Option For Hearing Rehabilitation Research Articles

Cochlear Implantation in Children With Congenital Unilateral Deafness: A Case Series.

Cochlear implant is regarded as a treatment option for hearing rehabilitation of adults with unilateral sensorineural hearing loss. A clear benefit has been experienced in regard to speech comprehension in noise, localization, and quality of life. The aim of this study was to investigate the benefit of cochlear implantation for children with congenital unilateral hearing loss. Retrospective case series. Tertiary referral center; cochlear implant program. Ten children with congenital unilateral hearing loss. After extensive consultation with the families and intensive counseling, the children received a cochlear implant. Categories of auditory performance, speech discrimination in open set, subjective assessment by Speech, Spatial and Qualities scale questionnaire. Eight of ten children use their cochlear implant consistently on a daily basis. Two children who were equipped with an implanted device at a later age tend to nonuse of the device. The evaluation of binaural hearing in small children is still difficult and methods have to be developed to allow objective assessment.

Limitation of High Pitch Sound Perception in Nontumor Patients with Auditory Brainstem Implantation

Background and Objectives Auditory brainstem implantation (ABI) is another option for hearing rehabilitation in non-neurofibromatosis type 2 patients who cannot undergo cochlear implantation (CI). However, the average performance of ABI is worse than that of CI. We analyzed the psycho-electrical parameters of each electrode and psycho-acoustic response to different frequency sounds in nontumor patients with ABI. Subjects and Method Sixteen patients with ABI from July 2008 to May 2013 were included in the study. They were followed up for 4 to 56 months. Among them, 12 were prelingual deaf with a narrow internal auditory canal or cochlear ossification. The remaining four were postlingual deaf adults with severely ossified cochleae. We analyzed the electrical parameters [impedance, threshold level (T level), and dynamic range] of each of the 12 electrodes. We also evaluated the sound field pure-tone threshold, Ling 6 sound detection-identification test (Ling 6 test), and pitch ranking data of these patients. Results The impedance, T level, and dynamic range did not significantly differ among electrodes. However, the pure-tone threshold to sound field stimulation was elevated in the high tone area, where more variables were found than in the low frequency area. Patients could not identify /S/ and /Sh/ sounds in the Ling 6 test. The mean T level and the dynamic range of the three highest pitch-perceiving electrodes in each patient was higher and narrower, respectively, than those of the three lowest pitch-perceiving electrodes. Conclusion The nontumor patients with ABI have difficulty perceiving high pitch sound. More sophisticated penetrating type electrodes and, if possible, bimodal stimulation with CI, could be considered. Key words: Auditory brainstem implantation ㆍ Cochlear ossification ㆍ Narrow internal auditory canal ㆍ nontumor ㆍ Outcomes.

Auditory Outcomes with Hearing Rehabilitation in Children with Unilateral Hearing Loss: A Systematic Review.

Objective Options for management of unilateral hearing loss (UHL) in children include conventional hearing aids, bone-conduction hearing devices, contralateral routing of signal (CROS) aids, and frequency-modulating (FM) systems. The objective of this study was to systematically review the current literature to characterize auditory outcomes of hearing rehabilitation options in UHL. Data Sources PubMed, EMBASE, Medline, CINAHL, and Cochrane Library were searched from inception to January 2016. Manual searches of bibliographies were also performed. Review Methods Studies analyzing auditory outcomes of hearing amplification in children with UHL were included. Outcome measures included functional and objective auditory results. Two independent reviewers evaluated each abstract and article. Results Of the 249 articles identified, 12 met inclusion criteria. Seven articles solely focused on outcomes with bone-conduction hearing devices. Outcomes favored improved pure-tone averages, speech recognition thresholds, and sound localization in implanted patients. Five studies focused on FM systems, conventional hearing aids, or CROS hearing aids. Limited data are available but suggest a trend toward improvement in speech perception with hearing aids. FM systems were shown to have the most benefit for speech recognition in noise. Studies evaluating CROS hearing aids demonstrated variable outcomes. Conclusions Data evaluating functional and objective auditory measures following hearing amplification in children with UHL are limited. Most studies do suggest improvement in speech perception, speech recognition in noise, and sound localization with a hearing rehabilitation device.

Intralabyrinthine schwannomas : Surgical management and hearing rehabilitation with cochlear implants.

Intralabyrinthine schwannomas (ILS) are a rare differential diagnosis of sudden hearing loss and vertigo. In an own case series of 12 patients, 6 tumors showed an intracochlear, 3 an intravestibular, 1 a transmodiolar including the cerebellopontine angle (CPA), 1a transotic including the CPA, and 1 a multilocular location. The tumors were removed surgically in 9 patients, whereas 3 patients decided for a “wait-and-test-and-scan” strategy. Of the surgical patients, 3 underwent labyrinthectomy and cochlear implant (CI) surgery in a single-stage procedure; 1 patient had extended cochleostomy with CI surgery; 3 underwent partial or subtotal cochleoectomy, with partial cochlear reconstruction and CI surgery (n = 1) or implantation of electrode dummies for possible later CI after repeated MRI follow-up (n = 2); and in 2 patients, the tumors of the internal auditory canal and cerebellopontine angle exhibiting transmodiolar or transmacular growth were removed by combined translabyrinthine–transotic resection. For the intracochlear tumors, vestibular function could mostly be preserved after surgery. In all cases with CI surgery, hearing rehabilitation was successful, although speech discrimination was limited for the case with subtotal cochleoectomy. Surgical removal of intracochlear schwannomas via partial or subtotal cochleoectomy is, in principle, possible with preservation of vestibular function. In the authors’ opinion, radiotherapy of ILS is only indicated in isolated cases. Cochlear implantation during or after tumor resection (i. e., as synchronous or staged surgeries) is an option for hearing rehabilitation in cartain cases and represents a therapeutic approach in contrast to a “wait-and-test-and-scan” strategy.

X-linked Malformation and Cochlear Implantation.

To evaluate if cochlear implantation is safe and constitutes an option for hearing rehabilitation of children with x-linked inner ear malformation. Retrospective patient review in combination with a multidisciplinary follow-up. Tertiary referral hospital and cochlear implant program. Ten children with severe-profound mixed hearing loss and radiological findings consistent with Incomplete Partition type 3 cochlear malformation received cochlear implants during the years 2007 to 2015. Nine of the children had a mutation affecting the gene POU3F4 on Xq21. Cochlear implantation. Surgical events, intraoperative measures and electrical stimulation levels, hearing and spoken language abilities. In all, 15 cochlear implantations were performed. In three cases the electrode was found to be in the internal auditory canal on intraoperative x-ray and repositioned successfully. One child had a postoperative rhinorrhea confirmed to be cerebrospinal fluid but this resolved on conservative treatment. No severe complications occurred. Postoperative electrical stimulation levels were higher in 9 of 10 children, as compared with typically reported average levels in patients with a normal cochlea. Eight patients developed spoken language to various degrees while two were still at precommunication level. However, speech recognition scores were lower than average pediatric cases. Cochlear implantation is a safe procedure for children with severe-profound mixed hearing loss related to POU3F4 mutation inner ear malformation. The children develop hearing and spoken language but outcome is below average for pediatric CI recipients.

Erratum to: Stability, survival, and tolerability of a 4.5-mm-wide bone-anchored hearing implant: 6-month data from a randomized controlled clinical trial

The objective of this study was to compare the stability, survival, and tolerability of 2 percutaneous osseointegrated titanium implants for bone conduction hearing: a 4.5-mm diameter implant (test) and a 3.75-mm diameter implant (control). Fifty-seven adult patients were included in this randomized controlled clinical trial. Sixty implants were allocated in a 2:1 (test-control) ratio. Follow-up visits were scheduled at 7, 14, 21, and 28 days; 6 and 12 weeks; and 6 months. At every visit, implant stability quotient (ISQ) values were recorded by means of resonance frequency analysis (RFA) and skin reactions were evaluated according to the Holgers classification. Implants were loaded with the bone conduction device at 3 weeks. Hearing-related quality of life was evaluated using the Abbreviated Profile of Hearing Aid Benefit (APHAB), the Glasgow Benefit Inventory (GBI), and the Glasgow Health Status Inventory (GHSI). ISQ values were statistically significantly higher for the test implant compared to the control implant. No implants were lost and soft tissue reactions were comparable for both implants. Positive results were reported in the hearing-related quality of life questionnaires. These 6-month results indicate that both implants and their corresponding hearing devices are safe options for hearing rehabilitation in patients with the appropriate indications. Loading at 3 weeks did not affect the stability of either implant.

Stability, survival, and tolerability of a 4.5-mm-wide bone-anchored hearing implant: 6-month data from a randomized controlled clinical trial.

The objective of this study was to compare the stability, survival, and tolerability of 2 percutaneous osseointegrated titanium implants for bone conduction hearing: a 4.5-mm diameter implant (test) and a 3.75-mm diameter implant (control). Fifty-seven adult patients were included in this randomized controlled clinical trial. Sixty implants were allocated in a 2:1 (test–control) ratio. Follow-up visits were scheduled at 7, 14, 21, and 28 days; 6 and 12 weeks; and 6 months. At every visit, implant stability quotient (ISQ) values were recorded by means of resonance frequency analysis (RFA) and skin reactions were evaluated according to the Holgers classification. Implants were loaded with the bone conduction device at 3 weeks. Hearing-related quality of life was evaluated using the Abbreviated Profile of Hearing Aid Benefit (APHAB), the Glasgow Benefit Inventory (GBI), and the Glasgow Health Status Inventory (GHSI). ISQ values were statistically significantly higher for the test implant compared to the control implant. No implants were lost and soft tissue reactions were comparable for both implants. Positive results were reported in the hearing-related quality of life questionnaires. These 6-month results indicate that both implants and their corresponding hearing devices are safe options for hearing rehabilitation in patients with the appropriate indications. Loading at 3 weeks did not affect the stability of either implant.

Auditory brainstem implant candidacy in the United States in children 0–17 years old

ObjectivesThe auditory brainstem implant (ABI) is an option for hearing rehabilitation in profoundly deaf patients ineligible for cochlear implantation. Over the past decade, surgeons have begun implanting ABIs in pediatric patients who are unable to receive cochlear implants due to congenital or acquired malformations of the inner ear. No study has examined the potential population-level demand for ABIs in the United States (US). Herein, we aim to quantify the potential need for pediatric ABIs. MethodsA systematic literature review was conducted to identify studies detailing the rates of congenital cochlear and/or cochlear nerve (CN) anomalies. Absolute indications for ABI include bilateral cochlea or CN aplasia (Group A), and relative indications for ABI include bilateral cochlea or CN hypoplasia (Group B). Data was subsequently correlated to the US Census Bureau, the National Health Interview Survey, and the Gallaudet Research Institute to provide an estimation of pediatric ABI candidates. ResultsEleven studies documented rates of bilateral findings. Bilateral cochlea aplasia was identified in 0–8.7% of patients and bilateral CN aplasia in 0–4.8% of patients (Group A). Bilateral cochlea hypoplasia was identified in 0–8.7% of patients and bilateral CN hypoplasia in 0–5.4% of patients (Group B). Using population-level sensorineural hearing loss data, we roughly estimate 2.1% of potential implant candidates meet absolute indications for an ABI in the United States. ConclusionCongenital cochlear and cochlear nerve anomalies are exceedingly rare. This study provides the first preliminary estimate of cochlea and CN aplasia/hypoplasia at the population level albeit with limitations based on available data. These data suggest the need for dedicated ABI centers to focus expertise and management.

Contemporary hearing rehabilitation options in patients with aural atresia.

Congenital aural atresia is the failure of development of the external auditory canal. It usually occurs in conjunction with microtia, which is the malformation of the auricle due to a failure of development of the external ear. Aural atresia, with or without microtia, may significantly affect the hearing and social life of the patients. It is important for every medical practitioner to be aware of the possible treatment options for hearing rehabilitation in this group of patients. In the era of modern technology, new choices, including Bone-Anchored Hearing Aid (BAHA) (Cochlear Ltd. and Oticon Medical), Vibrant Soundbridge (VSB) (MED-EL, Innsbruck, Austria), and Bonebridge system (BB) (MED-EL, Innsbruck, Austria), provide high-end alternatives to traditional Bone Conduction Hearing Aid and Auditory Canal Reconstruction. All these options have advantages and disadvantages, and they are appropriate for different patients and/or at different ages. This paper aims to provide an overview of the management of hearing rehabilitation in congenital aural atresia patients and a discussion of each treatment option.

Outcomes of cochlear implantation in children with CHARGE syndrome

Conclusion: Regardless of the extent of inner ear anomalies and intellectual faculties, cochlear implantation (CI) with careful treatment planning can be a highly effective option for hearing rehabilitation in children with sensorineural hearing loss and CHARGE syndrome. Objective: CHARGE is a mnemonic term for coloboma, heart defects, choanal atresia, retarded growth and development, genital abnormalities, and ear anomalies. CHARGE syndrome is one of the leading causes of congenital deafness and blindness in children. We evaluated the language performance of six pediatric patients with CHARGE syndrome. Methods: Six patients with CHARGE syndrome underwent CI at the Asan Medical Center in Seoul between 2002 and 2012. The mean age of these CI patients was 4.9 years (range 2–9.8 years). All six patients had inner ear malformations; five children had an auricle anomaly and otitis media with effusion; three of these cases showed ossicle anomalies and four patients had downward displacement of the facial nerve (FN) toward the promontory; five patients showed delayed development; four of these children had cardiovascular malformations; three patients had cryptorchidism; two had choanal atresia; and there was one case of coloboma. Results: In the initial operations, two patients each were implanted with a CI512, CI24R, or CI24RE device. One patient initially received a CI512 device in the right ear, followed by a CI24RE device in the left ear. Another patient (case 1) first received a CI24R and then CI24RST device in the right ear due to poor performance, but this was eventually removed due to exposure of the electrode from adhesive otitis media and continuing poor performance. Assessment of the meaningful auditory integration scale (MAIS) showed that four patients reached a score above 95% and one patient achieved 60%. The categories of auditory performance (CAP) score showed that one patient reached CAP 7 at 2 years postoperatively and two cases showed CAP 5.5 at 1 year after implantation, while the remaining two patients had CAP scores of 3 at 12 months and 4.5 at 4 years, respectively. The speech intelligibility rating (SIR) score at 18 months post-surgery was 4.5 in two patients, 2.5 in two patients, and 1.5 in one case. The remaining patient (case 1) who was explanted showed no meaningful speech improvement.

Auditory Brainstem Implantation in Children

Until last decade, use of auditory brainstem implant (ABI) was restricted to adults and children with severe inner ear malformations and cochlear nerve aplasia did not have any option for hearing rehabilitation. Use of ABI opened a new era for this group of patients. In this review article, definite and probable indications of ABI for children are stated. Among probable indications, children with hypoplastic nerves constitute the most challenging group in decision making between cochlear implant (CI) and ABI. In these patients, radiological findings should be utilized together with preoperative and intraoperative audiological evaluation. However, none of these assessment methods offer enough data to enable correct choice between CI and ABI. Intracochlear electrical ABR (eABR) seems to a better indicator compared to preoperative electrophysiological tests. Commonly, CI is the first choice in these patients; if benefit with CI is not satisfactory during follow up, then ABI is utilized. Further definitive diagnostic tools are require in order to overcome this uncertainty. The ABI team must be experienced on pediatric CI patients, also an experienced pediatric neurosurgeon is indispensable to achieve success and to avoid complications. Age limit for ABI is similar to CI candidate children. Better language outcome is expected at younger ages, i.e., between 1 and 2 years. Preoperative radiological work up involves both high resolution computed tomography and magnetic resonance imaging. The side with less severe inner ear malformation or with more developed neural structures can be preferred. In preoperative audiological evaluation, subjective tests should always be included in test battery as well as objective ones. Rehabilitative assessment is focused on auditory perception skills, language and speech skills and learning abilities. Surgical method of choice is retrosigmoid approach in children. Intraoperative eABR measurements are important to determine correct position of electrode. Interindividual variability in language development and audiological outcome is prominent. Overall complication rate is in an acceptable range when surgery is performed with an experienced team. In conclusion, ABI can provide satisfactory audiological outcomes and language development to a certain extent is possible when every aspect of whole process is applied properly.

Congenital Oval or Round Window Anomaly With or Without Abnormal Facial Nerve Course

To describe the audiometric results in a consecutive series of patients with congenital ossicular aplasia (Class 4a) or dysplasia of the oval and/or round window (Class 4b), which might include a possible anomalous course of the facial nerve. Retrospective chart study. Tertiary referral center. A tertiary referral center study with a total of 14 patients with congenital minor ear anomalies as part of a consecutive series (n = 89) who underwent exploratory tympanotomies (15 ears). Audiometric results. In 8 of 15 ears, ossicular reconstruction was attempted. In the short term (1 mo), there was a serviceable hearing outcome (air-bone gap closure to within 25 dB) in 4 ears. However, the long-term results showed deterioration because of an increased air-bone gap in all but 1 ear. No facial nerve lesion was observed postoperatively. Congenital dysplasia or aplasia of the oval and/or round window is an uncommon congenital minor ear anomaly. Classical microsurgical opportunities are rare in this group of anomalies. Newer options for hearing rehabilitation, such as the osseointegrated passive bone conduction devices, have become viable alternatives for conventional air conduction hearing devices. In the near future, upcoming active bone conduction devices might become the most preferred surgical option. In cases in which the facial nerve is only partially overlying the oval window, a type of malleostapedotomy procedure might result in a serviceable postoperative hearing level.

Speech Perception in Auditory Brainstem Implants in Neurofibromatosis Type 2

Background: In bilateral auditory nerve destruction by NF2, auditory brainstem implants (ABIs) are the only option for hearing rehabilitation.

Does Preoperative Hearing Predict Postoperative Hearing in Patients Undergoing Primary Aural Atresia Repair?

The purpose of this study is to explore the correlation between preoperative hearing and early postoperative hearing results in patients undergoing primary aural atresia repair. Retrospective review of 125 patients. Academic tertiary referral center. One hundred twenty-five patients (5-67 yr old) undergoing 133 primary aural atresia surgeries were included. Spearman correlation coefficients were calculated between preoperative and postoperative (mean, 7.5 wk; range, 3-40 wk after surgery) hearing outcome measures including 3-tone pure-tone average (PTA), speech reception threshold (SRT), speech discrimination scores (SDS), air-bone gap (ABG), change in ABG (ΔABG), and between preoperative SRT and Jahrsdoerfer score. Preoperative PTA, SRT, SDS, and ABG correlated strongly with their respective postoperative values (correlation coefficients rho of 0.356 [p < 0.01], 0.199 [p < 0.05], 0.480 [p < 0.01], and 0.223 [p < 0.05], respectively). Preoperative PTA (0.407; p < 0.01), SRT (0.348; p < 0.01), SDS (-0.247; p < 0.01), and ABG (0.514; p < 0.01) also were correlated with ΔABG. When postoperative results were dichotomized to either normal (SRT, <30dB HL) or abnormal (SRT, ≥30dB HL), preoperative SRT was found to be a positive predictor of normal postoperative hearing (p = 0.05). Probability of normal postoperative hearing was 66% when preoperative SRT was 50 dB HL or lower and 40% when greater than 60 dB HL. Preoperative hearing (SRT) also trended toward a correlation with Jahrsdoerfer score (-0.168 [p = 0.058]). Among patients undergoing primary atresia repair, better preoperative hearing strongly predicts better postoperative hearing and correlates with ear anatomy. Preoperative hearing status should be factored when counseling atresia patients on hearing rehabilitation options.

Contemporary options for lateral skull base reconstruction following tumor extirpation

This review is timely and relevant for several reasons. More lateral skull base tumors are being diagnosed, as the threshold to image patients with lower cranial nerve symptoms is very low. Patients are living longer and can potentially have more comorbidities that can affect wound healing. More surgical teams are attempting lateral skull base surgery. Patients with benign and malignant tumors of the lateral skull base may also be treated with radiotherapy and chemotherapy, which can have implications for wound healing. Abdominal fat grafting continues to be a viable option for closure of defects resulting from transtemporal skull base surgery. Hydroxyapatite cranioplasty remains controversial due to a high incidence of delayed infection. For malignant lateral skull base lesions such as advanced temporal bone and parotid malignancies, free tissue transfer is increasingly utilized, as it is reliable, is harvested outside the surgical field, and provides superior wound closure. Bone anchored hearing aids (BAHAs) can be a good option for hearing rehabilitation, whereas vistafix can reliably address auricular deformities. Proper reconstruction after tumor resection in skull base surgery is important to prevent postoperative complications. This article will address contemporary reconstruction options including primary closure, abdominal fat grafting, synthetic materials, microvascular free tissue transfer, BAHA and vistafix.

Power Stapes

To present power stapes, stapedotomy, and middle ear implantation with Vibrant SoundBridge (VSB) performed in a one-step surgery as an alternative option for hearing rehabilitation in patients with osteogenesis imperfecta (OI). Retrospective case series. Tertiary referral ear center. A family with genetically proven OI Type I. Two patients, mother and son, with severe to profound mixed hearing loss underwent 3 power stapes, 1 unilateral and 1 bilateral sequential. Thorough audiological diagnostic batteries including aided and unaided pure-tone and free-field audiometry and Freiburg monosyllabic word test were used to assess the preoperative status and the postoperative hearing outcome. High-resolution computed tomography of the temporal bones was performed as well. Surgical procedure and any special considerations were analyzed in detail. The hearing outcome was favorable in all cases, showing in comparison to the preoperative values an average improvement of 36.8 dB. Severe intraoperative bleeding of the middle ear mucosa was the only complication and could be easily controlled by allowing short time intervals. Inner ear trauma did not occur in any case. Power stapes represents a safe and promising procedure for treating hearing loss in selected patients with OI. Furthermore, it introduces a new, advantageous VSB application in cases of mixed hearing loss with severe otosclerosis and increased bone-conduction thresholds.

Options for hearing rehabilitation in children with cochleovestibular nerve dysplasia

Introduction Nowadays, children with limited abilities to develop open-speech understanding are considered as cochlear implant candidates. Children with anatomical abnormalities of the inner ear and particularly with deficiency of the cochleovestibular nerve (CVN) represent such great challenges. The achievement of speech development in these patients may be impossible (Nikolopoulos and Kiprouli, 2004). In 1997, Casselman et al. described for the first time the malformations of CVN, with the help of magnetic resonance imaging (MRI). A classification of abnormalities of three types, based on embryologic knowledge, was proposed. Type one includes aplasia of the CVN which is associated with stenosis of the internal auditory canal. Type two describes common CVN with aplasia or hypoplasia of its cochlear branch and finally type three refers to the presence of a common CVN with aplasia or hypoplasia of the vestibular branch (Casselman et al., 1997). Due to the wide use of MRI, CVN dysplasias were better identified and examined (Adunka et al., 2006). In this way, different methods of diagnostic assessment and therapeutic intervention were suggested in case series, presenting each of them one sole solution to the problem, either cochlear or auditory brainstem implantation (Govaerts et al., 2003; Colletti and Zoccante, 2008; Song et al., 2010). Systematic approach and universally established algorithms are still missing, though. The aim of this study was to present a variety of diagnostic and treatment options when dealing with CVN dysplasia.

Cochlear Implantation Outcomes in Patients With Autoimmune and Immune-Mediated Inner Ear Disease

To compare hearing outcomes in patients with autoimmune inner ear disease (AIED) undergoing cochlear implantation to a group of controls, postlingually deafened by non-immune-mediated causes. Hearing performance in AIED patients who receive unilateral or bilateral cochlear implants is comparable to similarly treated postlingually deafened adults without AIED. Retrospective chart review. Academic neurotologic tertiary referral center. Ten patients with AIED with 12 implanted ears who met the audiological criteria for cochlear implantation were compared with 12 randomly selected controls, postlingually deafened by non-immune-mediated causes. Cochlear implantation using commercially available devices. Preoperative and postoperative hearing thresholds, words, and sentence scores. A note was made regarding the presence or absence of ossification or fibrosis noted within the scala tympani at the time of implant. The mean age was 49.6 ± 14 years in the AIED group and 56.8 ± 17 years in the control group (p = 0.31). The mean duration of deafness was 14 ± 26 months in the AIED group and 6.5 ± 4 months in the control group (p = 0.34). In the AIED group, 42% were men and 58% were women. In the control group, 33% were men and 67% women. Five patients in the AIED group (6 implanted ears) were found to have cochlear fibrosis and variable degrees of ossification. Two patients in that group required drill-out procedures. All patients had full insertion. The mean preoperative pure-tone averages in the AIED and control groups were 102 ± 18 and 90 ± 13 dB, respectively (p = 0.13). In the AIED, the mean short-term (≤12 mo of follow-up) postoperative word and sentence scores were 74.8% ± 15% and 94% ± 6%, respectively. In the control group, the mean short-term postoperative words and sentence scores were 72% ± 12% and 96% ± 4%, respectively. No statistical difference was present in the short-term postimplantation words (p = 0.7) and sentence scores (p = 0.49) between both groups. The mean long-term (after 12 mo of follow-up) postoperative word and sentence scores in the AIED group were 87.2% ± 11% and 96.8% ± 4%, respectively. In the control group, the long-term words and sentence scores were 77.2% ± 14% and 77.2% ± 7%, respectively. No statistical significance was found in the long-term postimplantation words (p = 0.17) and sentence scores (p = 0.7) between both groups. Cochlear implantation is a safe and viable option for hearing rehabilitation in patients deafened by progressive AIED. Hearing outcomes in AIED patients are excellent and support transition to implantation when hearing is lost or long-term steroid therapy becomes undesirable. Cochlear fibrosis or ossification seems to affect nearly 50% of implanted ears (41.6% of patients) in this study and implies that the cochlea is at risk for ossification changes long term. In appropriate candidates, earlier implantation may be indicated before postinflammatory obliterative changes in the cochlea.

Hearing Rehabilitation Counseling for Patients With Otosclerosis-Related Hearing Loss

To standardize preoperative counseling for stapedectomy candidates. Retrospective case review. Tertiary referral center. Preoperative and postoperative hearing thresholds of 54 patients (55 stapedectomies) were retrospectively recorded. Patients (age range, 17-67 yr) were divided into 3 groups that differed significantly in their mean preoperative air conduction (AC) pure-tone averages (PTA) (up to 50, 50-70, and 70-90 dB). Comparison of the gap between the mean hearing thresholds achieved postoperatively and the target threshold (normal hearing) in the 3 groups. Preoperative and postoperative AC thresholds, bone-conduction (BC) thresholds, and air-bone gap at 0.5, 1.0, 2.0, and 4.0 Hz. Patients in the group with a mean preoperative AC below 50 dB had excellent postoperative results and achieved normal hearing thresholds. In the group with a mean preoperative AC of 50 to 70 dB, the postoperative results were good, but normal hearing thresholds were not achieved. Postoperative results in the third group were within the range of moderate hearing loss, allowing these patients to use hearing aids much more successfully than preoperatively. In each of the 3 groups, mean group differences between the preoperative and the postoperative values of AC-PTA thresholds, BC-PTA thresholds, and air-bone gap were statistically significant. Comparisons between each pairing of the stratified groups also yielded statistically significant differences. The preoperative AC-PTA threshold value can be viewed as a convenient, valid, and standardized basis for better informed and more comprehensive counseling of stapedectomy candidates with regard to options for hearing rehabilitation.

Neurofibromatosis type 2 and auditory brainstem implantation

Neurofibromatosis type 2 and auditory brainstem implantation