients with available data had good audiometric outcomes, with the exception of those reimplanted for soft failure who had poor immediate auditory function. Using the manufacturers’ recommended surgical technique, including drilling a bony recess with suture fixation, very low surgical revision rates were achieved. Pediatric patients experienced significantly higher complications requiring CIR. All hard failures in this series occurred in the pediatric group and in a single device. Continued follow-up will be needed to determine if additional devices will succumb to this mode of failure.

Risk analysis of unilateral severe-to-profound sensorineural hearing loss in children

OBJECTIVE To explore the etiology of pediatric unilateral severe-to-profound sensorineural hearing loss (USPSNHL) and the risk of hearing loss in the contralateral ear. METHODS Pediatric patients with USPSNHL referred to a tertiary childrens hospital for unilateral bone-anchored hearing aid implantation were identified for study. Clinical charts, audiograms, and radiographic imaging of the temporal bones were reviewed. RESULTS Eighty-four children with USPSNHL were reviewed (40 male, 44 female). The etiology of USPSNHL could be identified in 35 patients (41.7%), with perinatal events (16.2%) and family history of hearing loss (10.0%) being the most common identifiable risk factors. Forty percent of children with available newborn hearing screens passed in both ears but later developed USPSNHL. Progressive unilateral hearing loss leading to USPSNHL was found in 21% percent of patients. No patient experienced worsening hearing in the contralateral ear (mean follow-up=31.9 months). Of the 49 temporal bone scans available for review, 40.8% demonstrated aberrant inner ear anatomy, including semicircular canal dysplasia (10.2%), cochlear aperture stenosis (10.2%), hypoplastic cochlea (8.1%), enlarged vestibular aqueduct (14.3%), incomplete partition I/II (6.1%), and anomalous internal auditory canal (2.0%). CONCLUSION Pediatric USPSNHL can be attributed to a variety of sources with a cause identifiable in approximately 40% of patients. Temporal bone CT scan acquisition appears to be relatively high yield in this patient population, but only rarely do results have clinical implications. Early follow-up suggests that this population of children with USPSNHL does not appear to have a significantly increased risk for hearing loss in the contralateral ear.

Hearing aid tolerance after revision and obliteration of canal wall down mastoidectomy cavities.

Objective To review the tolerance of hearing aid use after revision and obliteration of a previously unstable canal wall down mastoidectomy cavity. Study Design Retrospective case series. Setting Academic tertiary referral center. Patients Adults and children who underwent the described surgical procedure followed by attempted hearing aid use. Intervention(s) Surgical revision and obliteration of a chronically unstable canal wall down mastoidectomy cavity and subsequent attempted use of a conventional, ear-level hearing aid. Main Outcome Measure(s) Stability of mastoid cavity after starting conventional hearing aid usage. Results From a review of 87 consecutive mastoid obliteration procedures performed on previously unstable open cavities, 20 ears in 19 subjects were identified for study inclusion. The indication for hearing aid use was mixed hearing loss in the majority of subjects (85%). Among included ears, 7 (35%) had at least one documented temporary period of hearing aid nonuse because of otorrhea; however, permanent discontinuation of hearing aid use in favor of bone-anchored hearing implant placement only occurred in 3 ears (15%). The mean follow-up from the start of hearing aid use was 49 months. Conclusion Although failures do exist, attempted use of a hearing aid after revision of an unstable canal wall down mastoidectomy cavity seems feasible and generally well tolerated. However, the exact likelihood of achieving this result is yet uncertain, and hearing performance was not assessed in this study.

Method and reproducibility of a standardized ossiculoplasty technique.

Objective To describe a standardized ossiculoplasty technique and to evaluate its reproducibility. Study Design Retrospective review. Setting Academic tertiary referral center. Patients The first 35 consecutive patients (adults and children) that underwent ossiculoplasty between 2009 and 2010 using a standardized technique that was newly adopted by a surgeon that had previous experience only using other ossiculoplasty techniques. Intervention Standardized technique consisted of the following: 1) reconstruction from the stapes to the malleus neck, 2) usage of standard length prostheses (2-mm partial ossicular replacement prosthesis and 4-mm total ossicular replacement prosthesis) in nearly all cases, 3) bending the prosthesis head 15 to 30 degrees according to the contour of the malleus, 4) freestanding reconstruction without packing, 5) usage of a large cartilage graft over the prosthesis head, 6) usage of a footplate stabilization prosthesis for total ossicular replacement prosthesis cases, and 7) tympanoplasty at the same stage as ossiculoplasty. The predicted postoperative air-bone (A–B) gap was calculated according to middle ear risk factors based on a scoring system developed using this technique, which was compared with the actual average postoperative A–B gap. Main Outcome Measure Predicted versus actual postoperative A–B gap. Results Thirty cases had audiometric data available for review. The average postoperative A–B gap was 15.3 dB (range, 1–31 dB), which was not different (p = 0.9060) than the predicted value. This also was statistically better (p < 0.0001) than the preoperative A–B gap of 27.8 dB. Conclusion Short-term data suggest that this standardized technique of ossiculoplasty leads to predictable hearing outcomes and seems to be reproducible by an experienced otologist with only limited specific instruction.

Rhabdomyosarcoma and Other Pediatric Temporal Bone Malignancies

This article outlines the nature of temporal bone malignancy in children, particularly from the viewpoint of a surgeon. This article includes a synopsis of the presentation, workup, and management options for children affected by these uncommon tumors. Particular attention is given to rhabdomyosarcoma, including an update of modern staging, risk classification, and prognosis; however, a concise review of other forms of pediatric temporal bone cancer and an overview of surgical approaches available for treatment is undertaken as well.

Immunotargeted photodynamic therapy for cholesteatoma: in vitro results with anti-EGFR-coated indocyanine green nanocapsules.

Hypothesis The objective was to test the hypothesis that immunotargeted photodynamic therapy (IT-PDT) using anti–epithelial growth factor receptor (EGFR)–coated indocyanine green (ICG) nanocapsules would selectively kill cholesteatoma-derived keratinocytes while sparing middle ear–derived mucosa cells in vitro. Background Rates of residual cholesteatoma caused by incomplete microsurgical removal are unacceptably high; thus, development of an adjuvant therapy to safely destroy undetected residual cholesteatoma cells would be desirable. IT-PDT is a possible means to achieve this end. Methods ICG nanocapsules coated with anti-EGFR were synthesized and applied to cholesteatoma-derived keratinocytes and middle ear mucosa cells in vitro. Selective binding to keratinocytes was evaluated by fluorescence microscopy. Activation of ICG was undertaken by applying near-infrared light (810 nm) at an applied energy dose of 1,080 J/cm2. Cell death was evaluated 2 hours after treatment with trypan blue staining. Results Selective and robust nanocapsule binding to keratinocytes, but not mucosa cells, was confirmed by preapplication and postapplication fluorescence measurements. A keratinocyte cell death rate of 70.12% ± 2.50% was achieved, whereas negligible mucosa cell death was observed. Negligible cell death was also observed for both cell types with application of the nanocapsules alone or with application of near-infrared light alone. Conclusion Anti-EGFR ICG nanocapsules applied topically and activated as part of an IT-PDT scheme results in a high rate of cholesteatoma-derived keratinocyte cell death while negligibly affecting middle ear mucosal cells in vitro. These preliminary findings suggest that this is a feasible concept and that further investigation is warranted.

Age-Related Change in Vestibular Ganglion Cell Populations in Individuals With Presbycusis and Normal Hearing

HYPOTHESIS We sought to establish that the decline of vestibular ganglion cell counts uniquely correlates with spiral ganglion cell counts, cochlear hair cell counts, and hearing phenotype in individuals with presbycusis. BACKGROUND The relationship between aging in the vestibular system and aging in the cochlea is a topic of ongoing investigation. Histopathologic age-related changes the vestibular system may mirror what is seen in the cochlea, but correlations with hearing phenotype and the impact of presbycusis are not well understood. METHODS Vestibular ganglion cells, spiral ganglion cells, and cochlear hair cells were counted in specimens from individuals with presbycusis and normal hearing. These were taken from within a large collection of processed human temporal bones. Correlations between histopathology and hearing phenotype were investigated. RESULTS Vestibular ganglion cell counts were positively correlated with spiral ganglion cell counts and cochlear hair cell counts and were negatively correlated with hearing phenotype. There was no statistical evidence on linear regression to suggest that the relationship between age and cell populations differed significantly according to whether presbycusis was present or not. Superior vestibular ganglion cells were more negatively correlated with age than inferior ganglion cells. No difference in vestibular ganglion cells was noted based on sex. CONCLUSION Vestibular ganglion cell counts progressively deteriorate with age, and this loss correlates closely with changes in the cochlea, as well as hearing phenotype. However, these correlations do not appear to be unique in individuals with presbycusis as compared with those with normal hearing.