Audie L. Woolley

COCHLEAR IMPLANTATION IN CHILDREN WITH INNER EAR MALFORMATIONS

We performed a case study and intervention study, with follow-up of 1 to 5 years, in 4 children with inner ear malformations who underwent implantation of a multichannel cochlear implant (Nucleus, Cochlear Corporation) at ages 3 to 12 years. Malformations included a common cavity deformity, 2 incomplete partitions, and 1 case of isolated bilateral vestibular aqueduct enlargement. One child had a single-channel implant placed at 3 years of age, and this was exchanged for a 22-channel implant at age 9. One child had her implant placed at age 4.5 years, but due to complications from a cerebrospinal fluid (CSF) leak had the initial implant removed and replaced at age 5 years during repair of the CSF leak. Intraoperative findings included a CSF leak at the time of surgery in 3 patients. One patient contracted bacterial meningitis 7 months postimplantation that was thought to be secondary to acute otitis media in the unoperated ear. Bilateral CSF leaks were noted in the middle ear by a lumbar puncture radionuclide and fluorescein dye study. Successful repair of the CSF leaks and reimplantation of the cochlear implant was carried out in this patient. Mapping and programing of the implant was found to be challenging in each of these patients. All patients demonstrated improved performance after implantation. Two patients demonstrated some open-set speech perception. One patient demonstrates improved use of temporal cues in a structured closed set. One patient has achieved no significant speech recognition at this time, but does have improved sound detection and awareness. Cochlear implantation in children with congenital inner ear abnormalities can be a successful method of rehabilitation. It should be recognized that the postoperative speech perception results may be highly variable among patients, and that intraoperative complications may occur.

The multidisciplinary evaluation and management of cleft lip and palate

Cleft lip with or without a cleft palate (CLP) and cleft palate alone (CPA) are common birth defects, with a combined birth prevalence of about 1 to 2/1,000.1 Affected children have a number of medical issues and potential complications, and therefore require a wide variety of healthcare specialists beyond plastic surgeons and dental specialists. For this reason, the best environment in which to deliver this care is a multidisciplinary cleft clinic (MCC) that features a team of healthcare providers, including audiology, pediatric otolaryngology, speech pathology, occupational/feeding therapy, and genetics. In this setting, the many medical issues that these children face are comprehensively addressed in the most convenient manner, as all the specialists can be seen in a single busy day. Furthermore, the referring primary care provider (PCP) will receive a concise letter that documents the team evaluation, including future management plans and recommendations for therapy. Unfortunately, few papers are available in the literature that review the workings of these clinics. In this paper we will provide such an overview, discussing the management issues for children with CLP/CPA, and how these are addressed by members of the MCC.

Major vascular injuries in children undergoing myringotomy for tube placement

Abstract Major vascular injuries in children undergoing myringotomy are extremely rare events. The anatomic deviations that put the internal carotid artery or jugular bulb at risk for injury are relatively uncommon. HOwever, given the frequency with which myringotomies are performed on children, simple arithmetic implies that dozens of children with an anatomic predisposition for a major vascular complication are unknowingly undergoing this procedure every year. Certainly most are unscathed, however, every now and then, a catastrophe will occur. Does this mean that every child with chronic otitis media shoujld have a preoperative CT scan to rule out a vascular anomaly? No, but the otolaryngologist must be alert to the potential for life-threatening complications in this most “routine” of procedures, and be knowledgeable about the proper management of these complications should they occur. These cases simply reinforce the often overlooked adage that even the most benign procedure is fraught with pitfalls

Operative Management of Choanal Atresia: A 15-Year Experience

OBJECTIVE To analyze factors affecting 15-year surgical outcomes of choanal atresia repair. DESIGN Case series. SETTING Tertiary care pediatric hospital. PATIENTS Between April 17, 1996, and March 23, 2010, a total of 42 patients aged 3 days to 15 years underwent endoscopic or transpalatal choanal atresia repair by our pediatric otolaryngology faculty. MAIN OUTCOME MEASURES Reoperation and restenosis rates, with consideration of effects of mitomycin C therapy, stenting, and postoperative dilation. RESULTS Three of 42 patients were excluded because of inadequate follow-up data; the follow-up time for the remaining 39 patients averaged 6.3 years (range, 1-14.9 years). Excluding 6 patients whose initial repair was performed by other physicians, 31 of 33 patients in whom we performed initial repair had a total of 43 endoscopic surgical procedures (19 patients had unilateral procedures, and 12 patients had bilateral procedures), and the other 2 underwent bilateral transpalatal repair. Of the total 43 sides we operated on endoscopically, 9 sides (21%) required revision surgery, including excision of scar tissue or additional drilling of persistent bony stenosis. No significant difference was observed in the rate of restenosis among cases treated endoscopically with mitomycin C (22 of 43 operative sides, P = .13), with stenting (36 of 43 operative sides, P = .99), or with subsequent dilation (P = .45). When we used stents, they were usually (in 28 of 36 patients) left in place for 15 days or longer. CONCLUSION Our revision rate after initial endoscopic repair of choanal atresia was low and was unaffected by adjuvant mitomycin C therapy or stenting.

Preoperative Temporal Bone Computed Tomography Scan and Its Use in Evaluating the Pediatric Cochlear Implant Candidate

Preoperative computed tomography (CT) scan evaluation of the temporal bones in cochlear implant candidates plays a crucial role in determining candidacy and the side of implantation. The CT scans allow the surgeon to carefully review the anatomy of the inner ear and mastoid cavity in order to predict any potential difficulties or complications that may be encountered during the implant insertion. We retrospectively reviewed 50 preoperative CT scans of the temporal bone in children who have been successfully implanted. In these scans, we assessed the degree of mastoid pneumatization, cochlear anatomy and patency, size of the vestibular aqueduct, cochlear aqueduct, and internal auditory canal. We analyzed our findings and measurements and compared our results with the degree of difficulty noted in the insertion of the implant, the number of electrodes inserted in each case, and the overall complication rate. From this radiographic review, we have created a checklist for cochlear implant surgeons in order to highlight key features that need to be recognized in the temporal bone scan in preoperative evaluation of cochlear implant candidates.

Fixation Methods in Pediatric Cochlear Implants: Retrospective Review of an Evolution of 3 Techniques

Objective. To review 3 techniques of cochlear implant (CI) fixation used by a single surgeon for the fixation of 320 consecutive CIs in a pediatric population and associated complications. Study Design. Case series with chart review. Setting. Tertiary referral children’s hospital. Subjects and Methods. Patients receiving CIs between July 1995 and July 2009 were reviewed. Clinical information obtained included age at implant, implant type, duration of follow-up, method of implant fixation (intraosseous suture ligature, prolene mesh with titanium screws, and a small periosteal pocket with periosteal sutures), and postoperative complications of fixation (migration or extrusion). Results. Three hundred twenty consecutive CIs were reviewed: 64 of which were bilateral (42 staged, 22 concurrent). The median age at implantation was 3.6 years (range, 8 months to 20 years). Manufacturers included Cochlear (223) and Advanced Bionics Corporation (97). Median follow-up was 26 months (range, 1 month to 12.7 years). The intraosseous suture ligation method of fixation was used for 182 CIs. Ninety-eight CIs were fixed using a small piece of polypropylene mesh and titanium screws. Forty implants were secured by using a tight periosteal pocket and placing the suture through the periosteum and soft tissue to collar the receiver in a modified well. No complications of device migration or extrusion were noted, nor were there any intracranial complications. Device failure occurred in 13 (4%) patients requiring explantation and reimplantation, but these were unrelated to surgical technique or fixation. Conclusions. This study illustrates that with an evolution toward less invasive and less complex methods of fixation, there has not been an associated increase in fixation-related complications.

The use of genetic testing in the evaluation of hearing impairment in a child

Purpose of review To review the role of genetic testing in the evaluation of hearing impairment in children. Recent findings The introduction of genetic testing has greatly enhanced the evaluation of deafness and hearing impairment in children. It can save time and money as well as providing patients, their families, and their physicians with important information; however, this testing is different from the medical testing that pediatricians typically order. Summary For patients and families to realize the benefits of genetic testing it must be done early in the evaluation process and must be accompanied by appropriate pretest and posttest counseling.

Foreign body simulating a hard palate lesion in a child

Foreign bodies embedded in the palate are unusual findings and can occasionally mimic other types of oral cavity pathology. We present a case report of a foreign body in the palate of a infant girl which mimicked an odontogenic cyst. A review of the literature and a brief discussion of the differential diagnoses are included.

The Management of Myringotomy Tubes in Pediatric Cochlear Implant Recipients

OBJECTIVE To investigate the impact of myringotomy tubes (MTs) on outcomes for pediatric cochlear implant (CI) recipients. DESIGN Retrospective case-control chart review. SETTING Tertiary care pediatric hospital. PATIENTS Sixty-two patients received an MT before CI (mean [SD] age at initial CI, 3.20 [2.45] years). Seventy-eight ears received CIs and MTs. INTERVENTION The MTs were removed and allowed to extrude before CI (59% [n = 46]) or kept in place until CI (41% [n = 32]). MAIN OUTCOME MEASURES Otorrhea, persistent tympanic membrane (TM) perforation, and need for additional procedures were recorded. Statistical analysis was performed with the Fisher exact test. RESULTS Forty ears (51%) required more than 1 set of MTs. Ten ears (22%) in which the MTs were removed before CI required a separate MT after CI compared with 6 ears (19%) in which the MTs remained in place until CI (P = .78). The MTs that were present during CI were either removed with myringoplasty (31% [n = 10]) or retained after surgery (69% [n = 22]). All TMs in which the tubes were removed before or during CI healed. There were 3 persistent TM perforations that required surgical treatment. There were no cases of meningitis and no removals of CIs because of infection. CONCLUSIONS Myringotomy tubes do not appear to adversely affect the final outcomes of pediatric CI recipients and can be managed similarly to MTs in other otitis media-prone children. They may be left in place in children who continue to experience recurrent acute otitis media or removed in children who no longer need them.

Familial transmission of oculoauriculovertebral spectrum (Goldenhar syndrome) is not due to mutations in either EYA1 or SALL1.

Familial Transmission of Oculoauriculovertebral Spectrum (Goldenhar Syndrome) Is Not Due to Mutations in Either EYA1 or SALL1 Kara Goodin, Sandra Prucka, Audie L. Woolley, Juergen Kohlhase, Richard J.H. Smith, John Grant, and Nathaniel H. Robin* Department of Genetics, University of Alabama, Birmingham, Alabama Department of Pediatrics, University of Alabama, Birmingham, Alabama University of Freiburg, Freiburg, Germany Department of Otolaryngology, University of Iowa, Iowa City, Iowa Department of Surgery, University of Alabama, Birmingham, Alabama