Joe Walter Kutz

Management of spontaneous cerebrospinal fluid otorrhea.

Objectives/Hypothesis: To describe the surgical approaches and materials used to repair spontaneous cerebrospinal fluid (CSF) otorrhea of temporal bone origin.

Cochlear Implantation in Children With Cochlear Nerve Absence or Deficiency

Objective: To evaluate speech perception after cochlear implantation in children with cochlear nerve absence or deficiency. Methods: A retrospective case review was performed to identify children who underwent cochlear implantation with cochlear nerve absence or deficiency. The cochlear nerve was evaluated by high-resolution three-dimensional T2-weighted fast spin echo MR in the oblique sagittal and axial planes. A deficient cochlear nerve was defined as a cochlear nerve that is smaller in diameter when compared with the adjacent facial nerve in the midportion of the internal auditory canal. The cochlear nerve was considered absent if there was no imaging evidence of a cochlear nerve. Speech awareness threshold and the speech perception category score were used to measure speech perception after cochlear implantation. Results: Seven children who underwent cochlear implantation in an ear without imaging evidence of a cochlear nerve were identified. One child developed early closed-set speech recognition. The other 6 children developed only speech detection or pattern perception. Two children underwent cochlear implantation with a deficient cochlear nerve. One developed consistent closed-set word recognition and the other developed early closed-set word recognition. The mean follow-up time for all patients was 3.8 years (range, 1.1-7.1 yr). Conclusion: Cochlear nerve deficiency is not an uncommon cause for profound sensorineural hearing loss and presents a challenge in the decision-making process regarding whether to proceed with a cochlear implant. Children with a deficient but visible cochlear nerve on magnetic resonance image can expect to show some speech understanding after cochlear implantation; however, these children do not develop speech understanding to the level of implanted children with normal cochlear nerves. Children with an absent cochlear nerve determined by magnetic resonance imaging can be expected to have limited postimplantation sound and speech awareness.

Labyrinthitis ossificans: How accurate is MRI in predicting cochlear obstruction?

Objective: To determine the accuracy of preoperative MRI in predicting cochlear obstruction in pediatric patients with a history of bacterial meningitis. Methods: A case series with chart review was performed at a tertiary care multidisciplinary cochlear implant program. Forty-five children with hearing loss that resulted from bacterial meningitis were implanted from 1991 to 2006. Twenty-five children had preoperative MRI with high-resolution axial T2-weighted images to assess for cochlear patency. Results: Seventeen of 25 patients (68%) had surgical evidence of cochlear obstruction. Six patients (37.5%) required circummodiolar drill-outs, and one patient (6.25%) underwent placement of a double array cochlear implant. The nine remaining patients (56%) with cochlear obstruction required removal of fibrous tissue or drilling of the inferior basal turn, but did not require manipulation of the ascending basal turn to achieve full electrode insertion. The sensitivity, specificity, and positive and negative predictive value of MRI predicting intraoperative cochlear obstruction with 95 percent confidence intervals was 94.1 percent (71–99), 87.5 percent (47–99), 94.1 percent (71–99) and 87.5 percent (47–99), respectively. Conclusion: Preoperative high-resolution T2 MRI may be useful in predicting cochlear obstruction in patients with a prior history of bacterial meningitis.

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.

Bone-Anchored Hearing Aid Subjective Benefit for Unilateral Deafness

Determine the benefit of the bone‐anchored hearing aid (BAHA) in patients with unilateral deafness.

Hearing Preservation Using the Middle Fossa Approach for the Treatment of Vestibular Schwannoma

BACKGROUND: The incidence of small vestibular schwannomas in patients with serviceable hearing is increasing because of the widespread use of MRI. The middle fossa approach provides the patient with an opportunity for tumor removal with hearing preservation. OBJECTIVE: To determine the rate of hearing preservation and facial nerve outcomes after removal of a vestibular schwannoma with the use of the middle fossa approach. METHODS: A retrospective case review at a tertiary, academic medical center was performed identifying patients from 1998 through 2008 that underwent removal of a vestibular schwannoma by the middle fossa approach. Preoperative and postoperative audiograms were compared to determine hearing preservation rates. In addition, facial nerve outcomes at last follow-up were recorded. RESULTS: Forty-six patients underwent a middle fossa craniotomy for the removal of a vestibular schwannoma. Of the 38 patients that had class A or class B hearing preoperatively, 24 (63.2%) retained class A or B hearing and 29 (76.3%) retained class A, B, or C hearing. When tumors were 10 mm or less in patients with class A or B preoperative hearing, 22 of 30 patients (73.3%) retained class A or B hearing. When the tumor size was greater than 10 mm in patients with class A or B preoperative hearing, 2 of 8 patients (25%) retained class A or B hearing. At most recent follow-up, 76.1% of patients had House-Brackmann grade I facial function, 13.0% had House-Brackmann grade II facial function, and 10.9% had House-Brackmann grade III facial function. CONCLUSION: Hearing preservation rates are excellent using the middle fossa approach, especially for smaller tumors. No patient experienced long-term facial nerve function worse than House-Brackmann grade III.

Lumbar subarachnoid drainage in cerebrospinal fluid leaks after lateral skull base surgery.

Objective To determine the efficacy of lumbar drainage in managing cerebrospinal fluid (CSF) leak after lateral skull base surgery. Study Design Retrospective case review. Setting Academic tertiary referral center. Patients Patients who had a lumbar subarachnoid drain placed after a lateral skull base procedure between July 1999 and February 2010 were included. Interventions Patients were identified by searching medical records for lateral skull base approach Current Procedural Terminology codes. The following variables were recorded for each subject: diagnosis, type of lateral skull base operation, duration of lumbar drainage, need for revision surgery, and presence of meningitis. Main Outcome Measure Successful cessation of postoperative CSF leakage. Results Five hundred eight charts were reviewed, and 63 patients were identified who received a lumbar drain after a lateral skull base operation. The most common diagnosis was acoustic neuroma in 61.9%. The most common skull base approaches were the translabyrinthine, middle fossa, and transpetrosal approaches. Approximately 60.3% of patients had CSF rhinorrhea, 23.8% had an incisional leak, and 14.3% had otorrhea. The mean duration of lumbar drainage was 4.6 days. Forty eight (76.2%) study subjects had resolution of their CSF leak with lumbar drainage. Fifteen patients (23.8%) required revision surgery to stop the CSF leak. Lumbar drainage was successful in 90% of leaks after the translabyrinthine approach but in only 50% of those undergoing a suboccipital approach, which was a statistically significant difference. Conclusion Postoperative CSF leaks after lateral skull base surgery can be managed with a lumbar subarachnoid drain in a majority of cases but is more successful after the translabyrinthine than the suboccipital approach. Recurrent CSF leaks after lumbar drainage is likely to require a revision operation.

The dizzy patient.

The dizzy patient often presents a challenge to the physician. The history is the most important component of the evaluation of the dizzy patient and often allows the cause of the dizziness to be categorized as peripheral or central. Peripheral causes include benign paroxysmal positional vertigo, Menieres disease, and vestibular neuritis. Central causes include migraine-associated dizziness, postconcussion syndromes, cerebrovascular disease, and multiple sclerosis. Treatment depends on the cause of the dizziness and may include dietary modifications, diuretics, vestibular suppressants, vestibular rehabilitation, or surgical intervention.

Pontine Tegmental Cap Dysplasia The Neurotologic Perspective

Objectives. Pontine tegmental cap dysplasia (PTCD) is a rare congenital malformation. Clinical and imaging findings in 3 patients and the authors’ experience with bilateral cochlear implantation in 1 patient are described. Study Design. Retrospective review. Setting. Two tertiary medical centers. Subjects and Methods. Three patients were evaluated by an otolaryngologist and underwent magnetic resonance imaging (MRI) of the temporal bones and brain. High-resolution computed tomography (CT) scanning of the temporal bones was performed in 2 patients. Imaging findings of the brain, the presence and course of resolvable cranial nerves, the membranous labyrinth, and internal auditory canals were reviewed. Clinical data were reviewed. Results. All patients demonstrated typical brain characteristics of PTCD. Mild, bilateral cochlear dysplasia was noted in 2, and all had a normal vestibular labyrinth. The cochleovestibular nerves were universally absent bilaterally. The facial nerves were subjectively deficient bilaterally in 1 patient, unilaterally in the second patient, and normal in the third. An accessory canal for the seventh cranial nerve, referred to as a duplicated internal auditory canal, was present in all patients. Auditory brainstem response testing revealed profound bilateral sensorineural hearing loss in all of the patients; none suffered facial weakness. A single patient underwent bilateral cochlear implantation with only minimal response. Conclusion. The authors report 3 cases of PTCD with emphasis on imaging of the seventh and eighth cranial nerves and clinical neurotologic findings. All patients manifested duplicated internal auditory canals, a previously unreported finding in PTCD. Bilateral profound sensorineural hearing loss is due to absence of the cochleovestibular nerve. Prognosis for cochlear implantation is poor.

Concurrent Vestibular Schwannoma and Meningioma Mimicking a Single Cerebellopontine Angle Tumor

Vestibular schwannomas account for ~;80% of cerebellopontine angle (CPA) tumors, with meningiomas being the second most common tumor of the CPA. The occurrence of both a schwannoma and a meningioma in the cerebellopontine angle is rare. After obtaining Institutional Review Board approval, we present a case of a concurrent vestibular schwannoma and meningioma in the CPA mimicking a single tumor.