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Hearing Disorders: HELP
Articles by John Patrick Carey
Based on 19 articles published since 2009
(Why 19 articles?)
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Between 2009 and 2019, John P. Carey wrote the following 19 articles about Hearing Disorders.
 
+ Citations + Abstracts
1 Review Canal dehiscence. 2011

Chien, Wade W / Carey, John P / Minor, Lloyd B. ·Department of Otolaryngology-Head & Neck Surgery, The Johns Hopkins University School of Medicine, Baltimore, Maryland, USA. wchien1@jhmi.edu ·Curr Opin Neurol · Pubmed #21124219.

ABSTRACT: PURPOSE OF REVIEW: The aim is to review canal dehiscence involving the superior, lateral, and posterior semicircular canals. The main focus will be on superior semicircular canal dehiscence. RECENT FINDINGS: Canal dehiscence involving the superior, lateral, and posterior semicircular canal can have different etiologies, including developmental abnormality, congenital defect, chronic otitis media with cholesteatoma, and high-riding jugular bulb. However, their clinical presentation can be very similar, with patients complaining of vertigo, oscillopsia, and sometimes hearing loss. Canal dehiscence causes an abnormal communication between the inner ear and the surrounding structures. This creates a third mobile window within the inner ear, disrupting its normal mechanics and causing symptoms. SUMMARY: Superior semicircular canal dehiscence is now a well-established entity in the medical literature. Surgical repair is effective at relieving patients' vestibular symptoms. Lateral semicircular canal dehiscence is usually associated with chronic otitis media. Posterior semicircular canal dehiscence is a rare entity, with similar clinical presentations and treatment options as the other canal dehiscences.

2 Article Safe Intraoperative Neurophysiologic Monitoring During Posterior Spinal Fusion in a Patient With Cochlear Implants. 2018

Abiola, Godwin / Ward, Bryan Kevin / Bowditch, Stephen / Ritzl, Eva Katharina / Carey, John Patrick. ·Johns Hopkins School of Medicine. · Department of Otolaryngology-Head and Neck Surgery. · Department of Neurology, Johns Hopkins School of Medicine, Baltimore, Maryland. ·Otol Neurotol · Pubmed #29738385.

ABSTRACT: OBJECTIVE: Cochlear implants are generally considered a contraindication for any procedure requiring electrical stimulation near the implant. We present a case of a patient undergoing intraoperative transcranial electrical motor-evoked potential monitoring with a cochlear implant without adverse outcomes. PATIENT: A 12-year-old girl with a history of VACTERL presented with worsening congenital kyphosis and bilateral severe-to-profound hearing loss. Since age 7 the patient used a cochlear implant in the right ear and hearing aid in the left ear. Physical examination and magnetic resonance imaging in 2016 revealed a left-sided 66-degree thoracolumbar kyphosis at T11 making the patient a candidate for surgical correction. INTERVENTIONS: She underwent a posterior spinal fusion surgery, performed with intraoperative transcranial electrical motor-evoked potential monitoring. Steps were taken to mitigate electrical stimulation of the patient's cochlear implant. MAIN OUTCOME MEASURES: Postoperative impedance of individual channels, audiometry, and neural response testing were compared with preoperative measurements. RESULTS: Significant (>10%) impedance changes were observed postoperatively in channels 1, 2, 4, and 6; however, the net variation across all the channels was low (3%). The patient reported no hearing changes, and no significant changes in hearing threshold were seen in postoperative audiometric testing or neural response testing. CONCLUSION: We present a case of successful posterior spinal fusion with intraoperative neurophysiological monitoring via transcranial electrical stimulation, in a patient with a cochlear implant. With proper precautions, motor-evoked potential monitoring can be safely performed in a patient with a cochlear implant.

3 Article Sudden Hearing Loss with Vertigo Portends Greater Stroke Risk Than Sudden Hearing Loss or Vertigo Alone. 2018

Chang, Tzu-Pu / Wang, Zheyu / Winnick, Ariel A / Chuang, Hsun-Yang / Urrutia, Victor C / Carey, John P / Newman-Toker, David E. ·Department of Neurology, Johns Hopkins University School of Medicine, Baltimore, Maryland; Department of Neurology/Neuro-medical Scientific Center, Taichung Tzu Chi Hospital, Buddhist Tzu Chi Medical Foundation, Taichung, Taiwan; Department of Medicine, Tzu Chi University, Buddhist Tzu Chi Medical Foundation, Hualien, Taiwan. · Department of Oncology, Johns Hopkins University School of Medicine, Baltimore, Maryland; Department of Biostatistics, Johns Hopkins University School of Public Health, Baltimore, Maryland. · Department of Neurology, Johns Hopkins University School of Medicine, Baltimore, Maryland. · Department of Research, Taichung Tzu Chi Hospital, Buddhist Tzu Chi Medical Foundation, Taichung, Taiwan. · Department of Otolaryngology- Head and Neck Surgery, Johns Hopkins University School of Medicine, Baltimore, Maryland. · Department of Neurology, Johns Hopkins University School of Medicine, Baltimore, Maryland; Department of Otolaryngology- Head and Neck Surgery, Johns Hopkins University School of Medicine, Baltimore, Maryland; Armstrong Institute Center for Diagnostic Excellence, Johns Hopkins University School of Medicine, Baltimore, Maryland. Electronic address: toker@jhu.edu. ·J Stroke Cerebrovasc Dis · Pubmed #29102540.

ABSTRACT: BACKGROUND: Because it is unknown whether sudden hearing loss (SHL) in acute vertigo is a "benign" sign (reflecting ear disease) or a "dangerous" sign (reflecting stroke), we sought to compare long-term stroke risk among patients with (1) "SHL with vertigo," (2) "SHL alone," and (3) "vertigo alone" using a large national health-care database. METHODS: Patients with first-incident SHL (International Classification of Diseases, Ninth Edition, Clinical Modification [ICD-9-CM] 388.2) or vertigo (ICD-9-CM 386.x, 780.4) were identified from the National Health Insurance Research Database of Taiwan (2002-2009). We defined SHL with vertigo as a vertigo-related diagnosis ±30 days from the index SHL event. SHL without a temporally proximate vertigo diagnosis was considered SHL alone. The vertigo-alone group had no SHL diagnosis. All the patients were followed up until stroke, death, withdrawal from the database, or current end of the database (December 31, 2012) for a minimum period of 3 years. The hazards of stroke were compared across groups. RESULTS: We studied 218,656 patients (678 SHL with vertigo, 1998 with SHL alone, and 215,980 with vertigo alone). Stroke rates at study end were 5.5% (SHL with vertigo), 3.0% (SHL alone), and 3.9% (vertigo alone). Stroke hazards were higher in SHL with vertigo than in SHL alone (hazard ratio [HR], 1.93; 95% confidence interval [CI], 1.28-2.91) and in vertigo alone (HR, 1.63; 95% CI, 1.18-2.25). Defining a narrower window between SHL and vertigo (±3 days) increased the hazards. CONCLUSIONS: The combination of SHL plus vertigo in close temporal proximity is associated with increased subsequent stroke risk over SHL alone and vertigo alone. This suggests that SHL in patients with vertigo is not necessarily a benign peripheral vestibular sign.

4 Article Long-Term Patient-Reported Outcomes After Surgery for Superior Canal Dehiscence Syndrome. 2017

Alkhafaji, Mohammed S / Varma, Sanskriti / Pross, Seth E / Sharon, Jeffrey D / Nellis, Jason C / Santina, Charles C Della / Minor, Lloyd B / Carey, John P. ·*Department of Otolaryngology-Head and Neck Surgery, Johns Hopkins University School of Medicine, Baltimore, Maryland †Department of Otolaryngology-Head and Neck Surgery, University of California, San Francisco ‡Department of Otolaryngology-Head and Neck Surgery, Stanford University, Palo Alto, California. ·Otol Neurotol · Pubmed #28902804.

ABSTRACT: OBJECTIVE: Evaluate the long-term patient-reported outcomes of surgery for superior canal dehiscence syndrome (SCDS). STUDY DESIGN: Cross-sectional survey. SETTING: Tertiary referral center. PATIENTS: Adults who have undergone surgery for SCDS with at least 1 year since surgery. MAIN OUTCOME MEASURE(S): Primary outcome: change in symptoms that led to surgery. SECONDARY OUTCOMES: change in 11 SCDS-associated symptoms, change in psychosocial metrics, and willingness to recommend surgery to friends with SCDS. RESULTS: Ninety-three (43%) respondents completed the survey with mean (SD) time since surgery of 5.3 (3.6) years. Ninety-five percent of respondents reported the symptoms that led them to have surgery were "somewhat better," "much better," or "completely cured." Those with unilateral symptoms were more likely to report improvement than those with bilateral symptoms. There was no difference between those with short (1-5 yr) versus long (5-20 yr) follow-up. Each of the SCDS-associated symptoms showed significant improvement. The largest improvements were for autophony, pulsatile tinnitus, audible bodily sounds, and sensitivity to loud sound. Headaches, imbalance, dizziness, and brain fog showed the least improvements. Most patients reported improvements in quality of life, mood, and ability to function at work and socially. Ninety-five percent of patients would recommend SCDS surgery. CONCLUSIONS: Respondents demonstrated durable improvements in the symptoms that led them to have surgery. Auditory symptoms had the greatest improvements. Headaches, imbalance, dizziness, and brain fog showed the least improvements. Nearly, all patients would recommend SCDS surgery to others. These results can be used to counsel patients regarding the lasting benefits of surgery for SCDS.

5 Article Rhesus Cochlear and Vestibular Functions Are Preserved After Inner Ear Injection of Saline Volume Sufficient for Gene Therapy Delivery. 2017

Dai, Chenkai / Lehar, Mohamed / Sun, Daniel Q / Rvt, Lani Swarthout / Carey, John P / MacLachlan, Tim / Brough, Doug / Staecker, Hinrich / Della Santina, Alexandra M / Hullar, Timothy E / Della Santina, Charles C. ·Vestibular NeuroEngineering Lab, Department of Otolaryngology-Head & Neck Surgery, Johns Hopkins University School of Medicine, 720 Rutland Ave., Ross Bldg Rm 830, Baltimore, MD, 21205, USA. cdai4@jhu.edu. · Department of Biomedical Engineering, Johns Hopkins University School of Medicine, 720 Rutland Ave., Ross Bldg Rm 830, Baltimore, MD, 21205, USA. cdai4@jhu.edu. · Vestibular NeuroEngineering Lab, Department of Otolaryngology-Head & Neck Surgery, Johns Hopkins University School of Medicine, 720 Rutland Ave., Ross Bldg Rm 830, Baltimore, MD, 21205, USA. · Department of Biomedical Engineering, Johns Hopkins University School of Medicine, 720 Rutland Ave., Ross Bldg Rm 830, Baltimore, MD, 21205, USA. · Novartis Institutes for Biomedical Research, 250 Massachusetts Ave, Cambridge, MA, 02139, USA. · GenVec, 910 Clopper Rd #220n, Gaithersburg, MD, 20878, USA. · Dept of Otolaryngology, Head & Neck Surgery, University of Kansas School of Medicine, 3901 Rainbow Blvd, Kansas City, KS, 66160, USA. · Department of Otolaryngology, Washington University School of Medicine, 660 S Euclid Ave, St. Louis, MO, 63110, USA. · Department of Anatomy and Neurobiology, Washington University School of Medicine, 660 S Euclid Ave, St. Louis, MO, 63110, USA. · Department of Audiology and Communication Sciences, Washington University School of Medicine, 660 S Euclid Ave, St. Louis, MO, 63110, USA. ·J Assoc Res Otolaryngol · Pubmed #28646272.

ABSTRACT: Sensorineural losses of hearing and vestibular sensation due to hair cell dysfunction are among the most common disabilities. Recent preclinical research demonstrates that treatment of the inner ear with a variety of compounds, including gene therapy agents, may elicit regeneration and/or repair of hair cells in animals exposed to ototoxic medications or other insults to the inner ear. Delivery of gene therapy may also offer a means for treatment of hereditary hearing loss. However, injection of a fluid volume sufficient to deliver an adequate dose of a pharmacologic agent could, in theory, cause inner ear trauma that compromises functional outcome. The primary goal of the present study was to assess that risk in rhesus monkeys, which closely approximates humans with regard to middle and inner ear anatomy. Secondary goals were to identify the best delivery route into the primate ear from among two common surgical approaches (i.e., via an oval window stapedotomy and via the round window) and to determine the relative volumes of rhesus, rodent, and human labyrinths for extrapolation of results to other species. We measured hearing and vestibular functions before and 2, 4, and 8 weeks after unilateral injection of phosphate-buffered saline vehicle (PBSV) into the perilymphatic space of normal rhesus monkeys at volumes sufficient to deliver an atoh1 gene therapy vector. To isolate effects of injection, PBSV without vector was used. Assays included behavioral observation, auditory brainstem responses, distortion product otoacoustic emissions, and scleral coil measurement of vestibulo-ocular reflexes during whole-body rotation in darkness. Three groups (N = 3 each) were studied. Group A received a 10 μL transmastoid/trans-stapes injection via a laser stapedotomy. Group B received a 10 μL transmastoid/trans-round window injection. Group C received a 30 μL transmastoid/trans-round window injection. We also measured inner ear fluid space volume via 3D reconstruction of computed tomography (CT) images of adult C57BL6 mouse, rat, rhesus macaque, and human temporal bones (N = 3 each). Injection was well tolerated by all animals, with eight of nine exhibiting no signs of disequilibrium and one animal exhibiting transient disequilibrium that resolved spontaneously by 24 h after surgery. Physiologic results at the final, 8-week post-injection measurement showed that injection was well tolerated. Compared to its pretreatment values, no treated ear's ABR threshold had worsened by more than 5 dB at any stimulus frequency; distortion product otoacoustic emissions remained detectable above the noise floor for every treated ear (mean, SD and maximum deviation from baseline: -1.3, 9.0, and -18 dB, respectively); and no animal exhibited a reduction of more than 3 % in vestibulo-ocular reflex gain during high-acceleration, whole-body, passive yaw rotations in darkness toward the treated side. All control ears and all operated ears with definite histologic evidence of injection through the intended site showed similar findings, with intact hair cells in all five inner ear sensory epithelia and intact auditory/vestibular neurons. The relative volumes of mouse, rat, rhesus, and human inner ears as measured by CT were (mean ± SD) 2.5 ± 0.1, 5.5 ± 0.4, 59.4 ± 4.7 and 191.1 ± 4.7 μL. These results indicate that injection of PBSV at volumes sufficient for gene therapy delivery can be accomplished without destruction of inner ear structures required for hearing and vestibular sensation.

6 Article Prevalence of Pulsatile Tinnitus Among Patients With Migraine. 2016

Weinreich, Heather M / Carey, John P. ·Division of Otology, Neurotology and Skull Base Surgery, Department of Otolaryngology-Head and Neck Surgery, The Johns Hopkins University School of Medicine, Baltimore, Maryland. ·Otol Neurotol · Pubmed #26859546.

ABSTRACT: OBJECTIVE: To examine the prevalence of pulsatile tinnitus (PT) among patients with a diagnosis of migraine and to determine if treatment of migraine improves symptoms. STUDY DESIGN: Single-institution retrospective patient review. SETTING: Academic tertiary referral center. PATIENTS: Billing data capturing ICD-9 codes 346.xx and 388.3x was used to identify patients with history of migraine and tinnitus. Patients were excluded if the symptom of PT could be attributed to an alternate diagnosis. Data were extracted from the patients' electronic medical records. INTERVENTION(S): Therapeutic patients were prescribed a strict migraine diet with or without migraine medication. MAIN OUTCOME MEASURE(S): Subjective improvement in tinnitus as documented in electronic medical records. RESULTS: One thousand two hundred four patients were identified with an ICD-9 code for migraine and of those patients, 12% (n = 145) had an ICD-9 code for tinnitus. After ruling out alternative causes, the prevalence of PT among all patients with migraine was 1.9%. Of migrainers with PT who underwent migraine treatment, 11 out of 16 reported resolution or improvement of their PT. CONCLUSION: PT can be observed in the context of migraine. Migraine treatment with avoidance of dietary triggers with or without medication can possibly lead to resolution of PT.

7 Article Intraoperative neuromonitoring for superior semicircular canal dehiscence and hearing outcomes. 2015

Wenzel, Angela / Ward, Bryan K / Ritzl, Eva K / Gutierrez-Hernandez, Sergio / Della Santina, Charles C / Minor, Lloyd B / Carey, John P. ·*Departments of Otolaryngology-Head and Neck Surgery, and †Neurology, Johns Hopkins School of Medicine, Baltimore, Maryland; and ‡Stanford University School of Medicine, Stanford, California, U.S.A. ·Otol Neurotol · Pubmed #25333320.

ABSTRACT: BACKGROUND: Recent findings in patients with superior semicircular canal dehiscence (SCD) have shown an elevated ratio of summating potential (SP) to action potential (AP), as measured by electrocochleography (ECochG). Changes in this ratio can be seen during surgical intervention. The objective of this study was to evaluate the utility of intraoperative ECochG and auditory brainstem response (ABR) as predictive tools for postoperative hearing outcomes after surgical plugging via middle cranial fossa approach for SCD syndrome (SCDS). METHODS: This was a review of 34 cases (33 patients) in which reproducible intraoperative ECochG recordings were obtained during surgery. Diagnosis of SCDS was based on history, physical examination, vestibular function testing, and computed tomography imaging. Simultaneous intraoperative ECochG and ABR were performed. Pure-tone audiometry was performed preoperatively and at least 1 month postoperatively, and air-bone gap (ABG) was calculated. Changes in SP/AP ratio, SP amplitude, and ABR wave I latency were compared with changes in pure-tone average and ABG before and after surgery. RESULTS: Median SP/AP ratio of affected ears was 0.62 (interquartile range [IQR], 0.45-0.74) and decreased immediately after surgical plugging of the affected canal to 0.42 (IQR, 0.29-0.52; p < 0.01). Contralateral SP/AP ratio before plugging was 0.33 (IQR, 0.25-0.42) and remained unchanged at the conclusion of surgery (0.30; IQR, 0.25-0.35; p = 0.32). Intraoperative changes in ABR wave I latency and SP amplitude did not predict changes in pure-tone average or ABG after surgery (p > 0.05). CONCLUSION: This study confirmed the presence of an elevated SP/AP ratio in ears with SCDS. The SP/AP ratio commonly decreases during plugging. However, an intraoperative decrease in SP/AP does not appear to be sensitive to either the beneficial decrease in ABGs or the mild high-frequency sensory loss that can occur in patients undergoing surgical plugging of the superior semicircular canal. Future work will determine the value of intraoperative ECochG in predicting changes in vestibular function.

8 Article Racial difference in cochlear pigmentation is associated with hearing loss risk. 2014

Sun, Daniel Q / Zhou, Xin / Lin, Frank R / Francis, Howard W / Carey, John P / Chien, Wade W. ·*Department of Otolaryngology-Head and Neck Surgery, †Johns Hopkins University School of Medicine, Baltimore; and ‡National Institute on Deafness and Other Communication Disorders, National Institutes of Health, Bethesda, Maryland, U.S.A. ·Otol Neurotol · Pubmed #25166018.

ABSTRACT: OBJECTIVES: The goals of this study are to characterize the distribution of melanin pigmentation in the human cochlea and to investigate differences in pigment content between races. METHODS: Human temporal bone specimens from the Johns Hopkins Temporal Bone Collection were examined. Demographic, clinical, and audiometric data were analyzed. Melanin pigmentation in the cochlea was quantified in each specimen. RESULTS: Nineteen African-American (AA) and 27 Caucasian specimens were selected for the study. The mean ages were 64 and 70 years for AA and Caucasian specimens, respectively (p = 0.21). At all cochlear turns, AA specimens contained significantly more pigmentation in the stria vascularis (p = 0.0003) and Rosenthal's canal (p < 0.0001) compared with Caucasian specimens. Strial melanin content increased significantly with age. Cochlear pigmentation content was not associated with sex or hearing thresholds. CONCLUSION: Melanin pigmentation is significantly more abundant in AA cochleae than in Caucasian cochleae. This study provides a detailed description of pigmentation in the cochlea and may help to explain the observed racial differences in hearing thresholds.

9 Article Stapedial synkinesis causing change in hearing threshold with facial motion. 2013

Brichacek, Michal A / Brandt, Michael G / Carey, John P / Byrne, Patrick J. ·Department of Otolaryngology-Head and Neck Surgery, Division of Facial Plastic and Reconstructive Surgery, Johns Hopkins University School of Medicine, Baltimore, Maryland, USA. ·Otol Neurotol · Pubmed #23370552.

ABSTRACT: OBJECTIVE: Synkinetic facial movement after facial nerve regeneration is a well-documented phenomenon. Rarely, patients recovering from facial nerve injury report feelings of auditory ringing, fullness, and a sensation of ear tightness as a result of stapedial muscle involvement. It is exceedingly rare for such synkinesis to produce perceivable changes in hearing threshold. We report a unique case of stapedial synkinesis causing pure-tone changes in hearing threshold with activation of the facial musculature. PATIENT: A single patient is presented who developed stapedial synkinesis after suboccipital resection of a unilateral acoustic neuroma. RESULTS: Despite facial nerve sparing, surgery resulted in House-Brackmann grade V/VI right facial nerve paralysis that improved to Grade III/VI after 7 months. Synkinesis developed that caused eye closure with puckering of the lips. Puckering of the lips likewise caused decreased hearing in the right ear, corresponding to a measured decrease of 10 dB in the PTA. Over the next several months, facial motion continued to improve, and hearing changes became less bothersome, so no intervention was undertaken. CONCLUSION: The changes presented in the hearing threshold fit within the classically described 15-dB attenuation provided by the stapedial reflex. Although no intervention was undertaken in this particular case, some patients with unremitting stapedial synkinesis might benefit from sectioning of the stapedial muscle. Thus, consideration should be made for audiometric evaluation with and without facial muscle contraction in the evaluation of individuals with synkinetic facial movement.

10 Article Association between hearing loss and saccular dysfunction in older individuals. 2012

Zuniga, Maria Geraldine / Dinkes, Roni E / Davalos-Bichara, Marcela / Carey, John P / Schubert, Michael C / King, W Michael / Walston, Jeremy / Agrawal, Yuri. ·Department of Otolaryngology-Head and Neck Surgery, Johns Hopkins University School of Medicine, Baltimore, Maryland 21287, USA. ·Otol Neurotol · Pubmed #23064383.

ABSTRACT: OBJECTIVE: 1) Describe the association between hearing loss and dysfunction of each of the 5 vestibular end-organs--the horizontal, superior, and posterior semicircular canals; saccule; and utricle--in older individuals. 2) Evaluate whether hearing loss and vestibular end-organ deficits share any risk factors. STUDY DESIGN: Cross-sectional study. SETTING: Academic medical center. PATIENTS: Fifty-one individuals age 70 years or older. INTERVENTIONS: Audiometry, head-thrust dynamic visual acuity (htDVA), sound-evoked cervical vestibular-evoked myogenic potential (cVEMP), and tap-evoked ocular VEMP (oVEMP). MAIN OUTCOME MEASURES: Audiometric pure-tone averages (PTA), htDVA LogMAR scores as a measure of semicircular canal function in each canal plane, and cVEMP and oVEMP amplitudes as a measure of saccular and utricular function, respectively. RESULTS: We observed a significant correlation between hearing loss at high frequencies and reduced cVEMP amplitudes (or reduced saccular function; r = -0.37, p < 0.0001) in subjects age 70 years or older. In contrast, hearing loss was not associated with oVEMP amplitudes (or utricular function), or htDVA LogMAR scores (or semicircular canal function) in any of the canal planes. Age and noise exposure were significantly associated with measures of both cochlear and saccular dysfunction. CONCLUSION: The concomitant decline in the cochlear and saccular function associated with aging may reflect their common embryologic origin in the pars inferior of the labyrinth. Noise exposure seems to be related to both saccular and cochlear dysfunction. These findings suggest a potential benefit of screening individuals with presbycusis-particularly those with significant noise exposure history-for saccular dysfunction, which may contribute to fall risk in the elderly.

11 Article Hearing outcomes after surgical plugging of the superior semicircular canal by a middle cranial fossa approach. 2012

Ward, Bryan K / Agrawal, Yuri / Nguyen, Elena / Della Santina, Charles C / Limb, Charles J / Francis, Howard W / Minor, Lloyd B / Carey, John P. ·Department of Otolaryngology-Head and Neck Surgery, Johns Hopkins School of Medicine, Baltimore, Maryland 21287, USA. bward15@jhmi.edu ·Otol Neurotol · Pubmed #22935810.

ABSTRACT: OBJECTIVE: To determine postoperative hearing outcomes after surgical plugging via middle cranial fossa approach for superior semicircular canal dehiscence syndrome (SCDS). STUDY DESIGN: Clinical review. SETTING: Tertiary care medical center. PATIENTS: Forty-three cases of SCDS based on history, physical examination, vestibular function testing, and computed tomography imaging confirming the presence of a dehiscence. All patients underwent surgical plugging of the superior semicircular canal via middle cranial fossa approach. INTERVENTION: Pure tone audiometry was performed preoperatively and at 7 days and at least 1 month postoperatively. MAIN OUTCOME MEASURES: Change in air-bone gap (ABG) and pure tone average (PTA). RESULTS: Preoperative average ABG across 0.25, 0.5, 1, and 2 kHz was 16.0 dB (standard deviation [SD], 7.5 dB). At 7 days postoperatively, average ABG was 16.5 dB (SD, 11.1; p = 0.42), and at greater than 1 month was 8.1 dB (SD, 8.4; p < 0.001). 53% (95% confidence interval, 33-69) of affected ears had greater than 10 dB increase in their 4-frequency (0.5, 1, 2, and 4 kHz) PTA measured by bone-conduction (BC) threshold 7 days postoperatively and 25% (95% confidence interval, 8-39) at greater than 1 month postoperatively. Mean BC PTA of affected ears was 8.4 dB hearing loss (HL) (SD, 10.4) preoperatively. Compared with baseline, this declined to 19.2 dB HL (SD, 12.6; p < 0.001) at 7 days postoperatively and 16.4 dB HL (SD, 18.8; p = 0.01) at greater than 1 month. No significant differences in speech discrimination score were noted (F = 0.17). CONCLUSION: Low-frequency air-bone gap decreases after surgical plugging and seems to be due to both increased BC thresholds and decreased AC thresholds. Surgical plugging via a middle cranial fossa approach in SCDS is associated with mild high-frequency sensorineural hearing loss that persists in 25% but no change in speech discrimination.

12 Article Audiology in the sudden hearing loss clinical trial. 2012

Halpin, Chris / Shi, Helen / Reda, Domenic / Antonelli, Patrick J / Babu, Seilesh / Carey, John P / Gantz, Bruce J / Goebel, Joel A / Hammerschlag, Paul E / Harris, Jeffrey P / Isaacson, Brandon / Lee, Daniel / Linstrom, Chris J / Parnes, Lorne S / Slattery, William H / Telian, Steven A / Vrabec, Jeffrey T / Rauch, Steven. ·Department of Audiology, Massachusetts Eye and Ear Infirmary, USA. chris_halpin@meei.harvard.edu ·Otol Neurotol · Pubmed #22805100.

ABSTRACT: OBJECTIVE: To report the pretreatment and posttreatment population characteristics and the overall stability of the audiologic outcomes found during the Sudden Hearing Loss Clinical Trial (ClinicalTrials.gov: Identifier NCT00097448). STUDY DESIGN: Multicenter, prospective randomized noninferiority trial of oral versus intratympanic (IT) steroid treatment of sudden sensorineural hearing loss (SSNHL). SETTING: Fifteen academically based otology practices. PATIENTS: Two hundred fifty patients with unilateral SSNHL presenting within 14 days of onset with 50 dBHL or greater pure tone average hearing threshold in the affected ear. INTERVENTION: Either 60 mg/d oral prednisone for 14 days with a 5-day taper (121 patients) or 4 IT doses for 14 days of 40 mg/ml methylprednisolone (129 patients). MAIN OUTCOME MEASURE: Primary end point was change in hearing [dB PTA] at 2 months after treatment. Noninferiority was defined as less than 10 dB difference in hearing outcome between treatments. In this article, pretreatment and posttreatment hearing findings will be reported in detail. RESULTS: A general (and stable) effect of treatment and a specific effect of greater improvement at low frequencies were found in both treatment groups. CONCLUSION: Hearing improvements are stable, and a significantly greater improvement occurs with lower frequency after either oral or IT steroid treatment of SSNHL.

13 Article Balance dysfunction and recovery after surgery for superior canal dehiscence syndrome. 2012

Janky, Kristen L / Zuniga, M Geraldine / Carey, John P / Schubert, Michael. ·Johns Hopkins University, 601 N Caroline Street, Baltimore, MD 21287-0910, USA. ·Arch Otolaryngol Head Neck Surg · Pubmed #22801722.

ABSTRACT: OBJECTIVE: To characterize (1) the impairment and recovery of functional balance and (2) the extent of vestibular dysfunction and physiological compensation following superior canal dehiscence syndrome (SCDS) surgical repair. DESIGN: Prospective study. SETTING: Tertiary referral center. PARTICIPANTS: Thirty patients diagnosed as having SCDS. INTERVENTIONS: Surgical plugging and resurfacing of SCDS. MAIN OUTCOME MEASURES: Balance measures were assessed in 3 separate groups, each with 10 different patients: presurgery, postoperative short-term (<1 week), and postoperative long-term (≥6 weeks). Vestibular compensation and function, including qualitative head impulse tests (HITs) in all canal planes and audiometric measures, were assessed in a subgroup of 10 patients in both the postoperative short-term and long-term phases. RESULTS: Balance measures were significantly impaired immediately but not 6 weeks after SCDS repair. All patients demonstrated deficient vestibulo-ocular reflexes for HITs in the plane of the superior canal following surgical repair. Unexpectedly, spontaneous or post-head-shaking nystagmus beat ipsilesionally in most patients, whereas contrabeating nystagmus was noted only in patients with complete canal paresis (ie, positive HITs in all canal planes). There were no significant deviations in subjective visual vertical following surgical repair (P = .37). The degree of audiometric air-bone gap normalized 6 weeks after surgery. CONCLUSIONS: All patients undergoing SCDS repair should undergo a postoperative fall risk assessment. Nystagmus direction (spontaneous and post-head-shaking) seems to be a good indicator of the degree of peripheral vestibular system involvement and central compensation. These measures correlate well with the HIT.

14 Article Superior canal dehiscence size: multivariate assessment of clinical impact. 2012

Chien, Wade W / Janky, Kristen / Minor, Lloyd B / Carey, John P. ·Department of Otolaryngology-Head and Neck Surgery, Johns Hopkins School of Medicine, Baltimore, Maryland, USA. wchien1@jhmi.edu ·Otol Neurotol · Pubmed #22664896.

ABSTRACT: OBJECTIVE: To examine the association between dehiscence length in patients with superior semicircular canal dehiscence syndrome and their clinical findings, including objective audiometric and vestibular testing results. STUDY DESIGN: Retrospective study. SETTING: Tertiary referral center. PATIENTS: Patients included in this study were diagnosed with superior semicircular canal dehiscence syndrome and underwent surgical repair of the dehiscence through middle fossa craniotomy. The dehiscence length was measured intraoperatively in all cases. MAIN OUTCOME MEASURES: Correlation between dehiscence length with pure-tone average (PTA), average bone-conduction threshold, maximal air-bone gap, cervical vestibular evoked myogenic potential thresholds, and presenting signs and symptoms. RESULTS: The correlation between dehiscence length and maximal air-bone gap was statistically significant on both univariate and multivariate regression analyses. The correlations between dehiscence length and PTA, average bone-conduction threshold, cervical vestibular evoked myogenic potential threshold, and presenting signs and symptoms were not statistically significant. CONCLUSION: The dehiscence length correlated positively with the maximal air-bone gap in patients with superior semicircular canal dehiscence. The correlation was statistically significant. The dehiscence length did not correlate with the other variables examined in this study.

15 Article Second-side surgery in superior canal dehiscence syndrome. 2012

Agrawal, Yuri / Minor, Lloyd B / Schubert, Michael C / Janky, Kristen L / Davalos-Bichara, Marcela / Carey, John P. ·Department of Otolaryngology-Head and Neck Surgery, Johns Hopkins University School of Medicine, Baltimore, Maryland 21287, USA. ·Otol Neurotol · Pubmed #22158019.

ABSTRACT: OBJECTIVE: Bilateral superior canal (SC) dehiscence syndrome poses a challenge because bilateral SC dehiscence (SCD) plugging might be expected to result in oscillopsia and disability. Our aims were as follows: 1) to evaluate which symptoms prompted patients with bilateral SCD syndrome (SCDS) to seek second-side surgery, and 2) to determine the prevalence of disabling imbalance and oscillopsia after bilateral SC plugging. STUDY DESIGN: Prospective observational study. SETTING: Tertiary referral center. PATIENTS: Five patients with bilateral SCDS based on history, audiometric and physiologic testing, and computed tomographic findings. This includes all of our patients who have had second-side plugging surgery to date. INTERVENTION(S): Bilateral sequential middle fossa craniotomy and plugging of SCs. MAIN OUTCOME MEASURE(S): Cochleovestibular symptoms, cervical and ocular vestibular-evoked myogenic potential testing, dizziness handicap inventory, short-form 36 Health Survey, dynamic visual acuity testing. RESULTS: The most common symptoms prompting second-side surgery were sound- and pressure-induced vertigo and autophony. Three of the 5 patients reported that symptoms shifted to the contralateral ear immediately after plugging the first side, whereas in 2 patients, contralateral symptoms developed several years after the first SC plugging. Two of 4 patients experienced ongoing oscillopsia after bilateral SCDS surgery; however, all patients reported relief from their SCD symptoms and were glad that they had pursued bilateral surgery. CONCLUSION: In patients with bilateral SCDS, sound- and pressure-induced vertigo most commonly prompted second-side surgery. Despite some degree of oscillopsia after bilateral SCDS surgery, patients were very satisfied with second-side surgery, given their relief from other SCDS symptoms.

16 Article Association of skin color, race/ethnicity, and hearing loss among adults in the USA. 2012

Lin, Frank R / Maas, Paige / Chien, Wade / Carey, John P / Ferrucci, Luigi / Thorpe, Roland. ·Department of Otolaryngology-Head and Neck Surgery, Johns Hopkins School of Medicine, Baltimore, MD 21287, USA. flin1@jhmi.edu ·J Assoc Res Otolaryngol · Pubmed #22124888.

ABSTRACT: Epidemiologic studies of hearing loss in adults have demonstrated that the odds of hearing loss are substantially lower in black than in white individuals. The basis of this association is unknown. We hypothesized that skin pigmentation as a marker of melanocytic functioning mediates this observed association and that skin pigmentation is associated with hearing loss independent of race/ethnicity. We analyzed cross-sectional data from 1,258 adults (20-59 years) in the 2003-2004 cycle of the National Health and Nutritional Examination Survey who had assessment of Fitzpatrick skin type and pure-tone audiometric testing. Audiometric thresholds in the worse hearing ear were used to calculate speech- (0.5-4 kHz) and high-frequency (3-8 kHz) pure-tone averages (PTA). Regression models were stratified by Fitzpatrick skin type or race/ethnicity to examine the association of each factor with hearing loss independent of the other. Models were adjusted for potential confounders (demographic, medical, and noise exposure covariates). Among all participants, race/ethnicity was associated with hearing thresholds (black participants with the best hearing followed by Hispanics and then white individuals), but these associations were not significant in analyses stratified by skin color. In contrast, in race-stratified analyses, darker-skinned Hispanics had better hearing than lighter-skinned Hispanics by an average of -2.5 dB hearing level (HL; 95% CI, -4.8 to -0.2) and -3.1 dB HL (95% CI, -5.3 to -0.8) for speech and high-frequency PTA, respectively. Associations between skin color and hearing loss were not significant in white and black participants. Our results demonstrate that skin pigmentation is independently associated with hearing loss in Hispanics and suggest that skin pigmentation as a marker of melanocytic functioning may mediate the strong association observed between race/ethnicity and hearing loss.

17 Article Functional variants in NOS1 and NOS2A are not associated with progressive hearing loss in Ménière's disease in a European Caucasian population. 2011

Gazquez, Irene / Lopez-Escamez, Jose A / Moreno, Antonia / Campbell, Colleen A / Meyer, Nicole C / Carey, John P / Minor, Lloyd B / Gantz, Bruce J / Hansen, Marlan R / Della Santina, Charles C / Aran, Ismael / Soto-Varela, Andres / Santos, Sofia / Batuecas, Angel / Perez-Garrigues, Herminio / Lopez-Nevot, Alicia / Smith, Richard J H / Lopez-Nevot, Miguel A. ·Otology and Neurotology Group CTS495, GENYO, Centro de Genómica e Investigación Oncológica-Pfizer, Universidad de Granada, Junta de Andalucía, Granada, Spain. ·DNA Cell Biol · Pubmed #21612410.

ABSTRACT: Hearing loss in Ménière's disease (MD) is associated with loss of spiral ganglion neurons and hair cells. In a guinea pig model of endolymphatic hydrops, nitric oxide synthases (NOS) and oxidative stress mediate loss of spiral ganglion neurons. To test the hypothesis that functional variants of NOS1 and NOS2A are associated with MD, we genotyped three functional variants of NOS1 (rs41279104, rs2682826, and a cytosine-adenosine microsatellite repeat in exon 1f) and the CCTTT repeat in the promoter of NOS2A gene (rs3833912) in two independent MD sets (273 patients in total) and 550 controls. A third cohort of American patients was genotyped as replication cohort for the CCTTT repeat. Neither allele nor genotype frequencies of rs41279104 and rs2682826 were associated with MD, although longer alleles of the cytosine-adenosine microsatellite repeat were marginally significant (corrected p = 0.05) in the Mediterranean cohort but not in a second Galicia cohort. Shorter numbers of the CCTTT repeat in NOS2A were significantly more frequent in Galicia controls (OR = 0.37 [CI, 0.18-0.76], corrected p = 0.04), but this finding could not be replicated in Mediterranean or American case-control populations. Meta-analysis did not support an association between CCTTT repeats and risk for MD. Severe hearing loss (>75 dB) was also not associated with any functional variants studied. Functional variants of NOS1 and NOS2A do not confer susceptibility for MD.

18 Article Oral vs intratympanic corticosteroid therapy for idiopathic sudden sensorineural hearing loss: a randomized trial. 2011

Rauch, Steven D / Halpin, Christopher F / Antonelli, Patrick J / Babu, Seilesh / Carey, John P / Gantz, Bruce J / Goebel, Joel A / Hammerschlag, Paul E / Harris, Jeffrey P / Isaacson, Brandon / Lee, Daniel / Linstrom, Christopher J / Parnes, Lorne S / Shi, Helen / Slattery, William H / Telian, Steven A / Vrabec, Jeffrey T / Reda, Domenic J. ·Department of Otology and Laryngology, Harvard Medical School, Massachusetts Eye and Ear Infirmary, 243 Charles St, Boston, MA 02114, USA. steven_rauch@meei.harvard.edu ·JAMA · Pubmed #21610239.

ABSTRACT: 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.

19 Article Improvement in autophony symptoms after superior canal dehiscence repair. 2010

Crane, Benjamin T / Lin, Frank R / Minor, Lloyd B / Carey, John P. ·Johns Hopkins School of Medicine, Baltimore, Maryland, USA. craneb@gmail.com ·Otol Neurotol · Pubmed #20050268.

ABSTRACT: OBJECTIVE: Autophony, or the unusually loud or disturbing sound of a patient's own voice, can be a prominent and disabling symptom of superior canal dehiscence (SCD) syndrome. The current study measures autophony symptoms before and after SCD plugging to quantify the benefits of surgery. STUDY DESIGN: Patients undergoing SCD plugging between September 2007 and October 2008 completed a questionnaire before and 3 months after surgery. The questionnaire consisted of 26 statements to assess the disability caused by the sound of the patient's own voice. Patients graded each item on a scale from 0 (never) to 4 (almost always) to how often they noted a symptom or experience. Typical statements included "hearing my voice has interfered with my ability to work" and "hearing my voice has caused me to avoid social situations." An autophony index (AI) was generated to grade patient symptoms. SETTING: Tertiary referral center. PATIENTS: Nineteen adults with SCD. INTERVENTION: Superior canal dehiscence plugging via a middle fossa approach. OUTCOME MEASURES: Change in AI. RESULTS: Preoperatively, the mean AI was 42 +/- 27 (mean +/- SD; range, 0-86; 1 patient had no autophony symptoms). Postoperative AI decreased 89% to 9 +/- 22, a significant (p < 0.01) decline. Of the 18 patients with preoperative autophony, 13 had complete postoperative resolution. In 3 remaining patients, the AI decreased but did not resolve. One of these had bilateral SCD with contralateral autophony. One patient's mild autophony remained unchanged, and another patient with coexisting patulous eustachian tube AI increased after SCD plugging. CONCLUSION: In patients with significant autophony symptoms, SCD plugging improved 94% of patients. A simple 5-item AI is provided that will be useful in grading autophony symptoms.