Vibrant Soundbridge implantable hearing device: critical review and single-surgeon short- and long-term results.

We conducted a retrospective descriptive study of a series of 31 consecutively presenting patients who had been implanted with the Vibrant Soundbridge middle ear hearing device. All implantations had been performed by the senior author. Three of these patients had undergone bilateral implantation, and 4 others had undergone subsequent explantation and reimplantation in response to known or suspected device failure, giving us a total of 34 ears and 38 implants. Our goal was to ascertain short- and long-term outcomes as measured by conventional audiometry (pure-tone average at 1 to 6 kHz) and long-term benefit as defined by the use or nonuse of the device. We found that at the initial activation session 2 months postoperatively, the average hearing thresholds were within 3 dB of the preoperative thresholds in all 34 ears and all 38 implants. The mean short-term gain at activation in the 38 implants was 28.1 dB. Nineteen patients (20 ears) were available for long-term evaluation, with the length of follow-up ranging from less than 1 year to 11 years (mean: 7.3). Of these 20 ears, 9 demonstrated further gain (mean: 10.8 dB) despite any natural hearing deterioration; of the remaining 11 ears, gain was unchanged in 2, diminished in 7 (mean: -3.6 dB), and gain data were unavailable in 2. In the final analysis, there were 20 user ears and 10 nonuser ears; 4 ears were lost to all follow-up. We conclude that direct-drive hearing with the Vibrant Soundbridge middle ear hearing device is beneficial and provides sustained audiometric gain. Factors that have a significant impact on patient use or nonuse include difficulty in obtaining audiologic support and the direct and indirect costs of the device. Without audiologic or financial support, some patients may choose to become nonusers and to either switch to conventional hearing aid amplification or become apathetic about hearing improvement.

Revision cochlear implant surgery in children.

OBJECTIVE: To determine the incidence of revision cochlear implant (CI) surgery in children and the indications for revision surgery and to examine the pattern of events that lead to revision CI surgery. STUDY DESIGN: Retrospective case review. SETTING: Two tertiary pediatric CI centers. PATIENTS: Pediatric CI patients who underwent revision surgery related to their CI. MAIN OUTCOME MEASURES: Reasons for revision, surgical outcomes, complications, performance, and device analyses were sought. RESULTS: Nine hundred fifty-two pediatric CI operations were performed between 1991 and 2005. Ninety-three patients underwent 107 (11.2%) revision operations. Hard device failure occurred in 46% (n = 49); soft failure occurred in 15% (n = 16); medical/surgical causes were responsible for 37% (n = 40); and magnet dislodgement requiring revision surgery occurred in 2% (n = 2). Head trauma was associated with 41% of the hard failure cases (n = 20). Device analyses revealed identifiable abnormalities in most of both hard and soft failure cases. In most patients, auditory performance equaled or surpassed the best preoperative performance by 6 to 12 months after revision. CONCLUSION: Revision CI surgery is common among pediatric CI recipients. Hard failure is the most common reason for undertaking revision surgery, and this mode of failure is frequently associated with preceding head trauma. Patients and parents should be counseled that performance is expected to equal or surpass the child's best level of performance before revision surgery, although this may take some time, and exceptions do exist.

Management of cerebrospinal fluid leaks after acoustic tumor removal.

OBJECTIVE: To present a logical algorithm for management of postoperative cerebrospinal fluid (CSF) leak that occurs after acoustic tumor removal, and to describe a method for eustachian tube resection. CLINICAL PRESENTATION: We present an algorithm in the form of a flow chart, describe middle fossa craniotomy for eustachian tube resection, and present three cases in which this technique was used. TECHNIQUE: For CSF leak, pressure dressing at the wound and bed rest for the patient are advised; lumbar drain is indicated if the leak does not resolve. Occasionally, wound exploration is required. CSF rhinorrhea is treated first with a lumbar drain. If this approach is unsuccessful, the choice of treatment depends on the patient's hearing status. If a patient has no hearing, we perform a blind sac closure of the ear canal and pack the eustachian tube. If a patient has hearing, wound reexploration and lumbar drainage are advised. When conservative and initial surgical procedures for resolving CSF leak fail, we perform a middle fossa craniotomy to identify, divide, and remove a segmental portion of the cartilaginous eustachian tube and then cauterize and occlude both ends. CONCLUSION: At centers where surgical removal of acoustic tumors is frequently performed, a logical protocol should be in place for treatment of postoperative CSF leak. The leak location and the patient's hearing status are factors in determining the appropriate treatment method. Middle fossa craniotomy for resection of the eustachian tube is a safe, definitive management option for treatment of recalcitrant CSF rhinorrhea that occurs after acoustic tumor surgery.

Evaluation of facial function with a questionnaire: reliability and validity.

OBJECTIVES: To analyze the validity and reliability of assigning House-Brackmann facial nerve grade based on a patient-administered questionnaire. Specifically, the use of a questionnaire was studied for evaluation of the long-term facial function after microsurgical resection of vestibular schwannoma (VS). STUDY DESIGN: Three neurotologists with more than 25 years of experience and 3 neurotology clinical fellows rated facial nerve grade in a blinded fashion based on the questionnaire. The standard to which the questionnaires were compared was the facial nerve grade assigned to the patient during an office visit near the same time as the completed patient questionnaire. METHODS: Thirty-four patients had an office evaluation of facial function at least 1 year after microsurgical resection of a VS. Thirty patients had a sporadic VS, whereas 4 patients had neurofibromatosis 2. The average tumor size was 2.2 cm. A postoperative questionnaire regarding facial function was completed within 2 months of the office evaluation. Statistical analyses were performed to compare the office evaluation rating with the questionnaire rating (validity) and to analyze interobserver reliability. RESULTS: The association between the facial nerve grade assigned by the office evaluation and the questionnaire was highly significant (p<0.01), with a mean correlation (Spearman [rho]) equal to 0.94. The interobserver reliability was high, with coefficients ranging from 0.93 to 0.99 (p<0.01; mean=0.97). Because a large proportion of patients had normal (House-Brackmann Grade I) facial function, an additional analysis was performed with these patients excluded. The high levels of interobserver reliability persisted (average=0.96). CONCLUSION: When compared with office evaluation, a patient-administered questionnaire provides a valid and reliable measure of long-term facial function after VS surgery.

Facial nerve function after translabyrinthine vestibular schwannoma surgery.

OBJECTIVES: To evaluate the long-term facial function of patients after translabyrinthine vestibular schwannoma [VS] surgery and identify factors that influence these outcomes. STUDY DESIGN AND SETTING: A retrospective review was performed that included 580 consecutive patients who underwent translabyrinthine craniotomy for removal of VS at a tertiary referral neurotologic practice between February 2000 and July 2004. A total of 512 patients who underwent primary microsurgical treatment of sporadic unilateral VS met inclusion criteria. Patient and tumor characteristics as well as perioperative complications are described. Perioperative and long-term facial function were evaluated in 392 patients who had at least 1-year follow-up. RESULTS: Complication rates after translabyrinthine craniotomy for VS are low. Patients with smaller tumors have significantly better postoperative facial function than those with larger tumors. CONCLUSIONS: Excellent long-term facial function can be expected in the majority of patients who undergo microsurgical removal of VS via the translabyrinthine approach. Alternative treatment strategies may need to be developed for the treatment of VS > 3.5 cm in order to maximize postoperative facial function.

Cochlear implants in Waardenburg syndrome.

OBJECTIVE: Waardenburg syndrome is an autosomal-dominant syndrome characterized by dystopia canthorum, hyperplasia of the eyebrows, heterochromia irides, a white forelock, and sensorineural hearing loss in 20% to 55% of patients. This patient population accounts for approximately 2% of congenitally deaf children. The purpose of this retrospective case review was to describe the outcomes for those children with Waardenburg syndrome who have undergone cochlear implantation. METHODS: Pediatric cochlear implant recipients with documented evidence of Waardenburg syndrome underwent retrospective case review. All patients received their cochlear implants at the study institution followed by outpatient auditory habilitation. Charts were reviewed for etiology and duration of deafness, age at time of cochlear implantation, perioperative complications, duration of use, and performance outcomes. Results of standard tests batteries for speech perception and production administered as a part of the patients' auditory habilitation were reviewed. RESULTS: Seven patients with Waardenburg syndrome and cochlear implants were identified. The average age at implantation was 37 months (range, 18-64 months) and the average duration of use was 69 months (range, 12-143 months). All of these patients are active users of their devices and perform very well after implantation. There were no major complications in this small group of patients. CONCLUSIONS: Children with congenital sensorineural hearing loss without other comorbidities (e.g., developmental delay, inner ear malformations) perform well when they receive cochlear implantation and auditory habilitation. Patients with Waardenburg syndrome can be expected to have above-average performance after cochlear implantation.

Cochlear implantation in patients with substantial residual hearing.

OBJECTIVES: Cochlear implantation is an effective means for providing auditory rehabilitation in adult patients with severe to profound sensorineural hearing loss. It has been hypothesized that patients with substantial, preoperative residual hearing would be excellent cochlear implant candidates because of surviving neural populations and a lack of auditory deprivation. The purpose of this study is to describe the outcomes of patients with substantial residual hearing who have undergone cochlear implantation. STUDY DESIGN: Retrospective chart review of patients with substantial preoperative residual hearing who underwent cochlear implantation. METHODS: Chart reviews were completed for patients with substantial residual hearing who underwent cochlear implantation (City University of New York Sentence Test [CUNY] > 60%, Hearing in Noise Test sentences presented in quiet [HINTQ] > 50%, or Consonant-Nucleus-Consonant [CNC] > 20% in the ear to be implanted). Preoperative and postoperative measures of audiologic performance as well as complications were assessed. RESULTS: All 12 patients who met inclusion criteria ultimately surpassed their preoperative aided performance level after implantation and gained significant benefit from their cochlear implant. At 6 months postimplantation, mean CUNY, HINTQ, and CNC scores were 93%, 78%, and 48% in the implant ear alone, respectively. However, progress was slower than expected for many patients, and at least one patient took 1 year to surpass his preoperative performance level. There were no complications from surgery in this selected group of patients. CONCLUSIONS: Patients with some degree of residual hearing do benefit from cochlear implantation. However, there may be an initial decline in performance as compared with preoperative levels. This decline is overcome in time in this patient population. These patients need to be counseled accordingly.

Cochlear implantation for children with GJB2-related deafness.

OBJECTIVES/HYPOTHESIS: Mutations in GJB2 are a common cause of congenital sensorineural hearing loss. Many children with these mutations receive cochlear implants for auditory habilitation. The purpose of the study was to compare the speech perception performance of cochlear implant patients with GJB2-related deafness to patients without GJB2-related deafness. STUDY DESIGN: Retrospective case review. METHODS: Pediatric cochlear implant recipients who have been tested for GJB2 mutation underwent chart review. All patients received cochlear implantation at a tertiary referral center, followed by outpatient auditory habilitation. Charts were reviewed for cause and duration of deafness, age at time of cochlear implantation, intraoperative and postoperative complications, duration of use, and current age. Results of standard tests of speech perception administered as a part of the patients' auditory habilitation were reviewed. RESULTS: Twenty patients with GJB2 mutations were compared with 27 patients without GJB2 mutations. There was no statistical difference between patients with and without GJB2-related congenital sensorineural hearing loss with regard to open-set and closed-set speech recognition performance at 12, 24, and 36 months after cochlear implantation. Surgical complications were uncommon. CONCLUSION: Pediatric patients with congenital sensorineural hearing loss without other comorbid conditions (eg, developmental delay, inner ear malformations) perform well when they receive cochlear implantation and auditory habilitation. The presence or absence of GJB2 mutation does not appear to impact speech recognition performance at 12, 24, and 36 months after implantation.

Endoscopic ablation of distal tracheal lesions using Nd:YAG contact laser.

Surgical lasers are an excellent tool for the treatment of airway lesions. Distal tracheal lesions offer a greater challenge in that they are less accessible to laser treatment relative to upper airway lesions yet retain the propensity to cause complete airway obstruction. The ideal treatment modality for the endoscopic ablation of distal tracheal lesions provides secure airway protection, excellent visualization of the lesion in question, and delivery of a safe and effective method of treatment. We have found that the use of a contact Nd:YAG fiberoptic delivery system through a rigid bronchoscope has performed very well in meeting these criteria.