Faculty Bibliography

Here are the facts about diagnosis and treatment and how Autoimmune Inner Ear Disease relates to progressive sensorineural hearing loss. If you think you have the disease, you are invited to contact one of the clinical research centers listed at the end of the article. <14>

When viable proximal facial nerve is inacessible, facial nerve paralysis has been classically managed with the hypoglossal facial anastomosis (HFA) for at least the past 70 years. While this procedure has proven its reliability, its problems with hemilingual atrophy (speech deglutition, drooling, mastication), hypertonia, synkinesis, and mimetic deficits indicate the need for a more perfect solution for facial paralysis. The jump interpositional graft hypoglossal facial anastomosis (JIGHFA) along with gold weight lid implantation and electromyographic (EMG) rehabilitation achieves substantial facial reanimation without hemilingual deficits. We present our results in 18 patients who underwent JIGHFA along with gold weight lid implantation and EMG rehabilitation for facial paralysis. These results were compared with those from published series of 30 patients treated with HFA with EMG rehabilitation evaluated with objective (House-Brackmann) criteria. Anonymous retrospective information from questionnaires from 22 of 48 patients who were treated with the classic HFA was also presented. In properly selected patients, the JIGHFA technique is capable of achieving substantial facial reinnervation (House-Brackmann grade III or better) in 83.3% of the patients without hemilingual sequelae which was seen in 45% of the HFA patients. In contrast to the HFA, this procedure can be used by patients with concomitant lower cranial nerve paralysis (except hypoglossal), and bilateral facial paralysis. Hypertonia, synkinesis, and lagophthalmus were less symptomatic in the JIGHFA patients. Mimetic expression was not improved in the JIGHFA population compared with the HFA group

Increased incidence of failed primary stapedectomy may be associated with the reduced stapedectomy training experiences in contemporary residency programs. It is well recognized that revision stapedectomy results are not as successful as those of the primary surgery. Nevertheless, in experienced hands, properly selected cases for revision can have highly successful outcomes in closing air-bone gaps. Fibrous adhesions and overhanging facial nerves are most refractory to revision surgery.

OBJECTIVE/HYPOTHESIS: Identify causes of primary and revision stapedectomy failure in 308 patients, assess whether these are different based on source of initial surgery, and evaluate hearing results in revision stapedectomy to improve outcome. Study Design: Retrospective, nonrandomized chart review of patients undergoing revision stapedectomy in a referral otology practice in a large metropolitan region. MATERIALS AND METHODS: Intraoperative findings, preoperative and postoperative revision stapedectomy air and bone conduction pure-tone averages, speech discrimination scores, postoperative air-bone gaps, complications, and repeated revisions were noted in 308 patients. RESULTS: Leading causes of primary stapedectomy failure included dislocated prosthesis (24.4%), inadequate prosthesis length (14%), long process resorption (14%), and fibrous adhesions (13.6%). Revision stapedectomy air-bone gaps were less than 10 dB in 80% and greater than 30 dB in 6.8% of cases. Increased sensorineural hearing loss occurred in 0.8% of revision stapedectomy cases. Five of seven cases of vertigo associated with primary stapedectomy resolved after revision surgery. CONCLUSION: Revision stapedectomy by experienced surgeons is highly effective in attaining successful air-bone gap closure in 80% and improved closure in 84.8% of operative cases. Risk of vertigo and/or sensorineural hearing loss was not any higher in this patient population when compared with reports of primary stapedectomy

OBJECTIVE: Intraoperative facial nerve monitoring has reduced the incidence of facial nerve paralysis associated with acoustic neuroma surgery, but poor facial nerve outcomes continue to occur. Intraoperative prediction of facial nerve outcome would be advantageous in patient management and counseling. This study seeks to evaluate intraoperative facial nerve stimulus thresholds as a tool for predicting postoperative facial nerve outcome. STUDY DESIGN: This study is a prospective clinical study of the prognostic value of intraoperative stimulus thresholds. SETTING: The study was performed at a tertiary referral center. PATIENTS: There were 109 patients undergoing excision of acoustic neuromas included in this study. INTERVENTIONS: The minimum current required to stimulate the facial nerve at the brain stem was prospectively recorded after excision of the acoustic neuroma. MAIN OUTCOME MEASURES: Facial nerve outcome was evaluated by the House-Brackmann grade. RESULTS: A statistically significant relationship was found between poor initial facial nerve outcome and higher stimulus thresholds. Long-term impaired facial function was also more common in the higher stimulus group compared to that of the lower stimulus groups. CONCLUSIONS: Although these findings suggest that intraoperative stimulus thresholds have prognostic potential, other prognostic factors should also be considered and additional research is needed

Spontaneous temporal bone cerebrospinal fluid leak may be defined as a leak without an apparent precipitating cause. These transdural fistulas occur rarely, and diagnosis is predicated upon a high index of suspicion. Leaks have been reported through both middle and posterior fossa defects, although the vast majority involve the middle fossa plate. In a previous study we reported 7 cases of spontaneous temporal bone cerebrospinal fluid leaks, all involving the middle fossa tegmen. Upon further review of these cases and 5 previously unreported cases, the defect was localized to the tegmen tympani in 9 of the total 12 cases. Diagnostic methods are discussed, with the importance of high-resolution computed tomography stressed. The role of contrast cisternography is also evaluated. An outline for surgical management is presented based upon residual hearing and defect location and accessibility. A transmastoid procedure offers the advantage of visualization of both the middle and posterior fossa plates, and this approach can be supplemented with an obliterative procedure when indicated. The middle fossa approach provides optimal exposure of the tegmen plate with less likelihood of ossicular injury when dealing with tegmen tympani defects. Adjuncts to surgical therapy include intrathecal fluorescein dye and continuous postoperative lumbar cerebrospinal fluid drainage

Massive skull base injuries require detailed preoperative neurological and neurovascular assessment prior to undertaking surgical repair of isolated cranial nerve deficits. We present the management of a patient with traumatic facial paralysis, cerebrospinal fluid leak, and carotid artery cavernous sinus fistula as the result of a gunshot wound to the skull base. The carotid artery cavernous sinus fistula was ultimately controlled with super-selective embolization via the vertebral artery. The facial nerve injury was then safely treated with mobilization of the labyrinthine and vertical segments to allow a primary anastomosis

Cerebrospinal fluid rhinorrhea is the result of transdural communication between the subarachnoid space and the skull base. A transdural fistula may originate from the anterior, middle, or posterior cranial compartments. All skull-base sites of leakage potentially lead to the nasal cavity. Recurrent meningitis is commonly associated with such a direct source of bacterial contamination. Organisms associated with recurrent meningitis secondary to cerebrospinal fluid leaks are commonly found in the upper respiratory tract. We report a case of recurrent meningitis in a 5-year-old girl that highlights the problem of cerebrospinal fluid rhinorrhea, and we discuss etiology, current diagnostic techniques, and surgical management