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Purpose Hamartomas of tuber cinereum present as ectopic tissue in the hypothalamic region. Clinically, the usual hypothalamic hamartomas manifest themself by gelastic seizures and pubertas praecox. We observed an increased coincidence of the presence of X-linked recessive deafness DFNX2 (DFN3) and a hamartoma of the tuber cinereum. Initially five patients presented with hearing loss in childhood, two additional were already adults, not showing any characteristic symptoms for a hamartoma but signs of delayed puberty. Methods Seven patients who underwent computed tomography imaging due to a sensorineural hearing loss and had a hamartoma of the tuber cinereum in addition to X-linked deafness DFNX2 (DFN3) were included in a retrospective study. Patients underwent initial neurologic, endocrinologic, and genetic evaluation. Long-term follow-up was performed after 10 to 12 years. Results The average age at the initial exam was 12.9 years (range 4–29). All patients genetically proven nonsyndromic, X-linked deafness associated with the POU3F4 gene. Three out of six patients presented signs of delayed puberty. None of all seven showed any evidence of pubertas praecox or gelastic seizures at mean age of 17 years (range 17–29 years) at any time. Conclusion Hamartomas of tuber cinereum are often coincident with DFNX2. Clinically, half of the cases are—in contrary to the usual pubertas praecox—associated with growth hormone deficiency and delayed puberty, in the sense of pubertas tarda, when coincident. Clinicians' and radiologists' knowledge and awareness of this rare combination are crucial to identify children early enough for hormone-sensitive treatment. [ABSTRACT FROM AUTHOR]

Objective: The implantation of biohybrid electrodes was introduced a few years ago in our clinic. These electrodes coated with autologous mononuclear cells releasing anti-inflammatory and neuroprotective factors are thought to reduce insertion trauma and maintain the vitality of surviving spiral ganglion neurons. The clinical feasibility of this approach has already been demonstrated. In the present retrospective study, the four-year results of the two sides (classical electrode and biohybrid electrode) in the bilaterally implanted patients were compared in order to investigate possible adverse long-term effects. Methods: All patients received a complete audiological diagnosis which also included a speech audiogram and impedance measurement. The measurements were carried out 1 month, 3 months, 6 months, 1 year, 2 years, 3 years and 4 years after implantation. The hearing results were assessed by pure tone audiometry. Results: All patients showed satisfactory speech understanding and similar impedances on both sides although they had a long-term deafness before implantation of the side provided with a biohybrid electrode array. The results of speech understanding and impedance measurements were stable for years. Cone beam computed tomography was performed in 4 patients three years after implantation and could rule out cochlear ossification. Other complications were also not registered in any of the patients. Conclusion: Due to satisfactory outcomes and lack of complications, the biohybrid electrode is considered to be a safe option in cochlear implantation. The simplicity of application of autologous cells as a source of anti-inflammatory and neuroprotective factors via a biohybrid electrode array is a key step for cell-based, regenerative therapies for deafness. [ABSTRACT FROM AUTHOR]

Objectives: The aim was to analyze the long‐term hearing results after simultaneous microsurgical extirpation via enlarged cochleostomy and cochlear implantation in intracochlear schwannoma as compared with non‐tumor single‐side deafness patients. Methods: Microsurgical extirpation via enlarged cochleostomy with simultaneous cochlear implantation was performed in 15 cases of intracochlear schwannoma between 2014 and 2021. Speech recognition tests in German language and impedance performances were collected over 36 months of observation and compared with an internal cohort of 52 age matched non‐tumor single‐side deafness patients. Retrospective cohort study in a tertiary referral center. Results: The surgery proved feasible and uneventful in all cases. In the case of intracochlear schwannoma, the hearing rehabilitation results were highly satisfactory and comparable to those of the non‐tumor single‐side deafness cohort. The speech recognition performance improved steadily in the first 12 months; afterward, it remained stable, providing indirect evidence against tumor recurrence during the follow‐up. One patient required implant revision surgery related to device failure, but no recurrence was registered in the 36 months of observation. Conclusions: Cochlear implantation is the strategy of choice for hearing rehabilitation in case of intracochlear schwannomas in the long term. In particular, the combination of tumor extirpation via cochleostomy with a cochlear implantation in the same surgical time offers a viable therapy for intracochlear schwannoma, granting a sufficient degree of radicality without compromising the cochlear integrity. This technique allows for revision surgery if required. Level of Evidence: 4 Laryngoscope, 134:1854–1860, 2024 [ABSTRACT FROM AUTHOR]

Purpose: To evaluate if a specific type of cochlear implant (CI) electrode array (EA) reveals higher rates/prevalence of vestibular symptoms and to characterize their respective relationship to intracochlear position and objective vestibular function. Methods: This retrospective study included 71 cochlear implantations in patients older than 18 years. The electrode position within the cochlea, electrode insertion angle, and cochlear coverage were determined from postoperative multiplanar reconstructed cone-beam computed tomography scans. All device manufacturers were represented. Data related to preoperative and postoperative PTA as well as vestibular symptoms in the preoperative and postoperative stages were collected from the patient's records. Results: Twelve of the 71 (16.9%) CI patients experienced vertigo symptoms in the early postoperative period. In 5 (7.0%) patients, the vertigo complaints lasted until the time of the first activation (5-6 weeks postoperative). Postoperative onset of vestibular symptoms was more often seen in patients receiving lateral wall (LW)/straight EAs (19%) compared to perimodiolar/precurved EAs (7%), but this was only a trend and no statistical significance was observed. Moreover, preoperative pathologic caloric responses (CRs) better predicted the postoperative onset of vestibular symptoms. Conclusion: The preoperative consideration of a complicated CI-induced vertigo is important in the counseling particularly of elderly patients. We identified some risk factors for post-CI vertigo that should be considered in the patient's counseling: preoperative pathologic CRs, the extent of surgical trauma, and possibly the use of an LW EA, regardless of the length. [ABSTRACT FROM AUTHOR]

Introduction: In a previous study, an inner ear catheter was used to deliver low- and high-dose steroids into the cochlea prior to cochlear implant electrode insertion. With this approach, more apical regions of the cochlea could be reached and a reduction of electrode impedances in the short term was achieved in cochlear implant recipients. Whether intracochlear application of drugs via the catheter is a safe method also for patients with residual hearing has not been investigated hitherto. The aim of the present study was therefore to investigate the effect of intracochlear triamcinolone application in cochlear implant recipients with residual hearing. Patients and methods: Patients with residual hearing were administered triamcinolone-acetonide (4 mg/ml; n = 10) via an inner ear catheter just prior to insertion of a MED-EL FLEX28 electrode. Impedances were measured at defined time points (intra-operatively, post-operatively and at first fitting) and retrospectively compared with a control group (no steroid application) and lowand high-dose group. Hearing thresholds were measured preoperatively, 3 days after surgery and at first fitting by pure tone audiometry. Pre- to postoperative hearing loss was determined at first fitting and compared to results from a previous study. Results: The median hearing loss after implantation (125-1,500 Hz) was 20.6 dB. Four patients (40%) showed a median hearing loss of less than 15 dB, three patients (30%) between 15 and 30 dB and three patients (30%) more than 30 dB. The median hearing loss was similar to the results obtained from our previous study showing a median hearing loss of 24 dB when using FLEX28 electrode arrays. Conclusion: No difference in residual hearing loss was found when comparing application of triamcinolone-acetonide using an inner ear catheter prior to the insertion of a FLEX28 electrode array to the use of the FLEX28 electrode array without the catheter. Thus, we conclude that application of drugs to the cochlea with an inner ear catheter could be a feasible approach in patients with residual hearing. [ABSTRACT FROM AUTHOR]