OBJECTIVES:Cochlear implants (CIs) have been shown to benefit patients with single-sided deafness (SSD) in terms of tinnitus reduction, localization, speech understanding, and quality of life (QoL). ...While previous studies have shown cochlear implantation may benefit SSD patients, it is unclear which point of comparison is most relevantbaseline performance before implantation versus performance with normal-hearing (NH) ear after implantation. In this study, CI outcomes were assessed in SSD patients before and up to 6 mo postactivation. Benefits of cochlear implantation were assessed relative to binaural performance before implantation or relative to performance with the NH ear alone after implantation.
DESIGN:Here, we report data for 10 patients who completed a longitudinal, prospective, Food and Drug Administration–approved study of cochlear implantation for SSD patients. All subjects had severe to profound unilateral hearing loss in one ear and normal hearing in the other ear. All patients were implanted with the MED-EL CONCERTO Flex 28 device. Speech understanding in quiet and in noise, localization, and tinnitus severity (with the CI on or off) were measured before implantation (baseline) and at 1, 3, 6 mo postactivation of the CI processor. Performance was measured with both ears (binaural), the CI ear alone, and the NH ear alone (the CI ear was plugged and muffed). Tinnitus severity, dizziness severity, and QoL were measured using questionnaires administered before implantation and 6 mo postactivation.
RESULTS:Significant CI benefits were observed for tinnitus severity, localization, speech understanding, and QoL. The degree and time course of CI benefit depended on the outcome measure and the reference point. Relative to binaural baseline performance, significant and immediate (1 mo postactivation) CI benefits were observed for tinnitus severity and speech performance in noise, but localization did not significantly improve until 6 mo postactivation; questionnaire data showed significant improvement in QoL 6 mo postactivation. Relative to NH-only performance after implantation, significant and immediate benefits were observed for tinnitus severity and localization; binaural speech understanding in noise did not significantly improve during the 6-mo study period, due to variability in NH-only performance. There were no correlations between behavioral and questionnaire data, except between tinnitus visual analog scale scores at 6 mo postactivation and Tinnitus Functional Index scores at 6 mo postactivation.
CONCLUSIONS:The present behavioral and subjective data suggest that SSD patients greatly benefit from cochlear implantation. However, to fully understand the degree and time course of CI benefit, the outcome measure and point of comparison should be considered. From a clinical perspective, binaural baseline performance is a relevant point of comparison. The lack of correlation between behavioral and questionnaire data suggest that represent independent measures of CI benefit for SSD patients.
The presence of a congenital anomaly is associated with increased childhood cancer risk, likely due to large effects of Down syndrome and chromosomal anomalies for leukemia. Less is known about ...associations with presence of non-chromosomal anomalies.
Records of children diagnosed with cancer at <20 years of age during 1984-2013 in Washington State cancer registries were linked to their birth certificates (N = 4,105). A comparison group of children born in the same years was identified. Congenital anomalies were assessed from birth records and diagnosis codes in linked hospital discharge data. Logistic regression was used to estimate odds ratios (OR) and 95% confidence intervals (CI) for cancer, and for specific cancer types in relation to the presence of any anomaly and specific anomalies.
Having any congenital anomaly was associated with an increased risk of childhood cancer (OR: 1.46, 95% CI 1.28-1.65). Non-chromosomal anomalies were also associated with increased childhood cancer risk overall (OR: 1.35; 95% CI: 1.18-1.54), and with increased risk of several cancer types, including neuroblastoma, renal, hepatoblastoma, soft-tissue sarcoma, and germ cell tumors. Increasing number of non-chromosomal anomalies was associated with a stronger risk of childhood cancer (OR for 3+ anomalies: 3.11, 95% CI: 1.54-6.11). Although central nervous system (CNS) anomalies were associated with CNS tumors (OR: 6.05, 95% CI 2.75-13.27), there was no strong evidence of other non-chromosomal anomalies being specifically associated with cancer occurring in the same organ system or anatomic location.
Non-chromosomal anomalies increased risk of several cancer types. Additionally, we found that increasing number of non-chromosomal anomalies was associated with a stronger risk of cancer. Pooling similar data from many regions would increase power to identify specific associations in order to inform molecular studies examining possible common developmental pathways in the etiologies of birth defects and cancer.
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DOBA, IZUM, KILJ, NUK, PILJ, PNG, SAZU, SIK, UILJ, UKNU, UL, UM, UPUK
Cochlear implantation as an approved clinical therapy ushered in an exciting era of innovation for the treatment of hearing loss. The U.S. Food and Drug Administration approved the use of cochlear ...implants as a treatment option for adults with profound sensorineural hearing loss in 1985. The landscape for treating adults and children with significant hearing loss has changed dramatically over the last three decades. The purpose of this paper is to examine the evolving regulatory process and changes to clinical care. A significant emerging trend in cochlear implantation is the consideration of steroids to preserve hearing during and following surgery. This parallels the quest for hearing preservation in noise-induced hearing disorders, especially considering the current interest in biological drug therapies in this population. The future will likely usher in an era of combination therapeutics utilizing drugs and cochlear implantation. For over 30+ years and following regulatory compliance, the Rocky Mountain Ear Center has developed an extensive candidacy and outcome assessment protocol. This systematic approach evaluates both unaided and aided auditory performance during candidacy stages and post-implantation. Adjunctive measures of cognition and quality-of-life augment the auditory assessment in specific populations. Practical insights into lessons learned have directed further clinical research and have resulted in beneficial changes to clinical care.
To comprehensively assess and describe functional auditory performance in a group of adults with bilateral, moderate sloping to profound sensorineural hearing loss who were dissatisfied users of ...well-fit bilateral hearing aids and presented for Cochlear implant evaluation. Participants were evaluated with bilateral hearing aids and after six months of bimodal (Cochlear implant and a contralateral hearing aid) hearing experience with a Cochlear implant and contralateral hearing aid.
Study participants were assessed using pure tone audiometry, aided speech understanding in quiet (CNC words) and in noise (AzBio sentences at +10 and +5 dB SNR) in the sound field with unilateral and bilateral hearing aids fit to target. Participants completed subjective scales of quality of life, (Health Utilities Index Mark 3), hearing disability, (Speech, Spatial and Qualities of Hearing Scale) and a device use satisfaction scale. Participants ≥55 years were administered the Montreal Cognitive Assessment screening tool. One-hundred enrolled individuals completed baseline evaluations.
Aided bilateral mean speech understanding scores were 28% for CNC words and 31%, and 17% for AzBio sentences at a +10 dB, and +5 dB SNR, respectively. Mean scale ratings were 0.46 for overall quality of life and 3.19 for functional hearing ability. Ninety percent of participants reported dissatisfaction with overall hearing performance.
Evaluation results, including functional performance metrics quantifying the deleterious effects of hearing loss for overall wellbeing, underscore that bilateral hearing aids are not an effective treatment for individuals with bilateral, moderate sloping to profound sensorineural hearing loss. Individuals with this degree of hearing impairment, who demonstrate poor aided speech understanding and dissatisfaction with hearing abilities in everyday life, require timely referral to a Cochlear implant clinic for further evaluation.
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GEOZS, IJS, IMTLJ, KILJ, KISLJ, NLZOH, NUK, OILJ, PNG, SAZU, SBCE, SBJE, UILJ, UL, UM, UPCLJ, UPUK, ZAGLJ, ZRSKP
Type 2 diabetes mellitus (T2D) is a prevalent long-term complication of treatment in survivors of childhood cancer, with marked racial/ethnic differences in burden. In this study, we investigated ...trans-ancestral genetic risks for treatment-related T2D.
Leveraging whole-genome sequencing data from the St Jude Lifetime Cohort (N = 3,676, 304 clinically ascertained cases), we conducted ancestry-specific genome-wide association studies among survivors of African and European genetic ancestry (AFR and EUR, respectively) followed by trans-ancestry meta-analysis. Trans-/within-ancestry replication including data from the Childhood Cancer Survivor Study (N = 5,965) was required for prioritization. Three external general population T2D polygenic risk scores (PRSs) were assessed, including multiancestry PRSs. Treatment risk effect modification was evaluated for prioritized loci.
Four novel T2D risk loci showing trans-/within-ancestry replication evidence were identified, with three loci achieving genome-wide significance (
< 5 × 10
). Among these, common variants at 5p15.2 (
), 2p25.3 (
), and 19p12 (
) showed evidence of modifying alkylating agent-related T2D risk in both ancestral groups, but showed disproportionately greater risk in AFR survivors (AFR odds ratios ORs, 3.95-17.81; EUR ORs, 2.37-3.32). In survivor-specific RNA-sequencing data (N = 207), the 19p12 locus variant was associated with greater
expression dysregulation after exposures to alkylators. Elevated T2D risks across ancestry groups were only observed with increasing values for multiancestry T2D PRSs and were especially increased among survivors treated with alkylators (top
bottom quintiles: OR
, 20.18;
= .023; OR
, 13.44;
= 1.3 × 10
).
Our findings suggest therapy-related genetic risks contribute to the increased T2D burden among non-Hispanic Black childhood cancer survivors. Additional study of how therapy-related genetic susceptibility contributes to this disparity is needed.
Conductive hearing loss (CHL) is known to produce hearing deficits, including deficits in sound localization ability. The differences in sound intensities and timing experienced between the two ...tympanic membranes are important cues to sound localization (ILD and ITD, respectively). Although much is known about the effect of CHL on hearing levels, little investigation has been conducted into the actual impact of CHL on sound location cues. This study investigated effects of CHL induced by earplugs on cochlear microphonic (CM) amplitude and timing and their corresponding effect on the ILD and ITD location cues. Acoustic and CM measurements were made in 5 chinchillas before and after earplug insertion, and again after earplug removal using pure tones (500 Hz to 24 kHz). ILDs in the unoccluded condition demonstrated position and frequency dependence where peak far-lateral ILDs approached 30 dB for high frequencies. Unoccluded ear ITD cues demonstrated positional and frequency dependence with increased ITD cue for both decreasing frequency (±420 μs at 500 Hz, ±310 μs for 1–4 kHz) and increasingly lateral sound source locations. Occlusion of the ear canal with foam plugs resulted in a mild, frequency-dependent conductive hearing loss of 10–38 dB (mean 31 ± 3.9 dB) leading to a concomitant frequency dependent increase in ILDs at all source locations. The effective ITDs increased in a frequency dependent manner with ear occlusion as a direct result of the acoustic properties of the plugging material, the latter confirmed via acoustical measurements using a model ear canal with varying volumes of acoustic foam. Upon ear plugging with acoustic foam, a mild CHL is induced. Furthermore, the CHL induced by acoustic foam results in substantial changes in the magnitudes of both the ITD and ILD cues to sound location.
►Acoustic cues in conductive hearing loss induced by foam earplug were studied.►Binaural acoustic cues of interaural time and level differences were computed.►The conductive loss induced by plugging leads to an azimuth shift of auditory space. ►The acoustic cue changes were confirmed by analysis of a model ear canal.
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GEOZS, IJS, IMTLJ, KILJ, KISLJ, NLZOH, NUK, OILJ, PNG, SAZU, SBCE, SBJE, UILJ, UL, UM, UPUK, ZAGLJ, ZRSKP
There are three main cues to sound location: the interaural differences in time (ITD) and level (ILD) as well as the monaural spectral shape cues. These cues are generated by the spatial- and ...frequency-dependent filtering of propagating sound waves by the head and external ears. Although the chinchilla has been used for decades to study the anatomy, physiology, and psychophysics of audition, including binaural and spatial hearing, little is actually known about the sound pressure transformations by the head and pinnae and the resulting sound localization cues available to them. Here, we measured the directional transfer functions (DTFs), the directional components of the head-related transfer functions, for 9 adult chinchillas. The resulting localization cues were computed from the DTFs. In the frontal hemisphere, spectral notch cues were present for frequencies from ∼6–18 kHz. In general, the frequency corresponding to the notch increased with increases in source elevation as well as in azimuth towards the ipsilateral ear. The ILDs demonstrated a strong correlation with source azimuth and frequency. The maximum ILDs were <10 dB for frequencies <5 kHz, and ranged from 10–30 dB for the frequencies >5 kHz. The maximum ITDs were dependent on frequency, yielding 236 μs at 4 kHz and 336 μs at 250 Hz. Removal of the pinnae eliminated the spectral notch cues, reduced the acoustic gain and the ILDs, altered the acoustic axis, and reduced the ITDs.
► Sound pressure transformations by the head and pinnae of the adult chinchilla (
Chinchilla lanigera) produced monaural spectral notch cues for frequencies from ∼6–18 kHz. ► The maximum interaural level differences were <10 dB for frequencies <5 kHz, and ranged from 10–30 dB for the frequencies > 5 kHz. ► The maximum interaural time differences were dependent on sound frequency, yielding 236 μs at 4 kHz and 336 μs at 250 Hz. ► Removal of the pinnae eliminated the spectral notch cues, reduced the acoustic gain and the interaural level differences, altered the acoustic axis, and reduced the interaural time differences.
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GEOZS, IJS, IMTLJ, KILJ, KISLJ, NLZOH, NUK, OILJ, PNG, SAZU, SBCE, SBJE, UILJ, UL, UM, UPUK, ZAGLJ, ZRSKP
Long-term retained foreign bodies in the human body have been reported across many specialties, but relatively few exist in the ENT literature.
We present a case report of a patient with a broken ...dental needle fragment in the posterior oral cavity with subsequent migration to the cochlea over the course of 4 years, eventually leading to hearing loss. CT scan and middle ear exploration demonstrated a 4-cm metallic fragment abutting the base of the cochlea, immediately adjacent to the internal carotid artery.
The needle segment was removed through an endaural approach without complication.
Postoperatively, the patient had improvements in PTA and speech discrimination, as well as the resolution of chronic otalgia and jaw pain. Imaging, audiologic results, and surgical details and pictures are presented herein.
To our knowledge, based on a thorough PubMed and Google Scholar search, there are no reports of such a foreign body migration from the oral cavity to the skull base.
(1) To describe the use of total ossicular prostheses (TOPs) in the setting of stapedectomy requiring an incus bypass procedure. (2) To analyze the short- and long-term audiometric results of TOP ...utilization in the setting of stapedectomy for an incus bypass procedure.
Case series with chart review.
Tertiary neurotologic referral center.
Seventeen cases of TOP reconstruction after stapedectomy were performed due to advanced incus erosion. The cases were assessed for pre- and postoperative bone conduction and air conduction pure-tone averages (PTAs; 0.5, 1, 2, 3 kHz), including high-tone bone conduction (1, 2, 4 kHz), air-bone gap, and speech discrimination scores. Hearing outcomes were measured: short-term (3 weeks) and long-term (average, 22 months).
Among 17 ears undergoing revision stapedectomy managed with TOP reconstruction, the average number of previous revision attempts was 1.0 (SD, 1; range, 1-5). The preoperative bone conduction PTA was 30.7 dB preoperatively, while the preoperative air conduction PTA was 64.3 dB. The mean postoperative air-bone gap significantly decreased to 18.9 dB (SD, 12.7; range, 5-46.25; P < .003) with a mean follow-up of 22.2 months (SD, 25.0; range, 0.75-78). No significant decrement in high-tone bone conduction PTA was observed (mean, 0 dB; SD, 12.8; range, -36.7 to 20; P = .427); however, 1 ear revealed a severe decrease in PTA and speech discrimination score postoperatively. No further revisions were noted in follow-up.
TOP reconstruction in the setting of previous revision stapedectomy with limited incudovestibular reconstructive options may lead to favorable hearing outcomes, but it carries an increased risk of sensorineural hearing loss.
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FZAB, GIS, IJS, KILJ, NLZOH, NUK, OILJ, SAZU, SBCE, SBMB, UL, UM, UPUK
Review audiological outcomes after revision stapedectomy using hydroxyapatite cement.
Retrospective case review.
Tertiary neurotological referral center.
Thirty-seven cases of previously treated ...otosclerosis where incus erosion was observed during revision stapedectomy.
Hydroxyapatite cement was used to rebuild the eroded incus and stabilize the prosthesis during revision stapedectomy.
Pre- and postoperative bone conduction (BC) and air conduction (AC) pure-tone averages (PTA) (0.5, 1, 2, 3 kHz) including high tone BC (1, 2, 4 kHz), air-bone gap (ABG), and speech discrimination scores were measured. Short-term (3 wk) and longer-term (average 12 mo) hearing outcomes were measured. Data are reported according to the most recent guidelines of the AAO-HNS Committee on Hearing and Equilibrium.
Among 37 ears undergoing revision stapedectomy with hydroxyapatite cement, the AC PTA was 59.8 dB preoperatively and 34.6 dB postoperatively (p < 0.0001) at latest follow-up. The mean postoperative ABG was 8.8 dB (SD = 8.6, range -1.3-36.3 dB) while a mean improvement of 2.1 dB (SD = 6.5, range -8.33-15.0 dB) of the high tone BC PTA was observed. One revision case was noted during the follow-up period.
Hydroxyapatite cement is useful to reconstruct and stabilize the prosthesis in revision stapedectomy when erosion of the long process is encountered. Short- and longer-term hearing results are favorable when compared to previously reported results of revision stapedectomy.
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