Cochlear implants in the management of hearing loss in Neurofibromatosis Type 2.

OBJECTIVE: Review of cochlear implant (CI) outcomes in patients with Neurofibromatosis Type 2 (NF2), implanted in the presence of an ipsilateral vestibular schwannoma (VS). Hearing restoration was combined in some cases with a Bevacizumab regime. METHOD: Retrospective review of 12 patients, managed over the period 2009-2016, at a tertiary referral multidisciplinary NF2 clinic. The patients are grouped by hearing outcomes to explore likely protective factors, and to generate a proposed decision-making tool for the selection of either CI or Auditory Brainstem Implant (ABI). RESULTS: Four of the 12 patients achieved speech discrimination without lip-reading. In these individuals there is reason to think that the mechanism of their hearing loss was cochlear dysfunction. A further four patients received benefit to lip-reading and awareness of environmental sound. For such patients their hearing loss may have been due to both cochlear and neural dysfunction. Two patients gained access to environmental sound only from their CI. Two patients derived no benefit from their CIs, which were subsequently explanted. Both these latter patients had had prior ipsilateral tumour surgery, one just before the CI insertion. CONCLUSION: Cochlear implantation can lead to open set speech discrimination in patients with NF2 in the presence of a stable VS. Use of promontory stimulation and intraoperative electrically evoked auditory brainstem response testing, along with case history, can inform the decision whether to implant an ABI or CI.

English consensus protocol evaluating candidacy for auditory brainstem and cochlear implantation in neurofibromatosis type 2.

OBJECTIVE: Hearing loss resulting from bilateral vestibular schwannomas (VSs) has a significant effect on the quality of life of patients with neurofibromatosis Type 2 (NF2). A national consensus protocol was produced in England as a guide for cochlear implantation (CI) and auditory brainstem implantation (ABI) in these patients. STUDY DESIGN: Consensus statement. SETTING: English NF2 Service. PARTICIPANTS: Clinicians from all 4 lead NF2 units in England. MAIN OUTCOME MEASURES: A protocol for the assessment, insertion and rehabilitation of CI and ABI in NF2 patients. RESULTS: Patients should undergo more detailed hearing assessment once their maximum aided speech discrimination score falls below 50% in the better hearing ear. Bamford-Kowal-Bench sentence testing scores below 50% should trigger assessment for auditory implantation, as recommended by the National Institute for Clinical Excellence guidelines on CI. Where this occurs in patients with bilateral stable VS or a unilateral stable VS where the contralateral cochlear nerve was lost at previous surgery, CI should be considered. Where VS surgery is planned, CI should be considered where cochlear nerve preservation is thought possible, otherwise an ABI should be considered. Intraoperative testing using electrically evoked auditory brainstem responses or cochlear nerve action potentials may be used to determine whether a CI or ABI is inserted. CONCLUSION: The NF2 centers in England agreed on this protocol. Multisite, prospective assessments of standardized protocols for auditory implantation in NF2 provide an essential model for evaluating candidacy and outcomes in this challenging patient population.

Hypertrophic effects of urocortin homologous peptides are mediated via activation of the Akt pathway.

The UCN homologues SCP and SRP bind specifically to the CRFR2 receptor, whereas UCN binds to both CRFR1 and CRFR2. We have previously demonstrated that all three peptides are cardioprotective, and both the Akt and MAPK p42/44 pathways are essential for this effect. Here we tested the hypertrophic effects of these peptides. We examined the effects of the peptides on cell area, protein synthesis, and induction of the natriuretic peptides ANP and BNP. All three peptides were able to increase all the markers of hypertrophy examined, with SCP being the most potent of the three, followed by UCN and SRP last. In addition, we provide a mechanism of action for the three peptides and show that Akt phosphorylation is important for their hypertrophic action, whereas MAPK p42/44 is not involved in this effect.

Diplacusis, hearing threshold and otoacoustic emissions in an episode of sudden, unilateral cochlear hearing loss.

Limited data are available on the relationship between diplacusis and otoacoustic emissions and sudden hearing threshold changes, and the detail of the mechanism underlying diplacusis is not well understood. Data are presented here from an intensively studied single episode of sudden, non-conductive, mild hearing loss with associated binaural diplacusis, probably due to a viral infection. Treatment with steroids was administered for 1 week. This paper examines the relationships between the hearing loss, diplacusis and otoacoustic emissions during recovery on a day-by-day basis. The hearing thresholds were elevated by up to 20 dB at 4kHz and upwards, and there was an interaural pitch difference up to 12% at 4 and 8 kHz. There was also a frequency-specific change in transient evoked otoacoustic emission (TEOAE) and distortion-product otoacoustic emission (DPOAE) level. DPOAE level was reduced by up to 20 dB. with the greatest change seen when a stimulus with a wide stimulus frequency ratio was used. Frequency shifts in the 2f2-fi DPOAE fine structure corresponded to changes in the diplacusis. Complete recovery to previous levels was observed for TEOAE, DPOAE and hearing threshold. The diplacusis recovered to within normal limits after 4 weeks. The frequency shift seen in the DPOAE fine structure did not quite resolve, suggesting a very slight permanent change. The time-courses of TEOAE. diplacusis and hearing threshold were significantly different: most notably, the hearing threshold was stable over a period when the diplacusis deteriorated. This suggests that the cochlear mechanisms involved in diplacusis, hearing threshold and OAE may not be identical.