Cerebrospinal fluid leaks and encephaloceles of temporal bone origin: nuances to diagnosis and management.

OBJECTIVE: Temporal bone encephalocele has become less common as the incidence of chronic mastoid infection and surgery for this condition has decreased. As a result, the diagnosis is often delayed, and the encephalocele is often an incidental finding. This situation can result in serious neurologic complications with patients presenting with cerebrospinal fluid leak and meningitis. We review the occurrence of, characteristics of, and repair experience with temporal encephaloceles from 2000-2012. METHODS: We conducted a retrospective review of 32 patients undergoing combined mastoidectomy and middle cranial fossa craniotomy for the treatment of temporal encephalocele. RESULTS: The diagnosis of temporal encephalocele was made in all patients using high-resolution temporal bone computed tomography and magnetic resonance imaging. At the time of diagnosis, 12 patients had confirmed cerebrospinal fluid leak; other common presenting symptoms included hearing loss and ear fullness. Tegmen defect was most commonly due to chronic otitis media (n = 14). Of these patients, 8 had undergone prior mastoidectomy, suggesting an iatrogenic cause. Other etiologies included radiation exposure, congenital defects, and spontaneous defects. Additionally, 2 patients presented with meningitis; 1 patient had serious neurologic deficits resulting from venous infarction. CONCLUSIONS: The risk of severe neurologic complications after the herniation of intracranial contents through a tegmen defect necessitates prompt recognition and appropriate management. Computed tomography and magnetic resonance imaging aid in definitive diagnosis. A combined mastoid/middle fossa approach allows for sustainable repair with adequate exposure of defects and support of intracranial contents.

Parental restriction of children's access to cochlear implants: case studies.

This article reports on four retrospective case studies in which parental behavioral management of the implanted child included withholding the cochlear implant or activities associated with it as a disciplinary measure or as a means of preventing device loss or damage. The need for parental counseling by health care and educational professionals as to the importance of a child's connection to the hearing world all day long through the implant for optimal speech, language, academic, and psychosocial development is emphasized.

How to measure cerebrospinal fluid pressure invasively and noninvasively.

We describe tympanic membrane displacement (TMD) testing for non-invasive estimation of intracranial pressure (ICP). With the TMD test, displacement of the tympanic membrane of the middle ear is recorded during elicitation of the acoustic middle-ear reflex (AR). Increased intracranial/perilymphatic pressure displaces the resting stapes footplate laterally so that TMD during the acoustic reflex is medial. Decreased intracranial/perilymphatic pressure displaces the baseline stapes footplate position medially (inward) so that TMD during the AR is lateral. The TMD typically is bidirectional when intracranial/perilymphatic pressure is normal. Discrepant findings have been reported for the sensitivity of the TMD test to ICP as the regression of TMD on invasive measurement of the ICP reveals substantial intersubject variability and overlap among patient and control groups. Large-sample research on TMD test performance in healthy persons and patients with various disorders affecting the ICP is needed using direct, invasive measures of the ICP as the gold standard. Research also is needed to examine whether non-invasive TMD testing can be used to investigate the trans-lamina cribrosa pressure difference in glaucoma.

Oral vs intratympanic corticosteroid therapy for idiopathic sudden sensorineural hearing loss: a randomized trial.

CONTEXT: Idiopathic sudden sensorineural hearing loss has been treated with oral corticosteroids for more than 30 years. Recently, many patients' symptoms have been managed with intratympanic steroid therapy. No satisfactory comparative effectiveness study to support this practice exists. OBJECTIVE: To compare the effectiveness of oral vs intratympanic steroid to treat sudden sensorineural hearing loss. DESIGN, SETTING, AND PATIENTS: Prospective, randomized, noninferiority trial involving 250 patients with unilateral sensorineural hearing loss presenting within 14 days of onset of 50 dB or higher of pure tone average (PTA) hearing threshold. The study was conducted from December 2004 through October 2009 at 16 academic community-based otology practices. Participants were followed up for 6 months. INTERVENTION: One hundred twenty-one patients received either 60 mg/d of oral prednisone for 14 days with a 5-day taper and 129 patients received 4 doses over 14 days of 40 mg/mL of methylprednisolone injected into the middle ear. MAIN OUTCOME MEASURES: Primary end point was change in hearing at 2 months after treatment. Noninferiority was defined as less than a 10-dB difference in hearing outcome between treatments. RESULTS: In the oral prednisone group, PTA improved by 30.7 dB compared with a 28.7-dB improvement in the intratympanic treatment group. Mean pure tone average at 2 months was 56.0 for the oral steroid treatment group and 57.6 dB for the intratympanic treatment group. Recovery of hearing on oral treatment at 2 months by intention-to-treat analysis was 2.0 dB greater than intratympanic treatment (95.21% upper confidence interval, 6.6 dB). Per-protocol analysis confirmed the intention-to-treat result. Thus, the hypothesis of inferiority of intratympanic methylprednisolone to oral prednisone for primary treatment of sudden sensorineural hearing loss was rejected. CONCLUSION: Among patients with idiopathic sudden sensorineural hearing loss, hearing level 2 months after treatment showed that intratympanic treatment was not inferior to oral prednisone treatment. TRIAL REGISTRATION: clinicaltrials.gov Identifier: NCT00097448.

Revision cochlear implant surgery in patients with suspected soft failures.

OBJECTIVE: To review our patient series who underwent revision cochlear implantation surgery, with special emphasis on the "soft failure" group. STUDY DESIGN: Retrospective chart review of cochlear implant revision surgeries from 1979 to 2008. An extensive review of these patients' medical, audiologic, and radiographic histories was performed. SETTING: Two tertiary care hospitals and 1 academic cochlear implant center. INTERVENTION: Explantation and reimplantation of cochlear implant, explanted device analysis, speech perception testing. MAIN OUTCOME MEASURES: Postoperative speech performance. RESULTS: Approximately 1,500 cochlear implant surgeries were performed from 1979 to 2008. Of these, 113 (7.53%) procedures in 98 patients were revision cases. The underlying reason for revision surgery was divided into 4 categories: 26 hard failures (23%), 31 medical failures (27.4%), 14 soft failures (12.4%), and 42 (37.2%) not classified/ambiguous cases. The last group was not categorized because of lack of available medical documentation or because of an ambiguous device failure analysis. The top 3 most common causes of hard failure were loss of hermiticity (8 patients [30.8%]), Vendor B defects (7 patients [26.9%]), and cracked casing (4 patients [15.4%]). The most common cause of medical failure was device extrusion (11 patients [35.5%]) followed by head trauma to the site of implantation (11 patients [35.5%]), and wound infection (5 patients [16.1%]). Fourteen patients (14.2%) were categorized as soft failures. All soft failure patients demonstrated a deterioration in pure-tone average and speech perception. Of the soft failure group, time to revision surgery was 4.7 years in contrast to 4.2 years for the hard failure group and 4.3 years for all revisions. An extensive review of patients' medical histories showed that 4 (28.5%) of the 14 patients had a previous diagnosis of meningitis. Two patients (14.3%) had evidence of inner ear malformations, and 2 patients (14.3%) had history of asthma. CONCLUSION: Our cochlear revision series are comparable to what is reported in the literature. However, an unexpected relationship between meningitis was identified among our soft failure group. More than one-quarter carried a history of meningitis. Moreover, nearly one-half of all soft failures had some form of inflammatory derangement. We used the soft failure criteria established by the 2005 Consensus Development Conference for our population analysis. Although we agree that audiologic data often are essential for defining soft failure, multiple patients in our series experienced pain that was severe enough to prevent a complete audiometric evaluation, therefore not rigorously fulfilling the criteria set forth by the 2005 Consensus. However, because their symptoms resolved after reimplantation, and their speech performance restored, we propose modifications of the current definition of "soft failure" to include these patients.

Repair issues associated with cochlear implants in children.

OBJECTIVE: To examine the number and type of repair issues associated with the use of cochlear implants in children who have worn either the body-level or ear-level style for 4 to 5 years. STUDY DESIGN: Retrospective review. SETTING: Specialty eye and ear institute. METHODS: Charts for all children who received cochlear implants from one of the authors between 1994 and 2002 and who received 4 to 5 years of follow-up for either the body or the ear-level style were examined. Based on the return merchandise authorizations and chart notes, implant problems were classified as external or internal. External problems were further subclassified as affecting the batteries, case, earhook, cords/cables, microphone, speech processor, coil, and/or external magnet; internal problems were subclassified as affecting the internal magnet, electrode(s), or other. MAIN OUTCOME MEASURES: Type and number of internal and external cochlear implant component breakdowns over time (including external component repair rates per year) and number of associated hospital visits. RESULTS: Of the 22 children, 4 (18.2%) had internal component problems. For the external components, the repair rate per year was 4.1 and 2.7 for the body style and ear-level style, respectively. For the group of children who was seen for each of 4 years, the mean number of repair problems declined 32% and 43% over that period for the body-worn and ear-level implants, respectively. The mean repair cost per year, based on Years 4 and 5 of use, was $794 and $317 for the body-worn and ear-level styles, respectively. CONCLUSION: The declines over time in repair problems and associated hospital visits suggest that children learn, over time, to better maintain and care for their cochlear implant. Although most had external component breakdowns, relatively few had internal component repair issues.

Conservative management of advanced external auditory canal cholesteatoma.

OBJECTIVE: To investigate the spectrum of disease presentation and clinical management of primary external auditory canal cholesteatoma (EACC). STUDY DESIGN: Case series with chart review. SETTING: Specialty teaching hospital. SUBJECTS AND METHODS: Ten cases of primary EACC were identified in nine patients treated over 14 years (1995-2009). Cases were reviewed with regard to demographics, presentation, physical examination, CT findings, and clinical management. RESULTS: The most common symptoms were otalgia and hearing loss, followed by otorrhea and tinnitus. Erosion was present in the mastoid air cells in seven patients, middle ear in six, temporomandibular joint in two, otic capsule in two, and fallopian canal in one patient. Eight of nine patients were managed with serial debridement. CONCLUSIONS: EACC is associated with adjacent bony erosion, most often involving the inferior EAC. Despite the potentially destructive nature of these lesions, most cases can be successfully managed with serial debridement.

Efficacy of the bone-anchored hearing aid for single-sided deafness.

OBJECTIVES/HYPOTHESIS: The aim was to examine short- and long-term efficacy of the bone-anchored hearing aid (BAHA) on adults with single-sided deafness. STUDY DESIGN: Prospective investigation. METHODS: The outcome measures included the Hearing in Noise Test (HINT), Abbreviated Profile of Hearing Aid Benefit (APHAB), and Single-Sided Deafness Questionnaire (SSD). The BAHA group comprised seven adults with single-sided deafness and the control group comprised 20 adults with essentially normal-hearing sensitivity, bilaterally. The outcome measures were administered in the unaided, directional BAHA, and omnidirectional BAHA conditions after 1 month, 6 months, and 12 months of BAHA use. A repeated measures analysis of variance (ANOVA) model was used to evaluate the data (for the SSD, a nonparametric analog of ANOVA was employed). RESULTS: None of the factors (time, HINT condition, amplification status) or their interactions were significant predictors of change in signal-to-noise ratio (SNR) from baseline over time on the HINT. The mean SNR (non-baseline-subtracted) was significantly lower in the directional BAHA versus the unaided status and in the omnidirectional BAHA versus the unaided status, but only under the noise in front, speech lateralized to the bad ear HINT condition. Significant short- and long-term BAHA benefit was observed on the APHAB (all subscales except Aversiveness) and SSD (all questionnaire items). CONCLUSIONS: Our results show short- and long-term efficacy for the BAHA in adults with single-sided deafness for recognition of speech in noise (noise in front, speech lateralized to the bad ear) and on subjective measures of benefit.

Glomus tumors in patients of advanced age: a conservative approach.

OBJECTIVES: Identify and discuss controversies in the management of paragangliomas in elderly patients. Assess and evaluate a conservative treatment strategy involving limited surgical resection and vigilant monitoring of the outcome measures of tumor control, peritreatment morbidity, symptom resolution, and hearing preservation. STUDY DESIGN: Retrospective case review. METHODS: All of the patients in this study were over age 60 with temporal bone glomus tumors. Primary outcome assessment included length of hospitalization, perioperative morbidity, symptom resolution, hearing preservation, and long-term tumor control. RESULTS: Twelve female patients with mean age of 74.5 years (range 61-85 years) with follow-up from 24 months to 33 years (mean/median: 5/7.8 years) were identified. Nine (75%) of the patients presented with pulsatile tinnitus. Seven patients (58%) underwent surgical excision of the middle ear component of the paraganglioma. Tumors extending to the jugular foramen were purposely not resected. Five patients (45%) had relative or absolute contraindications to surgical resection and were treated with observation or primary radiation therapy. Post-treatment audiometric evaluation confirmed stable or improved hearing. Pulsatile tinnitus resolved in all patients. No patient experienced cranial nerve deficits, extended hospitalization, or blood transfusions. All patients were followed closely with radiological imaging. The majority of patients demonstrated no disease or stable disease, while two patients demonstrated tumor growth 6 years after diagnosis. CONCLUSION: A prolonged natural history and the morbidity associated with surgical intervention have led to controversies in the treatment of glomus tumors in an elderly population. Our experience supports recent limited reports advocating conservative surgical excision and vigilant long-term monitoring in this population.

Perioperative management of intracranial aneurysms and otologic disease.

OBJECTIVE: Diagnosis and treatment of asymptomatic, unruptured intracranial aneurysms (UIA) are areas of longstanding controversy in the neurologic and neurosurgical literature. Treatment decisions require careful consideration of both the potential for aneurysm rupture as well as the risks associated with repair. Studies examining the natural history of UIA, the morbidity and mortality associated with subarachnoid hemorrhage (SAH), and the various treatment options have led to general guidelines for UIA management. In the literature, the simultaneous presence of otologic disease and UIA has not been introduced or addressed. It is unknown whether surgical treatment of middle ear disease has an effect on the natural history of UIA. STUDY DESIGN: Two illustrative patients with UIA discovered incidentally on radiographic evaluation of middle ear disease are presented. METHODS: Patients were managed according to diagnostic and treatment strategies developed and validated in the neurosurgical literature. RESULTS: Both patients underwent uneventful endovascular embolization of their intracranial aneurysms before middle ear surgery. The intra- and postoperative courses were uncomplicated without symptoms related to either their history of intracranial aneurysms or the prior embolization. CONCLUSIONS: Application of neurosurgical diagnostic and treatment algorithms to otology patients in the perioperative setting is appropriate. Established risk factors such as the size and location of the intracranial aneurysm, prior SAH, and patient age should guide treatment planning in all cases of UIA. Additional research is needed to evaluate the influence of otologic procedures on the natural history of UIA. In addition, studies are needed to evaluate the role of preoperative screening for UIA in otologic surgery candidates.

Malignant cylindroma of the external auditory canal.

OBJECTIVE: This report describes a case of a malignant cylindroma arising in the external auditory canal. STUDY DESIGN: Retrospective surgical case review. SETTING: Tertiary care teaching hospital. INTERVENTION: Wide local excision with margin control. RESULTS: Local control of disease at 2-year follow-up. CONCLUSION: To our knowledge, this is the first report describing a malignant degeneration of cylindroma in the external auditory canal. This tumor represents an extremely rare cutaneous neoplasm, thus raising diagnostic and therapeutic challenges.

Objective computerized versus subjective analysis of facial synkinesis.

OBJECTIVES/HYPOTHESIS: To compare the sensitivity of an objective, computerized approach to measurement of facial synkinesis with that for a subjective approach and to examine the test-retest reliability of these approaches. STUDY DESIGN: Prospective, nonrandomized, and blinded. METHODS: Remote facial motion at the upper eyelids and oral commissures during a closed-lip smile and eyelid closure were measured using a commercially available computerized motion analysis system. Thirty healthy adults with normal facial nerve function were enrolled to establish normative data and a threshold value for synkinesis. Thirty consecutive patients were analyzed based on the synkinesis threshold value. Blinded subjective evaluations by two observers were also performed independently on the same patients. RESULTS: Facial synkinesis was detected significantly more frequently with objective than subjective analysis for eyelid closure; no significant differences between approaches were shown for the closed-lip smile. Interestingly, five (17%) patients developed early synkinesis within 3 months from the onset of the facial nerve injury. The test-retest reliability of all objective measures was excellent for both expressions. Close agreement in percent of patients with synkinesis between test and retest subjective evaluations was obtained for both observers for both expressions. CONCLUSIONS: The objective, computerized approach to detection of facial synkinesis has excellent reliability and is more sensitive than the subjective approach to assessment to the presence of facial synkinesis associated with eyelid closure. The finding of early synkinesis suggests that central nervous reorganization plays a role in regeneration of the facial nerve.

Objective facial motion analysis in patients with facial nerve dysfunction.

OBJECTIVES/HYPOTHESIS: To objectively measure facial motion at various facial landmarks using a video-computer interactive system. STUDY DESIGN: Clinical, prospective, non-randomized. METHODS: A video-computer interactive system, The Peak Motus Motion Measurement System, was used to study linear displacement at preselected facial landmarks in the normal and abnormal face. Subjects with normal facial function (n = 34) and patients with abnormal facial function (n = 26) from various etiologies were studied. The sites studied were marked with reflective beads. Of a larger repertoire of expressions, two expressions (eyes closed and closed-lip smile) were studied in all subjects. The percent asymmetry in facial displacement between the sides of the face was calculated. The sensitivity of this measurement to facial dysfunction was evaluated. The presence of synkinesis was examined by quantifying the displacement at facial sites that were remote to the sites primarily involved in a given facial expression. Test-retest reliability of the percent asymmetry measurement was evaluated with the paired t test. RESULTS: The video-computer interactive approach used accurately detected and quantified gross and subtle changes in facial function. The sensitivity of the percent asymmetry measurement was 95% (both expressions) for patients with apparent facial dysfunction (House-Brackmann rating >I/VI). In patients with facial nerve dysfunction, displacement on the presumably normal side was significantly excessive in 27% to 35%, depending on the expression. With this interactive computer-video system, synkinesis was detected in 58% of the pathologic subjects during the eyes closed or closed-lip smile expressions. The paired t test indicated strong test-retest reliability (r = 0.73-0.99) of the percent asymmetry measurement. CONCLUSIONS: The present report indicates that this approach to the assessment of facial motion is sensitive to facial dysfunction. This computer-video interactive system is able to quantify synkinesis. A grading system for the magnitude of synkinesis, based on the magnitude of the displacement at remote facial sites, is proposed. The common occurrence of excessive facial motion on the presumably normal side of affected individuals indicates that patients with facial paralysis often overcompensate by exaggerating the normal side in an effort to move the affected side. This system is of value in the objective measurement of normal facial function and may prove a useful tool to quantify the outcomes of various medical and surgical treatments for facial nerve dysfunction.

Facial motion analysis with a video and computer system after treatment of acoustic neuroma.

OBJECTIVE: To objectively evaluate facial function with a computer and video system in a group of normal adults and a group of adults who have been treated for acoustic neuroma. STUDY DESIGN: A prospective descriptive study was performed in which the experimenter performing the objective facial motion analysis was blinded to the subjective rating of facial function. PATIENTS: The normal subjects comprised 18 women and 16 men. The subjects who had been treated for acoustic neuroma included 12 patients (seven women and five men) who underwent treatment for acoustic neuroma. METHODS: Light-reflective markers were placed at selected facial sites. A video and computer-assisted system was used to measure displacement that was unconfounded by head motion at these sites during two expressions. Proximal and remote displacement were measured for the x and y coordinates. Percentage of asymmetry relative to the total displacement was determined. RESULTS: Significant asymmetry in displacement for the y coordinate during the eyes-closed expression occurred in 100% of the subjects who had been treated for acoustic neuroma with apparent facial dysfunction and 0% of the subjects who had been treated for acoustic neuroma with no apparent facial dysfunction. Synkinesis was severe (>0.2 cm) in 17%, moderate (0.1-0.2 cm) in 25%, mild (>95th percentile for normal subjects but <0.1 cm) in 42%, and absent in 16% of the subjects who had been treated for acoustic neuroma. CONCLUSION: This method of objective assessment of facial function is useful in the evaluation of the asymmetry in facial motion and in the detection and quantification of synkinesis. The findings suggest that those subjective rating systems of facial function that compare the abnormal to the normal side may be confounded by compensatory motion on the presumed normal side.