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Very early bilateral implantation is thought to significantly reduce the attentional effort required to acquire spoken language, and consequently offer a profound improvement in quality of life. Despite the early intervention, however, auditory and communicative outcomes in children with cochlear implants remain poorer than in hearing children. The distorted auditory input via the cochlear implants requires more auditory attention resulting in increased listening effort and fatigue. Listening effort and fatigue may critically affect attention to speech, and in turn language processing, which may help to explain the variation in language and communication abilities. However, measuring attention to speech and listening effort is demanding in infants and very young children. Three objective techniques for measuring listening effort are presented in this paper that may address the challenges of testing very young and/or uncooperative children with cochlear implants: pupillometry, electroencephalography, and functional near-infrared spectroscopy. We review the studies of listening effort that used these techniques in paediatric populations with hearing loss, and discuss potential benefits of the systematic evaluation of listening effort in these populations. [ABSTRACT FROM AUTHOR]

Background: Cochlear implant indications have expanded over the last years, thanks to the advances in electrode array technology and surgical technique (soft surgery). It has been well demonstrated that patients with residual hearing on the low frequencies could benefit from electroacoustic stimulation, i.e. the combination of acoustic stimuli on the low frequencies and electrical stimulation on the higher ones. In order to use this kind of stimulation, successful postoperative hearing preservation must be obtained. Intraoperative active monitoring by intracochlear electrocochleography seems to be a promising method to assess cochlear trauma and to guide the surgeon during electrode insertion. Methods: Fifteen patients with useful residual hearing on the low frequencies were implanted with an intracochlear ECoG performed during electrode insertion. The system guided the surgeon with sound feedback and an intracochlear ECochG graph, by active and real-time testing of the cochlear function. Tone burst at 250 and 500 Hz were used as acoustic stimuli with an in-ear probe and the most apical contact of the cochlear implant electrode itself was used as the recording electrode. A pre- and postoperative pure tone audiogram was collected. Results: Comparing the pre- and postoperative pure tone audiogram, we observed the following mean dB decrease in hearing threshold: 15 dB at 125 Hz; 8.3 dB at 250 Hz; 9.1 dB at 500 Hz; 15.8 dB at 1000 Hz; 14.1 dB at 2000 Hz; 12.5 dB at 4000 Hz; 4.1 dB at 8000 Hz. These results show that hearing preservation can be obtained on low-medium frequencies. Conclusion: Residual hearing preservation is a feasible option in cochlear implant surgery using the soft surgery technique. With an intraoperative intracochlear ECochg, surgeons can have an electrode insertion real-time feedback in order to avoid damaging the intracochlear structures and by thus obtaining a better hearing preservation rate, allowing the patients to benefit from the electroacoustic stimulation. [ABSTRACT FROM AUTHOR]

Bacterial meningitis may cause inner ear fibrosis and progressive cochlear ossification with irreversible profound hearing loss (HL). Recognition of potential ossification is essential for effective management. We present a clinical case of a 4 year old boy who developed a progressive HL starting 3 weeks after meningitis. For the prospective risk of cochlear ossification, bilateral cochlear implantation (CI) was performed. Unexpectedly, unaided hearing threshold began to show improvement on the left ear, starting 4 months after meningitis and continuing for years post CI surgery. In order to explore the residual cochlear function, a trial of exclusively acoustic amplification was performed on the improved left side 5 years post implantation, providing good results. A certain degree of hearing recovery may be expected after meningitis related deafness. This case encourages surgeons to always adopt atraumatic surgical techniques that can enable the preservation of cochlear structure and residual function after CI surgery. [ABSTRACT FROM AUTHOR]

Background: Feeding and swallowing impairment are present in up to 80% of oculo-auriculo-vertebral spectrum (OAVS) patients. Salivary gland abnormalities have been reported in OAVS patients but their rate, features, and relationship with phenotype severity have yet to be defined., Material and Methods: Parotid and submandibular salivary gland hypo/aplasia was evaluated on head MRI of 25 OAVS patients (16 with severe phenotype, Goldenhar syndrome) and 11 controls., Results: All controls disclosed normal salivary glands. Abnormal parotid glands were found exclusively ipsilateral to facial microsomia in 21/25 OAVS patients (84%, aplasia in six patients) and showed no association with phenotype severity (14/16 patients with Goldenhar phenotype vs 7/9 patients with milder phenotype, p = 0.6). Submandibular salivary gland hypoplasia was detected in six OAVS patients, all with concomitant ipsilateral severe involvement of the parotid gland (p < 0.001). Submandibular salivary gland hypoplasia was associated to Goldenhar phenotype (p < 0.05). Parotid gland abnormalities were associated with ipsilateral fifth (p < 0.001) and seventh cranial nerve (p = 0.001) abnormalities. No association was found between parotid gland anomaly and ipsilateral internal carotid artery, inner ear, brain, eye, or spine abnormalities (p > 0.6)., Conclusions: Salivary gland abnormalities are strikingly common in OAVS. Their detection might help the management of OAVS-associated swallowing and feeding impairment.