Hypothesis: To study variations in human cochlea anatomy with potential implications for cochlear implantation surgery. Background: A comprehension of the anatomic variations of the human cochlea is essential for understanding the degree of surgical trauma induced by inserting various electrode arrays in cochlear implantation surgery. Variations in anatomy may also limit the potential for performing hearing preservation. Methods: We studied 73 archival, nonselected, adult, corrosion casts of human inner ears. Anatomic reference points were constructed from photographic reproductions taken at different angles, and various dimensions were assessed using planimetry. Anatomic variants with particular clinical/surgical interests were pinpointed. Results: Results showed that the human cochlea is individually shaped, varying greatly in dimensions ("fingerprint"). The outer cochlear wall length ranged from 38.6 to 45.6 mm with a mean length of 42.0 mm. The first turn represented 53% of the total length and ranged from 20.3 to 24.3 mm. The number of quadrants varied from slightly more than 8 to 12. The facial nerve canal ran in close proximity to the upper first turn explaining facial nerve excitement during stimulation of electrodes in this region in some instances. The internal diameter (height) of the cochlear tube in the first turn varied broadly (1.6-2.6 mm), occasionally with limited space for conventional implants. Conclusion: The human cochlea exhibits extensive anatomic variations. These variations will influence the location of cochlear implant arrays and affect the potential of hearing preservation surgery. Our results may explain the surgeon's difficulties sometimes to insert electrode arrays even in so-called "normal" cochleae.

Decision-making on treatment and (re)habilitation needs to be based on clinical expertise and scientific evidence. Research evidence for the impact of permanent unilateral hearing impairment (UHI) on children's development has been mixed and, in some of the reports, based on fairly small, heterogeneous samples. Additionally, treatment provided has been highly variable, ranging from no action taken or watchful waiting up to single-sided cochlear implantation. Published information about the effects of treatment has also been heterogeneous. Moreover, earlier reviews and meta-analyses published on the impact of UHI on children's development have generally focused on select areas of development. This systematic review aimed to summarize the impact of children's congenital or early onset unilateral hearing impairment on listening and auditory skills, communication, speech and language development, cognitive development, educational achievements, psycho-social development, and quality of life. Literature searches were performed to identify reports published from inception to February 16th, 2018 with the main electronic bibliographic databases in medicine, psychology, education, and speech and hearing sciences as the data sources. PubMed, CINALH, ERIC, LLBA, PsychINFO, and ISI Web of Science were searched for unilateral hearing impairment with its synonyms and consequences of congenital or early onset unilateral hearing impairment. Eligible were articles written in English, German, or Swedish on permanent unilateral hearing impairments that are congenital or with onset before three years of age. Hearing impairment had to be of at least a moderate degree with PTA ≥40 dB averaged over frequencies 0.5 to 2 or 0.5-4 kHz, hearing in the contralateral ear had to have PTA0.5-2 kHz or PTA0.5-4 kHz ≤ 20 dB, and consequences of unilateral hearing impairment needed to be reported in an unanimously defined population in at least one of the areas the review focused on. Four researchers independently screened 1618 abstracts and 566 full-text articles for evaluation of study eligibility. Eligible full-text articles were then reviewed to summarize the results and assess the quality of evidence. Additionally, data from 13 eligible case and multi-case studies, each having less than 10 participants, were extracted to summarize their results. Quality assessment of evidence was made adapting the Grades of Recommendations, Assessment, Development, and Evaluation (GRADE) process, and reporting of the results adheres to the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) standards. Three articles with the quality of evidence graded as very-low to low, fulfilled the eligibility criteria set. Due to the heterogeneity of the articles, only a descriptive summary could be generated from the results. Unilateral hearing impairment was reported to have a negative impact on preverbal vocalization of infants and on sound localization and speech perception both in quiet and in noise. No high-quality studies of consequences of early-onset UHI in children were found. Inconsistency in assessing and reporting outcomes, the relatively small number of participants, low directness of evidence, and the potential risk of confounding factors in the reviewed studies prevented any definite conclusions. Further well-designed prospective research using larger samples is warranted on this topic.

Studies have limitedly considered children with early-identified unilateral hearing impairment (UHI), and clinical practices regarding screening, diagnostics and habilitation in this group are rarely documented. In this study, routines for newborns with UHI from screening to diagnostics and habilitation were explored in Norway, Sweden and Finland. An online survey was sent to hospitals responsible for the hearing diagnostics of children requesting information about their practices regarding congenital UHI. Responses covered 95% of the children born in the three included countries. The results revealed large variations in ways of organising healthcare and in clinical decisions regarding hearing screening, diagnostics and habilitation of children with congenital UHI. Finally, implications for policy making and research are also discussed.

Objectives: To measure patient satisfaction and correlate to hearing results in partially deaf patients, after hearing preservation cochlear implant surgery with hybrid hearing strategy, and to evaluate the stability of residual low-frequency hearing (LFH) over time. Design: A patient satisfaction survey and a retrospective, 2-year follow-up journal study. Nineteen partially deaf patients intended for hybrid hearing responded to a questionnaire when they had used their cochlear implants for at least a year. The questionnaire consisted of the International Outcome Inventory for Hearing Aids, EuroQol Group visual analogue scale and nine questions about hybrid hearing. Pure-tone audiometry, monosyllables, and hearing in noise test results from the patients’ medical records were evaluated and compared with the results from the patient satisfaction survey. Results: All of the patients were satisfied with their CIs. The mean International Outcome Inventory for Hearing Aids score was 29. The CIs provided a major contribution to the speech comprehension of these partially deaf patients. Two years after surgery, the patients’ mean binaural score on tests of monosyllables was 58%, and the mean signal to noise ratio was 4.6 dB. We observed ongoing deteriorations in the residual hearing of the operated ears that surpassed the deteriorations observed in the contralateral ears. One month after surgery, the LFH loss (125–500 Hz) was 17 dB, and after 2 years, this loss was 24 dB compared with 5 dB in the nonoperated ear. There were no significant correlations between preserved LFH and patient satisfaction or speech perception results. Conclusions: Electric stimulation provided a major contribution to speech comprehension of partially deaf patients. The gain reached in speech understanding widely exceeded the downside in losing some residual hearing. All the patients showed a high degree of satisfaction with their CIs regardless of varying hearing preservation.

This study estimated the permanent hearing loss (PHL) prevalence among newborns and 6-year-olds in the Uppsala region (Sweden), compared how different definitions affected this estimation, and evaluated the outcome of the three hearing screening occasions for children. A retrospective investigation of medical records and audiograms was conducted for children born in 2011-2012. Data extraction was performed when the children had reached 7 years of age. Hearing loss was defined as > 20 dB hearing level in at least one frequency (125-8000 Hz) in one or both ears (clinician's definition). A total of 1385 children were included, and 95 were diagnosed with PHL. The prevalence of bilateral PHL at 6 years was 5.50 per 1000 children based on the clinician's definition, 3.74 per 1000 children based on the World Health Organization (WHO) 2021 definition, and 2.64 per 1000 children based on the WHO 1991 definition. Adding unilateral PHL resulted in a total prevalence of 10.11 (clinician's definition) and 6.16 (WHO 2021) per 1000 children. This study demonstrates the necessity of repeated hearing testing during childhood to identify PHL and suggests a higher prevalence in 6-year-olds than previously reported. The definition of PHL greatly impacted its estimated prevalence.