DeJong, Melissa D.

Objective: To remove barriers and improve access for patients seeking cochlear implantation. Study Design: Prospective quality improvement study at a large tertiary academic care center. Methods: A Kaizen quality improvement model was applied over the course of a year. Four weeklong meetings were used to identify areas for improvement and remediation. Data were collected at baseline, 90-days, and 1-year postcompletion of the project. Outcome measures included lead times, defined as the wait time between first contact with the clinic and the first appointment, and the wait time between surgery and activation, and cycle times defined as the total test time needed to determine cochlear implant candidacy, and total time needed to complete initial activation. The total inventory kept as clinic stock was also calculated Results: Kaizen team members collected data for each outcome measure. After the Kaizen principles were applied, the following outcomes were observed: median lead times between first contact with the clinic to candidacy testing, candidacy testing to surgery, and surgery to activation of the implant remained stable from baseline to 1-year follow-up. Median cycle time for candidacy testing decreased from 7.3 hours at baseline to 3.0 hours at 1-year follow-up. Cycle times for initial activation of the device did not change over time. The total inventory of clinic stock was reduced by 31%. Conclusions: Though outcomes for lead and cycle times varied, implementation of Kaizen principles was found to be an effective method for completing this quality improvement project at a large cochlear implant program overall. Level of Evidence: 3a

Objective: To describe a novel case of congenital profound bilateral sensorineural hearing loss in a patient with bilateral nodular internal auditory canal and labyrinthine enhancement and temporal bone dysplasia. Patients: A 76-day-old female was referred to the authors’ center for evaluation of congenital deafness. Behavioral observations and objective audiometric evaluation demonstrated bilateral profound sensorineural hearing loss and a comprehensive multidisciplinary evaluation identified compound heterozygous pathogenic variants in MYO7A , a gene associated with Usher Syndrome Type 1B or DFNB2. Computed tomography and contrast-enhanced magnetic resonance imaging studies demonstrated bilateral temporal bone anomalies with unique middle and inner ear malformations, as well as unique contrast enhancement in the membranous labyrinth, internal auditory canals, and cranial nerves, which have not been previously described with MYO7A variants. Interventions: Given the potential risk for progressive bilateral labyrinthitis ossificans, bilateral simultaneous cochlear implantation was performed at 4 months of age. Main Outcome Measures: Subsequent audiologic follow up after implantation shows significantly improved access to auditory information and increased vocalizations. At last testing, speech and language skills for both receptive and expressive language abilities were found to be commensurate with her chronological age. Conclusion: We report a novel presentation and imaging findings of congenital bilateral profound sensorineural hearing loss in a patient with nodular internal auditory canal and labyrinthine enhancement and coexisting inner ear dysplasia. Despite the multiple radiologic abnormalities, the patient has demonstrated good benefit from cochlear implantation. Future study of rare variants of congenital deafness, such as this, is critical toward defining new disease processes and determining optimal treatment.

Objectives/HypothesisThis study was designed to examine speech recognition and self‐perceived health‐related quality of life (HRQoL) received from cochlear implantation among a cohort of adults and children with a short duration of unilateral hearing loss greater than 6 months, but less than 2 years.Study DesignSingle‐subject repeated measures prospective study.MethodsThis study assessed changes in speech recognition and self‐perceived quality of life by prospectively analyzing data at the preoperative evaluation and at the 3‐month and 6‐month postactivation intervals. Measurement tools included Medical Outcomes Study Questionnaire Short Form 36, Nijmegen Cochlear Implant Questionnaire, Speech Spatial and Qualities of Hearing–Comparative, and speech recognition measures in quiet and in noise.ResultsResults indicated significant improvement in speech recognition, both in quiet and noise. Quality‐of‐life measures showed a significant increase in self‐perceived benefit with disease‐specific instruments, but remained constant with a generic HRQoL instrument.ConclusionsCochlear implantation was a successful intervention for improved hearing in quiet and noise, and a self‐perceived benefit for this group of adults and children with a short duration of unilateral hearing loss.Level of Evidence4 Laryngoscope, 127:1683–1688, 2017