Radiological Features and Pathognomonic Sign of Stapes Footplate Fistula in Inner Ear Malformations

Objective:Some inner ear malformations may cause recurrent meningitis, which may be fatal. The etiology is usually a stapes footplate fistula which enables microorganisms to pass into the inner ear containing cerebrospinal fluid (CSF), causing repeated attacks of meningitis. Radiological signs of the fistula are not obvious and are not reported in detail in the literature. The aim of the study is to investigate the radiological features of stapes footplate fistula in inner ear malformations.Methods:Radiological findings were analyzed for seventeen patients with inner ear malformations (IEMs) operated on because of recurrent meningitis. Using this information, images of 1,010 patients with IEMs were retrospectively reviewed to investigate the radiological findings of stapes footplate fistula and their relationship to IEMs. They were classified according to the Sennaroglu classification system, and according to different stages of stapes footplate fistula.Results:In the case of a stapes footplate cyst, computerized tomography shows an opacity at the oval window. On magnetic resonance imaging, a fluid filled cystic structure continuous with and having similar signal characteristics to the CSF in the inner ear is a pathognomonic finding of a stapes footplate cyst. It is most commonly found in common cavity anomaly (18.2%); the second most frequent finding is incomplete partition type I (15%). And it can even be seen in cases of cochlear aplasia where only the vestibule is present.Conclusion:If the history reveals recurrent meningitis, particular attention should be given to the oval window area, where an opacity, cyst or a leaking lesion should be looked for on the imaging. It is the responsibility of the otolaryngologist to notice these findings, and to operate on the patient to prevent further attacks of meningitis

Stapes Surgery Outcomes: The Practice of 35 Years

Objective: The objective of this study was to review patients who underwent stapes surgery in the Hacettepe University Ear Nose Throat and Head and Neck Surgery Department with subgroups, such as type of surgery, prosthesis used in the surgery, rate of revision, and audiological results.Methods: The data of 35 years were searched, and it was detected that 327 patients (190 females, 137 males) had undergone stapes surgery; the age was ranging between 11-70 years (mean: 39).In our clinic, stapes surgery is performed mostly by transmeatal incision. After entering the middle ear, we almost always look for stapes mobility. If there is mobility in the incus and malleus and no mobility in the stapes, a small fenestra is performed as stapedotomy and prosthesis and placed between the stapedotomy fenestra and incus long arm. To cover the space near the stapedotomy, small bony fragments are placed. In patients who had preoperative and postoperative audiograms, the mean value of 500, 1000, 2000, and 4000 Hz air-bone conduction thresholds of 199 cases were measured and compared.Results: In 62% of 327 patients who were operated on, the air-bone gap was less than 10 dB, less than 15 dB in 88%, and less than 20 dB in 94%. In 6% of patients, there was air-bone conduction gap of more than 20 dB. Revision surgery was performed in 20 patients. Of them, air-bone gap closure was achieved in 60%. One patient had sudden sensorineural hearing loss. There was also a perilymph fistula in one patient who had vertigo.Stapedotomy and using a Teflon piston with autogenous bone are successful methods of recovering conductive- type hearing loss in otosclerosis. In patients with advanced sensorineural hearing loss, a very low air-bone gap or unmeasured air-bone gap is not a contraindication.Conclusion: Stapes surgery (stapedectomy or stapedotomy) is a successful surgery in the case of conductive- type hearing loss with otosclerosis. Complications, such as total sensorineural hearing loss, facial nerve paralysis, and perilymph fistula, could be seen at variable rates, and the surgeon should be cautious, and the patients must be informed about these complications in the pre-operative period

Management of Far-Advanced Otosclerosis: Stapes Surgery or Cochlear Implant

Objective: The aim of this report is to share our experience and treatment outcomes with far-advanced otosclerosis (FAO) patients.Methods: Patients that underwent surgery from 2003 through 2014 at a tertiary referral center were retrospectively reviewed. Nineteen FAO patients were included in the study. Audiological results and the ability to communicate face to face and over telephone were considered as the main outcome measures.Results: Six FAO patients benefited well from stapedotomy with an average of 5.9-decibel (dB) air-bone gap and 86% median speech discrimination. Cochlear implantation (CI) was performed in 13 patients; two had disease progression after stapedotomy, five had failed stapes surgeries elsewhere, and six preferred CI as primary treatment. Median speech discrimination score of CI patients was 78.4%. Overall, all patients had satisfactory face-to-face communication and 90% could use telephone.Conclusion: Bilateral stapedotomy and wearing hearing aid is an effective and cost-effective solution for restoring natural binaural hearing and requires no specific training. Should stapedotomy fail, cochlear implantation is always a successful back-up option

Evaluation of Objective Test Techniques in Cochlear Implant Users With Inner Ear Malformations

Objective: The aim of the study was to compare results of objective test techniques in cochlear implant users with inner ear malformations and incomplete partition anomalies with types I and II and to show which techniques should be used in the evaluation and fitting of cochlear implant users with inner ear malformations

Factors affecting phoneme discrimination in children with sequential bilateral cochlear implants

Objectives To investigate the effects of a number of variables on phoneme discrimination (PD) performance in children with sequential bilateral cochlear implants (SeqBiCIs) and compare PD performance between the 2 implantation sides and between children with bilateral cochlear implants (BiCIs) and their age-matched peers with normal hearing (NH). Design All participants completed the Auditory Speech Sound Evaluation Phoneme Discrimination Test. Study sample The sample included 23 children with SeqBiCIs as the study group and 23 with NH as the control group. Results A significant difference was found between the scores of the two groups under the CI1 and CI2 conditions (p = 0.001), CI1 and BiCI conditions (p = 0.002), and CI2 and BiCI conditions (p = 0.001). PD scores with CI1 significantly depend on age at CI1 and duration of bilateral use. PD scores with CI1 were significant predictors of PD performance with CI2. Duration of BiCI use was a significant predictor of PD scores with BiCI. Conclusions The age at CI1 and the duration of bilateral cochlear implant use were found to improve phoneme discrimination performance in children with a sequential bilateral cochlear implant. According to the success of the CI1, it is possible to predict the success of CI2 use

Classification And Current Management Of Inner Ear Malformations

Morphologically congenital sensorineural hearing loss can be investigated under two categories. The majority of congenital hearing loss causes (80%) are membranous malformations. Here, the pathology involves inner ear hair cells. There is no gross bony abnormality and, therefore, in these cases high-resolution computerized tomography and magnetic resonance imaging of the temporal bone reveal normal findings. The remaining 20% have various malformations involving the bony labyrinth and, therefore, can be radiologically demonstrated by computerized tomography and magnetic resonance imaging. The latter group involves surgical challenges as well as problems in decision-making. Some cases may be managed by a hearing aid, others need cochlear implantation, and some cases are candidates for an auditory brainstem implantation (ABI). During cochlear implantation, there may be facial nerve abnormalities, cerebrospinal fluid leakage, electrode misplacement or difficulty in finding the cochlea itself. During surgery for inner ear malformations, the surgeon must be ready to modify the surgical approach or choose special electrodes for surgery. In the present review article, inner ear malformations are classified according to the differences observed in the cochlea. Hearing and language outcomes after various implantation methods are closely related to the status of the cochlear nerve, and a practical classification of the cochlear nerve deficiency is also provided.PubMedWoSScopu

Effects of Age at Auditory Brainstem Implantation: Impact on Auditory Perception, Language Development, Speech Intelligibility

Objective: To study the effect of age at auditory brainstem implant (ABI) surgery on auditory perception, language, and speech intelligibility. Study design: Retrospective single cohort design. Setting: Tertiary referral center. Patients: In this study, 30 pediatric ABI users with no significant developmental issues were included. Participants were divided into two groups, according to age at surgery (Early Group: < 3 yr old [n = 15], Late Group: ≥ 3 yr old [n = 15]). Groups were matched by duration of ABI use and participants were evaluated after 5 years (±1 yr) experience with their device. The mean age at ABI surgery was 22.27 (ranged ± 6.5) months in the early group, 45.53 (ranged ± 7.9) months in the late group. Intervention(s): Retrosigmoid craniotomy and ABI placement. Main outcome measure(s): Auditory perception skills were evaluated using the Meaningful Auditory Integration Scale and Categories of Auditory Performance from the Children's Auditory Perception Test Battery. We used a closed-set pattern perception subtest, a closed-set word identification subtest, and an open-set sentence recognition subtest. Language performance was assessed with the Test of Early Language Development and Speech Intelligibility Rating, which was administered in a quiet room. Results: In this study, the results demonstrated that the Early Group's auditory perception performance was better than the Late Group after 5 years of ABI use, when children had no additional needs (U = 12, p < 0.001). Speech intelligibility was the most challenging skill to develop, in both groups. Due to multiple regression analysis, we found that auditory perception categories can be estimated with speech intelligibility scores, pattern perception scores, receptive language scores, and age at ABI surgery variables in ABI users with no additional handicaps. Conclusions: ABI is a viable option to provide auditory sensations for children with cochlear anomalies. ABI surgery under age 3 is associated with improved auditory perception and language development compared with older users.Wo

Brain Stem Responses Evoked by Stimulation With an Auditory Brain Stem Implant in Children With Cochlear Nerve Aplasia or Hypoplasia

Objectives: The inclusion criteria for an auditory brain stem implant (ABI) have been extended beyond the traditional, postlingually deafened adult with Neurofibromatosis type 2, to include children who are born deaf due to cochlear nerve aplasia or hypoplasia and for whom a cochlear implant is not an option. Fitting the ABI for these new candidates presents a challenge, and intraoperative electrically evoked auditory brain stem responses (EABRs) may assist in the surgical placement of the electrode array over the dorsal and ventral cochlear nucleus in the brain stem and in the postoperative programming of the device. This study had four objectives: (1) to characterize the EABR by stimulation of the cochlear nucleus in children, (2) to establish whether there are any changes between the EABR recorded intraoperatively and again just before initial behavioral testing with the device, (3) to establish whether there is evidence of morphology changes in the EABR depending on the site of stimulation with the ABI, and (4) to investigate how the EABR relates to behavioral measurements and the presence of auditory and nonauditory sensations perceived with the ABI at initial device activation