Characterization of hearing status in children under 3 years of age with cleft palate.

OBJECTIVES: To describe the hearing thresholds of children under 3 years of age with cleft palate with or without cleft lip. METHODS: This retrospective study from a tertiary care children's hospital included children with cleft palate ± cleft lip born between January 01, 2008, and December 31, 2015. All patients who had the initial palate repair at our institution and at least one behavioral audiogram were included. Behavioral audiograms collected either in the sound field or under insert earphones showed results from the better hearing ear. We collected demographic information, cleft type, parent report of newborn hearing screen, syndrome diagnosis, comorbid conditions, and dates of tympanostomy tube placement and palatoplasty. The main outcome measure was hearing status as measured by behavioral audiograms, and, for a subset of patients, brainstem auditory evoked responses (BAER). Results were grouped by test type (behavioral vs BAER) and characteristics at time of testing (pre- or post-palatoplasty, with or without tympanostomy tube placement) to evaluate the range of severity of hearing loss. RESULTS: Three hundred and sixty patients were included in the cohort. The cohort had two groups: Group 1 included 37 patients who had BAER results prior to surgery and behavioral audiogram results post-surgery; Group 2 included 322 patients who had behavioral audiograms pre-surgery (n = 206) and/or post-surgery (n = 215). The median age of patients at time of BAER in Group 1 was 3.00 months [IQR: 1.00,3.75]; pre-surgery BAER results showed mild (45.9%, n = 17) or moderate hearing loss (29.7%, n = 11) by this age. Patients in Group 2 with pre-surgery behavioral audiograms had a median age of 12.00 months [IQR: 11.00, 14.00] at time of audiogram. Most patients had a mild (33.5%, n = 69) or moderate hearing loss (31.1%, n = 64). Post-surgery, patients had a median age of 13.00 months [IQR: 11.00, 15.00] at time of hearing assessment and typically had normal hearing (86.0%, n = 185). In most patients, hearing improved after palate repair with concurrent tympanostomy tube placement. CONCLUSIONS: Most patients with cleft palate ± cleft lip had evidence of mild or moderate hearing loss prior to cleft palate repair, and hearing loss was evident from early infancy. Hearing improved after concurrent tympanostomy tube placement and palatoplasty. Further research is needed to understand the clinical significance of untreated transient hearing loss during the first year of life in this patient population.

Reliability of Measuring Insertion Depth in Cochlear Implanted Infants and Children Using Cochlear View Radiography.

OBJECTIVES: Cochlear implant depth of insertion affects audiologic outcomes and can be measured in adults using plain films obtained in the "cochlear view." The objective of this study was to assess interrater and intrarater reliability of measuring depth of insertion using cochlear view radiography. STUDY DESIGN: Prospective, observational. SETTING: Tertiary referral pediatric hospital. SUBJECTS AND METHODS: Patients aged 11 months to 20 years (median, 4 years; interquartile range [IQR], 1-8 years) undergoing cochlear implantation at our institution were studied over 1 year. Children underwent cochlear view imaging on postoperative day 1. Films were deidentified and 1 image per ear was selected. Two cochlear implant surgeons and 2 radiologists evaluated each image and determined angular depth of insertion. Images were re-reviewed 6 weeks later by all raters. Inter- and intrarater reliability were calculated with intraclass correlation coefficients (ICCs). RESULTS: Fifty-seven ears were imaged from 42 children. Forty-nine ears (86%) had successful cochlear view x-rays. Median angular depth of insertion was 381° (minimum, 272°; maximum, 450°; IQR, 360°-395°) during the first round of measurement. Measurements of the same images reviewed 6 weeks later showed median depth of insertion of 382° (minimum, 272°; maximum, 449°; IQR, 360°-397°). Interrater and intrarater reliability ICCs ranged between 0.81 and 0.96, indicating excellent reliability. CONCLUSIONS: Postoperative cochlear view radiography is a reliable tool for measurement of cochlear implant depth of insertion in infants and children. Further studies are needed to determine reliability of intraoperatively obtained cochlear view radiographs in this population.

Auditory comprehension outcomes in children who receive a cochlear implant before 12 months of age.

OBJECTIVE: The U.S. Food and Drug Administration guidelines for cochlear implantation (CI) include age greater than 12 months. Studies have suggested that implantation in children younger than 12 months with congenital deafness may be associated with better spoken language outcomes. Compare auditory comprehension (AC) outcomes for children with congenital deafness who received CI less than 12 months of age to those implanted at 12 to 24 months of age. METHODS: Retrospective review of prospectively collected data in consecutively implanted patients under 2 years of age who received CI and had post-CI Preschool Language Scale (PLS)-AC scores. Receptive language was assessed with the AC subtest of the PLS. Patients without pre-CI PLS-AC scores were excluded. The association between age at implantation and post-CI PLS-AC scores up to 2 years after CI surgery was modeled using a linear mixed-effects model. Time from CI surgery, number of implants, risk factors for language delay, pre-CI PLS-AC score, and sex were included in the model. Patients implanted less than 12 months of age were compared to those implanted between 12 and 24 months. RESULTS: Twenty-nine patients who had CI surgery by 12 months and 82 who had CI surgery between 12 and 24 months were included in the analysis. Younger age at implantation and better pre-CI PLS-AC scores were significantly associated with better post-CI PLS-AC scores. CONCLUSION: Cochlear implantation in children with congenital deafness less than 12 months of age was associated with better PLS-AC than in children implanted over 12 months of age up to 2 years after implantation. LEVEL OF EVIDENCE: 4 Laryngoscope, 130:776-781, 2020.