Options and strategies for hearing restoration in pediatric neurofibromatosis type 2.

PURPOSE: In this article, we will review the mechanisms and natural history of hearing loss in neurofibromatosis type 2 (NF2) and discuss the hearing outcomes with different rehabilitation options. METHODS: Review of the published literature. RESULTS: NF2 is a rare autosomal dominant syndrome characterized by vestibular schwannomas and other intracranial and spinal tumors. Bilateral vestibular schwannomas are the hallmark of the disease which occur in 90 to 95% of the patients. As a result, hearing loss will eventually occur in almost all NF2 patients. Deafness can occur from tumor progression or from treatment of vestibular schwannomas and is among the most debilitating aspects of NF2. A number of surgical and non-surgical rehabilitation options are available for these patients including cochlear and auditory brainstem implants. The audiologic outcomes with surgical rehabilitation options have been variable but most patients are able to achieve sound awareness and benefit from auditory cues in lip reading. CONCLUSION: Early identification and treatment of NF2 patients can help in achieving better hearing outcomes in the pediatric population. An increasing number of NF2 patients are receiving open set word understanding with refinement in surgical techniques.

Tinnitus Patients Suffering from Anxiety and Depression: A Review.

OBJECTIVES: To review literature on the link between depression and anxiety in patients suffering from tinnitus. METHOD: A systematic review of published English-language literature was performed using PubMed, Ovid, and Cochrane databases. RESULTS: Of the 56 eligible abstracts 15 were chosen to be included in the review. All articles showed an association of depression and anxiety in tinnitus patients. CONCLUSIONS: Because of the strong association between tinnitus, depression, and anxiety- all tinnitus patients should be screened for psychiatric disorders. Treatment for these complex conditions should involve a multidisciplinary team with cognitive behavioral therapy and possible pharmacological therapy.

The National Ambulatory Medical Care Survey: PAs and NPs in outpatient surgery.

BACKGROUND: This study aimed to determine the prevalence and occupational characteristics of physician assistants (PAs) and nurse practitioners (NPs) in outpatient surgical subspecialty clinics. METHODS: The 2007 and 2008 National Ambulatory Medical Care Survey (NAMCS) databases were queried for the number and characteristics of office visits seen by different provider types (PAs or NPs, physicians, or both) in various surgical subspecialties. RESULTS: More than 250 million weighted sample visits were analyzed. PAs or NPs were involved in 5.9% of visits, though the percentage of patients seen by them alone (1.1%) was significantly lower (P<0.0001). PAs and NPs were more likely to be involved in pre- or postoperative visits, and often saw the same diagnoses alone as physicians only. The most common procedures performed by PAs and NPs varied according to subspecialty. CONCLUSIONS: PAs and NPs have a minor prevalence in the ambulatory surgical workforce during the time period studied. Further integration of these providers into the outpatient setting may help optimize efficiency in ambulatory surgical care.

A micro-drive hearing aid: a novel non-invasive hearing prosthesis actuator.

The direct hearing device (DHD) is a new auditory prosthesis that combines conventional hearing aid and middle ear implant technologies into a single device. The DHD is located deep in the ear canal and recreates sounds with mechanical movements of the tympanic membrane. A critical component of the DHD is the microactuator, which must be capable of moving the tympanic membrane at frequencies and magnitudes appropriate for normal hearing, with little distortion. The DHD actuator reported here utilized a voice coil actuator design and was 3.7 mm in diameter. The device has a smoothly varying frequency response and produces a precisely controllable force. The total harmonic distortion between 425 Hz and 10 kHz is below 0.5 % and acoustic noise generation is minimal. The device was tested as a tympanic membrane driver on cadaveric temporal bones where the device was coupled to the umbo of the tympanic membrane. The DHD successfully recreated ossicular chain movements across the frequencies of human hearing while demonstrating controllable magnitude. Moreover, the micro-actuator was validated in a short-term human clinical performance study where sound matching and complex audio waveforms were evaluated by a healthy subject.

Nasopharyngeal acid reflux and Eustachian tube dysfunction in adults.

OBJECTIVE: This study aimed to evaluate the relationship between nasopharyngeal pH and Eustachian tube dysfunction (ETD) in adults. STUDY DESIGN: Unmatched case-control study. METHODS: Forty-one subjects, 20 adults with a diagnosis of ETD and 21 healthy adults as controls, were enrolled from an outpatient clinic. All subjects had a Dx-pH probe placed near the torus tubarius in the posterior nasopharynx for 24 hours. The pH values were recorded every 0.5 second. Decreases in pH were considered as reflux events if the pH dropped below 5.5. RESULTS: The average nasopharyngeal pH value was 6.90 (range, 5.33-7.73) in the subjects with ETD and 7.07 (range, 5.99-7.94) in the controls. The difference between the 2 groups was not statistically significant (P = .30). The ETD group, on average, had a higher number of nasopharyngeal reflux events (2.3 +/- 1.6 vs 0.8 +/- 1.2, respectively; P = .002) and higher reflux finding score (3.6 +/- 2.7 vs 0.4 +/- 1.4, respectively; P < .00 I) than the control group. CONCLUSION: By using a novel pH probe that allows detection of acidity in a nonliquid environment, a comparison of nasopharyngeal pH between control patients and those with ETD was performed. Eustachian tube dysfunction was more likely to be associated with a higher number of nasopharyngeal reflux events and higher reflux finding score. Nasopharyngeal reflux may have a role in the pathogenesis of ETD.

Noise-induced hearing threshold shift among US adults and implications for noise-induced hearing loss: National Health and Nutrition Examination Surveys.

The objective of this study was to estimate the prevalence and evaluate the associated risk factors of the noise-induced hearing threshold shift (NITS) in the US adult population based on the National Health and Nutrition Examination Surveys (NHANES). The study population consisted of 5,418 individuals aged 20-69 years who had complete audiologic data from the NHANES database. Stringent criteria were used to define NITS. Prevalence of unilateral, bilateral, and total NITS and their association with several socio-demographic and hearing-related factors were evaluated. The prevalence of unilateral, bilateral, and total NITS was 9.4, 3.4 and 12.8%, respectively. Prevalence of bilateral NITS was higher in subjects with older age, male gender, white (non-Hispanic) and Hispanic ethnicities, education level less than or equal to high school diploma, married/living with partner status, Mexico as country of birth, service in armed forces, smoking history, diabetes, and different kinds of noise exposure. Odds of NITS were only higher in older people, males, and smokers. This study provides comprehensive information on the prevalence of NITS in the US adult population and its associated risk factors. More targeted interventions may be done for educational, preventative, and screening purposes.

Comparison of Surgeons' Assessment of the Extent of Vestibular Schwannoma Resection with Immediate Post Operative and Follow-Up Volumetric MRI Analysis.

(1) Background: Incomplete excision of vestibular schwannomas (VSs) is sometimes preferable for facial nerve preservation. On the other hand, subtotal resection may be associated with higher tumor recurrence. We evaluated the correlation between intra-operative assessment of residual tumor and early and follow-up imaging. (2) Methods: The charts of all patients undergoing primary surgery for sporadic vestibular schwannoma during the study period were retrospectively reviewed. Data regarding surgeons' assessments of the extent of resection, and the residual size of the tumor on post-operative day (POD) one and follow-up MRI were extracted. (3) Results: Of 109 vestibular schwannomas meeting inclusion criteria, gross-total resection (GTR) was achieved in eighty-four, near-total (NTR) and sub-total resection (STR) in twenty-two and three patients, respectively. On follow up imaging, volumetric analysis revealed that of twenty-two NTRs, eight were radiographic GTR and nine were radiographic STR (mean volume ratio 11.9%), while five remained NTR (mean volume ratio 1.8%). Of the three STRs, two were radiographic GTR while one remained STR. Therefore, of eighteen patients with available later follow up MRIs, radiographic classification of the degree of resection changed in six. (4) Conclusions: An early MRI (POD#1) establishes a baseline for the residual tumor that may be more accurate than the surgeon's intraoperative assessment and may provide a beneficial point of comparison for long-term surveillance.

Content Validity of a High-Fidelity Surgical Middle Ear Simulator: A Randomized Prospective International Multicenter Trial.

OBJECTIVE: After demonstration of face validity of a surgical middle ear simulator (SMS) previously, we assessed the content validity of the simulator with otolaryngology residents. STUDY DESIGN: Multicenter randomized prospective international study. SETTING: Four academic institutions. METHODS: Novice participants were randomized into control, low-fidelity (LF), and high-fidelity (HF) groups. Control and LF produced 2 recordings from 2 attempts, and HF produced 4 recordings from 10 attempts, with trials 1, 4, 7, and 10 used for scoring. Three blinded experts graded videos of the simulated stapedectomy operation using an objective skills assessment test format consisting of global and stapedotomy-specific scales. RESULTS: A total of 152 recordings from 61 participants were included. Baseline characteristics did not differ significantly between groups. Depending on the step of the operation, inter-rater reliability ranged from 24 to 90%. For LF and HF, years of training was significantly associated with improved scores in certain objective skills assessment test subparts. HF outperformed the control group on stapes and global scores ( p < 0.05). The HF group demonstrated improvement in global score over trials, but plateaued after four trials. Scores varied greatly for participants from different institutions in certain operative steps, such as transecting incudostapedial joints, likely due to differences in instrumentation and time elapsed since manufacture. CONCLUSION: Practice with SMS led to better performance in both global and stapes-specific scores. Further studies are needed to examine construct validity and to create otology-appropriate grading systems. Variables like instrumentation and decline in flexibility of the simulator after 12 months greatly affect performance on the simulator.

Accuracy of tinnitus pitch matching using a web-based protocol.

OBJECTIVES: We investigated the accuracy of a web-based protocol for tinnitus frequency matching compared to that of tinnitus pitch matching performed by an audiologist using an audiometer in an anechoic chamber. METHODS: Twenty subjects underwent tinnitus frequency-matching in a random order using an audiometer in an anechoic chamber and using web-based software with a multiple-choice protocol in octave or half-octave steps from 250 Hz to 12,000 Hz and a slider in 25-Hz steps from 20 to 20,000 Hz. Octave challenge testing was performed. The participants were asked to indicate which protocol resulted in the closest match to their tinnitus frequency. RESULTS: The median tinnitus frequency was 6,000 Hz (range, 2,000 to 12,000 Hz) with use of the audiometer and self-directed multiple-choice protocol. With the slider, the median frequency was 5,925 Hz (range, 1,850 to 16,000 Hz). The patients with a tinnitus frequency higher than 12,000 Hz experienced a greater level of satisfaction when using the computer-based slider system. Five patients experienced octave confusion with self-directed multiple-choice tinnitus matching that was corrected accurately after the octave challenge step. CONCLUSIONS: A web-based protocol for tinnitus frequency matching is as accurate as a standard audiometric protocol. An octave challenge test might be necessary for patient-directed tinnitus frequency matching.

Detecting nasopharyngeal reflux: a novel pH probe technique.

OBJECTIVES: We sought to ascertain the normal pH values in the aerosolized environment of the nasopharynx in healthy subjects and utilize a novel pH probe that allows measuring acidity in a nonliquid environment. METHODS: Between November 2009 and February 2011, healthy volunteers without a history of reflux or eustachian tube dysfunction were enrolled in the prospective study. A total of 20 subjects had a Dx-pH Measurement System Probe (Respiratory Technology Corp) placed near the torus tubarius. The pH probe records the pH throughout the 24-hour study. A pH below 5.5 while the subject was upright or below 5.0 while the subject was supine was used as a criterion to determine a reflux event. Recording was stopped during meals. RESULTS: For normal individuals with no history of reflux or eustachian tube dysfunction, the pH values obtained from the nasopharynx ranged from 6.10 to 7.92. The average pH was 7.03 (SD, 0.67). Eight subjects (40%) had at least 1 reflux event during the 24-hour pH study. CONCLUSIONS: By utilizing a novel self-condensing pH probe, we were able to perform a 24-hour pH study in the nasopharynx of 20 healthy individuals. In our study, the average pH for individuals without symptomatic reflux or eustachian tube dysfunction was 7.03. Interestingly, 8 control subjects had at least 1 episode of pH below 5.5 while awake or below 5.0 while asleep, which was considered to be a reflux event in our study.

Transmastoid approach to temporal bone cerebrospinal fluid leaks.

PURPOSE: The aim of the study was to evaluate various presentations and treatment options for spontaneous cerebrospinal fluid (CSF) leakage originating in the temporal bone. MATERIALS AND METHODS: Clinical data and imaging results for 18 ears (15 patients) presenting with spontaneous CSF leakage originating in the temporal bone were reviewed. Average follow-up period was 13.5 months. The main outcome measure was presence of persistent CSF leak postoperatively. A standard postauricular mastoidectomy was performed. RESULTS: Fifteen patients diagnosed with spontaneous CSF leakage over an 8-year period including 3 treated for bilateral disease were included in the study. The age ranged between 33 and 83 years. Presenting symptoms included serous otitis media (44%), persistent otorrhea after tympanostomy tube placement (28%), and meningitis (28%). Preoperative diagnosis was made using imaging studies and was substantiated by observation of CSF leakage and dural herniation intraoperatively. Treatment was eustachian tube plugging (5%), mastoidectomy with fat obliteration (61%), middle fossa approach with extradural (17%), intradural repair (5%), or combined middle fossa and transmastoid (TM) approach (11%). Successful treatment was obtained in 17 of the 18 cases. The last 9 patients in the series underwent TM approach alone for repair with no treatment failures. CONCLUSIONS: Repair of defects in tegmen mastoideum and posterior fossa can be successfully achieved on an outpatient basis without regard to size and multitude of defects via TM approach. This approach obviates the need for a craniotomy or lumbar drain.

Genetics of hearing loss: where are we standing now?

Hearing loss (HL) is the most common sensory impairment and is caused by a broad range of inherited to environmental causes. Inherited HL consists 50-60% of all HL cases. The inherited form of HL is further classified to different categories. More than 300 syndromes and 40 genes have been identified to result in different levels of HL. Although several diagnostic or screening tests have been developed, yet there are controversies around their use.

Customized web-based sound therapy for tinnitus.

INTRODUCTION: Traditional masking efforts have presented broadband noises, most typically white noise. Targeted (customized) sound therapies have been introduced to overcome the limitations of traditional maskers. OBJECTIVES: To evaluate the efficacy of a web-based delivery of customized sound therapy in reducing tinnitus loudness and increasing the residual inhibition. MATERIALS AND METHODS: Thirty-two subjects matched their tinnitus frequency using a web-based protocol. A customized Harmonic Sound Therapy file was produced by the web-based software and downloaded by the patient onto an MP3 player. The subjects listened to the sound file for one hour. RESULTS: Tinnitus loudness mean was 6.0 ± 2.3 standard deviation (SD) which decreased to 3.3 ± 1.9 SD after one hour of sound therapy. Some reduction in loudness was seen in 81%, while 72% experienced a reduction of 25% or more. Tinnitus annoyance decreased from an average of 6.1 ± 2.6 SD to 3.1 ± 2.0 SD. Median duration of residual inhibition was 32.5 minutes, with an average of 75 minutes ± 132 SD. CONCLUSION: Customized sound therapy can be delivered via the internet. Harmonic Sound Therapy is effective in reducing the loudness and annoyance of tinnitus.

Diagnostic efficacy of different methods in the assessment of adenoid hypertrophy.

OBJECTIVE: This study was designed for better understanding of the role of different methods of nasal endoscopy in the assessment of adenoid hypertrophy and comparing them with lateral neck radiography and patients' symptoms. SUBJECTS AND METHOD: From August 2007 until January 2009, in the otolaryngology ward of a tertiary referral center, 89 patients who had symptoms related to chronic mouth breathing participated in this study. History of the symptoms related to adenoid hypertrophy was obtained from them. In addition, all patients underwent nasal endoscopy and lateral nasopharynx x-ray. The clinician who did nasal endoscopy was blinded to information about clinical data and x-ray and vice versa. Afterward, the relationship between symptoms and each diagnostic procedure was evaluated. RESULTS: Patients had a mean age of 9.47 ± 4.68 years. In the evaluation of the relationship between symptoms grading and grading in lateral neck radiography, this relationship was significant about snoring. Furthermore, there was a significant relationship between the endoscopic size of adenoid and number of the episodes of acute otitis media. The sum of symptoms grading had a significant relationship with the size of adenoid in lateral neck x-ray, but not in nasal endoscopy. CONCLUSION: The results of the present study indicated that both radiography and nasal endoscopy could define the relationship between adenoid hypertrophy and associated symptoms and therefore are complementary. Between them, despite the popularity of nasal endoscopy, radiography can serve as a better planning tool.

Hypercoagulability in Vestibular Schwannoma Surgery.

OBJECTIVE: Patients with vestibular schwannoma who harbor a genetic predisposition for venous thromboembolism require special consideration when determining optimal therapeutic management. The primary objective of the current study was to provide recommendations on treatment of hypercoagulable patients with vestibular schwannoma through a case series and review of the literature. PATIENTS: Two patients who underwent resection of vestibular schwannomas. INTERVENTIONS: Surgical resection and diagnostic testing. MAIN OUTCOME MEASURES: Postoperative venous thromboses. RESULTS: One patient who underwent resection of vestibular schwannoma and suffered several postoperative thrombotic complications consistent with a clinical thrombophilia. One patient with known Factor V Leiden deficiency who underwent resection of vestibular schwannoma followed by postoperative chemoprophylaxis with a direct factor Xa inhibitor and experienced an uneventful postoperative course. CONCLUSIONS: In patients with a known propensity for venous thromboembolism, the skull base surgeon should consider nonsurgical management. If the patient undergoes surgical resection, we recommend careful effort to minimize trauma to the sigmoid sinus. In addition, the surgeon may consider retrosigmoid or middle fossa approaches. Best practice recommendations include the use of pneumatic compression devices, early ambulation, and consideration of postoperative prophylactic anticoagulation in patients with a known genetic predisposition.

False-Positive Cholesteatomas on Non-Echoplanar Diffusion-Weighted Magnetic Resonance Imaging.

OBJECTIVES: To investigate false-positive findings on non-echoplanar (non-EPI) diffusion-weighted magnetic resonance imaging (DWI) in patients under surveillance post-cholesteatoma surgery. STUDY DESIGN, SETTING, SUBJECTS, AND METHODS: A retrospective review was performed on patients diagnosed with cholesteatoma who underwent surgical resection and were then followed by serial non-EPI DWI using half-Fourier acquisition single-shot turbo spin echo (HASTE) sequence. All patients had at least two annual follow-up imaging studies. RESULTS: False-positive findings were identified in four patients. The size of the suspected lesions was 4 to 12 mm. Otoendoscopy was used during all primary cases and Argon laser was used in one case. In all cases, the entire cholesteatoma was removed, and no residual disease was detected at the end of the procedures. One patient underwent revision surgery but only cartilage graft was found in the area of concern. All patients had stable or resolved hyperintense areas in the subsequent HASTE sequences. CONCLUSION: False positive findings can occur with non-EPI DWI MRI and patients need to be counseled accordingly before revision surgery. Decreasing intensity and dimension of a suspected lesion and a positive finding in an area other than the location of the initial cholesteatoma may favor a false positive. If a false positive finding is suspected when the surgeon is confident of complete resection of the cholesteatoma, an MRI can be repeated in 6 to 12 months to assess changes in the dimension and intensity of the area of concern. Cartilage grafts may cause restricted diffusion on DWI sequences.

Hearing Difficulty and Risk of Mortality.

OBJECTIVE: Investigate whether hearing difficulty has an influence on the risk of mortality. METHODS: A nationally representative sample of individuals 18 years or older with data available for hearing and mortality status was selected from the National Health Interview Surveys (NHIS) 2005-2009. Self-perceived hearing status was regrouped as excellent/good, a little to moderate trouble, a lot of trouble, and deaf. Other independent variables investigated were demographics and comorbidities. Univariate analysis was performed to calculate the incidence of mortality, and 95% confidence intervals (CI) and multivariate analysis adjusted for demographics and comorbidities was performed to calculate odds ratios (OR) of mortality. Those with excellent/good hearing were considered as reference for ORs. RESULTS: Of 215.6 million Americans (mean age = 45.9 years; 51.7% female), approximately 16.0% (95% CI, 15.6%-16.3%) considered their hearing less than excellent or good. The 5-year mortality rate was 4.2% (95% CI, 4.0%-4.3%). In the univariate analysis, the mortality rate increased with the degree of hearing difficulty from 3.0% in excellent/good hearing to 19.5% in a lot of trouble hearing and 17.8% in deaf. With multivariate analysis, adjusted ORs of mortality were 1.5 (95% CI, 1.3-1.7) in those who had a lot of trouble hearing and 1.6 (95% CI, 1.1-2.3) in those who were deaf. CONCLUSION: Hearing difficulty may be associated with an increased risk of mortality, and this risk may correlate with the degree of hearing difficulty.

Visual and Endocrine Recovery Following Conservative and Surgical Treatment of Pituitary Apoplexy: A Meta-Analysis.

BACKGROUND: Pituitary apoplexy (PA) can manifest with visual and endocrine defects. The literature lacks strong support for either surgical or conservative management with respect to symptomatic improvement of these deficits. This meta-analysis compared visual and endocrine outcomes in conservative and surgical treatment of PA. METHODS: A systematic literature search was performed in PubMed, Cochrane, and Ovid MEDLINE for articles published between 1988 and 2018. Recovery outcomes were binarized, such that complete and partial improvements were combined as "improvement." Primary outcome variables evaluated via a binary random-effects model were improvements in endocrine dysfunction, visual field and acuity deficits, and ophthalmoplegia or ocular nerve palsy. RESULTS: Of 483 published articles, 14 studies comprising 457 cases (259 surgical treatments and 198 conservative treatments) were included. On initial examination, 58% of patients had endocrine dysfunction, 37% had visual acuity or field deficit, and 47% had ophthalmoplegia or ocular nerve palsy. Evaluation of outcomes for surgically and conservatively treated patients yielded odds ratios of 0.609 (95% confidence interval [CI], 0.199-1.859; P = 0.383), 0.763 (95% CI, 0.307-2.374; P = 0.763), 1.167 (95% CI, 0.433-3.146; P = 0.760), and 0.801 (95% CI, 0.305-2.105; P = 0.653) for improvements in endocrine dysfunction, visual acuity dysfunction, visual field dysfunction, and ophthalmoplegia or ocular nerve palsy. CONCLUSIONS: Both surgical intervention and conservative management of PA can lead to visual and endocrine recovery, although the management decision may heavily rely on severity of initial deficits. Treatment of PA can be multifaceted and tailored to the individual case and clinical judgment. Further investigation into appropriate intervention based on longitudinal outcome data is warranted.

Vision Outcomes in Early versus Late Surgical Intervention of Pituitary Apoplexy: Meta-Analysis.

BACKGROUND: Pituitary apoplexy (PA) is defined by hemorrhage and necrosis of the pituitary gland, often acute in onset, and frequently in the setting of an existing pituitary adenoma. Our objective was to conduct a meta-analysis of the available literature on vision outcomes following surgical intervention for PA on the basis of the timing from apoplexy to surgery (ATS). METHODS: A thorough literature search of the published English-language literature was performed in PubMed, Ovid, and Cochrane databases using the key words ("pituitary apoplexy") and ("surgery" or "vision") from database inception to August 2018 was conducted. The primary outcome variable evaluated using a binary random-effects model was vision recovery outcomes (metric: odds ratio). RESULTS: Of 234 articles found, 12 articles containing 200 patients met our eligibility criteria. The mean age was 46.1, with a male-to-female ratio of 2.9:1. A total of 86% of PA patients presented with visual deficits (ATS <7 days in 93 and >7 days in 79 patients). In patients with an ATS <7 days, 97.8% experienced visual recovery, compared with 84.8% with an ATS >7 days (odds ratio 2.6 [95% CI 0.94-7.31]; P value = 0.07). CONCLUSIONS: Despite readily accepted guidelines provided by the United Kingdom advocating for early surgical intervention in PA, the rates of vision outcomes we report demonstrate >80% recovery for patients in both the early and late surgical intervention group. As such, conservative management may be warranted for early stabilization before surgical intervention in PA patients with respect to vision outcomes.