Beatty, Charles W.

Although relatively rare, antrochoanal polyps represent one of the most common types of polyp diagnosed in children without cystic fibrosis. In an attempt to better define this entity and discuss treatment options, the histories and operative reports of all 25 children (aged 17 years and younger) diagnosed with an antrochoanal polyp between 1970 and 1997 at our institution were reviewed. All 25 children complained of nasal obstruction on presentation; other presenting symptoms included rhinorrhea (48%), snoring (36%), and mouth breathing (32%). All 25 patients were noted to have a mass in the nose on examination, and 16 (64%) also had a mass noted in the nasopharynx. All but 1 patient underwent surgical removal of the polyp: intranasal avulsion only, 2 patients; Caldwell-Luc procedures, 10 patients; intranasal procedures, 8 patients; and endoscopic procedures, 4 patients. Mean time to first recurrence was 44.5 months. Seven patients (29%) who underwent excision at our institution experienced recurrence, 3 after endoscopic procedures and 4 after intranasal procedures (with or without Caldwell-Luc; 1 of these patients had a second recurrence). Complications were unusual and included bleeding after pack removal (8.3%) and facial paresthesias (10%). Follow-up ranged from 2 days to almost 27 years and was aided by telephone interviews. We conclude that surgical treatment of these lesions is safe and effective. Endoscopic removal may result in a higher recurrence rate.

A review of 85 Mayo Clinic patients with carcinoma of the nasal septum revealed squamous cell carcinoma (58 patients) to be the predominant cell type, with adenocarcinoma (12 patients) and malignant melanoma (7 patients) being next in frequency. Twenty‐five (29%) of the 85 patients had metastatic disease. Twenty percent (17) of the patients had another malignancy at some time during their lives. The study suggests that tobacco smoking may have a role in the etiology of squamous cell carcinomas of the nasal septum. In most patients, wide surgical excision was the initial choice of 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

Purpose The purpose of this research note is to identify and prioritize diseases important for detection in adult hearing health care delivery systems. Method Through literature review and expert consultation, the authors identified 195 diseases likely to occur in adults complaining of hearing loss. Five neurotologists rated the importance of disease on 3 dimensions related to the necessity of detection prior to adult hearing aid fitting. Results Ratings of adverse health consequences, diagnostic difficulty, and presence of nonotologic symptoms associated with these diseases resulted in the identification of 104 diseases potentially important for detection prior to adult hearing aid fitting. Conclusions Current and evolving health care delivery systems, including direct-to-consumer sales, involve inconsistent means of disease detection vigilance prior to device fitting. The first steps in determining the safety of these different delivery methods are to identify and prioritize which diseases present the greatest risk for poor health outcomes and, thus, should be detected in hearing health care delivery systems. Here the authors have developed a novel multidimensional rating system to rank disease importance. The rankings can be used to evaluate the effectiveness of alternative detection methods and to inform public health policy. The authors are currently using this information to validate a consumer questionnaire designed to accurately identify when pre- fitting medical evaluations should be required for hearing aid patients.

The need to preserve hearing during acoustic neuroma removal has rekindled interest in labyrinth‐sparing procedures. This review of 11 years' experience with the retrosigmoid approach to acoustic neuroma removal includes 335 procedures in 332 patients. There were no intraoperative deaths; two patients died in the postoperative period. The facial nerve was preserved in 86.3% of procedures, and auditory function was preserved in 45 procedures (34% of those tumors were 2 cm or smaller). Postoperative complications occurred in 101 procedures, the most common being cerebrospinal fluid otorhinorrhea in 40 cases; 25 of these required secondary surgery. Meningitis occurred 16 times and aspiration 8 times; all other complications were less frequent. Tumor removal was incomplete in eight procedures; In only one of these cases has tumor recurred. The six recurrences usually were identified 5 or more years postoperatively. This has prompted us to follow patients for 7 years postoperatively. Major changes in our management include the use of the supine position and of electrophysiologic monitoring. Advantages of the approach are: (1) wide access to the tumor, (2) applicability to all tumor sizes, (3) potential to preserve facial and auditory function in all cases, and (4) ability to change procedure without sacrificing labyrinth. This review confirms our confidence in this approach to acoustic neuroma removal.

From 1966 through 1983, 408 patients underwent primary removal of an acoustic neuroma at the Mayo Clinic. Of these, 21 had preoperative pure tone hearing levels of 25 dB hearing loss or better at 500, 1000, and 2000 Hz, which for this study was defined as normal pure tone hearing. Fourteen patients (67%) had dysequilibrium and 13 (62%) had subjective hearing impairment. Nystagmus was the most common physical finding. Five patients (23%) had completely symmetric pure tone hearing levels through all frequencies tested. The mean speech discrimination score was 90%. Mean tumor size was 2.4 cm. Brain stem evoked response audiometry was the most helpful of the special tests in the evaluation of these patients with normal pure tone hearing levels. Our results suggest that 5% of patients with acoustic neuromas have normal pure tone hearing levels. A careful history, a thorough physical examination, and an appropriate selection of tests will identify these patients.

Cochlear and neural auditory function for a series of 30 patients with acoustic neuroma was assessed pre- and postoperatively by transiently evoked otoacoustic emission (TEOAE), auditory brain stem response (ABR), and acoustic reflex measures. Employing a retrosigmoid surgical approach, physical integrity of the acoustic nerve was preserved for 22 patients. TEOAEs were observed preoperatively for 13 patients and postoperatively for 6 of them. Postoperative neural function remained unchanged or improved following surgery for 7 patients, and cochlear function decreased. TEOAE, ABR, and acoustic reflex measurements were considered valuable in providing information about site of lesion and probable mechanisms of injury associated with tumor removal.

Audiologic test results were reviewed for 30 patients whose hearing was preserved following eighth nerve tumor surgery. Speech reception thresholds, 4,000-Hz thresholds, acoustic reflexes, and ABR wave I absolute latencies were generally unchanged or poorer following surgery; word recognition scores were essentially unchanged. By contrast, ABR waves III and V, and the I–III and I–V interpeak intervals, were generally improved postoperatively, including several patients who had clear waves following surgery despite absent preoperative responses. The improved ABR tracings likely indicate increased neural synchrony due to reduced pressure on the cochlear nerve following surgery, whereas the reduced hearing sensitivity probably reflects cochlear damage coincident to the surgery.

Object The aim in this study was to describe the clinical presentation, differential diagnosis, and risk for neurofibromatosis Type 2 (NF2) in pediatric patients presenting with cerebellopontine angle (CPA) and internal auditory canal (IAC) tumors. Methods The authors conducted a retrospective study at a tertiary care academic referral center. All patients with an age ≤ 18 years who had presented with an extraaxial CPA or IAC tumor between 1987 and 2012 were included in the study cohort. Data regarding symptoms, diagnosis, tumor characteristics, and NF2 status were collected and analyzed. Results Sixty patients (55% female, 45% male) harboring 87 tumors were identified. The mean age at diagnosis was 12.8 years (median 14.0 years, range 0.9–18.9 years). Schwannomas were the most commonly identified lesions (57 of 87 tumors, including 52 vestibular, 3 facial, and 2 trigeminal schwannomas), followed by meningiomas (5 of 87) and epidermoid cysts (4 of 87). Six malignant tumors were diagnosed, including small-cell sarcoma, squamous cell carcinoma, malignant meningioma, atypical rhabdoid-teratoid tumor, endolymphatic sac tumor, and malignant ganglioglioma. Headache, followed by hearing loss and imbalance, was the most common presenting symptom, whereas dysphagia, otalgia, and facial pain were uncommon. Neurofibromatosis Type 2 was diagnosed in 20 (61%) of 33 patients with vestibular schwannoma (VS), while the other 13 patients (39%) had sporadic tumors. Nineteen of the 20 patients with NF2 met the diagnostic criteria for that disorder on initial presentation, and 15 of them presented with bilateral VS. At the last follow-up, 19 of the 20 patients subsequently diagnosed with NF2 demonstrated bilateral VSs, whereas 1 patient with a unilateral VS and multiple other NF2-associated tumors has yet to demonstrate a contralateral VS to date. Only 1 patient presenting with an isolated unilateral VS and no family history of NF2 demonstrated a contralateral VS on subsequent radiological screening. Conclusions Cerebellopontine angle and IAC tumors in the pediatric population are rare. There are several noteworthy differences between the adult and pediatric populations harboring these lesions. While VS is the most common pathology in both age groups, the lesion was found in only 60% of the pediatric patients in the present study. Unlike in adults, VSs in the pediatric population were associated with NF2 in over one-half of all cases. The majority of pediatric patients with NF2 fulfilled the diagnostic criteria at initial presentation; however, approximately 7% of patients presenting with a seemingly sporadic (no family history of NF2) unilateral VS will meet the criteria for NF2 later in life. Finally, malignancies account for a significantly higher percentage (10%) of cases among pediatric patients. These findings underscore the importance of early screening and close radiological follow-up and may be helpful in patient counseling.