Active Bone Conduction Implant and Adhesive Bone Conduction Device: A Comparison of Audiological Performance and Subjective Satisfaction

Abstract Introduction Atresia of the external auditory canal affects 1 in every 10 thousand to 20 thousand live births, with a much higher prevalence in Latin America, at 5 to 21 out of every 10 thousand newborns. The treatment involves esthetic and functional aspects. Regarding the functional treatment, there are surgical and nonsurgical alternatives like spectacle frames and rigid and softband systems. Active transcutaneous bone conduction implants (BCIs) achieve good sound transmission and directly stimulate the bone. Objective To assess the audiological performance and subjective satisfaction of children implanted with an active transcutaneous BCI for more than one year and to compare the outcomes with a nonsurgical adhesive bone conduction device (aBCD) in the same users. Methods The present is a prospective, multicentric study. The audiological performance was evaluated at 1, 6, and 12 months postactivation, and after a 1-month trial with the nonsurgical device. Results Ten patients completed all tests. The 4-frequency pure-tone average (4PTA) in the unaided condition was of 65 dB HL, which improved significantly to 20 dB HL after using the BCI for 12 months. The speech recognition in quiet in the unaided condition was of 33% on average, which improved significantly, to 99% with the BCI, and to 91% with the aBCD. Conclusion The aBCD demonstrated sufficient hearing improvement and subjective satisfaction; thus, it is a good solution for hearing rehabilitation if surgery is not desired or not possible. If surgery is an option, the BCI is the superior device in terms of hearing outcomes, particularly background noise and subjective satisfaction.

Alternative Inverted Middle Fossa Approach in Bonebridge Surgery. Technique, Results and Complications

Abstract Introduction The transmastoid approach is the most recommended technique to Bonebridge surgery, while in patients with bad anatomy or in the canal wall down technique, retrosigmoid or Middle Fossa Approaches are the alternative surgical options. Objective To describe a novel alternative approach called inverted middle fossa approach (IMFA) and its technique and audiological outcomes. Methods Seven patients submitted to the IMFA were included. All patients presented conductive and mixed hearing loss with bone thresholds of the audiogram > 40 dB. The audiological test was conducted pre- and postoperatively. Results A total of 5 males and 2 females, aged 13,8 years old (range 6-25 years old) were studied. The average follow-up was of 20 months (12 to 32 months). All patients presented aural atresia, except one with severe osseous-fibrous dysplasia of the temporal bone. Two patients showed bilateral compromise, three patients had associated Goldenhar and Treacher Collins syndrome. On the preoperative audiograms, air conduction (AC) thresholds showed a PTA4 (0.5, 1, 2 and 4 kHz) of 66.7 dB (standard deviation [SD] = ± 7.8), while the bone conduction thresholds reached an average of 11.2 dB (SD = ± 6.9). The postoperative thresholds did not change, and additional sensorineural damage was not observed before activation. Four weeks after surgery, all the patients were fitted with the external processor. The postoperative audiological aided exam showed AC PTA 4 thresholds of 18.9 dB (SD = ± 5.9). Conclusion The IMFA allows the nearest position of the microphone to the external auditory canal. The technique is a suitable option to the 3 classical approaches with similar rate of audiological results. More investigation is needed to determine the benefit of the novel approach compared with the others.

Analysis of Inner Ear Anomalies in Unilateral Congenital Aural Atresia Combined With Microtia

OBJECTIVES: The aim of this study was to analyze the incidence of inner ear anomalies in patients with unilateral congenital aural atresia (CAA) combined with microtia. METHODS: We retrospectively reviewed 61 patients with unilateral CAA combined with microtia who underwent high-resolution temporal bone computed tomography (TBCT) and hearing examination. Inner ear anomalies were analyzed using TBCT and evaluated according to the Jahrsdoerfer grading system, Marx classification, and extent of inferior displacement of the mastoid tegmen. RESULTS: Inner ear anomalies were observed in 14 patients (23.0%). Lateral semicircular canal (LSCC) dysplasia was the most common inner ear anomaly, with an incidence of 16.4%. The incidence was significantly higher on the pathologic side than on the unaffected side (P=0.002). All vascular anomalies were observed in the high-riding jugular bulb, with an incidence of 24.6%. The incidence was significantly higher on the pathologic side than on the unaffected side (P<0.001). LSCC dysplasia was significantly more common in patients with a lower Jahrsdoerfer score (odds ratio, 0.66; P=0.004). CONCLUSION: The incidence of inner ear anomalies was relatively high in patients with unilateral CAA combined with microtia; LSCC dysplasia was the most common anomaly and the probability of coexistence was higher in patients with a lower Jahrsdoerfer score.

A Case of the Vibrant Soundbridge Implantation to Unilateral Congenital Aural Atresia / 대한이비인후과학회지

Middle ear implantation is indicted for patients who have sensorineural, conductive or mixed hearing loss when conventional surgery or hearing aids do not producde sufficient improvement. This study reports the long-term results of an active middle ear implant, the Vibrant Soundbridge® (VSB) implantation, in a patient with unilateral congenital aural atresia (CAA). VSB was implanted for hearing rehabilitation after failed canaloplasty in a 15-year-old girl. After exposure of deformed ossicle, the floating mass tranceducer was coupled to the stapes head and subsequent subtotal petrosectomy was completed. Preoperative pure-tone air conduction threshold was 68.8 dB, which improved to 15 dB at one year of surgery. The mean aided free field speech discrimination in quiet was 98%. The speech understanding in noise evaluated by the composite score of Hearing-In-Noise test improved from SNR −2.5 dB to −5.2 dB. We present a first report of VSB implantation in a patient with unilateral CAA in Korea. The patient showed a significant hearing gain after the implantation of VSB; moreover, her hearing in noisy environment also showed improvement.

Cochlear Implantation Using the Endomeatal Approach in a Child with a Extremely Hypoplastic Mastoid / 대한이비인후과학회지

The classic surgical technique for cochlear implantation was first introduced by House in 1961. This technique includes a mastoidectomy and a posterior tympanotomy, which has been most widely used for cochlear implantation. However, this approach may result in the injury of the chorda tympani nerve or the facial nerve. To avoid the risk of the neural injury, several alternative surgical methods have been developed. The endomeatal approach is one of the several alternative techniques. We present, along with a review of literatures, a case of cochlear implantation using the endomeatal approach performed in a child with an extremely hypoplastic mastoid.

Surgical and Audiologic Comparison Between Sophono and Bone-Anchored Hearing Aids Implantation

OBJECTIVES: Bone-anchored hearing aids (BAHA) occasionally cause soft tissue problems due to abutment. Because Sophono does not have abutment penetrating skin, it is thought that Sophono has no soft tissue problem relating to abutment. On the other hand, transcutaneous device's output is reported to be 10 to 15 dB lower than percutaneous device. Therefore, in this study, Sophono and BAHA were compared to each other from surgical and audiological points of view. METHODS: We retrospectively reviewed the medical records of 9 Sophono patients and 10 BAHA patients. In BAHA cases, single vertical incision without skin thinning technique was done. We compared Sophono to BAHA by operation time, wound healing time, postoperative complications, postoperative hearing gain after switch on, and postoperative air-bone gap. RESULTS: The mean operation time was 60 minutes for Sophono and 25 minutes for BAHA. The wound healing time was 14 days for Sophono and 28 days for BAHA. No major intraoperative complication was observed. Skin problem was not observed in the 2 devices for the follow-up period. Postoperative hearing gain of bilateral aural atresia patients was 39.4 dB for BAHA (n=4) and 25.5 dB for Sophono (n=5). However, the difference was not statistically significant. In all patients included in this study, the difference of air-bone gap between two groups was 16.6 dB at 0.5 kHz and 18.2 dB at 4 kHz. BAHA was statistically significantly better than Sophono. CONCLUSION: Considering the audiologic outcome, BAHA users were thought to have more audiologic benefit than Sophono users. However, Sophono had advantages over BAHA with abutment in cosmetic outcome. Sophono needed no daily skin maintenance and soft tissue complication due to abutment would not happen in Sophono. Therefore, a full explanation about each device is necessary before deciding implantation.

Anatomic Variants on Computed Tomography in Congenital Aural Atresia and Stenosis

OBJECTIVES: To quantitatively analyzing the anatomic variants on temporal computed tomography (CT) in congenital external auditory canal stenosis (EACS), congenital aural atresia (CAA), and normal ear structure. METHODS: Through a retrospective study, we analyzed 142 temporal high-resolution CT studies performed in 71 microtia patients. The following 6 parameters were compared among the three groups: Marx classification, medial canal diameter, vertical facial nerve (VFN) anterior displacement, tegmen mastoideum position, tympanic cavity volume, and malleus-incus joint or malleus-incus complex (MIC) area. RESULTS: The results showed that the microtia distributions in the Marx classification in these three groups were significantly different, as 86% (31 of 35) of ears with major microtia (third-degree dysplasia) had an atresia, and in 54.8% (23 of 42) of the minor microtic (first-degree or second-degree) ears, the bony or cartilaginous part of the external auditory canal was stenotic. Measurement data also showed that the potential medial canal diameter of the atresia group was obviously shorter than that of the stenosis group. The VFN anterior displacement and temporomandibular joint backward-shift together lead to medial canal diameters in ears with atresic canals that is smaller than those with stenotic canals. The tegmen mastoideum position was not significantly different between the three groups. CONCLUSION: The mal-development of the external auditory canal is significantly associated with auricle and middle ear developmental anomalies. Compared with CAA ears, EACS have better development of the auricle, canal, tympanic cavity and MIC and relatively safer surgical operation except for the position of the tegmen mastoideum and the VFN.

A Case of Image-Guided Surgery in Congenital Aural Atresia / 대한이비인후과학회지

The repair of congenital aural atresia remains one of the most challenging otologic procedures because of the scarcity of surgical landmarks, the complexity and the variety of the temporal bone anatomy, and the limited space for reconstruction. The risks of facial nerve injury and profound sensorineural hearing loss following atresia surgery are common concerns. Furthermore, the rarity of the disease makes it difficult to improve surgical learning. Image-guided surgery may aid otologic surgeons in repairing atresia as it allows identifying the exact position of surgical instruments in relation to the specific anatomy. To our knowledge, there have been no published reports on image-guided atresia surgery in Korea. Herein, we report a case of congenital aural atresia, which was repaired using image-guided surgical technique.

Two Cases of Malleostapedotomy in Congenital Oval Window Atresia

Congenital anomaly of the oval window with an abnormal facial nerve course is an uncommon embryological defect, which is related to the underdevelopment of second branchial arch derivatives. Some treatments for improving hearing levels are available; these include hearing aids, vestibulotomy, neo-oval window formation, and stapes surgeries, including incudostapedotomy and malleostapedotomy. However, surgery for congenital anomalies of the oval window has rarely been described, usually in very small series of patients. We describe two cases of congenital anomalies of the oval window with aberrant facial nerve courses. One was a 40-year-old male diagnosed with unilateral congenital oval window atresia; the other was a 10-year-old male diagnosed with bilateral congenital oval window atresia. We also describe the clinical manifestations and treatment outcomes of malleostapedotomy for congenital anomalies of the oval window with aberrant facial nerve courses.

Clinical Analysis of Neonatal Microtia

PURPOSE: Neonatal microtia is an external ear deformity that takes various forms in degrees from minor deformity to aural atresia. Microtia causes not only simple deformity of the auricle but also the deformity of the middle ear or/and the internal ear leading to hearing loss. We intended to analyze the clinical manifestation of microtia in neonate period. METHODS: 17 neonates born with microtia who were admitted to Kyung Hee Hospital from January 1994 to June 2003 were evaluated for clinical characteristics including associated anomalies, outcomes, findings of temporal computer tomogram (CT) and auditory brainstem response test. RESULTS: The most common type of microtia was grade III (64.7%) followed by grade II (29.4%) and grade I (5.9%) according to the Marx classification. Temporal CT was carried out in 15 cases which showed external ear abnormalities in 7 cases, external and middle ear abnormalities in 5 cases, and external middle and internal ear abnormalities in 3 cases. 7/15 (46.7%) cases had partial hearing loss, followed by 4/15 (26.7%) cases of total hearing loss and 4/15 (26.7%) cases of normal hearing. Associated anomalies among 8/17 (47%) cases included congenital heart disease, celft palate and lip, microcephaly, micrognathia, CONCLUSION: Neonate born with microtia should be evaluated and followed closely for combined anomalies such as middle or internal ear deformities as these may lead to hearing loss affecting language and emotional development.

Incidence of Congenital External Ear Anomalies in Taegu City and Kyungpook Province / 대한이비인후과학회지

BACKGROUND AND OBJECTIVES: Congenital anomalies of the external ear occurs rarely, and has negative effects on children due to its unaesthetic appearance and hearing impairment. Thus surgical management is needed to correct the deformity and to gain hearing. Authors report the incidence of congenital anomalies of the external ear because there is no available data in Korea. MATERIALS AND METHODS: We conducted a hospital based survey and analyzed data from 9 hospitals in Taegu city and Kyungpook Province for the year of 1996. RESULTS: 1) Of the total of 33,898 births, 63 new borns were affected, and the incidence of anomaly was 18.5 per 10,000 births. 2) Incidence of male and female was 43 (68%) and 20 (32%). 3) Incidence of abnormalities is as follows: preauricular sinus was 7.0 per 10,000 births, polyotia was 4.4 per 10,000 births, lop ear was 2.1 per 10,000 births, bifid lobule was 0.9 per 10,000 births, macrotia was 0.9 per 10,000 births, Satyr ear was 0.6 per 10,000 births, microtia was 9.2 per 10,000 births, aural atresia was 3.2 per 10,000 births. 4) According to Marx's classification of the microtia, Grade I was 20, Grade II was 4, and Grade III was 8 cases.

Klippel-Feil Syndrome with Bilateral Congenital Aural Atresia and Bilateral Congenital Deafness / 대한이비인후과학회지

BACKGROUND AND OBJECTIVES: The Klippel-Feil syndrome is a congenital anomaly characterized by fusion of the cervical vertebrae. It is often associated with serious congenital anomalies of the nervous, cardivascular, respiratory, and urogenital systems. Another anomaly often associated is hearing loss. For those cases of the Klippel-Feil syndrome accompanying hearing loss, the middle and inner ear deformities were reported in some, whereas aural atresia was extremely rare. The purpose of this paper is to report the fact that Klippel-Feil syndrome can be combined with congenital aural atresia and deafness. MATERIAL & METHOD: Recently we experienced a case of Klippel-Feil syndrome in a 28 year old female. In this case, the second and third cervical vertebrae were fused. The patient also presented a short neck, cardiovascular anomaly, bilateral congenital deafness, and bilateral congenital aural atresia. Radiographical observation showed soft tissue density in the bilateral external uditory canals and stenosis of bilateral internal auditory canals, but no definite middle and inner ear anomalies in the temporal bone were found by computerized tomogram. RESULT: As operative procedures, canaloplasty and meatoplasty were performed after cholesteatoma removal from the external auditory canal. The external auditory canal has been well maintained following the surgery. CONCLUSION: For patients with the Klippel-Feil syndrome, we must do full evaluation of the whole body in order to treat first any life threatening problems. Evaluation of hearing should then be followed to implement appropriate treatment and early rehabilitation.

Congenital Aural Atresia Associated with Cholesteatoma / 대한이비인후과학회지

Congenital aural atresia (CAA) with variable degrees of microtia are combined with many other otologic or facial anomalies. Among them, cholesteatoma developed behind atretic plate is rarely reported. Since Von Luders had first reported about this condition at 1912, several authors had described CAA associated with cholesteatoma. In their reports, atretic canal cannot prevent the development of cholesteatoma. We reviewed 4 cases of CAA associated with cholesteatoma with medical records and preoperative temporal bone CTs retrospectively. All the cases were unilaterally presented and corrected by canaloplsty. Keratin materials were demonstrated at the medial side of atretic ear canal.

Surgical Experiences of Congenital Aural Atresia Analysis of 53 Cases / 대한이비인후과학회지

Congenital aural atresia requires reconstructive surgery because of its negative effect against hearing and cosmetic problem. Two surgical methods, anterior and transmastoid approaches, have been designed for the surgery and many surgeons have advocated any techniques depending on their surgical experiences and advantages of each method. We analyzed retrospectively 53 cases of congenital aural atresia operated with the use of one of both approaches, and got the clinical, audiological and surgical results as follows. 1) Postoperative dressing period was shorter in the anterior approach(34.5 days) than in the posterior one(44.2 days). 2) Hearing results are better in the anterior approach. 3) Postoperative complications include granulation tissue formation, drum lateralization, external auditory canal stenosis and temporary facial nerve weakness. And it happened more frequently in the anterior approach than the posterior one. 4) Facial nerve anormaly was noted in 16 cases(30.2%) and cholesteatoma in 5 cases(9.4%).

Surgical Management of Congenital Aural Atresia / 대한이비인후과학회지

BACKGROUND: Some authors recommend the surgical correction at early childhood in the selected cases of unilateral atresia as well as bilateral atresia. It has been enabled due to the improvement of imaging technique and it gives cosmetic satisfaction and biaural hearing. OBJECTIVES: The authors laid emphasis on the timing of canaloplasty in unilateral atresia, stage of canaloplasty, and postoperaitve hearing results. MATERIALS AND METHODS: A retrospective review of surgical results of congenital aural atresia from 1983 to 1995 in Seoul National University Hospital was performed. RESULTS: Forty four patients had unilateral atresia and 16 patients had bilateral atresia. Age range at the time of surgery was from 5.3 years to 14.6 years: average age was 8.1 years. Otoplasty was performed in 22 ears by plastic surgeons. It took precedence over canaloplasty in 17 ears. The endaural anterior approach through incision from temporal area to cavum cocha was performed in these cases. Meatal stenosis was the most common postoperative complication, but it has been decreased since the introduction of modification of anterior approach. The surgical attempts to improve hearing could not be performed in 14 ears due to various reasons. The audiologic follow-up period in the remaining 60 ears ranged from 1 year 9 months to 8 year 8 months(mean, 3 year 8 months). By analyzing final air conduction level, it was possible to attain 30 dB or better in 18(30%) of 60 cases and 40 dB or better in 36(60%) of 60 cases. CONCLUSION: The authors recommend the surgical correction at early childhood in unilateral atresia. and the otoplasty should be performed at first and it seems to be better in view of early audiologic rehabilitation that canaloplasty would be performed before the stage of elevation of rib cartilage graft. Meatal stenosis can be reduced by modification of anterior approach.