Postsurgical outcome in patients with auditory auras and drug-resistant epilepsy.

PURPOSE: We assessed whether patients with auditory auras have similar outcomes after epilepsy surgery as patients without auditory auras, and hypothesized that patients with non-dominant hemisphere foci might fare better after temporal lobe surgery than patients with dominant resections. METHODS: In this retrospective study, outcome after temporal resection was assessed for patients with drug-resistant epilepsy. Preoperative demographic data, clinical data, and surgical outcome were prospectively registered in a database from 1986 through 2016. Seizure outcome was classified as either seizure-free or relapsed. RESULTS: Data were available in 1186 patients. Forty five patients (3.8%) reported auditory auras; 42 patients (93%) had temporal lobe epilepsy (TLE), and three patients (7%) had extratemporal epilepsy. Since most patients with auditory auras had TLE and in order to have comparable groups, we selected 41 patients with auditory auras and compared them with patients without auditory auras who had temporal lobe resections (767 patients). There were no significant demographic or clinical differences between TLE patients with auditory auras and those without. Patients who had auditory auras were more likely to relapse after temporal lobe surgery than those without (p=0.03). Among patients who had auditory auras and temporal lobe surgery, side of surgery was not related to postoperative outcome (p=0.3). CONCLUSION: Auditory auras are rare among patients with drug-resistant TLE. The presence of an auditory aura in a patient with drug-resistant TLE carries a worse prognosis for a postoperative seizure free outcome and this is not related to the side of surgery.

Clinical evaluation of the xDP output compression strategy for cochlear implants.

Technological advances in the domain of digital signal processing adapted to cochlear implants (CI) are partially responsible for the ever-improving outcomes observed with this neural prosthesis. The goal of the present study was to evaluate audiometric outcomes with a new signal processing strategy implemented in Oticon Medical-Neurelec cochlear implant systems, the xDP strategy. The core of this approach is a preset-based back-end output compression system, modulating a multi-channel transfer function depending on the intensity and information content of input sounds. Twenty adult CI patients, matched for age and CI experience, were included in this study. Pure-tone thresholds and vocal audiometry scores were measured with their former signal processing strategy and with xDP. Speech perception was assessed using dissyllabic words presented in quiet, at different intensity levels: 40, 55, 70, and 85 dB SPL, and in a cocktail party noise at a signal-to-noise ratio of +10 dB. Results with the xDP strategy showed, as awaited, no major modification of pure-tone thresholds. A global increase of speech perception scores was observed after a 1-month habituation period, with significant improvements for speech perception in quiet for moderate (55 dB SPL), loud speech sounds (85 dB SPL), and speech-in-noise comprehension. Subjective signal quality assessment showed a preference for Crystalis(xDP) over the former strategy. These results allow the quantification of improvements provided by the xDP signal processing strategy.

Contemporary surgical issues in paediatric cochlear implantation.

OBJECTIVE: To review the contemporary surgical issues in paediatric cochlear implantation (CI) based on published evidence. DESIGN: Narrative literature review. RESULTS: Surgical challenges in paediatric CI are discussed, with respect to post meningitic labyrinthitis ossificans; cochlear malformation; cochlear implantation in infants; auditory neuropathy and cochlear nerve deficiency; bilateral cochlear implantation; hearing preservation; otitis media; and device failure. CONCLUSION: Early CI is recommended if bacterial meningitis causes profound sensorineural hearing loss (SNHL). CI in cochleovestibular malformation requires pre-operative imaging to plan surgical technique, choice of electrode, and to anticipate complication. Children with congenital severe to profound SNHL should undergo early bilateral simultaneous implantation, preferably before 12 months of age, except those with auditory neuropathy spectrum disorder who should be implanted after one year. Soft surgical technique should be deployed in an attempt to preserve any residual hearing. Otitis media with effusion is not a contraindication to cochlear implantation, but active or recurrent acute otitis media requires resolution of infection with grommet insertion pre-operatively. Device failure in CI recipients requires a stepwise audiological, medical, radiological, and device integrity assessment to determine the need for reimplantation.

Barriers to early cochlear implantation.

OBJECTIVE: Identify variables associated with paediatric access to cochlear implants (CIs). DESIGN: Part 1. Trends over time for age at CI surgery (N = 802) and age at hearing aid (HA) fitting (n = 487) were examined with regard to periods before, during, and after newborn hearing screening (NHS). Part 2. Demographic factors were explored for 417 children implanted under 3 years of age. Part 3. Pre-implant steps for the first 20 children to receive CIs under 12 months were examined. RESULTS: Part 1. Age at HA fitting and CI surgery reduced over time, and were associated with NHS implementation. Part 2. For children implanted under 3 years, earlier age at HA fitting and higher family socio-economic status were associated with earlier CI. Progressive hearing loss was associated with later CIs. Children with a Connexin 26 diagnosis received CIs earlier than children with a premature / low birth weight history. Part 3. The longest pre-CI steps were Step 1: Birth to diagnosis/identification of hearing loss (mean 16.43 weeks), and Step 11: MRI scans to implant surgery (mean 15.05 weeks) for the first 20 infants with CIs under 12 months. CONCLUSION: NHS implementation was associated with reductions in age at device intervention in this cohort.

A practical guide to cochlear implantation in adults with long durations of monaural sound deprivation.

OBJECTIVE: This paper aims to summarize published findings by the authors and integrate these within current literature to support clinical guidelines when choosing an ear for cochlear implantation in adults with long-term monaural sound deprivation. STUDY SAMPLE: Four retrospective cohort studies based on data collected in five cochlear implantation centres with adults with bilateral hearing loss who used a single hearing aid for at least 15 years prior to unilateral or bilateral cochlear implantation. DESIGN: Review, integration and interpretation of retrospective cohort studies to support clinical recommendations. RESULTS: In this population, the prelingual nature of the hearing loss and the duration of bilateral significant hearing loss were the most reliable predictors of cochlear implantation outcomes. Importantly, the duration of sound deprivation in the ear to be implanted was not a significant predictor of speech recognition scores after cochlear implantation and should carry less weight in making recommendations. CONCLUSIONS: In most adults with postlingual hearing loss and long-term monaural sound deprivation, the sound-deprived sound deprivation ear should be preferred for implantation. For adults with prelingual deafness and monaural sound deprivation, the decision should weigh the risks of obtaining poorer results with the cochlear implant compared to the benefits of accessing binaural hearing.

Impact of the Degree of the Mastoid Pneumatization on Cartilage Type 1 Tympanoplasty Success.

OBJECTIVES: To evaluate the effect of the degree of the mastoid pneumatization on the success rate of cartilage type 1 tympanoplasty. STUDY DESIGN: A retrospective clinical chart review. METHODS: In total, 90 patients (44 females and 46 males; average age, 38.40 ±â€Š11.12 years; age range: 21-65 years) who underwent cartilage type 1 tympanoplasty were included. Demographics and anatomical outcomes were recorded. The patients were divided into 2 groups in terms of anatomical success. In group A, all patients had intact grafts without perforation, retraction, or lateralization, and a dry ear, at 6 months postoperatively. In group B, reperforation of the tympanic membrane was evident 6 months postoperatively. The mastoid air cell volumes of each groups were compared. RESULTS: The mean mastoid pneumatization in groups A and B was 5.32 ±â€Š1.96  and 5.06 ±â€Š2.12 cm, respectively. The mastoid pneumatization of diseased ears did not differ between the groups (P > 0.05). The mastoid pneumatization of diseased ears did not differ between males and females (P > 0.05). The mastoid pneumatization of normal ears did not differ between the groups (P > 0.05). The mastoid pneumatization of normal ears did not differ between males and females (P > 0.05). CONCLUSIONS: The degree of mastoid pneumatization did not affect the success rate of cartilage type 1 tympanoplasty. Further studies with larger numbers of patients are needed to evaluate the relationship between the degree of the mastoid pneumatization and anatomical outcomes after placement of various graft types.

Trans-tympanic catheter insertion for treatment of patulous eustachian tube.

OBJECTIVES: To evaluate the safety and therapeutic efficacy of trans-tympanic catheter insertion (TCI) in patients with refractory patulous eustachian tube (PET). METHODS: TCI was attempted in thirty-six ears of twenty-nine patients with chronic PET refractory to conservative treatment. The catheter was inserted under local anesthesia in an operating room through the bony orifice of the eustachian tube (ET) to occlude the isthmus of the tube via a myringotomy site on the tympanic membrane. Patients were evaluated postoperatively by nasal endoscopy and by interview to document symptoms. Successful treatment was defined as complete relief or significant improvement plus satisfaction with treatment. Patients had no concurrent disease and did not undergo any additional surgical procedure. RESULTS: TCI was performed in all except one ear, in which it failed because of an abnormally narrow tympanic ET orifice. Follow-up durations ranged from 6 to 37 months, with an average of 19.3 months. Successful treatment of subjective autophony was achieved in twenty-nine (82.4%) of the thirty-five ears. Ventilation tube (VT) placement was performed in the two ears because of otitis media with effusion (OME) after TCI. In one ear, the inserted catheter was finally removed due to additional unilateral mastoiditis after VT extrusion. CONCLUSION: TCI seems to be a minimally invasive and was used successfully to treat PET. The procedure had a good overall success rate and complications were rare in the long-term.

Long term surgical and hearing outcomes in the management of tympanomastoid paragangliomas.

OBJECTIVE: To analyze the long term outcomes after surgery in tympanomastoid paragangliomas. STUDY DESIGN: Retrospective study. METHODS: The charts of 145 patients with tympanomastoid paragangliomas managed between 1988 and 2013 were reviewed. The clinical features, audiological data, pre- and postoperative notes were noted. The tumors were staged according to the modified Fish and Mattox classification. The surgical approaches for all patients were formulated according to the surgical algorithm developed at our center. RESULTS: 34 (23.5%), 46 (31.7%), 22 (15.2%), 18 (12.4%) and 25 (17.2%) patients were diagnosed to have TMP class A1, A2, B1, B2 and B3 tumors respectively. Gross tumor resection was achieved in 141 (97.2%) patients. The facial nerve was uncovered in four patients and infiltrated in three. The cochlea was found eroded in seven cases. The mean follow-up was 48.4 months. Recurrence was seen in one patient (0.7%). In the cases where the facial nerve was preserved (n=143), the nerve function was graded as HB grade 1 in 138 patients (97%). Postoperatively, the mean AC showed an improvement in all categories except in class B2 and B3, which corresponds to the classes that include patients who underwent subtotal petrosectomy. CONCLUSION: We report the long term surgical outcomes in tympanomastoid paragangliomas in the largest series published till date. It is possible to completely eradicate all types of tympanomastoid paragangliomas with minimum sequelae by choosing the correct surgical approach to achieve adequate exposure for individual tumor classes as described in our classification and algorithm. LEVEL OF EVIDENCE: IIb.

Surgical outcomes of facial nerve hemangiomas in temporal bones.

OBJECTIVE: To report surgical outcomes of 15 cases who had facial nerve hemangiomas. METHODS: All cases underwent complete removal of hemangiomas, and preservation of nerve integrity was attempted. The postoperative outcomes of facial nerve was assessed. RESULTS: Nerve integrity was successfully preserved in 10 cases, all of which maintained or recovered to Grade I or Grade II, and facial nerve was sacrificed in 5 cases, who recovered to Grade III or Grade IV. CONCLUSIONS: When possible, facial nerve preservation should be attempted, which was critical to yield better outcomes of facial nerve.

The influence of word characteristics on the vocabulary of children with cochlear implants.

The goal of this study was to explore the effects of phonotactic probability, word length, word frequency, and neighborhood density on the words known by children with cochlear implants (CIs) varying in vocabulary outcomes in a retrospective analysis of a subset of data from a longitudinal study of hearing loss. Generalized linear mixed modeling was used to examine the effects of these word characteristics at 3 time points: preimplant, postimplant, and longitudinal follow-up. Results showed a robust effect of neighborhood density across group and time, whereas the effect of frequency varied by time. Significant effects of phonotactic probability or word length were not detected. Taken together, these findings suggest that children with CIs may be able to use spoken language structure in a manner similar to their normal hearing counterparts, despite the differences in the quality of the input. The differences in the effects of phonotactic probability and word length imply a difficulty in initiating word learning and limited working memory ability in children with CIs.

Exploring the discourse on communication modality after cochlear implantation: a Foucauldian analysis of parents' narratives.

After pediatric cochlear implantation, parents have to make decisions concerning which communication modality the child and the child's family will use. The choice has to be made against a background of opposing views on communication modality in follow-up after pediatric cochlear implantation. The opposing views form a discourse that has been a core issue in the international body of literature for a long time. For hearing parents caught up in this ongoing controversy, the choice can be a difficult one. The study reported in this article explores the discourse on communication modality and is based on 27 written parental accounts on experiences with follow-up. From the perspective of Foucault's thinking, discourses exert knowledge and power. Drawing on Foucault, the study explores how the discourse on communication modality is constructed, how it operates, and how it governs thinking and acting. The Foucauldian analysis brings to the fore the complex nature of the discourse and states that follow-up, which is intended to be helpful for parents, may induce insecurity and frustration. The study brings into conversation a broader understanding of the discourse on communication modality and addresses a need for increased awareness on how the discourse governs thinking and acting in follow-up.

[The hearing function in the patients presenting with the intralabyrinthine distribution of giant cholesteatoma].

The objective of the present study was to evaluate the state of the hearing function in the patients presenting with the intralabyrinthine distribution of giant cholesteatoma before and after the surgical treatment based on the results of examination by the subjective and objective methods. The possibility of preservation of the hearing function after the surgical intervention has been demonstrated.

Autologous meatal skin graft implantation and intratympanic injection of Pseudomonas aeruginosa: a new experimental mouse model of acquired middle ear cholesteatoma.

AIM: To create an experimental model for the biomedical research of middle ear cholesteatoma. METHODS: Cholesteatoma was induced in the right ears of mice. An autologous meatal skin graft was implanted into the middle ear via the tympanic membrane followed by an intratympanic injection of Pseudomonas aeruginosa. Six weeks after surgery, the formation of acquired cholesteatoma was evaluated by macroscopic examination, CT scan, and histological analysis. The expressions of TNF-α, IL-1ß, and IL-6 were measured with real-time PCR. Auditory-evoked brain stem response was used for assessing the changes in hearing levels. RESULTS: None of the mice died during the modeling time. By the sixth postoperative week, cholesteatoma had successfully formed in 23 out of 25 mice, which equals a success rate of 92%. A CT scan revealed that the cholesteatoma was eroding the cochlea. Furthermore, histological analysis demonstrated a cystic structure covered by stratified squamous epithelium and keratin desquamation in the lamellae inside the cystic cavity in the bullae. All mice with experimentally induced cholesteatoma showed hearing impairment and an upregulated expression of TNF-α, IL-1ß, and IL-6. CONCLUSION: The present study successfully developed a mouse model of acquired middle ear cholesteatoma, which provides an extremely valuable tool for auditory and biomedical research. The modeling approach is technically easy and has a high success rate.

[The results of primary tympanoplasty of extended tympanic defects in the patients presenting with chronic mesotympanitis].

The objective of the present study was to estimate the results of primary tympanoplasty of extended tympanic defects in the patients presenting with chronic mesotympanitis. The results of the surgical treatment of 68 patients with chronic suppurative otitis media (mesotympanitis) by primary tympanoplasty of extended tympanic defects. Tympanoplasty was performed with the use of a 0.2-0.3 mm cartilage plate from which a number of fragments mobile relative to one another were formed by an original method. Good clinical and anatomical results were obtained in 90.4% of the treated patients within 24 months after surgery. Moreover, the number of patients with socially adequate hearing increased up to 86.5% during the same period. The bone-air interval was reduced from 30 to 10 dB at all observation time-points.

Fallopian canal stapedotomy in congenital stapes fixation with aberrant facial nerve.

Congenital stapes ankylosis is reported to have an occurrence rate of 3% to 4%, and it represents 20% to 35% of ossicular malformations. Surgical treatment of congenital stapes ankylosis is described to be satisfactory in the large majority of the case series reported in the literature. In these cases, special attention should be paid to exclude any aberrations of the course of the facial nerve, which have been demonstrated to be frequently associated with congenital middle ear malformations. We describe a case of congenital stapes ankylosis associated with a previously unreported facial nerve abnormality, characterized by the presence of an empty fallopian canal in combination with a dehiscent facial nerve running over the footplate and almost totally covering it.

Endoscopic Ossiculoplasty for the Management of Isolated Congenital Ossicular Chain Malformation: Surgical Results in 16 Ears.

OBJECTIVE: The aim of this study is to describe the clinical characteristics and intraoperative findings and further evaluate the efficacy of endoscopic ossiculoplasty for the management of isolated congenital ossicular chain malformation. METHODS: A retrospective study was performed on 16 ears (15 patients) with the isolated congenital ossicular chain malformation who underwent endoscopic ossiculoplasty in our department from May 2017 to January 2019. Endoscopic exploratory tympanotomy was conducted to check the ossicular chain; at the same time, endoscopic ossiculoplasty was performed depending on intraoperative findings. Air-conduction thresholds, bone-conduction thresholds, and air-bone gaps (ABGs) were measured before and after surgery, and the hearing outcome was assessed at 6 months postoperatively. RESULTS: The most common malformations of ossicular chain were the missing of the incus long process and stapes suprastructure. A serial assessment of the hearing status was conducted before and 6 months after surgery. It showed the mean postoperative pure-tone average (PTA) was significantly reduced, and the mean postoperative ABG was obviously closed, respectively (P < .001). The mean PTA gain was 36.3 ± 8.6 dB, and the ABG closure was 35.1 ± 8.3 dB; ABG closure to 20 dB or less and ABG closure to 10 dB or less were achieved in 14 cases (87.5%) and 5 cases (31.3%), respectively. No differences were observed in postoperative hearing outcome between type Ⅲ cases and type Ⅳ cases; however, cases with partial ossicular replacement prosthesis implantation showed a larger hearing gain (P = .049) and a higher proportion of postoperative ABG less than 10 dB (P = .021). No facial palsy and significant sensorineural hearing loss occurred; all patients completed the surgery without the need of canalplasty, and the chorda tympani nerve was preserved in all patients. CONCLUSIONS: This research showed endoscopic surgery was effective in the diagnosis and management of isolated congenital ossicular chain malformation; the endoscopic ossiculoplasty provides an alternative method to manage congenital ossicular chain malformation, with comfortable hearing outcome and the advantage of excellent vision and less invasion.

Music perception of cochlear implant users: a questionnaire, and its implications for a music training program.

Current research has shown that although adult cochlear implant (CI) users generally find music to be less enjoyable following implantation, training may help some recipients to improve their music perception. This study developed and administered a questionnaire (The University of Canterbury Music Listening Questionnaire: UCMLQ), to collect information which could then be used to develop such a music training program (MTP). One hundred adult recipients completed the UCMLQ. Results showed that respondents generally found music to be less enjoyable post-implantation, and thought that music did not sound as they would expect it to sound to a person with normal hearing. However, it was reported that music listening could be enhanced by controlling the listening environment, being selective about the music chosen, and using a contralateral HA. The preferred logistics for a MTP were 30-minute sessions, 2-3 times per week, using a DVD format. The program should focus on improving recipients' ability to recognize tunes, and encompass a wide range of musical styles. The findings support the development of a MTP for CI users to better enable them to enjoy and appreciate music, and to maximize their potential with current technology.

[Stem cell therapy in the inner ear: recent achievements and prospects]. / Thérapie cellulaire dans l'oreille interne : nouveaux développements et perspectives.

Because of its high prevalence and social impact, hearing impairment is a major public health problem. Whatever the cause--heredity, acoustic trauma, aminoglycoside antibiotics, noise exposure or aging--the hearing impairment is often caused by an irreversible loss of sensory hair cells. So far, hearing aids and cochlear implants are the only possibility to "treat" profound deafness. With the advent of regenerative medicine, extensive studies aimed to repair, regenerate or replace lost hair cells have been initiated. Recently, Stefan Heller and colleagues described a guidance protocol to induce mouse embryonic stem cells (ESC) and induced pluripotent stem cells (iPSC) to differentiate into mechanosensitive hair cells. The resulting hair cells hold promise as a tool for hair cell molecular physiology and physiopathology, drug discovery, and possibly also hair cell replacement. The next challenges, alternative strategies, their limitations and prospects are also discussed.