Otology Neurotology 2021-01-01

Book Review

Maxwell, Anne K.

Publication date 01-01-2021


No abstract available

Pubmed PDF Web

Pharmacological Prevention of Noise-induced Hearing Loss: A Systematic Review

Gupta, Avigeet; Koochakzadeh, Sina; Nguyen, Shaun A.; Brennan, Emily A.; Meyer, Ted A.; Lambert, Paul R.

Publication date 01-01-2021


Objective: This study aims to explore and determine the effectiveness of current pharmacologic agents for the prevention of noise-induced hearing loss (NIHL) via a systematic review.
Databases Reviewed: The Pub Med, Scopus, Clinical Trials.gov, and Cochrane Library databases were searched from inception through February 6, 2020.
Methods: Full-text, English-language articles detailing prospective randomized and nonrandomized clinical trials with pharmacological interventions administered to prevent NIHL were included in accordance with PRISMA guidelines. The detailed search terms are included in the Appendix, http://links.lww.com/MAO/B67.
Results: Eleven articles were included in this review with 701 patients receiving a pharmacologic prevention for various noise exposures. Various regimens included administration of alpha-lipoic acid, ambient oxygen, beta-carotene, carbogen, ebselen, Mg-aspartate, N-acetylcysteine, and vitamins C, E, and B12. A number of studies demonstrated statistically significant amelioration of NIHL with pharmacologic intervention. Two studies demonstrated significantly better hearing outcomes for pharmacological prophylaxis with carbogen or ebselen as compared with placebo for the 4 k Hz frequency, where the noise-notch is most likely to be encountered. Given the considerable heterogeneity in agents and methodologies, however, it was not possible to conduct a meta-analysis.
Conclusions: While several heterogenous articles demonstrated promising results for Mg-aspartate, carbogen, vitamin B12, and alpha-lipoic acid, the clinical significance of these pharmaceuticals remains unclear. Initial data from this study alongside future clinical trials might potentially contribute to the generation of clinical practice guidelines to prevent NIHL.
Level of Evidence: 2

Pubmed PDF Web

Decision Making in Patients With Concomitant Otosclerosis and Superior Semicircular Canal Dehiscence: A Systematic Review of the Literature

Fernandez, Ignacio J.; Molinari, Giulia; Presutti, Livio

Publication date 01-01-2021


Objective: Concomitant otosclerosis (OTS) and superior semicircular canal dehiscence (SSCD) is a rare, but difficult-to-identify and treat diagnosis. A systematic review of the literature was performed to analyze the diagnostic and therapeutic approaches of concurrent OTS and SSCD cases and to identify possible factors that may help in predicting the surgical outcome.
Data Sources: Pub Med, Scopus, Medscape, Ovid databases.
Study Selection: Studies showing diagnosis of OTS documented by audiometric test with or without associated radiological signs (computed tomography), and concomitant diagnosis of SSCD, documented at least by high-resolution computed tomography (and possibly supported by neurophysiological testing) were included. Both surgically treated and untreated patients were considered for data analysis.
Data Extraction: The general characteristics of each study were recorded, when available. Clinical, audiological, vestibular testing, surgical, and radiological data were extracted from the published case reports and series, and recorded on a database. For each case, the diagnostic steps that confirmed OTS and concomitant SSCD in the same ear were extracted.
Data Synthesis: Twelve studies were included in the review and 18 cases affected simultaneously by the 2 conditions were extracted and analyzed. Seven of 12 ears (58.3%) undergoing stapes surgery experienced a hearing improvement. In four (33.3%) cases, vestibular symptoms (with or without associated acoustic symptoms) of SSCD were unmasked or worsened by stapes surgery.
Conclusions: A reliable preoperative diagnosis of the two concomitant conditions is possible with a proper interpretation of clinical signs, audiometric, and vestibular testing, in association with the radiologic assessment. Despite that the length and the location of the dehiscence may guide the surgical decision, definitive conclusions regarding the appropriate indications for surgical treatment cannot be drawn due to the limited number of cases with adequate data reported in the literature.

Pubmed PDF Web

Within-Subject Comparisons of the Auditory Brainstem Response and Uncomfortable Loudness Levels in Ears With and Without Tinnitus in Unilateral Tinnitus Subjects With Normal Audiograms

Shim, Hyun Joon; Cho, Yong tae; Oh, Hyeon Sik; An, Yong-Hwi; Kim, Dong Hyun; Kang, Yeon Shin

Publication date 01-01-2021


Objective: To evaluate whether cochlear synaptopathy is a common pathophysiologic cause of tinnitus in individuals with normal audiograms.
Study Design: Prospective study.
Setting: Tertiary referral center.
Methods: We enrolled 27 subjects with unilateral tinnitus and normal symmetric hearing thresholds, and 27 age- and sex-matched control subjects with normal symmetric hearing thresholds. We measured 1) the amplitudes of waves I and V with 90 dB nHL click stimuli in quiet conditions; 2) the latency shift of wave V with 80 dB nHL click stimuli in background noise, varying from 40 dB HL to 70 dB HL; and 3) uncomfortable loudness levels (UCLs) at 500 Hz and 3000 Hz pure tones.
Results: There were no significant differences in the wave V/I amplitude ratio or the latency shift in wave V with increasing noise levels among the tinnitus ears (TEs), nontinnitus ears (NTEs), and control ears. There were no significant differences in UCLs at 500 Hz or 3000 Hz between TEs and NTEs, but the UCLs were lower in TEs (mean 111.3 dB or 104.1 dB) and NTEs (mean 109.4 dB or 100.6 dB) than in control ears (mean 117.9 dB or 114.1 dB, p  < 0.017). No subject met our criteria for cochlear synaptopathy or increased central gain in terms of all three parameters.
Conclusion: Based on these results for UCL, increased central gain is a major mechanism of tinnitus in humans with normal audiograms. However, this compensatory mechanism for reduced auditory input may originate from other pathophysiologic factors rather than from cochlear synaptopathy.

Pubmed PDF Web

Cochlear Dysfunction Is a Frequent Feature of Facioscapulohumeral Muscular Dystrophy Type 1 (FSHD1)

Frezza, Erica; Fuccillo, Emanuela; Petrucci, Antonio; Greco, Giulia; Nucera, Gabriele; Bruno, Ernesto; Giardina, Emiliano; Tupler, Rossella; Di Mauro, Roberta; Di Girolamo, Stefano; Massa, Roberto

Publication date 01-01-2021


Introduction: Facioscapulohumeral muscular dystrophy type 1 (FSHD) represents one of the most common forms of muscular hereditary diseases and it is characterized by a great clinical variability with the typical muscular symptoms and other clinical features, including hearing impairment. However, etiopathogenetic mechanisms of auditory dysfunction are still not completely understood and it has been suggested that it could be assigned to a cochlear alteration that is present even in those subjects with a normal pure tonal audiometry (PTA) examination.
Methods: We found out the cochlear function in 26 patients with molecular diagnosis of FSHD1 and in healthy controls. All patients underwent complete neurological and audiological examinations, including FSHD clinical score, pure-tone audiometry (PTA), and otoacoustic emissions (OAEs), in particular transient evoked otoacoustic emissions (TEOAEs) and distortion product evoked otoacoustic emissions (DPOAEs).
Results: All FSHD1 patients showed significantly reduced DPOAEs and TEOAEs, bilaterally and at all frequencies, even when considering only subjects with a normal PTA or a mild muscular involvement (FSHD score ≤ 2). No correlation between OAEs and FSHD clinical score was found.
Discussion: Cochlear echoes represent a sensitive tool in detecting subclinical cochlear dysfunction in FSHD1 even in subjects with normal hearing and/or subtle muscle involvement. Our study is focused on the importance of evaluating the cochlear alteration through OAEs and, in particular, by performing TEOAEs and DPOAEs sequentially, to evaluate more frequent specificities of cochlear dysfunction with a wider spectrum of analysis.

Pubmed PDF Web

Otosclerosis in an Urban Population

Choi, Jonathan S.; Sweeney, Alex D.; Alava, Ibrahim; Lovin, Benjamin D.; Lindquist, Nathan R.; Appelbaum, Eric N.; Vrabec, Jeffrey T.

Publication date 01-01-2021


Objective: To investigate the evolving prevalence of otosclerosis in a large urban population.
Methods: A retrospective review of patients in a large, urban, public health system was conducted from January 2010 to August 2019 to identify subjects with otosclerosis. Diagnostic testing included audiometry and computed tomography scans. Sex, age at diagnosis, treatment received, race, ethnicity, and country of birth were analyzed for each subject and compared with all eligible patients in the reference population.
Results: A total of 134 patients from a reference population of 672,839 were diagnosed with otosclerosis and analyzed. The otosclerosis patients were predominantly Hispanic (73%), of which the majority were foreign born (87%). The average age at onset was 46 years and 59% were women. The overall prevalence of otosclerosis was 20 of 100,000 patients. The crude prevalence of otosclerosis by ethnicity was 43 of 100,000 for Hispanics, 12.6 of 100,000 for Caucasians, and 3 of 100,000 for African Americans. Within the Hispanic population, the prevalence of otosclerosis was 60 of 100,000 for foreign-born individuals and 16 of 100,000 for those born in the USA (odds ratio OR = 3.69, 95% confidence interval CI, 2.02–6.76, p < 0.0001). Prevalence was not significantly different among Caucasians and US-born Hispanics.
Conclusion: Otosclerosis in the studied population was most common among Hispanic patients, though it was strongly influenced by country of birth. The “imported” otosclerosis cases are best explained by environmental influence rather than ethnic susceptibility. This discrepancy is likely due to variance in measles immunization rates among North and Central American countries before 1990.

Pubmed PDF Web

Characterization of Sensorineural Hearing Loss in Adult Patients With Sickle Cell Disease: A Systematic Review and Meta-analysis

Kapoor, Elina; Strum, David; Shim, Timothy; Kim, Sunny; Sabetrasekh, Parisa; Monfared, Ashkan

Publication date 01-01-2021


Objectives and Main Outcome Measures: To determine the prevalence of sensorineural hearing loss (SNHL) attributable to sickle cell disease (SCD) in the global adult population and to identify factors contributing to its severity.
Study Design: Systematic Review and Meta-analysis.
Methods: We performed a comprehensive literature search for scientific articles in Pub Med, Scopus, CINAHL, Web of Science, and the Cochrane Library that reported the incidence of hearing loss in populations over 18 years of age with SCD.
Results: We identified 138 studies from the initial search, 12 of which met inclusion criteria and were utilized for data analysis. A total of 636 SCD patients and 360 controls were included in the Cochrane Review Manager 5.3 meta-analysis. There was a statistically significant increase in the prevalence of SNHL in adults with SCD compared with the general population with a cumulative risk ratio (RR) of 6.03.
Conclusions: This is the first systematic investigation of the relationship between SCD and SNHL in adult patients across the globe. SNHL is more prevalent in patients with SCD, specifically those of the HbSS genotype, than the general population likely due to the pathophysiology of the disease and its effects on labyrinthine microvasculature. The increased prevalence of SNHL in the adult SCD population warrants future research into the predictors of SNHL severity and merits routine audiometric monitoring of adult SCD patients.

Pubmed PDF Web

Bilateral Sudden Sensorineural Hearing Loss and Intralabyrinthine Hemorrhage in a Patient With COVID-19

Chern, Alexander; Famuyide, Akinrinola O.; Moonis, Gul; Lalwani, Anil K.

Publication date 01-01-2021


Objective: To describe a case of bilateral sudden sensorineural hearing loss (SSNHL) and intralabyrinthine hemorrhage in a patient with COVID-19.
Study Design: Clinical capsule report.
Setting: Tertiary academic referral center.
Patient: An adult woman with bilateral SSNHL, aural fullness, and vertigo with documented SARS-CoV-2 infection (IgG serology testing).
Interventions: High-dose oral prednisone with taper, intratympanic dexamethasone.
Main Outcome Measures: Audiometric testing, MRI of the internal auditory canal with and without contrast.
Results: A patient presented with bilateral SSNHL, bilateral aural fullness, and vertigo. Serology testing performed several weeks after onset of symptoms was positive for IgG COVID-19 antibodies. MRI showed bilateral intralabyrinthine hemorrhage (left worse than right) and no tumor. The patient was treated with two courses of high-dose oral prednisone with taper and a left intratympanic dexamethasone injection, resulting in near-resolution of vestibular symptoms, a fluctuating sensorineural hearing loss in the right ear, and a severe to profound mixed hearing loss in the left ear.
Conclusions: COVID-19 may have otologic manifestations including sudden SSNHL, aural fullness, vertigo, and intralabyrinthine hemorrhage.

Pubmed PDF Web

"Association of Subjective Hearing Loss and Apolipoprotein E ε4 Allele on Alzheimers Disease Neurodegeneration"

Neff, Ryan M.; Jicha, Gregory; Hawk, Gregory S.; Bush, Matthew L.; McNulty, Beth

Publication date 01-01-2021


Objective: Hearing loss (HL) and apolipoprotein E ε4 (ApoE4) allele are both dementia risk factors. No research has investigated the association of these variables regarding dementia, specifically Alzheimers disease. Our goal was to evaluate HL and ApoE4 allele positivity toward degree of Alzheimers neurodegeneration.
Study Design: Retrospective.
Setting: Academic.
Patients: Alzheimers neuropathology obtained from brain tissue databank. Documented demographics, subjective hearing status, cognition, and ApoE4. Subjects divided into four groups based on hearing status and ApoE4 positivity.
Main Outcome Measures: Differences in cognition (clinical dementia rating, mini mental state examination (MMSE), geriatric depression score) and Alzheimers neuropathology staging (Braak, CERAD) between groups.
Results: Two-hundred and fifty-nine subjects. No significant difference between groups, with regard to hearing status or ApoE4 positivity, in premorbid cognition, including scores for clinical dementia rating and MMSE (p = 0.2332). HL subjects had less severe neuropathology, as compared with normal hearing subjects. For example, high grade Braak stage was present in 27.1 and 51.0% of HL and normal hearing subjects, respectively (p = 0.0263). This finding was in context of equivocal clinical cognition between groups. ApoE4+ individuals had more severe neurodegeneration; for example, 65.7 and 33.5% with high grade Braak stage for ApoE4+ and ApoE4– subjects, respectively (p < 0.0001).
Conclusion: Subjective HL subjects had less severe neuropathology with no difference in cognition, suggesting an additive effect of HL to cognitive burden of Alzheimers neuropathology. HL appeared to increase cognitive burden, but wasn’t manifested by greater neurodegeneration. This is clinically relevant in that treating HL could slow Alzheimers disease progression.

Pubmed PDF Web

Audiologic Outcome of Cochlear Implantation in Children With Cochlear Nerve Deficiency

Yousef, Medhat; Mesallam, Tamer A.; Garadat, Soha N.; Almasaad, Ayna; Alzhrani, Farid; Alsanosi, Abdulrahman; Hagr, Abdulrahman

Publication date 01-01-2021


Objective/Hypothesis: The aim of this study was to investigate cochlear implantation (CI) outcome in children with nerve deficiency.
Study Design: Retrospective chart review.
Methods: A total of seven children with prelingual profound deficiency (hypoplasia or aplasia) were included. A control group of 10 CI children with no cochlear nerve anomalies was also included. In addition to implant stimulation levels, childrens performance on pure-tone audiometry, speech reception measure, and auditory and speech skills ratings were compared across groups. Additionally, pre- and postoperative audiologic results were evaluated for the group with nerve deficiency.
Results: In general, children with nerve deficiency performed poorer than those without nerve deficiency on all tested measures. Stimulation levels were considerably higher and more variable than the control group. Results further showed that performance was dependent on the diameter of the internal auditory canal.
Conclusion: Overall, cochlear implantation outcome in children with auditory nerve deficiency is poorer and extremely more variable than those without nerve deficiency. However, three of the patients had a noticeable improvement in auditory performance postimplantation suggesting that CI is a viable option in this population but expected benefit can be dependent on the status of the cochlear nerve.

Pubmed PDF Web

Real-World Compliance With Follow-up in 2,554 Cochlear Implant Recipients: An Analysis of the HERMES Database

Shapiro, Scott B.; Lipschitz, Noga; Kemper, Nathan; Abdelrehim, Lamiaa; Hammer, Theresa; Wenstrup, Lisa; Breen, Joseph T.; Grisel, Jedidiah J.; Samy, Ravi N.

Publication date 01-01-2021


Background: Traditional paradigms of care recommend close audiology follow-up and regular speech perception outcomes assessment indefinitely for cochlear implant (CI) recipients after device activation. However, there is scant published data on actual compliance with this paradigm in clinical practice.
Methods: A multi-center cochlear implant database was queried to identify follow-up rates after cochlear implantation. Follow-up rates where speech perception outcomes assessment occurred at 1-, 3-, 6-, 12-, 18-, and 24-months post-activation were determined by tabulating observed follow-up divided by expected follow-up (O/E ratio) expressed as a percentage. To determine all-cause audiology follow-up rates (with or without testing speech perception outcomes assessment), the database patients from two participating centers (one private practice and one academic center) were similarly analyzed using electronic health record (EHR) data to calculate O/E rates where audiology follow-up occurred for any reason.
Results: O/E follow-up rates where speech perception outcomes assessment occurred was 42, 40, 31, 29, 5, and 22% for 1-, 3-, 6-, 12-, 18-, and 24-months post-activation, respectively (n = 2,554). All-cause audiology follow-up rates (with or without speech perception outcomes assessment) using EHR-confirmed data from two individual centers were 97, 94, 81, 66, 41, and 35% at 1-, 3-, 6-, 12-, 18-, and 24-months post-activation visits, respectively (n = 118).
Conclusions: Compliance with audiology follow-up and speech perception outcomes assessment is generally low and decreases significantly as time post-activation increases. Future paradigms of care for CI should be designed recognizing the significant attrition that occurs with CI follow-up.

Pubmed PDF Web

Cochlear Implants and Magnetic Resonance Imaging: Experience With Over 100 Studies Performed With Magnets in Place

Fussell, Wanda L.; Patel, Neil S.; Carlson, Matthew L.; Neff, Brian A.; Watson, Robert E.; Lane, John I.; Driscoll, Colin L. W.

Publication date 01-01-2021


Objective: To evaluate adverse events and feasibility of performing 1.5-T MRI in patients with cochlear implants (CI) and auditory brainstem implants (ABI).
Setting: Single tertiary academic referral center.
Patients: CI and ABI recipients undergoing 1.5-T MRI without internal magnet removal.
Intervention(s): MRI after tight headwrap application.
Main Outcome Measures: Adverse events, patient tolerance.
Results: A total of 131 MR studies in 79 patients were performed, with a total of 157 study ears. Sixty-one patients (77%) had unilateral devices. Four patients (5%) underwent MRI with ABI magnets in place. Sixteen patients (20%) had MRI-compatible devices that did not require a head wrap. There were no instances of device stimulation, device malfunction, or excessive heating of the receiver-stimulator package. Magnet tilt requiring manual repositioning occurred during seven studies (4.5%) and magnet displacement requiring operative intervention occurred during seven studies (4.5%). Significant pain where imaging had to be discontinued occurred during three episodes (2%). No adverse events were noted among patients who underwent MRI with an MRI-compatible magnet.
Conclusions: MRI with CI or ABI magnets in place is associated with a low prevalence of adverse events when performed in a controlled setting. Many partial magnet displacements can be corrected with firm manual pressure. Devices with magnets that align with the field within their housing were not associated with any adverse events and do not require immobilization of the magnet during the scan. These may be valuable in patients with known or anticipated need for MRI.

Pubmed PDF Web

Investigating the Electrical Properties of Different Cochlear Implants

Swaddiwudhipong, Nol; Jiang, Chen; Landry, Thomas G.; Bance, Manohar

Publication date 01-01-2021


Aim: This study characterises and compares electrical properties and current spread across four different makes of cochlear implants with differing electrode designs using a 3D-printed artificial cochlear model.
Background: Cochlear implants are currently limited by current spread within the cochlea, which causes low spectral resolution of auditory nerve stimulation. Different cochlear implant makes vary in electrode size, shape, number, and configuration. How these differences affect cochlear implant current spread and function is not well known.
Method: Each cochlear implant was inserted into a linear cochlear model containing recording electrodes along its length. Biphasic monopolar stimulation of each implant electrode was carried out, and the resultant waveform and transimpedance matrix (TIM) data obtained from the recording electrodes. This was repeated with each implant rotated 180 degrees in the cochlea model to examine the effects of electrode orientation. Impedance spectroscopy was also carried out at the apex, middle, and base of the model.
Results: The four cochlear implants displayed similar TIM profiles and waveforms. One hundred eighty degrees rotation of each cochlear implant made little difference to the TIM profiles. Impedance spectroscopy demonstrated broad similarities in amplitude and phase across the implants, but exhibited differences in certain electrical parameters.
Conclusion: Implants with different designs demonstrate similar electrical performance, regardless of electrode size and spacing or electrode array dimension. In addition, rotatory maneuvers during cochlear implantation surgery are unlikely to change implant impedance properties.

Pubmed PDF Web

Comparison of Speech Recognition and Localization Ability in Single-sided Deaf Patients Implanted With Different Cochlear Implant Electrode Array Designs

Speck, Iva; Ketterer, Manuel Christoph; Arndt, Susan; Aschendorff, Antje; Jakob, Till F.; Hassepass, Frederike

Publication date 01-01-2021


Objective: Choice of electrode array (EA) design and differences in outcome are major concerns both to patients with single-sided deafness (SSD) and to surgeons before cochlear implant (CI) surgery. The present work investigates the effects of EA design on 1) insertion depths, and 2) audiological outcomes of SSD CI recipients.
Study Design: Retrospective study.
Setting: Tertiary academic center.
Patients: Forty patients with acquired SSD matched according to duration of deafness Main Outcome Measures: Fourteen CI recipients were implanted with a perimodiolar electrode (cochlear perimodiolar CPM), 12 with a shorter lateral wall electrode (cochlear lateral wall CLW), and 14 with a longer lateral wall electrode array (med El lateral wall MLW). Postoperative rotational tomography was evaluated to determine cochlear size and EA angle of insertion depth (AID). Binaural speech comprehension in noise (in three configuration presentations) and localization ability were assessed 12 months postoperatively with CI.
Results: AID was significantly deeper in MLW (mean 527.94 degrees) compared with the CPM (mean 366.35 degrees) and CLW groups (mean 367.01 degrees). No significant difference in AID was seen between the CPM and CLW groups (difference 0.66 degrees). Cochlear sizes revealed no significant differences between any groups. All three groups showed significant improvement in head shadow effect (difference on average CPM: 6.3 dB SPL, CLW 5 dB SPL, and MLW 4.05 dB SPL) and localization ability at 12 months postoperatively (difference on average CPM: 19.72 degrees, CLW: 24 degrees, and MLW: 12.9 degrees). No significant difference in the extent of audiological benefit was observed between any groups.
Conclusion: No effect on binaural benefit was apparent from the selection of the three EA designs in SSD CI recipients. Further studies focusing on subjective results, sound quality, and music perception depending on EA design in SSD CI recipients are needed.

Pubmed PDF Web

Cost-effectiveness of Pediatric Unilateral/Bilateral Cochlear Implant in a Developing Country

Swami, Himanshu; AP, Arjun; Shivanand, Shyamal

Publication date 01-01-2021


Objective: To determine the cost-effectiveness of unilateral and bilateral cochlear implantation (CI) for children suffering from bilateral profound SNHL.
Study Design: This was a cost benefit analysis study where quality-adjusted life years (QALY) and the expenditure involved post unilateral and bilateral CI were calculated to assess the cost per QALY gained.
Setting: The study was conducted in a tertiary-care hospital in a city of a developing country.
Patients: A total of 59 patients were studied during the years 2015 to 2019 of which a study cohort of 29 patients underwent bilateral cochlear implantation and a control cohort of 30 patients received U/L CI but did not undergo B/L CI.
Main Outcome: The QALY parameters HUI3 and VAS when patient had no implant, post unilateral and bilateral CI were calculated along with the total lifelong expenditure involved. The outcomes improvement post CI was compared in the study and control groups. The discounted net cost per QALY gained was assessed and compared with the PCGDP of the country for cost effectiveness.
Results: Significant QALY was gained post unilateral and bilateral CI and the expenditure for both was within the cost thresholds. The net costs per QALY gained post the discounting in unilateral and bilateral CI were found to be 1,345 US$ and 1,977 US$ respectively. The PCGDP (nominal) of India in 2018 was 2,016 US$. The cost involved per QALY gained was found to be lesser than the per capita GDP of India.
Conclusion: Unilateral CI was found to be highly cost effective with bilateral CI involving a little higher cost per QALY gained but well within the cost effectiveness threshold.

Pubmed PDF Web

Mastoid Obliteration Using Autologous Bone Dust Following Canal Wall Down Mastoidectomy

Sioshansi, Pedrom C.; Alyono, Jennifer C.; Blevins, Nikolas H.

Publication date 01-01-2021


Objective: To describe a technique for mastoid obliteration following canal wall down (CWD) mastoidectomy for chronic otitis media with cholesteatoma, and review its early results in producing a dry, safe ear, and a small mastoid cavity.
Study Design: Retrospective review.
Setting: Tertiary referral center.
Patients: Forty-three consecutive CWD procedures using bone dust obliteration for chronic otitis media.
Intervention: All patients underwent CWD mastoidectomy and, if indicated, concurrent tympanoplasty and ossicular chain reconstruction. Bone dust harvested from healthy mastoid cortex was used to obliterate selected portions of the tympanomastoid defect. Temporalis fascia and/or an inferiorly-based periosteal flap were used for coverage of the bone dust.
Main Outcome Measures: Postoperative infection, need for mastoid bowl cleaning, incidence of recurrent cholesteatoma, need for revision surgical intervention.
Results: At mean follow-up of 29 months, 95% of ears have remained dry and safe since mastoid obliteration, with a lack of symptoms and no evidence of recurrent disease. Cholesteatoma recurrence rate was 5%. Postoperative otorrhea, while rare, was managed successfully with topical medication in all affected patients. Clinical, radiographic, and surgical appearance of grafted bone dust suggests good take with long-term viability.
Conclusions: The described technique used for mastoid obliteration using autologous bone dust and cartilage is simple, effective, and safe to reduce the size of the mastoid cavity in patients undergoing CWD mastoidectomy. It might help to reduce morbidity by improving the surgeons control over mastoid bowl size and shape.

Pubmed PDF Web

Comparison of Ossiculoplasty Outcomes Using Different Materials in the Treatment of Chronic Otitis Media

Jung, Da Jung; Yoo, Myung Hoon; Lee, Kyu-Yup

Publication date 01-01-2021


Objective: This study aimed to evaluate success rates after ossicular chain reconstruction using different materials.
Study Design: Retrospective cohort study.
Setting: Tertiary referral center.
Patients: Four hundred forty-three participants who underwent ossiculoplasty at a tertiary medical center were included.
Intervention: Ossicular chain reconstruction using five materials: autologous malleus, incus, and cortical bone, as well as Hydroxyapatite (HA) and titanium.
Main Outcome Measures: Hearing data were measured 1 day preoperatively and 6 months postoperatively.
Successful hearing outcomes were defined by the fulfillment of more than one of the following criteria: postoperative air–bone gap of 20 dB or less, hearing air conduction (AC) gain of 15 dB or more, or postoperative AC hearing less than 30 dB.
Results: Preoperative median AC values (95% confidence interval) among participants with malleus, incus, cortical bone, HA, and titanium transplants or prostheses were 50 (39.6–54.6) dB, 51.3 (48.1–51.8) dB, 50 (45.2–52.2) dB, 56.3 (50.9–57.6) dB, and 54.3 (48.5–56.0) dB, respectively (p = 0.092). The success rates in malleus, incus, cortical bone, HA, and titanium were 53.3%, 60.3%, 51.7%, 61.6%, and 69.7%, respectively. Titanium had the highest success rate among the five materials, but the differences between the materials were not statistically significant (p = 0.283). Titanium had highest success rate among the participants with erosive stapes suprastructure or obstructed Eustachian tubes (p = 0.042 for erosive stapes suprastructure and p = 0.010 for obstructed Eustachian tubes).
Conclusion: Our study demonstrated that titanium prostheses would be a good alternative for ossiculoplasty in cases wherein autologous material is unavailable, especially in association with unfavorable conditions, such as with the presence of cholesteatoma, erosive stapes suprastructure, edematous middle ear mucosa, and obstructed Eustachian tube.

Pubmed PDF Web

Prevalence, Surgical Management, and Audiologic Impact of Sigmoid Sinus Dehiscence Causing Pulsatile Tinnitus

Ettyreddy, Abhinav R.; Shew, Matthew A.; Durakovic, Nedim; Chole, Richard A.; Herzog, Jacques; Buchman, Craig A.; Wick, Cameron C.

Publication date 01-01-2021


Objective: To evaluate the prevalence, surgical management, and audiologic impact of pulsatile tinnitus caused by sigmoid sinus dehiscence.
Study Design and Setting: Retrospective chart review at a tertiary care hospital.
Patients: Adults with unilateral pulsatile tinnitus attributable to sigmoid sinus dehiscence who underwent resurfacing between January 2010 and January 2020.
Interventions: Transmastoid sigmoid resurfacing.
Main Outcome Measures: Resolution of pulsatile tinnitus; audiologic outcomes; complications; tinnitus etiologies.
Results: Nineteen patients (89.4% women) had surgery for suspected sigmoid sinus dehiscence. The mean dehiscence size was 6.1 mm (range, 1–10.7 mm). Eight patients had concurrent sigmoid sinus diverticulum and one patient also had jugular bulb dehiscence. Only two patients (10.5%) had the defect identified by radiology. Low-frequency pure-tone average, measured at frequencies of 250 and 500 Hz, showed a significant median improvement of 8.8 dB following resurfacing (18.8 dB versus 10.0 dB, p = 0.02). The majority of patients had complete resolution of pulsatile tinnitus (16/19, 84.2%). Of those without complete resolution, two patients had partial response and one patient had no improvement. There were no significant complications. Of 41 consecutively tracked patients with a pulsatile tinnitus chief complaint, sigmoid pathology represented 32% of cases.
Conclusions: Sigmoid sinus dehiscence represents a common vascular cause of pulsatile tinnitus that, if properly assessed, may be amenable to surgical intervention. Sigmoid sinus resurfacing is safe, does not require decompression, and may improve low-frequency hearing. Radiographic findings of dehiscence are often overlooked without a high index of clinical suspicion. Its relationship with transverse sinus pathology and idiopathic intracranial hypertension remain unclear.

Pubmed PDF Web

A Study of Correlation Between Tympanic Membrane Perforation Size With Hearing Loss in Patients With Inactive Mucosal Chronic Otitis Media

Kolluru, Kartik; Kumar, Sunil; Upadhyay, Prabhakar

Publication date 01-01-2021


Purpose: This study is directed towards establishing a correlation between the objective measurement of tympanic membrane (TM) perforation size with hearing loss in patients with inactive mucosal chronic otitis media (COM), and to compare the otoscopic findings of the TM perforation vis-a-vis the results obtained by image analysis of the endoscopic image of the perforation.
Materials and methods: An observational, prospective and hospital based study was performed on 107 ears (78 patients) with inactive mucosal chronic otitis media. Objective measurement of the TM perforation size was done by capturing the images of the perforation on ImageJ analysis software. A correlation was established between the hearing loss and the perforation with respect to its size and location. The accuracy of the otoscopic finding of the perforation was established with respect to the endoscopic image of the perforation.
Results: The extent of the hearing loss was seen to increase with increasing size of the TM perforation (r = 0.435, p < 0.0001). Posterior perforations had a 12% greater hearing loss than anterior perforations but this was significant only at the frequency of 500 Hz (p = 0.02). There was a positive correlation between the otoscopic estimation of the size of the perforation with the endoscopically obtained image.
Conclusion: Hearing loss in chronic otitis media is directly proportional to the size of the TM perforation and posteriorly based perforations have worse audiometric thresholds at lower frequencies (500 Hz).

Pubmed PDF Web

Multinational Appraisal of the Chronic Otitis Media Questionnaire 12 (COMQ-12)

Phillips, John S.; Yung, Matthew W.; Nunney, Ian; Doruk, Can; Kara, Hakan; Kong, Taehoon; Quaranta, Nicola; Peñaranda, Augusto; Bernardeschi, Danièle; Dai, Chunfu; Kania, Romain; Denoyelle, Françoise; Tono, Tetsuya

Publication date 01-01-2021


Objectives: The Chronic Otitis Media Questionnaire-12 (COMQ-12) assesses patient-reported health-related quality of life. A multinational collaborative project was performed to translate and appraise the psychometric properties of the COMQ-12 across Europe, Asia, and South America.
Methods: Eight otology units from seven countries (China, Colombia, France, Italy, Japan, Korea, Turkey) created native versions of the COMQ-12 by the process of translation and back-translation. Questionnaire reliability was assessed on the basis of internal consistency by calculating Cronbachs coefficient alpha. Exploratory factor analysis was performed to identify underlying correlations between individual questionnaire items.
Results: This study included 478 participants from 8 countries. Calculated values for Cronbachs coefficient alpha were between 0.71 and 0.90. Exploratory factor analysis allowed the identification of three dominant factors, the primary factor (related to hearing problems) explaining 42% of the total variance, the secondary factor (related to daily activities) explaining 30% of the variance, and the third factor (related to acute disease activity) explaining 28% of the variance.
Conclusions: This is a large study of patients with chronic otitis media, from centers from within many different countries spanning Europe, Asia, and South America. This study supports the use of the COMQ-12 within the individual countries where it was tested.

Pubmed PDF Web

The Feasibility of Magnetic Resonance Imaging Without General Anesthesia Using the “Bundle and Scan” Technique for Infants With Sensorineural Hearing Loss

Grose, Elysia; Pigeon, Marie; Abdeen, Nishard; Belanger, Melissa; Schramm, David; Vaccani, Jean-Philippe

Publication date 01-01-2021


Objective: The purpose of this study is to determine the feasibility of magnetic resonance imaging (MRI) without general anesthesia (GA) for infants being evaluated for sensorineural hearing loss (SNHL) using the bundle and scan technique.
Study Design: Retrospective study.
Setting: Pediatric tertiary care hospital.
Patients: All infants who underwent MRI using the bundle and scan technique as part of the diagnostic workup for unilateral or bilateral SNHL between June 2016 to April 2019 were included.
Main Outcome Measure: The primary outcome was the proportion of clinically useful images produced.
Results: We reviewed 21 bundle and scan MRI examinations in infants being evaluated for SNHL. Patients had a median age of 10 (range: 6–25) weeks at the time of MRI. Motion artifact was noted in 38% (8/21) of cases. Eighty-six percent (18/21) of the magnetic resonance images produced using the bundle and scan technique were of diagnostic quality and/or sufficient for surgical planning for cochlear implantation. Repeat imaging with GA was required for three cases (14%) as the initial images were not clinically useful. All patients requiring GA had unilateral SNHL. All patients with bilateral SNHL successfully underwent MRI without GA using the bundle and scan technique.
Conclusion: The results of our study demonstrate that it is feasible to perform MRI using the bundle and scan technique in the majority of young infants being evaluated for SNHL. This has the potential to help determine cochlear implant candidacy earlier, reduce exposure to GA, and reduce healthcare costs.

Pubmed PDF Web

Hearing Loss in Enlarged Vestibular Aqueduct: A Prognostic Factor Systematic Review of the Literature

Saeed, Haroon Shakeel; Kenth, Jonny; Black, Graeme; Saeed, Shakeel R.; Stivaros, Stavros; Bruce, Iain A.

Publication date 01-01-2021


Objective: There is a need to highlight individual prognostic factors determining hearing loss in enlarged (wide) vestibular aqueduct, as currently clinicians cannot counsel parents about the expected clinical course, nor provide individualized hearing rehabilitation plans following identification at newborn screening. We apply a novel methodology to specifically outline and assess the accuracy of prognostic factors reporting for hearing loss in enlarged vestibular aqueduct.
Data Sources: A preferred reporting items for systematic reviews and meta-analyses compliant systematic review (Prospero ID: CRD42019151199), with searches applied to Medline, EMBASE, and Cochrane. Studies with longitudinal design were included between 1995 and 2019.
Study Selection: The CHARMS-PF tool was used to assess robustness of prognostic factor study designs.
Data Extraction: The QUIPS tool was used to assess for individual study risk of bias.
Data Synthesis & Results: Seventy papers were suitable for data extraction. In the six studies with low risk of bias, the domains of enlarged vestibular aqueduct (EVA) morphology, age, hearing thresholds, sex, head trauma, and genotype provided exploratory prognostic factors for hearing loss associated with enlarged vestibular aqueduct. Overall, study heterogeneity and risk of bias precluded reporting by forest plots and meta-analysis.
Conclusions: The majority of exploratory prognostic factor studies for hearing loss associated with enlarged vestibular aqueduct are hampered by risk of bias. However, this systematic review identifies potential independent prognostic factors which should be measured, and adjusted for, in subsequent confirmatory studies utilizing multivariate analysis. This would determine the true independent prognostic effects associated with hearing loss in enlarged vestibular aqueduct, while facilitating prognostic model development and the ability to predict individual hearing loss trajectory.

Pubmed PDF Web

Systematic Review of Endoscopic Ear Surgery Outcomes for Pediatric Cholesteatoma

Basonbul, Razan A.; Ronner, Evette A.; Kozin, Elliott D.; Lee, Daniel J.; Cohen, Michael S.

Publication date 01-01-2021


Objective: Endoscopic ear surgery (EES) has been increasingly adopted to enhance visualization during otologic surgery including pediatric cholesteatoma resection. The purpose of this study is to systematically review the current literature reporting rates of residual cholesteatoma to evaluate outcomes of EES in the pediatric population.
Data Sources: Pub Med, Embase, Cochrane.
Study Selection: Using the Preferred Reporting Items for Systematic Reviews and Meta-Analysis recommendations (PRISMA), we systematically reviewed studies that reported outcomes for cholesteatoma surgery in children using the endoscope. Studies were included if they described the use of the endoscope for middle ear surgery and measured outcomes following cholesteatoma resection in pediatric patients.
Data Extraction: Identified articles were assessed for study design, sample size, inclusion of pediatric population, the presence of a comparison group, outcomes measured such as: residual disease rate, recurrent cholesteatoma, audiologic outcome, quality of life measures, and complications.
Data Synthesis: Fourteen articles in the literature discussed cholesteatoma outcomes in children when the endoscope was employed. Residual disease rate ranged from 0 to 22% when endoscope was used for dissection compared with 11 to 55% when endoscope was used for inspection or not at all. A meta-analysis was conducted on 11 studies. There was a significant risk reduction in residual disease rates for endoscopic dissection when compared with microscope (RR = 0.48, 95% CI = 0.37–0.63, p < 0.001).
Conclusion: Among the studies included in this review, EES was associated with lower rates of residual cholesteatoma compared with traditional microscopic techniques. Additional prospective studies are needed to further assess these findings.

Pubmed PDF Web

The Natural Course of Tympanic Membrane Retractions in the Posterosuperior Quadrant of Pars Tensa: A Watchful Waiting Policy

Bayoumy, Ahmed B.; Veugen, Christianne C.A.F.M.; Rijssen, L. Bengt; Yung, Matthew; Bok, Jan-Willem M.

Publication date 01-01-2021


Introduction: Tympanic membrane retraction (TMR) is a relatively common otologic finding. Currently, there is no consensus on the optimal treatment of TMR. Some ENT-surgeons advocate surgical correction while others opt for a watchful-waiting policy. Our aim was to investigate the natural course of retraction pockets in the posterosuperior quadrant of the pars tensa in a large cohort of patients.
Methods: An observational retrospective cohort study was conducted including patients of all ages with a posterosuperior pars tensa retraction. Primary outcome measure was difference between audiometry at first and last visits. Secondary outcomes were patients’ complaints, otoscopic outcomes (Sade classification), and complications (perforation, ossicular chain damage, and/or cholesteatoma).
Results: A total of 71 patients with 81 ears and a median age of 23 years (IQR 14–47) were included. The median duration of follow-up was 64 months (IQR 44–102). The mean air-bone gap at first and last visits was 17.9 dB (SD 11.3) and 15.5 dB (SD 12.9), respectively, with a mean improvement of 2.4 dB (p = 0.08). In 10 ears (12%) the hearing level (air-bone gap) deteriorated with 10 dB or more. Patients who presented with a TMR Sade grade I at first visit had significantly better audiometric outcomes than patients presenting with Sade grade III (p = 0.001). Progression to cholesteatoma occurred in one patient (1%), progression to perforation occurred in five patients (6%), and progression to ossicular chain damage occurred in five patients (6%).
Conclusions: Otoscopic findings and audiometric results remained stable in most TMRs without treatment. Additionally, audiometry did not worsen during last follow-up. Progression to cholesteatoma, perforation, or ossicular chain damage was rare. Shared decision making regarding TMRs should include a discussion of a wait-and-see policy.

Pubmed PDF Web

“Mushroom” Autologous Partial Ossicular Replacement Prosthesis: Long-term Results

Malhotra, Manu; Priya, Madhu; Bhardwaj, Abhishek; Tyagi, Amit Kumar; Kumar, Amit; Varshney, Saurabh; Malhotra, Rashmi

Publication date 01-01-2021


Objective: Evaluate a new autologous mushroom-shaped cortical bone partial ossicular replacement prosthesis (MPORP) for cost-effective and sustainable hearing results.
Study Design: Prospective study.
Setting: Tertiary care center.
Patients: Forty-two patients suffering from chronic otitis media with intact superstructure of the stapes and partially or completely eroded incus.
Interventions: Group-1 (n = 24) underwent only tympanoplasty with MPORP; group-2 (n = 18) underwent intact canal wall mastoidectomy (ICW) with MPORP.
Main Outcome Measures: Hearing results were evaluated using a four frequency average (measured at 0.5, 1, 2, 3 k Hz) pure tone air conduction (PTA), air-bone gap (ABG), and word recognition scores (WRS) after 3, 6, and 12 months and compared with preoperative results.
Results: Overall, successful rehabilitation of ABG to 20 dB or less was achieved in 92% of patients. Mean postoperative ABG was 15.35 ± 4.18 dB showing mean improvement of 23.89 ± 5.95 dB. In group-1, mean postoperative ABG was 18.47 ± 3.65 dB, showing an improvement of 25.92 ± 5.3 dB. In group-2, mean postoperative ABG was 18.47 ± 3.65 dB showing an improvement of 20.14 ± 4.96 dB. Hearing improvement in all the cases together and both the groups checked separately was statistically significant (paired t test, p  0.05).
Conclusion: The MPORP is obtainable from the local site, easily constructed, bio-compatible, cost-effective, less bulky, adequately rigid for sound transmission, magnetic resonance imaging (MRI) compatible, and provides sustainable hearing gain because it has better chances of integration with the head of stapes.

Pubmed PDF Web

"Triple Canal Occlusion for the Treatment of Intractable Menières Disease"

Gill, Charn; Muzaffar, Jameel; Kumar, Raghu; Irving, Richard

Publication date 01-01-2021


Objective: Menières disease can be a debilitating condition but in most cases the symptoms are controlled by lifestyle changes and medical management. However, some patients remain symptomatic despite medical treatment and have the option of more invasive surgical treatments. Surgical intervention for Menières includes a range of interventions from grommet insertion, intratympanic steroids/Gentamicin, endolymphatic sac decompression, labyrinthectomy, and vestibular neurectomy. A recently described technique involves the occlusion of all three semi-circular canals as an alternative in intractable Menières disease.
Study Design: This is a case series of three patients who underwent triple canal occlusion for the treatment of intractable Menières disease.
Setting: Patients were selected from those who were referred to Queen Elizabeth Hospital in Birmingham, a tertiary referral center.
Patients: Patients who were severely symptomatic despite medical treatment who were considering ablative therapy were offered the option of triple canal occlusion as an alternative.
Intervention: We report a series of Menières patients treated by triple canal occlusion, describe the rationale behind this intervention, the surgical technique, and preliminary results.
Main Outcome Measure: Each patient was followed up for a minimum of 2 years following the procedure. The main outcomes measures were the class of vertigo control and hearing threshold levels according to the American Academy of Otolaryngology–Head and Neck Surgery guidelines.
Results: Of the three patients, two were men and one was woman, the age range was 45 years to 61 years old. Two patients with unilateral disease achieved class A control whereas one patient with bilateral disease achieved class B control. Two patients who underwent the procedure had little or no effect to their hearing on the treated side however one patient suffered a 30 dB hearing loss on the operative side.
Conclusions: Based on our limited experience and the early reports in the literature we consider that there are potential patient benefits for triple canal occlusion for intractable Menières disease as an alternative to vestibular neurectomy due to the reduced morbidity and long-term efficacy.

Pubmed PDF Web

Single Cycle Versus Multiple Cycles of Canalith Repositioning Procedure for Treatment of Posterior Canal Benign Paroxysmal Positional Vertigo: A Randomized Controlled Trial

Isaradisaikul, Suwicha Kaewsiri; Chowsilpa, Sanathorn; Hanprasertpong, Charuk; Rithirangsriroj, Tayaporn

Publication date 01-01-2021


Objective: To compare the treatment outcomes and complications of single cycle versus multiple cycles of the canalith repositioning procedure (CRP).
Study Design: Randomized controlled trial.
Setting: Academic tertiary referral center; Chiang Mai University, Thailand.
Patients: One hundred and forty-three adults who had unilateral posterior canal benign paroxysmal positional vertigo (BPPV).
Intervention: Therapeutic. Patients received either a single cycle or multiple cycles of the CRP in one session.
Main Outcome Measures: Rate of negative Dix-Hallpike test (DHT), rate of symptom improvement, dizziness handicap inventory score (DHI), and rate of complications.
Results: At the 1st week and the 4th week recall visits after CRP the treatment outcomes between the single-CRP group and the multiple-CRP group showed no significant difference.
The outcomes included: 1) rate of negative DHT (1st week: 76.9 and 76.7%, 4th week: 89.5 and 88.2%); 2) rate of complete recovery or improved symptoms (1st week: 92.3 and 91.7%; 4th week: 96.5 and 98.0%); 3) DHI scores (1st week: 22 and 28; 4th week: 6 and 10); and 4) complication rate (1st week: 12.5 and 20%; 4th week: 20 and 0%. Rate of complications in the single-CRP group (6.9%) during the 1st treatment was lower than in the multiple-CRP groups (21.1%) (p-value = 0.013).
Conclusion: A single cycle of CRP is as effective as multiple cycle CRP, with a lower incidence of complication and a decrease in the time for treatment. Single-cycle CRP is a more advantageous treatment for unilateral posterior canal BPPV.
Clinical Trials.
gov Identifier: NCT02701218.

Pubmed PDF Web

A Comparison of Dizziness Handicap Inventory Scores by Categories of Vestibular Diagnoses

Graham, Madison K.; Staab, Jeffrey P.; Lohse, Christine M.; McCaslin, Devin L.

Publication date 01-01-2021


Objective: The Dizziness Handicap Inventory (DHI) is a 25-item self-report quantifying dizziness-related physical and emotional symptoms and restrictions in daily activities. DHI scores do not correlate with severity of structural vestibular deficits; thus, high DHI scores may reflect other causes of morbidity. This study investigated the relationship between total DHI scores and the presence of structural, functional, and psychiatric disorders in tertiary neurotology patients.
Study Design: Retrospective.
Setting: Tertiary center.
Patients: Eighty-five patients who underwent multidisciplinary neurotologic evaluations.
Intervention: Diagnostic.
Main Outcome Measure(s): Active illnesses identified by a multidisciplinary neurotology team were abstracted from medical records, cataloged as structural, functional, or psychiatric disorders, and used to group patients into diagnostic categories: structural (structural disorders only), functional (functional disorders with/without structural disorders), and psychiatric (psychiatric disorders with/without other conditions). DHI scores were compared across diagnostic categories. Sensitivities and specificities of DHI scores for identifying structural versus functional or psychiatric disorders were calculated.
Results: Mean DHI scores differed significantly by diagnostic category (structural 35 ± 18, functional 64 ± 15, and psychiatric 65 ± 19), before and after adjusting for age and sex (p 1.62 for structural versus functional and psychiatric categories). DHI≤30 (mild handicap) had specificity = 0.98 for structural disorders alone, whereas DHI>60 (severe handicap) had specificity = 0.88 for functional or psychiatric disorders.
Conclusions: In this tertiary cohort, categories of illnesses had large effects on total DHI scores. Patients with scores ≤30 were likely to have structural disorders alone, whereas those with scores >60 were likely to have functional or psychiatric disorders, with or without coexisting structural conditions.

Pubmed PDF Web

Validation of the Binocular Vision Dysfunction Questionnaire (BVDQ)

Feinberg, Debby L.; Rosner, Mark S.; Rosner, Arthur J.

Publication date 01-01-2021


Objective: Among patients presenting with dizziness, visual dysfunction must be considered, including vertical heterophoria (VH), a frequently under-identified form of binocular vision dysfunction where there is vertical discrepancy between the lines of sight of the eyes when at physiologic rest. Current self-rated screening measures do not account for complex VH symptomatology including dizziness/ambulation difficulties, nausea, headache, anxiety, neck pain, and reading impairment. VH must be differentiated from vestibular/otolithic etiologies, as their treatment frequently provides inadequate relief, yet treatment of the VH can reduce/eliminate symptoms. The objective of this study is to create a valid measurement tool (binocular vision dysfunction questionnaire) to assist in identifying VH among dizzy patients to aid in appropriate referral.
Study Design: Retrospective case series.
Setting: Tertiary referral center.
Patients: One hundred twenty-six patients presenting to an optometric binocular vision subspecialist diagnosed with VH.
Intervention: Psychometric study. The measurement tools internal consistency and test–retest reliability was assessed. Confirmatory and exploratory factor analyses were performed. Validity was estimated through correlations with a visual analog scale and validated instruments for headaches, dizziness, and anxiety.
Main Outcome Measures/Results: Excellent reliability demonstrated including Cronbachs alpha of 0.91 and high test–retest reliability. Statistical correlations with established measurements established sound convergent/content validity. Analysis of participants who underwent treatment indicated change in BVDQ score correlates with perception of change in symptom burden.
Conclusions: Results suggest the BVDQ is a valid, reliable screening tool to assist otologists in identifying VH among their dizzy patients. The BVDQ may also be useful for measuring changes with various treatments, and in identifying diverse symptoms associated with BVD/VH

Pubmed PDF Web

Fundal Fluid Cap Is Associated With Hearing Preservation in the Radiosurgical Treatment of Vestibular Schwannoma

Bojrab, Dennis I. II; Fritz, Christian G.; Lin, Kenny F.; Schutt, Christopher A.; Hong, Robert S.; Babu, Seilesh C.; Chen, Peter Y.; Maitz, Ann; Bojrab, Dennis I.

Publication date 01-01-2021


Objective: To evaluate the relationship between fundal fluid and hearing outcomes after treatment of vestibular schwannoma (VS) with Gamma Knife radiosurgery (GKRS).
Study Design: Retrospective case series.
Setting: Tertiary neurotology referral center.
Patients: Patients treated with GKRS for vestibular schwannoma between March 2007 and March 2017 were considered for this study. Exclusion criteria included pretreatment pure-tone average (PTA) >90 dB, neurofibromatosis type II, history of previous surgical resection, and follow-up less than 1 year.
Main Outcome Measure(s): Hearing function was assessed both by preservation of serviceable hearing and by preservation of baseline hearing (≤20 dB change in PTA) after GKRS. Hearing preservation comparisons were made between groups of patients with and without a fundal fluid cap.
Results: Patients with a fundal cap had significantly higher rates of baseline hearing preservation (≤20 dB change in PTA) according to Kaplan–Meier survival analysis of all 106 patients (p = 0.006). By the 3rd year posttreatment, 70.9% of patients with a fundal cap had maintained a ≤20 dB change in PTA, while only 43.6% of patients without a fundal fluid cap achieved this outcome (p = 0.004).
Conclusions: Fundal fluid present on pretreatment magnetic resonance imaging is predictive of improved baseline hearing preservation rates in patients undergoing GKRS for vestibular schwannoma when considering all patients with PTA ≤90 dB. Fundal fluid cap presence may serve as a favorable prognostic indicator to help set hearing expectations and guide patient selection efforts.

Pubmed PDF Web

Middle Cranial Fossa Approach to Vestibular Schwannoma Resection in the Older Patient Population

Kohlberg, Gavriel D.; Lipschitz, Noga; Raghavan, Arun M.; Breen, Joseph T.; Pensak, Myles L.; Zuccarello, Mario; Samy, Ravi N.

Publication date 01-01-2021


Objective: Compare outcomes of middle cranial fossa approach (MCF) to vestibular schwannoma (VS) resection in patients 60 years of age and older to patients under 60.
Study Design: Retrospective case series.
Setting: Tertiary referral center.
Patients: Charts of 216 consecutive VS patients over 18 years of age were reviewed to identify 67 patients who underwent MCF approach to VS resection between 2006 and 2017.
Intervention(s): Age at time of surgery.
Main Outcome Measure(s): Measured outcomes included postoperative hearing results, facial nerve function, length of hospital stay, wound complications, cerebrospinal fluid leak, myocardial infarction, cerebrovascular accident, seizure, deep vein thrombosis, 30-day readmission, and return to operating room.
Results: Sixty-seven patients underwent VS resection via MCF approach including 16 patients > = 60 years (mean 64.4 SD 3.3) and 51 patients < 60 years (mean 45.7 SD 10.2). Between these two groups, there were no differences in sex, tumor laterality, tumor size (10.4 mm versus 9.8 mm, p = 0.6), or other demographic characteristics. Postoperatively, there were no differences between groups in complication rates. Rates of HB 1 or 2 facial nerve function were similar (93.8% versus 88.2%, p = 0.7) as were rates of maintenance of class A or B hearing (58.3% versus 44.4%, p = 0.7).
Conclusions: Patients over 60 undergoing MCF for VS resection experienced similar rates of postoperative complications, facial nerve outcomes, and hearing preservation compared with younger patients. MCF for VS may be considered in the older population. Further research is warranted to evaluate appropriate limitations for this approach based on age.

Pubmed PDF Web

Endoscopic Intervention of Aberrant Carotid Artery in the Middle Ear

Hashim, Noor Dina; Jang, Seung H.; Moon, In Seok

Publication date 01-01-2021


Objective: To describe surgical management for transcanal endoscopic ear surgery (TEES) in two patients with aberrant internal carotid artery (ICA) in the middle ear.
Patients: A young girl who complained of pulsatile tinnitus and an elderly man who presented with ear bleeding. Otoendoscopy examination revealed a pulsatile reddish mass protruding through the tympanic membrane in both patients.
Interventions: A combination of clinical assessments and imaging supported the diagnosis of aberrant ICA in the middle ear. Transcanal endoscopic reinforcement of the artery was performed; tragal cartilage was used as a shield to strengthen the carotid canal defect.
Results: Assisted by fine endoscopic instruments, the protruding arteries were separated without damage to the surrounding structures and reinforced using tragal cartilage. Both patients’ symptoms improved postoperatively; they reported tinnitus relief and hearing improvement.
Conclusion: To prevent catastrophic events, diagnosis of aberrant ICA is important before any surgical intervention. With appropriate management, surgical intervention using transcanal endoscopic ear surgery offers a clear view of the surgical field and is an excellent choice for management of aberrant ICA.

Pubmed PDF Web

Middle Ear Actuator Performance Determined From Intracochlear Pressure Measurements in a Single Cochlear Scala

Raufer, Stefan; Gamm, Ute A.; Grossöhmichen, Martin; Lenarz, Thomas; Maier, Hannes

Publication date 01-01-2021


Hypothesis: Intracochlear pressure measurements in one cochlear scala are sufficient as reference to determine the output of an active middle ear implant (AMEI) in terms of “equivalent sound pressure level” (eqSPL).
Background: The performance of AMEIs is commonly calculated from stapes velocities or intracochlear pressure differences (PDiff). However, there are scenarios where measuring stapes velocities or PDiff may not be feasible, for example when access to the stapes or one of the scalae is impractical.
Methods: We reanalyzed data from a previous study of our group that investigated the performance of an AMEI coupled to the incus in 10 human temporal bones. We calculated eqSPL based on stapes velocities according to the ASTM standard F2504-05 and based on intracochlear pressures in scala vestibuli, scala tympani, and PDiff.
Results: The AMEI produced eqSPL of ∼100 to 120 dB at 1 Vrms. No significant differences were found between using intracochlear pressures in scala vestibuli, scala tympani, or PDiff as a reference. The actuator performance calculated from stapes displacements predicted slightly higher eqSPLs at frequencies above 1000 Hz, but these differences were not statistically significant.
Conclusion: Our findings show that pressure measurements in one scala can be sufficient to evaluate the performance of an AMEI coupled to the incus. The method may be extended to other stimulation modalities of the middle ear or cochlea when access to the stapes or one of the scalae is not possible.

Pubmed PDF Web

JAK/STAT Dysregulation With SOCS1 Overexpression in Acquired Cholesteatoma-Adjacent Mucosa

Westerberg, Johanna; Tideholm, Ellen; Piersiala, Krzysztof; Drakskog, Cecilia; Kumlien Georén, Susanna; Mäki-Torkko, Elina; Cardell, Lars Olaf

Publication date 01-01-2021


Importance: Surgery remains the gold standard in cholesteatoma treatment. However, the rate of recurrence is significant and the development of new nonsurgical treatment alternatives is warranted. One of the possible molecular pathways to target is the Janus kinase/signal transducer and activator of transcription (JAK/STAT) pathway.
Objective: To investigate the JAK/STAT pathway in the middle ear mucosa in patients with acquired cholesteatoma compared with middle ear mucosa from healthy controls.
Design: Case-control study.
Setting: Linköping University Hospital, Sweden, and Karolinska Institutet, Stockholm, Sweden.
Sampling period: February 2011 to December 2016.
Participants: Middle ear mucosa from 26 patients with acquired cholesteatoma undergoing tympanoplasty and mastoidectomy, and 27 healthy controls undergoing translabyrinthine surgery for vestibular schwannoma or cochlear implantation was investigated.
Main Outcomes/Measures: The expression of Interleukin-7 receptor alpha, JAK1, JAK2, JAK3, STAT5A, STAT5B, and suppressor of cytokine signaling-1 (SOCS1) were quantified using quantitative polymerase chain reaction. In addition, expression level of cyclin D2, transforming growth factor beta 1, thymic stromal lymphopoietin, CD3, and CD19 was evaluated.
Results: In cholesteatoma-adjacent mucosa, SOCS1 was significantly upregulated (p= 0.0003) compared with healthy controls, whereas STAT5B was significantly downregulated (p = 0.0006). The expression of JAK1, JAK2, JAK3, and STAT5A did not differ significantly between groups.
Conclusions and Relevance: To the best of our knowledge, this is the first article reporting dysregulation of the JAK/STAT pathway in cholesteatoma-adjacent mucosa. The main finding is that important players of the aforementioned pathway are significantly altered, namely SOCS1 is upregulated and STAT5B is downregulated compared with healthy controls.

Pubmed PDF Web

Enhanced Otolith Function Despite Severe Labyrinthine Damage in a Case of Pneumolabyrinth and Pneumocephalus Due to Otogenic Meningitis Associated With Superior Canal Dehiscence

Castellucci, Andrea; Botti, Cecilia; Renna, Luigi; Delmonte, Silvia; Moratti, Claudio; Pascarella, Rosario; Bianchin, Giovanni; Ghidini, Angelo

Publication date 01-01-2021


Objective: To describe a rare case of pneumolabyrinth (PNL) and pneumocephalus (PNC) due to otogenic meningitis in a patient with superior canal dehiscence (SCD) resulting in profound sensorineural hearing loss (SNHL), semicircular canals impairment but preservation of SCD-related enhanced otolith function.
Patient: A 65-year-old woman with otogenic meningitis.
Intervention: Temporal bone high-resolution computed tomography (CT) scans, brain-magnetic resonance imaging, audiometry, bedside examination, video-head impulse test, and vestibular-evoked myogenic potentials (VEMPs).
Main outcome measures: Enhanced otolith function despite canal and cochlear loss.
Results: The patient developed right profound SNHL and acute labyrinthitis. Imaging showed middle ear inflammatory tissue, right PNL and PNC despite lack of bony fractures. Bilateral SCD and tegmen dehiscence were detected. The patient underwent mastoidectomy, drainage of effusion, and surgical repair of tegmen dehiscence. Exploratory tympanotomy was uneventful. She was treated with intravenous antibiotics and dexamethasone for 3 weeks with improvement of general condition. At 3-weeks follow-up, right profound SNHL persisted with global hypofunction for ipsilateral semicircular canals and selective impairment for left superior canal activity at video-head impulse test. Surprisingly, both cervical and ocular-VEMPs exhibited bilaterally abnormal amplitudes and reduced thresholds, consistently with preserved SCD-related macular hypersensitivity to sounds even on the affected side.
Conclusions: This case report exhibits a unique clinical scenario as it offers interesting insights concerning PNL aetiology despite lack of either bony fractures or barotrauma and PNC likely conveyed intracranially by SCD. Moreover, it provides an unusual pattern of functional dissociation among inner-ear receptors showing enhanced otolith function despite severe labyrinthine damage.

Pubmed PDF Web

Quinine in Otology and Neurotology: Ototoxicity and Historic Role in Therapy

Semedo, Maria Guilherme; Dias-Silva, Nuno; Miguéis, Jorge; Pita, João Rui

Publication date 01-01-2021


Objectives/Hypothesis: Quinine, a cinchona bark-derived antimalarial alkaloid, is a known ototoxic. Isolated and named in 1820 by the French scientists Pierre-Joseph Pelletier and Joseph-Bienaimé Caventou, it has since been employed in the treatment of different maladies. Quinine was also recommended as a local anesthetic in surgical procedures in the early 20th century. This article aims to identify early ototoxicity reports regarding quinine and to investigate if quinine was previously used in otology as an anesthetic agent or as an actual therapy.
Method: Historical review of medical and pharmaceutical literature from the 19th and 20th centuries in databases (Pub Med; Web of Science), as well as medical books on ototoxic drugs, quinine, and therapies in otology.
Results: The first identified reference of quinine ototoxicity was from 1824. Quinine also had a therapeutic role in otology and neurotology and was employed for its analgesic properties. It was used in Menières disease, vertigo, otalgia, purulent otitis media, neuralgia of the plexus tympani, furuncles in the auditory canal, and herpes zoster in the auricle.
Conclusion: Quinine was acknowledged as an ototoxic drug in the 19th century. Quinine was used in several otologic disorders, both as an analgesic (for herpes zoster, otalgia) and as a therapeutic agent (Menières disease, vertigo, purulent otitis media, furuncles in the auditory canal). This research demonstrates that, analogously to gentamicin, quinine was used in Menières disease specifically due to its ototoxic effects.

Pubmed PDF Web

Perilymphatic Fistula: Consideration Based on MRI and Surgery

Eliezer, Michael; Toupet, Michel; Guichard, Jean-Pierre; Houdart, Emmanuel; Kania, Romain; Hautefort, Charlotte

Publication date 01-01-2021


No abstract available

Pubmed PDF Web

Benign Paroxysmal Positional Vertigo: Canal Switching Affecting All Canals During a Single Session

Zanotti, Estefanía; Yacovino, Darío Andrés

Publication date 01-01-2021


SDC Video Link:http://links.lww.com/MAO/B86.

Pubmed PDF Web

Highlights From the ACI Alliance 16th Symposium on Cochlear Implants in Children in Hollywood FL: Introduction to the Special Issue: 16th Symposium on Cochlear Implants in Children

Sorkin, Donna L.; Telischi, Fred; Cejas, Ivette

Publication date 01-01-2021


No abstract available

Pubmed PDF Web

Development and Validation of the Spanish AzBio Sentence Corpus

Rivas, Alejandro; Perkins, Elizabeth; Rivas, Adriana; Rincon, Luz Adriana; Litvak, Leo; Spahr, Tony; Dorman, Michael; Kessler, David; Gifford, René

Publication date 01-01-2021


Objective: To create and validate a Spanish sentence test for evaluation of speech understanding of Spanish-speaking listeners with hearing loss or cochlear implants (CI).
Study Design: Two thousand sentences were recorded from two male and two female speakers. The average intelligibility of each sentence was estimated as the mean score achieved by five listeners presented with a five-channel cochlear implant simulation. The mean scores of each sentence were used to construct 42 lists of 20 sentences with similar mean scores. List equivalency was then validated by presenting all lists to 10 CI users and in a 2-list comparison in a clinical setting to 38 CI patients.
Setting: Tertiary referral center.
Patients: Normal-hearing listeners (n = 5), CI users in a research study (n = 10), and CI patients (n = 38) in routine clinical follow-up.
Intervention: Multiple sentence lists from a newly minted speech perception test.
Main Outcome Measures: List intelligibility and equivalence across sentence lists.
Results: Forty-two lists of sentences were equivalent when all lists were presented in random order to 10 adult CI recipients. The variability of scores observed on lists presented to the same listener in the same condition was captured using a binomial distribution model based on a 40-item list for 38 adult implant recipients.
Conclusion: The Spanish Az Bio Sentence Test includes 42 lists of 20 sentences. These sentences are roughly equivalent in terms of overall difficulty and confidence limits have been provided to assess the significance of variability in list scores observed within or across conditions. These materials will be of benefit when assessing native Spanish speakers in both research and clinical settings.

Pubmed PDF Web

Considerations for a Revised Adult Cochlear Implant Candidacy Evaluation Protocol

Biever, Allison; Amurao, Carly; Mears, Megan

Publication date 01-01-2021


Objective: To compare the current metrics used in adult cochlear implant candidacy evaluations for consistency and applicability, and to make a recommendation for an updated assessment battery.
Study Design: Prospective, multicenter, within-subject clinical trial.
Setting: North American cochlear implant programs including private practices, universities, and hospital centers.
Patients: One hundred adult hearing aid users scoring 40% or less on monosyllabic words in quiet in the aided test ear who presented for cochlear implantation evaluation.
Intervention: Subjects underwent unilateral cochlear implantation.
Main Outcome Measures: Speech perception measured via Consonant-Nucleus-Consonant words in quiet and Az Bio sentences in noise. Patient-reported hearing disability measured via the Speech, Spatial, and Qualities of Hearing Scale administered preoperatively and at 6 months postoperatively.
Results: Significant group mean improvement on all speech perception measures and SSQ subscales postoperatively with possible floor effects observed in objective background noise testing preoperatively and a broad range of variability seen postoperatively.
Conclusions: Consonant-Nucleus-Consonant words are an effective tool to clinically evaluate hearing ability over time. Adjustment of cochlear implant screening protocols to prioritize monosyllabic words over sentences in noise as the chief determining factor appears justified, and this test can be supplemented by the SSQ for a holistic and applicable assessment of cochlear implant candidacy.

Pubmed PDF Web

Assessment of Frequency-Place Mismatch by Flat-Panel CT and Correlation With Cochlear Implant Performance

Zanetti, Diego; Conte, Giorgio; Di Berardino, Federica; Lo Russo, Francesco; Cavicchiolo, Sara; Triulzi, Fabio

Publication date 01-01-2021


Objective: To calculate the frequency allocation mismatch in a group of very selected cochlear implant (CI) recipients and to contrast it with the speech perception performances.
Study Design: Cross-sectional observational prospective study.
Settings: Tertiary Audiological Department, University hospital.
Patients: Fifteen adults receiving the same CI array by the same surgeon through a posterior tympanotomy, round window approach.
Main Outcome Measures: 1) High definition flat panel computed tomography (FPCT) control of the intracochlear position of each electrode contact, and computation of the relative frequency allocation mismatch; 2) analysis of speech perception outcomes in relation with the mismatch.
Results: Despite a consistent and reproducible surgical procedure with the same intracochlear array, significant deviations from the frequency allocation tables (FAT) assigned by default by the manufacturer were observed in this study.
Their influences on speech perception performances were negligible in the simple tasks of words or sentences recognition in quiet (and, to a lesser extent also in noise). The greatest effect of a significant mismatch was observed for the vocal-consonant-vocal (VCV) sequences recognition under noise masking, the emotional and the linguistic prosody recognition, and the phonemes discrimination of the Auditory Speech Sound Evaluation (A§E) test.
Conclusions: The greatest frequency-to-place occurred at the high frequencies. The effect was rather irrelevant on simple words and sentences recognition, while it negatively impacted on the more complex perceptual tasks

Pubmed PDF Web

Principles of Executive Functioning Interventions for Children With Cochlear Implants: Guidance From Research Findings and Clinical Experience

McConkey Robbins, Amy; Kronenberger, William G.

Publication date 01-01-2021


Children with cochlear implants (CIs) are at risk for experiencing deficits in the development of neurocognitive processes known as executive functions (EF). Such deficits likely arise as a consequence of early-onset deafness, the degraded auditory input provided by CIs, and delays in spoken language development. Interventions specifically designed for pediatric CI users are needed to address challenges and delays in EF because of the unique influences of hearing loss and language delay on EF development, which have deleterious, cascading effects on speech and language development and subsequent reading and academic achievement. Because patterns of EF weakness emerge even in the preschool years, interventions to improve EF should be implemented at early ages, while children are young and neuroplasticity is high. Drawing on previous research findings and clinical experience, this paper highlights 10 principles to guide the development of EF interventions for children with CIs.

Pubmed PDF Web

Screening Strategies for Deafness Genes and Functional Outcomes in Cochlear Implant Patients

Nisenbaum, Eric; Prentiss, Sandra; Yan, Denise; Nourbakhsh, Aida; Smeal, Molly; Holcomb, Meredith; Cejas, Ivette; Telischi, Fred; Liu, Xue Zhong

Publication date 01-01-2021


Objectives: To review the current state of knowledge about the influence of specific genetic mutations that cause sensorineural hearing loss (SNHL) on cochlear implant (CI) functional outcomes, and how this knowledge may be integrated into clinical practice. A multistep and sequential population-based genetic algorithm suitable for the identification of congenital SNHL mutations before CI placement is also examined.
Data Sources, Study Selection: A review was performed of the English literature from 2000 to 2019 using Pub Med regarding the influence of specific mutations on CI outcomes and the use of next-generation sequencing for genetic screening of CI patients.
Conclusion: CI is an effective habilitation option for patients with severe-profound congenital SNHL. However, it is well known that CI outcomes show substantial inter-patient variation. Recent advances in genetic studies have improved our understanding of genotype–phenotype relationships for many of the mutations underlying congenital SNHL, and have explored how these relationships may account for some of the variance seen in CI performance outcomes. A sequential genetic screening strategy utilizing next-generation sequencing-based population-specific gene panels may allow for more efficient mutation identification before CI placement. Understanding the relationships between specific mutations and CI outcomes along with integrating routine comprehensive genetic testing into pre-CI evaluations will allow for more effective patient counseling and open the door for the development of mutation-specific treatment strategies.

Pubmed PDF Web

The Long-Term Stability of the Electrical Stapedial Reflex Threshold

Pitt, Cache; Muñoz, Karen; Schwartz, Sarah; Kunz, John M.

Publication date 01-01-2021


Objectives: To 1) describe changes in the electrical stapedial reflex threshold (eSRT), within and across patients over time and 2) to identify the clinical relationship between eSRT and an individuals upper limit of loudness.
Study Design: Retrospective chart review and analysis using a multilevel modeling approach to describe changes in eSRT over time.
Setting: Secondary care center.
Patients: Two-hundred five cochlear implant recipients treated at the cochlear implant center during a 3-year time period.
Intervention(s): Cochlear implantation, eSRT testing, and, electrical upper limits of loudness.
Main Outcome Measure(s): The eSRT over multiple appointments and the cochlear implant recipients’ final upper limits of loudness.
Results: Analysis of the eSRT testing indicated stability over time; no global trend was seen in trajectory across the population, b = –0.010, p = 0.899. The relationship between eSRT and user upper limits of loudness revealed a mean decrease of 19.47, units for manufacturer 1, 30.53 units for manufacturer 2, and 0.7 units for manufacturer 3.
Conclusion: Electrical stapedial reflex thresholds remain consistent for individual subjects over time with implant experience being the only variable correlated with eSRT stability (increase in 5% of one standard deviation with each year of experience). In addition, a clinical relationship between eSRT and behaviorally set upper limits of loudness was identified for all three cochlear implant manufacturers available in the United States.

Pubmed PDF Web

Effectiveness of Place-based Mapping in Electric-Acoustic Stimulation Devices

Dillon, Margaret T.; Canfarotta, Michael W.; Buss, Emily; Hopfinger, Joseph; O’Connell, Brendan P.

Publication date 01-01-2021


Background: The default mapping procedure for electric-acoustic stimulation (EAS) devices uses the cochlear implant recipients unaided detection thresholds in the implanted ear to derive the acoustic settings and assign the lowest frequency filter of electric stimulation. Individual differences for speech recognition with EAS may be due to discrepancies between the electric frequency filters of individual electrode contacts and the cochlear place of stimulation, known as a frequency-to-place mismatch. Frequency-to-place mismatch of greater than 1/2 octave has been demonstrated in up to 60% of EAS users. Aligning the electric frequency filters via a place-based mapping procedure using postoperative imaging may improve speech recognition with EAS.
Methods: Masked sentence recognition was evaluated for normal-hearing subjects (n = 17) listening with vocoder simulations of EAS, using a place-based map and a default map. Simulation parameters were based on audiometric and imaging data from a representative 24-mm electrode array recipient and EAS user. The place-based map aligned electric frequency filters with the cochlear place frequency, which introduced a gap between the simulated acoustic and electric output. The default map settings were derived from the clinical programming software and provided the full speech frequency range.
Results: Masked sentence recognition was significantly better for simulated EAS with the place-based map as compared with the default map.
Conclusion: The simulated EAS place-based map supported better performance than the simulated EAS default map. This indicates that individualizing maps may improve performance in EAS users by helping them achieve better asymptotic performance earlier and mitigate the need for acclimatization.

Pubmed PDF Web

Enlarged Vestibular Aqueduct: Hearing Progression and Cochlear Implant Candidacy in Pediatric Patients

Hodge, Sarah E.; Thompson, Nicholas J.; Park, Lisa R.; Brown, Kevin D.

Publication date 01-01-2021


Hypothesis/Objective: Investigate the rate of hearing loss progression and incidence of cochlear implant candidacy in children with enlarged vestibular aqueduct (EVA).
Background: EVA is the most common congenital malformation of the inner ear, is responsible for a large percentage of children with hearing loss, and is associated with hearing loss progression. Rates and degree of progression of hearing loss to cochlear implantation candidacy have not been well described.
Methods: Review of children with EVA who presented to a single academic medical center. Audiometric data were reviewed to determine subjects who met criteria for cochlear implantation (≥75 dB pure-tone average) at presentation. For those not meeting criteria, serial audiometric data were reviewed for progression to candidacy.
Results: A total of 257 ears met inclusion criteria. One hundred ninety-two (74.7%) met cochlear implant candidacy criteria by age 12, yet only 117 ears (60.9%) actually received implants before turning 13. One hundred fifty-three ears (59.5%) met implant candidacy criteria at presentation. Nearly 50% of those not initially meeting implantation criteria had a ≥15 dB shift in pure-tone average by age 12, with 37.5% of this subgroup meeting implant candidacy criteria before their teen years at an average age of 7.10 years.
Conclusion: The majority of children with EVA reach cochlear implant candidacy before reaching adulthood, yet implantation rates for candidate ears remain at 60% and delay in implantation persist. Parents of children with EVA should be counseled on the likelihood of progression and closely monitored for cochlear implant candidacy.

Pubmed PDF Web

Copyright © KNO-T, 2020 | R/Abma