Cochlear implantation in children without preoperative computed tomography diagnostics. Analysis of procedure and rate of complications.

INTRODUCTION: To evaluate the safety in cochlear implantation without preoperative computed tomography diagnostics, which was implemented into the protocol of cochlear implantation in 2013, since in the year before, new evidence concerning the risks of ionizing radiation especially in children arose. METHODS: In this retrospective data analysis 89 children under 36 months, which were cochlear implanted from 2008 until 2018 at a tertiary referral centre with a large cochlear implant program were analysed. Fortyfour of the children were implanted before the date of change in 2013 and 45 in the following years up to now. The data about the operative procedures, the postoperative care and the complication rate before and after implementation of the new protocol were compared. RESULTS: Before the date of change in 2013, 100% of patients received preoperative CT diagnostics, in the following years 13.3%. No difference in the duration of surgery, the procedure related and the late complications between the two groups was identified. CONCLUSION: Cochlear implantation in very young children under the age of 36 months without preoperative radiological diagnostics by CT scan of the temporal bone is a safe procedure without additional risks for the patients.

Long-term treatment outcomes in children with auditory neuropathy spectrum disorder (ANSD).

INTRODUCTION: The present article shows long-term results in the hearing and speech development of children with auditory neuropathy spectrum disorder (ANSD). Some children were followed up for nearly 20 years, monitoring their progress through childhood into adulthood. METHODS: This retrospective study examined data from 10 children who were diagnosed and treated at our tertiary referral center. All children were provided with hearing aids and/or cochlear implants. The children's hearing and speech development and their social and educational development were evaluated. RESULTS: Seven children were provided with cochlear implants on both sides; three children, one of which had single-sided deafness, received hearing aids. All children with cochlear implants on both sides used their devices full-time. Speech perception ranged between 100% and 0% on one side. Five children attended a school for the hearing impaired; four children attended a regular school. Four children attended vocational training. CONCLUSIONS: At present, there is a lack of literature on the long-term outcomes of treatment in children with ANSD. The data presented show that the hearing and speech development in children with ANSD are significantly heterogeneous. Regular school education and social integration of children with ANSD can be achieved with intensive and supportive rehabilitative methods.

Evaluation and therapy outcome in children with auditory neuropathy spectrum disorder (ANSD).

OBJECTIVES: The aims of the present study are to: describe diagnostic findings in patients with auditory neuropathy spectrum disorder (ANSD); and demonstrate the outcomes of different therapies like hearing aids (HAs) or cochlear implantation. METHODS: 32 children were diagnosed and treated at our tertiary referral center and provided with HAs or cochlear implants (CIs). All of them underwent free-field or pure-tone audiometry. Additionally, otoacoustic emissions (OAEs), impedance measurements, auditory brainstem responses (ABRs), auditory steady-state responses (ASSR), electrocochleography, and cranial magnetic resonance imaging (cMRI) were all performed. Some patients also underwent genetic evaluation. Following suitable provision pediatric audiological tests, psychological developmental diagnostic and speech and language assessments were carried out at regular intervals in all the children. RESULTS: OAEs could initially be recorded in most of the children; 17 had no ABRs. The other eight children had a poor ABR morphology. Most of the children had typical, long-oscillating cochlear microphonics (CMs) in their ABRs, which was also observed in all of those who underwent electrocochleography. Eight children were provided with a HA and 17 received a CI. The functional gain was between 32 and 65 decibel (dB) with HAs and between 32 and 50 dB with CI. A speech discrimination level between 35 and 100% was achieved during open-set monosyllabic word tests in quiet with HA or CI. With the Hochmair-Schulz-Moser (HSM) sentence test at 65 dB SPL (sound pressure level), 75% of the children with a CI achieved a speech discrimination in noise score of at least 60% at a signal to noise ratio (SNR) of 5, and four scored 80% or higher. Most of the children (72%) were full-time users of their devices. All the children with a CI used it on a regular basis. CONCLUSION: Only a few case reports are available in the literature regarding the long-term outcomes of ANSD therapy. The present study reveals satisfactory outcomes with respect to hearing and speech discrimination in children with CIs or HAs. The nearly permanent use of the devices reflects a subjective benefit for the children. Provision with a suitable hearing device depends on audiological results, the speech and language development of an individual child, and any accompanying disorders. Repeated audiological evaluations, interdisciplinary diagnostics, and intensive hearing and speech therapy are essential for adequate rehabilitation of this group of children.

Phenotypic Characterization of DFNB16-associated Hearing Loss.

HYPOTHESIS: We hypothesized that patients with DFNB16 caused hearing loss show characteristical audiological findings depending on genetic results. BACKGROUND: Hearing loss belongs to the most frequent congenital diseases. In 50-70% of individuals, hearing loss is caused by genetic defects. DFNB1 (deafness, neurosensory, autosomal-recessive) is the most frequently affected locus. Despite its great genetic heterogeneity, comprehensive analysis of genes like STRC, encoding stereocilin (DFNB16) is possible. The genetic architecture of the DFNB16 locus is challenging and requires a unique molecular genetic testing assay. The aim of the study is a systematic characterization of the audiological phenotype in DFNB16-positive patients. METHODS: Since 2011, 290 patients with suspicion of inherited hearing loss received a human genetic exploration. Eighty two DFNB1-negative patients advanced to further testing in the DFNB16 locus. STRC-positive patients obtained complete audiological diagnostic workup. Additionally, epidemiological data was collected. RESULTS: Nine of 82 (11%) of the examined patients (mean age 5 yr) showed mutations in the STRC (3 homozygous, 6 compound heterozygous). Aside from a moderate hearing loss in the pure tone audiogram, auditory brainstem response thresholds were 40-50 dB nHL. Otoacoustic emissions were detectable in only one patient. CONCLUSIONS: Examination of the DFNB16-locus should be a standard diagnostic test after negative DFNB1-gene screening result. Notably, DFNB16-associated hearing loss can be audiologically characterized as moderate sensorineural hearing loss in the main speech field with absent otoacoustic emissions. Our study is the first to correlate audiological findings with genetic results in patients with hearing loss due to STRC.