Efficacy of vibrant sound bridge in congenital aural atresia: an updated systematic review.

PURPOSE: The indications of Vibrant Soundbridge (VSB) have been expanded to include patients with conductive and mixed hearing loss due to congenital aural atresia (CAA). However, the current evidence supporting the auditory outcomes of VSB is based mainly on case reports and retrospective chart reviews. Therefore, the present systematic review aims to summarize and critically appraise the current evidence regarding the safety and effectiveness of VSB in children and adult patients with CAA. METHODS: A systematic literature search retrieved studies that evaluated the outcomes of unilateral or bilateral implantation of VSB in patients with CAA. The bibliographic search was conducted in PubMed, Scopus, EBSCO, and Cochrane Central Register of Controlled Trials (CENTRAL) databases from January 2000 to December 2022. RESULTS: Twenty-seven studies were included in the present systematic review. Overall, the speech perception after VSB was good, with a mean word recognition score (WRS) score ranging from 60 to 96.7%. The mean postoperative speech recognition threshold (SRT) after implantation ranged from 20.8 to 50 dB. The effective gain was reported in 15 studies, ranging from 31.3 to 45.5 dB. In terms of user satisfaction with VSB, the included studies showed significant improvements in the patient-reported outcomes, such as the Speech Spatial and Qualities of Hearing scale and Glasgow Hearing Aid Benefit Profile. The VSB implantation was generally safe with low incidence of postoperative complications. CONCLUSION: VSB provides significant benefits to individuals with hearing loss owing to CAA, with very good subjective outcomes and a low risk of complications.

Bilateral adhesive bone conduction devices in patients with congenital bilateral conductive hearing loss.

PURPOSE: This study aims to characterize the hearing benefits and sound localization accuracy of bilateral adhesive bone conduction devices (aBCDs) compared to unilateral devices in patients with congenital bilateral conductive hearing loss (BCHL). METHODS: Sixteen children and adolescents with congenital BCHL were enrolled and tested under four listening conditions: (1) unaided, (2) R aided: aided with a right-side aBCD, (3) L aided: aided with a left-side aBCD, and (4) B aided: aided with aBCDs on both sides. The sound field hearing threshold (SFHT, in dB hearing level [HL]) and the word recognition score (WRS) were measured. The mean absolute error (MAE) of sound source localization was calculated to assess the sound localization accuracy. RESULTS: The performance in SFHT and WRS was significantly higher in the B aided condition than that in the unaided, R and L aided conditions; moreover, no significant difference was observed between the R and L aided conditions. Concerning sound source localization, the accuracy of localization exhibited a sharp decline when using a single aBCD, while the application of bilateral aBCDs (B aided condition) resulted in a significantly improved localization accuracy as compared to the unilaterally aided conditions (both R and L); however, no significant difference was found between the unaided and B aided condition. CONCLUSION: Patients with congenital BCHL experienced suboptimal hearing benefits and manifested significant challenges in sound source localization when utilizing a single aBCD, as compared to the utilization of bilateral aBCDs.

Sound localization with bilateral bone conduction devices.

PURPOSE: To investigate sound localization in patients bilaterally fitted with bone conduction devices (BCDs). Additionally, clinically applicable methods to improve localization accuracy were explored. METHODS: Fifteen adults with bilaterally fitted percutaneous BCDs were included. At baseline, sound localization, (un)aided pure-tone thresholds, device use, speech, spatial and qualities of hearing scale (SSQ) and York hearing-related quality of life (YHRQL) questionnaire were measured. Settings to optimize sound localizing were added to the BCDs. At 1 month, sound localization was assessed again and localization was practiced with a series of sounds with visual feedback. At 3 months¸ localization performance, device use and questionnaire scores were determined again. RESULTS: At baseline, one patient with congenital hearing loss demonstrated near excellent localization performance and four other patients (three with congenital hearing loss) localized sounds (quite) accurately. Seven patients with acquired hearing loss were able to lateralize sounds, i.e. identify whether sounds were coming from the left or right side, but could not localize sounds accurately. Three patients (one with congenital hearing loss) could not even lateralize sounds correctly. SSQ scores were significantly higher at 3 months. Localization performance, device use and YHRQL scores were not significantly different between visits. CONCLUSION: In this study, the majority of experienced bilateral BCD users could lateralize sounds and one third was able to localize sounds (quite) accurately. The localization performance was robust and stable over time. Although SSQ scores were increased at the last visit, optimizing device settings and a short practice session did not improve sound localization.

Absent pyramidal eminence and stapedial tendon associated with congenital stapes footplate fixation: Intraoperative and radiographic findings.

OBJECTIVE: Report an association between congenital stapes footplate fixation (CSFF) and radiological absence of the pyramidal eminence and stapedial tendon. PATIENTS: Children and adults with intraoperatively confirmed CSFF and an absent stapedial tendon. INTERVENTIONS: Computed tomography (CT); exploratory tympanotomy with stapedotomy. MAIN OUTCOME MEASURES: Absence of a pyramidal eminence and stapedial tendon aperture identified on preoperative CT that was confirmed intraoperatively. RESULTS: Eight patients with intraoperative confirmation of CSFF and absent stapedial tendon were retrospectively identified. The average preoperative bone conduction and air conduction pure tone averages were 19.6 dB (SD 15.6 dB) and 55.9 dB (SD 23.6 dB), respectively. The average air-bone gap was 36.3 dB (SD 17.9 dB) preoperatively. In the seven patients who underwent preoperative CT, all were consistently identified to have an absent or hypoplastic pyramidal eminence and absent stapedial tendon aperture at the pyramidal eminence. In six cases, the stapedial footplate appeared normal, while in one case the footplate appeared abnormal which correlated with severe facial nerve prolapse observed intraoperatively. All eight cases underwent exploratory tympanotomy and demonstrated intraoperative stapes footplate fixation, absent stapedial tendon and either absent or hypoplastic pyramidal eminence, which correlated with preoperative CT findings. CONCLUSIONS: This study identifies a clinically pragmatic association between an absent pyramidal eminence identified on high-resolution CT and the diagnosis of CSFF. In a condition that otherwise generally lacks distinctive radiological features, the absence of a pyramidal eminence on CT in a patient with nonprogressive, congenital conductive hearing loss may strengthen clinical suspicion for CSFF.

Multicenter Study of Congenital Middle Ear Anomalies. Report on 246 Ears.

OBJECTIVES/HYPOTHESIS: Congenital middle ear anomalies represent a relatively rare condition. This study aimed to describe the characteristics and the surgical outcomes for patients with middle ear anomalies. METHODS: A multicenter study was conducted of consecutive patients with congenital middle ear anomalies who underwent primary surgical treatment between January 2008 and December 2017. Demographics, surgical procedures, and audiometric data were registered into the institutional database. Hearing changes and postoperative air-bone gap (ABG) were evaluated 1 year after surgery. RESULTS: A total of 246 patients (246 ears) (median age: 14 years, range: 4-75 years old) were included in this study. Anomalies were subdivided using the Teunissen and Cremers classification: 53 ears (22%) were categorized as class I, comprising only stapes ankylosis; 35 ears (14%) as class II, having ossicular chain anomalies with stapes ankylosis; 139 ears (57%) as class III, having ossicular chain anomalies with a mobile stapes-footplate; and 19 ears (8%) as class IV, with aplasia of the oval window. Evaluation of hearing outcomes for 198 ears with more than 1 year of follow-up revealed that good postoperative ABG (≤20 dB) was achieved in 82% of class I, 68% of class II, 74% of class III, and 23% of class IV anomalies. The postoperative ABG in class IV was significantly worse than in class I (P < .001) or class III (P < .01). CONCLUSIONS: This study demonstrated that class III anomalies comprised the majority of middle ear anomalies and surgical outcomes for class IV anomalies are unfavorable. LEVEL OF EVIDENCE: 4 Laryngoscope, 131:E2323-E2328, 2021.

Salivary Gland Choristoma of the Middle Ear in a Child: A Case Report.

Salivary gland choristoma is an extremely rare middle ear pathology. We present the case of a 10-year-old girl with unilateral conductive hearing loss. Tympanotomy showed a nonspecific middle ear mass, absence of stapes, anomaly of incus, and displaced facial nerve. It was not possible to remove the mass completely. Histology confirmed salivary gland choristoma. The hearing in this case can be improved with a bone-anchored hearing aid.

Is the Conductive Hearing Loss in NOG-Related Symphalangism Spectrum Disorder Congenital?

We describe a dominant Japanese patient with progressive conductive hearing loss who was diagnosed with NOG-related symphalangism spectrum disorder (NOG-SSD), a spectrum of congenital stapes fixation syndromes caused by NOG mutations. Based on the clinical features, including proximal symphalangism, conductive hearing loss, hyper-opia, and short, broad middle, and distal phalanges of the thumbs, his family was diagnosed with stapes ankylosis with broad thumbs and toes syndrome (SABTT). Genetic analysis revealed a heterozygous substitution in the NOG gene, c.645C>A, p.C215* in affected family individuals. He had normal hearing on auditory brainstem response (ABR) testing at ages 9 months and 1 and 2 years. He was followed up to evaluate the hearing level because of his family history of hearing loss caused by SABTT. Follow-up pure tone average testing revealed the development of progressive conductive hearing loss. Stapes surgery was performed, and his post-operative hearing threshold improved to normal in both ears. According to hearing test results, the stapes ankylosis in our SABTT patient seemed to be incomplete at birth and progressive in early childhood. The ABR results in our patient indicated the possibility that newborn hearing screening may not detect conductive hearing loss in patients with NOG-SSD. Hence, children with a family history and/or known congenital joint abnormality should undergo periodic hearing tests due to possible progressive hearing loss. Because of high success rates of stapes surgeries in cases of SABTT, early surgical interventions would help minimise the negative effect of hearing loss during school age. Identification of the nature of conductive hearing loss due to progressive stapes ankylosis allows for better genetic counselling and proper intervention in NOG-SSD patients.

3D Printing of a BAHA Protective Cap.

Mechanical feedback is one of the most common difficulties encountered when fitting hearing aids for toddlers and young children. We described the use of 3D printing to tailor a protective cap for a toddler with bilateral microtia/canal atresia to facilitate bone-anchoring hearing aid use.

Cleft Palate Patient With Conductive Hearing Loss Due to Stapes Fixation.

Congenital stapes fixation is characterized by congenital conductive hearing loss that is not progressive. This rare disease may be accompanied by additional middle ear anomalies. However, the occurrence of congenital stapes fixation with cleft palate and oligodontia was described by Gorlin et al. in 1973, and no further cases have been reported in the literature. Congenital stapes fixation was detected after the exploratory tympanotomy operation performed on a 15-year-old male patient admitted to our clinic with hearing loss, more prominent on the left. The patient also had a previous operation due to cleft lip and had congenital deficiencies in the upper and lower teeth. We present the second case report in the literature which we think belongs to this syndrome, which is defined as cleft palate, stapes fixation, and oligodontia syndrome by Gorlin et al. Laryngoscope, 131:E1279-E1281, 2021.

Isolated Incudostapedial Cholesteatomas: Unique Radiologic and Surgical Features.

OBJECTIVES: Congenital cholesteatomas originate from epithelial tissue present within the middle ear in patients with an intact tympanic membrane, no history of otologic surgery, otorrhea, or tympanic membrane perforation. They are diagnosed by a pearl-like lesion on otoscopy and computed tomography (CT) scan showing an expansile soft-tissue mass. We describe a series of patients with no prior otologic history presenting with progressive unilateral conductive hearing loss and normal otoscopy. The CT scans showed ossicular erosion without obvious soft-tissue mass. Surgery confirmed incudostapedial erosion found to be cholesteatoma. In this study, we characterize the clinical course of patients diagnosed with isolated incudostapedial cholesteatoma (IIC) and review possible pathologic mechanisms. METHODS: Retrospective review of IIC cases treated by the Department of Pediatric Otolaryngology, Rady Children's Hospital, San Diego, 2014 to 2020. Data included patient demographics, clinical features, imaging, surgical findings, and audiologic data. RESULTS: Five patients were diagnosed with IIC (3 [60%] female; mean age at presentation 10.7 years [range 5.5-16.0]). All patients presented with postlingual unilateral conductive hearing loss and normal otoscopy without any past otologic history; delay in diagnosis ranged from 4 months to several years. The CT scans showed ossicular chain erosion with an absent long process of the incus and/or stapes superstructure. All patients underwent middle ear exploration, revealing a thin layer of cholesteatoma in the incudostapedial region, confirmed by histopathology. Mean preoperative speech reception threshold was 55 dB and improved to a mean of 31 dB in the 4 patients who underwent ossicular chain reconstruction. CONCLUSION: Isolated incudostapedial cholesteatoma should be included as a possible etiology in pediatric patients with insidious onset of unilateral conductive hearing loss with normal otoscopy, unremarkable otologic history, and a CT scan showing ossicular abnormality/disruption without notable middle ear mass. These patients should be counseled preoperatively regarding the possibility of cholesteatoma and should undergo middle ear exploration with possible ossiculoplasty.

An atypical stapedial artery.

The persistence of the stapedial artery is a rare vascular anomaly. It is mostly asymptomatic but sometimes cause conductive hearing loss, pulsatile tinnitus, or vertigo. The estimated prevalence of this rare postembryonic persistence ranged from 0.02% to 0.48%. Four different anatomical forms have been identified, and their preoperative diagnostic is essential. We report the case of an incidental discovery of pharyngo-hyo-stapedial artery, the most uncommon form of persistent stapedial artery. Its per-operative finding has become rare because tomodensitometry is performed in case of conductive hearing loss. The continuous improvement of imagery resolution will probably help to revise the incidence of this malformation.

Hearing Aid Uptake in Children with Unilateral Microtia and Canal Atresia: A Comparison between a Tertiary Center and Peripheral Centers.

OBJECTIVES: To review the trialing and uptake of hearing aids in children with unilateral microtia or canal atresia, known collectively as congenital unilateral conductive hearing loss (CUCHL), observed in a tertiary hospital and local peripheral services. MATERIALS AND METHODS: A retrospective review of medical records for patients with CUCHL was conducted using data from a shared audiology database at a tertiary children's hospital. RESULTS: We identified 45 patients with CUCHL and excluded seven of them due to missing data. Of the 38 patients, 16 (16/38, 42%) did not have any subjective hearing complaints. Furthermore, 32% (12/38) of patients attended audiology at a tertiary centre and 83% (10/12) from this group trialled a hearing aid. In comparison, 46% (12/46) whose audiology care was delivered peripherally trialled aiding. Of the patients from the tertiary center, 58% (7/12) are still using a hearing aid compared to 27% (7/26) of patients from peripheral centers. CONCLUSION: Our analysis shows that patients with CUCHL are more likely to try hearing aids and continue using them if their audiology care is in a tertiary center. Allowing for a small sample size, this may indicate a health inequality. Agreeing on minimum standards for the management of patients with CUCHL or managing them in a designated center could increase consistency.

Isolated Congenital Round Window Atresia: Report of 2 Cases.

Round window atresia (RWA) is an uncommon condition and can result in a conductive hearing loss. Two cases of nonsyndromal bilateral RWA in 2 members of the same family are reported. Both cases presented with a conductive hearing loss of 20 to 30 dB. High-resolution computed tomography scanning was used to diagnose the condition. The patients were rehabilitated with hearing aids. Review of the literature has shown disappointing results in hearing improvement with cochlear fenestration in an attempt to address this condition. Patients presenting with unexplained conductive hearing loss should be offered computed tomography scanning. The cases we report add to the literature to benefit future patients in preoperative counseling and better inform management.

[Congenital middle ear malformation: clinical analysis and discussion of classification].

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.

Outcomes of the Baha Attract System combined with auricle reconstruction in mandarin speaking patients with bilateral microtia-atresia.

BACKGROUND: A transcutaneous bone-conduction hearing device (tBCHD), the Baha Attract System has been recently introduced to China, and very few studies have assessed the efficacy of this system in speakers of mandarin. OBJECTIVES: This study aims to analyze the functional and cosmetic outcomes of this system. MATERIALS AND METHODS: This prospective study included 11 patients (nine males, two females), of mean age 16 years (range 9-32 years). Seven patients were conducted the implantation simultaneously with auricle reconstruction, and the other four were before it. Auditory results were compared between unaided patients and implanted patients. Subjective satisfaction was analyzed using three questionnaires. RESULTS: The mean sound field thresholds were 65.9 ± 5.1 dB SPL unaided and 30.9 ± 4.7 dB SPL with an implanted Baha Attract System, resulting in a mean hearing gain of 35.0 ± 6.7 dB. The mean WRS scores were 47.8 ± 8.7% unaided and 92.1 ± 2.0% with the Baha Attract System, resulting in a mean improvement of 47.8 ± 8.7%. No adverse events were reported and questionnaires showed good patients satisfaction. CONCLUSIONS: The transcutaneous Baha Attract System is effective in mandarin speaking patients, and the combination of hearing rehabilitation and auricle reconstruction surgery is promising for patients with bilateral microtia-atresia.

Impact of unilateral congenital aural atresia on academic Performance: A systematic review.

BACKGROUND: Little is known about the academic performance of children with unilateral congenital aural atresia (CAA). OBJECTIVE: of review: Our objective was to summarize what is known about the academic performance of children with hearing loss by unilateral congenital aural atresia, in order to provide pragmatic recommendations to clinicians who see children with this entity. TYPE OF REVIEW: Systematic review. SEARCH STRATEGY: We conducted a systematic search in PubMed Medline, EMBASE, and Cochrane Library combining the terms "atresia" and synonyms with "unilateral hearing loss" and synonyms. Date of the most recent search was 16 May 2018. EVALUATION METHOD: Two independent authors identified studies, extracted data, and assessed risk of bias. This review was reported according to the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA). Observational studies on the academic achievements of patients of any age with unilateral conductive hearing loss of any level due to congenital aural atresia were included. We considered grade retention, special education, individualized education plans, and parental report of school performance as outcome measures for academic achievement. RESULTS: Two studies reporting on academic performance of patients with unilateral CAA, which both had a significant risk of bias. One study (n = 140) showed a grade retention rate of 3.6% (n = 5) in total. 15.7% (n = 22) needed special education, and 36.4% (n = 51) used an individualized education program. The second study, reporting on 67 patients with unilateral CAA, showed that 29.9% (n = 20) of the patients received school intervention, and 25.4% (n = 17) had learning problems. CONCLUSION: Current evidence regarding the effect of unilateral congenital aural atresia on academic performance is sparse, inconclusive and has a significant risk of bias. High quality observational studies assessing the effects of aural atresia on academic performance in these patients should be initiated.

Improved directional hearing of children with congenital unilateral conductive hearing loss implanted with an active bone-conduction implant or an active middle ear implant.

Different amplification options are available for listeners with congenital unilateral conductive hearing loss (UCHL). For example, bone-conduction devices (BCDs) and middle ear implants. The present study investigated whether intervention with an active BCD, the Bonebridge, or a middle ear implant, the Vibrant Soundbridge (VSB), affected sound-localization performance of listeners with congenital UCHL. Listening with a Bonebridge or VSB might provide access to binaural cues. However, when fitted with the Bonebridge, but not with a VSB, binaural processing might be affected through cross stimulation of the contralateral normal hearing ear, and could interfere with processing of binaural cues. In the present study twenty-three listeners with congenital UCHL were included. To assess processing of binaural cues, we investigated localization abilities of broadband (BB, 0.5-20 kHz) filtered noise presented at varying sound levels. Sound localization abilities were analyzed separately for stimuli presented at the side of the normal-hearing ear, and for stimuli presented at the side of the hearing-impaired ear. Twenty-six normal hearing children and young adults were tested as control listeners. Sound localization abilities were measured under open-loop conditions by recording head-movement responses. We demonstrate improved sound localization abilities of children with congenital UCHL, when listening with a Bonebridge or VSB, predominantly for stimuli presented at the impaired (aided) side. Our results suggest that the improvement is not related to accurate processing of binaural cues. When listening with the Bonebridge, despite cross stimulation of the contralateral cochlea, localization performance was not deteriorated compared to listening with a VSB.

Transcutaneous Osseointegrated Implants for Pediatric Patients With Aural Atresia.

Importance: Patients with aural atresia typically have maximal conductive hearing loss, which can have negative academic and social consequences. Transcutaneous osseointegrated implants (TOIs) can potentially restore hearing on the affected side. Objectives: To review the demographic, audiological, and surgical outcomes of TOI placement in pediatric patients with aural atresia and to describe a modification in incision technique in anticipation of later auricular reconstruction. Design, Setting, and Participants: This retrospective case series reviewed 41 cases of TOI placement in pediatric patients between January 1, 2014, and September 30, 2016, at Lurie Children's Microtia and Aural Atresia Clinic. Patients, all younger than 18 years and with atresia or microtia, received at least 6 months of follow-up and underwent testing before and after surgery. Main Outcomes and Measures: Patient age, indication for procedure, ear sidedness, case length, incision type, complications, and other postoperative events. Audiological outcomes before and after implantation were measured using pure-tone averages and the Hearing In Noise Test for Children, presented in variable signal to noise ratios. Results: In total, 46 TOIs were performed in 38 pediatric patients, but only 41 implantations in 34 patients were included in this study. Of the 34 patients, 13 (38%) were males and 21 (62%) were females, with a mean age of 8.9 (range, 5-17) years at the time of TOI placement. Microtia on the implanted side was present in 39 cases (95%). A modified posterior-superior scalp incision technique was used in 30 (73%) of 41 ears, all in cases of microtia. One perioperative surgical complication occurred: a seroma requiring drainage. Two patients developed minor skin irritation and erythema at the magnet site related to the overnight use of the processor, which resolved when removed while sleeping. The mean (SD; range) score for the Speech In Noise test at 5 dB signal to noise ratio improved from 75.3% (14.4%; range, 50%-92%) correct in unaided/preoperative condition to 93.6% (6.95%; range, 80%-100%) correct in the aided/postoperative condition. The mean improvement in score was 18.3% (95% CI, 10.8%-25.9%), with an effect size of 1.62 (95% CI, 0.95-2.29). The mean pure-tone averages (SD; range) similarly improved from 63.7 (13.2; range, 25-11) dB to 9.6 (4.9; range, 5-15) dB. Conclusions and Relevance: Transcutaneous osseointegrated implantation has a low complication rate among pediatric patients with atresia or microtia and can provide excellent audiological results. It should be included as a treatment option for this population of patients who meet audiological criteria.

Hearing Rehabilitation in Congenital Middle Ear Malformation.

Microtia and atresia cause significant conductive hearing loss of up to 60 dB HL. The bilateral cases suffer from severely restricted communication abilities and require immediate acoustic stimulation. There is also growing evidence that unilateral cases benefit from an early and selective stimulation of the affected side. Hearing restoration can be performed in selected cases of minor malformation by classic middle ear reconstruction. However, the majority of patients presumably benefit better from a hearing aid. There are 3 main types: active middle ear implants, active bone conduction implants and passive bone conduction implants. All implants improve speech perception, speech recognition, the signal-to-noise ratio and directional hearing. The extent varies among implants and requires further studies. Decision making on the implant type depends on the extent of malformation and hence the preoperative imaging. New scoring systems provide reliable risk stratification. Second it depends on the age of the patient. The active middle ear implants provide a selective stimulation of the affected side and are beneficial if implanted in the first years of life during the maturation period of the auditory system. In conclusion, hearing rehabilitation of congenital atresia should be performed as early as possible. This includes not only the bilateral but also the unilateral affected patients.

[Stapedo-Vestibular Ankylosis: Retrospective Study of Five Cases in São Tomé e Príncipe]. / Anquilose Estapedo-Vestibular: Estudo Retrospetivo de Cinco Casos em São Tomé e Príncipe.

INTRODUCTION: Otosclerosis is a common form of conductive hearing loss characterized by abnormal bone remodeling exclusively in the otic capsule. The prevalence of otosclerosis varies in racial populations and is described as being rare in black African populations. In this paper we aim to report five cases of clinical, and surgically confirmed, otosclerosis in black individuals, in São Tomé and Príncipe. MATERIAL AND METHODS: Since February 2011, Ear, Nose and Throat consultations and surgeries specialty have been carried out at Dr. Ayres de Menezes Hospital in cooperation with the project 'Health for all'. A retrospective analysis was undertaken of the records of all patients subjected either to stapedectomy or partial stapedectomy until February 2014. Information regarding clinical presentation, audiometric data and surgery reports was recorded. RESULTS: Five adult patients underwent stapedectomy or partial stapedectomy. All of them presented with normal otoscopy, conductive or mixed hearing loss on audiogram and normal tympanometry with absent stapedial reflexes. None of the patients had signs of infection or history of head trauma. Three cases showed improvement in the air-bone gap after surgery. The other two were lost to follow-up. DISCUSSION: We documented and surgically confirmed five cases of clinical otosclerosis in this population. A thematic review was carried out and concluded that, despite being described as a rare event in this race, available literature on this topic is not enough to state that there is lower prevalence of otosclerosis amongst the African population. CONCLUSION: Even if not common, otosclerosis cannot be disregarded as a possible cause for conductive hearing loss among the population of São Tomé and Principe.

Introdução: A otosclerose é uma causa frequente de hipoacusia de condução caracterizada pela alteração da remodelação óssea localizada exclusivamente à cápsula ótica. Diferenças rácicas são evidentes na literatura e, ao contrário dos caucasianos, as descrições na população de origem africana são raras. Neste trabalho pretende-se reportar cinco casos observados, e cirurgicamente confirmados, de otosclerose em indivíduos de raça negra, em São Tomé e Príncipe. Material e Métodos: Desde fevereiro de 2011, efetuam-se consultas e cirurgias de Otorrinolaringologia no Hospital Ayres de Menezes, em São Tomé e Príncipe, inseridas no projeto 'Saúde para todos ­ Especialidades'. Neste trabalho realizou-se um estudo retrospetivo dos doentes submetidos a estapedotomia ou estapedectomia parcial durante estas missões até fevereiro de 2014. Recolheu-se informação relativa à apresentação clínica, resultados audiométricos e relatórios cirúrgicos. Resultados: Cinco doentes, adultos, foram submetidos a procedimento cirúrgico. Todos apresentavam otoscopia normal, ausência de história de traumatismo cranioencefálico ou quadro infecioso, audiograma com hipoacusia mista ou de condução e timpanograma tipo A, sem reflexos estapédicos. Em três doentes foi possível realizar audiograma pós-operatório, verificando-se melhoria do gap aero-ósseo. Discussão: Neste trabalho documentam-se cinco casos de otosclerose clínica, e cirurgicamente confirmada, na população negra de São Tomé e Príncipe. Efetuada uma revisão temática, constata-se que, apesar de a otosclerose ser considerada rara nesta raça, os dados disponíveis parecem insuficientes para determinar se, de facto, diferentes raças têm diferentes incidências da doença. Conclusão: Apesar de rara, a otosclerose não pode ser ignorada como uma possível causa de hipoacusia de condução em São Tomé e Príncipe.