Labyrinthotomy or Vestibulotomy in Anatomic and Congenital Variations of the Oval Window and Facial Nerve

Perioperative Hearing Impairment

Active middle ear implants, such as the Vibrant Soundbridge, are used as an important part in the rehabilitation of sensorineural, conductive hearing, or mixed hearing loss. The attachment of the Vibrant Soundbridge at the round window and the usage of the Vibroplasty couplers strongly expanded the application of the Vibrant Soundbridge.The Vibrant Soundbridge is developed for patients who have an intolerance to hearing aids and a moderate to profound sensorineural hearing loss. The VSB also provides an optimal solution for patients with failed middle ear reconstructions or patients with atresia. To capture the improvement with the VSB Implant with different hearing losses a literature analysis was conducted. The functional gain was analyzed for 107 patients with conductive hearing loss and for 214 patients with sensorineural hearing loss out of 14 studies.Patients with conductive and mixed hearing loss resulted in a functional gain from 30 to 58 dB with the VSB. Patients with a pure sensorineural hearing loss showed a functional gain of 23-30 dB. The VSB bone conduction threshold shift was analyzed for all studies conducted in the years between 2000 and 2009. In 11 of the 16 studies there was no significant (p=0.05) change found. In 5 studies, the pre- to post-surgical bone conduction threshold shift was less than 10 dB. None of these studies measured a threshold shift of more than 10 dB.The flexible attachment at either the long process of the incus with sensorineural hearing loss, with an conductive hearing loss at the round window or the use of Vibroplasty couplers at the oval window, head of the stapes or round window makes the VSB an extremely versatile instrument. If patients can't wear conventional hearing aids, had failed middle ear reconstructions or atresia the VSB presents, due to the significant hearing improvement in any type of hearing loss, an ideal solution.

New Bone-Anchored Amplification Options for Children

Branchio-oto-renal syndrome (BOR) is an autosomal dominant mutation of the EYA1 and the more recently discovered the SIX1 gene.1 The phenotype and syndrome were comprehensively described by Melnick in 1975 to include hearing loss, auricular malformations, branchial arch remnants, and renal anomalies.2 The diagnosis of BOR is made using major and minor criteria as defined by Chang et al 2004.3 However, 60% of patients who meet phenotypic criteria do not have an identifiable mutation in the EYA1 gene, leading to recent interest in the EYA-SIX regulatory system.1 The most common manifestations include hearing loss (98.5%), preauricular pits (83.6%), branchial anomalies (68.5%), renal anomalies (38.2%), and external ear abnormalities (31.5%). In terms of the imaging characteristics, the most sensitive modality remains CT of the temporal bones. The most commonly reported anomalies on temporal bone imaging include but are not limited to 1)hypoplastic apical turn of the cochlea, 2) facial nerve deviated to the medial side of the cochlea, 3) funnel-shaped internal auditory canal, and 4) patulous eustachian tube. 4 The spectrum of hearing loss in BOR is variable but most commonly presents with mixed hearing loss (50%), pure sensorineural hearing loss (25%) and pure conductive hearing loss (25%) 5. The conductive component of the hearing loss is most often the result of ossicular chain abnormalities. A 42 year old male previously diagnosed with BOR using clinical criteria presented with a conductive hearing loss. His physical exam demonstrated small external auditory canals with a normal tympanic membrane. His audiogram demonstrated a mild left sensorineural hearing loss and a maximal conductive hearing loss in the right ear. Imaging with CT revealed several findings consisted with BOR: Bilateral enlarged air-filled eustachian tubes extending from the middle ear to the nasopharynx, a widened and flared internal acoustic meatus with the nervus intermedius extending into a funnel shaped labyrinthine segment of the temporal bone and hypo-plastic horizontal canal, hypo-plastic vestibular system/epitympanum, and lateral position of the facial nerve. The incus and malleus were malformed and fixed in the attic (Fig. 1). Figure 1 Computed tomography (CT) images. Panel A: Axial view of the head demonstrating enlarged eustachian tubes (arrow). Panel B: Axial view of right internal auditory (IAC) canal demonstrating hypoplastic horizontal semicircular canal(H), and funnel shaped ... Despite the findings on CT, the patient elected to pursue a middle ear exploration prior to pursuing other rehabilitative options. At surgery, middle ear exploration revealed a very small oval window niche with no clear oval window, or stapes footplate as shown on this view with a 30° endoscope (Fig. 2). The round window niche was visible. A dehiscent facial nerve was visible at the horizontal segment. Because no mobile footplate was found there was no attempt at ossiculoplasty. The patient recovered from surgery with no change in his hearing and later went on to a Baha which he found beneficial. Figure 2 Right middle ear as viewed through a 30° endoscope during surgery. There was no clear stapes footplate, but a narrow oval window (OW) niche. The round window (RW) niche was visible. The OW and RW are labeled to the right above the structures. ... The extreme ossicular abnormalities in this patient with BOR made his maximal conductive hearing loss not amenable to ossiculoplasty. We ultimately failed in our attempt to restore his conductive hearing loss due to agenesis of the oval window and lack of a mobile footplate. The intraoperative endoscopy revelaed the lack of suitable anatomy for an ossicular replacement prosthesis. The findings in our patient highlight the diagnostic findings in BOR on CT scan of the temporal bone and correlate well with his clinical findings. Although BOR patients may not be a homogeneous in terms of their middle ear anatomy, this patient suggests ossiculoplasty may not be a viable option in this population.

Conventional bone conduction hearing aid solutions often require a cumbersome retention system such as a headband or cap. This study aims to determine if a novel, nonsurgical bone conduction aid utilizing an adhesive attachment over the mastoid is equivalent to the conventional bone conduction hearing aid (BCHA) for the management of conductive hearing loss. Prospective, single-subject randomized, crossover trial. Tertiary referral center. Eleven adults and 1 child between 11 and 70 years of age with unilateral conductive hearing loss were enrolled. Patients had their baseline hearing assessed and were randomized to receive either the novel device or BCHA headband system. Hearing loss etiologies were varied and included cholesteatoma, otosclerosis, chronic otitis media, and previous head and neck surgery. Patients had their baseline hearing assessed and trialled both the novel device or the BCHA headband system. Patients were randomly assigned one device which was worn for 2 weeks followed by a 2-week trial with the alternate device. Pure-tone thresholds and speech discrimination in quiet and noise were tested and patients also completed the short form of the Speech Spatial and Quality of Hearing (SSQ) questionnaire. The mean unaided 4 frequency pure-tone average (PTA) air conduction threshold was 53.9 dB, bone conduction was 11.9 dB, and the mean air bone gap was 42 dB in the target ear. One patient with an adhesive skin reaction could not complete the protocol. Aided PTA and threshold testing between 250 Hz to 8000 Hz showed statistically equivalent results between both devices. The aided CNC word score, signal-to-noise ratio, and SSQ scores were also equivalent between both devices. The novel bone conduction aid demonstrates equivalent performance to the conventional BCHA headband solution. The novel device should be considered an alternative option where the cosmetic and comfort issues of a headband worn device are a concern.

Natural myringostapediopexy is an infrequent abnormality, and studies about resulting hearing loss are scarce. In several cases, natural myringostapediopexy may function as a Type III tympanoplasty. This study evaluated conductive hearing loss in myringostapediopexy. This cross-sectional comparative study included a historical and current sample of 46 patients, one with bilateral myringostapediopexy, at a total of 47 ears. All underwent pure tone and speech audiometry and were divided according to age into a pediatric (younger than 18 yr) and an adult group. The Statistical Package for Social Science (SPSS) 10.0 was used for statistical analysis, and the level of significance was set at p lower than 0.05. Patient distribution according to sex was similar (53.2% male), and 57.4% were adults. Mean conductive hearing loss in ears with myringostapediopexy ranged from 14.13 to 21.28 dB according to the frequency assessed. Pure tone average was 18.46 dB. A conductive hearing loss equal to or lower than 25 dB at all frequencies was found in 53% of the patients. The 2,000 and 3,000 Hz frequencies had the greatest prevalence of clinically nonsignificant conductive hearing loss (87% and 91%). Sensorineural hearing loss was found in 14 patients (30%), all adults, and 43% of the cases were mild. The comparison according to age did not reveal any significant differences in conductive hearing loss at any of the frequencies. Most patients with natural myringostapediopexy included in the study had clinically irrelevant conductive hearing loss. There were no differences in conduction loss between children and adults. The reconstruction of the ossicular chain and tympanoplasty, for purely functional reasons, are not justified in these cases, particularly not for patients with mixed hearing loss.

Idiopathic sudden sensorineural hearing loss (ISSHL) with or without tinnitus is common and presents a health problem with significant effect on quality of life. Hyperbaric oxygen therapy (HBOT) may improve oxygen supply to the inner ear and, it is postulated, may result in an improvement in hearing and/or a reduction in the intensity of tinnitus. To assess the benefits and harms of HBOT for treating ISSHL and/or tinnitus. We initially searched in June 2004 and repeated the search in June 2006. Our search included the Cochrane Ear, Nose and Throat Disorders Group Trials Register, the Cochrane Central Register of Controlled Trials (CENTRAL) (The Cochrane Library, Issue 2 2006), MEDLINE (1951 to 2006), EMBASE (1974 to 2006), CINAHL, Database of Randomised Trials in Hyperbaric Medicine (DORCTHIM), AMED, LILACS, KOREAMED, INDMED, National Research Register (NRR), CSA, ISI PROCEEDINGS and ZETOC. Randomised studies comparing the effect on ISSHL and/or tinnitus of therapeutic regimens which include HBOT with those that exclude HBOT. Three authors independently evaluated the quality of the relevant trials using the validated Oxford-Scale (Jadad 1996) and extracted the data from the included trials. Six trials contributed to this review (308 subjects). Pooled data from two trials involving 114 patients did not show any significant improvement in the chance of a 50% increase in hearing threshold on Pure Tone Average (PTA) when HBOT was used (relative risk [RR] with HBOT 1.53, 95% CI 0.85 to 2.78, P = 0.16), but did show a significantly increased chance of a 25% increase in PTA (RR 1.39, 95% CI 1.05 to 1.84, P = 0.02). There was a 22% greater chance of improvement with HBOT, and the number needed to treat (NNT) to achieve one extra good outcome was five (95% CI 3 to 20). A single trial involving 50 subjects also suggested significantly more improvement in the mean PTA threshold with HBOT, expressed as a percentage of baseline (WMD 37%, 95% CI 22% to 53%, P < 0.001). The significance of any improvement following HBOT in a subjective rating of tinnitus could not be assessed due to poor reporting. There were no significant improvements in hearing or tinnitus reported in the single study to examine chronic presentation (six months) of ISSHL and/or tinnitus. For people with early presentation of ISSHL, the application of HBOT significantly improved hearing loss, but the clinical significance of the level of improvement is not clear. We could not assess the effect of HBOT on tinnitus by pooled data analysis. The routine application of HBOT to these patients cannot be justified from this review. In view of the modest number of patients, methodological shortcomings and poor reporting, this result should be interpreted cautiously, and an appropriately powered trial of high methodological rigour is justified to define those patients (if any) who can be expected to derive most benefit from HBOT. There is no evidence of a beneficial effect of HBOT on chronic presentation of ISSHL and/or tinnitus and we do not recommend use of HBOT for this purpose based on the single study available.

Conductive hearing loss in newborns: Hearing profile, risk factors, and occasions of service.

Objective:The digits-in-noise test (DIN) has become increasingly popular as aconsumer-based method to screen for hearing loss. Current versions of allDINs either test ears monaurally or present identical stimuli binaurally(i.e., diotic noise and speech,NoSo). Unfortunately,presentation of identical stimuli to each ear inhibits detection ofunilateral sensorineural hearing loss (SNHL), and neither diotic normonaural presentation sensitively detects conductive hearing loss (CHL).Following an earlier finding of enhanced sensitivity in normally hearinglisteners, this study tested the hypothesis that interaural antiphasic digitpresentation (NoSπ) would improve sensitivityto hearing loss caused by unilateral or asymmetric SNHL, symmetric SNHL, orCHL.Design:This cross-sectional study, recruited adults (18-84 years) withvarious levels of hearing, based on a four-frequency pure tone average (PTA)at 0.5, 1, 2 and 4kHz. The study sample was comprised of listeners withnormal hearing (n=41; PTA ≤ 25 dB HL in both ears),symmetric SNHL (n=57; PTA > 25 dB HL), unilateral orasymmetric SNHL (n=24; PTA > 25 dB HL in the poorerear) and CHL (n=23; PTA > 25 dB HL and PTA air-bonegap ≥ 20 dB HL in the poorer ear). Antiphasic and diotic speechreception thresholds (SRTs) were compared using a repeated-measuresdesign.Results:Antiphasic DIN was significantly more sensitive to all three forms ofhearing loss than the diotic DIN. SRT test-retest reliability was high forall tests (ICC r > 0.89). Area under the receiveroperating characteristics (ROC) curve for detection of hearing loss(> 25 dB HL) was higher for antiphasic DIN (0.94) than for diotic DIN(0.77) presentation. After correcting for age, PTA of listeners with normalhearing or symmetric SNHL was more strongly correlated with antiphasic(rpartial[96]=0.69) than diotic(rpartial=0.54) SRTs. Slope of fittedregression lines predicting SRT from PTA was significantly steeper forantiphasic than diotic DIN. For listeners with normal hearing or CHL,antiphasic SRTs were more strongly correlated with PTA(rpartial[62]=0.92) than diotic SRTs(rpartial[62]=0.64). Slope of regressionline with PTA was also significantly steeper for antiphasic than diotic DIN.Severity of asymmetric hearing loss (poorer ear PTA) was unrelated to SRT.No effect of self-reported English competence on either antiphasic or dioticDIN among the mixed first-language participants was observedConclusions:Antiphasic digit presentation markedly improved the sensitivity ofthe DIN test to detect SNHL, either symmetric or asymmetric, while keepingtest duration to a minimum by testing binaurally. In addition, theantiphasic DIN was able to detect CHL, a shortcoming of previous monaural orbinaurally diotic DIN versions. The antiphasic DIN is thus a powerful toolfor population-based screening. This enhanced functionality combined withsmartphone delivery could make the antiphasic DIN suitable as a primaryscreen that is accessible to a large global audience.

Bone conduction systems are sometimes not well tolerated due to the tension on the skull. Therefore, a brand non-surgical device was developed, the ADHEAR (MED-EL, Innsbruck, Austria). It is based on a novel fixation system using an adhesive adapter, which allows for a more comfortable and efficient mechanical transmission of the acoustic signal and that can be used as treatment for conductive hearing loss. The purpose of the study is to evaluate the performance of ADHEAR in two patients with unilateral conductive hearing loss. An 8-years-old patient with external ear canal stenosis obtained a functional gain of 28.75 dB, while a 16-years-old patient with Goldenhar syndrome showed a recovery of 53.75 dB. Therefore, both cases suggest that ADHEAR appears to be an effective treatment for pediatric patients who due to the medical condition cannot be treated surgically or fitted with conventional hearing aids.

Sensorineural hearing loss (SNHL) has been associated with cognitive impairment and dementia. However, because SNHL contains a neural component, it is difficult to completely exclude reverse causation whereby dementia causes worse SNHL. Conductive hearing loss (CHL), a purely peripheral phenomenon, would not have this mechanistic connection. We investigate the association between CHL and dementia in a large national cohort. Cross-sectional study of ≥ 18-year-old (n = 338,048) voluntary participants from the NIH All of Us Research Program. The exposure was bilateral CHL defined by ICD-10 code (H90.0). The outcome was dementia defined by ICD-10 codes (F03, G30-32). The odds of dementia in subjects with and without CHL were assessed with multivariable regression, controlling for potentially confounding variables (age, sex, ethnicity, race, education, and smoking history), as well as surgical and medical interventions for CHL. The mean (SD, range) age was 54 years (±17). 62.9% (n = 212,623) of participants identified as female. The cohort included 393 (0.1%) individuals with CHL. 1.9% (n = 6597) had dementia. After controlling for covariates, the odds of dementia were 2.05 times (95% CI 1.18-3.32; p = 0.006) higher for those with CHL compared to those without CHL. The odds were variably reduced when surgical interventions (stapedectomy, tympanoplasty, mastoidectomy, myringoplasty) (OR = 2.03, CI 1.17-3.29; p = 0.007) or hearing devices (hearing aids, implants) (OR = 1.98, CI 1.13-3.21; p = 0.010) were added to the fully adjusted multivariable model. In the All of Us Research Program, CHL was strongly associated with dementia and treatment of CHL, both surgically and non-surgically, minimally reduced the odds of dementia.

Idiopathic sudden sensorineural hearing loss (ISSHL) with or without tinnitus is common and presents a health problem with significant effect on quality of life. Hyperbaric oxygen therapy (HBOT) may improve oxygen supply to the inner ear and thereby result in an improvement in hearing and/or a reduction in the intensity of tinnitus. To assess the benefits and harms of HBOT for treating ISSHL and tinnitus. We searched the Cochrane ENT Specialist Register (June 2004), CENTRAL (The Cochrane Library Issue 3, 2004), MEDLINE (1966 to 2004), EMBASE (1974 to 2004), CINAHL (1982 to 2004), DORCTHIM (1996 to 2004), and reference lists of articles. Researchers in the field were contacted. Randomised studies comparing the effect on ISSHL and/or tinnitus of therapeutic regimens which include HBOT with those that exclude HBOT. Three reviewers independently evaluated the quality of the relevant trials using the validated Jadad 1996 Oxford-Scale and extracted the data from the included trials. Five trials contributed to this review (254 subjects, 133 receiving HBOT and 120 control). Pooled data from two trials involving 114 patients (45% of the total) suggested there was a trend towards, but no significant increase in, the chance of a 50% increase in hearing threshold on Pure Tone Average (PTA) over four frequencies when HBOT was used (relative risk (RR) for good outcome with HBOT 1.53, 95% confidence interval (CI) 0.85 to 2.78, P = 0.16). The chance of achieving a 25% increase with HBOT was, however, statistically significant (RR 1.39, 95% CI 1.05 to 1.84, P = 0.02). Fifty-six per cent of the control subjects achieved this outcome versus 78% of the HBOT subjects, with the number-needed-to-treat (NNT) to achieve one extra good outcome being 5 (95% CI 3 to 20). A single trial involving 50 subjects (20% of the total) also suggested a significant improvement in the mean PTA threshold expressed as a percentage of baseline (61% improvement with HBOT, 24% with control, WMD 37%, 95% CI 22% to 53%). The effect of HBOT in tinnitus could not be assessed due to poor reporting. There were no significant improvements in hearing or tinnitus reported in the single study to examine the effect of HBOT on a chronic presentation (six months) of ISSHL and/or tinnitus. For people with early presentation of ISSHL, the application of HBOT significantly improved hearing loss, but the clinical significance of the level of improvement is not clear. We could not assess the effect of HBOT on tinnitus by pooled analysis. The routine application of HBOT to these patients cannot be justified from this review. In view of the modest number of patients, methodological shortcomings and poor reporting, this result should be interpreted cautiously, and an appropriately powered trial of high methodological rigour is justified to define those patients (if any) who can be expected to derive most benefit from HBOT. There is no evidence of a beneficial effect of HBOT on chronic presentation of ISSHL and/or tinnitus.

Objective: To analyze the clinical characteristics and appropriate surgical procedures, and discuss the classification of congenital middle ear malformation. Methods: All cases were from the Center of Otorhinolaryngology, the Sixth Medical Center of Department of PLA General Hospital. All of these cases, including 26 male patients (ears) , 10 female patients (11 ears) , aged from 7 to 57 years old, had normal external auditory canal, tympanic membrane, conductive hearing loss, type A tympanogram and negative Gelle's test. Tympanoplasty was performed in all cases. The deformity was classified to three types,i.e., Type I (stapes foot plate mobility): Ⅰa, ossicular chain deformity with normal stapes suprastructure; Ⅰb, ossicular chain deformity with abnormal stapes suprastructure; Type Ⅱ (stapes foot plate fixation): Ⅱ a,normal ossicular chain, Ⅱ b, ossicular chain malformation; and Type Ⅲ: vestibular window osseous atresia or undeveloped, or with round window atresia. The malformation of type Ⅱ and Ⅲ may be accompanied with abnormal facial nerve. In addition, the papers on middle ear malformation published from 1982 to 2017 were analyzed retrospectively. The clinical data of 451 ears malformation were summarized. Results: According to the revisional classification criteria in 37 ear samples from our hospital, 20 ears were type I. 6 type Ⅰa cases were used PORP (partial ossicular replacement prosthesis) to reconstruct the ossicular chain; 14 type Ⅰb cases were used TORP (total ossicular replacement prosthesis) to reconstruct the ossicular chain. For the 5 ears of type Ⅱ, 2 of which were type Ⅱ a and 3 were type Ⅱ b. 4 ear samples of type Ⅱ were implanted with Piston ossicular prosthesis, 1 was implanted with TORP in which the ossificated foot plate was removed with periosteum preserved. 12 ear samples were type Ⅲ, with vestibular window osseous atresia, facial nerve malformation, and stapes suprastructure malformation. The pistons ossicular prosthesis were implanted in vestibular window in 3 ears with facial nerve covering vestibular window partially. The surgery had to be given up in 5 ears, and TORP was implanted in 4 ears at the opening with preserved periosteum at the beginning of the tympanic scala because of facial nerve covering vestibular window totally. 30 ears with complete follow-up data had no sensorineural hearing loss and the average air-bone conduction decreased 23.3±10.7 dB (P<0.05).There were 234 ears of type Ⅰ in 451 ears of congenital middle ear malformation reported in the literature. 113 of which were type Ⅰa, the basic surgery was ossicular chain shaking and artificial or autogenous PORP implantation. Type Ⅰb was 121 ears, with autogenous or artificial TORP and PORP. Type Ⅱ was125 ears, including type Ⅱa 22 ears, Ⅱb 60 ears, and no subclassification for 43 ears. The surgery of type Ⅱ was the same as otosclerosis. The vestibular window atresia of type Ⅲ was 92 ears, the surgery of 17 ears had to be abandoned, the other ears underwent vestibular window, promontory or semicircular canal opening to reconstruct hearing with Piston, autogenous or artificial TORP. Conclusion: Referring to the classification of congenital middle ear malformation combining with appropriate surgical materials and methods, otologists can better understand and choose appropriate surgical method to the middle ear malformation.

Postoperative complications of myringotomy and tube placement often include otorrhea, tympanosclerosis, and tympanic membrane perforation. However, the incidence of sensorineural or conductive hearing loss has not been documented. Recent efforts to curb the use of preoperative audiometric testing requires documentation of this incidence. To define the incidence of conductive and sensorineural hearing loss associated with myringotomy and tube placement. A retrospective chart review of 550 patients undergoing myringotomy and tube placement was performed. A total of 520 patients undergoing 602 procedures (1204 ears), including myringotomy and tube placement, were assessed for preoperative and postoperative sensorineural and conductive hearing loss. No patient developed a postoperative sensorineural or conductive hearing loss. All patients resolved their conductive hearing loss after myringotomy and tube placement. There was a 1.3% incidence of preexisting sensorineural hearing loss. The incidence of sensorineural or conductive hearing loss after myringotomy and tube placement is negligible and the use of preoperative audiometric evaluation may be unnecessary in selected patients, but further studies need to be done to corroborate this small data set.

Context: There is a need for a quick reliable home test for detecting hearing loss (HL), especially in the present era of telemedicine. Hum test is one such which can be performed easily without instruments but not much documented in the literature. Aims: The aim is to find out the diagnostic accuracy of hum test in detecting unilateral HL. Settings and Design: Tertiary healthcare center, prospective, cross-sectional diagnostic test accuracy study. Subjects and Methods: The study included 85 controls and 85 patients with unilateral conductive or sensorineural HL (SNHL). The patients were divided into four groups-controls, simulated unilateral conductive HL (CHL) subjects, unilateral CHL, and SNHL patients. They underwent 4 tests-Weber test, Low pitch hum test, High pitch hum test, and pure-tone audiometry (PTA), the latter being the gold standard. Statistical Analysis Used: To test the statistical significance of the agreement between the hum test and the Weber test with PTA, McNemar's Chi-square test was used. Diagnostic measures such as sensitivity, specificity, positive predictive value, negative predictive value, and accuracy were computed. Results: The sensitivity (85%) and accuracy (95.2%) of Hum test were better in comparison to Weber test (70% and 88.8% respectively) in unilateral sensorineural patients. However, in the case of CHL the Weber test was more sensitive in comparison with hum test (95.6% and 83.35% respectively). Overall, the diagnostic accuracy of hum test was 92.5% when compared to the Weber test (93.5%). Conclusions: Hum test can be used as a reliable screening tool for suspected sudden HL cases.